A previously healthy 31-year-old female presented with a 1-year history of microcytic anemia, which was associated with abdominal pain and a 10-pound weight loss. On physical examination, there was a palpable tender mass in the left upper quadrant of the abdomen. Previous investigations with an esophagogastroduodenoscopy and capsule endoscopy were essentially normal. However, an ultrasound of the abdomen revealed a complex multiseptated cystic mass measuring 16.5 × 9 × 11.5 cm within the pancreatic tail, as well as splenomegaly. Subsequent computed tomography scan of the abdomen confirmed the presence of the multiseptated lesion and it appeared to be closely associated with both the stomach and the body and tail of the pancreas. Figure A shows a CT coronal image of the dumbbell-shaped multiseptated pancreatic mass, and an axial image is shown in Figure B. Further imaging with endoscopic ultrasound (EUS) revealed a multiseptated, predominantly cystic extrinsic mass, which appeared to be originating from the distal pancreas and also invading the stomach. Its ultasonographic features were consistent with a cystadenoma (Figure C) and scant mucin was visible on EUS-guided fine-needle aspiration. Serum tumor markers including carcinoembryonic antigen, carbohydrate antigen (CA) 19-9 and CA 125 were all within normal limits.
During surgical exploration, a 19.5-cm encapsulated multilocular cyst was completely resected via distal pancreatectomy and splenectomy with en bloc subtotal distal gastrectomy, as the lesion was inseparable from the stomach. A gross image of the pancreatic mucinous cystic neoplasm encased in a smooth outer wall is seen in Figure D, and Figure E shows the multilobulated nature of this lesion in a cross-sectional image. This mass was confirmed to be originating from the pancreatic tail, with invasion of the adjacent gastric wall possibly because of prior cyst rupture leading to local inflammation and adhesion. Furthermore, it was occluding the splenic vein, and hence causing extensive venous congestion with resultant splenomegaly. On histology, the pancreatic mucinous cystic neoplasm was composed of glands lined by tall, mucin-producing cells, and ovarian-type stroma was also present without any evidence of invasive carcinoma cells (Figure F; H&E; original magnification ×100). Additionally, the ovarian-type stromal cells tested positive for estrogen and progesterone receptors. The patient had an uneventful hospital course and was discharged home on postoperative day 9.
Mucinous cystadenomas, which are also known as mucinous cystic neoplasms (MCN), account for about 10% of pancreatic cysts.1 They usually occur in middle-aged perimenopausal women and are diagnosed 20 times more commonly in women than men.1, 2 The majority of these lesions arise from the pancreatic body or tail1, 2, 3 and can develop into fairly large cysts of up to 35 cm in size.3 They consist of a thick, smooth outer capsule enclosing mucoid cystic fluid, and the presence of ovarian-type stroma is a pathognomonic feature.2, 3 Complete surgical resection of a noninvasive MCN is curative2 and is associated with extremely favorable prognosis with 100% survival and 0% recurrence rates.3 However, 6%–36% of MCNs are invasive mucinous cystadenocarcinomas, and these are associated with poorer 5-year survival rates of 20%–60%.3 Therefore, prompt diagnosis and treatment with surgical excision is paramount in the management of mucinous pancreatic cysts and this diagnosis should be considered in all patients presenting with a cystic pancreatic mass.
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