bromochloroacetic-acid and Rhabdomyosarcoma

Topics: Chromosomal Proteins, Non-Histone; Diagnosis, Differential; DNA-Binding Proteins; Follow-Up Studies; Humans; Infant; Infant, Newborn; Keratins; Male; Mucin-1; Phosphopyruvate Hydratase; Rhabdoid Tumor; Rhabdomyosarcoma; S100 Proteins; Sarcoma; Sarcoma, Clear Cell; Skin Neoplasms; SMARCB1 Protein; Transcription Factors; Vimentin

Topics: Adenocarcinoma; Biomarkers, Tumor; Brain Neoplasms; Breast Neoplasms; Carcinoma, Hepatocellular; Carcinoma, Neuroendocrine; Carcinoma, Small Cell; Carcinoma, Squamous Cell; Cell Differentiation; Cytogenetic Analysis; Head and Neck Neoplasms; Humans; Immunohistochemistry; Keratins; Liver Neoplasms; Lymphatic Metastasis; Magnetic Resonance Imaging; Mesothelioma; Neoplasm Metastasis; Neoplasms, Unknown Primary; Peritoneal Neoplasms; Positron-Emission Tomography; Prognosis; Rhabdomyosarcoma; Tomography, X-Ray Computed; Urinary Bladder Neoplasms

True malignant mixed tumor (carcinosarcoma) of the salivary gland is an extremely rare tumor. By definition, it is composed of both malignant epithelial and malignant mesenchymal elements. The most common type of the former is squamous cell carcinoma or adenocarcinoma and the most common type of the latter is chondrosarcoma, followed in frequency by fibrosarcoma, leiomyosarcoma, osteosarcoma, and in rare instances liposarcoma. We report a case of true malignant mixed tumor of the parotid gland in association with a pleomorphic adenoma in a 47-year-old man that contained a very unusual type of malignant mesenchymal component, rhabdomyosarcoma. Cytologic and histologic features and immunohistochemical results are presented. In addition, the literature is reviewed, and the possible histogenesis and pathogenesis of malignant mixed tumor of the salivary gland are briefly discussed.

Topics: Actins; Carcinosarcoma; Humans; Immunohistochemistry; Keratins; Male; Mesoderm; Middle Aged; Parotid Neoplasms; Rhabdomyosarcoma

Three cases of neuroendocrine carcinoma showing skeletal muscle differentiation are presented. The tumors were located in the skin and subcutaneous tissue, the urinary bladder, and the nasal cavity respectively, and were composed by two cell types admixed intimately with each other. One cell type had features identical to those seen in conventional small cell neuroendocrine carcinoma, including scanty cytoplasm, round nuclei with fine granular chromatin, immunohistochemical reactivity for neuron-specific enolase, chromogranin and cytokeratins, and electron-dense granules on ultrastructural examination. The second cell type was either plasmacytoid or elongated and straplike, with abundant eosinophilic cytoplasm and irregular nuclei with prominent nucleoli. These cells showed immunohistochemical positivity for desmin, sarcomeric actin, myoglobin, and myogenin. They also exhibited ultrastructural evidence of rhabdomyoblastic differentiation in the form of contractile filaments with abortive Z-band formation. An origin from a cell capable of dual differentiation toward neuroendocrine and rhabdomyoblastic elements is postulated for these tumors.

Topics: Aged; Biomarkers, Tumor; Carcinoma, Neuroendocrine; Chromogranins; Cytoplasmic Granules; Fatal Outcome; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Muscle, Skeletal; Nose Neoplasms; Phosphopyruvate Hydratase; Rhabdomyosarcoma; Skin Neoplasms; Urinary Bladder Neoplasms

The term malignant rhabdoid tumor (MRT) has been used to describe a heterogeneous group of neoplasms, having in common distinct so-called "rhabdoid" cytologic features. The recent discovery of a candidate tumor suppressor gene for MRT, INI1 on chromosome (Ch)22q11.2, has re-established this neoplasm as a distinct entity. Malignant rhabdoid tumor may arise either de novo from nonneoplastic cells or through tumor progression from other types of neoplasms. These latter tumors, in which other nonrhabdoid tumor components are identified, may be termed composite MRT. In order to avoid misdiagnosing MRT as other types of neoplasia, one must keep in mind three distinct clinicopathologic features--young age of onset, variable histologic and immunohistochemical patterns, and an aggressive infiltrative character. In difficult cases, cytogenetics, fluorescence in situ hybridization (FISH), and molecular genetic analysis may assist in diagnosing MRT.

Topics: Child, Preschool; Humans; Infant; Keratins; Kidney Neoplasms; Neoplasm Invasiveness; Rhabdoid Tumor; Rhabdomyosarcoma; Vimentin

Topics: Adolescent; Biomarkers, Tumor; Carcinoma, Squamous Cell; Diagnosis, Differential; Diagnostic Errors; Gingival Neoplasms; Humans; Keratins; Male; Microscopy, Electron; Myosins; Neoplasm Proteins; Rhabdomyosarcoma

Malignant mixed mesodermal tumors (malignant mixed Müllerian tumors [MMMT]) occur rarely in extragenital sites.. The authors analyzed the clinical, pathologic, and immunohistochemical features of three cases of primary MMMT of the female peritoneum.. The neoplasms occurred in 60-, 64- and 84-year-old women and arose from pelvic peritoneum. Two patients died with disseminated disease 8 and 24 months postoperatively. The third died of cardiac failure 12 months postoperatively with questionable metastatic disease. Microscopically, two tumors were of the heterologous type, containing foci of rhabdomyosarcomatous (case 1) and chondrosarcomatous (case 3) differentiation. Immunohistochemically, coexpression of keratin and vimentin was observed focally in both carcinomatous and sarcomatous components in all three neoplasms, whereas coexpression of low molecular weight cytokeratin, vimentin and actin was observed focally in case 2. Rhabdomyosarcomatous areas were positive with desmin and actin, and chondrosarcomatous areas for S-100 protein. Both epithelial and mesenchymal components were positive for alpha-1 antichymotrypsin in all cases.. On the basis of the present cases and a review of 15 reports from the literature, primary MMMT of the female peritoneum proved to be a rare but highly malignant neoplasm occurring in elderly postmenopausal women. Of 15 patients with available follow-up, 12 died with disease, mostly within 1 year, regardless of the initial tumor stage, histology (homologous versus heterologous MMMT) or treatments attempted. The tumor developed within pelvic peritoneum in half the cases. Histogenetically, peritoneal MMMT are thought to represent "metaplastic" carcinomas originating from the secondary Müllerian system.

Topics: Actins; Aged; Aged, 80 and over; alpha 1-Antichymotrypsin; Chondrosarcoma; Desmin; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Mixed Tumor, Mullerian; Peritoneal Neoplasms; Prognosis; Rhabdomyosarcoma; S100 Proteins; Vimentin

Most mammalian nucleated cells contain a cytoplasmic fibril system called intermediate filaments. Unlike other cytoskeletal proteins, the subunit proteins of intermediate filaments show a remarkable cell-type-specificity in their expression: mesenchymal, muscle, epithelial, glial and neuronal cells containing each their cell type specific filaments. In this review we discuss the possibilities to use antibodies to these filaments in the diagnosis and histogenetic analysis of human tumors. This approach is based on findings which indicate that these filaments retain their cell-type specific expression also in tumors.

