Sure U, Bertalanffy H, Isenmann S, Brandner S, Berghorn W J, Seeger W, Aguzzi A
Department of Neurosurgery, RWTH Aachen, Federal Republic of Germany.
Acta Neurochir (Wien). 1995;136(3-4):117-26. doi: 10.1007/BF01410612.
Although primary treatment of medulloblastoma is now successful in a high percentage of patients, its secondary manifestations still bear a poor prognosis. Thorough studies of secondary manifestations are therefore pivotal to plan therapeutic approaches for the long-term management of medulloblastoma. Here we describe the incidence of secondary tumour manifestations in 66 patients of a single centre who underwent surgery for medulloblastoma between 1975 and 1990. No patient was excluded due to a poor postoperative course. Thirty-five patients showed evidence of secondary tumour growth. Of these, 17 suffered from local recurrence, and 27 developed metastastatic disease. The median latencies for secondary manifestations were 25 months for local recurrence (n = 17), 11 months for spinal metastases (n = 10), 15 months for supratentorial metastases (n = 8), 8 months for subleptomeningeal dissemination (n = 6), and 23 months for systemic metastases (n = 8). Two patients developed primary metastatic spread to the posterior fossa. Of 8 patients with supratentorial metastases, 6 developed fronto-basal lesions. In our patients, 89% of secondary lesions occurred within less than 3 years after primary diagnosis. 85% of patients with extra-axial tumour spread had been treated with a permanent shunt. Radical tumour resection and radiotherapy with 30 Gy to the neuraxis and 20 Gy boost to the posterior fossa was an important prognostic factor in this series. Patients with additional chemotherapy did not benefit significantly from this treatment. We conclude that optimal management of the primary lesions should aim at (i) total resection, (ii) avoid permanent shunting, and (iii) completion of the radiotherapy with inclusion of the medial frontobasal cisterns in the radiotherapeutic regimen. Our analysis suggests that adequate postoperative screening programmes should consist of 3-monthly scans of the neuraxis in the first three postoperative years and 6-monthly scans thereafter.
虽然髓母细胞瘤的初始治疗目前在高比例患者中取得成功，但其继发表现的预后仍然很差。因此，对继发表现进行全面研究对于制定髓母细胞瘤长期管理的治疗方案至关重要。在此，我们描述了1975年至1990年间在单中心接受髓母细胞瘤手术的66例患者的继发肿瘤表现发生率。没有患者因术后恢复差而被排除。35例患者有继发肿瘤生长的证据。其中，17例发生局部复发，27例发生转移性疾病。继发表现的中位潜伏期为：局部复发25个月（n = 17），脊髓转移11个月（n = 10），幕上转移15个月（n = 8），软脑膜下播散8个月（n = 6），全身转移23个月（n = 8）。2例患者发生原发性转移至后颅窝。在8例幕上转移患者中，6例发生额底部病变。在我们的患者中，89%的继发病变发生在初次诊断后不到3年。85%的轴外肿瘤播散患者接受了永久性分流治疗。根治性肿瘤切除以及对神经轴给予30 Gy放疗和对后颅窝给予20 Gy增强放疗是本系列中的一个重要预后因素。接受额外化疗的患者并未从该治疗中显著获益。我们得出结论，原发性病变的最佳管理应旨在：（i）完全切除，（ii）避免永久性分流，以及（iii）完成放疗，放疗方案应包括内侧额底脑池。我们的分析表明，术后适当的筛查方案应包括术后头三年每三个月进行一次神经轴扫描，此后每六个月进行一次扫描。
