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Peer-reviewedCC BYDiseases of the esophagus : official journal of the International Society for Diseases of the Esophagus

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

  • Adam Mylonakis1
  • Dimosthenis Stylios1
  • Diamantis Tsilimigras2
  • Georgios Charalampopoulos3
  • Ioannis Kotsantis4
  • Michail Liontos5
  • Ioannis Georgakopoulos6
  • Dimitrios Schizas1
6 affiliations
  1. 1First Department of Surgery, National and Kapodistrian University of Athens, Laikon General Hospital, Athens, Greece.
  2. 2Department of Surgery, Division of Surgical Oncology, The Ohio State University, Wexner Medical Center and James Comprehensive Cancer Center, Columbus, OH, USA.
  3. 3Second Department of Radiology, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece.
  4. 4Medical Oncology Unit, Second Propaedeutic Department of Medicine, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece.
  5. 5Department of Clinical Therapeutics, Division of Oncology, National and Kapodistrian University of Athens, Alexandra Hospital, Athens, Greece.
  6. 6Radiation Oncology Unit, First Department of Radiology, National and Kapodistrian University of Athens, Aretaieion Hospital, Athens, Greece.
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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.

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Cite

BibTeXRIS
BibTeX
@article{mylonakis2026leptomeningeal,
  title = {{Leptomeningeal carcinomatosis arising from esophageal cancer: a systematic review of the literature.}},
  author = {Adam Mylonakis and Dimosthenis Stylios and Diamantis Tsilimigras and Georgios Charalampopoulos and Ioannis Kotsantis and Michail Liontos and Ioannis Georgakopoulos and Dimitrios Schizas},
  journal = {Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus},
  year = {2026},
  month = nov,
  doi = {10.1093/dote/doag126},
  url = {https://europepmc.org/article/MED/42974869},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Leptomeningeal carcinomatosis arising from esophageal cancer: a systematic review of the literature.
AU  - Adam Mylonakis
AU  - Dimosthenis Stylios
AU  - Diamantis Tsilimigras
AU  - Georgios Charalampopoulos
AU  - Ioannis Kotsantis
AU  - Michail Liontos
AU  - Ioannis Georgakopoulos
AU  - Dimitrios Schizas
PY  - 2026
DA  - 2026/11/01
JO  - Diseases of the esophagus : official journal of the International Society for Diseases of the Esophagus
DO  - 10.1093/dote/doag126
UR  - https://europepmc.org/article/MED/42974869
AB  - 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.
SN  - 1120-8694
SN  - 1442-2050
ER  -