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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Five cases of histologically confirmed olfactory neuroblastoma (esthesioneuroblastomas) have been studied by computed tomography (CT). Both the clinical symptoms (in particular, unilateral nasal obstruction and recurring epistaxis) and radiological findings (opacity of the paranasal sinuses) are nonspecific. Computed tomography shows a contrast enhancing mass-lesion and is more useful than conventional tomography in estimating its extension and the associated bone destruction, secondary reaction of sinusitis, and tumoral calcification. In the two cases in which the tumor was associated with exophthalmos, CT demonstrated that the muscular cone acts as a barrier preventing tumoral invasion. In cases wtih intracranial involvement, it appears that the attenuation values can be useful for differentiating between invasion by contiguity (two cases) versus metastasis (one case). Computed tomography is valuable in the follow-up and during and after radiotherapy of the tumor.
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PMID:Computed tomography in olfactory neuroblastoma: one case of esthesioneuroepithelioma and four cases of esthesioneuroblastoma. 70 20

The features of neuroblastoma and outcome of its management in 15 Nigerian children seen in the Ahmadu Bello University Teaching Hospital, Zaria over a 7-year period are described. The majority were between the ages of 3 and 5 years and the male:female ratio was 2.75:1. Over half of the patients presented with abdominal masses, about half with proptosis and a third with skull nodules. The diagnosis of neuroblastoma was correctly made on admission in only five instances. The mainstay of treatment was chemotherapy and the outcome was uniformly poor. Only one patient attended follow-up for as long as 1 year. Based on the present study, the frequency of neuroblastoma ranks third, after Burkitt's lymphoma and Wilms' tumour, amongst malignant childhood solid tumours encountered in the paediatric unit of Ahmadu Bello University Teaching Hospital. The low index of suspicion and poor diagnostic facilities might, however, have contributed to the low reported incidence, late diagnosis and poor survival rates of children with this tumour.
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PMID:Neuroblastoma, an under-diagnosed tumour: a 7-year experience in Zaria. 246 Nov 49

Neuroblastoma is one of the commonest childhood malignancies. The most important prognostic factor is age at diagnosis; early diagnosis, when the tumor is still localized and surgically resectable, is second in importance. On retrospective review of children seen at the Hospital for Sick Children, ophthalmic involvement was seen in 80 of 405 (20%). The three major eye signs of neuroblastoma, proptosis, Horner's syndrome and opsoclonus, are closely related to the site, stage of tumor, and outcome of the patient. Proptosis or periorbital ecchymosis due to orbital metastases was present in 60 of 80 children (bilaterally in 33). The 3-year survival rate was 11.2%. In 53 of 60 cases with orbital metastases the neuroblastoma originated in the abdomen. Unilateral Horner's syndrome occurred in 14 children, as the presenting sign in 9, related to localized disease in 11 and in a favorable location (cervical or thoracic neuroblastoma) in 8. The 3-year survival rate was 78.6%. Opsoclonus-myoclonus was the presenting sign of occult, localized neuroblastoma in all 9 children in whom it occurred. The 3-year survival rate was 100%. For all presentations, girls had a significantly better survival rate than boys (48.7% vs. 22.4%). Children presenting with any of these ophthalmological signs should undergo thorough and repeated investigations searching for neuroblastoma.
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PMID:Ocular involvement in neuroblastoma: prognostic implications. 649 2

A nasal neuroblastoma (esthesioneuroblastoma) extending into the brain in a heifer produced mouth breathing and proptosis. The mass filled much of the left nasal cavity, palatine sinus and maxillary sinus, with turbinate atrophy and deviation of the septum. Caudally the neoplasm extended into the nasopharynx and olfactory bulb. It was a cellular neoplasm composed of small, undifferentiated piriform cells showing infrequent pseudorosettes and immature axonal processes. Mitosis was common is some areas.
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PMID:Olfactory neuroblastoma in a heifer. 725 93

