Primary non-necrotizing granulomatous hypophysitis mimicking pituitary adenomas

Abstract

AIm:The authors review their experience in the endoscopic endonasal transsphenoidal treatment of 5 patients, finally diagnosed as primary hypophysitis but initially assumed to be pituitary adenomas. mATERIAL and mETHods: A retrospective study was undertaken to review 5 cases of primary non-necrotizing granulomatous hypophysitis (1.61%) through 310 endoscopic transsphenoidally operated cases with the diagnosis of pituitary adenoma between 2009 and 2013. All 5 cases were female without any background of autoimmunity or recent pregnancy. The initial presumptive diagnosis was pituitary adenoma for all patients. The endocrinological diagnoses of the patients were suspected Cushing's Disease, anterior pituitary deficiency with hyponatremia, hyperprolactinemia, and acromegaly. One of the patients had normal hormonal levels. All patients had macroadenomas including one invasive adenoma with skull base involvement. One of the patients (20%) had visual field defects. All patients underwent endoscopic endonasal transsphenoidal surgery (EETS). REsuLTs:All patients had improvement of hormonal levels postoperatively except the one with anterior pituitary deficiency who required long term hormone replacement after the surgery. Mean follow-up duration was 14.8 months.CoNCLusIoN: Primary granulomatous hypophysitis without any known etiological factors is very rare in the literature. It can mimic pituitary adenomas in radiological and endocrinological aspects. EETS is an effective and safe treatment especially for visual and compression symptoms.KEywoRds: Primary hypophysitis, Non-necrotizing, Granulomatous, Endoscopic, Secretory adenoma ÖZ AmAÇ: Hipofiz adenomu ön tanısı ile endoskopik endonazal transsfenoidal (EETS) yöntemle tedavi edilen ve patoloji tanısı primer hipofizit olan 5 olguluk deneyim sunulmaktadır. yÖNTEm ve GEREÇLER: Çalışmada 2009 ile 2013 yılları arasında endoskopik endonazal yolla opere edilen 310 hipofiz adenomu olgusu içerisinden patoloji tanısı primer nonnekrotizan granulomatöz hipofizit olan 5 olgu (%1,61) geriye dönük olarak incelendi. Hastaların hepsi kadın cinsiyette olup, hiçbirinde gebelik ya da altta yatan otoimmün hastalık gibi bir neden yok idi. Tüm hastaların ön tanısı hipofiz adenomu idi. Hastalarda endokrinolojik olarak sırasıyla şüpheli Cushing Hastalığı, hiponatremi ile birlikte hipofizer yetmezlik, hiperprolaktinemi, normal hormonal tablo ve akromegali ile uyumlu laboratuvar değerleri saptandı. Bir tanesi kafa kaidesi tutulumu ile birlikte invaziv adenom olmak üzere tüm olgular makroadenomdu. Bir hastada görme alanı defisiti saptandı. Tüm hastalara EETS cerrahi uygulandı.BuLGuLAR: Preoperatif yetmezlik bulguları olan hasta hariç tüm hastalarda postoperatif normal hormonal değerler sağlandı.Hastalar ortalama 14,8 ay takip edildi.soNuÇ: Primer non-nekrotizan hipofizitler nedeni bilinmeyen ve nadir görülen hastalıklar olup radyolojik ve endokrinolojik olarak hipofiz adenomlarını taklit edebilirler. EETS yol ise görme ile ilgili ve bası semptomlarının kaldırılmasında güvenli ve etkili bir...

Granulomatous hypophysitis: rare disease with challenging diagnosis

“…In the present case, the absence of D.I. and pituitary stalk thickening mimicked pituitary macroadenomas . Headache and visual symptoms as a result of progressive chiasmal compression are the most common presenting symptoms of granulomatous hypophysitis.…”

“…Headache and visual symptoms as a result of progressive chiasmal compression are the most common presenting symptoms of granulomatous hypophysitis. Patients may also present with amenorrhea–galactorrhea, hyperprolactinemia, fatigue, and aseptic meningitis . Granulomatous hypophysitis is difficult to differentiate from simple adenoma based on its clinical manifestations or radiological findings, and the diagnosis is usually made by histopathologic findings .…”

“…Morning cortisol and T4 free levels were low while the rest of pituitary hormones were within normal level indicating hypopituitarism. Consideration was given to prompt surgical intervention to decompress both optic pathway and obtain a tissue diagnosis .…”

“…A trans‐sphenoidal biopsy with fast‐frozen pathology was able to diagnose hypophysitis likely to be granulomatous. Corticosteroid therapy may be a potential treatment for hypophysitis, as complete removal of pituitary masses may disable pituitary function .…”

Idiopathic Granulomatous Hypophysitis Mimicking Pituitary Abscess

“…Histologically, inflammatory hypophysitis is classified into 5 subtypes: lymphocytic, granulamatous, xanthomatous, xanthogranulomatous, and necrotizing. 4 , 5 Hypophysitis can also be classified as idiopathic hypophysitis and secondary hypophysitis which may be caused by systemic inflammatory disorders such as tuberculosis, Wegener's granulomatosis, histiocytosis-X, and sarcoidosis. 4 , 6 The majority of inflammatory pituitary lesions occur in women, are related with the postpartum period, and most of them are in the form of LH.…”