It is known that pNETs can occur in the setting of several genetic syndromes. These include MEN-1, tuberous sclerosis, neurofibromatosis, and von Hippel-Lindau (vHL) disease. MEN-1 is discussed in greater detail elsewhere in this book. Here, the discussion is limited to special considerations involved in the surgical management of MEN-1-related pNETs. In addition, the aspects of tuberous sclerosis, neurofibromatosis, and vHL disease as they relate to pNETs will be discussed. As in all genetic cancer syndromes, genetic counseling and cancer screening are recommended.

Only recently have surgeons considered the oncologic aspects of pancreatic neoplasia in regard to MEN-1 patients who have nonfunctioning pNETs.125,126 Due to the characteristic multifocality of the pNETs in patients with MEN-1 and to the desire to avoid total pancreatectomy, some investigators have discouraged early surgery in patients with MEN-1.62 It has been suggested that surgery for nonfunctioning pNETs should be limited to those tumors larger than 2 to 3 cm in diameter.62,111,127 – 132 In a single institution series, a trend was shown for larger tumors to be associated with the presence of synchronous distant metastases at the time of diagnosis. In one study none of the 19 pNETs 2.5 cm or smaller in maximum dimension had distant metastases at the time of diagnosis compared with 5 (23%) of the 22 pNETs larger than 2.5 cm (p = .05).66 However, tumor ...