Topics: Antibodies; Carcinoma; Cytoskeleton; Desmin; Glial Fibrillary Acidic Protein; Glioma; Humans; Intermediate Filaments; Keratins; Mesothelioma; Neoplasms; Rhabdomyosarcoma; Sarcoma; Vimentin

Topics: Antibodies, Monoclonal; Carcinoma, Small Cell; Cell Line; Desmosomes; Humans; Intermediate Filament Proteins; Keratins; Lung Neoplasms; Neoplasms; Neuroectodermal Tumors, Primitive, Peripheral; Rhabdomyosarcoma

The authors assessed a panel of immunohistochemical stains against 109 pediatric solid tumors, primarily rhabdomyosarcomas, under the auspices of the Intergroup Rhabdomyosarcoma Study. Fresh tumor tissue received from participating organizations was divided into portions that were either frozen or fixed in formalin, alcohol, or B5. Immunostaining was performed by the avidin-biotin complex method using monoclonal antibodies to desmin, neurofilaments, vimentin, cytokeratin, and leukocyte common antigen on cryostat sections. Tissue was also embedded in paraffin and stained with antimuscle-specific actin (MSA) and polyclonal antibodies to desmin, creatine kinase M subunit (CKM), myoglobin, and neuron-specific enolase (NSE). Antidesmin staining of cryostat sections was the most sensitive indicator of rhabdomyosarcoma (58 of 62 specimens positive). Results with this reagent in alcohol-fixed and formalin-fixed tissue were similar (46 of 56 positive versus 43 of 56 positive, respectively) and comparable with results with anti-MSA in formalin-fixed tissue (43 of 55 positive). However, the proportion of cells stained by antidesmin was higher in alcohol-fixed tissue than in formalin-fixed tissue. Staining with antimyoglobin and anti-CKM was much less satisfactory, with positivity rates of 17 of 37 and 11 of 57, respectively, in formalin-fixed rhabdomyosarcomas. Immunostaining of muscle markers revealed evidence of myogenesis in six undifferentiated sarcomas and in two sarcomas with inadequate histologic study on hematoxylin-eosin-stained sections. However, positivity was also noticed in samples of fibromatosis, Wilms' tumor, ectomesenchyoma, peripheral primitive neuroectodermal tumor, renal rhabdoid tumor, myositis ossificans, malignant fibrous histiocytoma, and embryonal sarcoma of the liver. The authors conclude that combined use of antidesmin and anti-MSA enhances the diagnosis of childhood sarcomas, especially when employed with other techniques such as electron microscopic study.

Topics: Actins; Biomarkers, Tumor; Child; Cytoplasm; Desmin; Fixatives; Humans; Immunoenzyme Techniques; Keratins; Myoglobin; Phosphopyruvate Hydratase; Prospective Studies; Rhabdomyosarcoma; Sarcoma; Staining and Labeling; Vimentin

We report the case of a dedifferentiated solitary fibrous tumor with heterologous rhabdomyosarcomatous differentiation in a 74-year-old male presenting with a rapidly growing, large soft tissue tumoral mass in the gluteal muscles of the right hip. Dedifferentiation in solitary fibrous tumor had not been recognized until very recently and is an extremely rare phenomenon in this tumor type. In the present case, the diagnosis of dedifferentiated solitary fibrous tumor was difficult because of the absence of areas of conventional solitary fibrous tumor with a predominantly poorly differentiated, anaplastic tumor component in the incision biopsy composed of heterogeneous areas with small blue round cell (Ewing sarcoma-like), rhabdoid, epithelioid, and pleomorphic morphology. Moreover, the "unforeseen" strong patchy to multifocal positivity for cytokeratin AE1/AE3 and desmin made the diagnosis of a dedifferentiated solitary fibrous tumor even more challenging in this case. The morphology (presence of branching thin-walled, hemangiopericytoma-like blood vessels) and the immunohistochemical profile (including STAT6 and GRIA2 positivity) were very useful to differentiate this very challenging case of a cytokeratin-positive dedifferentiated solitary fibrous tumor with heterologous rhabdomyosarcomatous differentiation from a broad list of differential diagnoses.

Topics: Aged; Biopsy; Buttocks; Cell Dedifferentiation; Humans; Immunohistochemistry; Keratins; Male; Receptors, AMPA; Rhabdomyosarcoma; Solitary Fibrous Tumors; STAT6 Transcription Factor

Small round blue-cell tumors (SRBCTs) of soft tissue, which mainly include rhabdomyosarcoma (RMS), synovial sarcoma (SS), and Ewing's sarcoma/peripheral primitive neuroectodermal tumors (EWS/ pPNETs), are malignancies with overlapping morphological and immunohistochemical characteristics. Immunohistochemistry is one of the most prevalent and convenient methods for pathological diagnosis; however, differentiation between SRBCT subtypes in the absence of valid diagnostic markers is still very challenging. The purpose of the present study was to investigate diagnostic immunohistochemistry for subtyping soft tissue SRBCTs.. Seventeen RMS, 25 SS, and 14 EWS/pPNETs were investigated. Reverse transcription RT-PCR and immunohistochemistry was performed to determine a diagnosis. Also, the expression of CD99, FLI1, PAX5, myogenin, and Keratin/EMA was assessed between subtypes. The sensitivity and specificity test was performed to evaluate their diagnostic significance.. The sensitivity and specificity of the target markers were evaluated as follows. FLI1 and CD99 expression displayed strong associations in EWS/pPNETs, with OR (95% CI) and p values of 3.82 (1.23 - 11.94), p = 0.021 and 123.50 (12.63 - infinity), p < 0.001, respectively. Keratin/EMA expression did not support the diagnosis of EWS/pPNETs [OR (95% CI) = 0.06 (0.01 - 0.53), p = 0.011]. Myogenin expression displayed strong association with RMS, with high sensitivity and specificity of 94.1% and 100%, respectively. Membrane expression of CD99 did not support the diagnosis of RMS [OR (95% CI) = 0.09 (0.01 - 0.75), p = 0.026]. Keratin/EMA expression strongly indicated SS [OR (95% CI) = 345.00 (29.44 - infinity), p = 0.00011. A ROC curve value of 0.94 indicated that keratin/EMA expression might be a promising biomarker for SS, while separate expression of FLI1 and CD99 did not support the diagnosis of SS. Similarly, myogenin expression in RMS might be a promising biomarker for RMS with a ROC curve value of 0.97.. Diagnosis of SRBCTs should be based on a comprehensive analysis involving morphology and immunoreactivity to a panel of markers.

