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Hypophysitis

Formen und Differenzialdiagnose

Hypophysitis

Types and differential diagnosis

  • Schwerpunkt: Entzündungen endokriner Organe
  • Published:
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Zusammenfassung

Entzündliche Hypophysenbefunde machen etwa 1 % der Operationen der Sellaregion aus. Es sind dabei primäre Entzündungen (lymphozytäre Hypophysitis, idiopathische granulomatöse Hypophysitis) von sekundären Formen (Begleitentzündung bei Rathke-Zysten, Kraniopharyngeomen und Germinomen) und Beteiligungen der Hypophyse an generalisierten Entzündungen (IgG4-assoziiert, Sarkoidose, Septikopyämie) zu differenzieren und auch die Langerhans-Histiozytose als Differenzialdiagnose zu berücksichtigen. Die lymphozytäre Hypophysitis ist durch wechselnd dichte lymphozytäre Infiltrate vorwiegend des T‑Zell-Typs geprägt, die granulomatöse Hypophysitis ist von einer Sarkoidose nur durch Ausschluss einer Generalisation zu unterscheiden. Die sekundäre Hypophysitis zeigt ein gemischtzelliges, besonders auch histiozytäres Infiltrat und entsteht überwiegend aus Rupturen oder Blutungen von Rathke-Zysten. Das dann oft nur sehr spärliche restliche Zystenepithel sollte immunhistologisch (Pankeratin) gesichert werden.

Abstract

Inflammatory findings in the pituitary glands account for approximately 1 % of operations in the sellar region. Primary inflammation (e.g. lymphocytic and idiopathic granulomatous hypophysitis) have to be differentiated from secondary types (e.g. concomittant inflammation with Rathke’s cleft cysts, craniopharyngiomas and germinomas) and involvement of the pituitary in generalized inflammation (IgG4-related disease, sarcoidosis and septicopyemia). Langerhans cell histiocytosis also has to be considered for the differential diagnostics. Lymphocytic hypophysitis shows lymphocytic infiltrations of varying density, predominantly of the T‑cell type. Granulomatous hypophysitis has the features of sarcoidosis and can only be diagnosed by exclusion of generalized sarcoidosis. Secondary hypophysitis has a mixed cell infiltration, especially by histiocytic infiltration and predominantly originates from ruptures or bleeding from Rathke’s cleft cysts. The frequently very sparse remnants of cyst epithelium should be confirmed by pankeratin immunostaining.

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Danksagung

Meinen einsendenden Neurochirurgen und Neuropathologen verdanke ich das vielfältige Operationsmaterial und viele anregende Gespräche als Basis dieser Arbeit sowie gemeinsame Projekte. Mein besonderer Dank gilt den Sponsoren des Hypophysentumorregisters, der Novartis Pharma GmbH (Nürnberg), der Novo NordiskPharma GmbH (Mainz), der PfizerPharma GmbH (Berlin) und der Ipsen Pharma GmbH (Ettlingen), ohne die die Untersuchungen nicht finanzierbar gewesen wären.

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Saeger, W. Hypophysitis. Pathologe 37, 230–237 (2016). https://doi.org/10.1007/s00292-016-0164-x

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