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Embryonal central neuroepithelial tumors and their differentiating potential

A cytogenetic view of a complex neuro-oncological problem

Lucien J. Rubinstein

the hit, and most of them are already committed to a limited line of differentiation in their progeny, then the window of neoplastic vulnerability will be correspondingly narrow. Viewed in the context of the currently accepted stages of normal neurocytogenesis, the relative width of that window determines the great prevalence of gliomas and the striking rarity of neuronal neoplasms among central neuroepithelial tumors in general. Cellular Displacement in Neuro-Oncogenesis If we accept the current view that the phenotypic expression of malignant

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Shunji Nishio, Takatoshi Tashima, Iwao Takeshita and Masashi Fukui

on light microscopy resembled oligodendroglioma and which showed the ultrastructural characteristics of neuronal neoplasms. They coined the term “central neurocytoma” for these tumors. This neoplasm has not become a distinct clinical and pathological entity because of its rare occurrence and its rather recent recognition. In this paper, the cases of six patients with so-called “intraventricular oligodendroglioma” are reviewed, and the ultrastructural resemblance of these tumors to central neurocytoma is confirmed. Clinical Material and Methods Six patients

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Hypothalamic hamartoma

Report of two cases

Shunji Nishio, Shigeru Fujiwara, Yasutaka Aiko, Iwao Takeshita and Masashi Fukui

neuronal populations based on neuropeptide patterns suggests that some neurons in our cases have functional similarities to the hypothalamic neurons, and may also indicate the hamartomatous nature of our lesion. Some neuronal neoplasms, including ganglioglioma, have also been shown to have neuropeptides, which are characteristic of the hypothalamus. 19 In addition, some neuronal elements in our hamartoma showed positive immunohistochemical reaction for a 210-kD neurofilament protein, a positivity found only in some processes of Purkinje cells in the normal cerebellum

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Isidro Ferrer, Fabián Isamat, Luis López-Obarrio, Gerardo Conesa, Jordi Rimbau, Soledad Alcántara, Isabel Español and María José Zújar

of two cases. Neurosurg Rev 3: 151–158, 1980 21. Giangaspero F , Burger PC , Budwit DA , et al : Regulatory peptides in neuronal neoplasms of the central nervous system. Clin Neuropathol 4 : 111 – 115 , 1985 Giangaspero F, Burger PC, Budwit DA, et al: Regulatory peptides in neuronal neoplasms of the central nervous system. Clin Neuropathol 4: 111–115, 1985 22. Hendry SH , Jones EG , Emson PC , et al : Two classes of cortical GABA neurons defined by differential calcium binding

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Douglas C. Miller, Frederick F. Lang and Fred J. Epstein

Histopathological features that suggest the diagnosis of ganglioglioma require, in most cases, confirmation by special stains to distinguish these tumors from other gliomas. For this purpose, immunostaining for synaptophysin, which has previously been shown to selectively label the cell surface of neoplastic ganglion cells, was used to retrospectively examine glioma tumor specimens. Sixty-three cases of ganglioglioma were identified. The files of the Division of Neuropathology of New York University Medical Center contained 45 tumors that had been diagnosed as ganglioglioma, of which 42 were verified by synaptophysin; three cases were reclassified, two as astrocytomas and one as a gangliocytic paraganglioma. Thus, a tumor identified as ganglioglioma based on other criteria was likely to be a ganglioglioma. The other 21 cases of gangliogliomas were originally diagnosed as astrocytoma or mixed glioma, but were shown by synaptophysin staining to be gangliogliomas. In some cases the ultimate diagnosis was obtained after radical surgery provided relatively abundant amounts of tissue, thereby limiting sampling errors, in contrast to the biopsies from which the original diagnoses were made.

Histopathological review of these cases demonstrated that four features represent important clues to the correct diagnosis: 1) clusters of large cells potentially representing neurons (without such cells the tumor cannot be classified as a ganglioglioma); 2) no perineuronal clustering of the glial cells around the alleged neoplastic neurons; 3) fibrosis (desmoplasia); and 4) calcification. Binucleate neurons, previously suggested to be common in gangliogliomas, were not frequently found in this series, and lymphocytic infiltrates, while common, are so often found in other tumors that they gave no specific hint that any single neoplasm was a ganglioglioma. The glial elements were astrocytic in all cases, except that one tumor also had oligodendroglial and ependymal patterns. Four tumors also had small mature neurons, as seen in neurocytomas. Cells from one tumor were successfully grown in short-term tissue culture; the culture contained large dividing neurons with synaptophysin immunoreactivity as well as smaller dividing cells, demonstrating that the neuronal cells are a proliferating element in gangliogliomas.

