1/24. Proximal-type epithelioid sarcoma in the pelvic soft tissues.A case of proximal-type epithelioid sarcoma arising in the pelvic soft tissues of a 46-year-old man is presented. The tumor showed a predominantly epithelioid component with rhabdoid features, cord-like arrangement, small nests, abundant eosinophilic cytoplasm containing intracytoplasmic, paranuclear, hyaline-like globules, large vesicular nuclei, and prominent nucleoli. There were foci with alveolar arrangement and a spindle-cell proliferation. The tumor cells were positive for cytokeratin CAM5.2, vimentin, epithelial membrane antigen, and CD34. flow cytometry showed a diploid dna content with high S-phase fraction. The patient had pelvic lymph-node metastases. He died of the disease 5 months after diagnosis. It is important to include proximal-type epithelioid sarcoma in the differential diagnosis of pelvic tumors.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
2/24. Pleomorphic liposarcoma of a young woman following radiotherapy for epithelioid sarcoma.A case of a metachronous epithelioid sarcoma and pleomorphic liposarcoma in a young woman is described. The first tumor was an epithelioid sarcoma (ES) with focal rhabdoid features localised in the left calf while the second lesion developed seven years later in the same region was diagnosed as pleomorphic liposarcoma resembling myxofibrosarcoma ( myxoid variant of malignant fibrous histiocytoma ) predominantly composed of moderately differentiated spindle cells. Multiple foci of uni- and plurivacuolated lipoblasts were seen. Following the resection of ES the patient received 57 Gy radiation to the region, therefore we regarded the second tumor as a radiation induced liposarcoma. A further interesting feature of this case is that the development of pleomorphic liposarcoma preceded by 6 months the solitary right parabronchial metastasis of ES and after 4 months of metastasectomy a third tumor developed at the site of the first lesion. This tumor showed dedifferentiation toward pleomorphic malignant fibrous histiocytoma. Our case represents a unique case of postirradiation liposarcoma developed on the base of ES.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
3/24. Fine-needle aspiration cytology of "proximal-type" epithelioid sarcoma.The cytologic and immunocytologic findings in a case of recurrent "proximal-type" epithelioid sarcoma (ES) of the vulva are presented. This is a recently described neoplasm that differs clinically and morphologically from conventional ES. Cytologic smears showed a dissociated population of large, atypical neoplastic cells with bi- and multinucleated cells, abundant cytoplasm, and rhabdoid-like morphology. Due to its different clinical management it must be differentiated from metastatic carcinoma and melanoma. From a practical perspective, its differentiation from other epithelial-like sarcomas is less important. In conclusion the cytopathologic findings of "proximal-type" ES show a good correlation with histopathology, permitting the diagnosis of recurrences and metastases. When accompanied by adequate clinical information and ancillary studies, a specific preoperative recognition seems possible.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
4/24. Aggressive uterine sarcoma with rhabdoid features: diagnosis by peritoneal fluid cytology and absence of INI1 gene mutation.We report a primary uterine sarcoma with classic histologic, immunohistochemical, and ultrastructural features of a malignant extrarenal rhabdoid tumor (MERT). It arose in a 71-year-old woman who presented with postmenopausal bleeding, ascites, and a right pelvic mass. Malignant cells with rhabdoid morphology were identified by cytologic examination of the peritoneal fluid. Exploratory laparotomy revealed a 10-cm right adnexal mass and disseminated peritoneal tumor. Pathologic study showed diffuse expansion of the endometrial stroma by rhabdoid-like cells with transmural infiltration of the myometrium and extensive involvement of uterine serosa and right ovary by tumor. Neoplastic cells were immunoreactive for vimentin, cytokeratin, and epithelial membrane antigen, and cytoplasmic whorls of intermediate filaments were observed by electron microscopy. fluorescence in situ hybridization (FISH) studies with chromosome 22-specific probes showed no loss of the INI1 gene, and no coding sequence mutation was identified.- - - - - - - - - - ranking = 7keywords = rhabdoid (Clic here for more details about this article) |
