spontaneous hemorrhage in medulloblastomas - ajnr. · pdf file986 spontaneous hemorrhage in...

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986 Spontaneous Hemorrhage in Medulloblastomas Zelig R. Weinstein 1 - 3 and Edward F. Downey, Jr .1. 2 Spont aneous he morrhage into an intracranial neoplasm is relatively uncommon , usually occurring in metastatic le- sions, glioblastomas , or pituitary adenomas [1 -4]. Hemor- rhage into medullob lastoma is reported to be extremely rare. We present two cases of hemorrh age into medulloblastoma with c omput ed tomogr aphic (CT) findings . Case Reports Case 1 A newborn gir l was de li vered vaginally by vacuum ex traction fo ll owing fetal cardiac deceleration and a pro longed second stage of labor . The baby had a right-sided cephalohematoma and right f ac ial palsy as well as opisthot onos. Nonc ontrast CT demonstrated hemorrhage into enlarged lateral ventricles and a high-d ensity ar ea in the right cerebellopontine angle suggesting an intraparenchymal hematoma (fig. 1 A). CT 4 days after birth was es sentially un- changed, but, beca use of a dec reasing hematocrit, the cerebell ar hematoma was evacuated. The baby continued to deterior at e, and, 10 days aft er birth, CT was repeated. It demonstr ated a high-d ensity mass in th e right cerebell um, larger than before, which e nhanced homogenously after administration of intravenous c ontrast material. The patient rema in ed stable for 3 weeks and then deteriorated further. CT 1 month after birth showed an enlarged enhancing mass co mpr ess in g the fourth ventricle (figs. 1 Band 1 C). Surg ery dem- onstr ated a ri g ht cerebellar hematoma with clusters of medu ll obla s- toma ce ll s within it as we ll as adj ace nt solid areas of medulloblas- toma. The infant died 4 days after surgery of res pir atory arr est resulting from in volvement of the br ain stem by tumor . Case 2 A 9-yea r-old bo y was we ll until 6 months before admission, at which time he developed periodic headac hes, most c ommon in th e morning, loca ted in the occ iput , and reli eved by Tylenol and res t. One day be for e admission, the patient developed nausea, vomiting, and ataxia. Co ntr ast CT de monstr ated a large left cerebell ar mass of both high and low density (fig. 2). (N on c ontr ast scanning was not performed.) Surge ry demonstr ated a large nec rotic medull oblas- toma wi th a ce ntral hemorrhage. The postop erative co ur se was uneventful and radiotherapy was begun. Received Ap ril 28, 1982; accep ted after revision September 15, 1982. Discussion Medulloblastoma is a neoplasm that arises in the neuroe- pithelial roof of the fourth ventricle and is composed of poorly differentiated germinative cells. These neuroepithe- lial cells form the externa l granu lar layer within the cerebellar hemispheres by migrating upward and laterally [5]. There- fore, although most medulloblastomas are midline in the fourth ventricle, they can occur anywhere along this path of migration as far laterally as the cerebellopontine angle. The CT characteristics of medulloblastomas are reported to be typical for the tumor whether midline or lateral, usually demonstrating slightly increased density that enhances uni- formly after administration of contrast material [6]. However, this pattern is not pathognomonic for medulloblastoma, with a similar appearance occurring with ependymomas and cerebellar astrocytomas. Pathologically , medulloblastoma is described as a soft, friable, fairly we ll demarcated neoplasm with hemorrhage, cyst formation , and calcification being uncommon [5]. In fact, hemorrhage into medulloblastoma was thought to be so rare that one author stated: " Hemorrhages do not occur in this tumor. " [7] Other major references do not mention intratumoral hemorrhage in medulloblastoma [5 , 8 , 9]. How- ever, there are eight reported cases of spontaneous hem- orrhage into this tumor [1, 2, 10-13]. In addit ion to these cases of spontaneous hemorrhag e, there are reports of hemorrhage into medulloblastomas after surgery and/ or radiation therapy [14-16]. Although rare in medulloblastom a, the incidence of mas- sive intratumoral hemorrhage in bra in tumors has been reported as 3% -7 .5% [16 , 17]. Zimmerman and Bilaniuk [1] described three CT patterns of intratumoral hemorrhage: (1) solid hematoma, indistinguishable from hematomas from other causes ex cept for contrast enhancement of contig- uous or remote masses or contrast enhancement along the margin of the hematoma ; (2) central hemorrhage into a necrotic tumor or into a less active but solid part of the tumor; and (3) hemorrhagic infarction , from small peripheral to total tumor infarction . Case 1 had a type 1 pattern of The opinions exp ressed in this article are solely those of the authors and should not be co nstrued as th ose of the Department of the Navy or the Department of Defense. , Department of Radiology, National Naval Medicial Center, Bethesda, MD 20814. Address reprint requ ests to E. F. Downey, Jr. 2 Department of Radiology / Nuclear Medicin e, Uniformed Services University of the Health Sciences Medicine Sc hoo l, Beth esd a, MD 208 14. 3 Present add ress: Department of Radiology, Baltimore City Hospital, Baltimore, MD 21 224. AJNR 4:986 - 988 , Juty / August 1983 01 95 -6108 / 83 / 0404-0986 $00.00 © American Roentgen Ray Society

