Leiomyomatosis peritonealis disseminata – 4 Fallberichte einer seltenen Erkrankung

 

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Zusammenfassung

Die Leiomyomatosis peritonealis disseminata (LPD) ist charakterisiert durch zahlreiche subperitoneale Leiomyome von wenigen Millimetern bis zu raumfordernden abdominalen Tumorknoten. Vorgestellt werden 4 unterschiedliche Verläufe der seltenen Erkrankung. Patientin 1 wurde initial bei malignem Aspekt ausgedehnt operiert, war anschließend unter endokriner Therapie lange stabil und wurde schließlich bei einem Rezidiv hysterektomiert. In den Leiomyomknoten wurden Endometrioseherde nachgewiesen. Bei Patientin 2 ergab eine laparoskopische PE von kleinsten Herden bei stärkster Dyspareunie und Miktionsbeschwerden überraschend eine LPD. Bei Patientin 3 wurde nach Myomenukleation von 880 g und Hysterektomie wegen Myomen eine mehrknollige LPD operiert, wonach ein Beckenwandrezidiv auftrat und sie mehrere endokrine Therapieversuche erhielt. Patientin 4 hatte zusätzlich zu einem Rezidiv einer LPD ein Leiomyom im linken Labium minus. In der Übersicht werden das Risiko maligner Verläufe, die Frage nach endokrinen Therapieoptionen und die Assoziation zur Endometriose diskutiert. Der Literaturüberblick beschreibt das Risiko einer Schwangerschaft bei Patientinnen mit LPD. Bei der histologischen Sicherung dieser seltenen Entität sollten Hormonrezeptoren mitbestimmt werden.

Abstract

Leiomyomatosis peritonealis disseminata (LPD) is a rare disease. LPD is associated with multiple subperitoneal leiomyomas throughout the entire peritoneal cavity. Patients usually become symptomatic due to the large size of the abdominal mass. We present four different cases of LPD. Patient 1 had a radical debulking operation because of presumed malignancy and recurring LPD, which remained stable for years under endocrine therapy. In Patient 2 with severe dyspareunia and lower abdominal pain, LPD was diagnosed in a small peritoneal lesion by laparoscopic biopsy. In Patient 3 LPD recurred several times after myomectomy and hysterectomy and was treated with GnRH analogues amongst other endocrine agents. Patient 4 had a simultaneous LPD in the peritoneal cavity and a leiomyoma of the labium minus. The risk of malignancy, endocrine therapeutic options and association with endometriosis are discussed, and the risk during pregnancy of LPD patients is reviewed. Part of the diagnosis of this entity should include the analysis of steroid receptors.

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Dr. Katharina Goppel

Klinikum rechts der Isar
Technische Universität München

Ismaninger Straße 22

81675 München

Email: katharina.goppel@lrz.tum.de

Email: endokrinologie@lrz.tum.de