bromochloroacetic-acid and Abdominal-Neoplasms

The malignant rhabdoid tumor (MRT) has been a controversial lesion since its seminal description. There is no consensus as to whether it represents a distinctive clinicopathological entity or, alternatively, a phenotypic pattern that is potentially common to several disparate neoplasms. MRT of the kidney is a childhood tumor that is associated with uniformly aggressive behavior, but it shows a wide spectrum of histologic, immunophenotypic, and cytogenetic findings. Malignant extrarenal rhabdoid tumors (MERTs) have been observed in pure form over a broader range of patient ages and anatomic locations, but they show substantial morphological and biological homology with renal MRT. Lastly, "composite" extrarenal rhabdoid tumors (CERTs)--in which recognizable "parent" neoplasms are admixed with MERTs--also have been recognized in several topographic sites. In aggregate, these observations suggest that "rhabdoid tumors" are a heterogeneous group of lesions with dissimilar lineages of differentiation. Particularly in CERTs, it is likely that the rhabdoid phenotype represents a common end point of clonal evolution in tumors of clearly different origins. Despite these caveats, the authors do support retention of the diagnosis of "rhabdoid tumor," because the affiliated morphological pattern is uniformly attended by aggressive biological behavior despite potential dissimilarities at a subcellular level.

Topics: Abdominal Neoplasms; Central Nervous System Neoplasms; Child, Preschool; Female; Head and Neck Neoplasms; Humans; Immunohistochemistry; Infant; Infant, Newborn; Keratins; Kidney Neoplasms; Male; Mucin-1; Rhabdoid Tumor

ATF1, CREB1, and CREM, which encode the CREB family of transcription factors, are fused with EWSR1 or FUS in human neoplasms, such as angiomatoid fibrous histiocytoma. EWSR1/FUS-CREB fusions have recently been reported in a group of malignant epithelioid tumors with a predilection to the peritoneal cavity and frequent cytokeratin expression. Here, we studied 8 cytokeratin-positive abdominal malignancies with these fusions for further characterization. The tumors affected males (15 to 76 y old) and presented as intra-abdominal masses with concurrent or subsequent peritoneal dissemination, ascites, and/or metastases to the liver or lymph nodes. Four patients died of the disease within 18 to 140 months. Cases 1 to 5 showed multinodular growth of monomorphic epithelioid cells with focal serous cysts. Lymphoplasmacytic infiltration was prominent and was associated with systemic inflammatory symptoms. Two patients suffered from membranous nephropathy with nephrosis. The tumors displayed partly overlapping phenotypes with malignant mesothelioma, including diffuse strong expression of AE1/AE3 and WT1 and membranous positivity of sialylated HEG1, although calretinin was negative. Case 6 showed similar histology to cases 1 to 5, but expressed smooth muscle actin diffusely, lacked WT1 and HEG1, and harbored prominent pseudoangiomatous spaces. Cases 7 and 8 displayed dense growth of small oval to short spindle cells, with occasional molding and minor swirling, superficially resembling small cell carcinoma. Lymphoplasmacytic infiltration was not observed. The tumors were positive for AE1/AE3 and CD34 (focal), whereas calretinin, WT1, and HEG1 were negative. The detected fusions were FUS-CREM (n=4), EWSR1-ATF1 (n=2), EWSR1-CREB1 (n=1), and EWSR1-CREM (n=1). We confirmed the prior observation that these tumors do not fit perfectly with known entities and provided additional novel clinicopathologic information. The tumors require wider recognition because of more aggressive behavior than angiomatoid fibrous histiocytoma despite similar genetics, and potential misdiagnosis as unrelated diseases, such as neuroendocrine neoplasms.

