Medulloblastoma with Extensive Nodularity Undergoing Post-Therapeutic Maturation to a Gangliocytoma: A Case Report and Literature Review

Chelliah, Daniel; Sarfo-Poku, Christian Mensah; Stea, Baldassarre; Gardetto, Jennifer S.; Zumwalt, Jonathan

doi:10.1159/000322896

Cited by 14 publications

(20 citation statements)

References 43 publications

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“…The cells had small round to oval nuclei with mild to moderate hyperchromasia and minimal cytoplasm. This maturation can be either focal as described by Kudo et al 12 or Kubota et al 13 or near complete as in the cases reported by Cai et al 11 or Chelliah et al 14 Spontaneous maturation of medulloblastoma into ganglioglioma at diagnosis has also been reported. 15 Notably, 3 of these cases described tumors that were desmoplastic (or MBEN), whereas histology was unclear in the remaining 3.…”

Section: Figurementioning

confidence: 78%

“…Desmoplastic medulloblastoma is more common in children younger than 3 years of age and has a better prognosis. 14 As in neuroblastoma, maturation of medulloblastoma cells may be associated with a better prognosis. 11 This case suggests that some types of medulloblastoma may have more potential for maturation, and research to identify the pathways that induce differentiation may facilitate the development of novel therapeutic approaches.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Valvi

Ziegler

2017

Pediatrics

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We describe a case of medulloblastoma maturating into ganglioglioma during therapy. A 10-month-old boy was diagnosed with a desmoplastic medulloblastoma and was treated with gross total resection followed by induction chemotherapy. A recurrence in the tumor bed during therapy was managed with focal radiation therapy and consolidation chemotherapy. After further progression, the recurrent tumor was resected completely. The histopathology revealed a benign ganglioglioma with no residual medulloblastoma. This case raises the possibility that a malignant medulloblastoma can differentiate into a benign tumor and suggests that differentiation therapy may have value in the treatment of medulloblastoma.

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Section: Figurementioning

confidence: 78%

Section: Discussionmentioning

confidence: 99%

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

Valvi

Ziegler

2017

Pediatrics

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“…The data together indicated a medulloblastoma with maturation, a finding rarely documented in medulloblastomas. 81 A young child presented with an enhancing dura-based mass adjacent to the cerebellum. Upon resection, the lesion was confidently classified as a meningioma, and clear cell features were noted.…”

Section: Embryonal Cns Neoplasmsmentioning

confidence: 99%

Genomic Analysis in the Practice of Surgical Neuropathology: The Emory Experience

Neill

Saxe

Rossi

et al. 2017

Archives of Pathology &Amp; Laboratory Medicine

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The evaluation of central nervous system tumors increasingly relies on molecular genetic methods to aid in classification, offer prognostic information, and predict response to therapy. Available assays make it possible to assess genetic losses, amplifications, translocations, mutations, or the expression levels of specific gene transcripts or proteins. Current molecular diagnostics frequently use a panel-based approach and whole genome analysis, and generally rely either on DNA sequencing or on hybridization-based methodologies, such as those used in cytogenomic microarrays. In some cases, immunohistochemistry can be used as a surrogate for genetic analysis when the mutation of interest consistently results in overexpression or underexpression of a known protein product. In surgical neuropathology practice, the diagnostic workup of diffuse gliomas, medulloblastomas, low-grade circumscribed gliomas, as well as other diseases, now routinely incorporates the results of genomic studies. Here we summarize our institution's current approach to diagnostic surgical neuropathology, using these contemporary molecular diagnostic applications.

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“…Hemispheric tumors tend to arise in older patients (adolescents and young adults) many of these tumors are desmoplastic medulloblastomas [ 18 ] However, molecular advancements have revolutionized our understanding of these tumor well beyond their histopathology (see below). Rare cases of maturation of medulloblastoma after therapy have been reported [ 19 ].…”

Section: Medulloblastoma and Its Variantsmentioning

confidence: 99%

“…These areas contain streaming small cells resembling the cells of a central neurocytoma. Maturation into tumors dominated by ganglion cells has been reported [ 19 ].…”

Section: Medulloblastoma and Its Variantsmentioning

confidence: 99%

Tumors of the Central Nervous System

Fung

Petropoulou

2014

Pediatric Malignancies: Pathology and Imaging

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Knowledge of normal neuroanatomy and neurodevelopment is critical to proper diagnosis. A few normal structures may be mistaken as tumor particularly on frozen sections.The external granular layer (Fig. 6.1a ) is a layer of small neurons that covers the external surface of fetal/infant cerebellum. It is quite prominent at the time of birth but is almost completely regressed by the ninth postnatal month. It should not be misinterpreted as medulloblastoma or lymphocytic infi ltration in younger infants. The internal granular layer is also composed of small neurons. Without a size comparison, these cells may be mistaken for medulloblastoma or other small blue cell tumors in minute specimens or cytologic preparations. Conversely medulloblastoma cells, particularly those with smaller nuclei, can be mistaken as internal granular cells. Purkinje cells are rather large and should not be mistaken as rhabdoid cells (Fig. 6.1b ). Other normal structures that can be mistaken for neoplastic tissue include the posterior pituitary and the pineal gland which may be mistaken as glial neoplasm and normal choroid plexus being mistaken as choroid plexus papilloma. These misinterpretations are likely to be committed when the specimen is minute and communication between the pathologist and the neurosurgeon is poor. Overview and Classifi cationTumor of the CNS is not uncommon in the pediatric age group and some of these entities occur only rarely in adults (Table 6.1 ). While meningiomas are very common in adults, it is uncommon in children and almost nonexistent in infants. The most common pediatric tumors in descending order of frequency are pilocytic astrocytoma, diffuse astrocytomas, medulloblastomas, and craniopharyngiomas. Ependymal neoplasms and schwannomas are the most frequent infratentorial tumors. Most tumors occur in the brain with a supratentorial-to-infratentorial ratio of approximately 2-to-1. Malignant CNS neoplasms represent 16.6 % of all malignancies in the pediatric age group. In general, the incidence is higher in males than females and among white compared to black children. The incidence is 36.2 per million between infancy and 7 years of age and 21.0 per million between 7 and 10 years of age. As a group, they are the second most frequent malignancy and most common solid tumor in pediatric patients. About 2,200 new cases are diagnosed annually in the United States. Among CNS malignancies, astrocytic tumors account for 52 %, while medulloblastoma and primitive neuroectodermal tumor (PNET), other gliomas, and ependymomas accounted for 21, 15, and 9 %, respectively [ 2 ].In contrast to adults, there is a high occurrence of malignancies in the cerebellum and brain stem in young children. About 40 % of all thalamic tumors arise in patients under 18 years old and account for 2-5 % of all intracranial tumors in children [ 3 ]. About two third or pediatric thalamic tumors are high grade in histology [ 4 ]. Tumor of the spinal cord represents about 9.9 % and extramedullary tumors are slightly more common than intramedullary...

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Medulloblastoma with Extensive Nodularity Undergoing Post-Therapeutic Maturation to a Gangliocytoma: A Case Report and Literature Review

Cited by 14 publications

References 43 publications

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

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

Genomic Analysis in the Practice of Surgical Neuropathology: The Emory Experience

Tumors of the Central Nervous System

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