bromochloroacetic-acid and Leiomyosarcoma

Leiomyosarcoma represents a rather uncommon malignancy, and reports of cases characterized by a renal genesis are particularly rare.. We describe 2 cases of leiomyosarcoma of the kidney, in a 63-year-old woman and in a 53-year-old man, respectively. Both tumors share a common immunohistochemical profile with a strong positivity for smooth muscle actin, a focal positivity for desmin, and negativity for cytokeratins and other markers.. We provide a comparison between our findings and the data available in the literature, and we note an interesting relatively long survival in our patients (10 months for the first case and 20 months for the second case).

Topics: Female; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Leiomyosarcoma; Male; Middle Aged; Soft Tissue Neoplasms

To investigate the clinicopathological features, histogenesis and prognosis of mucinous tubular and spindle cell carcinoma (MTSCC).. Five MTSCCs were studied with histochemical, immunohistochemical staining, electron microscopy, and review of the related literatures.. Four cases of MTSCC were females and one was male. Three patients presented with flank discomfort and two were incidentally found with health examination. In gross examination, the tumors were circumscribed. The cut surface was solid, gray-white, yellow or red. Necrosis was present in one case of high-grade MTSCC. Microscopically, low-grade MTSCC was mainly consisted of tubular, spindle cell and mucinous stroma with relatively bland morphology, and mitoses were rare. While in the high-grade area of one case, the cells were spindle or polymorphic with severe atypia and high mitotic activity, without mucinous stroma and tubular structure. Mucin was positive for Alcian blue. The neoplastic cells were positive for vimentin (5/5), CKpan (5/5), CK7 (5/5), CK19 (5/5), 34betaE12 (1/5), EMA (5/5), E-cadherin (3/5), CD10 (1/5), P504S (5/5), and CAM5.2 (5/5). The Ki-67 index was low (< or = 5%) in the low-grade component, while it was high (15%) in the high-grade component. Ultrastructural study showed short microvilli along glandular lumens. The nuclear membrane was focally invaginated. Four cases were followed up for 3 to 52 months, and recurrence and metastasis were not found.. MTSCC occurs predominantly in females and it is a rare kidney neoplasm. Most of MTSCCs are low-grade and the prognosis is relatively good. However, the patients of high-grade MTSCC should be closely followed up.

Topics: Adenocarcinoma; Adenocarcinoma, Mucinous; Adult; Carcinoma; Carcinoma, Medullary; Carcinoma, Renal Cell; Diagnosis, Differential; Female; Humans; Keratins; Kidney Neoplasms; Leiomyosarcoma; Male; Middle Aged; Mucin-1; Nephrectomy; Racemases and Epimerases; Vimentin

Topics: Actins; Adult; Eyelid Neoplasms; Female; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Vimentin

We describe five patients who recently presented with gross hematuria secondary to inflammatory pseudotumors of the bladder along with a review of the literature. At presentation, four of the five patients were clinically misdiagnosed as malignancies of which two were further believed to be leiomyosarcomas on initial histological examination because of their spindle-cell appearance. Conservative excision either by transurethral resection or partial cystectomy was curative in all cases. The main importance of these rare, benign lesions is to differentiate them from malignant tumors for which they may be mistaken, thus avoiding radical surgery and its attendant complications.

Topics: Adolescent; Adult; Cystectomy; Desmin; Diagnosis, Differential; Diagnostic Errors; Female; Granuloma, Plasma Cell; Hematuria; Humans; Keratins; Leiomyosarcoma; Male; Middle Aged; Urinary Bladder Diseases; Urinary Bladder Neoplasms; Vimentin

Since the concept of malignant fibrous histiocytoma (MFH) was introduced and subsequently popularized in the 1960's and 1970's, it has become widely regarded as the commonest soft-tissue sarcoma of adulthood. Although the initial notion that MFH was a true histiocytic tumor showing faculative fibroblastic differentiation has been disproved, and despite the lack of definable, reproducible diagnostic criteria and considerable immunophenotypic, ultrastructural and karyotypic heterogeneity, MFH is still accepted widely as a discrete clinicopathologic entity. On the other hand several recent studies have expressed considerable doubts about MFH, or at least pleomorphic MFH, as an "entity" and have suggested that it represents a common morphologic manifestation of a host of poorly differentiated sarcomas and, more rarely, other neoplasms. This article reviews the clinicopathologic features of MFH and its established variants in the context of this debate and considers the evidence for and against their continued acceptance as distinct entities or as a cohesive group. We conclude that the pleomorphic, giant cell and inflammatory variants each represent heterogeneous diagnostic groups which are hard to defend as cohesive entities, while the myxoid ("myxofibrosarcoma") and angiomatoid types are distinct, reproducible tumor types.

Topics: Desmin; Fibrosarcoma; Giant Cell Tumors; Histiocytoma, Benign Fibrous; Humans; Keratins; Leiomyosarcoma; Retroperitoneal Neoplasms; Soft Tissue Neoplasms

We report the case of a 94-year-old man with a rapidly growing nodule on the preauricular area, which on histology showed a poorly differentiated spindle cell tumor with negative p63 and p40 antibody immunostains, negative high- and low-molecular-weight cytokeratins albeit for a focal expression of cytokeratin AE1/AE3. Spindle cell melanoma, angiosarcoma, and leiomyosarcoma were excluded. We explore the diagnostic approach to this challenging conundrum. Certain authors have suggested that sarcomatoid carcinoma and atypical fibroxanthoma (AFX) may lie within a spectrum of "sarcoma-like tumors of the head and neck" and that they may all run a similarly indolent clinical course. However, AFX appears to remain a diagnosis of exclusion, and expert consensus is that by definition AFX cannot express any cytokeratin antigens.