5/24. Adenocarcinomas of the gastrointestinal tract with prominent rhabdoid features.rhabdoid tumor, first described in kidneys of infants and children, is an aggressive tumor that has been reported in several extrarenal locations. Gastrointestinal tumors with rhabdoid features are extremely rare. The effect of the rhabdoid phenotype on the aggressiveness of gastrointestinal tumors remains unclear. We present four cases of rhabdoid tumors of the gastrointestinal tract involving the esophagus, stomach, and small intestine and discuss the clinicopathologic, immunohistochemical, and ultrastructural features. In the four cases reported herein, the patients' ages ranged from 52 to 73 years, and tumor size ranged from 3.8 to 13 cm in greatest dimension. The noncohesive rhabdoid cells exhibited an eccentric nucleus with a paranuclear inclusion, which was shown by electron microscopic examination to be composed of intermediate filaments. On immunohistochemical staining, the tumor cells were positive for vimentin and cytokeratin. Three patients developed distant metastasis shortly after diagnosis and died of disease within 2 to 10 months after initial presentation. A retrospective review of outcomes of the current cases and previously published literature showed that 12 (75%) of the 16 patients died within 6 months of presentation. Recognition of the rhabdoid phenotype in gastrointestinal tract neoplasms is important because this feature is associated with poor prognosis and unresponsiveness to conventional therapy.- - - - - - - - - - ranking = 9keywords = rhabdoid (Clic here for more details about this article) |
6/24. Proximal-type epithelioid sarcoma: case report and result of comparative genomic hybridization.BACKGROUND: Epithelioid sarcoma is a rare mesenchymal neoplasm. Recently, a more aggressive, so-called "proximal type" epithelioid sarcoma has been described. CLINICAL CASE: A 40-year-old-woman presented with 5 x 4 cm, erythematous, indurated, non-movable, painful mass on the pubic area. Histopathology demonstrated diffuse tumor-cell infiltration into the subcutaneous and fascia, which was consisted of prominent epithelioid cells and scattered rhabdoid cells. A multinodular growth pattern or granulomatous appearance with central necrosis was not observed. The tumor cells showed positive reactions for vimentin, cytokeratin (AE1/AE3), and CD34. Despite the surgery, left inguinal mass with lymphadenopathy occurred one month later. We also carried out comparative genomic hybridization (CGH) with tumor cells. CGH revealed chromosomal gain of 5q32-qter, 12q24-qter, and 22q. CONCLUSION: We report a case of proximal-type of epithelioid sarcoma, which showed the chromosomal gains of 5q32-qter, 12q24-qter, and 22q by CGH.- - - - - - - - - - ranking = 1keywords = rhabdoid (Clic here for more details about this article) |
7/24. "Proximal-type" epithelioid sarcoma in a young girl.Epithelioid sarcoma is a rare, malignant soft-tissue neoplasm occurring on the distal extremities of young adults. Its histologic features consist of a proliferation of rounded to plump, spindle-shaped cells with abundant eosinophilic cytoplasm around areas of necrosis, simulating a granulomatous process. Recently a different type of epithelioid sarcoma has been described and named "proximal-type" epithelioid sarcoma. It is found mostly in the pelvic and perineal areas of young to middle-aged adults. On microscopic examination, the tumor is characterized by a proliferation of epithelioid-like cells with rhabdoid features in the absence of a granuloma-like pattern, and immunohistochemically it coexpresses vimentin, cytokeratin, epithelial membrane antigen, and CD34. As we found no report of proximal epithelioid sarcoma in the dermatologic literature, we describe an additional case in a 12-year-old girl. This type of tumor appears to be somewhat more aggressive than the classical, distal type and it should be considered in the differential diagnosis of tumors showing rhabdoid features, in particular extrarenal malignant rhabdoid tumor.- - - - - - - - - - ranking = 3keywords = rhabdoid (Clic here for more details about this article) |
