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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 433 records · Page 24Linked to original sources
PubMed1975

Olfactory neuroblastoma. Management and prognosis.

Olfactory neuroblastoma is an uncommon malignant tumor originating in the olfactory epithelium in the roof of the nose. It usually produces nasal obstruction and secondary sinus disease and is often mistaken by the physician for a nasal polyp. Olfactory neuroblastoma has been observed to cause death by distant metastasis or by invasion through the cribriform plate and secondary meningitis in most instances, and is generally associated with a low cure rate. During the past eight years, we have treated three patients with olfactory neuroblastoma by a combination of extensive local surgery using the lateral rhinotomy approach with ethmoidectomy and medial partial maxillectomy, combined with either preoperative or postoperative irradiation therapy. From a review of the literature we have tried to draw meaningful conclusions regarding treatment.

Adult↗
PubMed1976

Fume-induced fluorescence in diagnosis of nasal neuroblastoma.

Nasal neuroblastoma, esthesioneuroblastoma, is frequently difficult to distinguish from the more common poorly differentiated epidermoid carcinoma of the nasal cavity and nasopharynx. We present a simple alternate method to electron microscopy, formaldehyde-fume-induced fluorescence, to demonstrate biogenic amine granules in neoplastic cells. This method is more specific and more sensitive, since it reveals the presence of biogenic amines, not merely membrane-bound granules, and it deals with larger quantities of tissue, thus avoiding some of the sampling errors inherent in electron microscopy. We also describe the histochemical relationship of this tumor to other neural crest neoplasms.

Formaldehyde↗
PubMed1977

En bloc ethmoidectomy and medial maxillectomy.

A method is described which enables the surgeon to gain necessary visual access for en bloc removal of certain tumors of the nasoethmoid area. A "medial maxillectomy" has been performed on ten patients, three of whom underwent combined craniofacial surgery for extensive neoplasms. The procedure is especially suitable for those tumors in which piecemeal or "shelling out" techniques are not desirable, and in which one desires to preserve the eye, palate, and facial appearance.

Adenocarcinoma↗
PubMed1980

Olfactory neuroblastoma. Response to combination chemotherapy.

A patient with olfactory neuroblastoma who had bone marrow metastasis at the time of diagnosis is presented. Previous therapy for this disease consisted of surgery and radiation. There is limited information relating to the efficacy of chemotherapy. Our patient was treated with combination chemotherapy (dacarbazine [DTIC-Dome], cyclophosphamide [Cytoxan], doxorubicin hydrochloride [Adriamycin], and vincristine sulfate [Oncovin]) and radiation to the primary site. Objective findings, more than two years after diagnosis, support a good partial response. Although a 50%, five-year survival time has been reported, the five-year cure rate is 18%. This report suggests that the role of combination chemotherapy should be further evaluated in certain patients with olfactory neuroblastoma.

Adult↗
PubMed1980

Radical surgery for ethmoid cancer.

Radical surgery followed by radiotherapy offers the best chance of cure for ethmoid cancer. In operations on 15 patients with ethmoid cancer, intracranial spread was found in five instances. In every patient, a craniofacial ethmoidectomy was performed. Two approaches are recommended. If there is radiologic or clinical evidence of intracranial spread, a frontal craniotomy and lateral rhinotomy approach is employed. With the help of the neurosurgeon, the overlying dura can be resected with the anterior cranial floor. When the cribriform plate appears intact, the resection is accomplished via a lateral rhinotomy and transfrontal sinus approach to the anterior cranial fossa. These techniques were used in 15 patients. Nine are free of recurrence after an observation time of two to seven years.

Adenocarcinoma↗
PubMed1983

Hyponatremia secondary to olfactory neuroblastoma.

A patient with a four-year history of unexplained hyponatremia was seen with recurrent nasal discharge and was found to have a typical olfactory neuroblastoma. The clinical laboratory diagnostic studies suggested that the patient's sodium deficiency was secondary to the syndrome of inappropriate antidiuretic hormone. Subsequent resection of the neoplasm led to resolution of the hyponatremia, suggesting that a (tumor-associated) humoral factor, such as vasopressin or a vasopressinlike substance, was responsible for the electrolyte disturbance. A search of the literature disclosed one previous case of vasopressin-secreting nasal neuroblastoma.

