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Serous Neoplasms of the Pancreas: A Clinicopathologic Analysis of 193 Cases and Literature Review With New Insights on Macrocystic and Solid Variants and Critical Reappraisal of So-called "Serous Cystadenocarcinoma".

193例胰腺浆液性肿瘤的临床病理分析和文献复习:对大囊型和实性型浆液性囊腺瘤提出新见解,所谓的“浆液性囊腺癌”需重新评估

Reid MD,Choi HJ,Memis B,Krasinskas AM,Jang KT,Akkas G,Maithel SK,Sarmiento JM,Kooby DA,Basturk O,Adsay V

Abstract

The literature on "variants" and "malignant" counterparts of pancreatic serous cystic neoplasms (SCNs) is highly conflicted. Clinicopathologic characteristics of 193 SCNs were investigated, along with a critical literature review. For the macrocystic (oligocystic) variant, in this largest series, a demographic profile in contrast to current literature was elucidated, with 21% frequency, predominance in female individuals (4:1), body/tail location (1.7×), younger age of patients (mean age, 50 y), and frequent radiologic misdiagnosis as other megacystic neoplasms. Solid SCNs were rare (n=4, 2%) and often misinterpreted radiologically as neuroendocrine tumors. Available fine-needle aspiration in 11 cases was diagnostic in only 1. Radiologic impression was "malignancy" in 5%. Associated secondary tumors were detected in 13% of resections, mostly neuroendocrine. Secondary "infiltration" (direct adhesion/penetration) of spleen, stomach, colon, and/or adjacent nodes was seen in 6 (3%) fairly large SCNs (mean, 11 cm) with no distant metastasis. Three SCNs recurred locally, but completeness of original resection could not be verified. Our only hepatic SCN lacked a concurrent pancreatic tumor. Literature appraisal revealed that there are virtually no deaths that are directly attributable to dissemination/malignant behavior of SCNs, and most cases reported as "malignant" in fact would no longer fulfill the more recent World Health Organization criteria but instead would represent either (1) local adhesion/persistence of tumor, (2) cases with no histologic verification of malignancy, or (3) liver SCNs with benevolent behavior (likely representing multifocality, rather than true metastasis, especially considering there was no fatality related to this and no reported metastases to other remote sites). In conclusion, in contrast to the literature, the clinicopathologic characteristics of solid and macrocystic SCN variants are similar to their microcystic counterpart, although their radiologic diagnosis is challenging. Recurrence/secondary invasion of neighboring organs occurs rarely in larger SCNs but seems innocuous. An SCN should not be classified as "malignant" unless there is clear-cut evidence of histologic malignancy or documented distant metastasis.

摘要

文献中关于胰腺浆液性囊性肿瘤(SCNs)各亚型及浆液性囊腺癌的描述非常矛盾。本文调查了193例SCNs的临床病理特征并进行了文献复习。对照最近文献,该大宗病例描述了大囊型(寡囊型)浆液性囊腺瘤的分布特征:发生率21%,患者以女性为主(男:女=1:4),部位为胰体/胰尾(胰体:胰尾=1.7:1),患者更年轻(平均年龄50岁),影像学常误诊为其它大囊性肿瘤。实性SCNs少见(4/193,2%),影像学常误诊为神经内分泌肿瘤。有11例进行了细针穿刺检查,仅确诊1例。5%的病例影像学印象为恶性。13%胰腺切除SCNs病例检测到伴发第二种肿瘤,常是神经内分泌肿瘤。6例(3%)巨大囊型SCNs(直径平均11cm)见继发性脾脏、胃、结肠和/邻近淋巴结浸润”(直接粘连/渗透),但没有远处转移。3例 SCNs局部复发,首次手术肿瘤是否完整切除无从考证。我们仅有1例肝脏SCN,胰腺无并发肿瘤。文献评估显示几乎没有1例患者的死亡可直接归因于 SCNs播散/恶性行为,实际上大多数被诊断为恶性浆液性囊腺癌的病例并不满足最近WHO标准,但有如下之一表现:(1)局部粘连/肿瘤持久,(2)病例未经组织学证实为恶性或(3)肝脏发生SCNs,生物学行为温和(可能代表病变多灶性生长,而不是真正的转移,特别是考虑到SCNs并没有引起死亡,亦没有报道转移到其它远处部位)。总之, 跟文献报道不同,我们的研究表明,实性型和大囊型SCNs临床病理特征与微囊型SCNs相似,但影像学诊断富有挑战性。巨大SCNs病例很少发生复发/继发性浸润邻近器官,即使发生似乎也没有大的危害。SCN不应归类为恶性,除非有确凿的恶性组织学证据或明确的远处转移。

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