Peripheral primitive neuroectodermal tumor of the dura in a 51-year-old woman following intensive treatment for breast cancer.

PATIENT: Female, 51. FINAL DIAGNOSIS: Ewing sarcoma. SYMPTOMS: Visual disturbances. MEDICATION: -. CLINICAL PROCEDURE: -. SPECIALTY: Oncology. OBJECTIVE: Rare disease. BACKGROUND: Primitive neuroectodermal tumor/Ewing sarcoma (PNET/EWS) is a round blue cell sarcoma that shows varying degrees of neuroectodermal differentiation. PNET/EWS as a primary intracranial tumor is extremely uncommon. CASE REPORT: We report a unique case of peripheral PNET presenting as an intracranial mass in an adult following chemotherapy and radiotherapy for a solid tumor. A 51-year-old woman with previously treated left breast cancer was evaluated for a newly developed brain mass. She underwent craniotomy with resection. Surgical pathology was consistent with a peripheral PNET/EWS with Ewing sarcoma gene translocation. She was treated appropriately with vincristine, cyclophosphamide, and doxorubicin (later dactinomycin) alternating with ifosfamide and etoposide. CONCLUSIONS: Although development of PNET/EWS presenting along the CNS is exceedingly rare in adults, establishing the proper diagnosis of this "small blue cell tumor" is critical. The further distinction between central PNET and peripheral PNET can greatly impact both prognosis and treatment. Our case also highlights the importance of considering the impact of prior intensive therapies, including radiation and chemotherapy, on predisposing to future PNET/EWS.

An unusual primitive neuroectodermal tumor in the thoracic epidural space.

Primitive neuroectodermal tumor (PNET) is a generic term used to describe a group of histologically indistinguishable neoplasms, including cerebellar medulloblastomas, which are located at various sites in the central nervous system. Primary epidural PNETs are rare and few patients have been reported. We report a 15-year-old girl who presented with gradual onset, over 1 month, of upper back pain and bilateral lower leg weakness. A thoracic spine MRI showed a dumbbell-shaped epidural mass at T2-4 with right paraspinal and posterior mediastinal extension. Surgical resection of the epidural tumor for decompression was performed. The pathologic examination revealed a PNET. Primary spinal PNETs typically have a poor prognosis and optimal therapy has not yet been defined. Surgical resection, with the combination of chemo-radiotherapy or radiotherapy, leads to better outcomes. However, primary epidural PNETs may be classified as a subtype of spinal PNETs because they are free from intrathecal invasion. For these patients, surgery alone and surgery combined with radiotherapy or chemo-radiotherapy remain controversial. Our patient received surgery alone and, 1y ear later, has experienced no local recurrence within the epidural space but the mediastinal part of the tumor has enlarged.

Peripheral primitive neuroectodermal tumor after radiotherapy.

A 41-year-old man had a peripheral neuroectodermal tumor develop at the distal third of the fibula 4 years after radiotherapy for relapsed villonodular synovitis. This type of sarcoma usually is classified into the heterogeneic group of small round-cell bone tumors as a subdivision of Ewing's sarcomas. The immuno-staining positivity of the neoplastic cells for the neuron-specific enolase allowed the authors to make the diagnosis of a tumor with neuroectodermal origin. When the histologic study confirmed the diagnosis, the patient was treated with chemotherapy, surgical excision of the tumor, and adjuvant radiotherapy. Radiotherapy is thought to be involved in the genesis of osteogenic sarcomas as it has been shown in several reports, but there is no evidence in the literature of a peripheral neuroectodermal tumor developing after radiotherapy.

Peripheral primitive neuroectodermal tumour after allogeneic bone marrow transplantation.

We report a case of peripheral primitive neuroectodermal tumour (PNET) in a 36-year-old man who 10 years earlier received an allogeneic bone marrow transplantation (BMT) for a non-Hodgkin's lymphoma. Immunohistochemistry proved definitive elimination of a relapse of the original disease and confirmed the diagnosis of PNET. The role of total body irradiation pre-BMT in the genesis of this secondary tumour is discussed. The importance of registering secondary malignant solid tumours after BMT is emphasized. To our knowledge, an Ewing family tumour following BMT has not previously been reported.

[Esthesioneuroblastoma: histogenesis and diagnosis]. / Asthesioneuroblastom: Histogenese und Diagnose.

