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.
Schlüsselwörter
Uterus - Leiomyomatosis - peritoneale Tumoren - benigne uterine Erkrankungen - Myome
Key words
leiomyoma - leiomyomatosis - peritoneal neoplasm - benign uterine diesease - fibroid
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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