Literature DB >> 9554386

Renal angiomyolipoma in children: diagnositc difficulty in 3 patients.

Z Tchaprassian1, G Mognato, G Paradias, E S D'Amore, A Tregnaghi, G Cecchetto.   

Abstract

PURPOSE: Because angiomyolipoma is less common in children than in adults, its diagnosis can be difficult. We present 3 cases of pediatric angiomyolipoma in which diagnostic problems resulted due to the presenting characteristics.
MATERIALS AND METHODS: We report on 3 children with unilateral renal angiomyolipoma. Computerized tomography (CT) and ultrasonography revealed 3 large renal masses, 20, 7 and 8 cm. in diameter, respectively. A correct diagnosis was not made preoperatively in any case by CT, ultrasound or fine needle biopsy. Wilms tumor was suspected in the first patient who received preoperative chemotherapy. Imaging was inconclusive in the other 2 cases.
RESULTS: All patients underwent surgical exploration and subsequent nephrectomy due to the large size of the tumor. At followup 33, 23 and 13 months postoperatively all children were well without signs of recurrence.
CONCLUSIONS: It has been reported that the demonstration of fat on renal ultrasound and CT can diagnose angiomyolipoma in 95% of the cases. Most radiologists rely solely on CT demonstration of lipid density in the renal mass to diagnose angiomyolipoma but the identification at imaging of lipid tissue may be difficult in small tumors. In our cases the fat content of the tumors was less than 10% despite the large size. This low fat content results in misdiagnosis, since fatty tissue is also present in other renal tumors, such as lipoma, liposarcoma, teratoma and Wilms tumors. We recommend conservative surgery when tumor size permits in pediatric patients with angiomyolipoma to avoid chemotherapy.

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Year:  1998        PMID: 9554386     DOI: 10.1097/00005392-199805000-00083

Source DB:  PubMed          Journal:  J Urol        ISSN: 0022-5347            Impact factor:   7.450


  5 in total

1.  Life threatening hematuria in a patient with renal angiomyolipoma and selective renal embolization prior to nephrectomy.

Authors:  Cem Ozden Yeniyol; Nuri Zeyrek; Mustafa Parildar; Elif Selek; Funda Taşli; Ahmet Memiş
Journal:  Int Urol Nephrol       Date:  2002       Impact factor: 2.370

Review 2.  Tuberous sclerosis complex and renal angiomyolipoma: case report and review of the literature.

Authors:  Elisabeth B Winterkorn; Ghaleb H Daouk; Sudha Anupindi; Elizabeth A Thiele
Journal:  Pediatr Nephrol       Date:  2006-06-22       Impact factor: 3.714

3.  PEComa in a 12-year-old boy.

Authors:  Zeeshan Uddin; Asim Qureshi; Samia Fatima; Naila Kayani
Journal:  BMJ Case Rep       Date:  2010-03-23

4.  Giant renal Angiomyolipoma masquerading as a Wilms tumor.

Authors:  Anjan Kumar Dhua; Abhishek Ranjan; Sandeep Agarwala; Veereshwar Bhatnagar; Sandeep R Mathur; Kandasamy Devasenathipathy
Journal:  Indian J Urol       Date:  2017 Jan-Mar

5.  Abdominal ultrasonographic manifestations in pediatric patients with tuberous sclerosis complex.

Authors:  Zhihua Xu; Junbo Wu; Guimin Xu; Hongxia Luo
Journal:  Transl Pediatr       Date:  2020-12
  5 in total

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