Treatment of Medulloblastoma in Adults

Hartsell, William F.; Montag, Anthony; Lydon, Jean; Galinsky, Dennis L.; Sarin, Pramilla

doi:10.1097/00000421-199206000-00005

Cited by 50 publications

(21 citation statements)

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“…1,4,10,22,31,32,38,40 The distribution of histological variants was also comparable to that reported by other authors, 1,7,10,19,24,26,31,34,36,38,43 with predominance of the classic histological phenotype. However, our population showed some characteristics different from other series.…”

Section: Population Studysupporting

confidence: 90%

“…1,3,4,7,8,10,15,19,26,32,33,43 This female preponderance is unusual but not exceptional as it has also been reported in several other studies. 14,22,24,38 Moreover, despite the fact that our retrospective study began in the late 1970s before the modern era of neuroimaging and surgical tools, we observed that a gross-total removal of the primary tumor was achieved in 78% of all the patients, and in 95% of patients whose tumors did not invade the brainstem or the surrounding structures. These findings contrast with other authors' reports of complete resection in about half of the patients.…”

Section: Population Studymentioning

confidence: 78%

“…These findings contrast with other authors' reports of complete resection in about half of the patients. 10,19,22,24,26,31,33,36,45 For many years, our general surgical principle has been to attempt as complete a resection of the tumor as possible, but it was also guided by what the surgeon considered prudent at the time: no attempts were made to remove the tumor from within the brainstem in cases of infiltration into the floor of the fourth ventricle or the cerebellar peduncles. This series also demonstrated that adult patients with medulloblastoma generally present with symptoms and signs indicating a posterior fossa lesion, and that the majority of patients have disease limited to the posterior fossa at the time of initial staging.…”

Section: Population Studymentioning

confidence: 99%

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Survival and prognostic factors in a series of adults with medulloblastomas

Riffaud

Saïkali

Leray

et al. 2009

JNS

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Object In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control. Methods Between 1977 and 2005, 27 patients who were ≥ 16 years old and had medulloblastoma were treated consecutively. There were 16 women and 11 men with a median age of 21 years (range 16–54 years). Gross-total resection was performed in 21 patients, subtotal (≥ 90%) in 2, incomplete in 1, and biopsy in 3 patients. Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma. Staging according to the Chang classification showed 4 patients with tumors invading the brainstem (2 with Stage T3b and 2 with Stage T4), 3 patients with metastases (2 with Stage M2 and 1 with Stage M3), and 1 patient in whom the stage was unknown (Stage MX) who died 10 days postoperatively. Twenty patients were assigned to the standard-risk group and 7 to the high-risk group. All patients except the one whose status was classified as Stage MX underwent craniospinal radiotherapy at our institution. Seven patients received chemotherapy before radiotherapy. Results The 5- and 10-year overall survival rates for the present study were 81 and 62%, respectively. The median overall survival time was 17.7 years. The 5- and 10-year event-free survival rates were 72 and 57%, respectively. The median event-free survival time was 17.9 years. Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome). Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables. Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times. Eleven patients suffered tumor recurrence within a median time of 4.2 years. The posterior fossa was not the most common site of recurrence, and delayed recurrence was not rare. All patients in whom the tumor recurred have died despite aggressive treatments. The median survival time after diagnosis of recurrence was 2.5 years. Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment. The authors observed 1 supposed second malignancy (thyroid carcinoma) and no evidence of pituitary dysfunction. Conclusions Long-term survival is possible in adults treated for medulloblastoma. Although rare, metastasis seeding at presentation is a poor prognostic factor. The possibility of delayed recurrence necessitates close follow-up of all patients. Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response. Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.

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Section: Population Studysupporting

confidence: 90%

Section: Population Studymentioning

confidence: 78%

Section: Population Studymentioning

confidence: 99%

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Survival and prognostic factors in a series of adults with medulloblastomas

Riffaud

Saïkali

Leray

et al. 2009

JNS

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“…6,7 Some studies suggested that adjuvant chemotherapy decreases the subsequent risk of extraneural disease including metastasis to bone. 8 The prognosis for patients with extraneural relapse is poor, with a median survival of 5 to 10 months. 1 Bone metastases from medulloblastoma most commonly involve the axial skeleton, particularly the pelvis and vertebrae.…”

Section: Discussionmentioning

confidence: 99%

High-dose chemotherapy with hematopoietic stem cell transplantation in adults with bone marrow relapse of medulloblastoma: report of two cases