Topics: Aged, 80 and over; Biomarkers, Tumor; Carcinoma; Diagnosis, Differential; Face; Hemangiosarcoma; Humans; Keratins; Leiomyosarcoma; Male; Melanoma; Mohs Surgery; Skin; Skin Neoplasms

Primary mucoepidermoid carcinoma (MEC) of the skin is an unusual neoplasm with few cases reported in the English medical literature. It has to be differentiated from adenosquamous carcinoma, usually a high-grade neoplasm with poorer outcome, and metastasis from a primary MEC arising elsewhere in the body. We report a 78-year-old woman with an abdominal skin lesion of recent onset. Histopathological examination revealed a dermal located carcinoma with variable proportions of squamous differentiation and goblet cells. The patient died in a very short time for an unrelated disease. Immunohistochemical study showed staining for cytokeratins (AE1AE3, 7, and 34betaE12), epithelial membrane antigen (EMA), and p63, whereas cytokeratins 18 and 20 and gross cystic disease fluid protein (GCDFP15) were negative. We conclude that primary MEC of the skin is usually a slow-growing neoplasm that should be differentiated from adenosquamous carcinoma. The immunohistochemical staining for p63 is helpful to differentiate primary and metastatic MEC in the skin.

Topics: Aged; Biomarkers, Tumor; Carcinoma, Adenosquamous; Carcinoma, Mucoepidermoid; Diagnosis, Differential; Fatal Outcome; Female; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Liver Neoplasms; Neoplasms, Multiple Primary; Peritoneal Neoplasms; Skin Neoplasms; Trans-Activators; Transcription Factors; Tumor Suppressor Proteins

Cutaneous spindle cell squamous carcinoma is an uncommon variant of squamous cell carcinoma in which keratinocytes infiltrate the dermis as single cells with elongated nuclei rather than as cohesive nests or islands, and signs of keratinization of conventional squamous cell carcinoma are insubstantial or nonexistent. Spindle cell carcinoma must be distinguished from spindle cell/desmoplastic melanoma, cutaneous leiomyosarcoma, atypical fibroxanthoma (AFX), and scar. In instances when there is no definitive evidence of squamous differentiation, immunohistochemical studies may confer diagnostic discrimination. Twenty-four cases consisting of 12 spindle cell squamous cell carcinomas, 3 AFXs, 3 leiomyosarcomas, 3 desmoplastic melanomas, and 3 scars were evaluated with a battery of immunohistochemical stains, with the specificity and sensitivity of each marker calculated. The immunohistochemical battery consisted of S-100, desmin, CD68, and smooth muscle actin and cytokeratins P KER (keratins predominantly of molecular weight 56 and 69 kd) and low-molecular weight keratin (CAM 5.2), AE1/AE3, p63, and 34 beta E12 (CK903). Spindle cell squamous carcinomas were negative for S-100, CD68, smooth muscle actin, and desmin with the exception of 2 cases with weak staining for smooth muscle actin. 34 beta E12 provided positive results for each spindle cell squamous carcinoma. The other cytokeratin stains were less sensitive for spindle cell squamous carcinoma than 34 beta E12. The final immunohistochemical results were as follows: 34 beta E12 (12/12, 100%), p63 (10/12, 80%), AE1/AE3 (8/12, 67%), low-molecular weight keratin (7/12, 58%), and P KER (4/12, 33%). The 3 AFXs were positive for CD68 and negative for all other stains, whereas the 3 leiomyosarcomas stained positively for desmin and smooth muscle actin and negatively for all other stains. The 3 melanomas stained positively for S-100 and negatively for all other immunohistochemistry. The scars were negative for all stains. In conclusion, our study of 34 beta E12 proved most promising in distinguishing spindle cell squamous carcinoma from the histologic mimickers, AFX, spindle cell melanoma, scar, and leiomyosarcoma.

Topics: Adult; Aged; Aged, 80 and over; Biomarkers, Tumor; Carcinoma; Carcinoma, Squamous Cell; Cicatrix; Diagnosis, Differential; Female; Histiocytoma, Benign Fibrous; Humans; Immunohistochemistry; Keratinocytes; Keratins; Leiomyosarcoma; Male; Melanoma; Middle Aged; Predictive Value of Tests; Skin Neoplasms

Topics: Aged; Carcinoma; Diagnosis, Differential; Female; Giant Cells; Hemangioendothelioma; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Liver Neoplasms; Osteoclasts; Thrombosis; Vena Cava, Inferior