Topics: 12E7 Antigen; Adolescent; Adult; Aged; Antigens, CD; Cell Adhesion Molecules; Child; Child, Preschool; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Neuroectodermal Tumors; Odds Ratio; Proto-Oncogene Protein c-fli-1; Reverse Transcriptase Polymerase Chain Reaction; Rhabdomyosarcoma; Sarcoma, Ewing; Sarcoma, Synovial; Sensitivity and Specificity; Soft Tissue Neoplasms; Young Adult

Rhabdomyosarcoma is a malignant mesenchymal tumor with skeletal muscle differentiation. Primary cutaneous rhabdomyosarcoma is rare. We report a series of 11 cases of primary cutaneous rhabdomyosarcoma.. Cases diagnosed as rhabdomyosarcoma arising in the dermis/subcutis with no identified primary tumor elsewhere were retrospectively reviewed. Follow-up was obtained.. The tumors occurred in five children and six adults. The adult subset consisted of pleomorphic, epithelioid and not otherwise specified (NOS) subtypes while the pediatric subset showed alveolar and embryonal subtypes. All cases showed immunohistochemical staining consistent with the diagnosis of rhabdomyosarcoma. Three adult cases showed immunoreactivity for cytokeratins (one pleomorphic, one epithelioid and one NOS.. Primary cutaneous rhabdomyosarcoma shows a bimodal age distribution and male predominance, correlating with rhabdomyosarcoma in deep soft tissue. Follow-up, available on all patients, showed aggressive behavior in both children and adults. Primary cutaneous rhabdomyosarcoma should be considered in the differential diagnosis of tumors with abundant eosinophilic cytoplasm and those with "small round blue cell" morphology. Desmin, myogenin and MYOD1 are a trio of markers with high sensitivity and specificity for primary cutaneous rhabdomyosarcoma. Cytokeratin immunoreactivity in primary cutaneous rhabdomyosarcoma represents a potential diagnostic pitfall in the differential diagnosis with sarcomatoid carcinoma.

Topics: Adolescent; Age Factors; Aged; Aged, 80 and over; Biomarkers, Tumor; Child, Preschool; Cytoplasm; Dermis; Diagnosis, Differential; Female; Follow-Up Studies; Humans; Infant; Keratins; Male; Middle Aged; MyoD Protein; Neoplasm Proteins; Retrospective Studies; Rhabdomyosarcoma; Sex Factors; Skin Neoplasms

Topics: Adenocarcinoma; Carcinoid Tumor; Child; Chromogranin A; Common Bile Duct; Common Bile Duct Neoplasms; Diagnosis, Differential; Duodenum; Gallbladder; Humans; Keratins; Lymphoma; Male; Mucin-1; Neoplasm Invasiveness; Rhabdomyosarcoma; Stomach; Synaptophysin

Adult rhabdomyosarcoma (RMS) in the urinary bladder is rare, and is the subject of case reports and small series. It consists of sheets of small round blue cells with high nuclear cytoplasmic ratio, brisk mitosis and apoptosis. In this study, we reported one case of pure rhabdomyosarcoma and two cases of urothelial carcinomas with extensive rhabdomyosarcomatous differentiation. In addition, their immunohistochemical profile was compared to that of small cell carcinoma of the bladder. Our study showed that sufficient sampling was critical for the diagnosis of urothelial carcinoma with extensive rhabdomyosarcomatous differentiation. As adult RMS in the bladder and urothelial carcinoma with rhabdomyosarcomatous differentiation shared morphological features with small cell carcinoma of the bladder, appropriate immunohistochemical stains were necessary in the differential diagnosis. We showed both rhabdomyosarcoma and rhabdomyosarcomatous areas of the urothelial carcinoma were positive for myogenin, negative for cytokeratin and chromogranin stains. In contrast, small cell carcinoma was positive for cytokeratin, and 7 out of 9 cases were also positive for chromogranin. Both rhabdomyosarcoma and small cell carcinoma could be positive for synaptophysin, a potential pitfall to avoid. In addition, all of the tumors with rhabdomyosarcomatous differentiation were negative for FKHR rearrangement.

Topics: Biomarkers, Tumor; Carcinoma, Small Cell; Cell Differentiation; Chromogranins; Diagnosis, Differential; Female; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Keratins; Male; Middle Aged; Myogenin; Predictive Value of Tests; Rhabdomyosarcoma; RNA-Binding Protein EWS; Synaptophysin; Translocation, Genetic; Urinary Bladder; Urinary Bladder Neoplasms; Urothelium

Only a small number of malignant mesotheliomas with heterologous elements have been described. There are currently no criteria for diagnosis and little data regarding prognosis. We suggest that the term heterologous mesothelioma should be reserved for tumours that show malignant heterologous elements, notably osteosarcomatous, chondrosarcomatous, or rhabdomyoblastic elements but have immunohistochemical and clinical characteristics of mesothelioma. We identified 27 such cases and characterized the clinical and pathological characteristics of these tumours. In our series, 89% originated in the pleura, and 11% from the peritoneal cavity. The median age at diagnosis was 68 years, ranging from 27 to 85 years. Of these cases, 93% occurred in males and 7% in women. Of the 27 mesothelioma cases 16 (59%) were sarcomatoid, 10 (37%) were biphasic, and one was reported as epithelioid; 40% (11 cases) showed osteosarcomatous elements only, 19% showed areas of rhabdomyosarcoma only, 19% contained areas of chondrosarcoma only, and 22% exhibited osteochondromatous elements. Immunohistochemical labelling for cytokeratins was present in the majority of cases. Exposure to asbestos was identified in all the 17 cases for which an exposure history was available (63%). Median survival was 6 months after diagnosis, similar to the survival seen in sarcomatoid mesotheliomas. The differential diagnosis includes primary and secondary pleural sarcomas, including osteosarcomas and chondrosarcomas. Immunohistochemical labelling for cytokeratins is helpful in the distinction, but lack of labelling for cytokeratins in a spindle cell/sarcomatoid tumour does not exclude the diagnosis of mesothelioma, irrespective of the presence of heterologous elements. We suggest that if the anatomical distribution conforms to that of mesothelioma, a diagnosis of heterologous mesothelioma should be made in preference to a diagnosis of primary pleural osteosarcoma or chondrosarcoma, regardless of cytokeratin positivity, as for conventional non-heterologous sarcomatoid mesothelioma.

Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Chondrosarcoma; Diagnosis, Differential; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Mesothelioma; Middle Aged; Osteosarcoma; Peritoneal Neoplasms; Pleural Neoplasms; Rhabdomyosarcoma; Sarcoma; Survival Rate

A 61-year-old man with a sensation of chest compression was admitted to our hospital. He had hemothorax. After drainage with a chest tube, chest CT scan revealed multiple bilateral pulmonary nodules with slight pleural thickening. Open pleural biopsy was performed and the biopsy specimens showed tumor cells with sarcomatoid proliferation, but no definite epithelial pattern. Initial immunohistochemical staining was negative for keratin and carletinin, but positive for desmin, suggesting rhabdomyosarcoma. After supportive care, he died due to progression of the disease. Autopsy revealed extensive invasion suggesting mesothelioma, so the immunohistochemical staining was repeated. Because it revealed patchy staining for keratin and carletinin, this case was diagnosed as sarcomatoid mesothelioma. Differential diagnosis of sarcomatoid mesothelioma or rhabdomyosarcoma is made by immunohistochemical staining, but it is sometimes difficult. For the selection of the best treatment strategy for mesothelioma especially in the early stage, we should be aware of this difficulty.

