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 »  Introduction
 »  Case report
 »  Discussion
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Year : 1999  |  Volume : 47  |  Issue : 1  |  Page : 55-7

Medullomyoblastoma : A case report.


Department of Neurological Sciences and Pathology, Christian Medical College and Hospital, Vellore, Andhra Pradesh, India.

Correspondence Address:
Department of Neurological Sciences and Pathology, Christian Medical College and Hospital, Vellore, Andhra Pradesh, India.

  »  Abstract

Medullomyoblastoma is a rare tumour seen in childhood. We report a medullomyoblastoma occurring in the cerebellar vermis of a 4 year old boy. The light microscopic features, immunohistochemistry and histogenesis are described.

How to cite this article:
ArunKumar M J, Chacko G, Chandi S M, Chandy M J. Medullomyoblastoma : A case report. Neurol India 1999;47:55


How to cite this URL:
ArunKumar M J, Chacko G, Chandi S M, Chandy M J. Medullomyoblastoma : A case report. Neurol India [serial online] 1999 [cited 2019 Nov 13];47:55. Available from: http://www.neurologyindia.com/text.asp?1999/47/1/55/1659




   »   Introduction Top


Medullomyoblastoma is a primitive neuroepithelial tumour which contains muscle fibers and is regarded as a variant of malignant teratoma, or a `teratoid' tumour.[1] This tumour was first described by Marinesco and Goldstein in 1933, and since then there have been only 30 additional reports in the literature including three cases from India.[2],[3],[4],[5],[6],[7] In this report, we describe the clinicopathological features and treatment of a medullomyoblastoma arising from the cerebellar vermis.


   »   Case report Top


A 4 year old boy presented with intermittent bifrontal headache for one month and urinary incontinence for 2 weeks. He had two episodes of vomiting with deteriorating sensorium on the day of admission. On examination, he had a Glasgow coma score of 7/15 [E2 M3 V2], gross bilateral papilloedema and diminished pupillary reaction. There was no cranial nerve deficit. He had bilateral pyramidal signs with no lateralising deficit. The skull and spine were normal. Computed tomography showed a 5 x 4 cm mixed density, contrast enhancing mass in the vermis with shift of the partially effaced fourth ventricle anteriorly and to the left [Figure 1]. There was gross dilatation of the third and lateral ventricles. An emergency right frontal twist drill craniostomy and ventricular tap was done. The patient was then taken up for a right ventriculo peritoneal shunt, which improved the sensorium. He underwent a midline suboccipital craniectomy. The vermis appeared widened, greyish, moderately vascular and partly suckable tumour tissue with a small cystic area superiorly was encountered below the surface. There was evidence of intratumoural haemorrhage with tonsillar herniation which explained the rapid deterioration of his sensorium prior to admission. A near total excision of the tumour was done leaving behind a small portion of the tumour at the junction between the floor of the fourth ventricle and middle cerebellar peduncle, lateral to the stria medullaris on the left side. The rest of the floor of the fourth ventricle was normal with free flow of cerebrospinal fluid from the aqueduct.

Histopathological examination : Microscopic examination of the soft white fragments of the tissue submitted showed a cellular tumour composed of sheets of small cells with oval to elongated dark staining nuclei and scanty eosinophilic cytoplasm exhibiting increased mitotic activity [Figure 2a]. In foci, the tumour contained polygonal to elongated cells with eccentrically placed nuclei and bright eosinophilic cytoplasm resembling myoblasts [Figure 2b]. In areas the tumour had a desmoplastic appearance and exhibited a nodular pattern with abundant reticulin in the internodular areas. The blood vessels in the tumour were unremarkable. On immunohistochemistry by the avidin peroxidase method, the tumour cells were positive for neuron specific enolase and negative for GFAP. The cells resembling myoblasts were positive for actin and desmin [Figure 2c].

Clinical course and follow up : In the immediate post operative period the child developed shunt infection and hence the conduit had to be removed. At the time of discharge he was fully conscious but had residual lower cranial nerve palsy and bilateral cerebellar signs and required feeding through a ryles tube. He had a full course of radiation therapy and showed marked improvement in his neurological status with normal sensorium when he left the hospital. 14 months following surgery, he was asymptomatic and had mild residual left sixth nerve paresis and gait ataxia. CT scan did not show evidence of any tumour or hydrocephalus.


   »   Discussion Top


Medullomyoblastoma is a primitive neuroectodermal tumour of the cerebellum with vermis being the preferential site of origin and a male/female ratio of 3:8. Though it has been accepted that it is a distinct variant of medulloblastoma, its histogenesis has been a subject of controversy.[7] Ingraham and Bailey[8] and Misugi and Liss[9] proposed that this tumour be considered a `teratoma' consisting of a neuroectodermal element and a mesenchymal rhabdomyosarcoma component.[8],[9] Banerjee and Kak[10] favoured the teratoid origin for this tumour, as the case of medullomyoblastoma reported by them was admixed with an epithelial component resembling medulloepithelioma. In another study of three cases which had features of a medullomyoblastoma, one had distinct teratomatous area supporting a possible teratomatous origin for the tumour.[4]

