Department of Pediatric Surgery, Juntendo University Urayasu Hospital, 2-1-1 Tomioka, Urayasu-shi, Chiba, Japan.
Department of Pediatric General and Urogenital Surgery, Juntendo University Graduate School of Medicine, Tokyo, Japan.
Pediatr Surg Int. 2023 Nov 23;40(1):5. doi: 10.1007/s00383-023-05582-z.
Dysplasia, carcinoma in situ, and other malignant transformation or premalignant/malignant histopathology (PMMH) seem uncommon in pediatric choledochal cyst (CC). A literature review and the authors' experience are presented.
All reports about PMMH in CC patients 15 years old or younger published in English and all cases of PMMH in specimens excised from CC patients 15 years old or younger by the authors were reviewed.
Of 20 published reports, PMMH was adenocarcinoma (n = 4), sarcoma (n = 4), and dysplasia (n = 12). Treatment for malignancies was primary pancreaticoduodenectomy (PD; n = 2) or cyst excision/hepaticojejunostomy (Ex/HJ; n = 6). Outcomes at the time of writing for malignancies: 2 deaths, 4 survivors after follow-up of 2 years, and 2 lost to follow-up. No dysplasia case has undergone malignant transformation. The authors have experienced 7 cases of PMMH; adenocarcinoma in situ (AIS; n = 1) and dysplasia (n = 6).
The present study identified the youngest cases of AIS and dysplasia from specimens excised when they were 3 years old and 4 months old, respectively. Both are published for the first time as evidence that PMMH can complicate CC in young patients. Long-term protocolized postoperative follow-up is mandatory when PMMH is diagnosed in pediatric CC.
发育不良、原位癌和其他恶性转化或癌前/恶性组织病理学（PMMH）在小儿胆总管囊肿（CC）中似乎不常见。本文呈现了文献复习和作者的经验。
作者复习了所有以英文发表的关于 15 岁或以下 CC 患者 PMMH 的报告，以及作者切除的 15 岁或以下 CC 患者标本中 PMMH 的所有病例。
20 篇已发表的报告中，PMMH 为腺癌（n=4）、肉瘤（n=4）和发育不良（n=12）。恶性肿瘤的治疗方法是胰十二指肠切除术（PD；n=2）或囊肿切除/肝肠吻合术（Ex/HJ；n=6）。截至撰写本文时，恶性肿瘤的结局：2 例死亡，4 例随访 2 年后存活，2 例失访。无发育不良病例发生恶性转化。作者共经历了 7 例 PMMH；原位腺癌（AIS；n=1）和发育不良（n=6）。
本研究鉴定了分别为 3 岁和 4 个月时切除标本中出现的最小年龄的 AIS 和发育不良病例。两者均首次发表，证明 PMMH 可使年轻患者的 CC 复杂化。当在小儿 CC 中诊断出 PMMH 时，必须进行长期的、有组织的术后随访。
