Surgical management of solid-pseudopapillary neoplasms of the pancreas (Franz or Hamoudi tumors): a large single-institutional series

Abstract

Background: Solid-pseudopapillary neoplasms (SPNs) are rare pancreatic tumors with malignant potential. Clinicopathologic characteristics and outcomes of patients with SPN were reviewed.

Study design: Longterm outcomes were evaluated in patients with an SPN who were followed from 1970 to 2008.

Results: Thirty-seven patients were identified with an SPN. Thirty-three (89%) were women, and median age at diagnosis was 32 years. Most patients were symptomatic; the most common symptom was abdominal pain (81%). Thirty-six patients underwent resection; one patient with distant metastases was not operated on. There were no 30-day mortalities. Median tumor size was 4.5 cm. Thirty-four patients underwent an R0 resection, 1 had an R1 resection, and 1 had an R2 resection. Two patients had lymph node metastases, and one patient had perineural invasion. After resection, 34 (94%) patients remain alive. One patient died of unknown causes 9.4 years after resection, and another died of unrelated causes 25.6 years after operation. The patient with widespread disease who didn't have resection died 11 months after diagnosis. Thirty-five of the 36 patients having resection remained disease free, including those who died of unrelated causes (median followup, 4.8 years). One patient developed a recurrence 7.7 years after complete resection. She was treated with gemcitabine and remains alive 13.6 months after recurrence.

Conclusions: SPNs are rare neoplasms with malignant potential found primarily in young women. Formal surgical resection may be performed safely and is associated with longterm survival.

Figure 1

Cross-sectional imaging of a solid-pseudopapillary neoplasm (SPN). (A) Axial CT image of an SPN. (B) The same lesion but from a coronal view. Note that in the arterial phase, this particular lesion is mainly solid. A characteristic central calcification is clearly visible in both views. (C) A coronal CT image of an SPN that is mainly cystic. Note again the calcifications (white arrow).

Figure 2

Gross and histopathologic appearance of solid-pseudopapillary neoplasm (SPN). (A) Gross appearance of an SPN. Note the well-delineated mass with spaces (cysts) containing friable pale tan to yellow material. Hemorrhagic areas can be seen (arrow). (B) On microscopic examination, pseudopapillae are formed by loosely cohesive, monotonous short columnar cells surrounding delicate blood vessels. (C) Histologic patterns are variable. This particular example of an SPN exhibits islands of foamy macrophages (arrow), scattered cholesterol clefts with intracytoplasmic periodic-acid Schiff-diastase resistant hyaline globules. (D) Immunolabeling of the neoplasm for β-catenin exhibits both nuclear and cytoplasmic staining pattern (white arrow). Adjacent normal pancreas exhibits membranous pattern only (black arrow).

Figure 3

Solid-pseudopapillary neoplasm metastatic to a lymph node. (A) This lymph node contains metastatic solid-pseudopapillary tumor, leaving only a small contingent of lymphocytes (hematoxylin and eosin). (B) Resident lymphocytes stain pale blue with the counterstain (lower part of field); neoplastic cells exhibit nuclear and cytoplasmic labeling with an antibody for β-catenin.