Xanthomatous Hypophysitis Is Associated with Ruptured Rathke’s Cleft Cyst

Duan, Kai; Sylvia, L.; Winer, Daniel A.; Zadeh, Gelareh; Gentili, Fred; Mete, Özgür

doi:10.1007/s12022-017-9471-x

Cited by 34 publications

(27 citation statements)

References 46 publications

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“…None of these cases had cystic appearance on MRI, but recognized by pathological examination. Simply explained by the triggered inflammatory reaction occurring secondary to cyst rupture, this co-existence was previously reported in other cases of both lymphocytic and xantho-granulomatous hypophysitis [17,18]. Adrenal insufficiency was reported to be common in patients with Rathke's cleft cysts [19]; however, only one out of three of our cases had adrenal hormone deficiency.…”

Section: Total N Preliminary Diagnosis (N %) 20supporting

confidence: 74%

Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort

et al. 2020

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Primary hypophysitis (PH) is a rare autoimmune inflammatory disease of the pituitary gland. The aim of the study was to evaluate clinical characteristics, disease management, and outcomes of cases with PH. Medical records of PH patients admitted to Hacettepe University Hospital between 1999 and 2017 were analyzed retrospectively. Paraffin-embedded pathology blocks were obtained for both re-examination and IgG4 immunostaining. Twenty PH patients (15 females, 5 males) were evaluated. Mean age at diagnosis was 41.5±13.4 years. Some form of hormonal disorder was present in 63.2% of cases, hypogonadism (66.6%) being the most common. Panhypopituitarism was present in 36.8%. All patients had pituitary gland enlargement on magnetic resonance imaging; stalk thickening and loss of neurohypophyseal bright spot were present in 17.6 and 23.5%, respectively. Lymphocytic hypophysitis was the most common histopathological subtype (50%). Among pathology specimens available for IgG and IgG4 immunostaining (n=10), none fulfilled the criteria for IgG4-related hypophysitis. Four patients were given glucocorticoid treatment in diverse protocols; as initial therapy in 3. Sixteen cases underwent surgery, 7 of whom due to neuro-ophthalmologic involvement. Only 1 patient was observed without any intervention. Reduction of pituitary enlargement was seen in all surgical and glucocorticoid treated cases. None of the surgical patients showed hormonal improvement while one case in glucocorticoid group improved. PH should be considered in the differential diagnosis of sellar masses causing hormonal deficiencies. MRI findings are usually helpful, but not yet sufficient for definitive diagnosis of PH. Treatment usually improves symptoms and reduces sellar masses while hormonal recovery is less common.

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Section: Total N Preliminary Diagnosis (N %) 20supporting

confidence: 74%

Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort

et al. 2020

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“…LH is the commonest cause of PH, accounting for 76–86% of cases, whereas XH represents one of the rarest forms of PH, with 27 reported cases [11, 12]. It is unclear whether XH constitutes a distinct entity or a continuum of the autoimmune/lymphocytic spectrum.…”

Section: Resultsmentioning

confidence: 99%

“…The pathogenesis of XH remains unclear owing to the extremely small case numbers, and although it was first thought to be due to an infectious agent, more recent work suggest that XH may be a reactive hypophysitis occurring secondarily to a ruptured Rathke’s cleft cyst [6, 12, 41]. The histological findings of a metaplastic epithelium support this hypothesis.…”

Section: Resultsmentioning

confidence: 99%

“…XH is characterized by the presence of lipid-rich foamy histiocytes with a variable number of lymphocytes [1, 47]. The diagnosis of XH is only confirmed on tissue sections demonstrating foamy (xanthomatous) macrophages with or without cholesterol clefts, admixed with variable amounts of multinucleated giant cells [12]. In GH the pituitary is infiltrated by histiocytes, multinucleated giant cells, and lymphocytes along with plasma cells [15, 39, 48].…”

