Ngo Vincent Trung H, Meyers Michael P, Qureshi Kasim, Farooq Muhammad Umar
Neurology, Trinity Health Saint Mary's - Grand Rapids, Grand Rapids, USA.
Internal Medicine, Trinity Health Saint Mary's - Grand Rapids, Grand Rapids, USA.
Cureus. 2023 Jan 10;15(1):e33605. doi: 10.7759/cureus.33605. eCollection 2023 Jan.
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 the treatment of ESCC, the standard regimen is the same as the SCC of the lung. Documented accounts of paraneoplastic encephalomyelitis associated with NETs are also uncommon. We present a patient who suffered from neurologic deficits before being diagnosed with paraneoplastic encephalomyelitis from a duodenal ESCC. The patient presented with ear pain and hematemesis. New symptoms arose after the resolution of initial symptoms, including shortness of breath and numbness. Autoimmune workup was positive for anti-Hu antibodies. A position emission tomography (PET) scan showed increased uptake in the duodenal region. Biopsy results from a duodenal ulcer revealed poorly differentiated neuroendocrine carcinoma with positive synaptophysin and strong positivity of Ki-67, consistent with ESCC. Numerous treatments, including platinum-based chemotherapy, yielded no neurologic improvement for the patient. This case details an atypical presentation of ESCC, which should be considered in patients suspected of paraneoplastic encephalomyelitis.
小细胞癌（SCC）是一种常见于肺部的神经内分泌肿瘤（NET），以快速增殖和早期转移而闻名。肺外小细胞癌（ESCC）较为罕见，胃肠道癌更是如此。由于缺乏ESCC治疗的临床数据，标准治疗方案与肺癌的SCC相同。与NET相关的副肿瘤性脑脊髓炎的文献报道也不常见。我们报告一例患者，在被诊断为十二指肠ESCC伴发副肿瘤性脑脊髓炎之前就出现了神经功能缺损。该患者表现为耳痛和呕血。初始症状缓解后出现了新的症状，包括呼吸急促和麻木。自身免疫检查抗Hu抗体呈阳性。正电子发射断层扫描（PET）显示十二指肠区域摄取增加。十二指肠溃疡活检结果显示为低分化神经内分泌癌，突触素阳性，Ki-67强阳性，符合ESCC。包括铂类化疗在内的多种治疗方法均未使该患者的神经功能得到改善。本病例详细描述了ESCC的非典型表现，对于疑似副肿瘤性脑脊髓炎的患者应予以考虑。
