Renal Epithelioid Angiomyolipoma in Children

Main Article Content

Dhruv Mahajan
Vishesh Jain
Sandeep Agarwala
Manisha Jana
Prashant P Ramteke

Keywords

Angiomyolipoma; Kidney tumor; Pediatric cancer; Renal epithelioid angiomyolipoma; Tuberous sclerosis

Abstract

Renal angiomyolipoma is a rare cause of renal tumor in children. Most are associated with tuberous sclerosis, and the classic type is observed more commonly. Epithelioid angiomyolipoma is even rarer with only limited case reports and series published in literature, most of which are of adult patients. We describe a 12-year-old boy, a diagnosed patient of tuberous sclerosis, who presented with pain in the left flank. On evaluation, it was found to have a left renal mass with the clinical picture suggestive of renal cell carcinoma. Partial nephrectomy was performed and histopathology revealed epithelioid angiomyolipoma. The child was asymptomatic at follow-up after 3 months. Only a few such cases in children are found in literature, which are discussed alongside. Differential diagnosis of this rare tumor must be kept in mind in a renal tumor as surgery is generally curative in this possibly malignant tumor. Metastasis confers a poor prognosis. Chemotherapy is generally not effective, although various regimens have been tried. Tumor recurrence must be kept in mind and a follow-up after apparent complete remission is of paramount importance.

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References

1. Citak C, Yilmaz EB, Yaman E, Kaya S, Tasinlar H, Arpaci RB, et al. Malignant epitheloid angiomyolipoma of the kidney in a child treated with sunitinib, everolimus and axitinib. Can Urol Assoc J (CUAJ). 2015 Jul 17;9(7–8):542. https://doi.org/10.5489/cuaj.2823
2. Xi S, Chen H, Wu X, Jiang H, Liu J, Wu Q, et al. Malignant renal angiomyolipoma with metastases in a child. Int J Surg Pathol. 2014 Apr;22(2):160–6. https://doi. org/10.1177/1066896913497395
3. Tsai C-C, Wu W-J, Li C-C, Wang C-J, Huang C-H, Wu C-C. Epithelioid angiomyolipoma of the kidney mimicking renal cell carcinoma: A clinicopathologic analysis of cases and literature review. Kaohsiung J Med Sci. 2009 Mar;25(3):133–40. https://doi.org/10.1016/S1607-551X(09)70052-X
4. Paradis V, Laurendeau I, Vieillefond A, Blanchet P, Eschwege P, Benoît G, et al. Clonal analysis of renal sporadic angiomyolipomas. Hum Pathol. 1998 Oct;29(10):1063–7. https://doi.org/10.1016/S0046-8177(98)90414-2
5. European Chromosome 16 Tuberous Sclerosis Consortium. Identification and characterization of the tuberous sclerosis gene on chromosome 16. Cell. 1993 Dec 31;75(7):1305–15. https://doi.org/10.1016/0092-8674(93)90618-Z
6. Hornick JL, Fletcher CDM. PEComa: What do we know so far? Histopathology. 2006 Jan;48(1):75–82. https://doi. org/10.1111/j.1365-2559.2005.02316.x
7. Ho C, Chen S, Ho KMT, Chan WK, Leung YL, Cheng KC, et al. Dual-tracer PET/CT in renal angiomyolipoma and subtypes of renal cell carcinoma: Clin Nucl Med. 2012 Nov;37(11):1075–82. https://doi.org/10.1097/RLU.0b013e318266cde2
8. Chen L-S, Zhu Z-Q, Wang Z-T, Li J, Liang L-F, Jin J-Y, et al. Chemical shift magnetic resonance imaging for distinguishing minimal-fat renal angiomyolipoma from renal cell carcinoma: A meta-analysis. Eur Radiol. 2018 May;28(5):1854–61. https://doi.org/10.1007/s00330-017-5141-0
9. Nese N, Martignoni G, Fletcher CD, Gupta R, Pan C-C, Kim  H, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: Detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011 Feb;35(2):161–76. https://doi.org/10.1097/PAS.0b013e318206f2a9
10. Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg  BA, Frost M, Belousova E, et al. Everolimus for angiomy-olipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): A multi-centre, randomised, double-blind, placebo-controlled trial. Lancet. 2013 Mar;381(9869):817–24. https://doi.org/10.1016/ S0140-6736(12)61767-X
11. Davies DM, de Vries PJ, Johnson SR, McCartney DL, Cox JA, Serra AL, et al. Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic lymphangioleiomyomatosis: A phase 2 trial. Clin Cancer Res. 2011 Jun 15;17(12):4071–81. https://doi.org/10.1158/1078-0432.CCR-11-0445
12. Gupta RK, Sharma MC, Chitragar S, Sinha K, Agarwala S, Sharma R. Epithelioid angiomyolipoma in a child—A diagnostic dilemma. Urol. 2014 Jun;83(6):1394–7. https://doi.org/10.1016/j.urology.2013.12.044
13. Johnson MH, Song JB, Dehner LP, Figenshau RS. Epithelioid angiomyolipoma in a pediatric patient: Case report and review of literature. Urol Case Rep. 2014 Sep;2(5):159–61. https://doi. org/10.1016/j.eucr.2014.05.008
14. Kakaje A, Mujahed R, Hamdan O, Hakim J. A large epithelioid angiomyolipoma of the kidney in a very young child with idio-pathic chylothorax: A diagnostic dilemma. Urol Case Rep. 2020 Jan;28:101035. https://doi.org/10.1016/j.eucr.2019.101035