Korean J Urol.  2007 Jun;48(6):655-658. 10.4111/kju.2007.48.6.655.

Renal Angiomyolipoma Partially Containing Epithelioid Component

Affiliations
  • 1Department of Urology, Sanggye Paik Hospital, Inje University College of Medicine, Korea. chung90@sanggyepaik.ac.kr
  • 2Department of Pathology, Sanggye Paik Hospital, Inje University College of Medicine, Korea.
  • 3Department of Diagnostic Radiology, Sanggye Paik Hospital, Inje University College of Medicine, Korea.
  • 4Department of Urology, Soonchunhyang University College of Medicine, Seoul, Korea.

Abstract

Epithelioid renal angiomyolipoma is a recently recognized pathologic variant of angiomyolipoma, which represents a more malignant course than a classical renal angiomyolipoma. We report a case of histologically proven renal angiomyolipoma. The patient presented with the symptoms and signs of spontaneous rupture of the kidney. On surgical exploration, the ruptured kidney with upper pole kidney mass was resected. The enlarged regional lymph nodes were also removed, which showed the same pathology.

Keyword

Epithelioid cells; Angiomyolipoma; Kidney

MeSH Terms

Angiomyolipoma*
Epithelioid Cells
Humans
Kidney
Lymph Nodes
Pathology
Rupture, Spontaneous

Figure

  • Fig. 1 The computed tomography (CT) pre-enhanced (A) and enhanced (B) images. A tumor contains fat and an enhancing solid portion. The high-attenuating material suggests hematoma, which is visible in the perirenal space.

  • Fig. 2 An ill-defined fibrofatty mass (6.5×5.5×3cm) is located at the upper pole of the left kidney. The surrounding perirenal fat tissue contains a large number of blood clots.

  • Fig. 3 The tumor is composed of a heterogenous populations of a mature fat tissue, spindle cells and thick walled vessels (H&E, ×100).

  • Fig. 4 (A) The medium power view shows epithelioid tumor cells arising from the vessel (H&E, ×100). (B) HMB-45 immunostaining is focally positive in some epithelioid tumor cells (HMB-45, ×400).

  • Fig. 5 The para-aortic lymph nodes showed an aggregation of spindle-to-ovoid tumor cells in the juxtacapsular area. However, the pericapsular sinus or intranodal structures are intact and not involved with tumor emboli. This supports that this is multifocality rather than true metastasis (H&E, ×100).


Cited by  1 articles

Trends of Presentation and Clinical Outcome of Treated Renal Angiomyolipoma
Kyo Chul Koo, Won Tae Kim, Won Sik Ham, Jin Sun Lee, Hee Jeong Ju, Young Deuk Choi
Yonsei Med J. 2010;51(5):728-734.    doi: 10.3349/ymj.2010.51.5.728.


Reference

1. Martignoni G, Pea M, Bonetti F. Renal epithelioid oxyphillic neoplasm, a pleomorphic monophasic variant of renal AML. Int J Surg Pathol. 1995. 2:539.
2. Son YW, Lee SW, Jeong YR, Cho YJ, Kim SI, Lee TY. Epithelioid angiomyolipoma of the kidney with distant metastasis. Korean J Urol. 2002. 43:987–990.
3. Ha U, Lee JH, Lee KB, Choi HW, Cho DH, Lee CB. Epithelioid angiomyolipoma of the kidney misdiagnosed as a renal cell carcinoma. Korean J Urol Oncol. 2006. 4:91–94.
4. Kawaguchi K, Oda Y, Nakanishi K, Saito T, Tamiya S, Nakahara K, et al. Malignant transformation of renal angiomyolipoma: a case report. Am J Surg Pathol. 2002. 26:523–529.
5. Martignoni G, Pea M, Rigaud G, Manfrin E, Colato D, Zamboni G, et al. Renal angimyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions. Am J Surg Pathol. 2000. 24:889–894.
6. Eble JN, Amin MB, Young RH. Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component. Am J Surg Pathol. 1997. 21:1123–1130.
7. Pea M, Bonetti F, Martignoni G, Henske E, Manfrin E, Colato C, et al. Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: the identification of malignant epithelioid angiomyolipoma. Am J Surg Pathol. 1998. 22:180–187.
8. Sarrano Frago P, Del Agua Arias Camison C, Gil Sanz MJ, Allue Lopez M, Gonzalvo Ibarra A, Plaza Mas, et al. Controversies related to epithelioid variant of renal angiomyolipoma: a review of the literature. Urology. 2006. 67:846.
9. Acikalin MF, Tel N, Oner U, Pasaoglu O, Donmez T. Epithelioid angiomyolipoma of the kidney. Int J Urol. 2005. 12:204–207.
10. Pan CC, Jong YJ, Chai CY, Huang SH, Chen TJ. Comparative genomic hybridization study of perivascular epithelioid cell tumor: molecular genetic evidence of perivascular epithelioid cell tumor as a distinctive neoplasm. Hum Pathol. 2006. 37:606–612.
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