To determine the demographic characteristics and distribution of various reported prognostic factors of leiomyosarcoma (LMS) among subjects reporting to histopathology diagnostic centers of Aga Khan University (AKU) all over Pakistan between 2000-2004.. This study analyzed 205 consecutive confirmed cases of LMS received during a period of four years. The data regarding age, sex and size of tumor was obtained from the pathology medical records. Data on grade and positivity of immunohistochemical stains was assessed and all the variables analyzed using SPSS version 12.5.. Of the 205 specimens of LMS, 31 were received as blocks for second opinion. A hundred specimens were multiple fragments, and 74 were intact masses. Of the latter, 40% had clear margins. The mean size of the tumor was 7.23 cms. (95% CI 6.1; 8.4). There were no cases of childhood LMS. Teenage LMS comprised 3% of all tumors. Grade 1 tumors were 16.6% of the total, 56.6% were grade 2, and 8.5% were grade 3. Vimentin positivity was observed in 97% of the tumors, desmin positivity in 56.7%, HHF35 positivity in 81.0%, s-100 positivity in 15.6% and cytokeratin positivity in 11% of the cases. The median age of LMS patients was 48.4 years, the mean age was 48.6 years (95% CI 45.9; 50.8) and male to female ratio was 1:1.2. The malignancy occurred a decade earlier in the females as compared to the males. The mean age of male cases was 52.3 years (95% CI 48.8; 55.8); and of females was 45.2 years (95% CI 41.8; 48.6). The mean age of teenage LMS was 17.2 years (95% CI 15.6; 18.7). The most common symptom was a painless swelling. The most common sites were lower limb (24.4%) and pelvis (24.4%) followed by abdomen (20.6%) and head and neck (12.7%). The least common sites were upper limb and chest (8.0% each). Seventy five percent of the tumors with involved margins were more than 5.0 cms. in size whereas 47% of tumors with free margins were above 5.0 cms. in size. Approximately half the tumors in the males were less than 5.0 cms, as compared with 31.0% in the females. LMS in the extremities was equally common in both genders, but trunk LMS was nearly twice as common in the females.. In our population, leiomyosarcoma occurs at a relatively younger age, has a late presentation; is more common in females as compared to males and usually excised incompletely. No genetic study for LMS has been published in Pakistan; these studies are recommended to determine the biological pattern of LMS in our population.

Topics: Actins; Adolescent; Adult; Age Factors; Aged; Desmin; Female; Hospitals; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Male; Middle Aged; Neoplasm Staging; Prognosis; S100 Proteins; Sex Factors; Vimentin

The expression of desmin, h-caldesmon, calponin, CD10, CD34, CD99, inhibin, and keratin (AE1/3-Cam 5.2) was studied in 10 conventional leiomyomas, 9 highly cellular leiomyomas, 9 epithelioid smooth muscle tumors, 9 leiomyosarcomas, 10 endometrial stromal tumors (4 with smooth muscle metaplasia), and 7 uterine tumors resembling ovarian sex cord tumors (UTROSCTs). c-kit expression was tested in 10 endometrial stromal tumors, 7 UTROSCTs, and 9 leiomyosarcomas. Desmin was positive in almost all smooth muscle tumors except those of epithelioid type, which were positive in only about half of the cases. It also stained areas of smooth muscle differentiation in endometrial stromal tumors and five of seven UTROSCTs. h-caldesmon was positive in almost all nonepithelioid smooth muscle tumors and in areas of smooth muscle differentiation in endometrial stromal tumors; it was positive in only about half of the epithelioid smooth muscle tumors and negative in all UTROSCTs. Calponin was positive in most tumor types. CD10 was positive in nine of 10 endometrial stromal tumors and five of seven UTROSCTs, although very focally in the latter group. It was also expressed, however, in almost all leiomyosarcomas, almost 50% of highly cellular leiomyomas, and rarely in the other smooth muscle tumors. CD34 was negative in the tested tumors with rare exceptions. CD99 and inhibin were positive in four of seven and one of seven UTROSCTs. Keratin positivity was found in most (five of seven) UTROSCTs and occasionally in smooth muscle tumors (seven of 37). c-kit was negative in all endometrial stromal tumors, UTROSCTs, and leiomyosarcomas. The major conclusions of this study are as follows: 1) Pure endometrial stromal tumors are usually desmin negative. 2) In contrast to some previous studies, CD10 expression was often seen in smooth muscle tumors, including most leiomyosarcomas and almost half of highly cellular leiomyomas. As a result, a panel of CD10, h-caldesmon, and desmin should be used and will distinguish endometrial stromal tumors from highly cellular leiomyomas in most cases. 3) In contrast to a previous study, no significant differences in immunoreactivity were seen between h-caldesmon and desmin in tumors with smooth muscle differentiation. 4) The absence of h-caldesmon in UTROSCTs helps separate them from epithelioid smooth muscle tumors. 5) UTROSCTs may express epithelial, stromal, and smooth muscle markers, suggesting divergent differentiation. 6) Our study shows less fr

Topics: Adenosine Triphosphatases; Antigens, CD; Antigens, CD34; Apyrase; Calcium-Binding Proteins; Calmodulin-Binding Proteins; Calponins; Desmin; Female; Humans; Immunohistochemistry; Inhibins; Keratins; Leiomyoma; Leiomyosarcoma; Microfilament Proteins; Neprilysin; Proto-Oncogene Proteins c-kit; Uterine Neoplasms

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

We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). Neoplasms with a nodular growth pattern were characterized by high cellularity and prominent nuclear atypia, and they showed conspicuous mitoses, several necrotic cells, and sometimes extensive necrotic areas. By contrast, most cutaneous leiomyosarcomas with a diffuse growth pattern revealed low cellularity, well-differentiated smooth muscle cells, inconspicuous mitotic figures, and few or no necrotic cells. Immunohistologic investigations revealed all cutaneous leiomyosarcomas to express vimentin and smooth muscle actin. Pan-muscle actin (HHF-35) was also expressed in most cases (15 lesions). However, only 12 lesions showed positive staining for desmin. Remarkable was the expression of cytokeratins in five lesions. Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. Cutaneous leiomyosarcoma may show different immunophenotypes, thus emphasizing the importance of using a large panel of antibodies (smooth muscle actin, HHF-35, desmin, vimentin, cytokeratins, and S-100 protein) in immunohistologic diagnosis. Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases.