Topics: Abdominal Neoplasms; Adolescent; Adult; Aged; Biomarkers, Tumor; Cyclic AMP Response Element Modulator; Gene Fusion; Genetic Predisposition to Disease; Histiocytoma, Malignant Fibrous; Humans; Immunohistochemistry; In Situ Hybridization, Fluorescence; Keratins; Male; Mesothelioma, Malignant; Middle Aged; Oncogene Proteins, Fusion; Phenotype; RNA-Binding Protein FUS; RNA-Seq; Treatment Outcome; Young Adult

Topics: Abdominal Neoplasms; Adult; Aged; Aged, 80 and over; Antigens, CD34; Female; Humans; Keratins; Middle Aged; Solitary Fibrous Tumor, Pleural; Solitary Fibrous Tumors; STAT6 Transcription Factor

Topics: Abdominal Neoplasms; Female; Humans; Keratins; Solitary Fibrous Tumor, Pleural; Solitary Fibrous Tumors

Desmoplastic small round cell tumor (DSRCT) is a distinctive clinicopathologic entity with an aggressive clinical course that typically involves the abdominal and/or pelvic peritoneum of young males. A population of small round blue cells and a fibroesclerotic stroma are the usual morphologic features. This tumor is characterized by a typical polyphenotypic profile with expression of epithelial, mesenchymal and neural markers. Cytogenetically, this tumor presents a unique abnormality - t(11;22)(p13;q12).. A 29-year-old male without significant medical history was admitted to our institution with gastrointestinal symptomatology. The physical examination and medical imaging studies revealed an extensive soft tissue mass filling the entire peritoneal cavity/pelvis. A fine-needle aspiration (FNA) of the abdominal mass was performed. The FNA smears revealed fragments of collagenous desmoplastic stroma and clusters of loosely cohesive small round cells that showed positivity for epithelial and myogenic markers. Cytogenetic analysis demonstrated rearrangement of the genes EWSR1 and WT1, resulting from the t(11;22)(p13;q12).. DSRCT is an uncommon neoplasm that shares clinical and cytomorphologic features with other small round cell tumors. Therefore, a primary definitive diagnosis based on cytology specimens may be difficult but plausible and can be aided by a typical clinical presentation and ancillary immunocytochemical/cytogenetic studies.

Topics: Abdominal Cavity; Abdominal Neoplasms; Adult; Biomarkers; Biopsy, Fine-Needle; Calmodulin-Binding Proteins; Chromosomes, Human, Pair 11; Chromosomes, Human, Pair 22; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Fatal Outcome; Gene Rearrangement; Humans; Immunohistochemistry; Keratins; Male; RNA-Binding Protein EWS; RNA-Binding Proteins; Translocation, Genetic; Vimentin; WT1 Proteins

Topics: Abdominal Neoplasms; Adolescent; Diagnosis, Differential; Fibroblasts; Groin; Histiocytoma, Malignant Fibrous; Humans; Keratins; Lymph Nodes; Lymphoma; Male; Melanoma; Vimentin

Synovial sarcoma arising in the abdominal wall is a rare tumor. We report a case of a 38-year-old man who complained of abdominal pain. Physical examination revealed a firm mobile mass, 25 cm in diameter, in the left lower abdominal wall. The tumor was first thought to be a sarcoma arising from the omentum or mesentery. During surgery, a large tumor was found attached to the inner surface of the abdominal wall and compressing the gastrointestinal tract. On microscopic examination the tumor corresponded to a biphasic synovial sarcoma immunoreactive for cytokeratins (AE1/AE3, 7 and 19), epithelial membrane antigen and carcinoembryonic antigen in the epithelial tumor cells, for E-cadherin especially in their glandular structure, vimentin, CD99, and CD56 in the spindle cell component and for bcl-2 protein. The tumor recurred at the same site, and clinical course progressed to death 3 months after the initial diagnosis.

