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

Adult ; Antigens, Neoplasm ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Humans ; Immunohistochemistry ; Melanoma-Specific Antigens ; Neoplasm Proteins ; analysis ; Uterine Neoplasms ; chemistry ; diagnosis ; pathology