Roma Andres Anibal, Malpica Anais
Department of Pathology, The University of Texas M. D., Anderson Cancer Center, Houston, TX 77030, USA.
Am J Surg Pathol. 2009 Apr;33(4):526-33. doi: 10.1097/PAS.0b013e3181909018.
Primary retroperitoneal mucinous tumors (PRMTs) are uncommon neoplasms occurring almost exclusively in women. PRMTs are divided into mucinous cystadenoma (MC), mucinous borderline tumors or tumors of low malignant potential (MLMP), and mucinous carcinomas (MCas). In this retrospective study, we present the clinicopathologic features of 18 such cases, the largest series to date. All patients were women, ranging in age from 20 to 63 years (mean 38.6 y). All except 2 patients presented with an enlarged mass during a routine examination or by self-palpation. All tumors were located exclusively in the retroperitoneum, with histologic or clinical confirmation of the lack of ovarian involvement. The tumors ranged from 7 to 26 cm (mean 13.2 cm). The gross appearance was variable: unilocular cyst with a thin wall (4 cases), predominantly cystic with papillary areas or nodule(s) (8 cases), multiloculated cyst with or without nodules (1 case each), and predominantly solid with cystic areas (4 cases). Histologically, there were 2 cases of MC, 7 of MLMP (7 cases; 3 of them with intraepithelial carcinoma and 1 with microinvasion), and 9 of MCas (9 cases, 5 of them associated with MLMP and 1 associated with MC). Three of the MCas had areas of anaplastic or sarcomatoid carcinoma whereas 1 had an associated sarcoma. Immunohistochemical studies were performed in 6 cases. Cytokeratin 7 was diffusely positive in all cases studied, whereas cytokeratin 20 and cytokeratin 17 were focally positive in 4 and 2 cases, respectively. All patients underwent surgical resection of the entire tumor. Two patients with MCa and sarcoma or sarcomatoid carcinoma received chemotherapy. Follow-up was available in 16 cases, ranging from 1 to 148 months (mean 40 mo, median 22 mo). Two patients died of disease at 5 and 9 months; both had MCa with anaplastic carcinoma or sarcoma. Three patients with MCa were alive with disease at 14, 26, and 58 months. The remaining patients were alive with no evidence of disease. In this study, MLMP and MCa were more common than MC. PRMTs seem to be nonaggressive neoplasms, except in cases containing anaplastic carcinoma or sarcoma.
原发性腹膜后黏液性肿瘤（PRMTs）是一种罕见的肿瘤，几乎仅发生于女性。PRMTs分为黏液性囊腺瘤（MC）、黏液性交界性肿瘤或低恶性潜能肿瘤（MLMP）以及黏液性癌（MCas）。在这项回顾性研究中，我们呈现了18例此类病例的临床病理特征，这是迄今为止最大的病例系列。所有患者均为女性，年龄在20至63岁之间（平均38.6岁）。除2例患者外，所有患者均在常规检查或自我触诊时发现肿块增大。所有肿瘤均仅位于腹膜后，经组织学或临床证实无卵巢受累。肿瘤大小从7至26厘米不等（平均13.2厘米）。大体外观各异：薄壁单房囊肿（4例）、以囊性为主伴乳头区域或结节（8例）、多房囊肿伴或不伴结节（各1例）以及以实性为主伴囊性区域（4例）。组织学上，有2例MC，7例MLMP（7例；其中3例伴上皮内癌，1例伴微浸润），9例MCas（9例，其中5例与MLMP相关，1例与MC相关）。3例MCas有间变性或肉瘤样癌区域，1例伴有肉瘤。对6例进行了免疫组化研究。细胞角蛋白7在所有研究病例中均弥漫性阳性，而细胞角蛋白20和细胞角蛋白17分别在4例和2例中局灶性阳性。所有患者均接受了肿瘤全切手术。2例患有MCa且伴有肉瘤或肉瘤样癌的患者接受了化疗。16例患者获得随访，时间从1至148个月不等（平均40个月，中位数22个月）。2例患者分别在5个月和9个月死于疾病；二者均为伴有间变性癌或肉瘤的MCa。3例患有MCa的患者在14、26和58个月时仍有疾病存活。其余患者无疾病存活。在本研究中，MLMP和MCa比MC更常见。PRMTs似乎是侵袭性不强的肿瘤，含有间变性癌或肉瘤的病例除外。