Adult↗
PubMed1983

Olfactory neuroblastoma. Biologic and clinical behavior.

The biologic behavior of olfactory neuroblastoma in 21 persons treated at the Mayo Clinic, Rochester, Minn, from 1960 to 1980 is reviewed. Follow-up data of five years or more are available for 19 cases. Findings showed the tendency of these tumors to spread submucosally and intracranially without obvious involvement of the anterior cranial fossa dura. The aggressive nature of this lesion has not been appreciated. Metastatic disease occurred in 62% and local recurrence in 57%. Long-term follow-up showed that recurrent or metastatic disease could occur up to ten years after initial diagnosis. The five-year survival rate was 58%. Only four patients who have been follow-up for more than five years were without local recurrence or metastasis. This review should prompt a change in the current attitudes toward treatment of this tumor.

Adolescent↗
PubMed1984

Olfactory neuroblastoma. Chemotherapy and radiotherapy for extensive disease.

A patient with olfactory neuroblastoma had extensive involvement of the nasopharynx, sinuses and orbit, and cervical metastasis. After surgical biopsy and excision of metastatic disease, she was treated with chemotherapy: three cycles of high-dose cisplatin (100 mg/sq m) and infusion of fluorouracil (1 g/sq m/day for five days). Subsequent treatment included definitive radiation therapy followed by three additional cycles of chemotherapy. The patient is free of disease three years after initiating therapy. This case report demonstrates the potential efficacy of planned combined modality therapy, including early chemotherapy in the control of locally advanced, unresectable olfactory neuroblastoma.

Cisplatin↗
PubMed1985

Dynamic computed tomography. Its use in the assessment of vascular malformations and angiofibroma.

Reliable diagnostic methods for head and neck vascular lesions are needed. The technique of dynamic computed tomography (CT) is used to delineate two aberrant subclavian arteries, one carotid artery aneurysm, and one angiofibroma. Five or six 4.8-s CT scans were obtained sequentially, separated by a 1.2-s interscan interval. Computer-generated iodinated contrast concentration (CT number) v time curves showed rapid influx of contrast, high peak density, and rapid uninterrupted contrast runoff. Simultaneous bone and soft-tissue visualization at the time of peak contrast enhancement provides excellent delineation of the malformations and angiofibroma. The technique is noninvasive, and it eliminates the potential complications of arteriography and biopsy. We conclude that dynamic CT is an excellent method for diagnosis of head and neck vascular lesions.

Adult↗
PubMed1988

Preservation of the eye in paranasal sinus cancer surgery.

Forty-one patients undergoing surgery between 1977 and 1985 for malignant tumors of the paranasal sinuses abutting or eroding the orbital walls were studied for the need to remove the orbital contents. All patients had preliminary computed tomographic scans to delineate the extent of orbital invasion. All were treated with preoperative radiotherapy. If the tumor mass could be peeled from the periorbita, the eye was saved. Preservation of eyes in the patients without periosteal invasion did not alter survival. Frozen-section control may be used to determine periorbital involvement. If the periorbita was minimally involved, it was locally resected. If invasion of the periorbita was extensive, an orbital exenteration was done. Only five of 41 patients required exenteration. Local recurrence of disease in the orbit has not occurred in these patients.

Adenocarcinoma↗
PubMed1989

Superior rhinotomy for en bloc resection of bilateral ethmoid tumors.

Ethmoid sinus neoplasms have traditionally been associated with a poor prognosis. Their advanced stage at presentation and proximity to vital structures have made them difficult to treat. Bilateral ethmoidal tumors impinging on or actively infiltrating the cribriform plate have been particularly resistant to cure. We have used the superior rhinotomy in conjunction with a bifrontal craniotomy in three patients to effect an en bloc resection of both ethmoid labyrinths and cribriform plate in an oncologically acceptable fashion.

