Lymphocytic hypophysitis: Can open exploration of the sella be avoided?

Prasad, Ashoka Jahnavi; Madan, V S; Sethi, Prahlad K.; Prasad, Manju L.; Buxi, T. B. S.; Kanwar, Chandra K.

doi:10.3109/02688699109002889

Cited by 40 publications

(16 citation statements)

References 10 publications

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“…It is generally believed that conservative management is appropriate in the majority of patients with lymphocytic hypophysitis since spontaneous resolution of both the mass and the hypopituitarism has been reported [20][21][22][23]. However, the experience of this case implies that a diagnosis of lymphocytic hypophysitis from PPL is challenging.…”

Section: Discussionmentioning

confidence: 49%

Primary Pituitary Lymphoma Presenting as Hypophysitis

et al. 2005

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Abstract.A 47-year-old man had suffered from prolonged fever for two months without clinical evidence of infection. Blood biochemistry and endocrine dysfunction indicated that he had pituitary insufficiency. Thorough whole body imaging studies merely identified a 22 × 14 mm mass lesion in the sella turcica. Tumor pathology and special cell marker study revealed the infiltration of atypical T-lymphoid cells and concomitant presence of some B-lymphoid cells. The fever subsided gradually following subtotal tumor resection and steroid supplementation. However, the mass lesion had invaded the cavernous sinus and optic chiasma shortly after surgery. Six months after his initial visit, metastasis lesions in the liver, the left adrenal gland, and retroperitoneal lymph nodes were discovered. In contrast to cells in the pituitary, the pathological investigation of the liver mass confirmed it to be exclusively of T-cell origin. Therefore, it is plausible that the pituitary dysfunction was related to an inflammatory process, namely hypophysitis, as well as the T-cell lymphoma. This case exemplifies the rarely noted condition of primary pituitary lymphoma with concomitant hypophysitis. Clinical diagnosis is indiscernible until the occurrence of systemic tumor metastasis.

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Section: Discussionmentioning

confidence: 49%

Primary Pituitary Lymphoma Presenting as Hypophysitis

et al. 2005

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“…Transsphenoidal surgery is, however, both diagnostic and therapeutic and, therefore, should be performed in cases with progressive compression or those in whom radiographical and/or clinical progression occurs during conservative medical management (1,31,32). Hyperprolactinemia (33)(34)(35) and pituitary insufficiency (36,37) resolve after pituitary surgery, in most cases (1).…”

Section: Discussionmentioning

confidence: 97%

The Spectrum and Significance of Primary Hypophysitis

Cheung

Ezzat²,

Smyth

et al. 2001

The Journal of Clinical Endocrinology &Amp; Metabolism

162

119

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Hypophysitis can present clinically as a mass lesion of the sella turcica. Secondary hypophysitis occurs in cases where a definite etiologic agent or process inciting the inflammatory reaction can be identified. In contrast, primary hypophysitis refers to inflammation confined to the pituitary gland with no identifiable etiologic associations. We report three cases of primary hypophysitis to illustrate the spectrum of three clinicopathological entities that encompass this disease: lymphocytic hypophysitis, granulomatous hypophysitis, and xanthomatous hypophysitis. Our three patients underwent surgery, with variable response. However, conservative, supportive treatment with or without surgical decompression is generally favored over aggressive and extensive surgical resection that results in hypopituitarism. We conclude that the optimal management of patients with hyophysitis requires a high index of suspicion before extensive surgical resection. Histological confirmation of the diagnosis of hypophysitis can be obtained by performing a biopsy or by requesting an intraoperative frozen section consultation.

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“…At present, it is likely that most cases of suspected LYH with significant hormonal dysfunction and/or mass effect symptoms will end up necessitating tissue histology for definite diagnosis. Some have suggested transphenoidal stereotactic biopsy of the mass as a useful tool in the diagnosis of LYH in selected cases [62][63][64]. However, such procedures could be misleading by failing to obtain tissue from the actual lesion.…”

Section: Diagnosismentioning

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: Can open exploration of the sella be avoided?

Cited by 40 publications

References 10 publications

Primary Pituitary Lymphoma Presenting as Hypophysitis

Primary Pituitary Lymphoma Presenting as Hypophysitis

The Spectrum and Significance of Primary Hypophysitis

Lymphocytic hypophysitis: Disease spectrum and approach to diagnosis and therapy

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