Tumors of the rat induced by means of two different nitrosamines (N-nitrosopiperidine, 2,6-dimethylnitrosomorpholine) were analyzed with the light and electron microscope and by immunohistological investigation. The ultrastructural analysis showed mainly in the Flexner rosettes some clear characteristics of olfactory epithelium such as olfactory vesicles, cilia, and microtubules. With four exceptions, the immunohistologically investigated tumor cells showed no immunofluorescence after incubation with antibodies against intermediate filaments. The results resemble in all aspects the structures found in human esthesioneuroepitheliomas, so that it is reasonable to speak of an identical tumor. As the sensory cells of the normal olfactory epithelium as well as the light basal cells show a negative reaction with antibodies against intermediate filaments, too, the induced tumor is derived histogenetically from the olfactory epithelium and the light basal cell is assumed to be its stem cell.

Esthesioneuroblastoma: cerebrospinal fluid rhinorrhea and widespread metastasis.

Esthesioneuroblastoma, a tumor of neural crest origin arising in the nasal cavity, is uncommon, may be difficult to diagnose, and frequently is not cured. We report a case that was originally diagnosed as an inflammatory polyp and recurred with extension into the anterior cranial fossa. Surgery and radiotherapy were followed by local recurrence and cerebrospinal fluid rhinorrhea. Despite initial negative results of investigation for metastases, recurrence appeared in cervical nodes and was followed by widespread bone, soft tissue, and cerebral metastasis. There was no response to multiple-agent chemotherapy. The results of surgery, radiation, and chemotherapy in this rare and lethal tumor are reviewed.

Esthesioneuroblastoma: diagnosis and management.

Esthesioneuroblastoma is an uncommon nasal tumor originating from cells of neural crest origin. An increased awareness of this neoplasm by clinicians and pathologists has led to an increase in its diagnosis. In the Department of Otolaryngology--Head and Neck Surgery at the University of Virginia Medical Center, 22 cases have been treated. A review of the biologic course of these cases has allowed us to make certain recommendations for therapy. Patients with stage A and B disease are best treated with preoperative radiation therapy followed by craniofacial resection. Patients with stage C disease are treated with cyclophosphamide and vincristine before irradiation and craniofacial resection. If a significant response occurs these patients receive postoperative chemotherapy. We believe this treatment should result in five-year survivals in excess of 50%.

Olfactory neuroblastoma (esthesioneuroblastoma): a light and ultrastructural study of two cases.

Olfactory neuroblastoma is a malignant neoplasm with a varied biological behavior. Its clinical course is unpredictable and there is no correlation between its microscopic features and biological behavior. The present study deals with light and ultrastructural characteristics of two cases of olfactory neuroblastoma of the nasal cavity. In one patient, the definitive diagnosis was established on the basis of ultrastructural features of the lesion. The most consistent fine structural findings were the presence of intracytoplasmic densecored neurosecretory granules, "true" and "pseudo-" rosettes, and the neuritic processes emanating from the tumor cells. On the basis of their biochemical, histochemical, and ultrastructural characteristics, olfactory neuroblastomas are similar to neuroblastomas arising from the adrenals or sympathetic nervous system. These findings, therefore, support the hypothesis that olfactory neuroblastomas are most likely of neural crest origin and thus belong to a group of neoplasms collectively known as "apudomas" or neurocristomas. The literature review strongly favors combined surgery and postradiation as the most effective treatment of olfactory neuroblastoma.

Human adenovirus-induced medulloepitheliomatous neoplasms in Sprague-Dawley rats.

A direct causal relationship between a human virus and malignant transformations in target cells (sensory neuronal precursors) was suggested by the development of a medulloepitheliomatous neoplasm in the central nervous system. Twenty-six newborn Sprague-Dawley rats were given a single intracerebral inoculation of 0.05 ml of adenovirus fluid, 10(3.5) to 10(4.5) TCID(50) HeLa cells/0.1 ml, in the left frontal lobe. Within 37 to 151 days after the virus inoculation, 23 (88.7%) rats autochthonously developed an adenovirus-typical neoplasm in the central nervous system. Nine animals developed a multicentric neoplasm closely related to the ventricular system. Nine others developed solid variously sized neoplasms along the ventricular lumen. Some neoplasms showed multiple foci connected with the stratum subependymale ventriculi olfactorii and the velum medullare of the fourth ventricle. Six spinal cord tumors, located chiefly in the dorsal sensory column, developed within 37 to 61 days after intracerebral inoculation. The remarkably uniform histopathologic appearance of all 23 cases was attributed to a medulloepitheliomatous neoplasm derived from the ependymal anlage. Electron microscopy clearly revealed a solitary cilium within the apical region of many tumor cells. It consisted of a typical ring of nine doublets with no axial pair (a 9+0 pattern), the typical structure of cilia of sensory neuronal origin. The appearance of exuberant neuron-like tumor cells with argyrophile cytoplasmic expansions, neurosyncytial mosaic alignment and myelin-like configurations also suggested a neuronal origin. A paucity of mesenchymal stroma in the neoplastic tissue was noted. No control animals developed tumors.