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

Gupta

Indian Journal of Nuclear Medicine

et al. 2022

Langerhans cell histiocytosis (LCH), also known as histiocytosis X, is a rare systemic disorder arising from clonal proliferation of immature CD207-positive (langerin) myeloid dendritic cells (histiocytes) in the skin and visceral organs with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. The incidence of LCH is very less in adult and occurs almost exclusively in children. Genital, perianal, and lung lesions are considered to be rare manifestations of adult LCH. We describe a case of 31-year-old, nonsmoker female who presented in February 2020 with itching and burning sensation in perianal and vulvar regions accompanied with multiple nonhealing ulcers and papillomatous lesions. These lesions gradually increased in size with no response to antibiotics and topical steroids. She was advised positron-emission tomography– computed tomography (PET-CT) scan for further evaluation. After PET-CT scan, her provisional diagnosis of multisystem, multifocal Langerhans cell histiocytosis with high-risk organ involvement was made. Both vulvar and perianal lesions were biopsied which was suggestive of Letterer–Siwe variant of LCH. The prognosis of this variant is very poor even with aggressive chemotherapy and 5-year survival rate of only 50%. Hence, it requires careful consideration during diagnosis and management.

Section: Discussionmentioning

confidence: 74%

Multifocal, Multisystem Presentation of Adult-Onset Langerhans Cell Histiocytosis on 18F-Fluorodeoxyglucose Positron-Emission Tomography–Computed Tomography

Gupta

Indian Journal of Nuclear Medicine

et al. 2022

Clin Pediatr Endocrinol

“…For patients with only a solitary bone lesion, such as one in the hands or feet, spontaneous resolution may occur and no treatment is acceptable. Meanwhile, the indications for chemotherapy for solitary hypothalamic-pituitary or cranial bone lesions remain controversial, and active surveillance is an alternative strategy ( 18 , 19 ). There are only a few series with small numbers of patients or case reports regarding the treatment of LCH with central nervous system lesions ( 19 ).…”

Section: Discussionmentioning

confidence: 99%

Potential indication of chemotherapy for hypodipsia and arginine vasopressin deficiency secondary to hypothalamic-pituitary Langerhans cell histiocytosis: a case report and literature review

Ota,

Sato,

Nakano

et al. 2024

Hypothalamic-pituitary Langerhans cell histiocytosis (HP-LCH) is often associated with arginine vasopressin deficiency (AVD). Patients with AVD caused by HP-LCH rarely develop an impaired osmotic threshold for thirst (OTT). Improvement in OTT among such patients has not been reported in the literature. To our knowledge, here we report the first case of AVD due to HP-LCH in which hypodipsia resolved during chemotherapy. A nine-year-old Japanese girl presented with polydipsia, polyuria, anorexia, and hypernatremia (149.8 mEq/L) and was diagnosed with AVD secondary to HP-LCH. Visual analog scale examination showed a reduced OTT following the water deprivation test. During chemotherapy for Langerhans cell histiocytosis (LCH), serum sodium concentrations became stable between 138.9 and 142.9 mEq/L under the replacement of desmopressin. Repeated visual analog scale examinations showed that she experienced a sense of thirst at a serum sodium concentration of 142.3-144.6 mEq/L, at which she did not experience any thirst prior to the initiation of chemotherapy. These data suggest that chemotherapy directly improved the OTT in our patient. Improved mechanical compression or infiltration of the hypothalamus related to OTT may lead to the recovery of the sense of thirst. This report highlights the potential role of chemotherapy for solitary HP-LCH in patients with hypodipsia and AVD.

The Indian Journal of Chest Diseases and Allied Sciences

“…76,77 Although isolated CNS LCH is reported, CNS LCH is mainly diagnosed as multisystemic disease, and the majority of CNS LCH patients are men. 72,[78][79][80] The treatment of adult LCH is not well defined and standardized due to very less incidence of disease and inadequate knowledge regarding its pathophysiology. It has been still debated whether LCH might have a neoplastic nature rather than an inflammatory or reactive nature.…”

Section: Discussionmentioning

confidence: 99%

Langerhans Cell Histiocytosis in an Adult Female with Multisystem Involvement

Gupta¹,

Singh²,

Chugh³

et al. 2023

Langerhans cell histiocytosis (LCH) also known as histiocytosis X, is a rare systemic disorder arising from the clonal proliferation of myeloid dendritic cells (histiocytes) with a tendency to involve single or multiple organ systems with variable clinical course and prognosis. Clinical presentation usually depends on the site of involvement. The organs commonly affected in adults by order of decreasing frequency include lungs, bone, skin, pituitary glands, lymph nodes, and the liver. Vulval and perianal involvement is extremely rare in adults. We describe the case of a 31-year-old non-smoker adult female with multisystemic LCH involving the vulva, perianal region, and lung. Probable involvement of other sites with LCH included mandibular bone, pituitary gland, skin, lymph nodes, liver, thyroid, and colon. She is undergoing systemic chemotherapy and has completed two cycles of cytarabine and steroids without any complications. Treatment is not standardized due to the very less incidence of the disease and inadequate knowledge regarding its pathophysiology. Langerhans cell histiocytosis remains a major concern for treating physicians because of its rarity with many faces and requires careful consideration for management.