Extrarenal angiomyolipoma of paratesticular and subcutaneous region: a report of two cases at rare sites
Keywords:
Angiomyolipoma, Epididymo-orchitis, Extrarenal angiomyolipoma, PEComa, Tuberous sclerosis
Abstract
Angiomyolipomas are benign mesenchymal neoplasms consisting of variable proportion of mature adipose tissue, thick walled blood vessels and smooth muscle bundles. Although initially thought to be hamartomas, now they are considered to be tumours derived from perivascular epithelioid cells(PEC) hence grouped under a family of tumours known as PEComas. The tumour mainly involves kidneys and are more common in patients with tuberous sclerosis. However extrerenal angiomyolipomas are being increasingly described with liver and retroperitoneum being the most common extrarenal sites. Other sites described include nasal cavity, oral cavity, colon, lung, skin, adrenal glands and bladder. Here we report two cases of extrarenal angiomyolipomas of paratesticular and subcutaneous region, the rarely described sites in the literature. There were no clinical signs of tuberous sclerosis in both cases. Although literature suggests that these tumours show immunohistochemical coexpression of myogenic and melanocytic markers with consistent expression of HMB-45, our both cases were negative for HMB-45. However both cases showed classical triphasic morphology. Although rare, it is important to screen these patients for tuberous sclerosis associated lesions for early diagnosis.References
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12. Friis J. Hjortrup A. Extrarenal angiomyolipoma: diagnosis and management. J Urol. 1982;127:528–9.
13. Ditonno P, Smith RB, Koyle MA, Hannah J and Belldegrun A. Extrarenal angiomyolipomas of the perinephric space. J Urol. 1992;147:447–50.
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2. Yapanoglu T, Yılmaz AH, Ziypak T, Adanur S, Demirci E, Bilen H. Extrarenal Retroperitoneal Angiomyolipoma: A Rare Case. J Urol Surg 2017;4:134-6.
3. Vos N, Oyen R. Renal Angiomyolipoma: The Good, the Bad, and the Ugly. Journal of the Belgian Society of Radiology. 2018; 102: 41, pp. 1–9. doi: https://doi.org/10.5334/jbsr.1536
4. Thakur B, Kishore S, Bhardwaj A, Kudesia S. Extrarenal angiomyolipoma in an elderly male: A case to remember. Int J Med Public Health 2015;5:121-2.
5. Lokhande R, Goyal N, Bhargava S, Patni A. Retroperitoneal Angiomyolipoma: Rare Entity Asian Pac. J. Health Sci 2017; 4:15-18.
6. Hayashi Y, Shiyanagi S, Nagae I, Kasuya K, Katsumata K, Yamataka A et al. Angiomyolipoma of the tunica dartos of the scrotum in infancy. J Ped Surg Case Reports 2015;3:334-7.
7. Sultan G, Masood B, Qureshi H, Mubarak M. Angiomyolipoma of the scrotum: report of a rarely seen case and review of the literature. Turk J Urol 2017; 43: 223-6. doi: 10.5152/tud.2017.26779.
8. Jayaprakash PG, Mathews S, Azariah MB, Babu G. Pure epitheliod perivascular epitheloid cell tumor (epitheliod angiomyolipoma) of kidney: Case report and literature review. J Cancer Res Ther 2014;10:404-6.
9. Lin CY, Tsai CC, Kau HC, Yu WK, Kao SC, Liu CJ. HMB-45 negative angiomyolipoma of the orbit: a case report and review of the literature. BMC Ophthalmology 2016; 16:8.doi: 10.1186/s12886-016-0185-5.
10. Ceifo W, Qadri IA, Malik A. Angiomyolipoma of the Testis: A Case Report. Med Surg Urol 2015;4: 155. doi:10.4172/2168-9857.1000155
11. Venyo AKG. A Review of the Literature on Extrarenal Retroperitoneal Angiomyolipoma. International Journal of Surgical Oncology 2016; 2016: http://dx.doi.org/10.1155/2016/6347136.
12. Friis J. Hjortrup A. Extrarenal angiomyolipoma: diagnosis and management. J Urol. 1982;127:528–9.
13. Ditonno P, Smith RB, Koyle MA, Hannah J and Belldegrun A. Extrarenal angiomyolipomas of the perinephric space. J Urol. 1992;147:447–50.
14. Gupta C, Malani AK , Gupta V, Singh J, Ammar H. Metastatic retroperitoneal epithelioid angiomyolipoma. J Clin Pathol. 2007; 60:428–431.
Published
2020-01-31
Issue
Section
Case Report
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