Triponez Frederic, Goudet Pierre, Dosseh David, Cougard Patrick, Bauters Catherine, Murat Arnaud, Cadiot Guillaume, Niccoli-Sire Patricia, Calender Alain, Proye Charles A G
Department of General and Endocrine Surgery, University Hospital, Lille, France.
World J Surg. 2006 May;30(5):654-62; discussion 663-4. doi: 10.1007/s00268-005-0354-9.
The management of small, nonfunctioning pancreaticoduodenal endocrine tumors (NFPET) in multiple endocrine neoplasia type 1 (MEN1) patients is still controversial. We therefore investigated the effect of surgery on survival and tumor progression in MEN1 patients with NFPET < or = 2 cm by analyzing data from the Groupe des Tumeurs Endocrines (GTE) registry.
Among 579 MEN1 patients in the registry, 65 had NFPET < or = 2 cm. Fifteen (23%) underwent pancreatectomy, 9 at least segmental pancreatectomies and 6 biopsies or enucleations (the surgery group), and 50 (77%) were followed conservatively (the no surgery group). Age at MEN1 and NFPET diagnosis was similar in both groups, as was size of the primary tumor. Seven (10.8%) patients had metastases. Five metastases were synchronous, and 2 (one in each group) were metachronous. Tumor size was similar in patients with or without metastasis.
There was no perioperative mortality. The average follow-up time after NFPET diagnosis was 6.7 years in the surgery group and 3.3 years in the no surgery group. Three (4.6%) patients died during follow-up, 2 due to NFPET and 1 due to thymus tumor. The 2 patients who died of NFPET had undergone pancreatic surgery at the time of NFPET diagnosis. The 2 groups did not differ significantly with respect to tumor progression [5/15 (33%) vs 6/38 (16%), P = 0.16]. Overall life expectancy of patients with NFPET < or = 2 cm was not different than that of the 229 MEN1 patients in the registry without any pancreaticoduodenal tumor (P = 0.33).
This study suggests that surgery may not be beneficial for MEN1 patients with NFPET < or = 2 cm.
1型多发性内分泌腺瘤病（MEN1）患者中，小型无功能胰十二指肠内分泌肿瘤（NFPET）的治疗仍存在争议。因此，我们通过分析内分泌肿瘤研究组（GTE）登记处的数据，研究了手术对NFPET≤2 cm的MEN1患者生存及肿瘤进展的影响。
登记处的579例MEN1患者中，65例有NFPET≤2 cm。15例（23%）接受了胰腺切除术，其中9例至少进行了节段性胰腺切除术，6例进行了活检或肿瘤剜除术（手术组），50例（77%）接受保守观察（非手术组）。两组患者MEN1及NFPET诊断时的年龄、原发肿瘤大小相似。7例（10.8%）患者有转移。5例转移为同时性，2例（每组各1例）为异时性。有无转移患者的肿瘤大小相似。
无围手术期死亡。NFPET诊断后，手术组的平均随访时间为6.7年，非手术组为3.3年。3例（4.6%）患者在随访期间死亡，2例死于NFPET，1例死于胸腺肿瘤。2例死于NFPET的患者在NFPET诊断时接受了胰腺手术。两组在肿瘤进展方面无显著差异[5/15（33%）对6/38（16%），P = 0.16]。NFPET≤2 cm患者的总体预期寿命与登记处229例无任何胰十二指肠肿瘤的MEN1患者无差异（P = 0.33）。
本研究提示，手术可能对NFPET≤2 cm的MEN1患者无益。
