A Case Of Spontaneous Regression Of Adult Multi-system Langerhans Cell Histiocytosis Presenting As Liver Tumor Rupture
Oshima, S., Inano, S., Honjo, G., Tabata, S., Fujimoto, M., Haga, H., & Kitano, T. (2025). Spontaneous Regression of Adult Multi-system Langerhans Cell Histiocytosis Presenting as Liver Tumor Rupture. Internal medicine (Tokyo, Japan), 64(8), 1217–1222. https://doi.org/10.2169/internalmedicine.4060-24
View Original Source →Abstract
Adult multisystem Langerhans cell histiocytosis (MS-LCH) is rare and has a poor prognosis. A 67-year-old man with MS-LCH presented with a hepatic tumor rupture and multiple masses in the lungs, liver, and pancreas. Despite the initial aggressive disease course and involvement of organs at risk, the patient experienced spontaneous regression and lesion disappearance following smoking cessation without chemotherapy. A literature review revealed a distinct subset of MS-LCH that can be managed by smoking cessation and careful observation through follow-up imaging. This suggests that careful observation through follow-up imaging may be a reasonable alternative to chemotherapy in select adult cases of MS-LCH.
Case Details
Disease Location
Liver, lung, pancreas
Personal Characteristics
67-year-old man. Medical history of sjögren’s syndrome and psoriasis with no active treatment and hypothyroidism with levothyroxine supplementation. Smoker of 1 pack of cigarettes per day for more than 40 years.
Clinical Characteristics
Presented with pain in the right upper quadrant. Adominal examination revealed rebound tenderness in the upper right quadrant. Laboratory findings revealed a slightly elevated liver enzyme level and lactate dehydrogenase level. Elevated c-reactive protein level and leukocytosis. Computed tomography revealed rupture of a liver tumor, measuring 45 mm in the s5/s6 region, as well as multiple masses of different sizes in the liver, lungs, and pancreas. The lung masses exhibited necrotic changes. Metastatic hepatocellular carcinoma and pancreatic adenocarcinoma were suspected. Endoscopy-guided core biopsy of the pancreatic mass revealed proliferation of medium to large tumor cells with abundant eosinophilic cytoplasm, conspicuous nucleoli, nuclear atypia, and frequent mitotic figures. Lung mass biopsy revealed proliferation of large highly mitotic cells with nuclear atypia. Immunohistochemistry revealed that these cells were positive for cd1a, s-100a indicative of langerhans cell origin. A diagnosis of langerhans cell sarcoma (lcs) was initially made.
Remission Characteristics
PET/CT 3 weeks after admission showed that the multiple masses spontaneously regressed. A week later, a biopsy of the liver mass, which had decreased in size from 45 to 30 mm, revealed extensive necrosis in most areas of the mass and low ki-67 expression. At the fourmonth follow-up, the masses continued to regress, with some completely disappearing following smoking cessation and watchful waiting
Treatment & Mechanisms
Proposed Remission Mechanisms
Smoking cessation can contribute to the improvement of the tumor microenvironment, possibly leading to reactivation of immune cells and tumor regression
Clinical Treatment
Biopsy
Non-Clinical Treatment
Smoking cesassation