Topics: Actins; Adult; Aged; Aged, 80 and over; Desmin; Female; Follow-Up Studies; Humans; Immunohistochemistry; Immunophenotyping; Keratins; Leiomyosarcoma; Male; Middle Aged; Mitotic Index; Necrosis; Prognosis; Retrospective Studies; Skin Neoplasms; Vimentin

Ten renal smooth muscle tumors (seven leiomyomas, three leiomyosarcomas) and an unusual smooth muscle-invested renal cell carcinoma were studied for HMB-45 reactivity. All leiomyomas strongly expressed at least one, and most expressed three, smooth muscle markers (desmin, MSA, SMSA) and were negative for two cytokeratin markers (BDK, AE1). Six of seven leiomyomas and the smooth muscle investment of a renal carcinoma contained a population of cells strongly positive for HMB-45. A total of 15 blocks from the 6 HMB-45-positive leiomyomas were studied, and no adipocytes or abnormal vessels suggestive of angiomyolipoma were identified. The six HMB-45-positive leiomyomas seemed to arise from the renal capsule, whereas a seventh leiomyoma, which was negative for HMB-45, seemed to arise from the renal pelvis. Three leiomyosarcomas were also studied, which strongly expressed at least one smooth muscle marker and contained myofilaments at electron microscopic examination. No cytokeratin reactivity and no HMB-45-positive cells were detected in these leiomyosarcomas nor in the normal renal capsule adjacent to leiomyomas. This study showed that HMB-45-positive cells are detectable in a population of cells in some smooth muscle tumors, particularly in those that appear to arise from the renal capsule.

Topics: Actins; Aged; Antigens, Neoplasm; Desmin; Female; Humans; Immunohistochemistry; Keratins; Kidney Neoplasms; Leiomyoma; Leiomyosarcoma; Male; Melanoma-Specific Antigens; Middle Aged; Neoplasm Proteins

Even when clinical data strongly suggest the presence of a metastatic neoplasm in the breast, this occurrence almost invariably raises great problems in diagnostic pathology. Both cases presented here had a well-recognized primitive neoplasm located elsewhere. Nonetheless, great importance was given to the application of ancillary techniques; the immunostains for "breast discriminants"--GCDFP15, HMFG1, and HMFG2--on tissue sections helped the recognition of a metastatic renal cell carcinoma; and the stains for S100 protein, smooth muscle actin, cytokeratins, and neurofilaments on cytologic material allowed the identification of a metastatic mediastinal leiomyosarcoma.

Topics: Actins; Adult; Biopsy, Needle; Breast Neoplasms; Carcinoma, Renal Cell; Fatal Outcome; Female; Humans; Keratins; Kidney Neoplasms; Leiomyosarcoma; Mediastinal Neoplasms; Middle Aged; S100 Proteins; Vimentin

Primary subcutaneous leiomyosarcoma is a rare neoplasm composed of plump, elongated spindle cells arranged in interweaving fascicles.. Differential diagnosis might be facilitated by the use of immunohistochemistry.. A case of subcutaneous leiomyosarcoma was investigated histologically on paraffin-embedded tissue and immunohistochemistry was performed following standard procedures.. The case reported exhibited light microscopic features and an immunophenotype characteristic of leiomyosarcoma. However, the intermediate filament desmin could only be found on a small number of tumor cells.. Expression of these markers might vary considerably and immunoperoxidase stainings need to be carefully evaluated. Utilization of several antibodies directed against different desmin epitopes might be advantageous.

Topics: Aged; Desmin; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Immunophenotyping; Keratins; Leiomyosarcoma; Soft Tissue Neoplasms; Thigh; Vimentin

Atypical fibroxanthoma is a bizarre, cytologically malignant but usually clinically benign, lesion which typically arises in sun-damaged skin of the head and neck region in the elderly. Classically, its morphology is said to represent the dermal counterpart of pleomorphic malignant fibrous histiocytoma. We have identified 10 cases of a more monomorphic spindle-celled, fascicular variant which, paradoxically, was often mistaken for a clinically malignant lesion because it lacked the pleomorphism of conventional atypical fibroxanthoma. These tumours all arose in the head and neck region as polypoid lesions in the elderly. The tumours were confined to the dermis, often had an epidermal collarette, showed an eosinophilic fascicular morphology and were highly mitotic. All 10 were vimentin positive and five showed very focal actin positivity. Desmin, keratin and S-100 protein were negative in all cases. The clinical course was benign in all cases, justifying their accurate recognition. The principal differential diagnoses are spindle cell squamous carcinoma, spindle cell melanoma and leiomyosarcoma. Immunohistochemistry plays a key role in this distinction.

Topics: Aged; Aged, 80 and over; Carcinoma, Squamous Cell; Desmin; Diagnosis, Differential; Female; Fibrosarcoma; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Male; Melanoma; Middle Aged; Mitosis; S100 Proteins; Skin Neoplasms; Vimentin

The authors identified five leiomyosarcomas (LMS) in a review of 13 nonmatrix-producing spindle cell sarcomas of bone. Only two were initially recognized as LMS; the others had been diagnosed as malignant fibrous histiocytoma (two) and fibrosarcoma (one). The patients, four of whom were women, ranged in age from 32 to 70 years. Sites included proximal humerus (two), distal femur (two), and rib (one). All tumors presented with clinical and radiographic features consistent with a diagnosis of primary bone neoplasms, although one probably represented a solitary metastasis from a primary uterine LMS. Radiographs showed lytic bone destruction with a moth-eaten appearance, and three cases had soft tissue extension. Histologically, all tumors showed broad, interlacing fascicles of spindle cells with pleomorphic nuclei, frequent mitoses, and necrosis. Two cases had a focal storiform pattern and bizarre multinucleated cells, and two other cases had focally prominent osteoclast-like giant cells. Extensive immunoreactivity for muscle actin was seen in all cases and for desmin in three. In each case, electron microscopy showed definite smooth muscle differentiation including cytoplasmic filaments with densities. At this writing, two patients are free of disease (including the patient with a presumed metastasis), one is alive with locally recurrent disease, and two are dead of disease. Experience suggests that LMS of bone is a distinct clinicopathologic entity that may be more common than previously recognized. Application of immunohistochemistry and electron microscopy to nonmatrix-producing bone sarcomas should facilitate diagnosis of additional cases.

