PMC3894914_01
Abdominal CT scan of case 1 showing the gastric mass (A), and retroverted endoscopic view showing the tumor at the gastric fundus with adjacent two satellite lesions (B).
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Computerized tomography (CT) scan of the abdomen showed a mass in the stomach (Figure 1A) and hence was referred for further management. Repeated upper GI endoscopy showed a mass occupying the proximal part of the stomach with 2 satellite lesions (Figure 1B).
The operative specimen showing the en-block resection of stomach, spleen and tail of pancreas (A). The cut surface of the specimen eliciting the large nodular whitish tumour (B).
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An en-block total gastrectomy, splenectomy and distal pancreatectomy was performed (Figure 2A, 2B) with Roux-en-Y oesophago-jejunal anastmosis.
Histological photograph of the tumour. (A) Gastric tumor showing necrosis (Haematoxylin and Eosin stain 10x), (B) CD34 immunostain is positive (20x), (C) S100 immunostain is positive (10x), (D) Immunohistochemistry picture showing positivity of the tumor cells to S100 indicating its neural origin.
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Histology of the specimen showed a heterogeneous multifocal malignant spindle cell/epithelioid cell tumor exhibiting variable patterns ranging from low grade sclerostic spindle cell areas to high grade epithelioid cell areas/nodules with high cellularity, geographical/palisading necrosis (Figure 3A) and frequent mitoses (Mitotic rate is 40/10 HPF). Immunohistochemical staining revealed focal positive staining with for Vimentin, CD34 (Figure 3B), and S100 (Figure 3C), but were negative for c-kit (CD117), DOG1, SMA, Desmin, HMB45, ALK1, EMA, Pancytokeratin and NSE. The histological immunohistologic findings (Figure 3C, 3D) are consistent with a gastrointestinal autonomic nerve tumor which represents a phenotypic variant of gastrointestinal stromal tumor.
CT scan showing the lung metastatic lesion (A), and PET/CT showing the liver and adrenal metastases appearing 2 months after operative resection of the primary lesion (B). (C) Showing PET body scan in which the bony metastases in the left humorous, right scapula and right femur are clearly demonstrated.
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There was also a left chest solitary metastasis (Figure 4A) and an enlarged necrotic lymph node in the hepato-gastric ligament. A PET/CT 18F-FDP whole body scan showed FDG-avid pulmonary, upper abdominal, hepatic, adrenal, bony and muscular metastases (Figure 4B, 4C).