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A Consensus-Developed Morphological Re-Evaluation of 196 High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms and Its Clinical Correlations

Elvebakken, Hege (author)
Norwegian Univ Sci & Technol, Fac Med & Hlth Sci, Dept Clin & Mol Med, Trondheim, Norway.;More & Romsdal Hosp Trust, Alesund Hosp, Dept Oncol, Alesund, Norway.
Perren, Aurel (author)
Univ Bern, Inst Pathol, Bern, Switzerland.
Scoazec, Jean-Yves (author)
Inst Gustave Roussy, Dept Biopathol, Villejuif, France.
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Tang, Laura H. (author)
Mem Sloan Kettering Canc Ctr, Dept Pathol, 1275 York Ave, New York, NY 10021 USA.
Federspiel, Birgitte (author)
Copenhagen Univ Hosp, Dept Pathol, Rigshosp, Copenhagen, Denmark.
Klimstra, David S. (author)
Mem Sloan Kettering Canc Ctr, Dept Pathol, 1275 York Ave, New York, NY 10021 USA.
Vestermark, Lene W. (author)
Odense Univ Hosp, Dept Oncol, Odense, Denmark.
Ali, Abir, 1986- (author)
Uppsala universitet,Onkologisk endokrinologi
Zlobec, Inti (author)
Myklebust, Tor A. (author)
More & Romsdal Hosp Trust, Dept Res & Innovat, Alesund, Norway.;Canc Registry Norway, Dept Registrat, Oslo, Norway.
Hjortland, Geir O. (author)
Oslo Univ Hosp, Dept Oncol, Oslo, Norway.
Langer, Seppo W. (author)
Copenhagen Univ Hosp, Dept Oncol, Rigshosp, Copenhagen, Denmark.
Gronbaek, Henning (author)
Aarhus Univ Hosp, Dept Hepatol & Gastroenterol, Aarhus, Denmark.;ENETS Ctr Excellence, Aarhus, Denmark.
Knigge, Ulrich (author)
Univ Copenhagen, Fac Hlth Sci, Dept Surg & Endocrinol PE C, Rigshosp, Copenhagen, Denmark.
Tiensuu Janson, Eva (author)
Uppsala universitet,Onkologisk endokrinologi
Sorbye, Halfdan (author)
Haukeland Hosp, Dept Oncol, Bergen, Norway.;Univ Bergen, Dept Clin Sci, Bergen, Norway.
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Norwegian Univ Sci & Technol, Fac Med & Hlth Sci, Dept Clin & Mol Med, Trondheim, Norway;More & Romsdal Hosp Trust, Alesund Hosp, Dept Oncol, Alesund, Norway. Univ Bern, Inst Pathol, Bern, Switzerland. (creator_code:org_t)
2020-10-01
2021
English.
In: Neuroendocrinology. - : S. Karger. - 0028-3835 .- 1423-0194. ; 111:9, s. 883-894
  • Journal article (peer-reviewed)
Abstract Subject headings
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  • High-grade gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are classified according to morphology as well-differentiated neuroendocrine tumours (NETs) G3 or poorly differentiated neuroendocrine carcinomas (NECs). Little data exist concerning which morphological criteria this subdivision should be based on. Uncertainty exists if the NEC group should be further subdivided according to proliferation rate. Clinical data on NET G3 and NEC with a lower Ki-67 range are limited. A total of 213 patients with high-grade GEP-NEN (Ki-67 >20%) were included from the Nordic NEC Registries. Four experienced NET pathologists re-evaluated the cases to develop the best morphological criteria to separate NET G3 from NEC, assuming longer survival in NET G3. Organoid growth pattern, capillary network in direct contact to tumour cells, and absence of desmoplastic stroma were found to best separate NET G3 from NEC. Of 196 patients with metastatic disease, NET G3 was found in 12.3%, NEC with a Ki-67 <55% (NEC < 55) in 29.6%, and NEC with a Ki-67 >= 55% (NEC >= 55) in 56.6%. Only in 1.5%, the morphology was ambiguous. Of 164 patients receiving first-line chemotherapy, 88% received platinum/etoposide treatment. Response rate was higher for NEC >= 55 (44%) than that of NEC < 55 (25%) and NET G3 (24%) (p = 0.025 and p = 0.026). Median progression-free survival was 5 months for all groups. Median overall survival was 33 months for NET G3 compared to 11 months for both NEC < 55 and NEC >= 55 (p = 0.004 and 0.003). Specific morphological criteria can separate NET G3 from NECs and show prognostic significance. High-grade GEP-NEN patients stratified by morphology and proliferation rate demonstrate significant differences in response to chemotherapy and survival.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Endokrinologi och diabetes (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Endocrinology and Diabetes (hsv//eng)
MEDICIN OCH HÄLSOVETENSKAP  -- Klinisk medicin -- Kirurgi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Clinical Medicine -- Surgery (hsv//eng)

Keyword

Neuroendocrine carcinomas
Gastroenteropancreatic neuroendocrine neoplasms
Neuroendocrine tumour grade 3
Ki-67
Morphology

Publication and Content Type

ref (subject category)
art (subject category)

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