Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old girl: case report and review of the literature

Cemeroğlu, Ayşe Pínar; Blaivas, Mila; Muraszko, Karin M.; Robertson, Patricia L.; Vázquez, Delia M.

doi:10.1007/s004310050690

Cited by 45 publications

(25 citation statements)

References 33 publications

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“…Lymphocytic infundibuloneurohypophysitis was excluded because it has been reported in adults as being confined to the posterior pituitary and being self-limited (5). Lymphocytic hypophysitis as classically conceived was unlikely for several reasons: 90% of reported cases were in females but they were older except in one case (22) and in at least 65% associated with pregnancy; 60% had symptoms such as headache and visual defect; 40% had hyperprolactinemia with functional involvement confined to the anterior pituitary (19)(20)(21); the MRI picture was that of pituitary enlargement (80%) mimicking pituitary adenoma (20). None of these features was found in our patient.…”

Section: Discussionmentioning

confidence: 59%

Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

Maghnie¹,

Genovese²,

Sommaruga³

et al. 1998

Eur J Endocrinol

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We report on a 15-year-old girl who had presented with acute onset central diabetes insipidus at the age of 8 years; this was followed by growth failure due to acquired growth hormone deficiency. Initial magnetic resonance imaging showed a uniformly enlarged pituitary stalk and absence of posterior pituitary hyperintensity. Frequent patient examination and magnetic resonance imaging gave unchanged results until after 5 years a large hypothalamic mass and panhypopituitarism were found. Dynamic magnetic resonance imaging documented hypothalamic-pituitary vasculopathy. Histopathological examination revealed perivascular inflammatory lymphoplasmic infiltrates with no granulomatosis or necrosis and negative staining for S-100 protein, suggesting autoimmuneinflammatory disease (lymphocytic infundibuloneurohypophysitis?). The response to glucocorticoid pulses (30 mg/kg per day for 3 days i.v.) was favorable, the hypothalamic mass being halved and partial anterior pituitary function recovery maintained for 2 years after the start of treatment. We suggest that long-term surveillance is needed for isolated and chronic thickening of the pituitary stalk and that dynamic magnetic resonance imaging can contribute to the demonstration of hypothalamic-pituitary vascular impairment associated with local vasculitis.

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Section: Discussionmentioning

confidence: 59%

Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

Maghnie¹,

Genovese²,

Sommaruga³

et al. 1998

Eur J Endocrinol

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“…The anterior pituitary function in LINH is frequently intact. When there is adenohypophysis involvement, it may be mild and often transient, involving most commonly GH [17,20,53], but also gonadotropins and thyrotropin [5,14,19,22,53]. Prolactin is usually normal or may be just slightly elevated [13,23,25].…”

Section: Lymphocytic Adenohypophysitis (Lah)mentioning

confidence: 99%

“…Pathologic examination of tissue from patients with LINH has in fact revealed lymphocytic inflammation limited to the infundibulum, the pituitary stalk and the neurohypophysis [13]. Cases of LINH have been reported with increasing frequency in the last decade [14][15][16][17][18][19][20][21][22][23][24][25][26] and this condition is now regarded as a LYH variant. LINH is also referred to as stalkitis, infundibulo-stalkitis, neurohypophysitis and necrotizing infundibulo-hypophysitis [14,22].…”

Section: Introductionmentioning

confidence: 96%

Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy

2006

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Lymphocytic hypophysitis (LYH) is a neuroendocrine disorder characterized by autoimmune inflammation of the pituitary gland with various degrees of pituitary dysfunction. The histopathology consists of an initial monoclonal lymphocytic infiltrate, which can heal with minimal sequela or progress to fibrosis and result in permanent hypopituitarism. Coexistence of other autoimmune conditions is reported in 25-50% of cases and pituitary autoantibodies have been detected in up to 70% of biopsy-proven cases. The clinical presentation varies depending on the pituitary segment that is more severely affected. In lymphocytic adenohypophysitis (LAH) an early destruction of the ACTH-producing cells is characteristic. Other anterior pituitary hormones can also be affected but posterior pituitary involvement is absent or minimum. Lymphocytic Infundibuloneurohypophysitis (LINH) typically presents as acute onset diabetes insipidus (DI) with intracranial mass-effect symptoms. A combination of extensive anterior pituitary involvement and DI characterizes lymphocytic Infudibulopanhypophysitis (LIPH). The diagnosis can be challenging in many cases, because distinction from pituitary adenomas and other sellar masses is not obvious. Significant efforts have been made to identify specific serum markers, but it would seem unlikely that this approach will ever have the specificity to replace histopathological examination of a surgical specimen. Diagnostic criteria have been proposed to help in the decision-making process and to avoid, whenever possible, unnecessary invasive procedures. The therapeutic approach is controversial and, although transsphenoidal surgery is often performed, a conservative medical management is justified in many cases, given the self-limited nature of the inflammatory process. This paper reviews the etiology, epidemiology, clinical and radiological findings, diagnosis and management of LYH.

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“…Lymphocytic hypophysitis is a rare inflammatory disorder of the pituitary gland which occurs mostly in young women during pregnancy or postpartum period. This disease is considered to be an autoimmune process because of its frequent association with other autoimmune disorders, especially with Hashimoto’s thyroiditis [4]. Hypophysitis occurs rarely in children.…”

Section: Discussionmentioning

confidence: 99%

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

Edouard¹,

Stafford

Oliver³

et al. 2009

Horm Res Paediatr

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We report the clinical course of 2 patients with central diabetes insipidus and evolving to panyhypopituitarism which prompted the diagnosis of an isolated pituitary stalk thickening (PST). In both patients, all etiological investigations were normal and the first biopsy revealed an isolated lymphocytic infiltrate with no sign of malignancy. Close clinical follow-up accompanied by serial brain MRIs was proposed to determine a precise diagnosis and for early detection and treatment of neoplastic disease. In our first case, the diagnosis of germinoma was made 9 months after the PST diagnosis owing to tumor progression. In the second case, the time course was even longer with the diagnosis of germinoma 6 years following initial presentation. In these cases, it is speculated that the lymphocytic infiltrates represent the first sign of a host reaction to an occult germinoma. To our knowledge, this is the third case reported of lymphocytic infiltrates preceding a germinoma in a prepubertal girl, and the only case reported in a prepubertal boy. These cases underline the difficulties in establishing the diagnosis of germinoma in a patient with isolated PST.

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Lymphocytic hypophysitis presenting with diabetes insipidus in a 14-year-old girl: case report and review of the literature

Cited by 45 publications

References 33 publications

Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

Evolution of childhood central diabetes insipidus into panhypopituitarism with a large hypothalamic mass: is 'lymphocytic infundibuloneurohypophysitis' in children a different entity?

Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy

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

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