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Central nervous system gangliogliomas

Part 2: Clinical outcome

Frederick F. Lang, Fred J. Epstein, Joseph Ransohoff, Jeffrey C. Allen, Jeffrey Wisoff, I. Richmond Abbott and Douglas C. Miller

The records of 58 patients with gangliogliomas surgically treated between January 1, 1980, and June 30, 1990, were retrospectively reviewed in order to determine long-term survival, event-free survival, and functional outcome resulting after radical resection and to assess the impact of histological grading on outcome. Tumors were located in the cerebral hemisphere in 19 cases, the spinal cord in 30, and the brain stem in nine. Forty-four patients had gross total resection and 14 had radical subtotal resection. Only six patients underwent postoperative irradiation or chemotherapy and, therefore, the outcome was generally related to surgery alone. Of the 58 gangliogliomas, 40 were classified as histological grade I, 16 were grade II, and two were grade III. The median follow-up period was 56 months. There were no operative deaths, and the operative morbidity rate was 5%, 37%, and 33% for cerebral hemisphere, spinal cord, and brain-stem gangliogliomas, respectively. The 5-year actuarial survival rates for cerebral hemisphere, spinal cord, and brain-stem gangliogliomas were 93%, 84%, and 73%, respectively (p = 0.7). The event-free survival rate at 5 years was 95% for cerebral hemisphere gangliogliomas and 36% for spinal cord gangliogliomas (p < 0.05); for brain-stem gangliogliomas the event-free survival rate at 3 years was 53% (p < 0.05). Neurological function at recent follow-up evaluation was stable or improved in 81% of patients. Multivariate analysis (Cox linear regression) revealed tumor location to be the only variable predictive of outcome, with spinal cord and brain-stem gangliogliomas having a 3.5- and 5-fold increased relative risk of recurrence, respectively, compared to cerebral hemisphere gangliogliomas. Histological grade was not predictive of outcome, although in each location there was a trend for higher-grade tumors to have a shorter time to recurrence. It is concluded that radical surgery leads to long-term survival of patients with gangliogliomas, regardless of location, and adjuvant therapy can probably be reserved for special cases.

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Stephen B. Tatter, Lawrence F. Borges and David N. Louis

✓ Central neurocytoma is a neuronal neoplasm that occurs supratentorially in the lateral or third ventricles. The authors report the clinical, neuroradiological, and neuropathological features of two neurocytomas arising in the spinal cord of two men, aged 65 and 49 years. The patients presented with progressive neurological deficits referable to the cervical spinal cord. Magnetic resonance imaging revealed isodense intramedullary spinal cord tumors at the C3–4 level. Both tumors were initially misdiagnosed as gliomas. In Case 1 the correct diagnosis was made after electron microscopy revealed neuronal features. Immunostaining in Case 2 revealed that tumor cells were positive for synaptophysin and negative for glial fibrillary acidic protein, strongly indicating a neuronal tumor. It is suggested that this spinal cord neoplasm be included under the designation “central neurocytoma.”

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Boleslaw Lach, Peter Rippstein, Brien G. Benoit and William Staines

I ntrasellar neuronal neoplasms involving the pituitary gland are very uncommon. Li, et al. , 23 identified only six tumors with a ganglionic component in their collection of 2000 intrasellar neoplasms. Although clinically progressive, occasionally recurrent, and sometimes fatal, all of the tumors described were well-differentiated and histologically benign choristomas and hamartomas. All were composed of a mixture of mature neurons and glial stroma, occasionally accompanied by epithelial adenoma cells. 1, 4–6, 9, 11, 12, 18–20, 22, 23, 31, 34–36, 38–42 In

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Dan X. Cai, Manuela Mafra, Robert E. Schmidt, Bernd W. Scheithauer, Tae Sung Park and Arie Perry

subtotal resection, radiation therapy (30-Gy whole-brain radiation with an additional 20-Gy boost to the posterior fossa) and five cycles of chemotherapy (vincristine, lomustine, and intrathecal methotrexate). Although the tumor remained stable on radiographic imaging, a second resection was performed 8 years later to alleviate hydrocephalus. Histopathological analysis demonstrated a mature neuronal neoplasm with extensive calcification. Postoperative Course. No additional adjuvant therapy was given and the patient is alive and well as of his last follow-up examination 3

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Kenta Fujimoto, Hideyuki Ohnishi, Masahiko Tsujimoto, Tohru Hoshida and Yoichi Nakazato

neuroepithelial tumor is a benign supratentorial mixed glial—neuronal neoplasm characterized by its intracortical location, multinodular architecture, and heterogeneous cellular composition. 1, 3–5, 8 It is described as occurring in young patients with medically intractable epileptic seizures. 1, 3–5, 8 Although more than 300 cases of DNET have been reported in the literature, the vast majority of these tumors have been supratentorial, with the temporal lobe being the most common site. 1–4, 6 However, due to the putative origin of DNETs in secondary germinal layers, these