子宫苗勒氏管腺肉瘤的诊断与治疗

目的：提高对子宫苗勒管腺肉瘤的诊断和治疗水平。方法：回顾性分析我院收治的9例子宫苗勒氏管腺肉瘤病例,分析其临床病理特征、治疗和预后。结果：①主要表现为阴道流血及宫颈赘生物等；②组织学特征主要为良性或非典型腺上皮组织伴有肉瘤间质,具有“腺周套袖”和息肉样突入腺腔的结构,初诊诊断率44．4%；③8例行手术治疗,其中7例术后给予化疗,1例单纯宫颈病灶摘除；④随访7例,除1例Ⅲ期患者死亡,余存活。结论：阴道流血常为本病首发症状；年轻患者易误诊为良性疾病；治疗以较广泛的手术并辅以化疗为宜；该病通常为低度恶性,预后与临床分期、患者年龄关系密切。     

Uterine Adenosarcoma with Sarcomatous Overgrowth: A Case Report of Aggressive Disease in a 16-Year-Old Girl and a Literature Review

Background

Uterine adenosarcoma with sarcomatous overgrowth (ASSO) is a rare and aggressive disease. A case of a 16-year-old girl with uterine ASSO is reported herein. The patient received surgical resection and chemotherapy and remained alive without disease 11 months after the surgery.

Adenosarcoma mülleriano de útero: un tumor infrecuente de difícil diagnóstico

Uterine adenosarcoma is a rare neoplasm constituting only around 8% of all uterine sarcomas. This tumor is associated with tamoxifen therapy and pelvic radiation. The most characteristic clinical feature is a recurrent cervical polypoid lesion. Patients have often had previous biopsies for the same reason, but without an accurate diagnosis. Furthermore, a mistaken diagnosis may have been made, the most common being cervical polyp. Uterine adenosarcoma consists of neoplastic glands with a benign appearance and a sarcomatous stroma.     

Clinicopathologic analysis of uterine sarcomas from a single institution in the Canary Islands

Objective

To evaluate the clinicopathologic data and prognostic factors for patients with uterine sarcomas treated at a single institution, with special emphasis on malignant mixed müllerian tumors (MMMT).

Methods

Medical and anatomic pathology records were reviewed. Survival rates were analyzed using the Kaplan-Meier method.

Results

The study included 89 patients: 48.4% with MMMT; 22.4% with leiomyosarcomas; 20.2% with endometrial stromal sarcomas; and 9% with adenosarcomas. FIGO stages I, II, III, and IV were identified in 57.3%, 9.0%, 22.5%, and 7.8% of patients respectively. Event-free survival rates after 2, 5, and 10 years were 70%, 61%, and 55% respectively, with a median time of 90 months (95% CI, 41-140 months). Overall survival rates after 2, 5, and 10 years were 50%, 45%, and 39% respectively, with a median time of 43 months (95% CI, 3-83 months). Multivariate analysis showed that stage, histology, tumor size, and parity had an independent influence on overall survival.

Conclusions

MMMT are the most aggressive tumors and their behavior strongly resembles that of high-grade endometrial adenocarcinoma. Prognostic factors affecting survival were stage, histology, tumor size, and parity.     

子宫腺肉瘤的研究进展

腺肉瘤是由良性腺上皮和恶性间质构成的一种低度恶性的混合性肿瘤,大多数腺肉瘤原发于子宫内膜,偶可发生在卵巢或子宫外组织。子宫腺肉瘤是罕见的女性生殖道恶性肿瘤,以绝经后妇女多见,缺乏特异的临床症状,多数患者表现为阴道出血、宫颈口脱出物及子宫增大。该病早期诊断困难,超声、磁共振成像（MRI）等影像学检查有一定的诊断价值,“袖口”状结构是术后病理检查的特征性表现。子宫腺肉瘤的发生可能与子宫内膜异位症、长期雌激素暴露等因素有关,但其具体的发病机制目前尚未明确。该病的主要治疗方法为全子宫双附件切除（因淋巴结转移少见故不常规行淋巴结清扫）,放化疗及激素治疗疗效尚不确切。但患者的生存时间及肿瘤的术后复发与是否伴肉瘤过度生长、疾病分期、子宫肌层侵犯深度、有无淋巴结转移及脉管浸润等因素密切相关。     

