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High Incidence of Extrapancreatic Neoplasms in Patients with Intraductal Papillary Mucinous Neoplasms

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High Incidence of Extrapancreatic Neoplasms in Patients with Intraductal Papillary Mucinous Neoplasms ORIGINAL ARTICLE High Incidence of Extrapancreatic Neoplasms in Patients With Intraductal Papillary Mucinous Neoplasms Min-Gew Choi, MD; Sun-Whe Kim, MD; Sung-Sik Han, MD; Jin-Young Jang, MD; Yong-Hyun Park, MD Background: Intraductal papillary mucinous neo- (33%) and colorectal adenocarcinoma (17%) were the plasms (IPMNs) are associated with a high incidence of most common neoplasms in the 24 patients. During post- extrapancreatic neoplasms. operative follow-up, 3 patients died of malignant IPMNs, 3 of associated malignancies, and 1 of a nonmalignancy- Design: Retrospective study. related cause. Comparisons of the clinicopathological fea- tures in patients with IPMNs with and without associ- Setting: Tertiary care referral center. ated malignancies revealed no significant differences in age, sex, family history of malignancy, history of ciga- Patients: Sixty-one patients underwent surgical resec- rette smoking or alcohol abuse, or type of IPMN. The in- tion for IPMN between January 1, 1993, and June 30, cidence of extrapancreatic neoplasms in patients with 2004. Thirty-eight patients with mucinous cystic neo- IPMN was significantly higher than in those with other plasms and 50 patients with pancreatic ductal adenocar- pancreatic diseases such as mucinous cystic neoplasm cinoma also were examined for development of extra- (8%) or pancreatic ductal adenocarcinoma (10%). pancreatic neoplasms. Conclusions: Frequently, IPMNs are associated with the Main Outcome Measures: The incidence and clini- development of extrapancreatic neoplasms. Consider- copathological features of extrapancreatic neoplasms with able attention should be paid to the possible occurrence IPMNs were compared with those with mucinous cystic of other associated malignancies in patients with IPMNs, neoplasm and pancreatic ductal adenocarcinoma. either concurrently or postoperatively. Further molecu- lar studies may be necessary to elucidate the unusual as- Results: Of the 61 patients with IPMNs, 24 (39%) de- sociation between IPMN and other primary neoplasms. veloped 26 extrapancreatic neoplasms, and 18 (30%) had extrapancreatic malignancies. Gastric adenocarcinoma Arch Surg. 2006;141:51-56 NTRADUCTAL PAPILLARY MUCI- cause of a favorable prognosis in patients nous neoplasm (IPMN) is now a with IPMN, these second primary neo- well-recognized entity in the pan- plasms, especially malignant tumors, could creas and is being reported with in- be more important than IPMN in deter- creasing frequency.1,2 The tumor mining prognosis. These second primary Icovers a broad histological spectrum, which neoplasms also offer an important insight ranges from adenoma to invasive carci- into the cause of IPMNs and their associ- noma, and shows a variety of biological be- ated malignancies, which may have a com- haviors.3 Moreover, IPMNs are believed to mon carcinogenic process. In the present progress slowly showing a spectrum of neo- study, we evaluated the incidence of extra- plastic transformations and are character- pancreatic neoplasms in patients with IPMN ized by a more favorable prognosis than and analyzed clinicopathological features pancreatic ductal adenocarcinomas in these patients. The results obtained were (PDACs), even in malignant cases.1-6 compared with those for mucinous cystic neoplasm (MCN) and PDAC. See Invited Critique at end of article METHODS Author Affiliations: Between January 1, 1993, and June 30, 2004, Department of Surgery, Seoul Another important feature of IPMN is 61 patients underwent surgical resection for National University College of that the tumor is associated with a high fre- IPMN at our institution. Medical records were Medicine, Seoul, South Korea. quency of extrapancreatic neoplasms.2,7 Be- reviewed retrospectively for the following in- (REPRINTED) ARCH SURG/ VOL 141, JAN 2006 WWW.ARCHSURG.COM 51 ©2006 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 RESULTS Table 1. Clinicopathologic Features of 61 Resected Intraductal Papillary Mucinous Neoplasms Of the 61 patients with IPMN, 24 (39%) developed 26 Characteristic Result extrapancreatic neoplasms, and 18 (30%) had other ex- Follow-up, mo, mean ± SD 28.8 ± 27.3 trapancreatic malignancies (Table 2). The stomach, Age, y, mean ± SD 62.4 ± 7.4 colorectum, and thyroid gland were the organs most fre- Sex ratio (male:female) 1.7:1 Sex, No. (%) quently involved in IPMN-associated neoplasms, which Male 38 (62) developed in 9, 6, and 3 patients, respectively. The most Female 23 (38) frequently associated extrapancreatic neoplasms were gas- Type of intraductal papillary mucinous tric adenocarcinoma in 8 patients (33%) and colorectal