Topics: Autopsy; Diagnosis, Differential; Fatal Outcome; Hemothorax; Humans; Immunohistochemistry; Keratins; Male; Mesothelioma; Middle Aged; Pleura; Pleural Neoplasms; Rhabdomyosarcoma; Sarcoma

Topics: Adult; Biopsy; Cell Differentiation; Desmin; Diagnosis, Differential; Epithelium; Female; Humans; Immunophenotyping; Keratins; Myogenin; Pelvic Neoplasms; Rhabdomyosarcoma; Sarcoma

There has been no report on useful immunohistological markers for epithelioid sarcoma (ES) so far. The purpose of this study is to evaluate the positivity and specificity of CA125 as a marker for the correct diagnosis of ES.. This study was performed in 11 patients with ES (nine men and two women; distal type: 10 cases; proximal type: one case), 78 patients with other soft tissue tumors and nine with benign granulomas. The other soft tissue tumors consisted of six synovial sarcomas, six clear cell sarcomas, eight leiomyosarcomas, six rhabdomyosarcomas, five malignant peripheral nerve sheath tumors, ten malignant fibrous histiocytomas, 17 desmoid tumors, 14 liposarcomas, six squamous cell carcinomas (cutaneous SCC of the distal extremities), two rheumatoid nodules and seven foreign body granulomas. Immunohistochemical analysis for CA125 was performed for these 89 soft tissue tumors and nine granulomas using a labeled streptavidin biotin method. Immunohistochemical analysis of epithelial membrane antigen, cytokeratin, carcinoembrionic antigen, vimentin and CD34 was performed only for the 11 ES patients.. CA125 was strongly expressed in 10 out of the 11 ES patients. EMA, cytokeratin and vimentin were also positive in all the cases. CEA was positive in two of the 11 patients. Immunohistochemical study in six ES patients showed expression of CD 34. The other 78 soft tissue tumors and nine granulomas did not express CA125.. This study clearly revealed the specificity and positivity of CA125 in ES. These data indicate that CA125 may be a useful tumor marker for diagnosing ES.

Topics: Adolescent; Adult; Antibodies, Monoclonal; Antigens, CD34; Biomarkers, Tumor; CA-125 Antigen; Carcinoembryonic Antigen; Carcinoma, Squamous Cell; Female; Granuloma; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Mucin-1; Rhabdomyosarcoma; Sarcoma; Sarcoma, Clear Cell; Sarcoma, Synovial; Sensitivity and Specificity; Soft Tissue Neoplasms

To extend flow cytometry (FC) to the diagnosis of nonhematopoietic neoplasms, we have developed new flow cytometric assays to identify expression of cytokeratin, epithelial cell adhesion molecule (EpCAM)/epithelial glycoprotein-2, myogenin, and CD99. To validate these assays, we correlated the flow cytometric results with the histologic and immunohistochemical results on paraffin-embedded tissue in a series of 21 cases, including 17 carcinomas, 1 atypical carcinoid, 2 rhabdomyosarcomas, and 1 Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET). Six of 7 assayed carcinomas and the carcinoid were positive for cytoplasmic cytokeratin by the flow cytometric assay. EpCAM was expressed by 11 of 12 carcinomas that were assayed by FC. Both rhabdomyosarcomas expressed myogenin by FC, and the ES/PNET case expressed CD99. Interestingly, the blast-associated antigen CD90 was expressed uniformly on the ES/PNET case and on subsets of cells in the rhabdomyosarcoma and carcinoma cases. Potential applications of the flow cytometric assay to nonhematopoietic neoplasms will include evaluating samples with limited material, monitoring disease persistence and recurrence in patients with previous diagnoses, and making rapid diagnoses in urgent cases.

Topics: 12E7 Antigen; Antigens, CD; Antigens, Neoplasm; Carcinoma; Cell Adhesion Molecules; Cell Lineage; Epithelial Cell Adhesion Molecule; Feasibility Studies; Flow Cytometry; Immunohistochemistry; Immunophenotyping; Keratins; Leukocyte Common Antigens; Myogenin; Neoplasms; Rhabdomyosarcoma; Thy-1 Antigens

A primary malignant peripheral nerve sheath tumor (MPNST) of the pleura that clinically mimicked a malignant mesothelioma in a 57-year-old man is described. Histologically, the tumor had features similar to those described in cases of the so-called epithelioid MPNST. A unique finding in this case was the demonstration of keratin expression in the epithelioid component of the tumor, as well as the presence of rhabdomyoblasts. This is the first example of an MPNST with heterologous elements arising in the pleura. Immunohistochemical and ultrastructural studies were important in differentiating this tumor from other malignancies with sarcomatoid and epithelioid features involving the pleura.

Topics: Actin Cytoskeleton; Biomarkers, Tumor; Cell Differentiation; Diagnosis, Differential; Fatal Outcome; Glycogen; Humans; Keratins; Male; Mesothelioma; Middle Aged; Neoplasm Recurrence, Local; Nerve Sheath Neoplasms; Peripheral Nervous System Neoplasms; Pleural Neoplasms; Rhabdomyosarcoma

Epidemiological studies have implied that Chinese salted fish is a human nasopharyngeal carcinogen. In the present study, 162 Sprague-Dawley rats were randomly assigned to one of four experimental groups. Rats in groups 1 (n = 41) and 3 (n = 40) were exposed to salted fish from birth through the breast feeding period by giving the maternal rats a diet containing 10% and 5% salted fish, respectively, later feeding the rats with pellets containing 10% and 5% of salted fish respectively. In group 2, the rats (n = 41) were given pellets containing 10% of salted fish from 6 weeks of age. Rats in group 4 (n = 40), serving as controls, were only given ordinary pellets. Three rats had nasopharyngeal tumours, 2 from group 1 had a poorly differentiated carcinoma and a squamous cell carcinoma. One rat from group 2 had a squamous cell carcinoma. Four rats had nasal tumours, one fibrosarcoma and one adenocarcinoma were found in rats from group 1. One rhabdomyosarcoma was found in group 2, and one soft tissue sarcoma was found in a rat in group 3. No nasal or nasopharyngeal tumours appeared in the control group. The difference in the occurrence of malignant nasal and nasopharyngeal tumours among the four experimental groups was statistically significant (one tailed p for trend = 0.041). The frequency of tumours appearing in other organs such as the breast, kidney, lung, liver and brain was not significantly different between the salted fish treated groups and the control group.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Adenocarcinoma; Animals; Animals, Newborn; Body Weight; Carcinoma; Carcinoma, Squamous Cell; Desmin; Diet; Female; Fibrosarcoma; Fishes; Food Preservation; Keratins; Male; Nasopharyngeal Neoplasms; Nose Neoplasms; Pregnancy; Rats; Rats, Sprague-Dawley; Rhabdomyosarcoma; Sarcoma, Experimental; Survival Rate; Vimentin

Desmoplastic small round cell tumors (DSRCT) have been only recently identified.. The authors report DSRCT in two pediatric patients (an 8-year-old boy and 12-year-old boy). In both patients, the initial diagnosis was rhabdomyosarcoma. The resistance to standard chemotherapy and radiation therapy prompted the authors to review the initial biopsy specimens and perform complementary immunophenotypic characterization.. These analyses revealed that the tumor cells were strongly positive for keratin epithelial marker antigen, desmin, vimentin, neurospecific enolase, and S100 protein, corresponding to pleomorphic differentiation, characteristic of DSRCT:. The authors suggest that extensive immunohistologic characterization be performed in all cases of small round cell tumors of the abdomen so that the diagnosis of DSRCT is not overlooked. These rare tumors are refractory to chemotherapy, and initial aggressive surgery is warranted.