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Page 1: Spontaneous Hemorrhage in Medulloblastomas - ajnr. · PDF file986 Spontaneous Hemorrhage in Medulloblastomas Zelig R. Weinstein1-3 and Edward F. Downey, Jr.1. 2 Spontaneous hemorrhage

986

Spontaneous Hemorrhage in Medulloblastomas Zelig R. Weinstein 1

-3 and Edward F. Downey, Jr .1. 2

Spontaneous hemorrhage into an intracranial neoplasm is relatively uncommon , usually occurring in metastatic le­sions, glioblastomas, or pituitary adenomas [1 -4]. Hemor­rh age into medulloblastoma is reported to be extremely rare . We present two cases of hemorrhage into medulloblastoma with computed tomographic (CT) findings .

Case Reports

Case 1

A newborn girl was delivered vaginally by vac uum extraction following fetal cardiac deceleration and a prolonged second stage of labor . The baby had a right-sided cephalohematoma and right facial palsy as well as opisthotonos. Noncontrast CT demonstrated

hemorrh age into enlarg ed lateral ventric les and a high-density area in the right cerebellopontine angle sugg esting an intraparenc hymal hematoma (fig. 1 A) . CT 4 days after birth was essentially un­changed, but, because of a dec reasing hematocrit, the ce rebellar hematoma was evacuated. Th e baby continu ed to deteriorate, and , 10 days after birth, CT was repeated. It demonstrated a high-density mass in the right cerebellum , larger than before , which enhanced homogenously after administration of intravenous contrast material. The pati ent remained stable for 3 weeks and then deteriorated furth er. CT 1 month after birth showed an enlarged enhanc ing mass compressing the fourth ventric le (figs. 1 Band 1 C). Surgery dem­onstrated a right cerebellar hematom a with c lusters of medu lloblas­toma ce lls within it as well as adjacent solid areas of medulloblas­toma. Th e infant died 4 days after surgery of respiratory arrest resulting from involvement of th e brain stem by tumor.

Case 2

A 9-year-old boy was well until 6 months before admission, at which time he developed periodic headaches, most common in th e morning, located in the occ iput, and relieved by Tylenol and rest. One day before admission , the patient developed nausea , vomiting, and atax ia. Contrast CT demonstrated a larg e left cerebell ar mass of both high and low density (fig. 2). (N on contrast scanning was not performed. ) Surgery demonstrated a large necrotic medullobl as­toma wi th a central hemorrhage. The postoperative course was uneventful and radiotherapy was begun .

Received April 28, 1982; accepted after revision September 15, 1982.