Millot

Giraud

et al. 1999

Bone Marrow Transplant

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Summary:Extraneural relapses of medulloblastoma are associated with a very poor outcome. We present two cases of young adults who developed bone marrow metastases after treatment of medulloblastoma. A very good response to a sequentially scheduled combination of carboplatin and etoposide was observed. Then, high-dose chemotherapy was delivered consisting of busulfan and thiotepa followed by infusion of autologous hematopoietic stem cells. Toxicity of the conditioning regimen was acceptable. The patients remained free of disease 20 and 27 months from the time of relapse, respectively. Further studies are needed to evaluate the impact of high-dose chemotherapy in terms of survival of such patients. Keywords: adult medulloblastoma; metastases; chemotherapy; autologous stem cell transplantation Medulloblastoma, a malignant tumor originating in the cerebellum, generally occurs in the first decade of life. It is a rare disease in the adult population and represents less than 1% of adult brain tumors. Extraneural relapses of medulloblastoma represent 7 to 13% of recurrences and are associated with a very poor outcome. 1 Encouraging preliminary data have been reported in children with recurrent malignant brain tumors receiving high-dose chemotherapy with autologous bone marrow rescue. 2,3 We report two young adults who underwent autologous hematopoietic stem cell transplantation for bone marrow relapse of medulloblastoma. Case reports Patient 1In July 1995, a non-metastatic cerebellar medulloblastoma was diagnosed in a 22-year-old man. The tumor was totally resected and cranio-spinal radiation therapy was given postoperatively. Fifteen months later the patient complained of left hip pain. A pelvic magnetic resonance (MR) scan revealed diffuse low intensity signals on T1-weighted images, high intensity signals on T2-weighted images in the bone marrow of the left acetabulum and the body of the left ilium with enhancement after gadolinium administration ( Figure 1a). An open biopsy of the left ilium and a bone marrow biopsy of the right posterior iliac crest revealed marrow involvement with metastatic medulloblastoma cells. No evidence of recurrent tumor within the central nervous system was demonstrated. Combination chemotherapy consisting of carboplatin (160 mg/m 2 /day) and etoposide (100 mg/m 2 /day) for 5 days was administered. Cycles were repeated at 4 weekly intervals. The patient was symptom-free after the first course of chemotherapy. Bone marrow biopsy was normal after the second course of chemotherapy. He received a total of five courses of carboplatin-etoposide. There were no episodes of febrile neutropenia. The main toxicities were nausea and myelosuppression. After the fifth course of chemotherapy, MR revealed marked improvement in the left ilium and left acetabulum bone marrow signals (Figure 1b). Cytapheresis was performed after a 3 g/m 2 cyclophosphamide infusion followed by G-CSF administration, leading to the collection of 2 × 10 6 CD34 cells/kg (before cryopreservation) and 8.5 × 10 4 CFU-GM/kg (after ...

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“…In some series the prognosis in adults was reported as the same as that in children, 2,4 whereas others have reported better survival in adults because recurrences appear earlier in children. 4,20,37 Long-term survival is possible in adults with medulloblastoma; the reported rates range from 50 to 84% at 5 years and 40 to 50% at 10 years. 2,3,6,8 Because the data in the literature are based on retrospective studies, treatments are neither randomized nor uniform; however, some treatment cornerstones have been identified.…”

Section: Neurosurg Focus / Volume 14 / April 2003mentioning

confidence: 99%

De novo cerebellar medulloblastoma in a 26-year recurrence-free case of temporal glioblastoma multiforme

Fandiño¹,

Am²,

Rh³

et al. 2003

FOC

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The authors describe a patient who survived 26 years after resection of a right temporal glioblastoma multiforme (GBM) without signs of tumor recurrence. Preoperative emergency angiography demonstrated a hypovascular mass localized in the right temporal lobe with right-to-left shift of the vascular structures. At surgery, the tumor had cystic and solid components localized in the lateral occipitotemporal gyrus, reaching the posterolateral wall of the inferior horn of the right lateral ventricle and extending to the trigone and posterior horn. The initial pathological diagnosis of a GBM was reviewed and confirmed throughout the follow-up period. Twenty-six years after surgery and subsequent radiosurgery, the patient underwent resection of a medulloblastoma localized in the right cerebellum as well as stereotactic biopsy sampling of tissue at the original GBM site. Neither radiological nor histological evidence of recurrence of the GBM could be documented. The intraoperative, histological, and radiological findings are described.

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Treatment of Medulloblastoma in Adults

Cited by 50 publications

References 0 publications

Survival and prognostic factors in a series of adults with medulloblastomas

Survival and prognostic factors in a series of adults with medulloblastomas

High-dose chemotherapy with hematopoietic stem cell transplantation in adults with bone marrow relapse of medulloblastoma: report of two cases

De novo cerebellar medulloblastoma in a 26-year recurrence-free case of temporal glioblastoma multiforme

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