Topics: 12E7 Antigen; Abdominal Neoplasms; Abdominal Wall; Adult; Antigens, CD; Biomarkers, Tumor; Cadherins; Carcinoembryonic Antigen; Carcinosarcoma; CD56 Antigen; Cell Adhesion Molecules; Fatal Outcome; Humans; Keratins; Male; Neoplasm Recurrence, Local; Sarcoma, Synovial; Tomography, X-Ray Computed; Vimentin

To evaluate a series of synovial sarcomas arising in the abdomen, pelvic cavity, or retroperitoneum. Synovial sarcoma is rare within the abdomen. In this location, it can be confused with other biphasic tumours and with other spindle and round cell sarcomas.. Cases were retrieved from archives. There were 11 intra-abdominal tumours among 300 synovial sarcomas in two referral practices (3.7%). Three were pelvic (two midline, one sidewall) and eight were retroperitoneal. They occurred in six males and five females aged from 25 to 75 years (mean 49 years, median 46 years), and ranged in diameter from 65 to 470 mm (mean 210 mm, median 150 mm). Six examples were biphasic, five were monophasic and seven had poorly differentiated areas. Monophasic tumours displayed at least one epithelial marker. One biphasic tumour had a SYT-SSX2 fusion gene. Seven sarcomas were high-grade and four of intermediate grade malignancy. Follow-up data were available in 10 patients. In all but one case, tumour recurred or metastasized within the abdomen. The pelvic sarcomas also metastasized outside the abdomen. Eight of 10 patients (80%) died of disease with survival from 4 to 36 months (mean 17 months, median 18 months). Two patients were alive with disease at 43 and 48 months.. Synovial sarcomas rarely arise within the abdomen and pelvis. They occur mainly in middle age, attain a large size, are difficult to excise and recur locally. Pelvic tumours metastasize distantly. Retroperitoneal tumours remain confined to the abdomen and, unlike synovial sarcomas elsewhere, do not metastasize remotely, although mortality is high.

Topics: 12E7 Antigen; Abdominal Neoplasms; Adult; Aged; Antigens, CD; Cell Adhesion Molecules; DNA, Complementary; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Mucin-1; Oncogene Proteins, Fusion; S100 Proteins; Sarcoma, Synovial; Sequence Analysis, DNA

Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young male patients and is characterized by a reciprocal translocation t(11;22)(p13;q12) associated with the EWS-WT1 gene fusion transcript. Clinical, histopathologic, immunohistochemical, and molecular genetics features were reviewed for 32 tumors. There were 29 male and three female patients, with ages from 6 to 54 years (mean, 25 years). The main clinical signs and symptoms included abdominal pain (eight patients), weight loss (five patients), and presence of umbilical hernia (four patients). Two tumors primarily involved the ethmoid sinus and the soft tissues of the scalp; the other tumors (mean size, 10 cm) involved the abdominal cavity (88%). One patient presented initially with an axillary lymph node metastasis. Generally, all tumors showed the typical histologic findings of variably sized clusters of small, round, or spindled cells lying in a desmoplastic stroma. The neoplastic cells in formalin-fixed, paraffin-embedded tissue sections were positive for desmin (dot pattern) (81% of the cases), WT1 (91%), keratin (87%), neuron-specific enolase (84%), CD99 (23%), and actin (3%). The EWS-WT1 gene fusion transcript was detected in 29 of 30 tumors. One tumor with typical clinicopathologic and immunohistochemical features did not show the gene fusion. Follow-up for 27 patients showed that 19 patients (70%) died of uncontrolled, local, or widespread metastatic disease 3-46 months (mean, 20 months) after diagnosis, and eight patients were alive with known evidence of disease. Occasionally, desmoplastic small round cell tumor lacks the classic clinical, histologic, and immunohistochemical features. This study emphasizes the utility of analysis of the EWS-WT1 gene fusion transcript, which was performed on paraffin-embedded tissues, to confirm the diagnosis.

