European Journal of Radiology Extra 61 (2007) 15–18 Alveolar soft part sarcoma of the sphincter ani externus: Control of vaginal bleeding by recurrent embolisations Michael Grundbichler a , Andreas Leithner b,* , Horst Rupert Portugaller c , Markus Hochegger b , Reinhard Windhager b a Institute of Physiology, Paracelsus Private Medical University, Salzburg, Austria b Department of Orthopedic Surgery, Medical University Graz, Auenbruggerplatz 5-7, A-8036 Graz, Austria c Department of Radiology, Medical University Graz, Graz, Austria Received 3 July 2006; received in revised form 15 October 2006; accepted 16 October 2006 Abstract Alveolar soft part sarcoma is a rare malignancy mainly localized in the deep soft tissue of the extremities. This is the first report of an alveolar soft part sarcoma originating from the sphincter ani externus. A 27-year-old pregnant woman presented with a tumour located in the right ischiorectal fossa which had rapidly enlarged during pregnancy 6 years after first detection. Admitted to our hospital, she presented with a continuous strong vaginal bleeding, multiple pulmonal metastases and an inoperable local tumour mass. Palliative chemotherapy was unsuccessful, control of local bleeding was only possible with multiple embolisations. The patient died 43 months after initial diagnosis. Our intention is to point out the remarkable origin, a rapid tumour growth during pregnancy, and the possibility of controlling massive tumour bleeding by embolisation. © 2006 Elsevier Ireland Ltd. All rights reserved. Keywords: Alveolar soft part sarcoma; Pregnancy; Embolisation 1. Introduction Alveolar soft part sarcoma is an uncommon soft tissue tumour. It was first named as an own entity by Christopherson et al. [1] in 1952. It occurs at any age but is most com- mon between 15 and 35 years with a female predominance before the age of 30 [2]. It is a slow growing, highly vas- cularized, usually painless and therefore easily overlooked tumour [2–5]. It is malignant and there is no benign coun- terpart [5]. In adults the typical localisation is in the deep soft tissue of the extremities particularly in the anterior thigh [2,4,6–8]. Early metastasis mainly to the lung or brain is a characteristic feature [2,3,6,8]. To our knowledge this is the first report of an alveolar soft part sarcoma originating from the sphincter ani externus. * Corresponding author. Tel.: +43 316 385 81899; fax: +43 316 385 4806. E-mail address: andreas.leithner@meduni-graz.at (A. Leithner). 2. Case report In 1992, a 21-year-old woman first recognized a small paraanal knot which she did not care about. Six years later in November 1998, the knot had suddenly enlarged dur- ing pregnancy. The tumour located in the right ischiorectal fossa was diagnosed by chance by a local gynaecologist at an antepartum examination in the 23rd week of gravidity. Inzisional biopsy showed a malign highly vascularized mes- enchymal neoplasm. Preoperative sonography displayed up to 4 mm dilated vessels filled with tumour thrombi partic- ularly in the cranial part. A microscopically intralesional local excision (R1) was performed at a local centre. The tumour originated from the sphincter ani externus with a diameter of 6 cm. Histological and immunohistochemical findings proved an alveolar soft part sarcoma. After child- birth by caesarean section in April 1999 X-ray presented metastases to the lung. Subsequent a high dose chemother- apy using Epirubicin (Farmorubicin®, Pfizer Pharma Gmbh, 1571-4675/$ – see front matter © 2006 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2006.10.008