Pancreatic mucinous cystic neoplasm with sarcomatous stroma metastasizing to liver: a case report and review of literatu
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WORLD JOURNAL OF SURGICAL ONCOLOGY
CASE REPORT
Open Access
Pancreatic mucinous cystic neoplasm with sarcomatous stroma metastasizing to liver: a case report and review of literature Michael Wayne*, Deniz Gur, Gil Ascunce, Ben Abodessa and Violette Ghali
Abstract We report a case of mucinous cystic neoplasm of pancreas with sarcomatous stroma metastasizing to the liver. The tumor occurred in a male patient aged 46 years. Symptoms included persistent epigastric and right upper quadrant pain. Radiographically, the pancreas contained four large cystic masses located in the neck, body, and tail. Histologically, the cysts were lined with benign, mucinous epithelium with underlying bland, storiform, ovarian-like stroma. An undifferentiated focally hyalinized, sarcomatous stroma composed of bland spindle cells showing short fascicular growth pattern and focal nuclear palisading was associated with the epithelial component in one of the cysts. These cells showed strong immunoreactivity with vimentin and inhibin (weak), they were negative for CD34, estrogen receptor, progesterone receptor, androgen, calretinin, S-100, CD117, melan A, chromogranin, and synaptophysin. A morphologically and immunohistochemically identical metastatic sarcomatous focus was identified in the liver without any glandular component. This case is unique in its clinically malignant behaviour and metastatic nature despite its morphologically benign epithelial and stromal components.
Background Mucinous cystic neoplasms (MCNs) represent one of the major cystic neoplasms of the pancreas. They have distinct clinicopathological features. They are seen in women in the fifth or sixth decades of life; only rarely are examples documented in men. The tumor is usually located in the tail of the pancreas. Macroscopically, the cysts are multilocular and they do not communicate with the pancreatic ductal system. Histologically, the lining epithelium consists of tall, columnar cells with abundant apical mucin, although cuboidal cells that lack obvious mucin may also be present. The epithelium may be bland in appearance or might range from mild to severe atypia resembling ovarian counterparts and include mucinous cystadenomas, borderline mucinous cystic tumors, and mucinous cystadenocarcinomas. Another distinct histologic feature is the presence of subepithelial hypercellular, ovarian-like spindle cell stroma, and its presence has become a requirement for the diagnosis of this entity [1,2]. Here we report a case of mucinous cystic neoplasm of the pancreas with sarcomatous stroma metastasized to liver. * Correspondence: [email protected] Biliary and Pancreatic Surgery of New York, Beth Israel Medical Center, New York, USA
Case presentation
A 46-year-old man presented with persistent epigastric and right upper quadrant pain. Endoscopic ultrasound studies revealed multiple cysts in the pancreas (Figures 1A,B). Computed tomography (Figure 1C) of the abdomen showed a large mass in the neck and body of the pancreas measuring 6.8 × 5.7 cm in cross-section, consiste
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