Final Diagnosis -- Congenital gemistocytic astrocytoma

DIAGNOSIS:

CONGENITAL GEMISTOCYTIC ASTROCYTOMA

DISCUSSION:

Intracranial congenital tumors (ICT) are rare. They comprise only about 0.5-1.5% of those detected during childhood (1). Arnstein et al (2) first reviewed brain tumors of the newborn in 1951, and they defined neonatal brain tumors as those that developed up to 60 days after birth. Since then, many authors reported such cases according to this definition (1 - 4).

Dystocia secondary to the increased head size often results in cesarian sections in these cases. The infants may show vomiting, seizures, and irritability. ITC are most commonly supratentorial, as seen in this case.

The most common ICT are teratomas, followed by medulloblastomas, astrocytomas, choroid plexus papillomas, ependymomas and rarely ependymoblastomas.

The prognosis is poor in most ICT due to the large size of the tumor, especially teratomas, as well as the poor general conditions of the baby at presentation. Histologic malignancy is also assumed to be another complicating factor (1, 4). Our case had a large size and the histologic diagnosis was gemistocytic astrocytoma (grade 2: WHO). Gemistocytic astrocytomas are particularly prone to progress to anaplastic astrocytoma and even glioblastoma (5), although such changes were not detected in our case. Gemistocytic astrocytomas account for 9 to 24% of all astrocytomas (6, 7) and affect mainly adult patients. To date this seems to be the first report of congenital gemistocytic astrocytoma.

ACKNOWLEDGMENTS:
Lucy B. Rorke, MD, The Children s Hospital of Philadelphia, for her expertise contribution.

REFERENCES:

1. Wakai S, Arai T, Nagai M (1984) Congenital Brain Tumors. Surg Neurol 21: 597-609
2. Arnstein LH, Boldrey UR, Naffziger HC (1951) A case report and survey of brain tumors during the neonatal period. J Neurosurg 8: 315-319
3. Balajthy B (1965) Congenital hemispherial glioma in a premature female twin. Acta Neurophatol 4: 312-316
4. Rothman SM, Nelson JS, DeVivo DC, Coxe WS (1979) Congenital astrocytoma presenting with intracerebral hematoma. J Neurosurg 51: 237-239
5. Schiffer D, Chio A, Giordana MT, Leone M, Soffietti R (1988) Prognostic value of histologic factors in adult cerebral astrocytoma. Cancer 61: 1386-1393
6. Krouwer HGJ, Davis RL, Silver P, Prados M (1991) Gemistocytic astrocytomas: a reappraisal. J Neurosurg 74: 399-406
7. Shaw EG, Daumas DC, Scheithauer BW Radiation therapy in the management of low-grade supratentorial astrocytomas. J Neurosurg 70: 853-861

Contributed by L. F. Bleggi-Torres, M.D., PhD, MIAC, L. Z. B. Pope, M.D., A. Koerbel, M.D., C. V. Grande, M.D., J. C. Araújo, M.D.