Jakacki Regina I, Feldman Hilary, Jamison Cheryl, Boaz Joel C, Luerssen Thomas G, Timmerman Robert
Division of Pediatric Hematology-Oncology, Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
Int J Radiat Oncol Biol Phys. 2004 Oct 1;60(2):531-6. doi: 10.1016/j.ijrobp.2004.03.027.
Craniospinal irradiation (CSI) is necessary in the treatment of medulloblastoma, although it results in significant long-term sequelae, particularly in young children. We prospectively evaluated the feasibility of giving preirradiation chemotherapy followed by 1800 cGy CSI to young children with localized medulloblastoma.
Between January 1993 and July 1997, 7 consecutive patients (age, 20-64 months) with M0 medulloblastoma were enrolled. After surgical resection, patients received 4 months of multiagent chemotherapy followed by 1800 cGy CSI and 5400 cGy to the posterior fossa.
Median follow-up is 8.9 years. No patient developed progressive disease during chemotherapy. One patient developed widespread metastatic recurrence 2 months after completing radiation therapy and died. Two additional patients developed isolated frontal horn relapses 32 and 36 months after initial diagnosis and received further irradiation and chemotherapy. Both of these patients remain alive 7.1 and 3.6 years from the time of recurrence. Four of the six survivors have endocrine deficits. All of the survivors require special assistance in school.
Craniospinal irradiation doses of 1800 cGy may not be adequate to prevent exoprimary recurrences. Despite the CSI dose reduction, neuroendocrine and neurocognitive sequelae are substantial.
颅脊髓照射(CSI)在髓母细胞瘤的治疗中是必要的,尽管它会导致显著的长期后遗症,尤其是在幼儿中。我们前瞻性地评估了对患有局限性髓母细胞瘤的幼儿先进行放疗前化疗然后给予1800 cGy CSI的可行性。
1993年1月至1997年7月,连续纳入7例年龄在20 - 64个月的M0髓母细胞瘤患者。手术切除后,患者接受4个月的多药化疗,随后接受1800 cGy CSI以及后颅窝5400 cGy的照射。
中位随访时间为8.9年。化疗期间无患者出现疾病进展。1例患者在完成放疗后2个月出现广泛转移性复发并死亡。另外2例患者在初次诊断后32和36个月出现孤立的额角复发,接受了进一步的照射和化疗。这2例患者自复发后分别存活7.1年和3.6年。6名幸存者中有4名存在内分泌缺陷。所有幸存者在学校都需要特殊帮助。
1800 cGy的颅脊髓照射剂量可能不足以预防初次复发。尽管降低了CSI剂量,但神经内分泌和神经认知后遗症仍然很严重。