Anaplastic carcinoma associated with a mucinous cystic neoplasm of the pancreas during pregnancy: Report of a case and a review of the literature

Abstract

Oncogenesis of anaplastic carcinoma of the pancreas is a subject of controversy, because it shows sarcomatous nature with extremely poor prognosis. We herein report an unusual case of anaplastic carcinoma occurring with a recurrent mucinous cystic neoplasm in a 38-year-old female. A 10-cm retroperitoneal cystic mass was pointed out in the first pregnancy and a probable diagnosis of mucinous cystic neoplasm was made in October 2000. She refused surgery first and delivered her baby uneventfully. During her second pregnancy in 2002, however, she presented hematemesis and underwent urgent distal pancreatectomy, splenectomy and partial resection of the gastric wall where the tumor perforated. A diagnosis of borderline-type mucinous cystic neoplasm with ovarian-like stroma was made. Nine months later, CT visualized a recurrent cystic tumor near the pancreatic stump, which was subsequently resected. Pathology revealed that the tumor was composed of two different components of borderline-type mucinous cystic neoplasm and anaplastic carcinoma. The latter was intensely positive for vimentin, CD68, p53 and focally for cytokeratin, suggesting both sarcomatous and carcinomatous differentiation. She survived four years after the second surgery without tumor recurrence. Although the origin of anaplastic carcinoma has not been determined yet, it should be remembered that anaplastic carcinoma can occur in association with mucinous cystic neoplasm of more benign histology.

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

“…Among five cases of pancreatic cystadenocarcinoma in pregnant patients reported to date (Table 1), 5–9 two were ruptured cases during pregnancy 5,8 . Most of MCNs are located in the pancreatic body or tail, 1,2 and indeed, all of these previously reported tumors were located in the pancreatic body or tail 5–9 . Also, the communication of MCN with the pancreatic ducts was rarely reported, and it is considered to be due to erosion of the expanding tumor into the duct, rather than true intraductal origin 21,22 .…”

“…Pancreatic MCNs usually grow slowly and most of them show benign appearance without infiltrative growth into adjacent tissues 1,2 . However, there have been several case reports showing rapid growth during pregnancy and presenting the critical problem of fetal intrauterine growth restriction 3–14 . Ganepola et al 3 .…”

“…MCNs are often found incidentally and may occasionally cause symptoms. However, MCN in pregnant women is extremely rare and only a few cases have been reported to date 3–14 . The predilection of MCNs for women and its rapid growth in pregnancy indicate a major role for the sex hormones in the histogenesis and malignant transformation of MCN, but the details of this relationship remain unclear 3…”

Cytopathology of anaplastic carcinoma of the pancreas: Review of a rare entity and description of a variant with signet ring cell features

“…Squamous differentiation and neuroendocrine differentiation have been described in ACP. Pancreatic neoplasms that are known to harbor focal areas with anaplastic carcinoma include mucinous neoplasms . Awareness of this possible differentiation and careful examination of similar cases is warranted to avoid missing an aggressive component …”

Pancreatic Neoplasms in Pregnancy: Diagnosis, Complications, and Management

“…[12] In an interesting case of an MCN diagnosed during the first trimester, the patient refused surgical resection until the second trimester of her next pregnancy; she then developed a recurrence, which the pathology demonstrated to be anaplastic carcinoma. [3]…”