Discussion

Medulloblastoma is a neoplasm that arises in the neuroe­pithelial roof of the fourth ventricle and is composed of poorly differentiated germinative cells. These neuroepithe­lial cells form the external granu lar layer within the cerebellar hemispheres by migrating upward and laterally [5]. There­fore, although most medulloblastomas are midline in the fourth ventric le, they can occur anywhere along this path of migration as far laterally as the cerebellopontine angle. The CT characteristics of medulloblastomas are reported to be typical for the tumor whether midline or lateral, usually demonstrating slightly increased density that enhances uni­formly after administration of contrast material [6]. However, this pattern is not pathognomonic for medulloblastoma, with a similar appearance occurring with ependymomas and cerebellar astrocytomas.

Pathologically , medulloblastoma is described as a soft, friable, fairly well demarcated neoplasm with hemorrhage, cyst formation , and calcification being uncommon [5]. In fact, hemorrhage into medulloblastoma was thought to be so rare that one author stated : " Hemorrhages do not occur in this tumor. " [7] Other major references do not mention intratumoral hemorrhage in medulloblastoma [5 , 8 , 9]. How­ever, there are eight reported cases of spontaneous hem­orrhage into this tumor [1, 2, 10-13]. In addition to these cases of spontaneous hemorrhage, there are reports of hemorrhage into medulloblastomas after surgery and / or radiation therapy [14-16].

Although rare in medulloblastoma, the incidence of mas­sive intratumoral hemorrhage in brain tumors has been reported as 3 %-7 .5 % [16 , 17]. Zimmerman and Bilaniuk [1] described three CT patterns of intratumoral hemorrhage: (1) solid hematoma, indistinguishable from hematomas from other causes except for contrast enhancement of contig­uous or remote masses or contrast enhancement along the margin of the hematoma; (2) central hemorrhage into a necrotic tumor or into a less active but solid part of the tumor; and (3) hemorrhagic infarction , from small peripheral to total tumor infarction . Case 1 had a type 1 pattern of

The opinions expressed in this artic le are so lely those of the authors and should no t be construed as those o f the Department of the Navy or the Department of Defense.

, Department of Rad iology, Nat ional Nava l Med icial Center , Bethesda, MD 208 14 . Address reprint requests to E. F. Downey, Jr. 2 Department of Rad io logy / Nuclear Med ic ine, Uniformed Services University o f the Health Sciences Medic ine Schoo l, Bethesda, MD 20814. 3 Presen t address: Department of Rad iology, Baltimore City Hospital, Baltimore, MD 21224.

AJNR 4 :986- 988, Juty / August 1983 0195- 6 108 / 83 / 0404-0986 $00.00 © Ameri can Roentgen Ray Society

Page 2: Spontaneous Hemorrhage in Medulloblastomas - ajnr. · PDF file986 Spontaneous Hemorrhage in Medulloblastomas Zelig R. Weinstein1-3 and Edward F. Downey, Jr.1. 2 Spontaneous hemorrhage

AJNR:4, Jul. / Aug . 1983 HEMORRHAGE IN MEDULLOBLASTOMAS 987

A

B

c Fig. 1.- Case 1 . A, Noncontrast CT at birth shows increased density in

right cerebellopontine angle with little mass effec l and hydrocephalus. Non­contrasl (B) and contrast (C) scans 1 month later. Further en largement of high-density right cerebellar mass now invo lving midline stru ctures and left cerebellum. Lesion enhances after contrasl administ ration . Fourth vent ri cle is obliterated and hydrocephalus is present. There was " hematoma" evacu­ati on 3 weeks earlier.

intratumoral hemorrhage-a solid clot indistinguishable from hematoma due to other causes except for an atypi cal location and contrast enhancement. Case 2 demonstrated a type 2 pattern-bleeding into a necrotic part of the tumor.

The incidence of brain tumors in the first 2 years of life is surprising ly high , with most of these tumors probably con-

Fig . 2. - Case 2. CT after contrast enhancement. Large mixed densily left cerebellar mass. Fourth venlric le is compressed and shifted. Hydrocephalus is present.

genital and usually large by the time they are detected [1 8-21]. The abso lute number of brain tumors in the first 2 years of life is equal to other pediatric age groups. In one series of 203 pediatric brain tumors detected by CT during a 3 year period , 23 (11 % ) were in infants under 2 years of age [17]. Five of these were medulloblastomas, four of which appeared as solid isodense lesions on noncontrast CT. This appearance of a medulloblastoma as an isodense lesion on non contrast CT would exp lain why only the hemorrhagic part of the tumor was seen on the initial CT scan in case 1.

