title: Synchronous poorly-differentiated neuroendocrine carcinoma and gastrointestinal stromal tumor of the stomach: a case report with immunohistochemical and molecular genetic analyses of KIT and PDGFRA.
abstract: Although the stomach is the most common location for gastrointestinal stromal tumor (GIST) with co-primary tumors, the synchronous appearance of a poorly differentiated neuroendocrine carcinoma (NEC) and GIST in the stomach is extremely rare. To the best of our knowledge, this is the first case of gastric GIST coexisting with gastric NEC to be reported in the literature. The current study reports the case of a 71-year-old male with gastric poorly differentiated NEC and GIST discovered incidentally during surgical treatment of the NEC. Immunohistochemistry analysis showed that the NEC tumor cells were positive for CK (cytokeratin), CD57, synaptophysin, chromogranin, CD117 (KIT protein), Dog-1 (discovered on GIST-1 protein) and CD34. The synchronous GIST immunophenotype showed positivity for CD117, Dog-1 and CD34 (100%), whereas staining for CK, SMA, desmin and S100 was negative. Ki-67 labeling of proliferating cells was 90% in NEC and 1% in GIST. An accurate diagnosis was confirmed by immunohistochemical findings. Furthermore, genetic analysis using PCR direct sequencing identified no mutations in the KIT (exons 9, 11, 13 and 17) and PDGFRA (exons 12 and 18) genes. The patient developed lymph node metastases and underwent cisplatin-based chemotherapy after the operation. This is the first documented case of synchronous gastric GIST and NEC with the examination of protein expression and gene mutations in KIT and PDGFRA, which will help to further understand the etiology and pathogenesis of NEC coexisting with GIST in a gastric location.
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Jun
authors: J
authors: Ding
authors: Department of Gastroenterology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Ping
authors: P
authors: Sun
authors: Department of Gastroenterology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Xiao-Yan
authors: XY
authors: Cai
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Shao-Hua
authors: SH
authors: Fei
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Jian
authors: J
authors: Wu
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Yu-Kai
authors: YK
authors: Qi
authors: Department of Pathology, Fudan University Shanghai Cancer Center Shanghai, People's Republic of China.
authors: Ze-Bin
authors: ZB
authors: Liu
authors: Department of Pathology, Shanghai Jiaotong University Affiliated First People's Hospital Shanghai, People's Republic of China.
authors: Lin
authors: L
authors: Yuan
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Yu-Jie
authors: YJ
authors: He
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Hui
authors: H
authors: Song
authors: Department of Pathology, Shanghai Gongli Hospital Shanghai, People's Republic of China.
authors: Wei-Xiang
authors: WX
authors: Chen
mesh_terms: D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001706:Biopsy; D018278:Carcinoma, Neuroendocrine / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D002454:Cell Differentiation; D017024:Chemotherapy, Adjuvant; D002945:Cisplatin / Q000008:administration & dosage; D004252:DNA Mutational Analysis; D005091:Exons; D005743:Gastrectomy; D046152:Gastrointestinal Stromal Tumors / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D006801:Humans; D007150:Immunohistochemistry; D033162:Incidental Findings; D008207:Lymphatic Metastasis; D008297:Male; D009154:Mutation; D009378:Neoplasms, Multiple Primary / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D011237:Predictive Value of Tests; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis / Q000235:genetics; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000032:analysis / Q000235:genetics; D013274:Stomach Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome
chemical_list: D019009:Proto-Oncogene Proteins c-kit; D020796:Receptor, Platelet-Derived Growth Factor alpha; D002945:Cisplatin
keywords: GIST; Snchronous tumor; carcinoma; gastric; neuroendocrine