A major objection to the `teratoid origin' of medullomyoblastoma was the absence of endodermal elements, and in most cases the mesenchymal component was restricted to the presence of muscle fibres. Lewis proposed that the myoblastic component of the tumour arose from pluripotent mesenchymal cells derived from the neural crest surrounding blood vessels in the brain and meninges.[11] This was supported by Kartha et al who found occasional striated muscle cells in the leptomeninges of the ventral pons in a still-born preterm foetus.[12] The presence of melanin containing cells in some cases of medulloblastoma has been used as support for its ectomesenchymal or neural crest origin. Some authors suggested that the location of these tumours in the cerebellar vermis can be linked to the primitive germinal cells found in early life occupying the posterior lip of the roof of the fourth ventricle.[1] Marinesco and Goldstein suggested that the striated muscle in these tumours arose from metaplastic vascular smooth muscle cells.[2] In 1979, Walter and Brucher postulated that the myoblasts originated from `neoplastic transformation of endothelial cells'. This postulate found support since the muscle fibres in medullomyoblastoma have a tendency to be situated around the blood vessels.[3] The presence of mitotic figures in endothelial cells was considered by them to lend further support to their theory.

A fourth hypothesis on the pathogenesis of medull cells by Lennon and Patterson.[13] They postulated that primitive neuroepithelial cells can differentiate into rhabdomyoblastic or melanocytic lines.[13] Of late, medullomyoblastoma, with a hamartomatous component, has been reported supporting the view that these tumours could be derived from dysplastic elements of the adjacent cerebellum.[7],[14]

In our case, there was neither any evidence of endodermal element nor the mitotic figures were seen in endothelial cells. We therefore do not favour a `teratoid origin' or an endothelial cell origin for this tumour. We believe that the medullomyoblastoma represents either a histologic variant of a medulloblastoma or is a mixed neuroepithelial mesenchymal neoplasm. However, based on immunocytochemical studies, it has been widely accepted that the myogenic component represents a distinct differentiation pathway inistogenesis of medullomyo- blastoma.[7]

Medullomyoblastomas are rapidly progressive tumours seen in young children with clinical symptoms ranging from a few weeks to months.[6] The recommended treatment of this tumour include radical surgery and craniospinal irradiation.[1],[4],[11],[15] However, the survival period for medullomyoblastoma is very short despite surgery and radiation and has ranged from 4 days to one year.[6] Nevertheless, radiation does seem to prolong the period of survival. The case reported by Lewis, who had undergone radiation, was alive even after 4 years of follow up.[11] Our patient underwent near total excision of the tumour with radiation therapy and was alive and well on follow up at 14 months.

 

  »   References Top

1.Russell DS, Rubinstein LJ : Pathology of Tumours of the Nervous system, Edward Arnold, Great Britain 1989; 687-689.   Back to cited text no. 1    
2.Marinesco G, Goldstein M : Sur une forme anatomique, non encore decrite, de medulloblastome, Medullo-myoblastome. Annals of Anatomy and Pathology 1933; 10 : 513-525.   Back to cited text no. 2    
3.Walter GF, Brucher JM : Ultrastructural study of medullomyoblastoma. Acta Neuropathol (Berl) 1979; 48 : 211-214.   Back to cited text no. 3    
4.Chowdhury C, Roy S, Mahapatra AK et al : Medullomyoblastoma : A teratoma. Cancer 1985; 55 : 1495-1500.   Back to cited text no. 4    
5.Lata M, Mahapatra AK, Sarkar C et al : Medullomyoblastoma - A case report. Indian J Cancer 1989; 26 : 240-246.   Back to cited text no. 5    
6.Rao C, Friedlander ME, Klein E et al : Medullomyoblastoma in an adult. Cancer 1990; 65 : 157-163.   Back to cited text no. 6    
7.Lantos PL, Vandenberg SR, Kleihues P : Tumours of the nervous system. In : Greenfield's Neuropathology. Graham DI, Lantos PL Eds. Great Britain : 1997; 706-707.   Back to cited text no. 7    
8.Ingraham FD, Bailey OT : Cystic teratomas and teratoid tumours of the central nervous system in infancy and childhood. J Neuro Surg 1946; 3 : 511-532.   Back to cited text no. 8    
9.Misugi K, Liss L : Medulloblastoma with cross striated muscle : Afine structural study. Cancer 1970; 25 : 1279-1285.   Back to cited text no. 9    
10.Banerjee AK, Kak VK : Teratoid tumours of the cerebellum. J Pathol 1973; 11 : 285-287.   Back to cited text no. 10    
11.Lewis AJ : Medulloblastoma with striated muscle fibers : Case report. J Neurosurg 1973; 38 : 642-646.   Back to cited text no. 11    
12.Kartha C, Shankar SK, Roy S : Skeletal muscle in the leptomeninges. Neurol India 1980; 28 : 74-76.   Back to cited text no. 12    
13.Lennon VA, Patterson S : Neuroectoderm markers retained in phenotypical skeletal muscle cells arising from a glia cell line. Nature 1979; 281 : 586-588.   Back to cited text no. 13    
14.Holl T, Kleihues P, Yasargil MG et al : Cerebellar medullomyoblastoma with advanced neuronal differentiation and hamartomatous component. Acta Neuropathol (Berl) 1991; 82 : 408- 413.   Back to cited text no. 14    
15.Smith TW, Davidson RI : Medullomyoblastoma : A histologic, immunohistochemical and ultrastructural study. Cancer 1984; 54 : 323-332.   Back to cited text no. 15    

 

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