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Birt-Hogg-Dubé Syndrome and coexistence with adrenocortical carcinoma.

Dear Editor,Birt-Hogg-Dubé syndrome (BHDS), also known as Hornstein-Krickenberg syndrome, constitutes an autosomal dominant disorder caused by mutations in the tumor suppressor gene FLCN, codifying the folliculin protein 1 . Patients manifest benign hamartomas of the hair follicle, pulmonary cysts, recurrent pneumothoraces, and are predisposed to renal cell carcinoma 2 . Cutaneous manifestations are fibrofolliculomas, trichodiscomas, and acrochordons, usually located on the face, neck, and upper trunk 2 . Lung involvement rates 90 % of patients with BHDS and includes multiple pulmonary cysts of various sizes and usually located in the mediastinum. Lung predominance is generally low, nonetheless, recurrent pneumothoraces occur. Compared to the background population, BHDS patients have a 50 times higher risk of spontaneous pneumothorax, usually resulting from pulmonary cysts rupture. Regarding BHDS-associated renal tumors, histology discloses most commonly chro-mophobe renal cell carcinomas and hybrid oncocytic/chromophobe tumors, both of which show a low malignant potential. A 30-year-old female patient was examined for multiple white dome-shaped papules on her face and neck. Similar skin lesions were referred in her family history (father, brother, and paternal uncle) along with a personal and familial history of recurrent pneumothoraces. Histology of the skin lesions showed proliferation of follicular epithelial cells encircled by fibrosis. These findings were consistent with fibrofolliculomas. Suspecting a BHDS, computerized tomography was ordered, where multiple bilateral lung cysts with central and subpleural distribution were observed. To exclude a renal malignancy, the patient underwent a Magnetic Reso-nance Imaging (MRI) of the abdomen, where a heterogeneous right adrenal mass of a maximum diameter of 4 cm with central ne-crosis was revealed. Ectopic renal cancer was suspected; therefore, successful right adrenalectomy undertaken. Hematoxylin and Eosin stained sections concluded to a tumor comprised of malignant cells folliae with central necrosis. The immunohistochemical staining showed diffuse synaptophysin expression. The findings were consistent with adrenocortical carcinoma. Genetic screening of the patient in the Institut of Human Genetics in Munich revealed a heterozygous 2-base-pair de-letion in exon 9 (c.941_942del;p.P314fs) of folliculin gene ( FLCN ), confirming the diagnosis of BHDS. No recurrence is observed three years after resection of the adrenocortical tumor, while the patient and her next of kin are screened for renal carcinoma with an annual abdominal MRI. and genetic