Topics: Abdominal Neoplasms; Adolescent; Adult; Blotting, Southern; Carcinoma, Small Cell; Child; Desmin; Female; Follow-Up Studies; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Nucleic Acid Hybridization; Oncogene Proteins, Fusion; Phosphopyruvate Hydratase; Polymerase Chain Reaction

An 11-month-old male mixed breed dog was euthanized due to two months history of vomiting and anorexia. At necropsy, numerous, multifocal or coalescing, firm, protruding nodules, 5 to 40 mm in diameter were scattered throughout the mesentery and omentum. Histologically and immunohistochemically, the nodules were diagnosed as malignant mesothelioma. Metastasis to the regional mesenteric, mediastinal and tracheobronchial lymph nodes were observed.

Topics: Abdominal Neoplasms; Animals; Dog Diseases; Dogs; Fatal Outcome; Female; Immunohistochemistry; Keratins; Mesothelioma; Omentum; Vimentin

An unusual case of deciduoid mesothelioma occurring in the anterior abdominal wall of a 30-year-old woman is reported. The patient had a palpable mass that was resected. The mass appeared largely cystic with solid areas. Histologically, the tumor cells appeared epitheloid with eosinophilic cytoplasm and prominent nucleoli. The tumor was positive for keratins and vimentin and negative for CEA and Ber-EP4. Electron microscopy showed features of mesothelial cells characterized by well-formed desmosomes and long, slender microvilli. In contrast to previously reported cases of deciduoid mesothelioma, this tumor developed in the abdominal wall and appears to have a benign course.

Topics: Abdominal Muscles; Abdominal Neoplasms; Adult; Antigens, Neoplasm; Biomarkers; Biomarkers, Tumor; Decidua; Female; Humans; Immunohistochemistry; Keratins; Mesothelioma; Microvilli; Radiography

Although 'aberrant' expression of the epithelial markers, cytokeratin (CK) and epithelial membrane antigen (EMA), in leiomyosarcoma has been described previously, there has not been a study of this phenomenon with clinicopathological correlation in a large series of lesions at different anatomical sites. We investigated systematically the immunohistochemical reactivity for CK and EMA in 100 cases of leiomyosarcoma. CK and EMA were positive in 38% and 44% of the cases, respectively. Although staining was usually focal, extensive immunoreactivity was observed in 11% with CK and 6% with EMA. There was no correlation between immunoreactivity for CK and EMA in leiomyosarcomas and non-neoplastic smooth muscle at the same location. Immunoreactivity for CK and EMA was not correlated with the location, age, sex, histological grade, or histological features, except for more frequent EMA positivity in vascular and uterine tumors than in soft tissue cases. These results indicate that CK and/or EMA-positive leiomyosarcomas do not have distinctive clinicopathological features differing from those of negative cases. However, the considerable frequency of immunoreactivity for these epithelial markers in leiomyosarcoma, occasionally with diffuse and strong immunopositivity, should be recognized as a potentially serious diagnostic pitfall in the differential diagnosis of other malignant spindle cell neoplasms.

Topics: Abdominal Neoplasms; Adult; Aged; Aged, 80 and over; Biomarkers; Female; Humans; Immunoenzyme Techniques; Keratins; Leiomyosarcoma; Male; Middle Aged; Mucin-1; Muscle, Smooth; Skin Neoplasms; Soft Tissue Neoplasms; Uterine Neoplasms; Vascular Neoplasms

Desmoplastic small round-cell tumor typically occurs in the abdomen of young men, but it can also develop at other anatomic sites and in older people, presenting greater diagnostic difficulties. We report a case of this tumor arising from the paratesticular region in a 43-year-old man. The tumor showed histologic, immunohistochemical, and ultrastructural evidence of multilineage differentiation, including epithelial, mesenchymal, and neuronal features. In addition, the presence of an EWS and WT1 chimeric messenger RNA was demonstrated by the reverse transcriptase-polymerase chain reaction using an EWS exon 7 primer and WT1 exon 8 and exon 9 primers, which revealed single polymerase chain reaction products with a junction of EWS exon 7 to WT1 exon 8. Our study demonstrates that desmoplastic small round-cell tumors of the paratesticular region share not only morphologic but also molecular genetic features with those of the abdomen and that reverse transcriptase-polymerase chain reaction analysis using paraffin sections is useful for a conclusive diagnosis.