A review was made of the clinical data of 28 patients with metastatic orbital disease diagnosed in our clinic between 1972 and 1993. Fifteen were men and 13 were women. The average age at diagnosis was 58.7 years. The right orbit was involved in 12 and the left orbit in 14 cases. Two cases had bilateral orbital involvement. Breast carcinoma was the most frequent tumor (8 of 28, 28.6%) followed by neuroblastoma (7 of 28, 25.0%), lung carcinoma (6 of 28, 21.4%), prostate carcinoma (3 of 28, 10.7%), gastrointestinal carcinoma (2 of 28, 7.1%), renal cell carcinoma and thyroid carcinoma (1 of 28, 3.6% each). Proptosis (67.9%), motility disturbance (57.1%) and mass (50.0%) were the three most common presenting signs. Enophthalmos was noted in two cases with breast carcinoma. Nine cases presented with ophthalmic signs and metastatic tumor was recognized later. In the remaining 19 cases, the diagnosis of the primary tumor preceded the onset of orbital metastasis. The time interval between the detection of the primary malignancy and metastatic orbital tumor was shortest for lung carcinoma (mean: 2 months) and longest for breast carcinoma (mean: 34 months). Radiotherapy and chemotherapy were applied in 12 cases. Improvement in orbital signs and visual acuity was noted in 5 cases. Radiotherapy, chemotherapy and hormonal therapy were used in 8 patients and improvement in orbital signs was noted in 4 of these patients. Four of 28 patients (2 with breast carcinomas, one with prostate carcinoma and one with thyroid carcinoma) survived longer than 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Metastatic orbital tumors. 764 86

Even though the current limits of treatment for advanced stage neuroblastoma require an understanding of biology and new therapeutic approaches, few invasion models of human neuroblastoma (HNB) which evaluate experimental therapies have been reported. We describe herein a reproducible murine model of cranial invasion after the intraocular xenograft of HNB in congenitally athymic mice. Approximately 10 weeks after the intraocular injection of 5 x 10(6) NB-1 HNB cells, 70% (14/20) of the mice developed intracranial invasion with skull involvement. There was no operative mortality. Macroscopically, deformities of the cranium were revealed in all 14 mice, 5 of which developed exophthalmos. Microscopically, cranial invasion mainly involved the extradural space, skull, and orbita; however, brain involvement could not be seen, indicating that the dura may act as a barrier. These invasive characteristics are very similar to those seen in humans; thus, we believe that this model provides a useful tool for evaluating the biology of, and new therapeutic approaches against, cranial invasion of neuroblastoma in vivo.
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PMID:A cranial invasion model of human neuroblastoma using congenitally athymic mice. 791 35

Twenty children with biopsy-proven neuroblastoma were diagnosed and treated between 1984 and 1994 at King Fahd Hospital of the University in Al-Khobar, Saudi Arabia. There were 12 males and eight females with a ratio of 1.5/1. The median age at diagnosis was 3 years. Clinical staging showed: Stage I, 0 per cent; Stage II, 30 per cent; Stage III, 30 per cent; Stage IV, 35 per cent, Stage IVs, 5 per cent. Primary sites of involvement included: adrenal 55 per cent retroperitoneal, 15 per cent; thoracic, 10 per cent, cervical, 5 per cent; pharyngeal, 5 per cent; lumbar, 5 per cent; unknown, 5 per cent. Pathological features showed: neuroblastoma, 70 per cent; ganglio neuroblastoma, 25 per cent; ganglioneuroma, 5 per cent. Clinical presentation revealed: abdominal swelling, 55 per cent; fever, 40 per cent; weight loss, 35 per cent; anorexia, 25 per cent; proptosis, 20 per cent; opsomyoclonus, 5 per cent; skin nodules, 5 per cent; diarrhoea, 5 per cent. Twenty four-hour urine collection showed high level of VMA in 13 (65 per cent) patients. Follow-up was from 6 months-10 years (median 5 years). disease-free survival at 2 years were as followed: Stage II, 100 per cent; Stage III, 66 per cent; Stage IV, 14 per cent; Stage IVs, 100 per cent.
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PMID:Neuroblastoma in children: a 10-year experience in Saudi Arabia. 960 93

