Consecutive cerebellar and cerebral astrocytomas.MG Bhatjiwale, DD Muzumdar, AA Goel, TT Patankar
Department of Neurosurgery & Neuroradiology, KEM Hospital, Parel, Mumbai.
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 0009715306
Source of Support: None, Conflict of Interest: None
An unusual astrocytoma occurring in different anatomical compartments of the brain is reported. The child was operated upon for a cerebellar astrocytoma when he was eight-and-half years old. Seven years later, he was operated for a cerebral astrocytoma. The growth of the similar tumours at these two sites is analysed and literature on the subject is reviewed. The concept of multicentricity is emphasized.
Keywords: Astrocytoma, radiography,surgery,Brain Neoplasms, radiography,surgery,Case Report, Cerebellar Neoplasms, radiography,surgery,Cerebral Cortex, Child, Human, Male, Neoplasms, Second Primary, radiography,surgery,Tomography, X-Ray Computed,
Although the simultaneous occurrence of two primary tumours of either the same or different types is recognised but not common, the consecutive development of brain tumours after a long lime interval has rarely been recorded. Our report describes such a tumour genesis. The tumours demonstrate both spatial and chronological multicentricity. This is found to be exceptional in well-differentiated astrocytomas. The tumour in our eight-year-old patient was such a well-differentiated, extremely benign cerebellar astrocytoma.
A eight-and-half year old male child presented in March '89 with complaints of occipital headaches, vomiting and imbalance whilst walking for one and half months. On examination he had early papilledema and mild left cerebellar signs. A CT scan done showed a large cystic left cerebellar tumour with an isodense enhancing mural nodule in the posterolateral part of the cyst. [Figure - 1]
There was moderate hydrocephalus. Via a lateral suboccipital craniectomy excision of the solid component and evacuation of the cyst, both situated well within the cerebellar parenchyma, was carried out. The histological diagnosis was cerebellar astrocytoma. The patient showed complete recovery postoperatively. He subsequently completed external radiation with Cobalt-80 rays to the whole brain in the dose of 5100 Gy in 17 fractions over 39 days. He also completed a course of adjuvant chemotherapy. He was given 6 cycles of chemotherapy over one year period comprising of Inj. Vincristine 1.2 mg/ M2 and Capsule CCNU 100 mg/m2. He was asymptomatic apart from poor scholastic performance till 9.8.96. He then presented with complaints of headaches, vomiting and insidious onset progressive weakness of the left limbs over eight days. On examination, he had early papilledema and left spastic hemiparesis. CT scan on 10.8.96 showed a right fronto-parietal cystic lesion with an irregular thick wall. Another isodense oval lesion with peripheral enhancement was seen just posterior to the first lesion. [Figure - 2]
There was lesional edema with mass effect. No tumour was found in the posterior fossa. A frontoparietal craniotomy with total excision of the lesions, which were located beneath the pia mater was achieved. The histopathology of the tumour revealed a Grade 3 astrocytoma. He showed satisfactory recovery after surgery and was advised repetition of cranial radiotherapy and adjuvant chemotherapy.
Multiple gliomas were first discussed by Gowers in 1896 and this has been the subject of many subsequent reviews,,,. Multiple primary tumours are not rare, and although Courville, felt that almost 9% of all gliomas were multiple, he stated that he could find no instance in which a cerebellar and cerebral glioma were identified together. Zulch recorded 2 cases with both cerebral and cerebellar gliomas. Rusell and Rubinstein reported another similar case in a man aged 26, from whom a cerebellar astrocytoma had been removed 7 years before death and who, at necropsy, was found to have a malignant astrocytoma of the frontal lobe. No recurrence of the growth was found at the site of the scar in the cerebellum either macroscopically or microscopically.
A clear distinction must be drawn from dissemination or metastasis of a glial tumour via cerebrospinal fluid pathways, direct extension across commissures, or by satellitosis, and the truly multicentric tumours. The latter are those that arise at some distance from the other and in which no evidence can be found for dissemination by any of the pathways described above. All tumours in our case were considered primary tumours because both cerebellar and cerebral tumours were located beneath the pia mater. Multicentric tumours may be of different cell types. This was seen in our case. There is a variable interval between the development of clinical evidence of one tumour and that of the other, Bassoe, Pfeifer. Stumpf have reported cases of multiple brain tumours but their descriptions fail to fulfil the above criteria in as much as implantation' and subarachnoid seeding cannot be ruled out. Some reports lack histological confirmation,,,,. Cases of phakomatosis with multicentric gliomas in cerebrum and cerebellum have been cited,,,,,,,,,,,, but these probably represent a facet of the underlying disease and could be excluded from this group.
On our literature review, a total of only ten cases satisfying both gross and microscopic criteria for true multicentricity in cerebrum and cerebellum were identified,,. Furthermore, multicentric gliomas were found to be rare in a child,,,.
Multicentricity is most frequently seen in glioblastomas. Well-differentiated astrocytic or mixed gliomas are only exceptionally, capable of a similar development. The glioma in the cerebellum in our case was of low histological grade.
Two other possibilities which need to be considered in the discussion on cases of multiple tumours are secondary tumours caused by CSF dissemination and tumours induced by radiation.
Many workers in the field have noted that extension of tumour cells into the subarachnoid space is common in patients with cerebellar pilocytic astrocytomas but is not necessarily associated with aggressive behaviour or leptomeningeal seeding,,,.
Radiation has been implicated in the development of intracranial neoplasms for over 40 years. There are numerous reports in the literature of various intracranial tumours in patients following radiation therapy, but conclusive proof of a causative link has been difficult. There are no definitive biochemical abnormalities linked to radiation-induced tumours, and there are no distinguishing morphologic features or cytogenetic alterations. Therefore, the link between radiation and intracranial tumours remains controversial.
Of late, information obtainable in the living patient by computerised tomography and magnetic imaging has intensified the importance of multitocality in gliomas. The problem is also relevant to the origin of tumour recurrence after apparently total resection of the growth.
We sincerely thank Dr. Sunil Pandya for his encouragement and support in preparing this report Dr. Priyam Bhatjiwale proof-read the manuscript.
[Figure - 1], [Figure - 2]