Medulloblastoma: clinical presentation and management

Experience at the Hospital For Sick Children, Toronto, 1950–1980

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✓ The authors review the cases of 144 children with medulloblastoma treated between 1950 and 1980. Duration of time between onset of symptoms and initial treatment was less than 1½ months in 51% of cases, and less than 3 months in 76%. The tumor was located in the cerebellar vermis in 93% of patients. Brainstem infiltration was noted in 32%. Classical medulloblastomas comprised 82% of the total number reviewed, and desmoplastic medulloblastomas 15%. The majority of desmoplastic medulloblastomas were found in the midline of the cerebellum and in patients under 10 years of age. The prognosis for patients with desmoplastic medulloblastomas was worse than that for children with classical medulloblastomas. Spontaneous hemorrhage associated with primary or recurrent medulloblastoma occurred in 5.6% of the patients. Supratentorial metastases were found in 14.6% of cases, spinal cord metastases in 12.5%, and systemic metastases in 9%. The overall 5-year survival rate was 47%, and the 10-year survival rate 42%. Extent of surgical excision proved to be a statistically significant prognostic factor. Two patients developed recurrence after the “period of risk” as defined by Collins' rule. Delayed complications of radiotherapy were found to be substantial. Intelligence quotient (IQ) testing on 16 survivors revealed verbal IQ, performance IQ, and full-scale IQ to be within the normal range in 11, seven, and nine cases, respectively. Two were retarded on all scores.

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Address reprint requests to: Harold J. Hoffman, M.D., Division of Neurosurgery, Suite 1504, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.

© AANS, except where prohibited by US copyright law.

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Figures

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    Overall survival rate in 129 patients. Dotted line = 95% confidence limits.

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    Surgical excision in relation to survival rates. Degree of excision is defined as partial, subtotal, and total. N = number of cases of each.

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    Treatment period in relation to survival rates. N = number of cases.

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    Collins' rule for period of risk of recurrence applied to medulloblastomas in this series. There were two exceptions to this rule.

References

1.

Arseni CConstantinescu ALDanaila L: Supratentorial dissemination of medulloblastomas. Schweiz Arch Neurol Neurochir Psychiatr 119:2993061976Arseni C Constantinescu AL Danaila L: Supratentorial dissemination of medulloblastomas. Schweiz Arch Neurol Neurochir Psychiatr 119:299–306 1976

2.

Berger ECElvidge AR: Medulloblastomas and cerebellar sarcomas. A clinical survey. J Neurosurg 20:1391441963Berger EC Elvidge AR: Medulloblastomas and cerebellar sarcomas. A clinical survey. J Neurosurg 20:139–144 1963

3.

Berry MPJenkin RDTKeen CWet al: Radiation treatment for medulloblastoma. A 21-year review. J Neurosurg 55:43511981Berry MP Jenkin RDT Keen CW et al: Radiation treatment for medulloblastoma. A 21-year review. J Neurosurg 55:43–51 1981

4.

Bloom HJGWallace ENKHenk JM: The treatment and prognosis of medulloblastoma in children. A study of 82 certified cases. AJR 105:43621969Bloom HJG Wallace ENK Henk JM: The treatment and prognosis of medulloblastoma in children. A study of 82 certified cases. AJR 105:43–62 1969

5.

Chang CHHousepian EMHerbert C: An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 93:135113591969Chang CH Housepian EM Herbert C: An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 93:1351–1359 1969

6.

Chatty EMEarle KM: Medulloblastoma. A report of 201 cases with emphasis on the relationship of histologic variants to survival. Cancer 28:9779831971Chatty EM Earle KM: Medulloblastoma. A report of 201 cases with emphasis on the relationship of histologic variants to survival. Cancer 28:977–983 1971

7.

Collins VPLoeffler RKTivey H: Observations on growth rates of human tumors. AJR 76:98810001956Collins VP Loeffler RK Tivey H: Observations on growth rates of human tumors. AJR 76:988–1000 1956

8.

Cutler SJEderer F: Maximum utilization of the life table method in analyzing survival. J Chronic Dis 8:6997121958Cutler SJ Ederer F: Maximum utilization of the life table method in analyzing survival. J Chronic Dis 8:699–712 1958

9.