Adenocarcinoma↗
PubMed2003

Adult soft tissue Ewing sarcoma or primitive neuroectodermal tumors: predictors of survival?

BACKGROUND: Ewing sarcoma (ES) is the second most common primary osseous malignancy in childhood and adolescence. The improvement in survival is primarily associated with the combination of surgery and chemotherapy. HYPOTHESIS: Little is known about the outcome of adults with soft tissue ES or primitive neuroectodermal tumors (PNET). Certain prognostic factors from soft tissue sarcomas (tumor size, tumor location, margin status, and initial presentation) in adults (>16 years) with ES/PNET will help to identify factors associated with outcome. METHODS: Between July 1, 1982, and June 30, 2000, we identified 59 adult patients with primary soft tissue ES/PNET. Clinicopathologic factors were correlated with the end points studied: patient factors, tumor factors, pathologic factors, status of surgical margins, adjuvant chemotherapy, and radiation therapy. RESULTS: There were 41 male and 18 female patients, with a median age of 27 years (range, 16-72 years). Median tumor size was 8 cm, with all lesions being high grade. The most common site was the trunk (n = 22), with an even distribution of retroperitoneal, pelvis, buttock, and lower extremity (all n = 5). The median follow-up was 29 months (range, 6-222 months), with local recurrence identified in 13 patients (22%), with a median time to recurrence of 15 months (range, 5-200 months). Overall 5-year survival was 60%. Initial presentation was the only predictor of long-term survival, with primary tumor-only presentation having a 5-year survival of 60% (median not reached) compared with primary tumor plus metastatic disease having a 5-year survival of 33% (median, 17 months) (P =.02). CONCLUSION: Initial presentation of disease represents the only predictor of survival identified in this small group of adult patients with ES/PNET.

Adolescent↗
PubMed1998

Brain abscesses in children with cancer.

BACKGROUND: Brain abscesses in pediatric patients are rare events, and the causative organism and prognosis vary with the population under study. Children with cancer seem to be particularly susceptible to the development of brain abscesses because of the immunological changes induced by cancer and its treatment. We reviewed the records of children who developed a brain abscess during treatment of a malignancy to define the clinical characteristics, prognosis, and management of these patients. PROCEDURE: We performed a retrospective review of the clinical and laboratory characteristics of all cancer patients younger than age 20 years who were admitted to our institution between 1980 and 1996 for a brain abscess. RESULTS: Twelve children were identified. Cancer diagnoses were brain tumor in two, systemic PNET in two, and leukemia in eight. Six patients had multiple abscesses. Eleven received prior chemotherapy. Abscesses were surgically excised or aspirated in seven, and empiric antibiotics were given to the other five. At surgery, Listeria monocytogenes, Aspergillus fumigatus (3), Fusarium, and Candida lusitanea were cultured. Aspergillus was identified in other locations in four patients. Abscesses were successfully treated in seven patients, two of whom received antibiotics only; five patients (42%) died from infection. CONCLUSIONS: Mortality is high in this immunosuppressed population, in part due to the preponderance of fungal infection. The finding of very rare organisms suggests that drainage and culture should be performed whenever possible; empiric antibiotics that include an antifungal agent may, on occasion, be successful.

Abscess↗
PubMed1998

A Phase II clinical trial of idarubicin administered to children with relapsed brain tumors.

