Ross Claire, Sharma Sunita, Louca Onsy, Scurr Michelle, Hayes Andrew, Judson Ian
St Mary's Hospital, Imperial Healthcare NHS Trust, Praed Street, London W2 1UL, UK.
J Med Case Rep. 2011 Aug 16;5:383. doi: 10.1186/1752-1947-5-383.
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.
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.
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.
血管周上皮样细胞瘤是一类罕见的间叶组织肿瘤，由组织学和免疫组化上独特的血管周上皮样细胞组成。它们可起源于任何内脏器官或软组织，包括一系列病变，如血管平滑肌脂肪瘤、肺透明细胞“糖”瘤、淋巴管平滑肌瘤病以及镰状韧带/圆韧带的透明细胞肌黑色素细胞瘤。由于其罕见性、多样的发病部位和临床表现，这些肿瘤的治疗仍然极具挑战性。
一名46岁、孕2产的白种女性最初因腹痛就诊于伦敦西北部我院的普通外科医生处。腹腔镜检查发现右侧阔韧带血肿，考虑为手术时Veress针穿刺所致的医源性血肿，遂进行保守治疗。两个月后她因剧痛再次就诊，超声检查显示血肿增大，随后她接受了全腹子宫切除术及双侧输卵管卵巢切除术。血肿坏死组织的组织学检查结果诊断为血管周上皮样细胞瘤。之后她被转诊至三级肿瘤中心，在那里她又接受了几次手术，试图切除肿瘤以缓解疼痛症状，但效果有限。她目前正在服用免疫抑制药物西罗莫司，肿瘤大小有一定程度的缩小。自首次就诊以来，她已随访47个月。
文献检索仅发现另外6例阔韧带血管周上皮样细胞瘤的病例报告，其临床表现和治疗方法各异。最长随访时间为21个月。仅报告了另外5例使用西罗莫司治疗的血管周上皮样细胞瘤病例。因此，我们认为本报告突出了血管周上皮样细胞瘤诊断中的一些困难，除了分享我们使用西罗莫司治疗这种非常罕见的妇科肿瘤的经验外，还阐明了其治疗策略。
