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Clinico-Pathological Features and Survival of Resected Pancreatic Neuroendocrine Tumors Associated With Von Hippel Lindau Disease and Multiple Endocrine Neoplasia Type 1
Ilaria Pergolini*1, Othon Iliopoulos3, Cristina R. Ferrone1, Vikram Deshpande2, Keith D. Lillemoe1, Carlos Fernandez-del Castillo1
1Surgery, Massachusetts General Hospital, Boston, MA; 2Pathology, Massachusetts General Hospital, Boston, MA; 3Oncology, Massachusetts General Hospital, Boston, MA
Patients with von Hippel Lindau disease (VHL) and multiple endocrine neoplasia 1 (MEN1) are at a risk of developing pancreatic neuroendocrine tumors (PNETs), which may behave in a malignant fashion. We retrospectively evaluated a 15-year experience with resected PNETs associated with these genetic syndromes.
Between January 2001 and November 2015, 334 patients underwent resection for a PNET at a single institution. 10 of these had VHL (3%) and 24 MEN1 (7%). 1 patient in each group was found to have distant metastases at the time of diagnosis. Age at diagnosis of PNET in patients with VHL was 17 years younger than in MEN1 (30.5 vs 47.5 years, p=0.1), although the age at the diagnosis of the inherited syndrome was similar (27 vs 27.5 years). Median time of observation between diagnosis and surgical resection was 14 months (2-153) in VHL cases and 1.5 months (0-96) in MEN1 patients (p=0.002). PNETs led to the diagnosis of the genetic syndrome in only one (10%) patient with VHL and in 9 (37.5%) patients with MEN1; 7 of these were functioning tumors. Family history was positive in 90% of VHL patients and in 71% of those with MEN1; the median size of the largest tumor was 2.4 cm (1.8-4.2) and 2.8 cm (0.7-9.0), respectively (p=0.353). PNETs were frequently multifocal, particularly in MEN1 (54% vs 30%, p=0.270). In VHL, PNET was more frequently associated with multifocal cystic lesions (60% vs 4%, p=0,001). Distal pancreatectomy was the most frequent procedure (50% in VHL and 59% in MEN1). Invasive lesions represented almost 60% of cases in both groups. Lymph nodes were positive in 30% and 25% of patients, respectively. There were no post-operative deaths, but morbidity occurred in 5 patients with VHL (50%) and in 21% of MEN1 patients. Median follow-up was 25 months (1-126) for VHL patients and 73.5 months (2-169) in the MEN1 group (p=0.259); during follow-up, 1 (10%) and 6 (25%) patients, respectively, experienced recurrence, and 1 patient in each group died of the disease. No correlation between size of the tumor and recurrence was found. Median disease free survival was 25 (1-126) and 56.5 (2-146) months, respectively.
Conclusions. 10% of resected PNETs are associated with either VHL or MEN1. Both require careful management due to their malignant potential, young age of diagnosis, frequent multifocal presentation and risk of recurrence.
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