Secondary central nervous system metastases in children with neuroblastoma

Astigarraga, Itziar; Lejarreta, R.; Navajas, A.; Fernández‐Teijeiro, Ana; Imaz, Igone; Bezanilla, J.L.

doi:10.1002/(sici)1096-911x(199612)27:6<529::aid-mpo4>3.3.co;2-7

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“…Although surgery, radiotherapy, and chemotherapy often controlled tumor progression from localized CNS metastases, remissions have been temporary. The median survival from time of CNS recurrence ranges from 1 to 5 months 5–8. Neoplastic meningitis remains consequently a significant treatment challenge for clinical oncologists.…”

Section: Discussionmentioning

confidence: 99%

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Favorable response of intraommaya topotecan for leptomeningeal metastasis of neuroblastoma after intravenous route failure

Sirachainan

Visudtibhan

Tuntiyatorn

et al. 2007

Pediatric Blood & Cancer

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of which were totally effaced by tumor. Surgical margins were positive. The patient was then referred to our institution for postoperative RT. At 9 months of age, she was treated using intensity-modulated RT (IMRT) to a total dose of 3,600 cGy. Despite the large target volume encompassing nearly the entire left neck, great care was taken to spare normal structures such as the uninvolved bones, larynx, inner ears, and salivary glands. She required daily anesthesia, suffered episodes of apnea, and missed several days of treatment as a result.After her first week of RT, she underwent a postoperative MRI. This revealed enhancing soft tissue along the course of the resected tumor down to the level of the left lobe of the thyroid gland, with a suspicion of residual tumor and persistent lymphadenopathy. Another MRI scan after the completion of RT showed persistent residual tumor as well as lung metastases. The patient ultimately expired at the age of 11 months, 6 months after diagnosis. DISCUSSIONMRTs, defined by mutations or deletions of the hSNF5/INI1 gene on chromosome 22, have been described in multiple anatomic locations. These are rare tumors without clear treatment guidelines. The role of RT is not well defined. However, there is some suggestion that it may be beneficial in the context of multimodality therapy, as has been shown in patients with CNS MRT [4,8]. A recent report by Tekautz et al. showed improved survival in children with AT/RT 3 years treated with RT and high-dose alkylator-based CMT. The only patient in the older cohort who died was not treated with up-front RT and the only two long-term survivors in the younger patient cohort both received RT [9].Extracranial extrarenal MRTs have not been studied to the same extent as their CNS counterparts, primarily because of their extreme rarity. Our limited experience with three cases suggest that RT in the setting of CMTand surgery has the potential to prolong survival with acceptable toxicity, but outcomes are inconsistent and no definite treatment recommendations can be made. Further study is clearly warranted for non-CNS extrarenal MRT and will likely require an international cooperative effort [6]. In the meantime, an aggressive multimodality approach is appropriate for these lethal tumors.

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Section: Discussionmentioning

confidence: 99%

“…Since therapy for children with neuroblastoma involving CNS is limited, the prognosis for these children remains poor 6–8. Future studies directed at treating neoplastic meningitis are needed.…”

Section: Discussionmentioning

confidence: 99%