Tonsillar langerhans cell sarcoma with gastrointestinal metastasis: a rare case report

Langerhans cell sarcoma (LCS) is a rare, aggressive neoplasm of Langerhans cells with potential for multi-organ involvement. LCS appearing in the tonsils and spreading to the gastrointestinal (GI) tract is extremely rare. This report describes a case of tonsillar LCS with GI metastasis in an elderly female.

An 83-year-old female with a history of over 50 years of heavy smoking presented in February 2024 with progressive left tonsillar swelling, dysphagia, significant weight loss, and pain impacting her ability to eat. Physical examination revealed a left tonsillar mass and cervical lymphadenopathy, with an Eastern Cooperative Oncology Group performance status of 2-3. Initial neck computed tomography (CT) imaging demonstrated a 3 cm × 3.5 cm × 6.4 cm left oropharyngeal mass with extensive local invasion and necrotic cervical lymphadenopathy. Positron emission tomography-CT (PET-CT) confirmed hypermetabolic activity in the left nasopharyngeal region and further invasion. Histological examination of an incisional biopsy, from the tonsillar mass, confirmed LCS, with positive immunohistochemical markers (S100, CD1a, BCL6, Langerin) and a high Ki-67 index (90%). The patient underwent radiation therapy and pembrolizumab for metastatic control. Despite treatment, a PET-CT revealed progression with abdominal lymph node involvement, pleural effusion, and gastric wall thickening. In July 2024, she experienced acute upper GI bleeding managed with blood transfusions and supportive care, with endoscopic biopsy confirming metastatic spread. By August 2024, the patient was readmitted with an acute abdomen due to gastric perforation, leading to a transition to palliative care due to LCS progression and GI complications. The patient died six months after initial diagnosis.

Further studies are needed to explain the pathogenesis of LCS, investigate targeted therapeutic approaches, and develop standardized management protocols for localized and metastatic LCS presentations to improve outcomes.