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Abstract Details

Anti-hu Associated Encephalomyelitis as a presentation of Primary Extrapulmonary Small Cell Carcinoma of the Small Bowel
Autoimmune Neurology
P17 - Poster Session 17 (11:45 AM-12:45 PM)
9-001
N/A
Introduction: Small cell carcinoma (SCC) is a neuroendocrine tumor (NET) commonly found in the lung, known for rapid proliferation and early metastasis. Extrapulmonary small cell carcinomas (ESCC) are rare, with GI tract carcinomas exceedingly so. Due to the lack of clinical data on treatment of ESCC, the standard regimen is the same as SCC of the lung. NETs are often considered only after extensive workup, increasing the risk of metastasis and severity of symptoms before diagnosis. Documented accounts of paraneoplastic encephalitis associated with NETs are rare. We present a 53 year-old female who suffered from neurologic deficits before being diagnosed with ESCC in their duodenum.
N/A

Case description: The patient initially presented with ear pain and was noted to have developed hematemesis from a duodenal ulcer treated with PPIs. Months after resolution of ear pain, new symptoms arose including diplopia, numbness, and shortness of breath. Autoimmune workup was positive for anti-GAD-65 and ANNA-1 antibodies. During an IVIG session, the patient had respiratory distress requiring BiPaP. A PET scan showed increased uptake in the duodenal region. Due to impaired respiratory function EGD was delayed. After a tracheostomy, EGD showed persisting duodenal ulcer. Biopsy results revealed poorly differentiated neuroendocrine carcinoma with positive synaptophysin and strong positivity of Ki-67, consistent with ESCC. Subsequent plasmapheresis and rituximab treatment had minimal effect. A Whipple procedure was recommended, though timing the procedure was complicated by tenuous respiratory function and rituxan dosing. The patient was discussed at the tumor board where platinum-based chemotherapy was recommended. The patient decided on comfort care after no symptomatic relief with chemotherapy.

Discussion: This case details an atypical presentation of ESCC, which should be included in the differential for paraneoplastic encephalitis patients. Further clinical data on treatment of these carcinomas would benefit future patients.
Authors/Disclosures
Vincent Trung Ngo, MD
PRESENTER
Dr. Ngo has nothing to disclose.
Kasim Qureshi, MD (Henry Ford Hospital) Dr. Qureshi has nothing to disclose.
No disclosure on file
Muhammad Farooq, MD (Neuroscience Program,Saint Mary'S Health) Dr. Farooq has nothing to disclose.