Reid Michelle D, Choi Hye-Jeong, Memis Bahar, Krasinskas Alyssa M, Jang Kee-Taek, Akkas Gizem, Maithel Shishir K, Sarmiento Juan M, Kooby David A, Basturk Olca, Adsay Volkan
Departments of *Pathology §Surgery, Emory University Hospital, Atlanta, GA ∥Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY †Department of Pathology, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan ‡Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, South Korea.
Am J Surg Pathol. 2015 Dec;39(12):1597-610. doi: 10.1097/PAS.0000000000000559.
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.
关于胰腺浆液性囊性肿瘤(SCN)的“变异型”及其“恶性”对应物的文献存在高度冲突。我们对193例SCN的临床病理特征进行了研究,并对相关文献进行了批判性综述。在这个最大规模的系列研究中,我们阐明了大囊型(少囊型)变异型的人口统计学特征,其发生率为21%,女性占优势(4:1),多位于体部/尾部(1.7倍),患者年龄较轻(平均年龄50岁),并且经常在放射学上被误诊为其他巨大囊性肿瘤。实性SCN很少见(n = 4,2%),在放射学上常被误诊为神经内分泌肿瘤。11例可进行细针穿刺抽吸的病例中,只有1例具有诊断价值。放射学诊断为“恶性”的占5%。在13%的切除病例中检测到相关的继发性肿瘤,大多为神经内分泌肿瘤。在6例(3%)相当大的SCN(平均11 cm)中观察到脾脏、胃、结肠和/或相邻淋巴结的继发性“浸润”(直接粘连/穿透),无远处转移。3例SCN局部复发,但无法证实初次切除的完整性。我们唯一的肝SCN没有合并胰腺肿瘤。文献评估显示,实际上没有直接归因于SCN播散/恶性行为的死亡病例,大多数报告为“恶性”的病例实际上不再符合世界卫生组织的最新标准,而是代表以下情况之一:(1)肿瘤局部粘连/持续存在;(2)没有组织学恶性证据的病例;(3)具有良性行为的肝SCN(可能代表多灶性,而非真正的转移,特别是考虑到没有与此相关的死亡病例,也没有报告转移到其他远处部位)。总之,与文献报道相反,实性和大囊型SCN变异型的临床病理特征与其微囊型对应物相似,尽管其放射学诊断具有挑战性。较大的SCN很少发生复发/继发性侵犯邻近器官,但似乎无害。除非有明确的组织学恶性证据或记录在案的远处转移,否则SCN不应被归类为“恶性”。