Section: Resultsmentioning

confidence: 99%

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Hypophysitis (Including IgG4 and Immunotherapy)

et al. 2020

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Hypophysitis is characterized by inflammation of the pituitary gland that can be primary (PH) or secondary (SH) to other diseases or following drug administration. It may also be classified according to anatomical and histopathological criteria, leading to variable degrees of hypopituitarism and/or compressive symptoms to nearby structures. There has recently been an increase in the number of hypophysitis cases, raising the interest on the spectrum of its pathogenesis, clinical, biochemical/endocrinological, and imaging features. However, the use of conventional biomarkers, including currently utilized pituitary autoantibodies, has relatively limited diagnostic accuracy. Lymphocytic hypophysitis (LH) is the commonest cause of PH, whereas IgG4-related hypophysitis is increasingly being recognized. Histiocytosis and granulomatous diseases are the most frequent causes of SH, although infections and lymphoma have also been reported. The increasing use of immune checkpoint inhibitors in oncology is associated with a high incidence of hypophysitis, providing further understanding of its pathogenesis. Hy-pophysitis can occur silently and be easily missed, potentially leading to substantial morbidity or mortality due to adrenal insufficiency, requiring a high index of clinical suspicion and timely initiation of appropriate treatment. In most cases of LH or drug-induced hypophysitis, active surveillance along with replacement of established hormonal deficiencies is needed. In the presence of compressive and/or evolving symptoms, treatment with glucocorticoids either alone or in combination with other immunosuppressive agents can be used. Surgical decompression is reserved for nonresponsive cases with threatened vital structures. Timely diagnosis and intervention are important to minimize disease-related morbidity and mortality. We aimed to review current concepts and recent developments in the pathogenesis, diagnosis, and management of hypophysitis.

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“…A rare type of hypophysitis is xanthomatous inflammation (about 3% of cases [4]), discovered in 1998. It is believed that this variant may be an inflammatory reaction to Ratke's cleft cyst rupture [20,21]. It has been more frequently reported in women (3:1), in their fourth decade of life.…”

Section: Xanthomatous Inflammationmentioning

confidence: 99%

Hypophysitis — new insights into diagnosis and treatment

Kluczyński

Gilis‐Januszewska

Rogoziński

et al. 2019

Endokrynologia Polska

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Hypophysitis is a broad term used to describe conditions leading to inflammation of the pituitary gland and the pituitary stalk. It may develop as a primary condition or secondary to other diseases. Hypophysitis is classified based on aetiological, anatomical, and histological criteria. Clinical symptoms result from enlargement of the pituitary gland, hormonal deficiencies, diabetes insipidus, and hyperprolactinemia. Histopathological verification of tissue samples from a pituitary biopsy remains the gold standard in diagnosing hypophysitis. However, due to the invasiveness and risk of the procedure it is rarely performed. The diagnosis is based mainly on clinical presentation, laboratory tests, and imaging. The rarity of the disease and the deficit in reliable data result in a lack of clear guidelines in the treatment of hypophysitis. The basic therapy relies on hormonal replacement. High doses of steroids are the first-line treatment of symptoms caused by mass effect in sella and compression of surrounding structures. In steroid-resistant patients or in cases of inacceptable sides effects, treatment with other immunosuppressant drugs was administered with success. The course of the disease varies: some patients present remission, in other cases hypophysitis leads to fibrosis and atrophy of the pituitary gland, which is reflected in persistent hormonal deficiencies and images of an empty sella. The objective of this article is to present the most important information: the epidemiology, clinical image, diagnostic procedures, and treatment of primary hypophysitis, in order to allow better understanding of this disease and implementation of proper management. Posttraumatic and immunotherapyrelated hypophysitis are also briefly characterised.

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Xanthomatous Hypophysitis Is Associated with Ruptured Rathke’s Cleft Cyst

Cited by 34 publications

References 46 publications

Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort

Clinical Characteristics, Management, and Treatment Outcomes of Primary Hypophysitis: A Monocentric Cohort

Hypophysitis (Including IgG4 and Immunotherapy)

Hypophysitis — new insights into diagnosis and treatment

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