REFERENCES

1. Zimmerman RA , Bilan iuk LT. Computed tomography 01 acute intratumoral hemorrhage. Radiology 1980; 135: 355- 359

2. Glass B, Abbott KH. Subarachnoid hemorrhage consequent to intracranial tumors. Review 01 the literature and report 01 seven cases. Arch Neurol Psychiatr 1955;73 : 369- 379

3. Globus JH, Sapirstein M. Massive hemorrhage into brain tu­mors. JAMA 1942; 120: 348-352

4. Wright RL, Ojemann RG, Drew JH . Hemorrhage into pituitary adenomata. Arch Neural 1965; 12: 326- 331

5. Rubinstein LJ . Tumors 01 the central nervous system. In : Atlas of tumor pathology, series 2, lasc 6 . Washington DC: Armed Forces Institute 01 Pathology, 1972

6. Zimmerman RA, Bilaniuk L T, Pahlajani H. Spectrum 01 medul­loblastomas demonstrated by computed tomography. Radiol­ogy 1978;126:137-141

7. Zulch KJ . Brain tumors , their biology and pathology. (Ameri can ed ition translated by Rothballer AB, Olozewsk i J.) New York :

Springer, 1957 : 1 31 8. Chatty EM , Earle KM . Medu lloblastoma. A report 01 201 cases

with emphasis on the rel ationship 01 hi stologic variants to surviva l. Cancer 1971 ;28 : 977 -983

9. Russell OS, Ru binstein LJ . Pathology of tumors of the nervous system , 2d ed. Baltimore: Williams & Wilkins, 1963

10. Carieri G, Cazzato G. On a case 01 hemorrhag ic medulloblas­tom a 01 the cerebell um (anatoma-c lin ica l contribution) . G Psi­chia tr Neuropatol 1960;88: 871 - 884

11 . McCormick WF, Ugaj in K. Fatal hemorrhage into a medu llo­blastoma. Case report. J Neurasurg 1967;26:78- 81

12. Roosen K, Reinhard t V, Liesegang J. Die spontane Subarach­noidalblutung aus Symptomlosen inlratentoriell en Tumoren. Neurachirurgia (Stuttg) 1980;23: 1-6

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988 WEINSTEIN AND DOWNEY AJNR:4 , Jul. / Aug . 1983

13. Zee CS, Segall HD, Miller C, et al. Less common CT features of medulloblastoma. Radiology 1982; 144 : 9 7 -102

14. Vaquero J, Cabezullo JM , DeSola RG, Nombela L. Intratumoral hemorrhage in posterior fossa tumors after ventricular drain­age. J Neurosurg 1981 ;54 : 406-408

15. Berry MP, Jenkin ROT, Keen CW, Nair BD, Simpson WJ. Radiation treatment for medulloblastoma. A 2 1 year review . J Neurosurg 1981 ;55: 43- 51

16 . Old berg E. Hemorrhage in gliomas. A review of eight hundred and th irty-two verifi ed cases of glioma. Arch Neuro l Psychiatr 1953;30 : 1 061-1 071

17. Courville CB. Pathology of the central nervous system. Moun-

tain View , CA: Pacific Press, 1937 : 67 -69 18. Tadmor R, Harwood-Nash DCF, Savoiardo M, et al. Brain

tumors in th e first two years of life: CT diagnosis. AJNR 1980; 1 : 411 -41 7

19. Harwood-Nash DC, Fitz CR. Neuroradiology in in fa nts and children, vol 2 . St Louis: Mosby, 1976

20 . Koos WT, Miller MH . lntracranial tumors of infants and children.

St Louis: Mosby, 1971 21. Taboada 0 , Froufe A, Alonso A, Alvarey J, Muro 0 , Vila M .

Congenital medulloblastoma. Report of two cases. Pediatr Radio/1980; 9: 5-10