Topics: Antigens, Neoplasm; Child; Desmin; Diagnosis, Differential; Humans; Keratins; Male; Membrane Glycoproteins; Mucin-1; Neoplasm Invasiveness; Pelvic Neoplasms; Peritoneal Neoplasms; Phosphopyruvate Hydratase; Rhabdomyosarcoma; Vimentin

Rhabdoid tumor of kidney (RTK) is a rare, highly malignant childhood neoplasm of uncertain histogenesis. Several recent studies have described considerable histochemical heterogeneity among cases of RTK, with confusing combinations of epithelial, mesenchymal, myogenous, and neuroepithelial markers in some tumors. The present study characterizes the histology, ultrastructure, histochemistry, cytogenetics, and oncogene expression in a cell line derived from RTK. The surgical specimen, nude mouse xenograft, and cell cultures demonstrated characteristic intermediate filament whorls by electron microscopy and expressed vimentin (diffusely) and cytokeratin (focally, in hyaline cytoplasmic inclusions) without detectable desmin, Thy-1, or epithelial membrane antigen. S-100 protein was absent in the surgical specimen and heterotransplant, and was seen very weakly and focally in the cell cultures. Light microscopic features of cultures were unchanged by several compounds (tissue plasminogen activator, nerve growth factor, cyclic adenosine monophosphate) which induce differentiation of some other pediatric neoplasms. The growth factor requirements of RTK cultures indicate a cell with mesenchymal features. Insulin-like growth factor-2 mRNA was detected in the RTK and in three Wilms' tumors also studied. Unlike most Wilms' tumors, RTK expresses the c-myc rather than the N-myc oncogene.

Topics: alpha 1-Antichymotrypsin; Desmin; Gene Expression; Humans; Immunohistochemistry; Infant, Newborn; Insulin-Like Growth Factor II; Keratins; Kidney Neoplasms; Male; Membrane Glycoproteins; Microscopy, Electron; Mucin-1; Myoglobin; Phenotype; Phosphopyruvate Hydratase; Proto-Oncogene Proteins c-myc; Rhabdomyosarcoma; RNA, Messenger; S100 Proteins; Thymidine; Tritium; Tumor Cells, Cultured; Vimentin

Sixty-three pure mesenchymal tumors of the uterus were studied to explore the value of immunostaining in the diagnosis of unusual mesenchymal tumors encountered in the uterus, some not reported previously. Each tumor was evaluated using a panel of immunostains including actin, desmin, vimentin, S-100 protein, and cytokeratin. The final classification, which incorporated the immunohistochemical findings, resulted in the identification of 33 relatively common pure mesenchymal tumors (13 benign and malignant endometrial stromal tumors and 20 benign and malignant smooth muscle tumors) and 30 uncommon tumors (five leiomyosarcomas with osteoclastic giant cells, two xanthomatous leiomyosarcomas, one melanotic schwannoma, one pure rhabdomyosarcoma, one neurofibroma, five plexiform tumorlets, and 15 combined smooth muscle-stromal tumors). The normal endometrial stroma, present in 14 cases, invariably showed a negative reaction for all antibodies. With rare exceptions, the pure endometrial stromal tumors displayed a negative immunoreaction for all antibodies utilized, while the pure smooth muscle tumors consistently showed a positive reaction for actin. Only the two tumors of neural origin (a neurofibroma and a melanotic schwannoma) reacted with S-100 protein. Immunostaining influenced most the final classification of neoplasms initially interpreted as uterine tumors with a sex-cord stromal pattern, endometrial stromal tumors that diverged from the classic lesions by having a spindle cell component, and intravascular leiomyomas with areas of compact proliferation of small round cells with prominent vascularity. All tumors in these three groups were reclassified as combined smooth muscle-stromal tumors following immunohistochemical studies.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Actins; Aged; Aged, 80 and over; Child, Preschool; Desmin; Female; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Mesenchymoma; Middle Aged; Neurilemmoma; Neurofibroma; Rhabdomyosarcoma; S100 Proteins; Uterine Neoplasms; Vimentin

Twenty-six cases of malignant soft tissue tumors with features similar to renal rhabdoid tumors were identified among approximately 3,000 childhood sarcomas entered on Intergroup Rhabdomyosarcoma Studies I-III. The tumors consisted of polygonal cells with vesicular nuclei and prominent nucleoli and cytoplasmic intermediate filament inclusions as identified by electron microscopy and immunohistochemistry. The growth pattern was predominantly solid or solid-trabecular. Immunohistochemistry showed vimentin, wide spectrum keratin, and epithelial membrane antigen to be the most consistent antigenic phenotypes. Eleven patients were infants less than 1 year of age. The tumors affected predominantly soft tissues of proximal extremities, trunk, and retroperitoneum/pelvis/abdomen. Nineteen patients died within 1 to 82 months (median, 6 months) from the start of treatment. Five patients have survived the disease for 2 to 13 years. When compared with the survival analysis of 991 Intergroup Rhabdomyosarcoma Study II patients, it was obvious that this group of tumors fares very poorly (P less than .001). The tumor belongs to the group of soft tissue neoplasms showing mesenchymal and subtle epithelial differentiation, similar to epithelioid sarcoma. Because of its identifiable histology, site and age distribution, and poor outcome, it warrants a status as an independent entity.

Topics: Adolescent; Antigens, Neoplasm; Brain Neoplasms; Child; Child, Preschool; Female; Humans; Infant; Keratins; Kidney Neoplasms; Male; Membrane Glycoproteins; Mucin-1; Rhabdomyosarcoma; Soft Tissue Neoplasms; Vimentin

Nineteen primary intracranial sarcomas out of a total of about 25,000 brain tumour biopsies are reported. Subtypes included malignant fibrous histiocytoma (6 cases), leiomyosarcoma (3), rhabdomyosarcoma (2), angiosarcoma (2), and one case each of fibrosarcoma, low-grade fibromyxoid sarcoma, malignant ectomesenchymoma, mesenchymal chondrosarcoma, differentiated chondrosarcoma and Ewing's sarcoma. Histological and immunohistochemical features corresponded to those of extracranial sarcomas. Nests of pleomorphic astrocytes mimicking glioma were detected in the five storiform-pleomorphic malignant fibrous histiocytomas. Our results indicate that intracranial sarcomas can be classified like their extracranial counterparts. The low incidence compared with earlier series is related to changes in classification and progress in histogenetic clarification.

Topics: Adult; Aged; Biomarkers, Tumor; Brain Neoplasms; Child; Child, Preschool; Desmin; Female; Glial Fibrillary Acidic Protein; Hemangiosarcoma; Histiocytoma, Benign Fibrous; Humans; Immunohistochemistry; Infant; Keratins; Leiomyosarcoma; Male; Membrane Glycoproteins; Middle Aged; Mucin-1; Rhabdomyosarcoma; Sarcoma; Vimentin

A case is presented of a rhabdomyosarcoma of the oesophagus with a description of the cytology, light microscopy, and immunocytochemical findings and a discussion of spindle cell tumours occurring at this site. Cytologically, large bizarre shaped pleomorphic cells were seen in which desmin was demonstrated in order to confirm the diagnosis after destaining a Papanicolaou stained slide and restaining it with antibody to desmin.

