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Acute and repeated haemoperitoneum: a challenging case of lymphangioleiomyomatosis with uterine PEComa
  1. Anjeza Xholli1,
  2. Chiara Kratochwila2,
  3. Valerio Gaetano Vellone3 and
  4. Maria Giulia Schiaffino2
  1. 1DINOGMI, Obsterics and Gynecology Clinic, IRCCS Ospedale San Martino, Genova, Italy
  2. 2Obstetrics and Gynecology Clinic, IRCCS AOU San Martino, Genova, Italy
  3. 3Department of Surgical and Diagnostic Sciences, IRCCS Ospedale San Martino, Genova, Italy
  1. Correspondence to Dr Anjeza Xholli; anj160583{at}yahoo.it

Abstract

A 39-year-old woman presented in the emergency ward for abdominal pain and acute anemiation. Abdominal–thoracic CT scan showed haemoperitoneum, with a parauterine mass and a pathological pulmonary pattern suspicious for lymphangioleiomyomatosis (LAM), a systemic disease belonging to perivascular epithelioid cell tumours (PEComas). Gynaecological ultrasound showed a hypoechoic irregular solid mass of the uterine right wall. Ultrasonographic virtual organ computer-aided analysis showed the mass completely formed by arteriovenous vessels, and that allowed distinction from leiomyosarcoma. Repeated haemoperitoneum required uterine artery embolisation. Mass revascularisation occurred in the following 7 days. A laparotomic hysterectomy with removal of the uterus and right parametrium was performed in epidural analgesia. Histological features were consistent with the diagnosis of uterine PEComa of uncertain malignant features, in the presence of coexisting pulmonary LAM. In women with LAM, acute haemoperitoneum may indicate the presence of a uterine PEComa whose diagnosis can be challenging.

  • ultrasonography
  • cancer - see oncology
  • interventional radiology
  • lung function

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Footnotes

  • Contributors AX: conception of the work, data interpretation, drafting the article and critical revision; VGV, MGS and CK: data collection.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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