Ross et al. Journal of Medical Case Reports 2011, 5:383 http://www.jmedicalcasereports.com/content/5/1/383 C A S E R E P O R T JOURNAL OF MEDICAL CASE REPORTS Open Access A patient presenting with a perivascular epithelioid cell tumor in the broad ligament: a case report Claire Ross1*, Sunita Sharma2, Onsy Louca3, Michelle Scurr4, Andrew Hayes4 and Ian Judson4 Abstract Introduction: Perivascular epithelioid cell tumors are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They can originate in any visceral organ or soft tissue and include a range of lesions such as angiomyolipoma, clear cell ‘sugar’ tumor of the lung, lymphangioleiomyomatosis and clear cell myomelanocytic tumors of the falciparum ligament/ligament teres. Due to their rarity and varied sites and presentation, management of these tumors remains highly challenging. Case Presentation: A 46-year-old para 2 Caucasian woman initially presented to the general surgeons at our hospital in North West London with abdominal pain. Laparoscopy revealed a right broad ligament hematoma, which was thought to be iatrogenic in origin, from insertion of the Veress needle at the time of surgery, and was managed conservatively. Upon her re-presentation two months later with severe pain, ultrasound scanning revealed the hematoma had increased in size and she underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histology results from necrotic tissue from the hematoma led to a diagnosis of perivascular epithelioid cell tumor. She was then referred to a tertiary oncology center, where she underwent several further operations in an attempt to debulk the tumor for symptomatic relief of her pain, with limited success. She is now taking the immunosuppressive drug sirolimus, which has produced a modest reduction in tumor size. She is now 47 months on from initial presentation. Conclusions: A literature search has revealed only six other case reports of broad ligament perivascular epithelioid cell tumors, with varied presentations and management. The longest duration of follow-up was 21 months. Only five other cases of perivascular epithelioid cell tumor managed with sirolimus have been reported. We therefore feel that this report highlights some of the difficulties in diagnosing perivascular epithelioid cell tumors, and sheds light on management strategies for a very rare gynecological tumor in addition to sharing our experience in the use of sirolimus in its treatment. Introduction Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal tumors composed of histo- logically and immunohistochemically distinctive perivas- cular epithelioid cells [1]. They can originate in any visceral organ or soft tissue and have been reported in the lung, pancreas, liver, kidney, bone, uterus, vulva, heart, breast, common bile duct, bladder, skull base, skin and soft tissue of thigh and abdominal wall, and * Correspondence: claire.ross@doctors.org.uk 1St Mary’s Hospital, Imperial Healthcare NHS Trust, Praed Street, London W2 1UL, UK Full list of author information is available at the end of the article include a range of lesions such as angiomyolipoma, clear cell ‘sugar’ tumor of the lung, lymphangioleiomyomato- sis and clear cell myomelanocytic tumors of the falci- parum ligament/ligament teres. The majority of PEComas arise in women, and have been reported in females ranging from 3 to 97 years of age. We report the case of a patient with a broad ligament PEComa whose history highlights some of the difficulties associated with diagnosing and managing these rare tumors. Case presentation A 46-year-old, para 2 Caucasian woman presented to the general surgeons at our hospital with a four-day © 2011 Ross et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.