Topics: Actins; Adult; Aged; Bone Neoplasms; Combined Modality Therapy; Cytoplasm; Desmin; Female; Femur; Humans; Humerus; Immunohistochemistry; Keratins; Leiomyosarcoma; Male; Middle Aged; Neoplasm Recurrence, Local; Radiography; Ribs; Vimentin

The presence of individual keratin polypeptides and desmoplakins was immunohistochemically studied in 25 spindle cell sarcomas of different types using acetone-fixed frozen sections. Results revealed that keratins 8 and 18 were present in a high number of tumors: 9 of 9 synovial sarcomas, 5 of 7 leiomyosarcomas, 5 of 5 malignant schwannomas, and 1 of 4 undifferentiated spindle cell sarcomas. In addition to keratins 8 and 18, the glandular component of synovial sarcoma showed prominent reactivity with antibodies to keratins 7 and 19. Also the glandular epithelial cells in synovial sarcoma showed desmoplakin immunoreactivity preferentially in a luminal distribution, but desmoplakin was absent in other spindle cell sarcomas. Furthermore keratin 13 was seen focally in 4 of 9 synovial sarcomas. In contrast, keratins 7, 13, and 19 were practically absent in leiomyosarcomas, malignant schwannomas, and undifferentiated spindle cell sarcomas. The widespread presence of keratins 8 and 18 in various spindle cell sarcomas may reflect aberrant keratin expression in mesenchymal cells, previously described in cultured transformed fibroblasts. The presence of keratins 7 and 19 and desmoplakin is highly associated with morphologically observable epithelial differentiation restricted to synovial sarcoma among spindle cell sarcomas.

Topics: Cytoskeletal Proteins; Desmoplakins; Desmosomes; Humans; Keratins; Leiomyosarcoma; Neurilemmoma; Peptides; Peripheral Nervous System Neoplasms; Sarcoma; Sarcoma, Synovial; Soft Tissue Neoplasms

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

Six cases of primary hepatic carcinomas with a significant amount of sarcomatoid elements were examined by using immunohistochemical stainings. Four of the six cases were associated with ordinary hepatocellular carcinoma (HCC), one with cholangiocellular carcinoma (CCC), and one with mixed HCC and CCC. Alpha-fetoprotein and alpha-1-antitrypsin were negative in sarcomatoid cells of all cases; vimentin stained positively in sarcomatoid tumor cells in two of the six cases; and cytokeratin (CK8) was detected in five cases. The CK8 was not detected in tumor cells of two cases of hepatic angiosarcoma, two of metastatic leiomyosarcomas, and one of metastatic fibrosarcoma, although vimentin stained positively in all these true sarcomas. It was concluded that sarcomatoid dedifferentiation of liver carcinomas might derive from both HCC and CCC. In addition CK8 might be an excellent marker to make a differential diagnosis of sarcomatoid cancers from true metastatic or primary sarcomas of the liver.

Topics: Aged; alpha-Fetoproteins; Carcinoembryonic Antigen; Carcinoma, Hepatocellular; Cell Transformation, Neoplastic; Diagnosis, Differential; Female; Fibrosarcoma; Hemangiosarcoma; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Liver Neoplasms; Male; Middle Aged; Sarcoma; 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

The expression of fine and intermediate cytofilaments in 10 cutaneous and seven subcutaneous leiomyosarcomas was studied immunohistochemically. All the tumors contained tumor cells which showed a positive immunoreactivity for desmin in formaldehyde-fixed and paraffin-embedded sections, but none of the seven anti-desmin antibodies used alone produced a distinct positive staining in all the tumors. A lack of correspondence in terms of immunoreactivity between tumor cells and the supposed muscle of origin was observed, especially in the subcutaneous leiomyosarcomas. In all cases, antibodies to muscle-specific and smooth muscle-specific actin were found to produce a positive staining in both the tumors and the supposed muscle of origin. Vimentin was detected in 8/10 cutaneous and 4/7 subcutaneous leiomyosarcomas, while the supposed muscle of origin was positive in 3/10 and 7/7 cases, respectively. Four of the cutaneous and three of the subcutaneous leiomyosarcomas contained tumor cells which stained positively for cytokeratins, while the supposed muscle of origin showed no positivity. It thus appears that a phenotypic shift in terms of vimentin and cytokeratin expression occurs in the tumor cells of cutaneous and subcutaneous leiomyosarcomas compared with the supposed muscle of origin. It is recommended that more than one monoclonal anti-desmin antibody is used to characterize these tumor entities. It is also concluded that the immunoreactivity for muscle-specific actins in superficial leiomyosarcomas is more constant, although less specific, than that of desmin and that the demonstration of the simultaneous expression of muscle-specific actins and desmin is helpful.

