Primary orbital Ewing's sarcoma: report of a case and review of the literature.

Primary orbital Ewing's sarcoma is a very rare condition. Since the first case was reported in 1950, only 7 other cases have been reported in the English literature. Herein we describe the ninth, the only bilateral and the youngest case of primary orbital Ewing's sarcoma occurring in a 2-year-old boy, who presented to us with bilateral painless proptosis. Tissue biopsy of the tumour was obtained through the nasal sinus. Immunohistological studies of the biopsy tissue confirmed the diagnosis of Ewing's sarcoma. No distant site of the tumour was found so this was considered a primary orbital tumour. Combined chemotherapy and radiotherapy without surgical resection achieved an encouraging result in that the patient has remained in remission for 30 months after completion of treatment. The successful use of combined chemotherapy and radiotherapy, without surgery, adds further support to evidence that surgical excision may be avoided in selected cases of primary orbital Ewing's sarcoma.

A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases.

Four examples of a novel glioneuronal neoplasm are presented. All tumors affected adults (including two males and two females aged 25-40 years) as supratentorial, cerebral hemispheric masses with associated seizure activity and, in one case, symptoms of raised intracranial pressure and progressive hemiparesis. CT scans in two cases revealed hypodense masses without calcification. MRI scans at presentation demonstrated, in all cases, solid T1-hypointense and T2-hyperintense tumors with mass effect in one instance but no edema or contrast enhancement. Only one was relatively circumscribed on neuroradiologic study. All were infiltrative in their histologic growth pattern and predominantly glial in appearance, being composed mainly of fibrillary, gemistocytic, or protoplasmic astroglial elements of WHO grade II to III. Their distinguishing feature was their content of sharply delimited, neuropil-like islands of intense synaptophysin reactivity inhabited and rimmed in rosetted fashion by cells demonstrating strong nuclear immunolabeling for the neuronal antigens NeuN and Hu. These cells included small, oligodendrocyte-like ("neurocytic") elements as well as larger, more pleomorphic forms. Two cases contained, in addition, well-differentiated neurons of medium to ganglion-cell size. Proliferative activity was observed principally within the glial compartment; two cases contained mitotic figures and exhibited relatively elevated MIB-1 indices (6.8% and 8.2%). One of the latter progressed and proved fatal at 30 months following subtotal resection and radiotherapy. The three other patients are alive at intervals of 14 to 83 months, two tumor-free and one with extensive disease associated with the appearance of enhancement on MRI. This glioneuronal tumor variant may pursue an unfavorable clinical course.

MRI demonstration of subarachnoid neurocysticercosis simulating metastatic disease.

We present a patient with neurocysticercosis with spinal subarachnoid spread who presented with lower back pain and progressive numbness and weakness of the left leg. MRI of the spine simulated metastasis. MRI of the brain demonstrated a "bunch of grapes" appearance in the basal cisterns, characteristic of cysticercosis.

Intraparenchymal clear cell meningioma of the brainstem in a 2-year-old child. Case report and literature review.

We report an unusual case of an intraparenchymal clear cell meningioma of the brainstem, occurring in a 22-month-old girl. She presented with bulbar dysfunction and a right hemiparesis due to an intrinsic tumor of the medulla, which was confirmed by radiologic imaging to be focal and with an exophytic component. At surgery, a partial resection was achieved and no dural attachment was found. Pathologic examination revealed a clear cell meningioma. In reviewing the literature, there have been fewer than 20 reported cases of clear cell meningioma, none of which were intraparenchymal, involved the brainstem or occurred in such a young patient. The pathologic findings, treatment options and present understanding of this tumor are discussed.

Cytodiagnosis of pilocytic astrocytoma in smear preparations.

OBJECTIVE: To assess the cytologic features in smear preparations of 23 pilocytic astrocytomas. STUDY DESIGN: Examination of 23 smears. RESULTS: In all cases, the diagnosis was subsequently confirmed on histologic sections. The smears were moderately cellular, with a variably myxoid background. They showed bipolar "hair" cells with elongated, coarse cytoplasmic processes; bland, oval nuclei; Rosenthal fibers; eosinophilic granular bodies; and hyalinized vessels. Stellate cells and bare tumor nuclei were also present. Nuclear pleomorphism, vascular proliferation, perivascular pseudorosettes, microcalcification and hemosiderin-laden macrophages were infrequent features. Mitoses and necrosis were absent. The differential diagnosis should include diffuse fibrillary astrocytomas of all histologic grades, pleomorphic xanthoastrocytoma, ganglion cell tumor, ependymoma and reactive gliosis. CONCLUSION: Correlation of cytologic features with clinical and radiologic information allows pilocytic astrocytoma to be distinguished from other conditions.

Intracranial vascular calcifications, glioblastoma multiforme, and lead poisoning.

A 72-year-man with previous lead poisoning presented with raised intracranial pressure and localizing neurologic signs. CT scans showed a high-grade glioma and extensive intracranial calcifications, which proved to be vascular in distribution on postmortem examination. The latter findings support the concept of dystrophic calcification following lead-induced cerebrovascular injury. Lead poisoning should be considered in the differential diagnosis of unexplained intracranial calcifications. There is also evidence from previous studies to suggest a causative relationship between lead poisoning and development of glioma.

Prevalence and prognostic significance of tumor-associated tissue eosinophilia in nasopharyngeal carcinoma.

BACKGROUND: Tumor-associated tissue eosinophilia (TATE) has been associated with an improved prognosis in a variety of neoplasms. METHODS: Diagnostic biopsy specimens from 96 patients with nasopharyngeal carcinoma (NPC) were reviewed for the presence of TATE by an observer blinded to the patients' clinical histories. Comparisons between patients with and without TATE with respect to the probabilities of local recurrence, distant metastasis, and survival were performed using the log rank test on Kaplan-Meier product-limit estimates and the Cox proportional hazards model. RESULTS: The prevalence of TATE in these patients was 32%, and was not significantly associated with local recurrence, distant metastasis, or survival. CONCLUSIONS: These results are discordant with those of studies in other tumor models, although comparison is hampered by varying definitions of TATE. The differing results may be due to variations in the degree of activation of the eosinophils present in TATE in different tumors.

Waterhouse-Friderichsen syndrome complicating primary biliary sepsis due to Pasteurella multocida in a patient with cirrhosis.

Pasteurella multocida is an opportunistic pathogen causing bacteraemia in patients with liver dysfunction. A fulminant case of acute cholecystitis and septicaemia caused by P multocida, complicated by Waterhouse-Friderichsen syndrome without skin haemorrhage, is reported in a previously healthy 64 year old Chinese woman. The patient presented with a six hour history of sudden onset epigastric pain, vomiting, chills, and rigors. A presumptive diagnosis of cholangitis with septicaemic shock was made. Disease progression was rapid and the patient died within eight hours of symptom onset. This case is further proof that skin and mucosal haemorrhages are not an essential feature of Waterhouse-Friderichsen syndrome and this condition should be suspected in all patients presenting with sudden illness and fulminant septicaemia.