Das PECom des Uterus - ein seltener mesenchymaler Tumor: Fallbericht und Literaturübersicht

Perivascular Epithelioid Cell Neoplasm (Pecoma) of the Uterus: A Case Report and Review of the Literature

“…Tumor size ≥ 5cm Infiltrative growth pattern Mitotic rate > 1/50 high power fields (HPF) High nuclear grade cellularity Necrosis Vascular invasion Three categories: benign: no criteria for malignancy uncertain malignant potential: one criterium for malignancy malignant: ≥ 2 criteria for malignancy Staging [20,21] in analogy with uterine (leiomyo-) sarcomas with a thoracic and an abdominal CT scan Therapy [26,28,29] -no unanimous consensus -surgical excision as mainstay of primary treatment -in PEComas with aggressive behavior or in metastatic disease:  Possible adjuvant or palliative chemotherapy regimens: Adriamycin, high-dose Ifosfamide, Trabectedin, Docetaxel/Gemcitabine, dacarbazine-based regimens  radiation therapy  targeted immunotherapies with mTOR inhibitors (e.g. Sirolismus, Temsirolismus or Everolismus) Prognosis [32,33] Relapse: 20% Lethality: 10% The spectrum of possible differential diagnoses for uterine PE-Comas include endometrial stromal sarcomas, epithelioid smooth muscle tumors and paragangliomas. PEComas can display several histomorphological characteristics and morphological diversities.…”