Unusually long survival in a case of medullomyoblastoma

Jaiswal, Awadhesh Kumar; Jaiswal, Sushila; Mahapatra, Ashok Kumar; Sharma, Mehar Chand

doi:10.1016/j.jocn.2004.11.019

Cited by 8 publications

(10 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although there have been reported cases of survival of individuals with MMB for more than 10 years after presentation, the previous average survival for such patients has been documented at less than 3 years in the absence of available long-term follow-up data [3–4]. The persistent leptomeningeal involvement and occurrence of intra-abdominal metastases in the absence of a ventriculoperitoneal shunt in this case highlight the aggressive nature of the disease when in the presence of negative prognostic markers, specifically LC/A phenotype and MYC amplification [5–10, 13–16].…”

Section: Discussionmentioning

confidence: 99%

“…Prognosis has been described as unfavorable with few patients surviving more than 3 years [3–4], implying there may be an argument that these patients would benefit from high risk therapeutic regimens; however, evidence is lacking in this regard. Current treatment for patients with MMB parallels the standard treatment for those with MB, including tumor resection followed by irradiation and chemotherapy [1, 4].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Isochromosome 17q, MYC amplification and large cell/anaplastic phenotype in a case of medullomyoblastoma with extracranial metastases

Wright

Embse

Coleman

et al. 2011

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Medullomyoblastoma is a rare variant of medulloblastoma, a member of the family of central nervous system (CNS) embryonal tumors. The outcome of standard therapy for CNS embryonal tumors is often unpredictable in the setting of medullomyoblastoma. Here, we present the clinical course and treatment of an almost 4-year-old girl with medullomyoblastoma that was characterized by MYC amplification, isochromosome 17q and large cell/anaplastic histopathology.

show abstract

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Isochromosome 17q, MYC amplification and large cell/anaplastic phenotype in a case of medullomyoblastoma with extracranial metastases

Wright

Embse

Coleman

et al. 2011

Pediatric Blood & Cancer

View full text Add to dashboard Cite

show abstract

“…The patient was found to be disease-free at 187 months post-diagnosis. In a separate report an 8-year-old boy was diagnosed in 1999 and treated following GTR, CSI and 6 cycles of combination chemotherapy and was disease-free 11 years after diagnosis [8]. …”

Section: Discussionmentioning

confidence: 99%

“…Two cases of long-term survival have been reported in children [2, 8], but there no reports of long-term survival in an adult. We present a case of a 21-year-old diagnosed with MMD in 1999 treated with aggressive combination therapy who remains a long-term disease-free survivor 11 years following his diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Medulloblastoma with myogenic differentiation: long-term survival in a patient treated with aggressive combination therapy and autologous stem cell transplantation

et al. 2011

View full text Add to dashboard Cite

show abstract

“…In the fourth edition WHO classification, MMB is described as medulloblastoma with myogenic differentiation and is considered to be any variant of medulloblastoma because genetic changes in medulloblastoma with myogenic differentiation are similar to those in other medulloblastomas, suggesting that this is not a distinct entity. Prognosis in medulloblastoma with myogenic differentiation is poor according to the descriptions in published papers, 5,6 although there have been reports of survival up to 5 years or >10 years after presentation 6–8 …”

mentioning

confidence: 99%

Medulloblastoma with myogenic differentiation showing double immunopositivity for synaptophysin and myoglobin

Kido¹,

Ueno²,

Onodera³

et al. 2009

Pathology International

View full text Add to dashboard Cite

Reported herein is a case of medulloblastoma with myogenic differentiation in a 3-year-old girl who died 1 year after appearance of clinical signs. Magnetic resonance imaging indicated a mass lesion in the cerebellar vermis. She underwent total resection of the tumor, followed by chemotherapy and radiotherapy in the brain and spinal cord. The resected specimen mainly consisted of densely packed cells with round-to-oval highly chromatic nuclei surrounded by scanty cytoplasm and focally of long spindle-shaped cells with elongated nuclei and eosinophilic cytoplasm showing discernible cross-striations. Immunohistochemistry indicated partial expression of synaptophysin in the former area and focal expression of desmin in the latter area. The diagnosis was medulloblastoma with myogenic differentiation, also known as medullomyoblastoma. Autopsy indicated disseminated proliferation of immature neuroglial cells with highly chromatic nuclei and scanty cytoplasm showing partial expression of synaptophysin, neurofilaments, and GFAP, and focal proliferation of round-to-oval immature cells showing immunoreactivity of myoglobin. The tumor cells had large nuclei, frequent mitoses, apoptoses, nuclear molding, and cell wrapping, indicating moderate anaplasia. Their Ki-67 labeling index was 54%. In addition, some tumor cells had double immunopositivity for synaptophysin or neurofilament and myoglobin, suggesting that the neuroectodermal cells may undergo differentiation into rhabdomyoblasts.

show abstract

Unusually long survival in a case of medullomyoblastoma

Cited by 8 publications

References 13 publications

Isochromosome 17q, MYC amplification and large cell/anaplastic phenotype in a case of medullomyoblastoma with extracranial metastases

Isochromosome 17q, MYC amplification and large cell/anaplastic phenotype in a case of medullomyoblastoma with extracranial metastases

Medulloblastoma with myogenic differentiation: long-term survival in a patient treated with aggressive combination therapy and autologous stem cell transplantation

Medulloblastoma with myogenic differentiation showing double immunopositivity for synaptophysin and myoglobin

Contact Info

Product

Resources

About