1. 2epart EXTRAPULMONARY SMALL CELL CANCER OF THE ESOPHAGUS INTRODUCTION
N George1,3, Saad Khan3, S Adepally1, M Girotra2, B Tharian2,3
1 Division of Internal Medicine 2Division of Gastroenterology and Hepatology University of Arkansas for Medical Sciences (UAMS)
3Department of Gastroenterology and Hepatology, Peninsula Health, Australia
2epart .
Immunohistochemistry showed positive staining with AE1/AE3, synaptophysin and faint staining with chromogranin confirming small cell or poorly differentiated neuroendocrine tumor (Fig 4). He was commenced on chemotherapy, which was complicated by fatal febrile neutropenia.
DISCUSSION
Primary small cell cancer of the esophagus is an extremely rare entity, accounting for 1-3% of all esophageal tumours. Extra pulmonary small cell cancer (EP-Smcc), which comprises 2-5% of all small cell carcinomas, has characteristics similar to Lung-Smcc with increased propensity for early distant metastases. EP-Smcc of the gastrointestinal tract commonly spreads to the liver and lymph nodes. The prognosis is poor in patients with extensive disease. Palliative chemotherapy remains the main stay of treatment for metastatic disease. Although controversial, surgery with neoadjuvant chemotherapy and or radiotherapy appears to play an important role in early disease, with improvement in prognosis and a possibility of long-term remission.
CONCLUSIONS
Esophageal Smcc is rather rare but highly aggressive tumor. It is imperative to identify this early, since the outcome depends on the stage of the disease at the time of diagnosis.
INTRODUCTION
Small cell carcinoma of the oesophagus is an extremely rare aggressive tumour with very poor prognosis. Timely diagnosis is crucial as chemotherapy if commenced early may achieve a long disease free survival
CASE PRESENTATION
A 64-year-old non-smoker man with history of gout, hypertension and reflux presented with intermittent fevers, night sweats and back pain. Clinical examination was pertinent for mild pyrexia and crackles at lung bases, without any lymphadenopathy.
Initial workup demonstrated mild leukocytosis, high CRP, and cholestatic hepatic panel with normal bilirubin and preserved synthetic function. Infectious workup with blood, urine and sputum cultures was negative. There was evidence of sub-segmental atelectasis on chest Xray with concern for pneumonia, for which he was initiated on empirical antibiotics. CT abdomen indicated a large mass anterior to the gastric cardia with regional lymphadenopathy and multiple hepatic lesions (Fig 1).
Esophagogastroduodenoscopy (EGD) revealed a raised ulcerated lesion with irregular borders on the anterior esophageal wall, concerning for carcinoma (Fig 2). Biopsies confirmed poorly differentiated high-grade malignancy, with features of small cell cancer(Fig 3a ).
Figure1-Oesophageal Tumour on CT Chest/Abdomen. Fusiform thickening of lower oesophagus with regional lymphadenopathy and liver metastasis.
Figure2-Endoscopic Images. An ulcerated tumour is seen from 30 to 38cm.
3b- Immunohistochemistry positive for AE1/AE3, synaptophysin, faint staining with chromogranin
Figure 3a – Histology showing poorly differentiated highgrade malignancy, with features of small cell lung cancer