Topics: Abdominal Neoplasms; Adult; Base Sequence; Desmin; Diagnosis, Differential; Genitalia, Male; Humans; Immunohistochemistry; Keratins; Male; Molecular Sequence Data; Mucin-1; Neural Cell Adhesion Molecules; Oncogene Proteins, Fusion; Paraffin Embedding; Phosphopyruvate Hydratase; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; Testicular Neoplasms; Vimentin

Desmoplastic small round cell tumor (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects male adolescents and young adults. This tumor is characterized by nests of small undifferentiated cells that show immunohistochemical evidence of epithelial, mesenchymal, and neural differentiation. We report two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation, but were found to have the fusion transcripts characteristic of this tumor. Both patients (a 41-year-old male and a 31-year-old female) presented with large intra-abdominal masses. After diagnostic biopsy, both were treated with multi-agent chemotherapy. One patient expired 18 days after diagnosis, and the other is currently alive 28 months later. Histologically, both tumors had the characteristic features of DSRCT and were composed of small round cells with hyperchromatic nuclei and scanty cytoplasm. In one of the cases, perinuclear intracytoplasmic hyaline inclusions were seen. Immunohistochemically, neither case expressed any of the epithelial markers tested, including AE1/AE3, CAM 5.2 and EMA. Both tumors were diffusely immunoreactive for desmin with a prominent globoid "dot-like" pattern of staining in one case. Both tumors stained for vimentin, neuron specific enolase, and synaptophysin, but were negative for CD99, muscle-specific actin, and myogenin. Reverse transcriptase-polymerase chain reaction revealed EWS-WT1 fusion transcripts characteristic of this neoplasm. In conclusion, we describe two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation but had histologic and other immunohistochemical features which suggested this diagnosis. The ability to confirm the diagnosis of this rare tumor using molecular genetic techniques is particularly useful in those cases with unusual histologic or immunophenotypic features.

Topics: Abdominal Neoplasms; Adult; Carcinoma, Small Cell; Female; Humans; Immunohistochemistry; Keratins; Male; Oncogene Proteins, Fusion; Phosphopyruvate Hydratase; Reverse Transcriptase Polymerase Chain Reaction; Synaptophysin; Tumor Cells, Cultured; Vimentin

Adenocarcinomas may arise primarily from the urinary bladder, but secondary involvement from adenocarcinomas arising in adjacent organs is more common. In the present study we tried to differentiate primary urinary bladder adenocarcinomas from adenocarcinomas arising from the surrounding organs, based on their antigen profiles in routinely processed, paraffin-embedded tissue specimens. We analysed the staining results using stepwise linear discriminant analysis.. We investigated the usefulness of a panel of antibodies against cytokeratin 7, E48, cytokeratin 20, PSA, PSAP, CEA, vimentin, OC125 and HER-2/neu, to discriminate primary bladder adenocarcinoma from adenocarcinomas arising from the prostate, urachus, colon, cervix, ovary and endometrium. In the differential diagnosis with urinary bladder adenocarcinoma, an overall correct classification was reached for 77% and 81% of urachal and colonic carcinomas, respectively, using CEA, for 93% of prostatic adenocarcinomas using PSA, for 82% and 70% of cervical and ovarian adenocarcinomas, respectively, using OC125, and for 91% of endometrial adenocarcinomas using vimentin. Adding other antibodies did not improve the classification results for any of these differential diagnoses.. For the surgical pathologist, a panel of antibodies consisting of CEA, PSA, OC125 and vimentin is helpful to differentiate primary urinary bladder adenocarcinomas from adenocarcinomas originating from prostate and endometrium, less helpful in differentiation with urachal carcinoma, and not helpful in differentiation with colonic, cervical and ovarian carcinoma.

