Angiomyolipoma is a common tumor of the kidney but has rarely been found in the mediastinum. We report a case of angiomyolipoma of the posterior mediastinum in a 62-year-old woman. She experienced exertional dyspnea and intermittent cough at admission. Computed tomography indicated a tumor located at the left paravertebral and upper posterior mediastinum and MRI imaging demonstrated a mass with low signal intensity in T1-weighted image at T4-5 level. Thoracotomy was done for surgical removal of the tumor and histologic examination revealed a mesenchymal tumor composed of mature fat, capillaries and smooth muscle fibers. The tumor was immunohistochemically positive for CD34 and factor-VIII (for vascular component) smooth muscle actin (for smooth muscle component) and S-100 protein (for fat component). There have been four case reports about mediastinal angiomyolipoma, namely three Japanese cases and one French case. It is suggested that angiomyolipoma could be considered for the differential diagnosis of mediastinal tumors.
Angiomyolipoma/*pathology/surgery ; Case Report ; Female ; Human ; Mediastinal Neoplasms/*pathology/surgery ; Middle Age

PURPOSE: The purpose of this study is to set guidelines for the management of renal angiomyolipoma (AML), clinical prognosis according to tumor size, in association with tuberous sclerosis complex (TSC), multiplicity, radiographic finding, and treatment modality. MATERIALS AND METHODS: Between March 1998 and October 2008, 129 out of 254 patients with AML who underwent surgical intervention or angioembolization were enrolled. Diagnosis of AML was determined by the presence of a low attenuated component on CT imaging or by pathological confirmation. Indications of treatment were intractable pain, hematuria, suspicion of malignancy, large tumor size, spontaneous rupture, and radiographically equivocal tumors in which a differential diagnosis was needed to rule out malignancy. Parameters including age, sex, tumor size, multiplicity, radiographic characteristics, association with TSC, and treatment modality were reviewed. RESULTS: Age at presentation was 50.6 years and mean tumor size was 3.5 cm. Presentation symptoms were flank pain, hematuria, spontaneous rupture, and fatigue. 97 (75.2%) patients were incidentally discovered. 100 (77.5%) were females. 68 (52.7%) underwent nephron-sparing surgery (NSS), 35 (27.1%) radical nephrectomy, and 26 (20.2%) angioembolization. TSC was accompanied in 12 (9.3%) patients. No patient developed renal function impairment during the mean follow-up period of 64.8 months. Patients with TSC presented at a younger age, along with larger, bilateral, and multiple lesions. CONCLUSION: Significant differences in clinical manifestations and treatment outcomes were noted in respect to tumor characteristics, association with TSC, and treatment modality. Considering the benign nature of AML, these parameters ought to be considered when deciding upon active surveillance or prophylactic intervention.
Adult ; Angiomyolipoma/*pathology/*surgery/therapy ; Female ; Humans ; Kidney Neoplasms/*pathology/*surgery/therapy ; Male ; Middle Aged ; Retrospective Studies ; Treatment Outcome

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

Adrenalectomy ; Adult ; Angiomyolipoma ; pathology ; surgery ; Follow-Up Studies ; Humans ; Kidney ; pathology ; Kidney Neoplasms ; pathology ; surgery ; Lymph Node Excision ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Male ; Nephrectomy ; Ureter ; surgery

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

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

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

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*

OBJECTIVETo present one cases of the use of balloon catheter in surgical treatment of renal angiomyolipoma involving the renal vein and vena cava as a tumor thrombus and review literatures.

METHODSAbdominal ultrasound and CT and MRI demonstrated a large right renal mass with tumor thrombus in the inferior vena cava. Right nephrectomy and en-bloc removal of the intra caval tumor thrombus were performed. A balloon catheter was used to block vena cava under the level of liver vena during the operation.

RESULTSThe pathological diagnosis was angiomyolipoma. The length of the tumor thrombus was 6.5 cm. The patient recovered well 1 year after surgery.

CONCLUSIONRenal angiomyolipoma with a tumor thrombus should be paid more attention.

Adult ; Angiomyolipoma ; complications ; surgery ; Balloon Occlusion ; Embolectomy ; methods ; Embolism ; etiology ; surgery ; Female ; Humans ; Kidney Neoplasms ; complications ; surgery ; Liver ; blood supply ; pathology ; surgery ; Nephrectomy ; Vena Cava, Inferior

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