• Asploro Journal of Biomedical and Clinical Case Reports
  • ISSN: 2582-0370
  • Article Type: Case Report
  • DOI: 10.36502/2020/ASJBCCR.6193
  • Asp Biomed Clin Case Rep. 2020 Apr 8;3(2):99-101

Epidemiology Analysis of Pelvic Floor Medicine Center in Southern Israel Using PFDI-20 and PISQ-12 Quality of Life Questionnaires

Rasso A1*, Chaoui R1, Elloudi S1, Baybay H1, Mernissi FZ1
1Department of Dermatology CHU Hassan II, Fez, Morocco

Corresponding Author: Asmae Rasso ORCID ID
Address: Department of Dermatology, CHU Hassan II Fez, Morocco.
Received date: 18 February 2020; Accepted date: 02 April 2020; Published date: 19 April 2020

Citation: Rasso A, Chaoui R, Elloudi S, Baybay H, Mernissi FZ. When Aesthetic Concern Discovered the Mister: A Renal Angiomyolipoma. Asp Biomed Clin Case Rep. 2020 Apr 8;3(2):99-101.

Copyright © 2020 Rasso A, Chaoui R, Elloudi S, Baybay H, Mernissi FZ. This is an open-access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium provided the original work is properly cited.

Keywords: Tuberous Sclerosis Complex, Renal Angiomyolipoma, Abdominal CT scan

Introduction

Tuberous sclerosis complex (TSC) is a rare genetic disorder that affects about 1 in 5000 individuals worldwide. It does can affect many organs, leading to benign tumors presenting preferentially in the skin, brain, and kidneys [1]. We report the case of a young man with cutaneous angiofibromes who is incidentally diagnosed with renal angiomyolipoma.

Case Report

An 18-year-old patient, with no notable pathological antecedents, consults for the management of a major aesthetic problem caused by lesions on the face evolving since the age of 3 years, with increase in size and number, asymptomatic, without any signs including no behavioral disorder or epilepsy. The clinical examination objectively multiple angiofibromes in the nasogénien fold, about 3 mm in size, with two chagrin patches on the scalp (Fig-1), the rest of the examination was without particularity. A radiological check-up was requested, where the abdominal ultrasound and abdominal CT scan had shown two bilateral renal tumors, of which the right side measures about 10 cm (Fig-2), without oligoanuria or hematuria. Urea and creatinine in the blood were normal. Cerebral MRI showed linear subcortical hypersigns, without contrasting in favor of cortical tubers.

When Aesthetic Concern Discovered the Mister: A Renal Angiomyolipoma

Fig-1

A: Angiofibromes in the nasogénien fold

B: Chagrin patches on the scalp

When Aesthetic Concern Discovered the Mister: A Renal Angiomyolipoma

Fig-2

Renal tumor 10 cm in size

Discussion

Renal angiomyolipoma is a mesenchymal tumor composed of blood vessels, smooth muscle and fat elements in varying proportions, uncommonly, they may become extremely large. It is a rare entity, with an incidence of 0.1-0.22% in the general population [2]. It can be associated with tuberous sclerosis complex (TSC) or sporadic. Renal angiomyolipoma associated with tuberose sclerosis tend to be larger, bilateral and multifocal grow during adolescence and into adulthood, as in the case of our patient. The main complication is retroperitoneal hemorrhage, which can be fatal due to the associated blood loss. The risk is greater as the tumors become larger. The management of renal angiomyolipoma is challenging for the physician who will choose between surveillance or a surgery [3]. Recently mTOR inhibitors are recommended as the most effective first-line treatment for renal angiomyolipoma associated with CST >3 cm in size, even when it is asymptomatic. However, it’s a relatively rare disease and there are no established criteria for its treatment [4].

Conclusion

Tuberous sclerosis complex is a rare disease with multi-organic disorders, some of which are severe and often asymptomatic`. The role of the doctor is to make a good clinical and paraclinical examination and a good follow-up of these patients in order to save and improve their quality of life.

Consent

The examination of the patient was conducted according to the Declaration of Helsinki principles.

Conflict of Interest

I declare no conflict of interest.

References

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[2] Rakowski SK, Winterkorn EB, Paul E, Steele DJ, Halpern EF, Thiele EA. Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors. Kidney Int. 2006 Nov;70(10):1777-82. [PMID17003820]

[3] Cockerell I, Guenin M, Heimdal K, Bjørnvold M, Selmer KK, Rouvière O. Prevalence of Renal Angiomyolipomas and Spontaneous Bleeding Related to Angiomyolipomas in Tuberous Sclerosis Complex Patients in France and Norway-a Questionnaire Study. Urology. 2017 Jun;104:70-76. [PMID28232177]

[4] Schillinger F, Montagnac R. Chronic renal failure and its treatment in tuberous sclerosis. Nephrology Dialysis Transplantation. 1996 Mar 1;11(3):481-85.