Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1.

Selberherr, Andreas; Koperek, Oskar; Riss, Philipp; Scheuba, Christian; Niederle, Martin B; Kaderli, Reto; Perren, Aurel; Niederle, Bruno (2019). Intertumor heterogeneity in 60 pancreatic neuroendocrine tumors associated with multiple endocrine neoplasia type 1. Orphanet journal of rare diseases, 14(1), p. 54. BioMed Central 10.1186/s13023-019-1034-4

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BACKGROUND

Patients with multiple endocrine neoplasia type 1 (MEN-1) develop multiple pancreatic neuroendocrine neoplasias (PNENs). Size at diagnosis and growth during follow-up are crucial parameters. According to the WHO 2017, grading is another important parameter. The impact of grading compared to size (WHO 2000) on the clinical course needs to be evaluated.

METHODS

Sixty PNENs of six patients with MEN-1 were retrospectively evaluated.

RESULTS

Fifty-one tumors with a diameter of < 20 mm were graded as G1. Two of 9 tumors with diameters of ≥20 mm were graded as G2. Tumor size of ≥20 mm correlated significantly with higher proliferation (p = 0.000617). Lymph node metastases were documented in two patients with a total of 19 tumors. In one patient, all 13 tumors (diameter: 0.4 to 100 mm) were classified as G1. However, metastases were documented in 9/29 lymph nodes. In the other patient, 5 tumors (3.5 to 20 mm) were classified as G1. The sixth tumor (30 mm) was classified as G2 (Ki-67: 8%). Metastases were revealed in 2/20 lymph nodes.

CONCLUSIONS

Tumor size of ≥20 mm seems to correlate with more aggressive MEN-1 related pancreatic disease, regardless of individual proliferation. Tumors ≥20 mm and tumors graded as G2 should be treated surgically regardless of their size.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Visceral Surgery
04 Faculty of Medicine > Service Sector > Institute of Pathology

UniBE Contributor:

Kaderli, Reto Martin and Perren, Aurel

Subjects:

500 Science > 570 Life sciences; biology
600 Technology > 610 Medicine & health

ISSN:

1750-1172

Publisher:

BioMed Central

Language:

English

Submitter:

Aurel Perren

Date Deposited:

13 Nov 2019 17:02

Last Modified:

28 Nov 2020 02:30

Publisher DOI:

10.1186/s13023-019-1034-4

PubMed ID:

30795813

Uncontrolled Keywords:

Intertumor heterogeneity MEN-1 Multiple endocrine neoplasia NET Pancreatic neuroendocrine tumors

BORIS DOI:

10.7892/boris.134737

URI:

https://boris.unibe.ch/id/eprint/134737

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