Del Papa Mauro, D'Amata Gabriele, Manzi Fulvio, Musmeci Luca, Crovaro Marco, Buonocore Carlo, Florio Gaetano, Giannetti Andrea
Department of General Surgery, Ospedale "Leopoldo Parodi Delfino", Colleferro, Italy.
Department of General Surgery, Ospedale "Leopoldo Parodi Delfino", Colleferro, Italy.
Int J Surg Case Rep. 2017;41:323-326. doi: 10.1016/j.ijscr.2017.11.003. Epub 2017 Nov 9.
Primary malignant mixed mesodermal tumor (MMMt, also called malignant mixed Mullerian tumor and designated in the WHO classification of female genital tract neoplasms as carcinosarcoma) is an infrequent tumor that develops usually in the uterus and more rarely in the ovary. Extragenital tumor, including primary peritoneal MMMt, is an extremely rare and aggressive neoplasm with only few case reported in the literature.
We report a case of a 70-year's old female who presented with nausea and abdominal discomfort for 6 months. Workup revealed an abdominal mass. Patient was treated with surgical removal in a general hospital.
Most peritoneal carcinosarcomas originate in the pelvic peritoneum, followed by decreasing frequency in the serosal surface of the colon, retroperitoneum, anterolateral abdominal peritoneum, and omentum. Surgical excision is the most effective treatment in carcinosarcomas. A complete cytoreduction, with resection of cancer to a status of no evidence of disease by the surgeon's unaided eye should be attempted.
Owing to the rarity of the disease, limited data regarding the management of peritoneal MMMT exists. Recommendations for the treatment of MMMT are based on individual cases only. In our case, the patient is alive with a follow-up of 15 months and she did not receive any cycle of chemotherapy.
原发性恶性混合中胚层肿瘤(MMMt,也称为恶性苗勒管混合瘤,在世界卫生组织女性生殖道肿瘤分类中被指定为癌肉瘤)是一种罕见的肿瘤,通常发生于子宫,很少发生于卵巢。包括原发性腹膜MMMt在内的生殖器外肿瘤是一种极其罕见且侵袭性强的肿瘤,文献中仅有少数病例报道。
我们报告一例70岁女性患者,出现恶心和腹部不适6个月。检查发现腹部有肿块。患者在一家综合医院接受了手术切除治疗。
大多数腹膜癌肉瘤起源于盆腔腹膜,其次是结肠浆膜面、腹膜后、腹前外侧腹膜和网膜,其发生频率依次降低。手术切除是癌肉瘤最有效的治疗方法。应尝试进行完全细胞减灭术,即外科医生肉眼观察将癌症切除至无疾病证据的状态。
由于该疾病罕见,关于腹膜MMMT治疗的资料有限。MMMT的治疗建议仅基于个别病例。在我们的病例中,患者存活,随访15个月,未接受任何化疗周期。