2012, Number 6
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Rev Mex Urol 2012; 72 (6)
Renal tumor associated with Birt-Hogg-Dubé syndrome: presentation of a clinical case
Monges-Jones JE, González-Jáuregui DF, Sánchez-González JA, Meza-Díaz R, Carrera-Muńoz A, González-Acosta J
Language: Spanish
References: 13
Page: 293-296
PDF size: 305.17 Kb.
ABSTRACT
Renal cancer represents 3% of malignant tumors. Its
presentation can be sporadic or hereditary. One of the
hereditary presentations is renal oncocytoma, which is
associated with Birt-Hogg-Dubé syndrome (BHDS). This
syndrome presents with cutaneous lesions (fibrofolliculoma,
trichodiscoma), renal tumors, and pulmonary alterations
(cysts, spontaneous pneumothorax).
A 16-year-old female patient had disease onset in June
2010 with moderate pain in the right iliac fossa. Physical
examination revealed fibrofollicular lesions on the cheeks
and a tumor in the right renal fossa that was firm, mobile,
and painless when palpated.
Computerized axial tomography (CAT) scan of the abdomen
identified a renal tumor. Right radical nephrectomy was
performed. The histopathologic diagnosis was renal
cancer.
A control thoracic CAT scan was carried out when the
patient presented with left hydropneumothorax, for which
a pleural catheter was placed.
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