Ganglioglioma Arising From Desmoplastic Medulloblastoma: A Case Report and Review of Literature

Valvi, Santosh; Ziegler, David S.

doi:10.1542/peds.2016-1403

Cited by 8 publications

(7 citation statements)

References 22 publications

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“…We identified five publications that reported six cases of histologically confirmed MB with post-treatment maturation (4 boys, 2 girls) (Tables 1 and 2). [4][5][6][7][8] The patients' mean age was 7.8 years (range, 3 months to 11 years). The MB subtypes were DMB (n¼2), MBEN (n¼1), CMB (n¼1), and unclassified (n¼2).…”

Section: Literature Searchmentioning

confidence: 99%

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

Zhou

et al. 2018

J Int Med Res

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We herein report two cases of post-treatment maturation of medulloblastoma (MB). We also conducted a literature review to summarize the clinical and pathologic features of MB maturation. From January 1992 to February 2017, 52 patients with MB underwent surgical resection followed by radiotherapy and chemotherapy. Tumor cell maturation was identified in two patients who underwent a second surgery. We conducted a systematic search of PubMed and identified six such cases. In both of our patients, the pathologic type was MB with extensive nodularity (MBEN). Both patients underwent radiotherapy and chemotherapy. The tumor differentiated to gangliocytoma in both patients. In the overall analysis that also included the six cases identified in the literature, the pathologic types were classic MB (n=1), desmoplastic/nodular MB (n=2), MBEN (n=3), and unclassified MB (n=2). MB differentiated into the following types: gangliocytoma (n=2), ganglioglioma (n=1), melanocyte (n=1), neuronal differentiation (n=2), and classic MB (n=1). Desmoplastic/nodular MB and MBEN can differentiate into less malignant cells types after radiotherapy and chemotherapy. Maturation of MB may be affected radiotherapy and chemotherapy.

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Section: Literature Searchmentioning

confidence: 99%

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

Zhou

et al. 2018

J Int Med Res

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“…Disease dissemination is an early event in MB, with up to ∼40% of patients showing metastases at diagnosis, with poor survival. Metastatic disease and tumor recurrence are responsible for the low survival rates, and those who survive frequently show treatment-related adverse effects (5,20,35,36,38,40,(50)(51)(52)(53).…”

Section: Discussionmentioning

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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“…The Neuropathological results for the 20 children in our study were desmoplastic medulloblastoma, some cases with poorly differentiated small cells with Homer-Wright Rosette and perivascular pseudo-Rosette showing lobular pattern with regions of dense intercellular reticulin and nodular reticulin-free zones, with moderate desmoplasia. Chelliah D et al and santosh Valvi et al found that desmoplastic medulloblastoma is common in paediatric age less than 3 years and has better prognosis (23,24), while Rubinstein LJ et al found that the desmoplastic type occur commonly in adult patients in the cerebellar hemispheres (40% laterally located) and that the classical type occurred in children and commonly is the midline (32).…”

Section: Discussionmentioning

confidence: 99%

Paediatric desmoplastic medulloblastoma – Surgical treatment, adjuvant therapy, outcome survival (case series study)

Hussein¹,

Al-Jumaily²,

Hamed³

et al. 2020

Ro J Neurol.

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Ganglioglioma Arising From Desmoplastic Medulloblastoma: A Case Report and Review of Literature

Cited by 8 publications

References 22 publications

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

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

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

Paediatric desmoplastic medulloblastoma – Surgical treatment, adjuvant therapy, outcome survival (case series study)

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