Adult ; Epithelioid Cells ; pathology ; Humans ; Male ; Perivascular Epithelioid Cell Neoplasms ; pathology

Epithelioid Cells ; pathology ; Humans ; Soft Tissue Neoplasms ; pathology

Child, Preschool ; Epithelioid Cells ; Heart Atria ; pathology ; Heart Neoplasms ; Humans ; Perivascular Epithelioid Cell Neoplasms

Epithelioid renal angiomyolipoma is a recently recognized pathologic variant of angiomyolipoma, which represents a more malignant course than a classical renal angiomyolipoma. We report a case of histologically proven renal angiomyolipoma. The patient presented with the symptoms and signs of spontaneous rupture of the kidney. On surgical exploration, the ruptured kidney with upper pole kidney mass was resected. The enlarged regional lymph nodes were also removed, which showed the same pathology.
Angiomyolipoma* ; Epithelioid Cells ; Humans ; Kidney ; Lymph Nodes ; Pathology ; Rupture, Spontaneous

OBJECTIVETo study the clinicopathological features of epithelioid angiosarcoma (EA) and to propose its differential diagnosis.

METHODS15 EAs were observed by means of HE staining, immunohistochemical staining (ABC method) and in part by electron microscopy.

RESULTSTwelve cases occurred in men and three in women. Clinical symptoms included a focal mass with pain, weight loss and weakness. The tumors were located in the retroperitoneum (n = 3), extremities (n = 3), femur (n = 4), thyroid (n = 2), supraobit (n = 1), soft tissue of thorax (n = 1), soft tissue of neck (n = 1). Radiographic evaluation demonstrated solid to cystic neoplasms ranging from 3 to 20 cm in diameter. Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis. Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells. FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes. Ultrastructurally, intracytoplasmic vacuoles, Weibel-Palades, intermediate filaments and basal lamina could be seen. Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up. Two patients are presently alive 19 months and 7 years following diagnosis. Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy. One patient was lost to follow up. There were 8 cases which had metastases to lungs, bones, lymph nodes and abdominal cavity.

CONCLUSIONSEA is a high-grade sarcoma. Endothelial derivation can be confirmed by immunohistochemical staining and ultrastructural findings. The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.

Adult ; Aged ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Hemangioendothelioma, Epithelioid ; diagnosis ; Humans ; Immunohistochemistry ; Male ; Melanoma ; diagnosis ; Middle Aged ; Sarcoma ; diagnosis

Pregnancy ; Female ; Humans ; Ovary/pathology* ; Gestational Trophoblastic Disease/pathology* ; Trophoblastic Neoplasms/pathology* ; Uterine Neoplasms/pathology* ; Epithelioid Cells/pathology*

Adult ; Epithelioid Cells ; pathology ; Fatal Outcome ; Female ; Humans ; Hysterectomy ; Middle Aged ; Trophoblastic Neoplasms ; pathology ; surgery ; Uterine Neoplasms ; pathology ; surgery

Epithelioid hemangioendothelioma (EHE) is a relatively rare and moderately aggressive type of vascular tumor. We describe a case of EHE that presented in a 24-year-old woman as a palpable nodule in the left cervical area. Radiological examinations and fine-needle aspiration cytology led to a preliminary diagnosis of metastatic lymphadenopathy with an unknown primary site. However, during surgery, we determined that the nodule was an intra-vascular tumor arising from the left internal jugular vein. The histopathological examination revealed cords of epithelioid endothelial cells distributed in a hyaline stroma, compatible with a diagnosis of EHE.
Biopsy, Fine-Needle ; Diagnosis ; Endothelial Cells ; Female ; Hemangioendothelioma, Epithelioid* ; Humans ; Hyalin ; Jugular Veins* ; Lymphatic Diseases* ; Pathology ; Vascular Diseases ; Young Adult

Actins ; metabolism ; Angiomyolipoma ; pathology ; Carcinoma, Renal Cell ; pathology ; Diagnosis, Differential ; Epithelioid Cells ; metabolism ; pathology ; Female ; Gastrointestinal Neoplasms ; pathology ; Gastrointestinal Stromal Tumors ; pathology ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; Lung Neoplasms ; pathology ; Lymphangioleiomyomatosis ; pathology ; Male ; Melanoma ; pathology ; Melanoma-Specific Antigens ; metabolism ; Pancreatic Neoplasms ; pathology ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; Sarcoma, Clear Cell ; pathology ; Skin Neoplasms ; pathology ; Uterine Neoplasms ; pathology

OBJECTIVETo investigate clinicopathologic features, pathologic diagnosis, differential diagnosis and biological behavior of epitheioid myxofibrosarcoma (EMFS).

METHODSThe clinical and pathological data of 10 cases were collected, and microscopic examination and immunostains were performed along with a review of the literatures.

RESULTSThere were 5 males and 5 females with age ranging from 53 to 74 years, and the mean and median age was 63.6 and 62.5 years, respectively. Six cases developed in the extremities, including upper limbs (n=3) and lower limbs (n=3). Three developed in the trunk and 1 case in the mesentery of sigmoid colon. Tumor size ranged from 4.2 to 7.0 cm (mean, 5.3 cm). Most patients presented with painless masses with duration of 1 to 24 months (mean, 8 months). All 10 patients were treated by surgery, with adjunctive chemotherapy and/or radiotherapy in 4 patients and interventional therapy in 1 patient. Histologically, 8 cases were high grade and 2 were intermediate grade. Like the conventional myxofibrosarcomas, all primary tumors presented a multinodular growth pattern consisting of hypocellular myxoid and hypercellular areas. Prominent curvilinear vessels and pseudolipoblasts were observed in the hypocellular myxoid areas. Besides the spindled neoplastic cells, all tumors were characterized by a variable proportion of epithelioid cells with vesicular nuclei, prominent nucleoli and moderate to abundant eosinophilic cytoplasm. They were arranged singly or in small clusters in the myxoid areas, and in compact sheets in the solid areas. The epithelioid component comprised 30% to 90% of the tumors. In addition, areas with resemblance to undifferentiated pleomorphic sarcoma were also noted, especially in the recurrent tumors. Immunohistochemically, tumor cells showed diffuse staining of vimentin in 6 tested cases with focal expression of smooth muscle actin and epithelial membrane antigen in 1 case each. Ki-67 index ranged from 30% to 80% (mean, 58%). Follow-up data (range, 2 to 74 months; mean, 23 months) were available in 10 cases: 4 patients were alive with unresectable or recurrent disease and 6 patients were alive with no evidence of disease. Five patients experienced local recurrence and 2 cases developed metastasis. The median interval to recurrence/metastasis was 7 months (mean, 9 months).

CONCLUSIONSThe presence of epithelioid cells in a myxofibrosarcomatous background portends an aggressive clinical behavior.EMFS should be differentiated from other myxoid sarcomas with epithelioid morphology.

Actins ; metabolism ; Aged ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Fibrosarcoma ; diagnosis ; pathology ; Humans ; Male ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local