Lymphocytic hypophysitis with central diabetes insipidus and subsequent hypopituitarism masking a suprasellar germinoma in a 13-year-old girl

Abstract

Lymphocytic hypophysitis in children may be the first sign of a host reaction to an occult germinoma. The diagnosis of central diabetes insipidus with a thickened pituitary stalk requires long-term follow-up to establish the underlying cause.

Isolated Lymphocytic Infiltration of Pituitary Stalk Preceding the Diagnosis of Germinoma in 2 Prepubertal Children Treated with Growth Hormone

“…To our knowledge, this is the third case reported of lymphocytic infiltrates preceding a germinoma in a prepubertal girl [2, 3] and the only case reported in a prepubertal boy. In the first case report, the diagnosis of germinoma was established in a 12 6 / 12 -year-old girl 2 years after PST diagnosis and 5 months after pituitary biopsy showing an isolated lymphocytic infiltrate [2].…”

“…In the first case report, the diagnosis of germinoma was established in a 12 6 / 12 -year-old girl 2 years after PST diagnosis and 5 months after pituitary biopsy showing an isolated lymphocytic infiltrate [2]. In the second case report, the diagnosis of germinoma was established in a 14-year-old girl 10 months after PST diagnosis with isolated lymphocytic infiltration on pituitary stalk biopsy [3]. In these 2 prior cases, the diagnosis of lymphocytic hypophysitis was suspected.…”

Perifocal Inflammatory Reaction with Volume Fluctuation Caused by Diagnostic Radiation-Induced Regression in Germinoma Makes Histological Diagnosis Difficult despite Its Disappearance following Treatment: A Significant Pitfall and Countermeasures to It

“…The diagnosis of DI in children requires a long-term follow-up beyond the pubertal age in order to establish the underlying cause [35] . In contrast to lymphocytic hypophysitis in adults, lymphocytic hypophysitis in prepubertal children may represent the first sign of a host reaction to an occult germinoma [4,8,9] . In other words, a germinoma can cause an inflammatory lymphocytic process [6] .…”

“…Some case reports of granulomatous neurohypophyseal germinoma are available [2,3] as well as germinoma masquerading as lymphocytic hypophysitis [4][5][6][7][8][9][10][11] . A granulomatous inflammation/ reaction can mislead histopathological diagnosis if biopsy shows mainly granulomatous tissues [1,2,12,13] .…”

An unusual suspect causing behavioural problems and pituitary failure in a child

“…The radiotherapy and/or surgery following diagnosis often results in hypopituitarism even if the primary tumour does not 6. Non-neoplastic causes include trauma7 and lymphocytic hypophysitis 8. Children with hypopituitarism often present with symptoms and signs of pituitary hormone deficiency (hypoglycaemia, growth failure, hypernatraemia, etc).…”