Post-treatment maturation of medulloblastoma in children: two cases and a literature review

Wu, Xuanxuan; Zhou, Yanmin; Li, Lusheng; Liang, Ping; Zhai, Xuan

doi:10.1177/0300060518788251

Cited by 6 publications

(15 citation statements)

References 33 publications

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“…When recurrent tumor is treated with surgical resection, pathological analysis almost universally reveals recurrent medulloblastoma. Only 6 prior case reports (8 patients in total) have described medulloblastoma undergoing posttreatment maturation into a benign neoplasm [3][4][5][6][7][8]. Of the 8 patients previously described, 3 underwent maturation into gangliocytoma.…”

Section: Discussionmentioning

confidence: 99%

“…Ten years later, the patient underwent surgical resection of recurrent posterior fossa tumor, with a resultant diagnosis of gangliocytoma. Two cases of posttreatment maturation of medulloblastoma into gangliocytoma were described by Wu et al [8]. In the first case, a 13-month-old girl presented with a right cerebellar hemisphere tumor.…”

Section: Discussionmentioning

confidence: 99%

“…Treatment comprised of cyclophosphamide, cisplatin, and vincristine, along with craniospinal irradiation with a boost to the posterior fossa. After 6 months recurrence was noted, and reoperation yielded a diagnosis of gangliocytoma [8].…”

Section: Discussionmentioning

confidence: 99%

“…Here we discuss 2 cases of World Health Organization (WHO) grade IV medulloblastoma with posttreatment maturation into gangliocytoma, a WHO grade I neoplasm. The phenomenon of posttreatment maturation of medulloblastoma is rare and poorly characterized, having been reported in the literature in only a few instances [3][4][5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%

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Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases

et al. 2020

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Introduction: We report 2 cases of medulloblastoma maturing into gangliocytoma after receiving multimodal therapy. Here we present 2 cases of diagnosed medulloblastoma which on re-resection were noted to be gangliocytoma without heterogeneity, which is an extremely rare occurrence. Case Presentation: The first patient, an 11-year-old boy diagnosed with high-risk (non-WNT, non-SHH) medulloblastoma, was treated with near-total surgical resection followed by craniospinal radiation therapy with weekly vincristine. He then received maintenance chemotherapy with vincristine, cyclophosphamide, and cisplatin. On surveillance MR imaging studies residual tumor in the lateral aspect of the tumor bed was noted to be slowly growing, eliciting gross-total resection of the residual tumor. Histopathology showed benign gangliocytoma without residual medulloblastoma. The second patient, a 3-year-old girl, was diagnosed with medulloblastoma, desmoplastic nodular variant. She was initially treated with gross total resection and chemotherapy with etoposide, carboplatin, and high-dose methotrexate. At 4 months off therapy, she was noted to have local recurrence along the resection cavity. Second-line therapy was started with irinotecan and temozolomide, but MRI assessment during treatment showed further disease progression. She then received craniospinal radiation. Eleven months off therapy, further radiographic progression was noted, and the patient underwent second-look surgery, with pathology showing gangliocytoma and treatment-related gliosis. Discussion/Conclusion: The maturation of medulloblastoma into a ganglion cell-rich lesion is very rare, with few well-characterized previous reports. Given the rare nature of this entity, it would be of great value to understand the process of posttreatment maturation and the genetic and treatment factors which contribute to this phenomenon.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases

et al. 2020

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“…Medulloblastoma (MB) is the most common malignant brain tumor in children, accounting for ∼12% of childhood cancer deaths around the globe (1)(2)(3)(4)(5). Over the last 10 years, integrative genomics has rapidly advanced our understanding of the molecular mechanisms governing MB pathogenesis, revealing the syndrome to be more heterogeneous than previously predicted (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Section: Introductionmentioning

confidence: 99%

Methylation Profiling of Medulloblastoma in a Clinical Setting Permits Sub-classification and Reveals New Outcome Predictions

et al. 2020

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Medulloblastoma (MB) is the most common childhood malignant brain tumor and is a leading cause of cancer-related death in children. DNA methylation profiling has rapidly advanced our understanding of MB pathogenesis at the molecular level, but assessments in Saudi Arabian (SA)-MB cases are sparse. MBs can be sub-grouped according to methylation patterns from FPPE samples into Wingless (WNT-MB), Sonic Hedgehog (SHH-MB), Group 3 (G3), and Group 4 (G4) tumors. The WNT-MB and SHH-MB subgroups are characterized by gain-of function mutations that activate oncogenic cell signaling, whilst G3/G4 tumors show recurrent chromosomal alterations. Given that each subgroup has distinct clinical outcomes, the ability to subgroup SA-FPPE samples holds significant prognostic and therapeutic value. Here, we performed the first assessment of MB-DNA methylation patterns in an SA cohort using archival biopsy material (FPPE n = 49). Of the 41 materials available for methylation assessments, 39 could be classified into the major DNA methylation subgroups (SHH, WNT, G3, and G4). Furthermore, methylation analysis was able to reclassify tumors that could not be sub-grouped through next-generation sequencing, highlighting its superior accuracy for MB molecular classifications. Independent assessments demonstrated known clinical relationships of the subgroups, exemplified by the high survival rates observed for WNT tumors. Surprisingly, the G4 subgroup did not conform to previously identified phenotypes, with a high prevalence in females, high metastatic rates, and a large number of tumor-associated deaths. Taking our results together, we demonstrate that DNA methylation profiling enables the robust sub-classification of four disease sub-groups in archival FFPE biopsy material from SA-MB patients. Moreover, we Alharbi et al. Clincal Methylation Profiling of Medulloblastomashow that the incorporation of DNA methylation biomarkers can significantly improve current disease-risk stratification schemes, particularly concerning the identification of aggressive G4 tumors. These findings have important implications for future clinical disease management in MB cases across the Arab world.

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Embryonal tumor with multilayered rosettes: Post‐treatment maturation and implications for future therapy

et al. 2023

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Background Embryonal tumor with multilayered rosettes (ETMR) is a deadly grade IV pediatric brain tumor. Despite an intensive multimodal treatment approach that includes surgical resection, high‐dose chemotherapy, and radiotherapy, the progression‐free survival at 5 years is less than 30%. Case We report a case of long‐term survival in a 5‐month old female with a large mass in the posterior fossa, diagnosed as ETMR, which subsequently underwent treatment‐induced maturation. Prior to chemotherapy, histopathology revealed an abundance of highly proliferative, undifferentiated cells and multilayered rosette structures. Conversely, post‐treatment histopathology revealed cell populations that differentiated into neuronal and ganglionic phenotypes. At 5‐year follow‐up, the patient remains progression‐free. Conclusion This finding contributes to the few reports to date of post‐treatment differentiation/maturation of ETMR cell populations, with an implication for less cytotoxic therapeutic interventions aimed at differentiation.

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Post-treatment maturation of medulloblastoma in children: two cases and a literature review

Cited by 6 publications

References 33 publications

Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases

Posttreatment Maturation of Medulloblastoma into Gangliocytoma: Report of 2 Cases

Methylation Profiling of Medulloblastoma in a Clinical Setting Permits Sub-classification and Reveals New Outcome Predictions

Embryonal tumor with multilayered rosettes: Post‐treatment maturation and implications for future therapy

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