A clinical analysis on functioning pancreatic neuroendocrine tumors (focusing on VIPomas): a single-center experience

Murakami, Masatoshi; Fujimori, Nao; Matsumoto, K.; Ohno, Akihisa; Teramatsu, Katsuhito; Takamatsu, Yu; Takeno, Ayumu; Ueda, Keijiro; Oono, Takamasa; Ito, Tetsuhide; Ogawa, Yoshihiro

doi:10.1507/endocrj.ej22-0111

Cited by 7 publications

(6 citation statements)

References 19 publications

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“…In the present cohort there was a predominant tumor location in the left pancreas (75%), which is in line with previous case series of VIPomas, glucagonomas and CT-pNEN [7,14,22,32,33]. The median diameter in our cohort was 82 (range 12-220), and only one patient with a glucagonoma had a tumor size < 20mm.…”

Section: Discussionsupporting

confidence: 91%

“…As in other studies patients with rf-pNEN frequently show liver metastases at the time of initial diagnosis as in 50% of patients in our cohort. This is in the range of reported rates for VIPoma of 36-75% [32,33,37,38] and glucaogonoma of 50-78% [8, 22,36]. It is of note, in the present series, 2 of 5 CT-pNEN presented with liver metastases, whereas in a previous series only 4 of 23 (17%) tumors did so [14].…”

Section: Discussionsupporting

confidence: 67%

“…The study from Murakami et al showed, that the median survival time for patients with VIPoma was 71 months [33]. In the present study the three patients with VIPoma showed the 5 years tumor survival 33% and the median survival time 100 months.…”

Section: Discussionsupporting

confidence: 60%

“…In our series, patients with rf-pNEN had a median age of 61.5 (range 28-73 years), which is in the range reported in the literature [7,14,22,32,33]. In addition, no sex predominance was noted in either our series, nor in the literature.…”

Section: Discussionsupporting

confidence: 60%

“…In an older literature review of 407 patients with glucagonoma 80 (29%) patients had small tumors of 20mm or less and these patients had a rate of metastasis of 8,8% [7]. The median tumor size in the present cohort was larger than that previously reported for VIPoma with a median diameter of 32-57,5mm [33], glucagonoma with a median size from 50 to 55mm [22,36], and CT-pNEN with a median size of 48mm [14].…”

Section: Discussionmentioning

confidence: 46%

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Longterm outcome of rare functioning pancreatic neuroendocrine neoplasms

Sevcik,

Albers,

Wiese

et al. 2024

Preprint

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Introduction: Rare functional pancreatic neuroendocrine neoplasms (rf-pNENs), such as VIPomas, calcitoninomas and glucagonomas are extremely rare tumors. Thus, their characteristics and long-term prognosis have not yet been well defined. Methods:Clinicopathological characteristics, including preoperative diagnostics, surgical procedures, other treatments and long-term outcome of patients with rf-pNEN operated in the ENETS Center of Excellence Marburg were retrospectively analyzed. Results: Between 2002 and 2022, 12 of 216 (5,5%) patients with pNEN had rf-pNEN, including three VIPomas, four glucagonomas and five calcitoninomas. Among these 5 were women and 7 were men with a median age of 60 (range 28-73) years at the time of the first surgery. The tumor was visualized by preoperative imaging in all 12 patients, and six patients had distant metastases at the time of diagnosis. The tumor was located in the pancreatic tail in 9 patients and the median tumor size was 82 (range 12-220) mm. Eleven patients underwent tumor resections (2 robotic, 9 conventional), and 9 of these 11 patients received R0 resections. After a median follow-up of 75 (range 1-247) months, six patients were alive, five of whom had no evidence of disease. All patients who remained disease-free had an initial R0 resection of the primary tumor and no initial liver involvement. Conclusion: rf-pNENs are a heterogeneous group of tumors with a good long-term prognosis, if detected early and initially radically resected. Long-term survival, however, can be also achieved in patients with metastasized tumors using multimodal treatment.

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