Publication: Isolated pituitary neurosarcoidosis: A case report and review of the Lliterature
Abstract
Sarkoidoz genellikle genç erişkinlerde görülen ve sıklıkla bilateral hilar lenfadenopati ve pulmoner infiltrasyonla presente olan, birçok sistemi tutan granülamatoz bir hastalıktır. Santral sinir sistemi (SSS) tutulumu oldukça nadirdir. Sarkoidozun nedeni bilinmemektedir. Sarkoidoz tanısı etkilenen organlarda histopatolojik incelemede gözlenen kazeifiye olmayan granulomların klinik ve radyolojik bulgularla uyumlu olması ile konur. Burada, kliniğimize amenore ve poliüri ile başvuran 30 yaşında bir kadın hastayı sunmaktayız. Pituiter stalkı tutan lezyonun radyolojik görünümü sarkoidoz, sifilis, tüberküloz ve yabancı cisim granülamatozu için patognomik olan inflamatuar infiltrasyon görüntüsündeydi. Laboratuar testleri ile sifilis ve tüberküloz tanıları ekarte edildi. Olası tanı sarkoidozdu. Diğer sistemleri araştırdığımızda akciğerler, lenf nodları, deri ve gözlerde sarkoidoz tutulumu gözlenmedi. Transkranyal insizyonel biyopsi histopatolojik incelemesi makrofaj, epiteloid hücreler, sitokin salgılayan multinükleer dev hücrelerden oluşan kazeifiye olmayan granülom göstermekteydi. Santral korda CD4 ve CD8 lenfositler, B lenfositler, plazma hücreleri ve fibroblastlar gözlendi. Tanı nörosarkoidozdu. Bu olguyu pituiter lezyonların ayırıcı tanısında izole nörosarkoidozun da yeri olduğunu hatırlatmak ve bu durumun tanı ve tedavisinde son bilgileri gözden geçirmek için sunmaktayız.
Sarcoidosis is a multisystem granulomatous disorder, commonly affecting young adults and usually presenting with bilateral hilar lymphadenopathy and pulmonary infiltration. Central nervous system (CNS) involvement is extremely rare. The cause of sarcoidosis is unknown. The diagnosis of sarcoidosis is firmly established when histopathological evidence of non-caseating granulomas in affected organs supports compatible clinicoradiographic findings. Here, we present a case of a 30-year-old woman referred to our clinic with amenorrhea and polyuria. The radiological appearance of a lesion involving the pituitary stalk was an image of inflammatory infiltration, which is pathognomic for sarcoidosis,syphilis, tuberculosis and foreign body granulomatosis. Laboratory tests were done to rule out syphilis and tuberculosis. A possible diagnosis was sarcoidosis. When we searched other systems for the involvement of sarcoidosis, lungs, lymph nodes, skin and eyes were not involved by the disease. Histopathological examination of a transcranial incisional biopsy revealed a non-caseating granuloma, consisting of macrophages, epithelioid cells, and multinucleated giant cells that secrete cytokines. Around this central core, CD4 and CD8 lymphocytes, B lymphocytes, plasma cells, and fibroblasts were detected. The diagnosis was neurosarcoidosis. We present this case to draw attention to the possibility of isolated neurosarcoidosis as the differential diagnosis of pituitary lesions and review recent advances in the investigation, diagnosis and treatment of this condition.
Sarcoidosis is a multisystem granulomatous disorder, commonly affecting young adults and usually presenting with bilateral hilar lymphadenopathy and pulmonary infiltration. Central nervous system (CNS) involvement is extremely rare. The cause of sarcoidosis is unknown. The diagnosis of sarcoidosis is firmly established when histopathological evidence of non-caseating granulomas in affected organs supports compatible clinicoradiographic findings. Here, we present a case of a 30-year-old woman referred to our clinic with amenorrhea and polyuria. The radiological appearance of a lesion involving the pituitary stalk was an image of inflammatory infiltration, which is pathognomic for sarcoidosis,syphilis, tuberculosis and foreign body granulomatosis. Laboratory tests were done to rule out syphilis and tuberculosis. A possible diagnosis was sarcoidosis. When we searched other systems for the involvement of sarcoidosis, lungs, lymph nodes, skin and eyes were not involved by the disease. Histopathological examination of a transcranial incisional biopsy revealed a non-caseating granuloma, consisting of macrophages, epithelioid cells, and multinucleated giant cells that secrete cytokines. Around this central core, CD4 and CD8 lymphocytes, B lymphocytes, plasma cells, and fibroblasts were detected. The diagnosis was neurosarcoidosis. We present this case to draw attention to the possibility of isolated neurosarcoidosis as the differential diagnosis of pituitary lesions and review recent advances in the investigation, diagnosis and treatment of this condition.