Topics: Actin Cytoskeleton; Actins; Aged; Antibodies; Antibodies, Monoclonal; Desmin; Female; Humans; Immunohistochemistry; Intermediate Filaments; Keratins; Leiomyosarcoma; Male; Middle Aged; Skin Neoplasms

A case of dedifferentiated leiomyosarcoma of the uterus was examined using immunohistochemistry. The tumor arose in the myometrium, and was a whitish large nodule with hemorrhage and necrosis. Histologically it was a well differentiated leiomyosarcoma with foci showing epithelioid pattern, and in part resembling malignant fibrous histiocytoma (MFH) and giant cell tumor (GCT). Additionally, small round neoplastic cells arranged in an alveolar manner, simulating alveolar rhabdomyosarcoma, were seen in some areas. Neoplastic cells in well differentiated areas expressed desmin, muscle-specific actin and LeuM1, whereas those in epithelioid and poorly differentiated areas lacked these antigens. Instead, tumor cells in epithelioid and small round cell areas were positive for keratin. Interestingly, most tumor cells in well differentiated, epithelioid and small round cell areas were also positive for MB1. However, tumor cells in GCT- and MFH-like areas reacted with none of the antibodies used. Ultrastructurally, some tumor cells possessed various amounts of microfilaments with or without dense patches, whereas others lacked them. These findings suggest that the divergent antigen expression was attributable to different levels of differentiation, and that poorly differentiated components had lost their native features.

Topics: Actins; Antigens, Differentiation, Myelomonocytic; Desmin; Female; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Microscopy, Electron; Middle Aged; Uterine Neoplasms; Vimentin

An autopsy case of high-grade endometrial stromal sarcoma (ESS) is reported. Immunohistochemical study of the ESS was done in comparison with carcinosarcoma (CS), leiomyosarcoma (LMS) and normal endometrium in the uterus in order to trace the origin of ESS, which is a point of some controversy. Co-expression of keratin and vimentin and/or desmin positivity in several elements of CS and LMS, and glandular or stromal tissues in normal endometrium made it difficult immunohistochemically to be certain of the origin of ESS.

Topics: Antigens; Antigens, Differentiation; Autopsy; Carcinosarcoma; CD57 Antigens; Desmin; Endometrium; Female; Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Middle Aged; Phosphopyruvate Hydratase; S100 Proteins; Sarcoma; Uterine Neoplasms; Vimentin

Forty-six smooth muscle tumors, including 35 of gastrointestinal origin, were studied immunohistochemically for the localization of cytokeratin using a variety of monoclonal antibodies. In formalin-fixed, paraffin-embedded material, six of 40 leiomyomas, two of five leiomyosarcomas and one leiomyoblastoma were immunoreactive for cytokeratin in a few tumor cells. A proportion of non-neoplastic smooth muscle cells also exhibited a positive reaction. In fresh frozen sections of one gastric leiomyosarcoma, a high percentage of tumor cells were reactive with nine of the eleven anti-cytokeratin monoclonal antibodies examined. Cytokeratin-positive cytoplasmic filaments were further demonstrated by immunoelectron microscopy. By immunoblot analysis, an extract of this immunohistochemically cytokeratin-positive leiomyosarcoma showed a distinct band at the same position as an extract of pancreas, whereas no bands were seen in an extract of a cytokeratin-negative esophageal leiomyosarcoma. These immunohistochemical and immunoblotting findings indicate that a certain subset of smooth muscle neoplasms express genuine cytokeratin filaments.

Topics: Humans; Immunoblotting; Immunohistochemistry; Keratins; Leiomyoma; Leiomyosarcoma; Microscopy, Electron; Neoplasms, Muscle Tissue

Specimens of neoplastic tissues from 19 dogs and 4 cats were examined immunohistochemically for intermediate filament expression, using commercially available antibodies. Staining was observed in a wide range of tumor tissues and in normal internal controls by use of antibodies to vimentin, desmin, glial fibrillary acidic protein, and low and high molecular weight cytokeratins. Intermediate filament expression was found to be consistent with light and/or electron microscopic findings, and hence believed to be an accurate indicator of tumor histogenesis in cats and dogs. Three fixatives were evaluated for their relative abilities to preserve antigenicity. Absolute alcohol was superior to B5 fixative and both were superior to formalin. Some tissues that clearly displayed intermediate filament antigens with alcohol and B5 fixative failed to stain when fixed in formalin.

Topics: Adenocarcinoma; Animals; Astrocytoma; Cat Diseases; Cats; Cytoskeleton; Desmin; Dog Diseases; Dogs; Immunohistochemistry; Intermediate Filament Proteins; Intermediate Filaments; Keratins; Leiomyosarcoma; Melanoma; Neoplasms; Neurilemmoma

Immunoperoxidase techniques for S-100 protein, keratin, cytokeratin, vimentin and desmin were applied to 65 canine skin tumours. These included eight squamous cell carcinomas, eight fibrosarcomas, eight melanomas, eight mastocytomas, eight haemangiosarcomas, eight leiomyosarcomas, five liposarcomas and 12 poorly differentiated tumours. Consistent results were obtained within each group. Cytokeratin and keratin immunoreactivity was detected only in squamous cell carcinomas. Vimentin was present in fibrosarcomas, melanomas, haemangiosarcomas, mastocytomas, leiomyosarcomas and liposarcomas. S-100 protein immunoreactivity was detected in melanomas, haemangiosarcomas, liposarcomas and leiomyosarcomas. Only leiomyosarcomas were positive for desmin. According to these results the 12 anaplastic tumours were diagnosed either as carcinomas, fibrosarcomas or malignant melanomas.

