Perlman Elizabeth J, Faria Paulo, Soares Andreia, Hoffer Fred, Sredni Simone, Ritchey Michael, Shamberger Robert C, Green Daniel, Beckwith J B
Department of Pathology, Children's Memorial Medical Center and the Robert H. Lurie Comprehensive Cancer Center of Northwestern University's Feinberg School of Medicine, Chicago Illinois 60614, USA.
Pediatr Blood Cancer. 2006 Feb;46(2):203-21. doi: 10.1002/pbc.20386.
This study provides insight into the clinical behavior, diagnostic complexities, and long-term management of patients with hyperplastic perilobar nephroblastomatosis (HPLN).
Fifty-two patients with HPLN with available long-term follow-up were retrospectively analyzed for pathologic, radiologic, and clinical features.
The mean age at diagnosis was 16 months; the lesions were bilateral in 49 patients. Of 33 patients who initially underwent diagnostic biopsy and adjuvant chemotherapy, 18 (55%) developed Wilms tumor (WT) at a mean of 35 months from diagnosis. Of 16 patients whose initial therapy included nephrectomy and adjuvant therapy, three (19%) developed WT at a mean of 36 months from diagnosis. All three patients who underwent initial diagnostic biopsy and received no adjuvant therapy during their initial course developed WT 4, 4, and 10 months following diagnosis. 24/52 patients developed either a single (13 patients) or multiple (11 patients) WT throughout their course; 8/24 (33%) of WT were anaplastic. The time from initial diagnosis to the development of the last WT ranged from 13 to 116 months (mean 42 months). Three children with HPLN died of WT at 3, 5, and 6 years of age; 2/3 were anaplastic.
HPLN is a self-limited, pre-neoplastic proliferative process associated with a high risk of developing WT. The accurate diagnosis and the choices of therapy during the often-complex course of HPLN depend on the availability and accurate interpretation of a combination of pathologic, radiologic, and clinical information. When such information is appropriately obtained, the long-term survival of patients with HPLN is excellent.
本研究深入探讨了增生性叶旁肾母细胞瘤（HPLN）患者的临床行为、诊断复杂性及长期管理。
对52例有长期随访资料的HPLN患者的病理、放射学和临床特征进行回顾性分析。
诊断时的平均年龄为16个月；49例患者的病变为双侧性。在最初接受诊断性活检和辅助化疗的33例患者中，18例（55%）在诊断后平均35个月时发生了肾母细胞瘤（WT）。在最初治疗包括肾切除术和辅助治疗的16例患者中，3例（19%）在诊断后平均36个月时发生了WT。在最初接受诊断性活检且在初始病程中未接受辅助治疗的所有3例患者中，分别在诊断后4、4和10个月时发生了WT。52例患者中有24例在整个病程中发生了单发（13例）或多发（11例）WT；24例WT中有8例（33%）为间变性。从最初诊断到最后发生WT的时间为13至116个月（平均42个月）。3例HPLN患儿分别在3岁、5岁和6岁时死于WT；其中2/3为间变性。
HPLN是一种自限性的肿瘤前增殖过程，发生WT的风险很高。在HPLN通常复杂的病程中，准确的诊断和治疗选择取决于病理、放射学和临床信息的综合可用性及准确解读。当适当地获取这些信息时，HPLN患者的长期生存率很高。
