A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus

Nicholas, P. Daniel; Garrahy, Ian

doi:10.1080/20009666.2019.1698231

Cited by 4 publications

(4 citation statements)

References 12 publications

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“…Several adult hypothalamic-pituitary LCH patients were reported to present central diabetes insipidus as first symptom. 9,10) Thus, we should have considered LCH at first time she came to our hospital. The initial diagnosis of the pituitary stalk lesion was gangliocytoma.…”

Section: Discussionmentioning

confidence: 99%

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy

Kono

Inomoto

Horiguchi

et al. 2021

NMC Case Report Journal

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Langerhans cell histiocytosis (LCH) is a disease characterized by the proliferation of Langerhans cells. Most cases of LCH occur in children, although it can be seen in adults as well. We encountered an adult case of LCH. A 44-year-old woman who was diagnosed as diabetes insipidus underwent a magnetic resonance imaging (MRI) of the head which revealed sellar and suprasellar gadolinium-enhanced mass. Prolactin level was high and cabergoline was prescribed. The size of this mass had reduced, so we supposed the tumor was prolactinoma. However, after 4 years of observation, it had increased once again. The biopsy of pituitary stalk lesion was performed via transcranial approach. The histological diagnosis was initially gangliocytoma. The patient complained of back pain after surgery. Three months after the biopsy, a computed tomography (CT) scan revealed multiple osteolytic lesions throughout the entire body. One of the osteolytic lesions of the skull was removed to determine the diagnosis. The pathological examination of the skull led to a diagnosis of LCH. We concluded retrospectively that the lesion of the pituitary stalk was LCH mimicking gangliocytoma though classical pathological findings were not obtained. In conclusion, LCH should be considered as a differential diagnosis in adult cases of diabetes insipidus with hypothalamic-pituitary lesion.

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Section: Discussionmentioning

confidence: 99%

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy

Kono

Inomoto

Horiguchi

et al. 2021

NMC Case Report Journal

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“…It may result when the hypothalamic-pituitary axis is involved with consequent impairment of antidiuretic hormone secretion from the posterior pituitary gland. 10 The condition typically presents with polydipsia, polyuria, and nocturia. LCH may affect any organs of the body.…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, CDI is the most frequent CNS manifestation of LCH, occurring in 10-50% of all patients. 10,12 Polyuria and polydipsia in DI and primary polydipsia do not necessarily differ in their specific manifestations, even though the underlying impairment of the urinary concentrating mechanism is different in the two conditions. In a day patient could urinate 4000-5000 cc and drink 4000-5000 cc per day.…”

Section: Discussionmentioning

confidence: 99%

Central Diabetes Insipidus in Langerhans Cell Histiocytosis: A Case Report

Lestari

Rini

2023

Bioscmed

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Background: Diabetes insipidus (DI) is part of a group of hereditary or acquired polyuria and polydipsia diseases. Diabetes insipidus can be caused by central and nephrogenic disorders. This study aimed to describe the etiologies, clinical symptoms, and management of central diabetes insipidus in Langerhans cell histiocytosis. Case presentation: A 4 years 4 months old boy came with excessive and frequent micturition since 9 months ago. The patient drinks 4-5 L per day and still feels thirsty. The patient had a history of Langerhans cell histiocytosis (LCH). During laboratory work-up, urine osmolarity decreased, and serum osmolarity and electrolyte were normal. The patient was diagnosed with central diabetes insipidus with Langerhans cell histiocytosis. The treatment given to the patient is desmopressin. Conclusion: Langerhans cell histiocytosis may affect any organs of the body. The long-term management of diabetes insipidus in Langerhans cell histiocytosis requires measurement to prevent dehydration and, at the same time to prevent water intoxication. The focus of management is based on the education of the patient about the importance of regulating their fluid intake according to the patient’s hydration status.

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“…It has been reported that the initial symptoms of adult patients with hypothalamic pituitary LCH include diabetes insipidus. [ 11 ] Central diabetes insipidus is an important clinical manifestation of posterior pituitary involvement and a marker for evaluating LCH disease activity, [ 12 ] Enhanced MRI showed a decreased signal of the posterior pituitary gland and/or thickened pituitary stalk (>3 mm). In this case, the signal of the posterior pituitary gland was significantly reduced, but the pituitary stalk was not thickened (about 2.67 mm).…”

Section: Discussionmentioning

confidence: 99%

A rare imaging presentation with multisystemic clinicopathological features of Langerhans cell histiocytosis: Case report and literature review

Li,

Wang,

Liu

et al. 2023

Medicine

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Rationale: Langerhans cell histiocytosis (LCH) is a kind of rare disease in which dendritic cells proliferate abnormally. It often occurs in children and can involve any tissue and organ. The affected sites usually include bone, skin, pituitary gland, and lungs, while the thyroid gland and external auditory canal are rarely observed. The perineal and labial involvement of this disease has not been reported yet. Patient concerns: A 47-year-old female patient experienced a swelling of the anterior neck area without an obvious inducement. She noticed a quail egg-like mass on the left side, and the mass increased progressively within 3 months. The anterior neck area was found to be swollen, and some flaky red rashes were seen on the scalp and bilateral external auditory canals. Diagnoses: Imaging examination showed enlarged thyroid and cervical lymph nodes, multiple low-density nodules in the liver, and reduced signal in the posterior pituitary gland. The biopsy pathological result of the increased left cervical lymph node indicated that LCH was detected. Interventions: VP regimen (vincristine, dexamethasone per os) and related supportive treatments were given as inducing chemotherapy for 6 weeks. Outcomes: After the second chemotherapy, the rash on the scalp and external auditory canal improved, and the neck mass was significantly reduced. After the third chemotherapy, the rash was mostly disappeared, while the neck lumps increased during chemotherapy. Thus, clatribine chemotherapy was recommended as the follow-up. Lessons: Imaging examinations played an important role in the diagnosis and follow-up of the disease, especially 18F-FDG PET/CT, which could show multiple involving organs at the same time. When a patient suffering from diabetes insipidus, skin rash, or fever, has a high FDG uptake PET/CT result in multiple tissues and organs throughout the body, it is necessary to consider the possibility of LCH.

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A case of multisystem Langerhans cell histiocytosis presenting as central diabetes insipidus

Cited by 4 publications

References 12 publications

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy

Adult Langerhans Cell Histiocytosis Diagnosed by Biopsy of the Skull Tumor Generated after Craniotomy

Central Diabetes Insipidus in Langerhans Cell Histiocytosis: A Case Report

A rare imaging presentation with multisystemic clinicopathological features of Langerhans cell histiocytosis: Case report and literature review

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