Topics: Animals; Carcinoma, Squamous Cell; Desmin; Dog Diseases; Dogs; Fibrosarcoma; Hemangiosarcoma; Immunoenzyme Techniques; Keratins; Leiomyosarcoma; Liposarcoma; Mast-Cell Sarcoma; Melanoma; S100 Proteins; Skin Neoplasms; Vimentin

Leiomyosarcomas of the heart are rare, with only 12 cases reported in the literature. An example of the epithelioid variant of leiomyosarcoma is described. The tumour cells expressed vimentin, desmin, muscle-specific actin and smooth muscle-specific actin. In addition, they were also labelled by monoclonal and polyclonal antibodies to cytokeratin intermediate filaments and displayed cell junctions at the ultrastructural level, features which highlight the potential pitfalls in the application of immunohistochemistry and electron microscopy in the diagnosis of poorly differentiated tumours.

Topics: Female; Heart Atria; Heart Neoplasms; Humans; Immunohistochemistry; Infant, Newborn; Keratins; Leiomyosarcoma; Microscopy, Electron; Middle Aged

Thirty-three leiomyosarcomas (LMS), which were verified by electron microscopy and by desmin and muscle actin immunoreactivity, were immunohistochemically evaluated for the presence of cytokeratin and epithelial membrane antigen (EMA) with monoclonal antibodies. None of the tumors showed any epithelial component by structure; all were homogeneous spindle cell neoplasms. Cytokeratin immunoreactivity was found in 14 of 33, and EMA in 20 of 33 LMS, usually in a large number of tumor cells. One case was confirmed as cytokeratin-positive in frozen sections with four different monoclonal antibodies. These results show that immunoreactivity for epithelial markers can be present in pure smooth-muscle sarcomas. Thus, such immunoreactivity cannot be regarded as a specific feature of carcinomas, synovial sarcoma, or epithelioid sarcoma, which are already known to be cytokeratin- and EMA-positive.

Topics: Actins; Antibodies, Monoclonal; Antigens, Neoplasm; Biomarkers, Tumor; Desmin; Fluorescent Antibody Technique; Humans; Immunoenzyme Techniques; Immunohistochemistry; Keratins; Leiomyosarcoma; Membrane Glycoproteins; Mucin-1

Topics: Humans; Immunohistochemistry; Keratins; Leiomyosarcoma; Male; Middle Aged; Muscle, Smooth; Neoplasms, Muscle Tissue

Cytokeratins are a set of 19 proteins that together constitute the class of intermediate filament protein expressed by epithelial cells and tumors. Using a panel of 9 different monoclonal anti-cytokeratin antibodies, the authors have performed immunocytochemistry on methanol-fixed, frozen sections and methacarn-fixed, paraffin-embedded tissue of human myometrial specimens. Anomalous cytokeratin expression (ACE) by smooth muscle cells was found in all specimens. Immunoblots of this tissue confirmed the presence of cytokeratin 19, and possibly 8. In addition, immunocytochemical studies demonstrated ACE in human fetal tissues within the intestinal muscularis and the heart, especially in the region of the aortic outflow tract, and in 8 of 19 cases of leiomyosarcoma from adults. Indirect immunofluorescence studies were also performed on cells explanted from myometrial tissue; the overwhelming majority of cells derived from these cultures were smooth muscle cells as verified by expression of muscle actins, and a subpopulation of these cells was found to be cytokeratin-positive. ACE was confirmed in vitro by double labeling experiments demonstrating simultaneous expression of muscle actins and cytokeratins within the same cell. The significance of this smooth muscle cell ACE is unknown, but it may be a phenotypic marker of smooth muscle in a proliferative state. ACE could be a source of confusion in the immunocytochemical analysis of poorly differentiated malignancies if a complete panel of antibodies is not employed.

Topics: Cells, Cultured; Electrophoresis, Polyacrylamide Gel; Epithelium; Female; Fetus; Humans; Immunochemistry; Immunologic Techniques; Keratins; Leiomyoma; Leiomyosarcoma; Muscle, Smooth; Myometrium; Uterus

We report here one case of post-irradiation leiomyosarcoma. The diagnosis of this was confirmed using anti-intermediate filament antibodies: tumor cells were positive for anti-vimentin and anti-desmin but negative for anti-prekeratin and anti-epithelial membrane antigen. The positive staining with anti-desmin clearly indicates a muscular origin although the tumor cells were not stained by two anti-actin antibodies, one directed against alpha-smooth muscle actin and the other against alpha-striated muscle actin. Irradiation was motivated by a wrong diagnosis of breast carcinoma when the patient was 13 year-old. The laps of time separating irradiation and the occurrence of the leiomyosarcoma was 13 years. The total dose was 6,000 rads. This is the ninth case of post-irradiation leiomyosarcomas reported in the literature.

