Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review

Radojkovic, Danijela; Pešić, Milica; Dimic, Dragan; Petković, Tatjana; Radenković, Saša; Velojic-Golubovic, Milena; Novak, Vesna; Ilić, Ivan; Radojković, Milan

doi:10.1007/s42000-018-0024-6

Cited by 20 publications

(14 citation statements)

References 23 publications

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“…Although some stalk lesion presentations and features might favor some etiologies, most of them are nonspecific; this creates a clinical conundrum [15]. Also other etiologies such as Langerhans cell histiocytosis [LCH] are indiscernible from a germinoma by MRI [17, 18]. Therefore, as in our case initially, the majority of reported cases are treated as lymphocytic hypophysitis [3, 4] (Table 2).…”

Section: Discussionmentioning

confidence: 87%

A Rare Localized Pituitary Stalk Germinoma Presenting in the Third Decade

Bossolo

Garcia

Davila

et al. 2018

Case Reports in Endocrinology

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We report the case of a 34-year-old male Hispanic patient who presented with an 8-month history of polyuria and decreased libido. An evaluation revealed hypopituitarism, central diabetes insipidus, and a pituitary stalk lesion. No evidence of a neoplasm or an inflammatory/infiltrative disease was found. We treated the patient conservatively with steroid therapy. However, as a result of disease progression, transsphenoidal surgery was performed to obtain tissue for a pathological analysis. Histopathology revealed an intrasellar germinoma, confirmed by immunochemistry. Due to localized disease, radiotherapy was initiated and clinical improvement was noted. This case emphasizes the importance of histopathological analysis; for many physicians, the presentation of a pituitary stalk lesion in a young male adult creates a clinical conundrum. Although the most common etiologies are an inflammatory or secondary neoplasm, the possible presence of an intrasellar neoplasm should not be ruled out. In many cases, imaging characteristics and tumor markers may aid in the diagnosis without the need of an aggressive diagnostic approach. However, in this case, histopathological analysis was the only way to make a correct diagnosis and provide adequate treatment for the patient.

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Section: Discussionmentioning

confidence: 87%

A Rare Localized Pituitary Stalk Germinoma Presenting in the Third Decade

Bossolo

Garcia

Davila

et al. 2018

Case Reports in Endocrinology

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“…In sarcoidosis-induced hypophysitis hypogonadism (87.5–89%), hypothyroidism (56%), hypocortisolism (37%), and growth hormone deficiency (30%) are common. The commonest endocrine abnormality, reported in 30% of patients with LCH, is DI, although anterior pituitary deficiencies can occur either at presentation or during follow-up [18, 66, 67]. These patients usually have multiorgan and craniofacial involvement, although localized disease of the hypothalamic-pituitary region has also been reported [66].…”

Section: Resultsmentioning

confidence: 99%

“…The commonest endocrine abnormality, reported in 30% of patients with LCH, is DI, although anterior pituitary deficiencies can occur either at presentation or during follow-up [18, 66, 67]. These patients usually have multiorgan and craniofacial involvement, although localized disease of the hypothalamic-pituitary region has also been reported [66]. Identification of BRAF-V600E mutation in the peripheral blood or cerebrospinal fluid rules out other pathologies, but cannot distinguish LCH from ECD [68].…”

Section: Resultsmentioning

confidence: 99%

“…The rate of recurrence/relapse of PH after surgery is 11–25% [3, 41, 51, 52]. Only a few cases of LH have been treated with stereotactic radiotherapy, leading to resolution of symptoms after 2 weeks to 6 months [39, 66]. Radiation-induced hypopituitarism is observed in 20–30% of patients, typically occurring within the first 5 years following radiosurgery [84].…”

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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“…Limited literature data show that low-dose irradiation (≤ 22 Gy) is usually the first-line treatment and adequate in most cases of isolated HPR involved LCH. Although previous studies have shown that the natural course of LCH cannot be changed by the available treatment, a few literatures have shown that radiation treatment applied in the early stages may be effective, with partial or even complete remission [21]. According to current standard therapy in children, total surgical excision achieves good efficacy only in treating isolated CDI patients.…”

Section: Discussionmentioning

confidence: 99%

Isolated Langerhans cell histiocytosis in the hypothalamic-pituitary region: a case report

Zhou

Rao

2019

BMC Endocr Disord

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BackgroundLangerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15 years. In this disease, any organ can be involved. The skeleton, skin and lung are commonly affected, and isolated hypothalamic-pituitary (HP) involvement is relatively rare. Here we report a 17-year-old adolescent with isolated HP-LCH of enlarged pituitary stalk presented with central diabetes insipidus (CDI).Case presentationA 17-year-old male adolescent with polydipsia and polyuria accompanied with elevated serum sodium level and low urine osmolality for 3 weeks was referred to our hospital. After admission, hormonal evaluation showed that his growth hormone (GH) was slightly elevated, and serum osmolality and glucose were normal. The fluid deprivation-vasopressin test demonstrated CDI. Imaging examination showed an obvious thickening of the pituitary stalk. Lymphocytic hypophysitis, sarcoidosis and granulation tissue lesions were suspected. After oral 1-deamino-8-Darginine vasopressin (DDAVP) and prednisone were administered for 2 months, symptoms were relieved, and he discontinued taking the drugs by himself. On reexamination, imaging revealed changes in the size and shape of the pituitary stalk, with thickened nodules. Then, a diagnostic biopsy of the pituitary stalk lesion was performed. Immunohistochemistry confirmed the definitive diagnosis of LCH. The clinical symptoms subsided with oral hormone replacements.ConclusionCDI is a rare symptom in children and adolescents. Most of the causes are idiopathic, while others are caused by central nervous system (CNS) disorders. Meanwhile, lymphocytic hypophysitis, germinoma, LCH and other CNS disorders can all present as thickening of the pituitary stalk, diffuse enlargement of the pituitary gland, and weakening of high signal intensity in the neurohypophysis on magnetic resonance imaging (MRI). The differential diagnosis among these diseases depends on immunohistochemistry evidence.

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Localised Langerhans cell histiocytosis of the hypothalamic-pituitary region: case report and literature review

Abstract: Anterior pituitary deficiency can appear without DI and not only as a consequence of LCH treatment. All patients with LCH should be screened for this endocrine abnormality so that appropriate substitution therapy may be provided.

Cited by 20 publications

References 23 publications

A Rare Localized Pituitary Stalk Germinoma Presenting in the Third Decade

A Rare Localized Pituitary Stalk Germinoma Presenting in the Third Decade

Hypophysitis (Including IgG4 and Immunotherapy)

Isolated Langerhans cell histiocytosis in the hypothalamic-pituitary region: a case report

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