Topics: Abdominal Neoplasms; Acid Phosphatase; Adenocarcinoma; Antibodies, Monoclonal; Antibody Specificity; CA-125 Antigen; Carcinoembryonic Antigen; Carcinoma, Papillary; Cell Adhesion Molecules; Diagnosis, Differential; Endometrial Neoplasms; Female; Glycoproteins; GPI-Linked Proteins; Humans; Immunohistochemistry; Intermediate Filament Proteins; Keratin-20; Keratin-7; Keratins; Male; Neoplasms, Unknown Primary; Ovarian Neoplasms; Prostate; Prostate-Specific Antigen; Receptor, ErbB-2; Urachus; Urinary Bladder Neoplasms; Uterine Cervical Neoplasms; Vimentin

Several reverse transcriptase polymerase chain reaction (RT-PCR) assays have been described for the detection of circulating tumour cells in blood and bone marrow. Target mRNA sequences for this purpose are the cytokeratins (CK) 19 and 20, the carcinoembryonic antigen (CEA), and the prostate-specific antigen messages. In this study, we investigated biological factors influencing the specificity of the CK19 and CEA RT-PCR assays. Bone marrow, granulocyte colony-stimulating factor (G-CSF)-mobilized blood stem cells and peripheral blood samples obtained from healthy volunteers (n = 15; CEA n = 7), from patients with epithelial (n = 29) and haematological (n = 23) cancer and from patients with chronic inflammatory diseases (n = 16) were examined. Neither CEA nor cytokeratin 19 messages could be amplified from bone marrow samples from healthy subjects and from patients with haematological malignancies. In contrast, specimens from patients with inflammatory diseases scored positive up to 60%. To investigate the influence of inflammation on target mRNA expression, haemopoietic cells were cultured with and without cytokine stimulation in vitro. CK19 messages could be easily detected in cultured marrow cells without further stimulation, CEA messages only after gamma-interferon (gamma-INF) stimulation. In contrast, G-CSF-mobilized peripheral blood stem cells were positive for CK19 messages only after stem cell factor (SCF) or interleukin stimulation. We conclude that transcription of so-called tissue-specific genes is inductible in haemopoietic tissues under certain conditions. These factors have to be considered in future applications of RT-PCR for the detection of minimal residual disease.

Topics: Abdominal Neoplasms; Bone Marrow; Carcinoembryonic Antigen; Cytokines; Granulocyte Colony-Stimulating Factor; Hematopoietic Stem Cell Mobilization; Hematopoietic Stem Cells; Humans; Inflammation; Keratins; Leukapheresis; Neoplastic Cells, Circulating; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger; Sensitivity and Specificity; Stimulation, Chemical; Stromal Cells

While undergoing a repeat Caesarian section, a 21-year-old woman was found to have a subcutaneous, 3.5-cm tumor-like lesion in the abdominal wall. It had a lobulated contour and consisted of gelatinous tan nodules with intervening fibrous septa. It had a composite histology, including a solid pattern of large glassy cells with a predominant perivascular and pericystic/cisternal topography (35% surface area); a pattern of dyscohesive, vacuolated cells including physaliphorous-like forms (35% surface area); and a myxoid pattern of spindle to stellate cells showing frequent cell contact and lying within an optically clear or pale eosinophilic and bubbly extracellular matrix with a prominent capillary framework. A strong and diffuse cytoplasmic expression of vimentin (only) was present in all cytoarchitectural patterns. The extracellular matrix and intracytoplasmic vacuoles contained abundant acid mucosubstance, mostly hyaluronic acid. No distinct endometrial gland, stigma of hemorrhage, or nuclear estrogen/progesterone receptor protein expression was observed. There were variously sized and shaped cystic spaces lined by a flat to cuboidal cytokeratin-positive cell lining and focally containing neutral and acid mucosubstance. These spaces are interpreted as dilated endometrial glands rather than mechanically entrapped inclusions of mesothelial origin. In this setting, florid decidual reaction represents a potential diagnostic pitfall because it could be confused with more commonly encountered myxoid or epithelioid tumors of mesenchymal, epithelial, or melanocytic cell lineage.

