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Metastatic Patterns of Duodenopancreatic Neuroendocrine Tumors in Patients With Multiple Endocrine Neoplasia Type 1.
Hackeng, Wenzel M; van Beek, Dirk-Jan; Kok, Aranxa S M; van Emst, Madelon; Morsink, Folkert H M; van Treijen, Mark J C; Borel Rinkes, Inne H M; Dreijerink, Koen M A; Offerhaus, G Johan A; Valk, Gerlof D; Vriens, Menno R; Brosens, Lodewijk A A.
Afiliação
  • Hackeng WM; Department of Pathology, University Medical Center Utrecht, Utrecht University.
  • van Beek DJ; Department of Endocrine Surgical Oncology, University Medical Center Utrecht.
  • Kok ASM; Department of Pathology, University Medical Center Utrecht, Utrecht University.
  • van Emst M; Department of Pathology, University Medical Center Utrecht, Utrecht University.
  • Morsink FHM; Department of Pathology, University Medical Center Utrecht, Utrecht University.
  • van Treijen MJC; Department of Endocrine Oncology, University Medical Center Utrecht Cancer Center, Utrecht.
  • Borel Rinkes IHM; Department of Endocrine Surgical Oncology, University Medical Center Utrecht.
  • Dreijerink KMA; Department of Endocrinology and Internal Medicine, Amsterdam University Medical Centers, Amsterdam, The Netherlands.
  • Offerhaus GJA; Department of Pathology, University Medical Center Utrecht, Utrecht University.
  • Valk GD; Department of Endocrine Oncology, University Medical Center Utrecht Cancer Center, Utrecht.
  • Vriens MR; Department of Endocrine Surgical Oncology, University Medical Center Utrecht.
  • Brosens LAA; Department of Pathology, University Medical Center Utrecht, Utrecht University.
Am J Surg Pathol ; 46(2): 159-168, 2022 02 01.
Article em En | MEDLINE | ID: mdl-34560682
ABSTRACT
Patients with multiple endocrine neoplasia 1 syndrome (MEN1) often develop multifocal duodenopancreatic neuroendocrine tumors (dpNETs). Nonfunctional pancreatic neuroendocrine tumors (PanNETs) and duodenal gastrinomas are the most frequent origins of metastasis. Current guidelines recommend surgery based on tumor functionality, size ≥2 cm, grade or presence of lymph node metastases. However, in case of multiple primary tumors it is often unknown which specific tumor metastasized. This study aims to unravel the relationship between primary dpNETs and metastases in patients with MEN1 by studying endocrine differentiation. First, it was shown that expression of the endocrine differentiation markers ARX and PDX1 was concordant in 18 unifocal sporadic neuroendocrine tumors (NETs) and matched metastases. Thereafter, ARX, PDX1, Ki67 and gastrin expression, and the presence of alternative lengthening of telomeres were determined in 137 microscopic and macroscopic dpNETs and 36 matched metastases in 10 patients with MEN1. ARX and PDX1 H-score clustering was performed to infer relatedness. For patients with multiple metastases, similar intrametastases transcription factor expression suggests that most metastases (29/32) originated from a single NET of origin, while few patients may have multiple metastatic primary NETs. In 6 patients with MEN1 and hypergastrinemia, periduodenopancreatic lymph node metastases expressed gastrin, and clustered with minute duodenal gastrinomas, not with larger PanNETs. PanNET metastases often clustered with high grade or alternative lengthening of telomeres-positive primary tumors. In conclusion, for patients with MEN1-related hypergastrinemia and PanNETs, a duodenal origin of periduodenopancreatic lymph node metastases should be considered, even when current conventional and functional imaging studies do not reveal duodenal tumors preoperatively.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Pancreáticas / Carcinoma Neuroendócrino / Neoplasia Endócrina Múltipla Tipo 1 / Neoplasias Duodenais Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Am J Surg Pathol Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Neoplasias Pancreáticas / Carcinoma Neuroendócrino / Neoplasia Endócrina Múltipla Tipo 1 / Neoplasias Duodenais Limite: Adult / Aged / Female / Humans / Male / Middle aged Idioma: En Revista: Am J Surg Pathol Ano de publicação: 2022 Tipo de documento: Article