An Unusual Presentation of a Solid Pseudopapillary Tumor of the Pancreas Mimicking Adenocarcinoma

Abstract

Solid pseudopapillary tumors of the pancreas are rare and typically occur in young women. Compared with pancreatic adenocarcinoma, solid pseudopapillary tumors are characterized by notable indolent biological behavior associated with a favorable prognosis. Despite their large size, these tumors rarely metastasize. Even in cases of hepatic metastasis, most lesions are usually solitary in distribution and are amenable to resection. We report a case of a 55-year-old man with a small solid pseudopapillary tumor (≤3-cm diameter) mimicking a pancreatic adenocarcinoma, with multiple hepatic metastases. The diagnosis was confirmed by endoscopic ultrasoundguided fine-needle biopsy using a 22-G core needle. Unfortunately, rapid tumor progression led to patient mortality 5 months after diagnosis. To our knowledge, this is the first case report that describes a small solid pseudopapillary tumor of the pancreas with multiple hepatic metastasis and poor prognosis in a patient who was diagnosed with this condition at the time of initial diagnosis. Clin Endosc Nov 22. [Epub ahead of print]

Huge solid pseudopapillary tumor of the pancreas ‘Frantz tumor’: a case report

“…The tumor location tends to be in the body or tail of the pancreas in 60% of the cases, while the remaining are located in the head of the pancreas (1,6,7). Even though SPNs are labeled as low malignant tumors, there have been multiple reports of metastasis (6,8,9), with an estimated incidence reaching up to 10% of the cases (1,2). We report the following case in accordance with the CARE reporting checklist (available at http://dx.doi.org/10.21037/jgo-20-180).…”

“…The pre-operative diagnosis of SPNs remains a clinical challenge despite all the current advances in diagnostic modalities (9). This is due to the overlap with a wide range of differential diagnoses such as benign cystic lesions, including pseudocysts, hydatid cysts, cystadenoma, lymphangioma, and hemangioma; as well as, different malignant lesions such as; cystadenocarcinoma or intraductal papillary mucinous neoplasms (5,7).…”

“…Tumor markers like alfa fetoprotein, carcinoembryonic antigen, CA199, CA125, and CA242 might be elevated, but they are not specific for SPNs (6). Nevertheless, these tumor markers and pancreatic tumor markers should be considered during the work-up as other malignant tumors are still part of the differential diagnosis of SPNs (9).…”

“…Surgical management with free surgical resection margins is the mainstay of treatment even with metastasis and vascular invasion, surgical excision should be performed whenever feasible (9). Radical lymphadenectomy is not indicated in these cases (5).…”

“…Even in case of recurrence or metastasis, surgery remains the treatment of choice. However, in unresectable lesions, surgical debulking might be justified (7,9).…”