Ruptured mucinous cystic neoplasm with an associated invasive carcinoma of pancreatic head in a pregnant woman: Report of a case and review of literature

Abstract

Mucinous cystic neoplasm (MCN) of the pancreas is characterized by mucin-producing columnar epithelium and an ovarian-type stroma. It occurs almost exclusively in women and is almost always located in the pancreatic body or tail. Here, we report a case of large MCN located in the pancreatic head but not in the body nor tail in a 32-year-old pregnant woman, which was thought to have grown rapidly during pregnancy. It was ruptured at 34 weeks of gestation and the patient was admitted to the emergency department of the University of Fukui Hospital with an acute abdomen. Emergency cesarean section followed by pancreaticoduodenectomy was performed. The tumor consisted of many small cysts lined by a single-layer of mucinous epithelium with papillary growth and partial solid parts showing invasive growth and sarcomatoid changes, indicating mucinous cystic neoplasm with an associated invasive carcinoma (previously referred as mucinous cystadenocarcinoma). Thickened septa revealed ovarian-type stroma strongly positive for α-inhibin and partly positive for progesterone receptor immunohistochemically. We also review and discuss previous reports of MCNs including those with an associated invasive carcinoma in pregnant patients.

Large Pancreatic Mucinous Cystic Neoplasm during Pregnancy: What Should Be Done?

“…In the English literature there are only two reports of patients who delivered vaginally at term [4,5], and in 1 case the tumor was discovered only 2 months after delivery [5]. Of the other 8 PMCNs reported, 7 were resected during the second trimester of pregnancy [6,7,8,9,10,11,12] and one at 34 weeks (due to cyst rupture [13]). In the majority of these cases, the diagnosis of PMCN was incidental during routine ultrasound evaluation in the first two trimesters of gestation, the tumors being completely asymptomatic.…”

“…Naganuma et al [13] report a PMCN ruptured at 34 weeks of pregnancy, which probably grew during pregnancy (the event imposed an emergency caesarian section, followed immediately by a pancreaticoduodenectomy). Immunohistochemistry showed only a partial immunopositivity for progesterone receptors and a strong one for α-inhibin.…”

“…This following up has to be done with great attention, due to the malignant potential of all PMCNs and due to a possible rapid tumor growth with occurrence of symptoms [8,9,11,26], IUGR [6,11] or cyst rupture [13]. …”

“…From the other 8 PMCNs reported, 7 were resected in the second trimester of pregnancy [6,7,8,9,10,11,12] and, except for one spontaneous abortion at the 10th week of pregnancy [10], all other patients uneventful vaginally delivered, giving birth to healthy newborns (table 1). The eighth PMCN was ruptured at 34 weeks of pregnancy and imposed an emergency cesarean section, immediately followed by pancreaticoduodenectomy [13]. …”

Spontaneous rupture of a pancreatic acinar cell carcinoma presenting as an acute abdomen

“…In review of literature we found only five cases of spontaneous rupture of cysadenoma of pancreas. [10][11][12][13][14][15] Naganuma et al 10 reported ruptured mucinous cystic neoplasm with associated invasive carcinoma of pancreatic head in a pregnant woman. Heim et al 11 reported ruptured cystadenoma of the pancreas in a 85-year-old woman.…”