BACKGROUND: Idarubicin (IDR), an anthracycline that is a derivative of daunorubicin, was synthesized in an attempt to find new analogs of daunorubicin with an improved spectrum of activity and diminished acute or chronic toxicity. Because of the favorable pharmacokinetic profile of IDR (with the persistence of its active metabolite [idarubicinol], the penetration of idarubicinol into the cerebrospinal fluid, and the lipophilicity of IDR/idarubicinol compared with other anthracyclines), its more favorable therapeutic index regarding cardiotoxicity in animals, and its potential for oral administration, a Phase II trial of IDR in children with relapsed brain tumors was undertaken. METHODS: Patients received IDR at a dose of 5 mg/m2/day x 3 days by intravenous bolus, followed by granulocyte-colony stimulating factor (G-CSF) at a dose of 5 microg/kg/day, starting on Day 7 of each cycle and continuing for at least 7 days, until the absolute neutrophil count was > or =10,000/mm3. RESULTS: Three of 19 patients with high grade astrocytoma achieved a partial response, 1 of 20 patients with medulloblastoma had a complete response, and 0 of 13 patients with ependymoma and 0 of 13 patients with brainstem tumors had responses. In nine other brain tumor patients there were no responses. The most significant toxicity was myelosuppression. CONCLUSIONS: IDR, given at a dose of 5 mg/m2/day x 3 days, is not sufficiently active against relapsed medulloblastoma, ependymoma, or brain stem tumors to warrant further study of this agent in a Phase III setting. The response rate for patients with relapsed high grade astrocytoma was 15% (95% confidence interval, 3.3-40%).

Adolescent↗
PubMed1999

Vasoactive intestinal peptide (VIP) and VIP receptors: gene expression and growth modulation in medulloblastoma and other central primitive neuroectodermal tumors of childhood.

Vasoactive intestinal peptide (VIP) is a neuromodulator and growth regulator in the developing nervous system. We analyzed 10 primitive neuroectodermal tumor (PNET) cell lines, 29 central PNET (cPNET) and 17 tumors of the Ewing's sarcoma/peripheral PNET family (ESFT) using reverse transcriptase-polymerase chain reaction (RT-PCR) and Southern hybridization. Each of the 10 cell lines and 86.2% of cPNET expressed mRNA for VIP receptor 1 (VIPR1) compared to 52.9% of ESFT. VIPR2 was expressed in 75.8% of cPNET, in 28.6% of ESFT and in all 10 cell lines. cPNET demonstrated high-affinity binding of 125I-VIP on quantitative autoradiography and in competitive binding assays. VIP inhibited tumor cell proliferation in a dose-dependent manner in 5 of 7 PNET cell lines. We conclude that VIPR1 and VIPR2 are highly expressed in cPNET and demonstrate that VIP is a growth modulator in these tumors.

Autoradiography↗
PubMed1999

Anterior craniofacial resection for malignant ethmoid tumors--a series of 91 patients.

BACKGROUND: Anterior craniofacial resection is now recognized as the best treatment for ethmoid tumors involving the cribriform plate with or without invasion of anterior cranial fossa. METHODS: Ninety-one patients underwent an anterior craniofacial resection for ethmoid malignant tumors at the Milan Cancer Institute between 1987 and 1994. The patient population was divided into two sections (30 and 61 patients) based upon some important variants (type of craniotomy, antibiotic treatment, postoperative care). RESULTS: The mean age was 53.4 years (range, 24 to 78 years). There were 62 men and 29 women. Forty-nine patients had a recurrence after previous treatments (surgery and/or radiotherapy). The subdivision by histology was as follows: 50 cases of adenocarcinoma, 16 cases of epidermoid and undifferentiated carcinoma, 8 cases of esthesioneuroblastoma, 5 cases of adenoid cystic carcinoma, 5 cases of melanoma, and 6 rare tumors. The stages (according to our new staging) were as follows: 37 cases with T2, 27 cases with T3, and 27 cases with T4. The mean follow-up was 47 months. Seven patients died after surgery (6 in the first series). The survival at 3 and 5 years was, respectively, 52% and 47%, and the disease-free survival (DFS) was 30% and 24%, with a statistically significant difference at multivariate analysis in favor of patients without prior treatment (p = .033) or T2 versus T3 and T4 (p<.007). CONCLUSIONS: An anterior craniofacial resection should be performed in cases of ethmoid tumors reaching or eroding the cribriform plate. A scrupulous intra- and postoperative approach is necessary to avoid severe complications. The patients often survive for a long time with recurrence ongoing. Our new staging identifies the critical extensions of ethmoid tumors.

Adenocarcinoma↗