Dawson WB: Growth impairment following radiotherapy in childhood. Clin Radiol 19:2412561968Dawson WB: Growth impairment following radiotherapy in childhood. Clin Radiol 19:241–256 1968

10.

Dell SCohen MKushner M: Period of risk hypothesis after prophylactic radiotherapy. Childs Brain 8:3723811981Dell S Cohen M Kushner M: Period of risk hypothesis after prophylactic radiotherapy. Childs Brain 8:372–381 1981

11.

Deutsch MReigel DH: The value of myelography in the management of childhood medulloblastoma. Cancer 45:219421971980Deutsch M Reigel DH: The value of myelography in the management of childhood medulloblastoma. Cancer 45:2194–2197 1980

12.

Epstein FMurali R: Pediatric posterior fossa tumors: hazards of the “preoperative” shunt. Neurosurgery 3:3483501978Epstein F Murali R: Pediatric posterior fossa tumors: hazards of the “preoperative” shunt. Neurosurgery 3:348–350 1978

13.

Fuks ZGlatstein EMarsa GWet al: Long-term effects of external radiation on the pituitary and thyroid glands. Cancer 37:115211611976Fuks Z Glatstein E Marsa GW et al: Long-term effects of external radiation on the pituitary and thyroid glands. Cancer 37:1152–1161 1976

14.

Gehan EA: A generalized Wilcoxon test for comparing arbitrarily singly-censored samples. Biometrika 52:2032231965Gehan EA: A generalized Wilcoxon test for comparing arbitrarily singly-censored samples. Biometrika 52:203–223 1965

15.

Harisiadis LChang CH: Medulloblastoma in children: a correlation between staging and results of treatment. Int J Radiat Oncol Biol Phys 2:8338411977Harisiadis L Chang CH: Medulloblastoma in children: a correlation between staging and results of treatment. Int J Radiat Oncol Biol Phys 2:833–841 1977

16.

Hirsch JFRenier DCzernichow Pet al: Medulloblastoma in childhood. Survival and functional results. Acta Neurochir 48:1151979Hirsch JF Renier D Czernichow P et al: Medulloblastoma in childhood. Survival and functional results. Acta Neurochir 48:1–15 1979

17.

Hoffman HJHendrick EBHumphreys RP: Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma. J Neurosurg 44:5625661976Hoffman HJ Hendrick EB Humphreys RP: Metastasis via ventriculoperitoneal shunt in patients with medulloblastoma. J Neurosurg 44:562–566 1976

18.

Humphreys RP: Posterior cranial fossa brain tumors in children in Youmans JR(ed): Neurological Surgeryed 2. Philadelphia: WB Saunders1982 Vol 5 pp 27332758 (see p 2741)Humphreys RP: Posterior cranial fossa brain tumors in children in Youmans JR (ed): Neurological Surgery ed 2. Philadelphia: WB Saunders 1982 Vol 5 pp 2733–2758 (see p 2741)

19.

Iacono RPApuzzo MLJDavis RLet al: Multiple meningiomas following radiation therapy for medulloblastoma. Case report. J Neurosurg 55:2822861981Iacono RP Apuzzo MLJ Davis RL et al: Multiple meningiomas following radiation therapy for medulloblastoma. Case report. J Neurosurg 55:282–286 1981

20.

Kessler LADugan PConcannon JP: Systemic metastases of medulloblastoma promoted by shunting. Surg Neurol 3:1471521975Kessler LA Dugan P Concannon JP: Systemic metastases of medulloblastoma promoted by shunting. Surg Neurol 3:147–152 1975

21.

King GASagerman RH: Late recurrence in medulloblastoma. AJR 123:7121975King GA Sagerman RH: Late recurrence in medulloblastoma. AJR 123:7–12 1975

22.

Kleinman GMHochberg FHRichardson EP: Systemic metastases from medulloblastoma: report of two cases and review of the literature. Cancer 48:229623091981Kleinman GM Hochberg FH Richardson EP: Systemic metastases from medulloblastoma: report of two cases and review of the literature. Cancer 48:2296–2309 1981

23.

