An Unusual Oncocytic Sellar Neoplasm in a Patient with Birt-Hogg-Dubé Syndrome
نویسندگان
چکیده
Spindle cell oncocytoma (SCO) is a rare and recently described neoplasm arising from the pituitary adenohypophysis thought to be derived from the folliculostellate cells. Even though SCO has been classified as a distinct World Health Organization (WHO) grade I tumor, sellar neoplasms with an oncocytic appearance are uncommon and have a wide differential diagnosis that includes primary and metastatic lesions. Herein we describe an oncocytic sellar neoplasm of uncertain histiogenesis arising in a 49-year-old man with BirtHoggDubé (BHD) syndrome. This case is also unusual in that individuals with BHD have a propensity to develop oncocytomas, yet to the best of our knowledge this is the first occurrence of a primary oncocytic neoplasm of the sella in BHD syndrome.
منابع مشابه
Birt-Hogg-Dube syndrome presenting as multiple oncocytic parotid tumors
Mutations in FLCN cause Birt-Hogg-Dubé syndrome, an autosomal dominant disorder notable for development of cutaneous fibrofolliculomas or trichodiscomas, a variety of renal tumors, and spontaneous pneumothorax due to cystic lung changes. We present a woman referred for genetic evaluation due to bilateral parotid gland tumors, who was subsequently diagnosed with Birt-Hogg-Dubé syndrome.
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Birt-Hogg-Dubé syndrome is a rare autosomal dominant genodermatosis that is characterized by the presence of fibrofolliculomas and/or trichodiscomas, pulmonary cysts, spontaneous pneumothorax, and renal tumors. The most common histological types found in renal tumors from patients with the syndrome are oncocytoma-chromophobe carcinoma hybrids and pure forms of chromophobe carcinoma, oncocytic c...
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