Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear. Moreover, it is often misdiagnosed. One patient with renal AML complicated with renal vein, IVC, and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University, who was a 35-year-old female, without any previous medical history, presented with right low back pain for more than 3 years. Computed tomography (CT) scan showed irregular lobulated fatty density mass in the right kidney, renal vein, IVC, and right atrium. The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components. Radical resection of the right kidney and removal of tumor thrombus were performed, and there was no recurrence 1 year after the operation. It is rare for renal AML to grow along the renal vein, IVC, and extend to the right atrium. Imaging examination is extremely important, and the CT findings of this case are characteristic, but the diagnosis eventually depends on pathological and immunohistochemical examinations.
Female ; Humans ; Adult ; Vena Cava, Inferior/pathology* ; Angiomyolipoma/surgery* ; Atrial Fibrillation ; Kidney Neoplasms/surgery* ; Embolism/pathology* ; Heart Atria/diagnostic imaging* ; Leukemia, Myeloid, Acute/pathology*

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

Renal angiomyolipomas are benign neoplasms composed of varying amounts of adipose tissue, smooth muscles and blood vessels. They typically contain macroscopic fat, which is seen as negative attenuation on computed tomography. Calcification and scarring is rarely seen in renal angiomyolipomas. We report the case of a 40-year-old man who was found to have a renal angiomyolipoma with a central stellate scar and focal calcification. The lesion was initially misdiagnosed as a calyceal calculus.
Adipose Tissue ; diagnostic imaging ; pathology ; Adult ; Angiomyolipoma ; diagnosis ; surgery ; Biopsy, Needle ; Calcinosis ; diagnostic imaging ; pathology ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Kidney Calculi ; diagnosis ; surgery ; Kidney Calices ; diagnostic imaging ; pathology ; Kidney Neoplasms ; diagnosis ; surgery ; Low Back Pain ; diagnosis ; etiology ; Male ; Risk Assessment ; Tomography, X-Ray Computed ; methods ; Treatment Outcome ; Urography ; methods

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 clinicopathological characteristics of sporadic renal hemangioblastoma.

METHODSTwo cases of sporadic renal hemangioblastoma were studied by histological and immunohistochemical staining, along with review of the literature.

RESULTSBoth cases were presented with a renal mass during annual physical examination with an age of 40 and 45 years, respectively. The lesions located in the middle portion of the kidney with a sharp tumor border. Histopathologically, the tumors were characterized by two major components: capillary and stromal cells. Immunohistochemically, the stromal cells were positive for vimentin, NSE, S-100 protein and inhibin-α, and negative for CK, HMB45, Melan A, EMA, CD56 and syn. Both tumors showed a very low level of Ki-67 labeling. The endothelial cells were positive for CD34.

CONCLUSIONSporadic renal hemangioblastoma is a rare benign neoplasm, the diagnosis of which is based on the characteristic follicular architecture and an exclusion of other carcinomas of the kidney.

Adult ; Angiomyolipoma ; metabolism ; pathology ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioblastoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Immunohistochemistry ; Inhibins ; metabolism ; Kidney Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Nephrectomy ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; methods ; Vimentin ; metabolism

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

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

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

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

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