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

OBJECTIVETo study the clinicopathological characteristics of hepatic angiomyolipoma (HAML) and to evaluate the correlation between clinicopathological parameters and tumor subtypes.

METHODSRetrospective analysis of clinicopathological features was conducted in 182 cases of HAML.

RESULTSHAML patients were predominantly female (M:F=1:4) and most commonly presented with non-specific symptoms. The median age at diagnosis was 46 years, ranged from 17 to 77 years. Tumor diameter was ranged from 0.3 to 32.0 cm with an average of 5.0 cm. Majority of the tumor was epithelioid type (112/165, 67.9%). Extramedullary hematopoiesis, multinucleated giant cells, intranuclear inclusions, nucleolus, cellular atypia, invasive growth pattern, multiple masses, hyperpigmentation and purpura-like changes mostly occurred in the epithelioid type (P<0.05). Extramedullary hematopoiesis was commonly seen in HAML, the significance of which was still uncertain.

CONCLUSIONSMost of HAML are epithelioid type, characterized by a proliferation of predominantly epithelioid cells, in which extramedullary hematopoiesis is commonly seen. Some morphologic features that may predict malignant such as necrosis, mitotic figures, and tumor emboli are only found in the epithelioid HAML. Mitotic activity, tumor necrosis, tumor thrombus, giant cells, periportal invasion, multiple lesions and tumors size over 10 cm are closely related with tumor recurrence and metastasis.

Adolescent ; Adult ; Aged ; Angiomyolipoma ; diagnosis ; pathology ; Epithelioid Cells ; cytology ; Female ; Gastrointestinal Neoplasms ; diagnosis ; pathology ; Giant Cells ; pathology ; Humans ; Middle Aged ; Necrosis ; Neoplasm Recurrence, Local ; Prognosis ; Retrospective Studies ; Young Adult

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

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

OBJECTIVETo further elucidate the clinical and pathologic features of retiform hemangioendothelioma (RH) and its clinical spectrum.

METHODSEight cases of RH were reviewed. The clinicopathologic profiles, immunophenotypes and outcome data were investigated.

RESULTSAll 8 cases occurred in females with a mean age at presentation of 40 years (range, 13-69 years). Five tumors arose in the skin of the head and neck region and lower extremities, two in the long bones and one in the spleen. Clinically, the patients presented with a slowly growing cutaneous plaque or subcutaneous nodule, pain of the upper arm, and dull pain and discomfort in the left hypochondrium respectively. Grossly, the tumor appeared as a non-encapsulated gray-yellowish to tan-brown mass with a mean diameter of 2.6 cm (range, 0.8-5.0 cm). On histology, it was composed of delicate branches of elongated vessels lined by a layer of hobnail or matchstick endothelium, exhibiting a retiform pattern with close resemblance of the normal rete testis. Cords or solid nests of epithelioid cells were noted adjacent to the well-formed vessels. In three cases, dilated vascular spaces with formation of intravascular papillary tufts were observed, features overlapping with Dabska tumor. There was usually marked lymphocytic infiltration in the stroma which was also hyalinized in some cases. One case had regional lymph node metastasis. By immunohistochemistry, all cases consistently expressed endothelial markers, including CD31 (8/8), human coagulation factor VIII (5/8), CD34 (5/7) and D2-40 (1/2). Two of six cases with follow-up information (18-67 months) developed local recurrences, but distant metastasis was not identified.

CONCLUSIONSRH is a distinctive vascular tumor of adulthood characterized by retiform growth of vessels with striking hobnail endothelium. Although the tumor occurs predominantly in the skin, the long bones and the spleen can be occasionally affected. The presence of Dabska tumor-like areas in RH may suggest a morphologic continuum between these two entities, comprising the family of hobnail hemangioendothelioma. Familiarity with the characteristic features of this vascular tumor of intermediate malignancy will help in the differential diagnosis of vascular neoplasms with hobnail endothelium.

Adolescent ; Adult ; Aged ; Biomarkers, Tumor ; Bone Neoplasms ; pathology ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; Female ; Hemangioendothelioma ; pathology ; Humans ; Immunohistochemistry ; Immunophenotyping ; Middle Aged ; Neoplasm Recurrence, Local ; Skin Neoplasms ; pathology ; Splenic Neoplasms ; pathology ; Young Adult

Cell Proliferation ; Cytoplasm ; metabolism ; Female ; Giant Cells ; pathology ; Humans ; Hysterectomy ; Immunophenotyping ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; diagnosis ; Salpingectomy ; Uterine Neoplasms ; diagnosis

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

Angiofibroma ; metabolism ; pathology ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; Extremities ; Fibroma ; metabolism ; pathology ; Fingers ; Hemangioblastoma ; metabolism ; pathology ; Hemangioendothelioma ; metabolism ; pathology ; Humans ; Rhabdomyosarcoma ; metabolism ; pathology ; Sclerosis ; Soft Tissue Neoplasms ; classification ; metabolism ; pathology

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 study the pathologic features and immunophenotype of 3 cases of melanotic epithelioid clear cell tumor of kidney.

METHODSMore than 2000 cases of renal tumors were retrospectively reviewed. Three cases of melanotic epithelioid clear cell tumor were identified. Immunohistochemical study was carried out using the paraffin-embedded tissue samples. Electron microscopy was also performed in 1 case.

RESULTSAmongst the 3 cases studied, the male-to-female ratio is 1:2. Histologically, 2 cases showed a clear cell carcinoma-like pattern. Papillary structures covered by clear cells and eosinophilic cells were observed in 1 case. Immunohistochemical study showed that the tumor cells in all cases expressed HMB 45. Two of them were also positive for Melan A. The staining for epithelial markers and S-100 protein was negative. Melanosomes were not identified by ultrastructural examination.

CONCLUSIONSMelanotic epithelioid clear cell tumor is a rarely seen neoplasm of kidney. There are some histologic overlaps with renal cell carcinoma, epithelioid angiomyolipoma and melanoma. Immunohistochemical study is useful in confirming the diagnosis. The tumor represents a morphologic variant of epithelioid angiomyolipoma.

Adolescent ; Adult ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Carcinoma, Renal Cell ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Epithelioid Cells ; metabolism ; pathology ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; MART-1 Antigen ; metabolism ; Male ; Melanoma-Specific Antigens ; metabolism ; Retrospective Studies