Ferrari Andrea, Rognone Alessia, Casanova Michela, Zaffignani Elena, Piva Luigi, Collini Paola, Bertario Lucio, Sala Paola, Leo Ermanno, Belli Filiberto, Gallino Gianfranco
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.
Pediatr Blood Cancer. 2008 Mar;50(3):588-93. doi: 10.1002/pbc.21220.
Colorectal carcinoma (CRC) is one of the most common tumors in adults, but extremely rare in young age. This study retrospectively reports on a group of 27 patients <30 years of age, and particularly on 7 cases <18 years old, treated at the Istituto Nazionale Tumori, Milan, Italy, between 1985 and 2005.
Among the children/adolescents (age 9-18, median 12 years), 5/7 had unfavorable CRC histotypes (poorly differentiated or mucinous adenocarcinoma) and all but one had advanced disease at onset. Initial surgical resection was complete in 5/7 cases, and all patients received postoperative chemotherapy.
In the subset of patients <18 years, 6/7 had tumor progression or relapse, and 5 died of their tumor: overall survival (OS) was 23% at 5 years. In the group of 19- to 29-year-olds (young adults), 5-year OS was 72.6%.
This study confirms the rarity and poor prognosis of CRC in children and adolescents: advanced stage and an aggressive biology are hallmarks of this tumor in pediatric age, while clinical findings and outcome in young adults seem more similar to those observed in adult series. Therapeutic recommendations should stay the same as for adults. Surgery remains the mainstay of treatment and early diagnosis is crucial: it is important for pediatricians to be aware that CRC does occur in children, in order to refer suspected cases to expert physicians professionally dedicated to the management of this cancer in adults.
结直肠癌（CRC）是成人中最常见的肿瘤之一，但在年轻人中极为罕见。本研究回顾性报告了1985年至2005年间在意大利米兰国家肿瘤研究所治疗的一组27例年龄小于30岁的患者，特别是7例年龄小于18岁的患者。
在儿童/青少年（年龄9 - 18岁，中位年龄12岁）中，7例中有5例具有不良的CRC组织学类型（低分化或黏液腺癌），除1例之外所有患者发病时均为晚期疾病。7例中有5例初始手术切除完整，所有患者均接受了术后化疗。
在年龄小于18岁的患者亚组中，7例中有6例出现肿瘤进展或复发，5例死于肿瘤：5年总生存率（OS）为23%。在19至29岁的年轻成人组中，5年OS为72.6%。
本研究证实了结直肠癌在儿童和青少年中罕见且预后不良：晚期阶段和侵袭性生物学行为是该肿瘤在儿童期的特征，而年轻成人的临床表现和结局似乎与成人系列中观察到的更相似。治疗建议应与成人相同。手术仍然是主要的治疗方法，早期诊断至关重要：儿科医生必须意识到儿童确实会发生结直肠癌，以便将疑似病例转诊给专门从事成人癌症管理的专家医生。