Topics: Abdominal Neoplasms; Adult; Biomarkers, Tumor; Diagnosis, Differential; Embryo Implantation; Endometriosis; Female; Humans; Immunohistochemistry; Keratins; Sarcoma

We report an intra-abdominal round cell tumor in a young man which exhibited the light and electron microscopic appearance of a peripheral primitive neuroectodermal tumor (PNET), in addition to the clinical and topographic characteristics, desmoplasia and a complex immunophenotypic profile of the intra-abdominal desmoplastic round cell tumor (DSRCT). Reverse transcription polymerase chain reaction revealed a EWS/FLI-1 fusion transcript as in PNET/Ewing's sarcoma, instead of the EWS/WT1 transcript of DSRCT. The tumor was also strongly positive for the mic2 protein. This is a unique case of a hybrid tumor arising in the peritoneal cavity of a young male. The existence of such a hybrid tumor in this location suggests that DSRCT and PNET may be related and possibly share a common histogenesis.

Topics: Abdominal Neoplasms; Adult; Biomarkers; Blotting, Western; Carcinoma, Small Cell; Desmin; DNA-Binding Proteins; Fatal Outcome; Humans; Immunohistochemistry; Keratins; Male; Microscopy, Electron; Neuroectodermal Tumors, Primitive; Oncogene Proteins, Fusion; Polymerase Chain Reaction; Proto-Oncogene Protein c-fli-1; Proto-Oncogene Proteins; Sarcoma, Ewing; Tomography, X-Ray Computed; Trans-Activators

Topics: Abdominal Neoplasms; Animals; Cattle; Cattle Diseases; Female; Immunoenzyme Techniques; Keratins; Lymph Nodes; Lymphatic Metastasis; Mesothelioma; Vimentin

The present report describes the case of a 9-yr-old boy with an abdominal desmoplastic small round cell tumor (DSRCT) which on fine-needle aspiration cytology and histology revealed a biphasic pattern, making initial diagnosis difficult. Epithelial-like clusters of cells and loosely-arranged poorly-differentiated cells with scant cytoplasm associated with cells having a larger nucleus and multinucleated larger cells represented the smears' counter-part of epithelial clusters and lobules and sarcomatous-like tissue recognized in the histologic sections. Multinucleated cells were common in the sarcomatous-like areas of the tumor. The biphasic pattern was highlighted by immunohistochemistry. Keratin and epithelial membrane antigen stained predominantly or only the epithelial component, while desmin diffusely decorated the sarcomatous areas and the epithelial cells as a paranuclear cytoplasmic dot. Immunosera 013 mainly stained the sarcomatous component.

Topics: Abdominal Neoplasms; Biopsy, Needle; Cell Nucleus; Child; Chromogranins; Cytoplasm; Desmin; Humans; Immunohistochemistry; Keratins; Male; Mucin-1; Tomography, X-Ray Computed; Ultrasonography; Vimentin

Five cases of intraabdominal small-cell tumor with divergent differentiation are reported. All patients were of male sex. They were 10, 15, 20, 21, and 30 years of age at time of diagnosis, respectively. By light microscopy, the tumors consisted of small cells arranged in groups, nests, and clusters separated by a collagen-rich desmoplastic stroma. Immunohistochemical studies revealed the coexpression of mesenchymal, epithelial, and neural markers. Notably, all tumors coexpressed vimentin, cytokeratin, and desmin, the latter in a remarkable paranuclear dot-like fashion. In contrast to other authors, we did not find chromogranin. DNA image cytometry on four cases demonstrated two diploid and two aneuploid (hyperdiploid) cases. No correlation was found between ploidy and prognosis. One patient died from disease, another died from veno-occlusive disease after bone marrow transplantation, and the remaining patients are alive, but have progressive intraabdominal disease. Thus, our findings support the poor prognosis in this type of tumor.

