The treatment of adults with medulloblastoma: a prospective study

Brandes, Alba A.; Ermani, Mario; Amistà, Pietro; Basso, Umberto; Vastola, Francesca; Gardiman, Marina Paola; Iuzzolino, P.; Turazzi, S.; Rotilio, Antonino; Volpin, Lorenzo; Mazza, Carlo; Sainati, Laura; Ammannati, Franco; Berti, Franco

doi:10.1016/s0360-3016(03)00643-6

Cited by 84 publications

(63 citation statements)

References 17 publications

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“…1,7,30,31 Among these patients, 2 had early relapse in the spinal axis despite more aggressive therapy. In agreement with other reports on adults 1,4,19,32,36,40 and children, 17,37,48 we found that the presence of metastatic disease at presentation was an important prognostic factor for overall and event-free survival times. In a larger multicenter series, 7 metastatic disease did not appear to be correlated with a worse prognosis, but data were available for only 61% of the patients, and half of the patients with Stage M0 medulloblastomas received chemotherapy either before or after, or both before and after, radiotherapy.…”

Section: Prognostic Factorssupporting

confidence: 93%

“…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: 89%

“…We observed a female preponderance, unlike most previous reports that described a male preponderance, as in childhood. 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.…”

Section: Population Studymentioning

confidence: 51%

“…This impact has already been reported in other adult series 12,18,32,40 as well as in children. 13,45,47 Others 1,3,4,10,25,26,36 have found that sex did not affect survival or, on the contrary, noted better outcome for male patients. 23 Some explanations for these findings have been suggested as follows: a behavior of medulloblastoma intrinsically different between sexes, favoring a more indolent, well-localized, and more easily resectable form in female patients; the presence of female hormones, which may provide protection against tumor recurrence; and the precocious puberty induced by therapy that confers a survival advantage in children as well as the fact that girls were usually more sensitive to therapy.…”

Section: Prognostic Factorsmentioning

confidence: 99%

“…1,4,5,7,10,26,31,33,36 The best outcomes from medulloblastoma to date are from Kunschner et al, 31 who reported a 5-year overall rate of 84% for 28 adults treated between 1978 and 1998. The event-free survival rates (median 17.9 years; 72 and 57% at 5 and 10 years, respectively) in the present study are also within the published range.…”

Section: Survival Ratementioning

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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