Topics: Carcinoma; Carcinosarcoma; Desmin; Diagnosis, Differential; Esophageal Neoplasms; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Rhabdomyosarcoma; Sarcoma; Staining and Labeling; Vimentin

Ultrasound-guided percutaneous needle biopsy proved to be a reliable and safe method to obtain material for histopathological and immunohistochemical diagnosis prior to treatment in childhood malignancies. A principal tumour identification could be obtained by a combined morphological and phenotypic examination of 38 small-sized tumour biopsy specimens using a fairly limited panel of immunological reagents, including antibodies to leucocyte common antigen (CD 45), certain B- and T-cell markers, various intermediate filaments (cytokeratin, desmin and vimentin), and neuroblastoma cells (UJ 167.11, A2B5, and UJ 13A; the latter recognizes NCAM). Five undifferentiated neuroblastomas were all positive with the neuroblastoma antibodies but negative for the other markers, including vimentin. The negative reactivity for desmin and vimentin was the major immunohistochemical distinction between neuroblastomas and rhabdomyosarcomas. In addition, limited reactivity with the neuroblastoma antibodies was seen in blastematous parts of Wilms' tumour, duct-like structures in a hepatoblastoma, and in tumour cells in a few undifferentiated myelo- and lympho-proliferative lesions. This study shows the importance of a combined evaluation of morphology and the pattern of immunoreactivity employing multiple markers.

Topics: Antibodies, Monoclonal; Antigens, CD; Biomarkers, Tumor; Biopsy, Needle; Child; Child, Preschool; Desmin; Diagnosis, Differential; Fluorescent Antibody Technique; Histocompatibility Antigens; Humans; Immunohistochemistry; Keratins; Leukocyte Common Antigens; Lymphoproliferative Disorders; Neuroblastoma; Rhabdomyosarcoma; Vimentin; Wilms Tumor

A malignant rhabdoid tumor occurring as a primary hepatic neoplasm in a six-month-old white infant is reported. It was treated by an attempt at total resection involving right hepatic lobectomy and by chemotherapy with cis-platinum, VP-16, and Adriamycin. Despite this, recurrence of the tumor resulted in the girl's death within three months. The neoplasm showed typical light microscopic features of malignant rhabdoid tumor as well as filamentous cytoplasmic inclusions by electron microscopic examination and staining for both cytokeratin and vimentin by immunohistochemistry. The classic clinicopathologic features of this tumor support the concept that malignant rhabdoid tumors similar to those of the kidney may occur in extrarenal sites.

Topics: alpha 1-Antitrypsin; Female; Humans; Immunohistochemistry; Infant; Keratins; Liver Neoplasms; Microscopy, Electron; Rhabdomyosarcoma; Vimentin

Topics: Biomarkers, Tumor; Cell Transformation, Neoplastic; Child; Connective Tissue; Humans; Immunoenzyme Techniques; Keratins; Phosphopyruvate Hydratase; Rhabdomyosarcoma; S100 Proteins; Soft Tissue Neoplasms

Twenty-five rhabdomyosarcomas (RMSs), including 12 alveolar and 13 embryonal types, were immunohistochemically studied for the presence of different classes of intermediate filament proteins and muscle actins (MAs). For the most part, formaldehyde-fixed and paraffin-embedded tissue was used in immunostaining. All RMSs showed desmin and MAs, usually in a major portion of tumor cells. The number of MA-positive cells was sometimes higher than that of desmin-positive cells. Vimentin was present in all tumors studied in frozen sections. Eight of 12 alveolar RMSs showed small number of cytokeratin-positive neoplastic cells. Cytokeratin-positive cells were present less commonly in embryonal RMS (3/13 cases). The 68-kD neurofilament protein was found in frozen sections of two embryonal RMSs. The cytokeratin and neurofilament immunostaining could be reproduced by immunofluorescence technique. In addition, we studied three childhood sarcomas, which showed abundant desmin and MA immunostaining but did not conform to the ultrastructural criteria of RMS. Scattered cytokeratin-positive cells were found in two of these tumors, and neurofilaments were found in the two cases for which frozen sections were available. The results show that typical RMS may demonstrate immunohistological pleomorphism with cytokeratin and neurofilament immunoreactivity suggesting the presence of multidirectional differentiation. In addition, there are tumors that by morphology look like RMS and have muscle cell markers but cannot be verified as RMS by electron microscopy; also, these tumors seem to show immunohistological pleomorphism. The presence of nonmyoid markers in RMS should be considered when making immunohistological diagnosis of soft tissue sarcomas.

Topics: Adolescent; Adult; Animals; Biomarkers, Tumor; Child; Child, Preschool; Female; Head and Neck Neoplasms; Humans; Immunohistochemistry; Infant; Intermediate Filament Proteins; Keratins; Male; Mice; Molecular Weight; Neurofilament Proteins; Prostatic Neoplasms; Rhabdomyosarcoma; Thigh

Sixteen cases of malignant rhabdoid tumor (MRT) were studied by conventional light microscopy, immunohistochemistry, electron microscopy and flow cytometry. The age of the 16 patients varied from two months to 25.9 years. There were 11 males and five females. Eleven tumors were located in the kidney. The remaining five were found in the chest wall (n = 2) and the head and neck (n = 3). Particular histopathological findings included myxoid, pseudoalveolar and hyalinized areas. By immunohistochemistry, 15/15 cases stained positively for vimentin, 9/14 for cytokeratin, 6/15 for desmin, 9/14 for epithelial membrane antigen (EMA), 10/14 for neuron specific enolase (NSE) and 10/15 for protein S-100. Stains for neurofilaments, myoglobin and Ulex europaeus aggl. I (UEA I) were negative. The characteristic finding by electron microscopy in three cases were large numbers of intermediate filaments arranged either randomly or in concentric whorls. None of the 11 cases studied revealed aneuploid DNA stem lines as determined by flow cytometry. Of the 16 patients 12 died, one is living with disease and three are living without evidence of disease. Postoperative treatment consisted of chemotherapy, in some cases combined with radiotherapy. Two patients developed a medulloblastoma in addition to a renal and extrarenal MRT, respectively. Our findings demonstrate that MRT may present more histopathological patterns than hitherto recognized. In addition, they show that MRT may express a wide range of antigenic "markers", similar to epithelioid sarcoma with which it may be confused on cytological grounds. Despite aggressive postoperative chemotherapy prognosis is still poor.

Topics: Adolescent; Adult; Age Factors; Aneuploidy; Cell Nucleus; Child; Child, Preschool; Desmin; DNA; Female; Flow Cytometry; Head and Neck Neoplasms; Humans; Immunohistochemistry; Infant; Keratins; Kidney Neoplasms; Male; Microscopy, Electron; Phosphopyruvate Hydratase; Rhabdomyosarcoma; S100 Proteins; Sex Factors; Thoracic Neoplasms; Vimentin; Wilms Tumor

Soft tissue sarcomas were induced by 20-methylcholanthrene in NMRI-mice. The tumors were characterized as rhabdomyosarcomas by light and electron microscopy as well as immunohistochemistry (vimentin, desmin and myoglobin expression). Cytokeratins could be demonstrated by a panel of different poly- and monoclonal antibodies in original rhabdomyosarcomas, their allotransplants and the re-established tumors from cell culture in nude mice. The cytokeratin positive tumor cells were arranged in small clusters and/or haphazardly single dispersed in the rhabdomyosarcomas. By means of monoclonal antibodies cytokeratins No. 8 and No. 19 could be evidenced and cytokeratin No. 18 could be made probably. Behind the background of cytokeratin expression in developing fetal cross striated muscle cells our findings are discussed as a reminiscence of embryonal muscle development in these tumors. The significance of cytokeratin expression in rhabdomyosarcomas for diagnostic histopathology is emphasized.

