Surgical Management of Solid-Pseudopapillary Neoplasms of the Pancreas (Franz or Hamoudi Tumors): A Large Single-Institutional Series - 23/08/11

Doi : 10.1016/j.jamcollsurg.2009.01.044

Sushanth Reddy, MD ^a,^b, John L. Cameron, MD, FACS ^a,^b, Jennifer Scudiere, MD ^b,^c, Ralph H. Hruban, MD ^b,^c, Elliot K. Fishman, MD ^b,^d, Nita Ahuja, MD, FACS ^a,^b,^e, Timothy M. Pawlik, MD, FACS ^a,^b, Barish H. Edil, MD, FACS ^a,^b,^e, Richard D. Schulick, MD, FACS ^a,^b,^e, Christopher L. Wolfgang, MD, PhD, FACS ^a,^b,^⁎
^a Department of Surgery, Johns Hopkins Medical Institutions, Baltimore, MD
^b Sol Goldman Pancreatic Cancer Research Center, Johns Hopkins Medical Institutions, Baltimore, MD
^c Department of Pathology, Johns Hopkins Medical Institutions, Baltimore, MD
^d Russell H Morgan Department of Radiology, Johns Hopkins Medical Institutions, Baltimore, MD
^e Department of Oncology, the Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins Medical Institutions, Baltimore, MD

Correspondence address: Christopher L Wolfgang, MD, PhD, John L Cameron Division of Surgical Oncology, the Sol Goldman Pancreatic Cancer Research Center, Department of Surgery, Johns Hopkins Medical Institutions, 606 Blalock Bldg, 600 N Wolfe St, Baltimore, MD 21287

Résumé

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.

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Plan

Methods

Results

Demographic characteristics

Presentation and workup

Operative and postoperative courses

Pathology

Survival and followup

Discussion

Author Contributions

	Disclosure Information: Nothing to disclose.
	Supported in part by the NIH SPORE (Specialized Programs of Research Excellence) in Gastrointestinal Cancer Grant CA62924.