Well-Differentiated Papillary Mesothelioma of the Female Peritoneum: A Clinicopathologic Study of 26 Cases
Well-Differentiated Papillary Mesothelioma of the Female Peritoneum: A Clinicopathologic Study of 26 Cases
复制标题
DOI：
10.1097/pas.0b013e3182354a79
复制
发表时间：
2012-01-01
影响因子：
5.6
通讯作者：
Silva, Elvio G.
中科院分区：
文献类型：
--
作者：
Malpica, Anais;Sant'Ambrogio, Sara;Silva, Elvio G.
高分化乳头状间皮瘤（WDPM）是一种罕见的间皮瘤，发生在女性腹膜，年龄范围很广。虽然被认为是一种不确定恶性潜能的肿瘤，但关于其生物学行为的信息仍然有限。在本研究中，我们报告了我院20年间（1990年至2010年）26例女性腹膜WDPM的临床病理特征。我们回顾了所有病例的临床资料和病理资料。患者年龄从23岁到75岁不等（中位47岁，平均48.6岁）。我们所有的病例都没有石棉接触史。10例患者既往有手术史，6例有子宫内膜异位症病史。在24例患者中，WDPM是在手术中偶然发现的良性或恶性病变。仅有2例患者出现症状：1例急性腹部疼痛，另1例慢性盆腔疼痛。前者因其中1处WDPM病变出血而发展为小腹膜出血，而后者则有2厘米的WDPM累及远端输卵管。病变为单发或多发（各13例），大小从0.1 cm到2 cm不等。受累部位：未明确的腹膜或盆腔腹膜（10例）、大网膜（7例）、囊尾膜（6例）、结肠浆膜（4例）、小肠肠系膜（2例）、子宫浆膜（2例）、胃浆膜（1例）、大肠肠系膜（1例）、输卵管（1例）、卵巢（1例）、腹股沟疝（1例）。所有病例均切除病变。显微镜下，我们所有的病例都具有WDPM的典型特征（即，乳头状结构可能伴有腺/管状结构，细胞巢和单个细胞，淡色间皮细胞，缺失或罕见的有丝分裂象）。我们病例的最初诊断是可变的，包括WDPM、间皮瘤增生、恶性间皮瘤、腹膜低恶性潜能浆液性肿瘤、乳头状输卵管内肿大和慢性输卵管黄色肉芽肿。25例患者获得随访，随访时间为4 ~ 192个月（平均47.5个月，中位32个月）；在5至144个月的随访后，22例患者存活，无WDPM证据。其中一名患者在初次诊断后46.5个月出现WDPM复发。本例患者为浆液性囊腺纤维瘤行全腹子宫切除术和双侧输卵管卵巢切除术时偶然发现WDPM。复发也是在结肠腺癌的结肠切除术中偶然发现的。该患者在初次诊断后73个月和诊断复发后36个月存活，无其他复发。3名患者死于其他原因：4个月和12个月时死于胰腺癌，192个月时死于白血病。认识WDPM的组织学特征和适当的临床相关性可以正确诊断该实体。如有必要，免疫组织化学研究，如calretinin和角蛋白5/6，有助于识别这种肿瘤的间皮性质。虽然在这一系列病例中没有患者死于疾病，但基于可能的复发或样本不足的恶性间皮瘤的误诊，对这种诊断的患者进行随访是有必要的。
Well-differentiated papillary mesothelioma (WDPM) is an uncommon mesothelial tumor that occurs in the peritoneum of women over a wide age range. Although considered a tumor of uncertain malignant potential, information about its biological behavior is still limited. In this study, we present the clinicopathologic features of 26 cases of WDPM of the female peritoneum seen in our institution over a 20-year period (1990 to 2010). Clinical information and pathology material were reviewed in all cases. Patients ranged in age from 23 to 75 years (median, 47 y; mean, 48.6 y). There was no history of asbestos exposure in any of our cases. Ten patients had undergone surgery previously, and 6 had a history of endometriosis. In 24 patients, the WDPM was an incidental finding during surgery for a benign or malignant lesion. Only 2 patients presented with symptoms: 1 with an acute abdomen and the other with chronic pelvic pain. The former had developed a small hemoperitoneum because of bleeding of 1 of the lesions of WDPM, whereas the latter had a 2-cm WDPM involving the distal fallopian tube. The lesions were single or multiple (13 cases each) and ranged in size from 0.1 cm to 2 cm. The following sites were involved: abdominal or pelvic peritoneum not otherwise specified (10 cases), omentum (7 cases), cul-de-sac (6 cases), colonic serosa (4 cases), small bowel mesentery (2 cases), uterine serosa (2 cases), stomach serosa (1 case), large bowel mesentery (1 case), fallopian tube (1 case), ovary (1 case), and inguinal hernia (1 case). In all cases the lesions were excised. Microscopically, all of our cases had the typical features described for WDPM (ie, a papillary architecture that may be accompanied by glandular/tubular patterns, nests of cells and individual cells, bland mesothelial cells, absent or rare mitotic figures). The initial diagnosis in our cases was variable, including WDPM, mesothelial hyperplasia, malignant mesothelioma, serous tumor of low malignant potential of the peritoneum, papillary endosalpingiosis, and chronic xanthogranulomatous salpingiosis. Follow-up was obtained for 25 patients, and it ranged from 4 to 192 months (mean, 47.5 mo; median, 32 mo); 22 patients are alive with no evidence of WDPM after a follow-up that ranged from 5 to 144 months. One of these patients experienced recurrence of WDPM 46.5 months after initial diagnosis. In this patient, WDPM was an incidental finding during a total abdominal hysterectomy and bilateral salpingo-oophorectomy for serous cystadenofibroma. The recurrence was also an incidental finding during a colectomy for colonic adenocarcinoma. This patient is alive with no other recurrences 73 months after initial diagnosis and 36 months after diagnosis of the recurrence. Three patients died of other causes: pancreatic cancer at 4 months and 12 months and leukemia at 192 months. Recognition of the histologic features of WDPM and proper clinical correlation allow for the correct diagnosis of this entity. If necessary, immunohistochemical studies such as calretinin and keratin 5/6 facilitate the recognition of the mesothelial nature of this neoplasm. Although no patient died of disease in this series, follow-up of patients with this diagnosis is warranted on the basis of possible recurrences or misdiagnosis of an undersampled malignant mesothelioma.