Pulmonary Metastases of a Uterine Smooth Muscle Tumour with Undefined Malignancy Potential.

Abstract:

:Smooth muscle neoplasms with atypical proliferative behaviour, but without clear histopathological malignancy represent a diagnostic and therapeutic challenge, as distinction from a sarcoma can be difficult and no guaranteed treatment recommendations are available due to the rarity of these changes. In the event of uncertain primary histology, even metastases cannot be assessed as malignancy criteria, but may contribute to the clarification of the histology. Similarities with other smooth muscle proliferations, such as lymphangioleiomyomatosis, are striking. The diagnostic difficulties and treatment options are explained based on the example of a 59-year-old patient, in whom a retroperitoneal mass and pulmonary lesion of such a tumour occurred 4 years after a hysterectomy. Even though the genesis and histological diagnostics have not been conclusively clarified, slow growth and a low recurrence rate for post-menopausal patients allow for a wait-and-see approach, whereby the option for anti-hormonal treatment exists in the event of positive evidence of hormone receptors. ZUSAMMENFASSUNG:Glattmuskuläre Neoplasien mit zwar atypischem Wachstumsverhalten, aber ohne eindeutige histopathologische Malignitätszeichen stellen eine diagnostische und therapeutische Herausforderung dar, da die Abgrenzung zu einem Sarkom schwierig sein kann und es aufgrund der Seltenheit dieser Veränderungen keine gesicherten Therapieempfehlungen gibt. Bei unsicherer Primärhistologie können selbst Metastasen nicht als Malignitätskriterium gewertet werden, aber zur Klärung der Histologie beitragen. Auffällig sind Ähnlichkeiten zu anderen glattmuskulären Proliferationen, wie der Lymphangioleiomyomatose. Anhand des Beispiels einer 59-jährigen Patientin, bei der 4 Jahre nach Hysterektomie eine retroperitoneale Raumforderung sowie pulmonale Herde eines solchen Tumors auftraten, werden die Schwierigkeiten der Diagnostik sowie die therapeutischen Optionen erläutert. Obwohl Genese und histologische Diagnostik nicht abschließend geklärt sind, erlauben ein langsames Wachstum sowie eine niedrige Rezidivrate bei postmenopausalen Patientinnen ein abwartendes Verhalten, wobei bei positivem Nachweis von Hormonrezeptoren die Möglichkeit einer antihormonellen Therapie besteht.

authors

Esch M,Teschner M,Braesen JH

doi

10.1055/s-0034-1368182

subject

Has Abstract

pub_date

2014-03-01 00:00:00

pages

288-292

issue

3

eissn

0016-5751

issn

1438-8804

journal_volume

74

pub_type

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