Topics: Abdominal Neoplasms; Adolescent; Adult; Antigens, Differentiation; Biomarkers, Tumor; Child; Desmin; DNA, Neoplasm; Ferritins; Humans; Immunohistochemistry; Keratins; Male; Phosphopyruvate Hydratase; Ploidies; Vimentin

Topics: Abdominal Neoplasms; Adult; Carcinoma, Small Cell; Child; Desmin; Female; Humans; Keratins; Male; Vimentin

Two cases are reported of intra-abdominal small cell tumours expressing concomitant neural and epithelial differentiation. These features were discernible on conventional microscopy and supported immunocytochemically. Immunoreactive vimentin was also revealed in both tumours, and, in addition, one showed focal desmin positivity. Epithelial differentiation in both tumours was confirmed ultrastructurally. The tumours were interpreted to represent a variant of peripheral primitive neuroectodermal tumour, and the report serves to emphasize a potential among such tumours for complex differentiation. The neoplasms are compared with other similar tumours reported recently in children.

Topics: Abdominal Neoplasms; Adolescent; Cell Transformation, Neoplastic; Epithelium; Humans; Immunohistochemistry; Keratins; Male; Membrane Glycoproteins; Microscopy, Electron; Mucin-1; Neoplasms, Germ Cell and Embryonal; Phosphopyruvate Hydratase

Nineteen cases of a distinctive type of malignant small-cell tumor are presented. The main features of the entity are as follows: a predilection for adolescent males (mean age: 18.6 years); predominant or exclusive intra-abdominal location, with only inconstant and secondary organ involvement; nesting pattern of growth; focal rhabdoid features; intense desmoplastic reaction; immunohistochemical reactivity for epithelial [keratin, epithelial membrane antigen (EMA)], neural [neuron-specific enolase (NSE)], and muscle (desmin) markers; and highly aggressive behavior. It is proposed that this represents yet another member of the continuously enlarging and evolving family of small round (blue) cell tumors of infancy and childhood that features, more than any other member of this group, the capacity for simultaneous multidirectional phenotypical expression.

Topics: Abdominal Neoplasms; Adolescent; Adult; Carcinoma, Small Cell; Child; Desmin; Female; Humans; Immunohistochemistry; Keratins; Male; Membrane Glycoproteins; Microscopy, Electron; Mucin-1; Phenotype; Phosphopyruvate Hydratase; Vimentin

A 28-year-old man with a large abdominal mass was found to have a tumor involving the peritoneum that had no other apparent primary origin. By light microscopy, the histologic pattern of the tumor was that of a small-cell epithelial neoplasm. Electron-microscopic examination demonstrated desmosomes and paranuclear aggregates of intermediate filaments. Immunohistochemical studies revealed reactivity for keratin, desmin, and vimentin. The globoid staining observed for the last two markers correlated with the paranuclear concentration of intermediate filaments observed by electron microscopy. We believe this case represents an unusual small-cell epithelial tumor, expressing mesenchymal-type intermediate filaments, that should be distinguished from other small-cell neoplasms.

Topics: Abdominal Neoplasms; Adult; Antibodies, Monoclonal; Carcinoma; Desmin; Humans; Intermediate Filament Proteins; Keratins; Male; Peritoneum; Vimentin

Topics: Abdominal Neoplasms; Adolescent; Adult; Antibodies, Monoclonal; Cell Transformation, Neoplastic; Child; Desmin; Diagnosis, Differential; Female; Fibroma; Humans; Keratins; Male; Neoplasms, Germ Cell and Embryonal