Verrucous esophageal carcinoma is a unique indolent subtype of squamous cell carcinoma: a systematic review and individu
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ORIGINAL ARTICLE—ALIMENTARY TRACT
Verrucous esophageal carcinoma is a unique indolent subtype of squamous cell carcinoma: a systematic review and individual patient regression analysis Darrick K. Li1 • Samir Haffar2 • Masayasu Horibe3,8 • Haya A. Homsi4 Lawrence Zukerberg5 • Mohammad H. Murad6 • Kavel H. Visrodia1 • Manish Gala7,9 • David A. Katzka8 • Fateh Bazerbachi9
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Received: 24 August 2020 / Accepted: 26 September 2020 Ó Japanese Society of Gastroenterology 2020
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. 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. Results A total of 135 patients were reported in 82 publications, and four unpublished cases from Massachusetts Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00535-020-01736-1) contains supplementary material, which is available to authorized users.
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). 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.
& Manish Gala [email protected]
5
Department of Pathology, Massachusetts General Hospital, Boston, MA, USA
& Fateh Bazerbachi [email protected]
6
Evidence-Based Practice Center, Robert D. and Patricia E. Kern Center for the Science of Health Care Delivery, Mayo Clinic, Rochester, MN, USA
7
Clinical and Translational Epidemiology Unit, Massachusetts General Hospital, Boston, MA, USA
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Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN, USA
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Assistant in Medicine, Instructor in Medicine, Harvard Medical School, Division of Gastroenterology, Department of Medicine, Massachusetts General Hospital, 55 Fruit Street, GRJ 825D, Boston, MA 021
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