8/24. Fine needle aspiration cytology of primary proximal-type epithelioid sarcoma of the perineum: a case report.BACKGROUND: Epithelioid sarcoma is a malignant soft tissue tumor of unknown histogenesis. We describe the cytologic findings in a case of primary proximal-type epithelioid sarcoma of the perineum and results of an immunofluorescence analysis of rhabdoid cells from this tumor. To the best of our knowledge, the 3-color immunofluorescence features of proximal-type epithelioid sarcoma have never before been reported. CASE: An 8-cm-diameter mass with a 2.5-cm ulcer was found in the perineum of a 36-year-old man. After excision of the tumor, histopathologic examination of the resected specimen suggested a diagnosis of proximal-type epithelioid sarcoma. Fine needle aspiration cytology showed numerous rhabdoid cells with globular intracytoplasmic inclusions. A few isolated cells and polygonal cells were also observed in the smears. Three-color immunofluorescence analysis indicated that the intracytoplasmic inclusions in the rhabdoid cells were positive for cytokeratin, vimentin and CD34. The cytoplasmic staining pattern differed between rhabdoid and epithelioid sarcoma cells. CONCLUSION: Immunofluorescent staining of rhabdoid cells from a primary perineal proximal-type epithelioid sarcoma revealed an uneven distribution of cytokeratin in intracytoplasmic inclusions, with the highest concentration at the periphery of the inclusions.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
9/24. Proximal-type epithelioid sarcoma: report of two cases in the perineum: differential diagnosis and review of soft tissue tumors with epithelioid and/or rhabdoid features.The authors report two cases of perineal proximal-type epithelioid sarcoma in middle-aged men, age 51 and 43 years old. Both tumors were located in the right side. In the first patient a 7.5-cm, well-encapsulated tumor was completely excised. The second patient was a referral case with incomplete excision, but the computed tomography scan and magnetic resonance imaging showed a 14-cm nonencapsulated tumor involving the soft tissues of the inner thigh and perineum, as well as metastasis in right inguinal and retroperitoneal lymph nodes. Both neoplasms had a predominant solid pattern alternating with occasional discohesive areas. Both were composed of large oval to polygonal cells with vesicular nuclei, conspicuous nucleoli, and amphophilic to eosinophilic cytoplasm. Rhabdoid phenotype was identified in the second case only. The first neoplasm displayed 15% necrosis, 7 mitoses per 10 high-power field, focal vascular invasion, and no extracapsular invasion. The other exhibited 60% necrosis, 12 mitoses per 10 high-power fields, extensive vascular invasion, no distinct capsule, and invasion of the surrounding fatty tissue. Both were positive for vimentin, cytokeratin, epithelial membrane antigen, and CD34. Muscle-specific actin was negative in the first case and focally positive in the second. CD56 was positive in the second case and negative in the first case. desmin, CD45, CD30, factor viii, CD31, S100, HMB45, calretinin, and synaptophysin were negative in both. Since proximal-type epithelioid sarcoma can be confused with a number of other soft tissue tumors with epithelioid and/or rhabdoid features, the authors emphasize the immunohistochemical differential diagnosis.- - - - - - - - - - ranking = 5keywords = rhabdoid (Clic here for more details about this article) |
10/24. Poorly differentiated primary orbital sarcoma (presumed malignant rhabdoid tumor). Radiologic and histopathologic correlation.A 47-year-old man presented with acquired hyperopia and gaze-evoked visual loss. Computed tomography and magnetic resonance imaging demonstrated a well-circumscribed intraconal tumor. Histopathologic studies indicated that the tumor was a poorly differentiated primary orbital sarcoma compatible with malignant fibrous histiocytoma or malignant rhabdoid tumor. To our knowledge, no case of primary orbital malignant rhabdoid tumor has been previously described in an adult.- - - - - - - - - - ranking = 6keywords = rhabdoid (Clic here for more details about this article) |
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