The sinonasal undifferentiated carcinoma (SNUC) is an aggressive and rare neoplasm arising in the nasal cavity and the paranasal sinuses. To date, over 50 cases of histologically proven SNUCs have been reported since its original description in 1986. Presenting symptoms include facial pain, nasal obstruction, diplopia, epistaxis, proptosis, and periorbital swelling. The histologic features of this neoplasm include cohesive cells arranged in nests, ribbons, and trabeculae. The cells exhibit hyperchromatic nuclei and a high nuclear to cytoplasmic ratio. A brisk mitotic rate, tumor necrosis, and vascular invasion are prominent features. Confirming the diagnosis of SNUC at the light microscopic level can be challenging, since the microscopic differential diagnosis includes olfactory neuroblastoma, rhabdomyosarcoma, undifferentiated nasopharyngeal carcinoma (lymphoepithelioma), malignant lymphoma, malignant melanoma, and neuroendocrine (small cell undifferentiated; oat cell) carcinoma. Sinonasal undifferentiated carcinoma can be differentiated from these other neoplasms by correlating clinical, light microscopic, histochemical, immunohistochemical, and ultrastructural characteristics. Aggressive, multimodal therapy can provide the best opportunity for local control of this neoplastic process, but the optimal treatment has yet to be determined.
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PMID:Sinonasal undifferentiated carcinoma: a distinctive clinicopathologic entity. 1056 93

Neuroblastoma is a common solid tumor of childhood that can involve the abdomen, thorax, pelvis, or the head and neck. The clinical manifestations are dependent on the widespread distribution of neural crest tissue and the length of the sympathetic chain involvement. Abdominal pain and hypertension may occur as a result of renal vasculature compression; respiratory distress may be evident in thoracic tumors; and Homer's syndrome or heterochromia of the iris may manifest from neuroblastoma of the head and neck. In addition, symptoms of cord compression and back pain may result from spinal cord compromise due to epidural invasion. Metastatic involvement of the liver, skin, periorbital regions, or bone may cause hepatomegaly, skin nodules, proptosis, or bone marrow failure, respectively. Clinical findings along with tumor metastasis may be studied by various imaging modalities to assess the nature and extent of the tumor. Diagnostic tests include plain radiography, ultrasonography, CT scanning, and MR imaging. Bone marrow studies, bone scans, and scintigraphy with 131I-metaiodobenzylmandelic may be utilized for metastatic evaluation. By using these imaging studies to detect the nature and behavior of neuroblastoma, early intervention may indeed improve patient survival.
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PMID:Imaging neuroblastoma in children. 1262 83

Orbital granulocytic sarcoma is a localized tumor consisting of malignant cells of myeloid origin. This tumor may present in association with acute myelogenous leukemia. Granulocytic sarcoma may be found in a variety of locations throughout the body including the orbit and typically affects children and young adults. There is a slight male predominance in these cases. This is an uncommon case report of a 33-year-old Latin-American woman who was admitted to the Hospital for rapidly progressive orbital proptosis. There was no systemic manifestation of leukemia. The occurrence of orbital granulocytic sarcoma before the development of systemic leukemia in children and young adults is not uncommon and these cases frequently develop hematological evidence within 2 months after initial orbital disease. In this case report, there was no systemic manifestation of leukemia in the last 30 months, even in the presence of orbital tumors. Granulocytic sarcoma is most frequently confused with malignant lymphoma, rhabdomyosarcoma and neuroblastoma. The differential diagnosis of these cases can be challenging, particularly when there is no evidence of systemic leukemia, when imaging features are not sufficiently specific to distinguish granulocytic neoplasms from other tumors. To establish the diagnosis often a biopsy is required. The treatment in such cases (orbital granulocytic sarcoma) is not standardized. Orbital granulocytic sarcoma may be suspected in cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.
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PMID:[Orbital granulocytic sarcoma: case report]. 1632 47


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