Topics: Animals; Female; Fluorescent Antibody Technique; Keratins; Male; Methylcholanthrene; Mice; Mice, Inbred Strains; Microscopy, Electron; Neoplasm Transplantation; Rhabdomyosarcoma; Transplantation, Homologous

The recognition of myogenic differentiation is not always possible using routine light or electron microscopic techniques. In this article, we describe our experience with two small round-cell neoplasms occurring in young children, each of which exhibited an undifferentiated light microscopic appearance. In both cases, myogenic features were revealed by immunocytochemical methods applied to fine-needle aspiration (FNA) biopsies. Each was immunoreactive for desmin and vimentin and failed to react with antibodies to leukocyte-common antigen. Moreover, formalin-fixed tissue sections of one case were negative for cytokeratin and epithelial membrane antigen. Sporadic reactivity for neuron-specific enolase and Leu-7 antigen was observed in occasional cells in FNA specimens, but synaptophysin was not identified. The rapidity of the method and the reliability of the immunocytochemical findings, when compared with routine cytologic evaluation, emphasize the diagnostic utility of immunocytochemical analysis when FNAs of pediatric soft-tissue tumors are obtained.

Topics: Actins; Biopsy, Needle; Desmin; Diagnosis, Differential; Female; Head and Neck Neoplasms; Humans; Immunoenzyme Techniques; Infant; Keratins; Male; Membrane Glycoproteins; Mucin-1; Phosphopyruvate Hydratase; Rhabdomyosarcoma; S100 Proteins; Vimentin

Intermediate filaments (IFs) of the cytokeratin (CK) type are cytoskeletal elements typical for epithelial differentiation. However, in diverse transformed culture lines of non-epithelial origin, rare cells emerge spontaneously, which synthesize, in addition to their vimentin IFs, CKs 8 and 18. We enriched such cells by cloning and studied the level(s) of regulation at which these changes occur. We found that in SV40-transformed fibroblasts the CK 18 gene is constitutively transcribed into translatable mRNA but that the protein is rapidly degraded in the absence of its complex partner, CK 8. In contrast, cells immunocytochemically positive for CK IFs contained both CKs 8 and 18, which apparently stabilized in heterotypic complexes. These findings and related observations of active genes for CKs 8 and/or 18 in several other transformed non-epithelial cell lines indicate that the genes for CKs 18 and, less frequently, 8 can be active in diverse different non-epithelial cell lines; synthesis of type I and type II CK pair partners can be uncoupled; control of CK IF formation can take place at different levels. We suggest that the intrinsic instability of the inactive state of these genes is responsible for the occurrence of CKs 8 and 18 in certain non-epithelial tissues and tumors, a caveat in tumor diagnosis.

Topics: Animals; Blotting, Northern; Cell Line, Transformed; Cell Transformation, Viral; Clone Cells; Cricetinae; Fibroblasts; Gene Expression Regulation; Genes; Humans; Keratins; Mice; Protein Biosynthesis; Rats; Rhabdomyosarcoma; RNA, Messenger; Simian virus 40; Transcription, Genetic; Tumor Cells, Cultured

The cytoplasmic filament composition of two pure high-grade endometrial stromal sarcomas and five pure endometrial rhabdomyosarcomas was studied using the immunoperoxidase avidin-biotin method. Ultrastructural correlates were performed on the same tissues. While four of the seven tumors presented as high-grade undifferentiated neoplasms, they were readily categorized on the basis of immunocytochemical findings in which the five rhabdomyosarcomas were positive for muscle-specific actin (HHF 35) and the two stromal sarcomas were positive for vimentin only. Ultrastructure on the HHF 35-positive cases showed the presence of thick filaments and Z-band material, whereas the other tumors showed no cytoplasmic differentiation. Muscle-type actin differs stromal cells, and is useful when used in conjunction with a panel of antibodies in the categorization of undifferentiated endometrial sarcomas.

Topics: Biomarkers, Tumor; Cytoskeletal Proteins; Desmin; Female; Humans; Immunohistochemistry; Keratins; Middle Aged; Myoglobin; Rhabdomyosarcoma; Sarcoma; Uterine Neoplasms; Vimentin

Rhabdoid tumors are highly malignant neoplasms resembling rhabdomyosarcomas, which most commonly occur in the kidney in young children. Several cases of extrarenal malignant rhabdoid tumors have been described, many in adolescents and young adults. We describe a 46-year-old woman with a primary uterine tumor that has the histologic, immunohistochemical, and ultrastructural characteristics of a malignant rhabdoid tumor. To our knowledge, this is the first report of a rhabdoid tumor in this site. Furthermore, this woman's age expands the range for which pathologists and clinicians should consider this diagnosis, even in extrarenal sites.

Topics: Female; Humans; Immunohistochemistry; Keratins; Membrane Glycoproteins; Microscopy, Electron; Middle Aged; Mucin-1; Rhabdomyosarcoma; Uterine Neoplasms

An extrarenal malignant rhabdoid tumor arising from soft parts in the prepubic region of a 37-year-old man is described. This case appears to be unusual with regard to its location and age at onset. To our knowledge, the patient is the oldest recorded in whom such a lesion has arisen. Histologically, the tumor consisted of an admixture of polygonal and elongated cells with abundant eosinophilic cytoplasm frequently containing hyaline-like globules. Ultrastructurally, these cytoplasmic inclusions were compatible with intermediate filaments. Immunohistochemical staining disclosed keratin (non-squamous epithelial type) and epithelial membrane antigen positivity. These characteristic features were identical to malignant rhabdoid tumor of the kidney seen in infants and young children. This extrarenal malignant rhabdoid tumor showed an aggressive clinical course, although its exact histogenesis was unclear.

Topics: Adult; Cell Nucleus; Cytoplasm; Humans; Immunoenzyme Techniques; Intermediate Filaments; Keratins; Male; Membrane Glycoproteins; Microscopy, Electron; Mucin-1; Pubic Bone; Rhabdomyosarcoma; Soft Tissue Neoplasms

The immunohistochemical study of 60 cases of rhabdomyosarcomas made it possible to test eight different antibodies currently used in tumour pathology: i.e., antisera to vimentin, desmin, myoglobin, cytokeratin, epithelial membrane antigen, S100 protein, neurofilaments, and leukocyte common antigen. Vimentin was found in 58 cases (97 per cent), desmin in 49 cases (82 per cent), myoglobin in 23 cases (38 per cent), S100 protein in 7 cases (12 per cent), and cytokeratin in 3 cases (5 per cent). Other markers were negative. S100 protein was present in large round tumour cells with abundant eosinophilic cytoplasm (round rhabdomyoblasts), whereas cytokeratin was present in small tumour cells similar to those observed in rhabdoid sarcoma. This unexpected staining should become common knowledge for the correct interpretation of the immunohistochemical study of small cell tumours in the young.

