Dustin Uhlenhopp, DO1, Klint Knutson, BS2, Vinaya Gaduputi, MD1, Tagore Sunkara, MD1; 1Mercy Medical Center, Des Moines, IA; 2Des Moines University, Des Moines, IA
Introduction: Small cell esophageal carcinoma (SCEC) is a highly aggressive and rare cancer, only accounting for 0.4%-2.0% of all GI neuroendocrine tumors and less than 1% of all esophageal cancers. These malignancies are typically diagnosed at advanced stages of disease, often with metastasis. Survival from time of diagnosis is 11-12.5 month with a 7.5% 5-year survival rate. We present a patient with advanced primary SCEC.
Methods: 73-year-old female with history of end-stage COPD, iron deficiency anemia, pulmonary hypertension, daily alcohol consumption, and former 20-pack-year smoker presented with hypoxia (saturation 80% on baseline home 4L oxygen), progressive dyspnea, persistent dysphagia, and unintentional 20-pound weight loss over previous 2 months. Chest x-ray showed 4x8 cm right paratracheal opacity, which was new compared to x-ray from 5 months prior. CT chest demonstrated large midthoracic esophageal mass abutting the trachea and descending aorta with malignant appearing right hilar lymph nodes. Once stabilized, EGD visualized near obstructing ulcerated lesion at 25cm in the esophagus with biopsies demonstrating SCEC. Patient started carboplatin and etoposide, the same regimen typically used for small cell lung carcinoma (SCLC). Chemotherapy was discontinued within 1 week of initiation and the patient transitioned to hospice after continued respiratory decline.
Discussion: Chest pain, dysphagia, and weight loss are the most common presenting signs of SCEC with smoking and alcohol consumption being common risk factors. By time of diagnosis, this cancer has usually invaded nearby structures or metastasized, most commonly to liver, lung, and lymph nodes with a much lower rate of metastasis to the brain than SCLC. SCEC demonstrates affinity for the middle and lower esophagus. There are several modalities for diagnosing incidental mediastinal masses, such as percutaneous, endobronchial, endoscopic, or surgical approaches depending on location. EGD is utilized for neoplasms invading the esophagus and allows for immediate cytology and accurate diagnosis. This disease is so rare that no standard treatment has been determined, but in general, treatment is similar the multimodality approach seen with SCLC. A select combination of chemotherapy agents, typically etoposide and cisplatin, radiation and surgical resection have shown to have the best prognosis by reducing tumor burden, metastasis, and preventing recurrence, although overall length of survival remains poor.
Chest x-ray demonstrating a 4 x 8 cm opacity (red arrow) in the right paratracheal-suprahilar/perihilar region later found to be esophageal small cell carcinoma.
Chest computed tomography scan with intravenous contrast demonstrating a large 8.4 x 6.0 x 9.5 cm soft tissue mediastinal mass (red arrow) involving the midthoracic esophagus abutting the trachea and descending aorta resulting in tracheal (blue arrow) and bronchi narrowing. This mass was later found to be small cell esophageal carcinoma.
Near obstructing ulcerated, friable lesion at 25cm in the esophagus visualized and biopsied with EGD (left image). Lesion bled upon touch and biopsy (right image). Cytology demonstrated small cell esophageal carcinoma.
Disclosures: Dustin Uhlenhopp indicated no relevant financial relationships. Klint Knutson indicated no relevant financial relationships. Vinaya Gaduputi indicated no relevant financial relationships. Tagore Sunkara indicated no relevant financial relationships.