Adult Langerhans cell histiocytosis of skull in a patient with synchronous papillary thyroid carcinoma and Castleman disease

• Published in: BMJ case reports Vol. 14; no. 1; p. e239341
• Main Authors: Kim, In Kyeong, Lee, Kyoung Yul
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 25.01.2021; BMJ Publishing Group
• Subjects: Abdomen; Antibodies, Monoclonal - therapeutic use; Antineoplastic Agents - therapeutic use; Autoimmune diseases; Biopsy; Bone Diseases - complications; Bone Diseases - diagnostic imaging; Bone Diseases - pathology; Bone Diseases - surgery; Bone marrow; Case Report; Case reports; Castleman Disease - complications; Castleman Disease - diagnostic imaging; Castleman Disease - drug therapy; Castleman Disease - pathology; Cell growth; Dendritic cells; Disease; Epidural; Epstein-Barr virus; Etiology; Hemoglobin; Histiocytosis, Langerhans-Cell - complications; Histiocytosis, Langerhans-Cell - diagnostic imaging; Histiocytosis, Langerhans-Cell - pathology; Histiocytosis, Langerhans-Cell - surgery; HIV; Human immunodeficiency virus; Humans; Hyperplasia; Immunotherapy; Kinases; Lymphatic system; Magnetic Resonance Imaging; Male; Metastasis; Middle Aged; Monoclonal antibodies; Nervous system; Neurosurgery; Patients; Pediatrics; Plasma; Positron Emission Tomography Computed Tomography; Scintigraphy; Skull; Stains & staining; Targeted cancer therapy; Thymus gland; Thyroid cancer; Thyroid Cancer, Papillary - complications; Thyroid Cancer, Papillary - diagnostic imaging; Thyroid Cancer, Papillary - pathology; Thyroid Cancer, Papillary - surgery; Thyroid Neoplasms - complications; Thyroid Neoplasms - diagnostic imaging; Thyroid Neoplasms - pathology; Thyroid Neoplasms - surgery; Tomography; Tomography, X-Ray Computed; thyroid disease; pathology; neurosurgery; CNS cancer; haematology (incl blood transfusion)
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2020-239341

We report an extremely rare case of adult Langerhans cell histiocytosis (LCH) in a patient with papillary thyroid carcinoma (PTC) and Castleman disease (CD). A 46-year-old man visited our hospital with anaemia; systemic imaging showed an abdominal and a left thyroid mass. Biopsy confirmed CD for the abdominal mass and PTC for the thyroid mass. Two months after, he presented with headache and a right parietal lump. Brain CT and enhanced MRI revealed an osteolytic mass with enhancement in the right parietal skull. Surgical removal and biopsy confirmed the diagnosis of skull LCH. The BRAF mutation was positive on PTC and negative on CD and LCH. We conducted surgical resection only for PTC and LCH; surgical resection with siltuximab for multicentric CD. At the 25-month follow-up, there was no recurrence or progression. We may consider of syndromic nature of these diseases to establish a treatment strategy.