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Adult Langerhans Cell Histiocytosis: Solitary and Multisystemic Involvement Detected on FDG PET-CT Scan
Address for correspondence: Dr. Aishwarya Wagle, Nuclear Medicine, Department of Imaging, P. D. Hinduja Hospital and Medical Research Centre, Mahim West, Mumbai - 400 016, Maharashtra, India. E-mail: aishwaryawagle47@gmail.com
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Received: ,
Accepted: ,
This article was originally published by Wolters Kluwer - Medknow and was migrated to Scientific Scholar after the change of Publisher.
Abstract
Langerhans Cell Histiocytosis (LCH) is a rare neoplasm of the hematopoietic system, characterized by the clonal proliferation of Langerhans cells and it is more common in children. We present two interesting adult cases of LCH, diagnosed with the help of FDG PET-CT imaging which is often challenging to diagnose due to its rarity and nonspecific clinical presentation.
Keywords
Adult langerhans cell histiocytosis
cholangitis
fluorodeoxyglucose positron emission tomography-computed tomography
liver
pituitary
thyroid
Introduction
Langerhans cell histiocytosis (LCH) is a rare neoplasm of hematopoetic system which involves reactive clonal proliferation of Langerhans cells. It affects both children and adults, with the incidence in adults being much less and usually reported as disseminated disease.[1] LCH can involve single or multiple systems, bone being the most common. Adult LCH is usually multisystemic, with involvement of skin, nodes, liver, thyroid, and also central nervous system.[2] We report the two cases presenting with rare solitary and multisystemic LCH involvement in adults detected on fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET-CT) scan.
Case Reports
Case 1
A 26-year-old female presented to us with complains of abdominal pain and jaundice. Liver function tests were deranged, with raised liver enzymes. Magnetic resonance cholangiopancreatography (MRCP) was suggestive of sclerosing cholangitis involving liver-biliary tree. Patient was referred for FDG PET-CT scan for further evaluation with a suspicion of granulomatous etiology. FDG PET-CT scan revealed multiple FDG avid lesions showing involvement of the liver with multiple hypodense lesions, thickening enhancement in dilated proximal common bile duct, common hepatic duct, left and right hepatic ducts, with intrahepatic biliary radical dilatation (IHBRD), infiltrative hypodense lesions within thyroid gland, mild plaque like soft tissue along posteroinferior walls of left external auditory canal, soft tissue along anterior pelvic wall rectus muscles midline, and C5 vertebral lytic lesion. In addition, unusually prominent bilateral lacrimal glands were noted with no significant uptake [Figure 1]. Overall picture raised possibility of metastasis; however, no obvious primary was identified. FDG PET-guided biopsy from the metabolically active liver lesion was performed which revealed LCH. Adult LCH is extremely rare in occurrence, 1–2 cases per million annually, and has a nonspecific clinical presentation which together contributes to a delayed diagnosis.[3] In adults, LCH usually disseminates with multiorgan involvement and dysfunction and thus poor prognosis in patients with risk organ involvement (liver, spleen, and bone marrow).[3]
Case 2
A 23-year-old-girl, presented with complains of memory loss, amenorrhea, excessive sleeping, weight gain, and raised serum prolactin levels. Magnetic resonance imaging (MRI) of the brain detected lesion in suprasellar-hypothalamic region suggesting possibility of optic pathway/hypothalamic glioma. FDG PET CT scan was performed which showed solitary FDG avid nodular enhancing lesion in the suprasellar region of the brain with no other abnormal lesion in rest of the body [Figure 2]. Biopsy of this lesion was suggestive of LCH. Central nervous system involvement in Langerhans cell histiocytosis (CNS LCH) usually presents as a part of multisystemic involvement, more prevalent in men. Typically, the hypothalamic-pituitary axis, cerebellum, and basal ganglia are affected, with prevalence ranging from 3.4% to 57%.[4] Diabetes insipidus, anterior pituitary hormone deficiency, and neurodegeneration are the common manifestations in CNS-LCH; hence, such classic presentation associated with intracranial lesion should raise a suspicion for the same.[5] The rarity of LCH in adults, its multisystemic involvement, and nonspecificity of symptoms can hinder the early diagnosis. In both our cases of adult LCH, we see how FDG PET-CT can help determine solitary organ involvement or disease dissemination and further guide biopsy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Conflicts of interest
There are no conflicts of interest.
Nil.
References
- International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults. Blood. 2022;139:2601-21.
- [Google Scholar]
- Hepatic Langerhans cell histiocytosis: A review. World J Clin Oncol. 2021;12:335-41.
- [Google Scholar]
- Adult disseminated Langerhans cell histiocytosis: Incidence, racial disparities and long-term outcomes. Br J Haematol. 2018;182:579-81.
- [Google Scholar]
- Clinicoradiological discrepancy in multisystem Langerhans cell histiocytosis with central nervous system involvement. Gulf J Oncolog. 2019;1:72-7.
- [Google Scholar]
- Intracranial Langerhans cell histiocytosis: A review. Interdiscip Neurosurg. 2020;21:2214-7519.
- [Google Scholar]