Work

Leptomeningeal carcinomatosis arising from esophageal cancer: a systematic review of the literature

Year: 2026

Type: review

Abstract: Leptomeningeal carcinomatosis (LMC) is a rare but devastating complication of solid tumors. Involvement from esophageal cancer is exceedingly uncommon and poorly characterized, with evidence limited to case reports and small series. PubMed/MEDLINE, Scopus, Embase, Web of Science, and the Cochrane Library were systematically searched by two investigators for LMC originating from esophageal cancer. Thirty-one studies reporting 43 patients were included (93% male; mean age 59.3 years). Esophageal adenocarcinoma was the predominant histology (86%), followed by squamous cell carcinoma (11.6%), most commonly from the distal esophagus or esophagogastric junction. LMC occurred synchronously in 27.9% and metachronously in 72.1%, with a median interval of 11.2 months between primary diagnosis and leptomeningeal disease. At diagnosis, 37.2% had isolated leptomeningeal involvement without extracranial metastases. Presentation was heterogeneous, most frequently including headache or meningeal symptoms (55.8%), vestibulocerebellar manifestations (39.5%), and cranial nerve involvement (37.2%). Cerebrospinal fluid cytology was positive in 81.1% of tested cases, often requiring repeat lumbar punctures. Molecular data were reported for 27.9% of patients; Human Epidermal Growth Factor Receptor 2 (HER2) was positive in 3 of 10 tested cases (30%). Management was individualized: radiotherapy in 37.2%, intrathecal therapy in 20.9%, and supportive care alone in 46.5%. Median survival was 1.1 months, though three patients achieved 12, 14, and 19 months following Central Nervous System (CNS)-directed therapy. LMC from esophageal cancer is rare and clinically aggressive, characterized by heterogeneous presentation, frequent diagnostic delay, and poor prognosis. Early recognition and palliative care are essential, while rare long-term survivors suggest selected patients may benefit from individualized CNS-directed treatment.

Source: Diseases of the Esophagus

DOI: 10.1093/dote/doag126