Pure lymphocytic infundibuloneurohypophysitis caused by the rupture of rathke's cleft cyst; report of 2 cases and review of the literature

Abstract

The major symptoms that are caused by Rathke's cleft cysts (RCCs) are visual disturbances, headaches, and endocrine insufficiencies. Among these symptoms, the endocrine insufficiencies are thought to result from the spreading of inflammation that is induced by the cyst contents onto the pituitary gland or the compression of the gland and the pituitary stalk by RCCs. Here, we present 2 rare cases with lymphocytic infundibulohypophysitis with the sudden onset of headaches and subsequent diabetes insipidus (DI). Magnetic resonance imaging revealed remarkable swelling of the pituitary gland with a small mass that was located between the anterior and the posterior lobe of the pituitary gland. Transsphenoidal surgery was performed to remove the mass, and pathological examinations of the cyst wall demonstrated that the epithelial tissue of the RCC and the posterior lobe were affected by massive lymphocytic infiltration. The clinical courses and pathological results of these patients strongly suggested that the rupture of the RCC onto the posterior lobe caused the lymphocytic hypophysitis. Postoperatively, the DI could be controlled with a smaller amount of anti-diuretic hormone replacement compared to that required preoperatively. KEywoRds:Hypophysitis, Rathke's cleft cyst, Lymphocyte, Diabetes insipidus ÖZ Rathke yarığı kistlerinin (RCC'ler) neden olduğu ana belirtiler görme bozuklukları, baş ağrıları ve endokrin yetmezliklerdir. Bu belirtiler arasında endokrin yetmezliklerin, kist içeriğinin oluşturduğu enflamasyonun hipofize yayılması veya hipofizin ve hipofiz sapının RCC'ler ile kompresyonu sonucunda oluştuğu düşünülmektedir. Makalede, aniden baş ağrısı ve sonrasında diabetes insipidus (DI) gelişen 2 nadir lenfositik infundibulohipofizit olgusu sunuldu. Manyetik rezonans görüntüleme hipofizin anterior ve posterior lobu arasında küçük bir kitleyle birlikte hipofizde belirgin şişme göstermiştir. Kitleyi çıkarmak için transsfenoidal cerrahi yapılmış ve kist duvarının patoloji incelemeleri epitelyal RCC dokusu ve posterior lobda masif lenfositik infiltrasyon göstermiştir. Bu hastalarda klinik seyir ve patoloji sonuçları, lenfositik hipofizitin nedeninin RCC'nin posterior loba rüptürü olduğunu kuvvetle düşündürmüştür. Postoperatif olarak DI preoperatif olarak gerekenden daha az miktarda antidiüretik hormon replasmanıyla kontrol altına alınabilmiştir.

Revisitation of autoimmune hypophysitis: knowledge and uncertainties on pathophysiological and clinical aspects

“…• The rupture of Rathke' cleft cyst may cause lymphocytic-infundibulo-neurohypophysitis. Two cases were published in 2016 by Hayashi et al [133] presenting with visual disturbances, headaches and endocrine insufficiencies, followed by diabetes insipidus. These are two rare cases of lymphocytic-infundibulo-neurohypophysitis confirmed by post-surgery pathological examination, showing a massive lymphocytic infiltration of the cyst wall and the posterior lobe [133].…”

“…Two cases were published in 2016 by Hayashi et al [133] presenting with visual disturbances, headaches and endocrine insufficiencies, followed by diabetes insipidus. These are two rare cases of lymphocytic-infundibulo-neurohypophysitis confirmed by post-surgery pathological examination, showing a massive lymphocytic infiltration of the cyst wall and the posterior lobe [133]. • Specific characteristics of possible autoimmune hypophysitis may also be observed in patients in late phase of complete or selective hypopituitarism, as in patients with Sheehan's syndrome [59,60,134], with hypopituitarism occurring some years after a brain injury [125,135] or after meningo-encephalitis [136] or in children with celiac disease and short stature, who do not show significant increase in stature on gluten-free diet [96].…”

Rathke Cleft Cyst with Entirely Ossified Cyst Wall and Partially Solid Cyst Content: A Case Report and Literature Review

“…6,19 In the symptomatic RCCs, mucus, hemorrhage from the cyst wall result in chronic inflammations of the adjacent hypophysis leading to irreversible changes. 1,2,7 In our patient, preoperative panhypopituitarism was thought to be caused by irreversible changes to the hypophysis. Levels of all hormones secreted from the anterior lobe were significantly lower, and the DI was apparent after hormonal replacement.…”

“…1,2,5 The inflammation spread by the cyst content and the compressive action of the cyst on the surrounding structures rarely induce headaches, endocrinological insufficiency, and visual function disturbance. [6][7][8] Calcification of the cyst wall in RCC is rare and ossification occurs even less frequent. To our knowledge, only four cases have been reported so far.…”

Clinical presentation and outcome of children with central diabetes insipidus associated with a self‐limited or transient pituitary stalk thickening, diagnosed as infundibuloneurohypophysitis

“…At neuroimaging follow‐up, a range of pituitary stalk size variations can be observed, from spontaneous resolution to further enlargement . In those cases with a self‐limited and/or transient stalk thickening and a normal (or sometimes decreasing) anterior pituitary size at serial imaging, the diagnosis of (lymphocytic or autoimmune) infundibuloneurohypophysitis (INH) has been made more frequently during the last years, even in the absence of circulating auto‐antibodies or a histological confirmation of lymphocytic infiltration. The diagnosis of INH is frequently made by exclusion and remains in the first years of presentation uncertain, even with histological confirmation .…”