Topics: Actin Cytoskeleton; Adult; Antibodies, Monoclonal; Desmin; Female; Fluorescent Antibody Technique; Humans; Intermediate Filaments; Keratins; Leiomyosarcoma; Membrane Proteins; Mucin-1; Muscle, Smooth; Neoplasms, Radiation-Induced; Radiotherapy; Vimentin

A series of 3 benign and 10 malignant smooth muscle (SM) neoplasms and of 2 malignant fibrous histiocytomas was examined by light microscopy, transmission electron microscopy, two-dimensional gel electrophoresis (2D-GE) and indirect immunofluorescence, using polyclonal monospecific or monoclonal antibodies to desmin, vimentin, cytokeratin, alpha-SM and alpha-sarcomeric (alpha-SR) actins. Benign neoplasms displayed typical light-microscopic features of SM, whereas leiomyosarcomas demonstrated variations in their histologic pattern. In 6 sarcomas, light microscopy suggested a SM differentiation, whereas in the other 4, a predominant nondistinctive spindle-cell pattern was observed. By transmission electron microscopy, all 13 neoplasms showed the minimal essential features of SM differentiation. Immunofluorescence disclosed heterogeneity of cytoskeletal protein expression: 5 neoplasms (3 benign and 2 malignant well-differentiated) expressed desmin, vimentin, and alpha-SM-actin; 2 malignant neoplasms expressed desmin and vimentin; 1 malignant neoplasm expressed desmin, vimentin and alpha-SR actin; 1 malignant neoplasm expressed vimentin and alpha-SR actin; and 4 malignant neoplasms expressed vimentin alone. By 2D-GE, 3 benign and 4 malignant SM neoplasms expressed alpha, beta, and gamma actins, and the remaining expressed only beta and gamma actins. The presence of alpha-SM actin in all benign neoplasms and in 2 well-differentiated leiomyosarcomas suggests that this actin isoform reflects a high degree of cellular differentiation. In 2 leiomyosarcomas, alpha-SR actin was detected by immunofluorescence, which suggested a skeletal muscle differentiation of these neoplasms. This study supports the assumption that leiomyosarcomas represent a heterogeneous group of neoplasms and furnishes new criteria for their characterization.

Topics: Actins; Antibodies, Monoclonal; Cell Differentiation; Desmin; Fluorescent Antibody Technique; Histiocytoma, Benign Fibrous; Humans; Intermediate Filament Proteins; Keratins; Leiomyosarcoma; Microscopy, Electron; Muscle, Smooth; Neoplasms, Muscle Tissue; Sarcoma; Vimentin

Formalin fixed and paraffin wax embedded tissue from 13 leiomyosarcomas, three leiomyoblastomas, five leiomyomas and fresh tissue from one leiomyosarcoma and one leiomyoma was studied. Tumours were stained by the avidin-biotin-peroxidase technique with antibodies to desmin, vimentin, cytokeratins and S-100 protein. Neoplastic cells in all the cases were positive for desmin and vimentin but were negative for S-100; antibody CAM 5.2 gave strong reactivity with tumour cells of 12 leiomyosarcomas and all leiomyomas, but failed to stain the leiomyoblastomas; LP34 weakly stained two leiomyosarcomas and one leiomyoblastoma. Immunoblot studies using whole cell extracts of one leiomyosarcoma and one leiomyoma respectively, showed 55-58 kd and 35-38 kd bands reactive with monoclonal antibody CAM 5.2. However, no cytokeratin components were demonstrated when cytoskeletal extracts of the same tissue samples were immunoblotted with CAM 5.2. The implications of these findings for the diagnostic histopathologist are discussed.

Topics: Adult; Antibodies, Monoclonal; Desmin; Female; Fetus; Histocytochemistry; Humans; Immunochemistry; Keratins; Leiomyoma; Leiomyosarcoma; Muscle, Smooth; Neoplasms, Muscle Tissue; S100 Proteins; Vimentin

The expression of cytokeratin intermediate filaments by a tumour has been accepted as evidence of an epithelial origin. Although there have been anecdotal reports of cytokeratin expression within tissues and neoplasms of non-epithelial origin, particularly muscle, there have been no comprehensive studies of its frequency and distribution. In order to investigate this we have studied 51 cases of normal smooth muscle and benign and malignant smooth muscle tumours using a panel of monoclonal antibodies against a range of intermediate filaments (cytokeratins, desmin and vimentin). Cytokeratin expression was noted overall in 50% of normal, benign and malignant smooth muscle tissues. Such expression tended to have a focal or patchy distribution. No case expressed cytokeratins in the absence of both desmin and vimentin. The implication of these findings for diagnostic immunocytochemistry is that intermediate filaments alone are not completely reliable markers of tumour histogenesis and should be used as part of a larger panel of monoclonal antibodies.

Topics: Desmin; Female; Gastrointestinal Neoplasms; Histocytochemistry; Humans; Keratins; Leiomyoma; Leiomyosarcoma; Muscle, Smooth; Myometrium; Neoplasms, Muscle Tissue; Skin Neoplasms; Uterine Neoplasms; Vimentin

We studied the expression of cytoskeletal intermediate filaments in different types of ovarian and uterine sarcomas and carcinomas. In both uterine and ovarian leiomyosarcomas, in endometrial stromal sarcomas, and also in ovarian sarcomas, most tumor cells appeared to be positive for desmin, the muscle type of intermediate filament protein. In most of the tumors, vimentin was present only in some neoplastic cells and in the vascular endothelia. Interestingly, both uterine and ovarian malignant mixed mesodermal tumors appeared to express several types of intermediate filaments, most of the stromal cells being positive for vimentin or desmin, and the epithelial component expressing keratin. The results show that most of the sarcomatous tumors of the ovary and uterus express mainly muscle type of intermediate filament protein. The results also demonstrate the ability of cells of mesodermal origin to express epithelial cytoskeleton markers--cytokeratins.

Topics: Adenocarcinoma; Cystadenocarcinoma; Desmin; Female; Histocytochemistry; Humans; Intermediate Filament Proteins; Keratins; Leiomyosarcoma; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Sarcoma; Uterine Neoplasms; Vimentin