Koos WTMiller MH: Intracranial Tumors of Infants and Children. Stuttgart: George Thieme1971 pp 205315Koos WT Miller MH: Intracranial Tumors of Infants and Children. Stuttgart: George Thieme 1971 pp 205–315

24.

Landberg TGLindgren MLCavallin-Stahl EKet al: Improvements in the radiotherapy of medulloblastoma, 1946–1975. Cancer 45:6706781980Landberg TG Lindgren ML Cavallin-Stahl EK et al: Improvements in the radiotherapy of medulloblastoma 1946–1975. Cancer 45:670–678 1980

25.

Laurent JPBruce DASchut L: Hemorrhagic brain tumors in pediatric patients. Childs Brain 8:2632701981Laurent JP Bruce DA Schut L: Hemorrhagic brain tumors in pediatric patients. Childs Brain 8:263–270 1981

26.

Matson DD: Neurosurgery of Infancy and Childhooded 2. Springfield, Ill: Charles C Thomas1969 pp 448456Matson DD: Neurosurgery of Infancy and Childhood ed 2. Springfield Ill: Charles C Thomas 1969 pp 448–456

27.

McComb JGDavis RLIsaacs H Jr: Extraneural metastatic medulloblastoma during childhood. Neurosurgery 9:5485511981McComb JG Davis RL Isaacs H Jr: Extraneural metastatic medulloblastoma during childhood. Neurosurgery 9:548–551 1981

28.

McCormick WFUgajin K: Fatal hemorrhage into a medulloblastoma. Case report. J Neurosurg 26:78811967McCormick WF Ugajin K: Fatal hemorrhage into a medulloblastoma. Case report. J Neurosurg 26:78–81 1967

29.

McFarland DRHorwitz HSaenger ELet al: Medulloblastoma—a review of prognosis and survival. Br J Radiol 42:1982141969McFarland DR Horwitz H Saenger EL et al: Medulloblastoma—a review of prognosis and survival. Br J Radiol 42:198–214 1969

30.

McIntosh N: Medulloblastoma—a changing prognosis? Arch Dis Child 54:2002031979McIntosh N: Medulloblastoma—a changing prognosis? Arch Dis Child 54:200–203 1979

31.

Mealey J Jr Hall PV: Medulloblastoma in children. Survival and treatment. J Neurosurg 46:56641977Mealey J Jr Hall PV: Medulloblastoma in children. Survival and treatment. J Neurosurg 46:56–64 1977

32.

Modan BBaidatz DMart Het al: Radiation-induced head and neck tumours. Lancet 1:2772791974Modan B Baidatz D Mart H et al: Radiation-induced head and neck tumours. Lancet 1:277–279 1974

33.

Nelson AA: Metastases of intracranial tumors. Am J Cancer 28:1121936Nelson AA: Metastases of intracranial tumors. Am J Cancer 28:1–12 1936

34.

Neuhauser EBDWittenborg MHBerman CZet al: Irradiation effects of roentgen therapy on the growing spine. Radiology 59:6376501952Neuhauser EBD Wittenborg MH Berman CZ et al: Irradiation effects of roentgen therapy on the growing spine. Radiology 59:637–650 1952

35.

Norris DGBruce DAByrd RLet al: Improved relapse-free survival in medulloblastoma utilizing moder techniques. Neurosurgery 9:6616641981Norris DG Bruce DA Byrd RL et al: Improved relapse-free survival in medulloblastoma utilizing moder techniques. Neurosurgery 9:661–664 1981

36.

Nüchel BAnderson AP: Medulloblastoma. Treatment results. Acta Radiol Oncol Radiat Phys Biol 17:3053111978Nüchel B Anderson AP: Medulloblastoma. Treatment results. Acta Radiol Oncol Radiat Phys Biol 17:305–311 1978

37.

Paterson EFarr RF: Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Acta Radiol 39:3233361953Paterson E Farr RF: Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Acta Radiol 39:323–336 1953

38.

