University of Massachusetts Medical School Worcetser, MA
Khaled Alsabbagh Alchirazi, MD1, Emily Weng, DO, BS2, Lidia Spaho, MD, BS3, Gustavo Churrango, MD4, Jaroslav Zivny, MD4; 1University of Massachusetts Medical School, Worcetser, MA; 2University of Massachusetts Medical School, Worcester, MA; 3University of Massachusetts Medical Center, Worcester, MA; 4UMass Memorial Medical Center, Worcester, MA
Introduction: Primary small cell esophageal carcinoma (SCEC) is extremely rare, accounting for 0.5%-2.4% of all esophageal malignancies. Though highly aggressive and often presenting with distant metastasis, it is often chemosensitive. Treatment algorithms include neoadjuvant chemoradiation followed by surgery. The data and evidence surrounding its treatment is limited as there are no randomized clinical trials or prospective clinical studies. Despite aggressive treatment, morbidity and mortality rates remain high.
Methods: A 53 year old male with history of heavy tobacco and alcohol use presented for evaluation of unintentional weight loss, dysphagia and severe GERD-like symptoms resistant to PPI and H2 blockers. An endoscopic evaluation revealed a large, partially circumferential, ulcerating mass in the middle third of the esophagus. Biopsies were significant for small cell neuroendocrine carcinoma positive for pancytokeratin, CK7, CKLMW, chromogranin and synaptophysin. The Ki67 proliferation index was 90%. A CT chest revealed a prominence in the left posterior tracheal membrane at the superior portion of the tumor concerning for invasion, but was not found on ensuing bronchoscopy. Tumor staging was T3 N2 M0. The patient was started on chemotherapy with cisplatin and etoposide, and concurrent radiation therapy. Despite treatment, he continued to experience worsening dysphagia and required palliative esophageal stent placement for progressive inability to tolerate oral intake. The patient continued to deteriorate and presented for the last time with persistent cough and shortness of breath, found to be a result of a new tracheoesophageal fistula. After a multidisciplinary discussion, the patient decided to pursue hospice care. Discussion: Small cell esophageal carcinoma is a rare disease which is often associated with both alcohol and tobacco use, as seen in our patient. Current literature recommends treatment similar to small cell lung cancer with neoadjuvant cisplatin and etoposide along with concurrent radiation therapy, however this is not without risk. Just as demonstrated with small cell lung cancer, chemoradiation used to treat SCEC can induce tracheoesophageal fistula formation. To the author’s knowledge, this is the first reported case of tracheoesophageal fistulae formation in SCEC treated with chemoradiation in the setting of persistent stenosis and esophageal stent placement. Further studies are needed to better understand this very rare disease and its deadly complications.
Disclosures: Khaled Alsabbagh Alchirazi indicated no relevant financial relationships. Emily Weng indicated no relevant financial relationships. Lidia Spaho indicated no relevant financial relationships. Gustavo Churrango indicated no relevant financial relationships. Jaroslav Zivny indicated no relevant financial relationships.