Retroperitoneal extrarenal angiomyolipoma: A case report

سال انتشار: 1396
نوع سند: مقاله کنفرانسی
زبان: انگلیسی
مشاهده: 481

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شناسه ملی سند علمی:

ACPLMED19_057

تاریخ نمایه سازی: 20 آبان 1397

چکیده مقاله:

Angiomyolipoma is a benign mesenchymal tumor which is composed of three components: fat ,blood vessles,myoid tissue. It is previously classified as hamartomas but now is regarded as belonging to the family of perivascular epithelioid cell tumor (PECOMA). Most common location of this tumor is kidney and rare cases of retroperitoneal angiomyolipomas are reported in medical literatures until now. Diagnosis of retroperitoneal angiomyolipoma is rather difficult due to the nonspecific symptoms and exclusion of other malignancies. Primary treatment of angiomyolipoma is surgical excision and less often embolization of the tumor supplying vessels. We present a case of retroperitoneal extrarenal angiomyolipoma and discus difficulties of diagnosis of this rare tumor. Case report A 37 years old healthy woman, who was planning for a pregnancy, was referred for checkup. In the abdominopelvic ultrasonography a right side retroperitoneal mass was reported. She was underwent surgery and the specimen was sent to the pathology. On gross examination kidney M:9x5x4cm with attached perinephric fat. External surface of kidney was smooth and grayish tan. Cut sectioning show normal appearance parenchyma. The mentioned mass M: 18*13*8cm and was attached to the hilum of right kidney. External surface of the mass was smooth and dark-brown. Cut sections show encapsulated tan-yellow surface with multiple hemorrhagic areas. On the microscopic examination, a neoplastic tissue composed of three componentswas seen: mature adipose tissue, epithelioid cells, and dilated vessels. The epithelioid cells have abundant clear to eosinophilic cytoplasm with distinct borders and roundnuclei. No mitotic activity or immature adipose tissue seen. For ruling out of differential diagnoses including adrenal cortical tumor, liposarcoma, melanoma, renal cell carcinoma, paraganglioma, etc IHC study was done. HMB45,Vimentin, SMA and Melan-A show positive staining in most epithelioid cells. Negative stained markers include: CK, CD10, S100, PAX8, Myogenin, Calretinin, C-Kit ,desmin, EMA, Synaptophysin, inhibin, CD68 ,MDM2. Ki67 show proliferative activity in 1% of tumor cells. According morphologic study and IHC staining and excluding differential diagnoses, retroperitoneal angiomyolipoma with more epithelioid features was confirmed. Disscusion Retroperitoneal angiomyolipoma is a rare and challenging tumor and could be confused with other retroperitoneal tumors. Patient’s symptoms and radiographically features of tumor are non-specific. These makes the diagnosis more difficult and it is important to differentiate it from other tumors. IHC staining helps to exclude differential diagnoses and prove the diagnosis of angiomyolipoma. Our case highlights problems and difficulties in the diagnosis of retroperitoneal angiomyolipoma.

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نویسندگان

Hedieh Moradi Tabriz

Department of pathology, Tehran University of Medical Sciences

Masoumeh Molavi

Department of pathology, Tehran University of Medical Sciences

Arezoo Eftekhar Javadi

Department of pathology, Tehran University of Medical Sciences