Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Epiglottis ; Factor VIII ; metabolism ; Humans ; Laryngeal Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged

Aged ; Angiomyolipoma ; metabolism ; pathology ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Leiomyoma ; metabolism ; pathology ; Lymphocytes ; pathology ; Male ; Nephrectomy ; Neurilemmoma ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

Abdominal Neoplasms ; metabolism ; pathology ; surgery ; Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; surgery ; Liposarcoma ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Retroperitoneal Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

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

Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Antigens, Neoplasm ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Leiomyosarcoma ; metabolism ; pathology ; Mediastinal Neoplasms ; metabolism ; pathology ; surgery ; Mediastinum ; pathology ; surgery ; Melanoma-Specific Antigens ; Middle Aged ; Neoplasm Proteins ; metabolism

BACKGROUND/AIMS: Hepatic angiomyolipoma (AML) is a rare mesenchymal tumor of the liver and demonstrates a marked histologic diversity. HMB-45 is a promising immunomarker for this tumor and especially helpful to diagnosis of some AMLs with unusual morphology. The purpose of this study was to better define the variable histologic feature of hepatic AML. METHODS: Eight hepatic AMLs were examined, and all of that were resection specimens. The diagnosis was confirmed by the presence of HMB-45 positive cells. Median age was 41.5 years old, and mean size of tumor was 8.94 cm. RESULTS: Conventional mixed type was 5 cases which showed myomatous, angiomatous and lipomatous component, and 3 cases were myomatous predominant. Variable patterns including spider web cell morphology, solid sheet-like and trabecular pattern were identified on myomatous component and variable amount and patterns of inflammatory cell infiltration was identified. CONCLUSIONS: With only histologic features, it is difficult to distinguish hepatic AML from other hepatic tumor including hepatocellular carcinoma or inflammatory pseudotumor. A correct diagnosis of hepatic AML is possible by a close histologic examination with immunohistochemical stainings such as HMB-45 which is important to patient's prognosis.
Adult ; Aged ; Angiomyolipoma/metabolism/*pathology/surgery ; Carcinoma, Hepatocellular/metabolism/*pathology/surgery ; Female ; Granuloma, Plasma Cell/metabolism/pathology ; Humans ; Liver Neoplasms/metabolism/*pathology/surgery ; Male ; Melanoma-Specific Antigens/*metabolism ; Middle Aged ; Tomography, X-Ray Computed

Adult ; Angiomyolipoma ; pathology ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Hemangiopericytoma ; pathology ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Nephrectomy ; Sarcoma, Synovial ; pathology ; Solitary Fibrous Tumors ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Actins ; metabolism ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; S100 Proteins ; metabolism ; Skin Neoplasms ; metabolism ; pathology ; surgery

Adult ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; MART-1 Antigen ; metabolism ; Male ; Melanoma-Specific Antigens ; metabolism ; Tumor Suppressor Protein p53 ; metabolism

OBJECTIVETo study the histopathologic features, differential diagnosis and prognosis of epithelioid angiomyolipoma (EAML) of kidney.

METHODSTwo cases of EAML (including one case with recurrence) of kidney were retrieved from the archival files of Departments of Pathology, Navy General Hospital of PLA and Health Science Center of Peking University. The clinicopathologic features, immunohistochemistry, ultrastructural findings and follow-up data were studied and literature reviewed.

RESULTSHistologically, the tumors were predominantly composed of epithelioid cells with marked cellular pleomorphism. Focal perivascular arrangement was seen. Hemorrhage and necrosis were present and tumor emboli were found in the venous structures. The renal hilar lymph nodes were also involved by tumor cells. Immunohistochemical study showed that the tumor cells (including those in the hilar lymph nodes) were strongly and diffusely positive for HMB45, smooth muscle actin, neuron-specific enolase and vimentin. They were focally positive for S-100 protein, melan-pan and CD68. The staining for epithelial membrane antigen, AE1/3, CK7, CD117, muscle-specific actin, desmin, leukocyte common antigen, CD20, CD45RO, CD30, CD15, chromogranin A, synaptophysin, bcl-2, estrogen receptor, progesterone receptor and p53 were negative. Ultrastructural examination revealed the presence of melanosome-like dense granules, myofilaments and dense bodies in the tumor cell cytoplasm. Discontinuous and focally thickened basal lamina was seen surrounding the tumor cells. On follow up, both patients remained well and disease-free 10 months after operation.

CONCLUSIONSEAML is predominantly or almost entirely composed of epithelioid cells. Perivascular cellular arrangement, focal features of otherwise classic angiomyolipoma, as well as coexpression of HMB-45 and smooth muscle actin are clues to the correct diagnosis. The degree of cytologic atypia, presence of hemorrhage and necrosis and high mitotic activity may indicate the malignant potential of this tumor. On the other hand, the presence of lymph node involvement and even tumor emboli in renal veins may represent multifocaltumorigenicity rather than true malignancy. Definitive evidence of malignancy requires demonstration of distant metastasis.

Adult ; Angiomyolipoma ; metabolism ; pathology ; surgery ; Antigens, Neoplasm ; metabolism ; Diagnosis, Differential ; Epithelioid Cells ; pathology ; ultrastructure ; Humans ; Immunohistochemistry ; Kidney ; pathology ; ultrastructure ; Kidney Neoplasms ; metabolism ; pathology ; surgery ; Male ; Melanoma-Specific Antigens ; Microscopy, Electron ; Neoplasm Proteins ; metabolism