Topics: Adolescent; Adult; Antibodies, Monoclonal; Biomarkers, Tumor; Child; Child, Preschool; Female; Humans; Immune Sera; Immunoenzyme Techniques; Infant; Intermediate Filaments; Keratins; Male; Rhabdomyosarcoma; S100 Proteins

Two cases of malignant rhabdoid tumor of the kidney (MRT) and two cases of bone metastasizing renal tumor (BMRT) were studied using electron microscopy and immunohistochemistry, in an attempt to evaluate the histogenetic relation to classical Wilms' tumor (2 cases). The two cases of MRT showed ultrastructural features of intermediate filament clusters in the cytoplasm. Vimentin, keratin, laminin, and peanut (PNA) lectin were immunohistochemically demonstrated in two cases of MRT. Tissue polypeptide antigen (TPA) was detected in one case. As for BMRT, the metastatic lesion exhibited numerous rosette-like structures of tumor cells. Vimentin was immunohistochemically demonstrated in one case, but no other antibodies were stained in two cases. Two cases of classical Wilms' tumor immunohistochemically demonstrated vimentin, keratin, PNA lectin, and TPA. MRT and BMRT fall within the broad spectrum of Wilms' tumors from the histogenetic aspect, but their clinical behavior is distinct.

Topics: Bone Neoplasms; Child; Creatine Kinase; Humans; Immunoenzyme Techniques; Isoenzymes; Keratins; Kidney; Kidney Neoplasms; Laminin; Lectins; Microscopy, Electron; Myoglobin; Peanut Agglutinin; Rhabdomyosarcoma; Sarcoma; Vimentin; Wilms Tumor

A rapidly fatal bladder tumour which had the features of a rhabdoid tumour was studied by sequential biopsies and at autopsy. This is the first rhabdoid tumour recorded at this site and the first in which there was co-existent transitional cell carcinoma. The possibility that rhabdoid tumour is histogenetically heterogeneous is discussed.

Topics: Carcinoma, Transitional Cell; Female; Humans; Immunohistochemistry; Keratins; Microscopy, Electron; Middle Aged; Neoplasms, Multiple Primary; Phosphopyruvate Hydratase; Rhabdomyosarcoma; Urinary Bladder Neoplasms; Vimentin

Topics: Antigens, Neoplasm; Desmin; Humans; Keratins; Mesenchymoma; Rhabdomyosarcoma; Vimentin

The present study describes 11 cases (10 carcinomas, one rhabdomyosarcoma) in which immuno-alkaline phosphatase labelling with monoclonal antibodies was used to demonstrate metastatic cells in routine smears of aspirated bone marrow. Carcinoma cells were detected using antibodies against epithelial cytokeratins, milk fat globule membrane antigen and carcinoembryonic antigen, and rhabdomyosarcoma cells with monoclonal anti-desmin. In four of the carcinoma cases it had not been possible to identify malignant cells in routinely stained marrow smears, whilst the case of disseminated rhabdomyosarcoma had initially been diagnosed (and treated) as a case of acute lymphoblastic leukaemia. The anti-cytokeratin antibody was found to be the most valuable of the anti-epithelial reagents used, since it labelled malignant cells in all of the 10 cases of carcinoma and gave the strongest reactions. These results suggest that immunocytochemical labelling should be used in cases of suspected carcinoma whenever conventional examination of marrow smears yields negative results, and furthermore (as illustrated by the case of rhabdomyosarcoma) that the technique is of value for identifying the true nature of poorly differentiated neoplasms in bone marrow.

Topics: Aged; Antibodies, Monoclonal; Antigens, Neoplasm; Bone Marrow Diseases; Carcinoembryonic Antigen; Female; Humans; Immunoenzyme Techniques; Keratins; Male; Membrane Proteins; Middle Aged; Mucin-1; Neoplasm Metastasis; Rhabdomyosarcoma

The intermediate filament proteins of rhabdoid tumors of the kidney were investigated with a panel of monoclonal antibodies to different intermediate filament proteins. Rhabdoid tumor cells are decorated by an antivimentin antibody and by an antibody made against a 54-kilodalton (kd) cytokeratin from human hepatoma cells. The rhabdoid tumor cells fail to react with an antibody generated against keratin from stratum corneum or with an anti-200-kd neurofilament protein antibody. Cytoskeleton preparations of rhabdoid tumor cells grown in vitro demonstrate the presence of vimentin (58 kd) and the 54-kd cytokeratin. Thus, these cells contain two different intermediate filament proteins characteristic of epithelial and mesenchymal cells. We also demonstrate that rhabdoid tumor cells can form tumors in athymic (nude) mice and that the intracytoplasmic globules are present in the nude mouse lesions.

Topics: Animals; Antibodies, Monoclonal; Cells, Cultured; Child, Preschool; Electrophoresis, Polyacrylamide Gel; Female; Humans; Intermediate Filament Proteins; Keratins; Kidney Neoplasms; Male; Mice; Mice, Nude; Neoplasms, Experimental; Rhabdomyosarcoma; Vimentin

Four embryonal rhabdomyosarcomas, one tumor diagnosed as an undifferentiated sarcoma, probably a rhabdomyosarcoma, and six different non-muscular sarcomas were investigated with antibodies specific for different intermediate filament types. The tumor cells in the rhabdomyosarcomas and the undifferentiated tumor were stained clearly by antibodies to desmin, the intermediate filament type characteristic of muscle. The staining of tumor cell by antibodies to vimentin, the intermediate filament type characteristic of certain cell types of mesenchymal origin including myoblasts, was different in these 5 cases. In one case of embryonal rhabdomyosarcoma nearly all tumor cells were stained, but in the remaining cases few or no tumor cells were positive with the vimentin antibody. In these rhabdomyosarcomas not only the large rhabdomyoblasts, but also the small undifferentiated cells were labeled by antibodies to desmin. In the latter cell type the desmin filaments were arranged typically in coils. In contrast, tumor cells in the non-muscular mesenchymal sarcomas were stained only by antibodies to vimentin but not by antibodies to desmin or prekeratin. The retention of the desmin marker characteristic of normal muscle in cases of rhabdomyosarcoma not only allowed the undifferentiated desmin-positive sarcoma to be classified as rhabdomyosarcoma but also suggests that the use of antibodies to desmin could be very helpful in the future for the diagnosis of undifferentiated rhabdomyosarcomas.

Topics: Adult; Aged; Antibodies; Child; Child, Preschool; Desmin; Female; Humans; Infant; Keratins; Male; Muscle Proteins; Protein Precursors; Rhabdomyosarcoma; Vimentin

Fifty primary gastrointestinal and breast carcinomas, four embryonal rhabdomyosarcomas, six nonmuscular mesenchymal malignant tumors and one mesothelioma have been studied to determine what type of intermediate filaments they express, using affinity purified antibodies to prekeratin, vimentin and desmin and FITC or peroxidase labeled second antibodies. The tissues were alcohol fixed and paraffin embedded before use. In all carcinoma cases the tumor cells are stained by antibodies to prekeratin, while the vimentin antibody only decorates the stroma. Prekeratin positive tumor cells are not only seen in well differentiated tumors, but also in signet ring cell carcinomas. In the case of rhabdomyosarcoma the tumor cells clearly were decorated by antibodies to desmin, while the vimentin antibody only stained very few tumor cells. In cases of nonmuscular mesenchymal tumors, the tumor cells could only be labeled by antibodies to vimentin and not by antibodies to prekeratin or desmin. In biphasic tumors like mesothelioma, different parts of the tumor were separated by antibodies to prekeratin and vimentin.

Topics: Adenocarcinoma, Mucinous; Breast Neoplasms; Carcinoma; Child; Cytoskeleton; Desmin; Female; Gastrointestinal Neoplasms; Humans; Intermediate Filament Proteins; Keratins; Mesothelioma; Neoplasms; Neoplasms, Germ Cell and Embryonal; Protein Precursors; Rhabdomyosarcoma; Sarcoma; Vimentin