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Verrucous esophageal carcinoma is a unique indolent subtype of squamous cell carcinoma: a systematic review and individual patient regression analysis.
- Source :
-
Journal of gastroenterology [J Gastroenterol] 2021 Jan; Vol. 56 (1), pp. 12-24. Date of Electronic Publication: 2020 Oct 20. - Publication Year :
- 2021
-
Abstract
- Background and Aims: Verrucous esophageal carcinoma (VEC) is a rare malignancy that presents a diagnostic challenge. We aim to characterize the clinical and genomic features, tumor behavior, and treatment outcomes of VEC to guide clinical practice.<br />Methods: We performed a systematic review of the literature and identified additional cases from Massachusetts General Hospital records and The Cancer Genome Atlas (TCGA). We obtained individual VEC patient data and analyzed publicly available clinicogenomic data from TCGA. We performed a regression analysis comparing cases of VEC to esophageal squamous cell carcinoma (ESCC) to identify factors influencing survival.<br />Results: A total of 135 patients were reported in 82 publications, and four unpublished cases from Massachusetts General Hospital (median age 65 years, 69% males, 48% smokers, 33% consumed alcohol). Symptoms were present at diagnosis in 95% of patients, most commonly dysphagia and weight loss. Median symptom onset to diagnosis time was 11.5 months with frequent misdiagnosis as Candida esophagitis. Among VEC cases with pathologic staging, lymph node metastases were rare (5%) compared to ESCC (40%). VEC was genomically characterized by enrichment of SMARCA4 missense mutations and a lack of pathogenic TP53 mutations. Despite its diagnostic elusiveness, in a multivariate regression analysis, VEC was detected at earlier stages (p = < 0.001) compared to ESCC, and advanced stage was the only significant factor affecting survival (p = 0.013).<br />Conclusions: VEC is a rare, clinically and genomically distinct subtype of ESCC. Recognition and diagnosis of this lesion may allow the pursuit of curative and less morbid treatment strategies.
- Subjects :
- Adult
Aged
Aged, 80 and over
Biomarkers, Tumor genetics
Combined Modality Therapy
DNA Helicases genetics
Early Detection of Cancer
Female
Follow-Up Studies
Humans
Male
Middle Aged
Mutation, Missense
Nuclear Proteins genetics
Regression Analysis
Survival Analysis
Transcription Factors genetics
Treatment Outcome
Tumor Suppressor Protein p53 genetics
Carcinoma, Verrucous diagnosis
Carcinoma, Verrucous genetics
Carcinoma, Verrucous mortality
Carcinoma, Verrucous therapy
Esophageal Neoplasms diagnosis
Esophageal Neoplasms genetics
Esophageal Neoplasms mortality
Esophageal Neoplasms therapy
Esophageal Squamous Cell Carcinoma diagnosis
Esophageal Squamous Cell Carcinoma genetics
Esophageal Squamous Cell Carcinoma mortality
Esophageal Squamous Cell Carcinoma therapy
Subjects
Details
- Language :
- English
- ISSN :
- 1435-5922
- Volume :
- 56
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Journal of gastroenterology
- Publication Type :
- Academic Journal
- Accession number :
- 33079233
- Full Text :
- https://doi.org/10.1007/s00535-020-01736-1