neoplasm, No. (%) adenocarcinoma in 4 (17%). Of 26 extrapancreatic neo- Main duct 18 (30)* plasms, 8 were diagnosed 3.7 to 91.0 months (mean, 29.4) Branch duct 37 (61)* Combined 5 (8)* before the IPMN operation, 15 concurrently, and 3 at 9.5 Not classified 1 (2) to 44.2 months (mean, 23.3) postoperatively. Location, No. (%) All associated neoplasms were proved pathologically Head 28 (46) and treated surgically or by means of colonoscopic re- Head and body 1 (2) moval, except a case of low-grade B-cell lymphoma and Body 16 (26) a case of thyroid adenoma, which were diagnosed at lymph Body and tail 9 (15) node biopsy and fine-needle aspiration, respectively, and Tail 5 (8) Entire 2 (3) managed medically. In IPMN cases with concurrently as- Histologic classification, No. (%) sociated extrapancreatic neoplasms, thyroid cancer and Adenoma 10 (16) colon tubular adenoma were detected at physical exami- Borderline adenoma 34 (56) nation and at an additional preoperative, but not rou- Carcinoma in situ 6 (10) tine, colonoscopic examination. In other cases, most Invasive carcinoma 11 (18) IPMNs were detected secondarily at computed tomog- raphy during preoperative evaluation of concurrent gas- *Percentage of those classified. trointestinal tumors. During postoperative follow-up, 3 patients died of ma- formation: patient characteristics, perioperative clinical and labo- lignant IPMNs and 3 of associated malignancies (ie, co- ratory data, operative management, pathology examination lon cancer, common bile duct cancer, and lymphoma). results, and postoperative course. History of a previous extra- Another patient died of a nonmalignancy-related cause. pancreatic neoplasm or the concurrent development of an ex- The 5-year disease-specific survival rates were 93.4% for trapancreatic neoplasm was investigated thoroughly. Overall all tumors, 100.0% for all noninvasive tumors (includ- survival and follow-up information, including the postopera- ing adenoma, borderline, and carcinoma in situ le- tive development of neoplasms in other organs, was obtained sions), and 68.2% for invasive cancers. by contacting the Cancer Registration and Biostatics Branch of A comparison of clinicopathological features in pa- the National Cancer Center Research Institute, Goyang-si, Gyeonggi-do, Korea, by direct patient contact, and by review- tients with IPMN with and without associated extrapan- ing outpatient medical records. Perioperative mortality was de- creatic neoplasms revealed no significant differences in fined as in-hospital death or death within 30 days after sur- mean age, sex ratio, mean follow-up, family history of gery, and a positive family history was defined as a history of malignancy, family history of gastrointestinal malig- malignancy among first-degree relatives. nancy, history of cigarette smoking or alcohol abuse, and Mean follow-up was 28.8 months and mean patient age was type of IPMNs (Table 3). However, borderline lesions 62.4 years (Table 1). Sixty of the 61 IPMNs were classified as and distally located IPMNs were found more frequently main duct (18 [30%]), branch duct (37 [61%]), or combined in patients with extrapancreatic neoplasms. When pa- (5 [8%]). Of the 61 patients, 10 (16%) had adenomas, 34 tients were grouped according to the presence of asso- (56%) had borderline adenomas, 6 (10%) had carcinoma in ciated extrapancreatic malignancies, no significant dif- situ, and 11 (18%) had invasive carcinoma. No postoperative death occurred. ference was observed in any characteristic except mean The incidence and clinicopathological features of extrapan- follow-up, which was significantly shorter in those with creatic neoplasms in 38 patients with MCNs who underwent associated malignancies (Table 4). Of the 11 invasive surgical resection at our institution between January 1, 1993, IPMNs, 2 cases were included in the malignancy-asso- and June 30, 2004, and in 50 patients with PDAC who under- ciated group and 9 in the nonassociated group. Three pa- went surgical resection at our institution between January 1, tients died of extrapancreatic malignancies in the malig- 2000, and December 31, 2002, also were analyzed and com- nancy-associated group and 3 of malignant IPMNs in the pared with those of the patients with IPMN. One postopera- nonassociated group. No statistical difference in sur- tive death occurred among the patients with MCN, for an over- vival rates was found between patients with IPMN with all perioperative mortality of 3%. Of the 38 MCNs, 21 (55%) and without extrapancreatic malignancies (Figure). were benign adenomas; 12 (32%), borderline; 3 (8%), nonin- vasive carcinoma; and 2 (5%), invasive carcinoma. No postop- The incidence of extrapancreatic neoplasms in pa- erative death occurred among the 50 patients with PDAC. Data tients with IPMN (39%) was significantly greater than in were compared by using the ␹2
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