卵巢腺肉瘤CT表现1例

患者女,54岁。8年前患者因子宫肌瘤接受子宫次全切除术,1个月前自觉憋尿后腹部胀痛,排尿后缓解,排便时有阻力感,并扪及腹部质硬包块。外院检查:膀胱镜示膀胱底部左侧壁肿物压迫膀胱明显,CA125 517U/ml,CA19-9 919U/ml。为求进一步诊治入本院。CT:平扫盆腔见一巨大囊实混合性肿块影,最大截面11.8cm×12.0cm,向上达脐水平,其内散在致密影,与周围组织分界清楚(图1A),增强呈不均匀强化(图1B)。     

ERBB-2 gene overexpression and amplification in uterine sarcomas

BACKGROUND: The aim of this study was to determine ERBB-2 (HER-2/neu) gene alterations in different subtypes of uterine sarcomas. METHODS: After central review, representative biopsies were immunohistochemically stained and semiquantitatively scored as negative, weakly (1+), moderately (2+), or strongly (3+) positive. Subsequently, fluorescence in situ hybridization (FISH) was performed on cases with 2+ and 3+ expression. RESULTS: Seventy tumors (52 primaries and 18 recurrent) were evaluated. All 10 adenosarcomas, 21 endometrial stromal sarcomas, and 10 leiomyosarcomas were negative both in the primary and recurrent setting. Twenty-two primary carcinosarcomas were scored. The epithelial component was negative/1+ in 16 (73%), 2+/3+ in five (22.5%) tumors, and could not be evaluated in one case (4.5%), whereas the sarcoma component stained negative/1+ in 21 cases (95.5%) and 3+ (4.5%) in one case. In two recurrent carcinosarcomas, the epithelial component stained 3+ in both cases, whereas the sarcoma component scored negative and 1+. Amplification of the ERBB-2 gene as determined by FISH was observed in 3/7 (43%) carcinosarcomas with 2+ or 3+ overexpression, resulting in an overall 3/22 (14%) amplification rate. One out of four undifferentiated uterine sarcomas stained 2+. ERBB-2 immunopositivity (3+) and ERBB-2 amplification by FISH were confirmed in the recurrent tumor, resulting in a gene amplification rate of 1/4 in undifferentiated uterine sarcomas. CONCLUSION: The current results suggest absence of ERBB-2 overexpression in uterine leiomyosarcoma, uterine adenosarcoma, and endometrial stromal sarcoma, whereas the ERBB-2 gene might have a biologic role in uterine carcinosarcoma and undifferentiated uterine sarcomas.     

子宫苗勒管腺肉瘤临床病理分析

目的 探讨子宫苗勒管腺肉瘤的临床病理特点、诊治方法及预后.方法 回顾性分析北京协和医院2003年1月至2009年2月收治的9例子宫苗勒管腺肉瘤患者的临床病理资料,并进行随访.结果 9例患者中,子宫内膜腺肉瘤6例,宫颈腺肉瘤3例.主要表现为异常阴道出血和盆腔疼痛.妇科检查见宫颈或阴道肿物,可扪及子宫增大和盆腔包块.肿瘤间质以腺周套袖样及息肉样突入腺腔结构为特征性病理表现.子宫苗勒管腺肉瘤术前正确诊断率为66.7%,以临床Ⅰ期为主(7/9).9例患者均行手术治疗,术后辅以化疗7例,辅以放疗1例,高效孕激素治疗3例.有5例患者保留单侧或双侧卵巢,3例行病灶局部切除术.2例子宫内膜腺肉瘤复发,其中1例临床Ⅲ期患者合并肉瘤成分过度生长,术后13个月死于肿瘤复发;另1例患者行宫腔病灶切除术后2年复发,切除子宫后随访正常.另7例患者随访至今无复发.结论 子宫苗勒管腺肉瘤临床罕见,患者症状、体征无特异性,其诊断依赖病理形态学检察.子宫苗勒管腺肉瘤呈低度恶性,临床以早期病例为主.治疗应采用手术为主的综合疗法,临床早期且完整切除病灶者的预后较好,临床晚期且合并肉瘤成分过度生长者的预后差.子宫苗勒管腺肉瘤的复发率较高,应长期密切随诊.