Probert JCParker BRKaplan HS: Growth retardation in children after megavoltage irradiation of the spine. Cancer 32:6346391973Probert JC Parker BR Kaplan HS: Growth retardation in children after megavoltage irradiation of the spine. Cancer 32:634–639 1973

39.

Quest DOBrisman RAntunes JLet al: Period of risk for recurrence in medulloblastoma. J Neurosurg 48:1591631978Quest DO Brisman R Antunes JL et al: Period of risk for recurrence in medulloblastoma. J Neurosurg 48:159–163 1978

40.

Raimondi AJTomita T: The disadvantages of prophylactic whole CNS postoperative radiation therapy for medulloblastoma in Paoletti PButti WGKnerich R(eds): Multidisciplinary Aspects of Brain Tumor Therapy. Amsterdam: Elsevier/North-Holland Biomedical Press1979 pp 209218Raimondi AJ Tomita T: The disadvantages of prophylactic whole CNS postoperative radiation therapy for medulloblastoma in Paoletti P Butti WG Knerich R (eds): Multidisciplinary Aspects of Brain Tumor Therapy. Amsterdam: Elsevier/North-Holland Biomedical Press 1979 pp 209–218

41.

Raimondi AJTomita T: Medulloblastoma in childhood: comparative results of partial and total resection. Childs Brain 5:3103281979Raimondi AJ Tomita T: Medulloblastoma in childhood: comparative results of partial and total resection. Childs Brain 5:310–328 1979

42.

Richards GEWara WMGrumbach MMet al: Delayed onset of hypopituitarism: sequalae of therapeutic irradiation of central nervous system, eye and middle ear tumors. J Pediatr 89:5535591976Richards GE Wara WM Grumbach MM et al: Delayed onset of hypopituitarism: sequalae of therapeutic irradiation of central nervous system eye and middle ear tumors. J Pediatr 89:553–559 1976

43.

Riseborough EJ: Irradiation induced kyphosis. Clin Orthop 128:1011061977Riseborough EJ: Irradiation induced kyphosis. Clin Orthop 128:101–106 1977

44.

Rubinstein LJ: Tumors of the Central Nervous System. Atlas of Tumor Pathology Series 2 Fascicle 6. Washington, DC: Armed Forces Institute of Pathology1972Rubinstein LJ: Tumors of the Central Nervous System. Atlas of Tumor Pathology Series 2 Fascicle 6. Washington DC: Armed Forces Institute of Pathology 1972

45.

Sachs ERubinstein JEArneson AN: Results of roentgen treatment of a series of one hundred and nineteen gliomas. Arch Neurol Psychiatry 35:5976161936Sachs E Rubinstein JE Arneson AN: Results of roentgen treatment of a series of one hundred and nineteen gliomas. Arch Neurol Psychiatry 35:597–616 1936

46.

Schnitzler ERRichards MJSChun RWM: Cerebellar medulloblastoma. An analysis of four cases of extraneural metastasis. Am J Dis Child 132:100410081978Schnitzler ER Richards MJS Chun RWM: Cerebellar medulloblastoma. An analysis of four cases of extraneural metastasis. Am J Dis Child 132:1004–1008 1978

47.

Shalet SMBeardwell CGMorris-Jones PHet al: Pituitary function after treatment of intracranial tumours in children. Lancet 2:1041071975Shalet SM Beardwell CG Morris-Jones PH et al: Pituitary function after treatment of intracranial tumours in children. Lancet 2:104–107 1975

48.

Tan BCKunaratnam N: Hypopituitary dwarfism following radiotherapy for nasopharyngeal carcinoma. Clin Radiol 17:3023041966Tan BC Kunaratnam N: Hypopituitary dwarfism following radiotherapy for nasopharyngeal carcinoma. Clin Radiol 17:302–304 1966

49.

Vaquero JCabezudo JMde Sola RGet al: Intratumoral hemorrhage in posterior fossa tumors after ventricular drainage. Report of two cases. J Neurosurg 54:4064081981Vaquero J Cabezudo JM de Sola RG et al: Intratumoral hemorrhage in posterior fossa tumors after ventricular drainage. Report of two cases. J Neurosurg 54:406–408 1981

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