Epithelioid angiomyolipoma is a recently described rare variant of renal angiomyolipoma. It can occur in patients with or without tuberous sclerosis. We report the imaging findings of a case of epithelioid angiomyolipoma that showed the presence of fatty tissue undifferentiated from the typical angiomyolipoma at the beginning. After partial nephrectomy, tumour recurrence occurred two years later, presenting as completely solid tumours with no adipose tissue, and with invasion into the psoas muscle and left adrenal gland. Differentiation of this tumour from renal cell carcinoma is difficult. Both the radiologist and surgeon should be aware of the existence of this tumour and its potentially malignant behaviour.
Aged ; Angiomyolipoma ; diagnosis ; pathology ; surgery ; Carcinoma, Renal Cell ; diagnosis ; Diagnosis, Differential ; Female ; Humans ; Kidney ; pathology ; Kidney Neoplasms ; diagnosis ; pathology ; surgery ; Neoplasm Recurrence, Local ; pathology ; Tomography, X-Ray Computed

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

OBJECTIVETo investigate the clinicopathologic features, diagnosis and treatment of hepatic angiomyolipoma (HAML).

METHODSThe clinical, histopathological, treatment and prognosis data of 51 patients treated for HAML from October 1998 to October 2007 were retrospectively analyzed.

RESULTSHAML had a female predilection (female/male = 41/10) and the mean age was 44 years old. The main symptoms were abdominal mass (33 cases) and abdominal pain or discomfort (15 cases), the other 2 cases presented as fever. Histopathologically, HAML was composed of a heterogeneous mixture of blood vessels, smooth muscle, and adipose cells. Immunohistochemical staining showed relatively high positive rate of HMB-45 (50/51), SMA (47/49) and S-100 (39/42). All 51 patients underwent partial hepatectomy. The mean hospital stay was 13.8 days and mean intraoperative blood loss was 263 ml. There was no recurrence or metastasis after a mean follow-up of 55.4 months.

CONCLUSIONSHAML is a rare benign mesenchymal tumor of the liver. Definitive diagnosis of HAML depends on the pathohistological findings and HMB-45 positive myoid cell is an important diagnostic marker. Complete surgical resection is the optimal treatment for HAML with favorable prognosis.

Adolescent ; Adult ; Angiomyolipoma ; diagnosis ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Liver Neoplasms ; diagnosis ; pathology ; surgery ; Male ; Middle Aged ; Prognosis ; Retrospective Studies ; Treatment Outcome ; Young Adult

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

No abstract availble.
Angiomyolipoma/*diagnosis/pathology/surgery ; Antigens, Neoplasm/immunology ; Carcinoma, Hepatocellular/diagnosis/pathology/surgery ; Diagnosis, Differential ; Humans ; Liver Neoplasms/*diagnosis/pathology/surgery ; Male ; Middle Aged ; Neoplasm Proteins/immunology ; Tomography, X-Ray Computed

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

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

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

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

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