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Grading neuroendokriner Tumoren

Grading of neuroendocrine tumors

  • Schwerpunkt: Grading-Systeme
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Zusammenfassung

Die aktuelle WHO-Klassifikation der neuroendokrinen Tumoren (NET) der Lunge unterscheidet gemäß ihrer Prognose zwischen typischen Karzinoiden (Low-grade-NET), atypischen Karzinoiden (Intermediate-NET), klein- und großzelligen NET bzw. Karzinomen als High-grade-NET. Den neuroendokrinen Neoplasien (NEN) des Gastrointestinaltrakts und des Pankreas wurde ein einheitliches Gradingsystem, das ENETS-/WHO-Grading, zugeschrieben. Es ermöglicht zusammen mit dem TNM-System präoperativ, an Biopsien oder Feinnadelpunktionen (FNP) eine Risikoabschätzung vorzunehmen. Die gut differenzierten Tumoren werden nach ihrer Mitosezahl und dem Ki-67-Index in G1- (<2 Mitosen/10 HPF [„high power field“], Ki-67 < 3 %) und G2- (2–20 Mitosen/10 HPF, Ki-67 3–20 %) Tumoren eingeteilt. Bei den nicht seltenen Diskrepanzen zwischen Mitosezahl und Ki-67-Index sollte der jeweils höhere Wert berücksichtigt werden. Von den neuroendokrinen Karzinomen des G3-Typs sind undifferenzierte Karzinome des klein- wie des großzelligen Typs mit noch schlechterer Prognose zu unterscheiden. Ein Grading der Schilddrüsenkarzinome mit Prognoserelevanz ergibt sich aus der speziellen Subtypisierung, sodass ein G1- bis G3-System entfällt. Für die sehr seltenen Nebenschilddrüsen- und Hypophysenkarzinome ist sinnvollerweise kein Grading etabliert. Die Nebennierentumoren besitzen ebenfalls kein Grading. Die Prognose richtet sich dort nach dem Ki-67-Index und in sehr eingeschränktem Maße nach den etablierten Scoringsystemen.

Abstract

The current WHO classification of neuroendocrine tumors (NET) differentiates between typical carcinoids (low grade NET), atypical carcinoids (intermediate grade NET) and small cell and large cell carcinomas (high grade NET) according to the prognosis. Neuroendocrine neoplasms (NEN) of the gastrointestinal tract and the pancreas are graded in an identical way. Together with the TNM system this enables a preoperative estimation of the prognosis in biopsies and fine needle aspirates. Well-differentiated tumors are graded into G1 tumors by the number of mitoses, <2 per 10 high-power fields (HPF) and the Ki-67 (index <3 %) and G2 tumors (2–20 mitoses/10 HPF, Ki-67 3–20 %). Discrepancies between the number of mitoses and the Ki-67 index are not uncommon and in these cases the higher value of the two should be applied. The more differentiated tumors of the G3 type have to be differentiated from undifferentiated carcinomas of the small cell type and large cell type with a much poorer prognosis. Prognosis relevant grading of thyroid cancers is achieved by special subtyping so that the G1-G3 system is not applicable. The rare cancers of the parathyroid gland and of the pituitary gland are not graded. Adrenal tumors also have no grading system. The prognosis is dependent on the Ki-67 index and with some reservations on the established scoring systems.

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Danksagung

Die Autoren danken allen Pathologen, die ihren Konsiliardienst nutzen. Daraus ergaben sich anregende Gespräche, neue Ideen und interessante Kasuistiken.

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Correspondence to W. Saeger.

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W. Saeger, P. A. Schnabel und P. Komminoth geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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C. Wittekind, Leipzig

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Saeger, W., Schnabel, P.A. & Komminoth, P. Grading neuroendokriner Tumoren. Pathologe 37, 304–313 (2016). https://doi.org/10.1007/s00292-016-0186-4

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