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  • Author or Editor: Venita Jay x
  • By Author: Becker, Laurence E. x
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Robert J. B. Macaulay, Venita Jay, Harold J. Hoffman and Laurence E. Becker

✓ Pleomorphic xanthoastrocytoma is a recently characterized neoplasm with a favorable prognosis despite aggressive histological features. The authors report a case of pleomorphic xanthoastrocytoma that recurred 4 years after complete gross resection. The original tumor exhibited histological features characteristic of this neoplasm, but up to 4 mitoses/10 high-power fields were present focally. The recurrent tumor contained small foci of classical pleomorphic xanthoastrocytoma, but consisted predominantly of glioblastoma multiforme. Transitional zones contained nests of glial fibrillary acidic protein (GFAP)-immunopositive cells surrounded by delicate collagenous and reticulin-rich septa. Electron microscopy of the transitional zone showed continuous basal lamina investing cells containing bundles of intermediate filaments. These were GFAP-positive by immunogold electron microscopy, confirming the astrocytic nature of pleomorphic xanthoastrocytoma. This example illustrates the capacity of this tumor to evolve into glioblastoma. The indolent clinical behavior of most pleomorphic xanthoastrocytomas is evident from a literature review, which confirms the prolonged survival of many patients after onset of symptoms. Completeness of excision, subjectively assessed at surgery, did not influence the risk of recurrence or survival up to 10 years after initial resection. Postoperative radiotherapy did not improve survival, but may reduce the probability of recurrence; more studies are needed to corroborate this finding.

The data compiled herein support the designation of pleomorphic xanthoastrocytoma as a distinct astrocytic neoplasm with a favorable prognosis. An increased mitotic rate has not previously been correlated with a worse outcome, and should not be used to exclude this diagnosis. However, anaplastic transformation of pleomorphic xanthoastrocytoma confers a much worse prognosis, and this case suggests that increased mitotic activity may be a negative prognostic indicator since it may herald subsequent anaplastic transformation.

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Abhaya V. Kulkarni, Laurence E. Becker, Venita Jay, Derek C. Armstrong and James M. Drake

✓ Primary cerebellar glioblastomas multiforme are exceedingly rare in children. The authors therefore retrospectively characterized the clinical behavior and pathological features of these tumors. A review of the database at the Hospital for Sick Children, Toronto, Canada revealed four patients with cerebellar tumors that displayed significant pleomorphism, hypercellularity, mitoses, and necrosis with pseudopalisading. The authors performed a detailed clinical, radiological, histological, and immunohistochemical analysis of the tumors in these four children (three boys and one girl; average age at presentation 7 years; range 21 months–15 years). Magnetic resonance imaging and computerized tomography most commonly revealed a large lesion with minimal edema, inhomogeneous contrast enhancement, and a discrete border. Tumor resection was subtotal in one patient and gross total in three patients. Immunostaining of the tumor cells with antisera to glial fibrillary acidic protein and vimentin was positive in varying degrees. Initial adjuvant therapy consisted of local radiation only (one patient), chemotherapy only (one patient), and radiation and chemotherapy (one patient). One patient received no adjuvant therapy. Tumor recurrence was documented in all patients: two local recurrences (at 3.5 and 7 months), one spinal recurrence (at 14 months), and one local recurrence with ventricular and spinal spread (at 8 months). Ultimately, three of the four patients developed leptomeningeal tumor spread. Patient follow up ranged from 8 to 17 months (mean 12.5 months). Three patients were dead at last follow up with a mean survival of 15 months.

The prognosis for patients with cerebellar glioblastomas is extremely poor, and the tumor has a tendency for cerebrospinal fluid dissemination. The optimal management of patients harboring of these difficult-to-treat tumors, including the role of craniospinal radiation and chemotherapy, has not yet been achieved.

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Pathology of temporal lobectomy for refractory seizures in children

Review of 20 cases including some unique malformative lesions

Venita Jay, Laurence E. Becker, Hiroshi Otsubo, Paul A. Hwang, Harold J. Hoffman and Derek Harwood-Nash

✓ Significant pathological abnormalities were encountered in a series of 20 temporal lobectomies in children with intractable complex partial seizures. In particular, “dual pathology” (mesial temporal sclerosis with other lesions) was found rather than mesial temporal sclerosis as the only lesion. Unusual pathological findings included capillary penetration of neurons in a neuronal heterotopia in one patient, and foci of extensive cortical disorganization in some cases of mixed tumors and gangliogliomas. A high proportion of neuronal migration disorders was also seen with overlapping pathological features between cortical dysplasia and tuberous sclerosis. In this correlative clinical, radiological, electroencephalographic, and pathological study, some of the pathological lesions in children did not fit the classical categories of neoplasia and malformation and transitional forms were rarely encountered.

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Venita Jay, Laurence E. Becker, Hiroshi Otsubo, Paul Hwang, Harold J. Hoffman and Derek C. Armstrong

✓ Unusual pathological findings were encountered in a temporal lobectomy specimen from a 9-year-old boy with intractable complex partial seizures. Magnetic resonance imaging revealed an enlarged left temporal lobe, with diffuse high signal intensity over the cortex and poor gray-white differentiation on T2-weighted imaging; single-photon emission computerized tomography showed decreased blood flow. Active epileptiform discharges were identified in the left temporal lobe with focal slow waves and generalized epileptiform paroxysms. Pathologically, the cortex revealed changes of focal cortical dysplasia with extensive disorganization of neuronal morphology, layering, and orientation as well as focal polymicrogyria. The cortical-white matter junction was indistinct with extensive neuronal heterotopias in the white matter. Large pale balloon cells akin to those seen in tuberous sclerosis were found scattered within the cortex and white matter. The most striking finding was that of a heterotopic nodule in the white matter, which revealed abnormal neurons with penetration of cell bodies by capillaries. Ultrastructurally, there were no degenerative changes in these neurons, and this unusual phenomenon is attributed to a developmental disturbance affecting neuronal, glial, and vascular elements.