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Clinicopathologic characteristics of 29 invasive carcinomas arising in 178 pancreatic mucinous cystic neoplasms with ovarian-type stroma: implications for management and prognosis.

起源于178例伴卵巢型间质的胰腺粘液性囊性肿瘤的29例侵袭性癌临床病理学特征:对处理和预后的影响

Jang KT,Park SM,Basturk O,Bagci P,Bandyopadhyay S,Stelow EB,Walters DM,Choi DW,Choi SH,Heo JS,Sarmiento JM,Reid MD,Adsay V

Abstract

Information on the clinicopathologic characteristics of invasive carcinomas arising from mucinous cystic neoplasms (MCNs) is limited, because in many early studies they were lumped and analyzed together with noninvasive MCNs. Even more importantly, many of the largest prior studies did not require ovarian-type stroma (OTS) for diagnosis. We analyzed 178 MCNs, all strictly defined by the presence of OTS, 98% of which occurred in perimenopausal women (mean age, 47 y) and arose in the distal pancreas. Twenty-nine (16%) patients had associated invasive carcinoma, and all were female with a mean age of 53. Invasion was far more common in tumors with grossly visible intracystic papillary nodule formation ≥1.0 cm (79.3% vs. 8.7%, P=0.000) as well as in larger tumors (mean cyst size: 9.4 vs. 5.4 cm, P=0.006); only 4/29 (14%) invasive carcinomas occurred in tumors that were <5 cm; however, none were <3 cm. Increased serum CA19-9 level (>37 U/L) was also more common in the invasive tumors (64% vs. 23%, P=0.011). Most invasive carcinomas (79%) were of tubular type, and the remainder (5 cases) were mostly undifferentiated carcinoma (2, with osteoclast-like giant cells), except for 1 with papillary features. Interestingly, there were no colloid carcinomas; 2 patients had nodal metastasis at the time of diagnosis, and both died of disease at 10 and 35 months, respectively. While noninvasive MCNs had an excellent prognosis (100% at 5 y), tumors with invasion often had an aggressive clinical course with 3- and 5-year survival rates of 44% and 26%, respectively (P=0.000). The pT2 (>2 cm) invasive tumors had a worse prognosis than pT1 (≤2 cm) tumors (P=0.000), albeit 3 patients with T1a (<0.5 cm) disease also died of disease. In conclusion, invasive carcinomas are seen in 16% of MCNs and are mostly of tubular (pancreatobiliary) type; colloid carcinoma is not seen in MCNs. Serum CA19-9 is often higher in invasive carcinomas, and invasion is typically seen in OTS-depleted areas with lower progesterone receptor expression. Invasion is not seen in small tumors (<3 cm) and those lacking intracystic papillary (mural) nodules of ≥1 cm, thus making the current branch-duct intraductal papillary mucinous neoplasm management protocols also applicable to MCNs.

摘要

对起源于粘液性囊性肿瘤(MCNs)的侵袭性癌临床病理学特征认识有限,因为许多早期研究都与非侵袭性MCNs集中分析。更为重要的是,先前许多大型研究诊断时不需要卵巢型间质(OTS)。我们分析了178例MCNs,全部病例严格确定出现OTS,98%的病例发生于围绝经期妇女(平均年龄,47岁)并发生于胰腺尾部。29(16%)名患者出现相关侵袭性癌,均为女性,平均年龄53岁。侵袭更常见于大体可见囊内形成≥1.0cm乳头状结节(79.3% vs. 8.7%,p=0.000)的肿瘤和较大肿瘤(囊平均直径:9.4 vs. 5.4cm, p=0.006);仅4/29(14%)的侵袭性癌发生于直径<5cm的肿瘤;但是,直径<3cm的肿瘤未见侵袭性癌。血清CA19-9水平(>37U/L)升高也更多见于侵袭性肿瘤(64% vs. 23%,p=0.011)。 除1例具有乳头状特征外,大多数侵袭性癌(79%)为管状,其余(5例)大部分为未分化癌(2例伴破骨样巨细胞)。有趣的是,没有胶样癌;2名患者诊断时出现淋巴结转移,并分别于第10个月和35个月时死于该病。非侵袭MCNs预后极好(5年生存率100%),而侵袭性肿瘤常呈侵袭性临床经过,3年和5年生存率分别为44%和26%(p=0.000)。pT2(大于2cm)侵袭性肿瘤比pT1(≤2cm)肿瘤预后差(p=0.000),但3名T1a(<0.5cm)疾病患者也死于该病。总之,侵袭性癌见于16%的MCNs,大多数为管状(胰胆管)型;MCNs中未见胶样癌。侵袭性癌中血清CA19-9常升高,侵袭性病变典型见于孕激素受体低表达的OTS缺乏区域。小肿瘤(<3cm)和无≥1cm的囊内乳头状(附壁)结节的肿瘤未见侵袭,因此,当前的分支导管导管内乳头状粘液性肿瘤的处理治疗方案也适用于MCNs。


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