Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Testicular and paratesticular tumors are rare in childhood, representing 1-2 percent of the solid tumors in the pediatric age. In patients under 14 years, the incidence is 0.5-2/100,000. The evolution differs from that of the adult. Since 1981 to 1994 we have treated 14 cases of testicular and paratesticular tumors. Mean age was 2.54 years, the younger patients presenting germinal tumors. The most frequent tumor was the yolk sac tumor (36%), followed by teratoma mature (29%), and Leydig cell tumor, epidermoid cyst, paratesticular fibrous hamartoma, paratesticular neuroblastoma and paratesticular rabdomyosarcoma (7% each). A testicular mass was present in all cases (100%). Testicular ultrasound was used in 100%. In all the patients with malignant tumors a thoraco-abdominal CT scans was done to rule out extension or the tumor, being negative in all cases. Alphafetoprotein were high in all the cases of yolk sac tumor, being within normal range one month after surgery except in one case. In 11 patients an orquiectomy was done through an inguinal approach, and in three cases a simple tumorectomy without orquiectomy. No lymphadenectomy was done. All the malignants neoplasms were stage I tumors, except one yolk sac tumor stage III. Postop chemotherapy was applied in this one and in the embrionary rabdomyosarcoma. There were no recurrences after a follow-up mean time of 3.98.
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PMID:[Testicular tumors in childhood. Review of cases in the course of 13 years]. 896

A retrospective review of 16 patients operated upon for primary mediastinal tumours was carried out. Anterior mediastinal tumours seen included retrosternal goitre (2), benign cystic teratoma (1), benign thymoma (1), malignant thymoma, spindle cell type (1) and Hodgkin's lymphoma, nodular sclerosing type (1). Mid-mediastinal tumours included bronchial cyst (1), mediastinal granuloma (1), and pulmonary arterio-venous fistula (1). Neurilemmoma (2), neuroblastoma (1), ganglioneuroma (1), Askin tumour (1), neurofibroma (1) and benign histiocytoma (1) constituted the tumours of the posterior mediastinum. The tumours were successfully resected with minimal operative morbidity and no mortality.
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PMID:Primary mediastinal tumours in the southern region of Saudi Arabia. 901 77

This report describes the fine-needle aspiration (FNA) cytology of a case of pleuropulmonary blastoma in a 3-yr-9-mo-old male. Pleuropulmonary blastoma is considered by most authors to be distinct from pulmonary blastoma and is a rare malignant tumor of the intrathoracic cavity. FNA smears were cellular with numerous small ovoid to spindled cells with oval to elliptical nuclei exhibiting finely granular chromatin and inconspicuous nucleoli. The cytoplasm was scant and eosinophilic with indistinct borders. Focal chondroid material and blastema-like cells were noted. The differential diagnosis suggested by the cytologic findings included rhabdomysosarcoma, teratoma, neuroblastoma, malignant mesenchymoma, pleuropulmonary blastoma, and metastatic tumor. To our knowledge, this is the first report of the cytology of this tumor.
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PMID:Fine-needle aspiration cytology of pleuropulmonary blastoma: case report and review of the literature. 914 27

Impact of surgery on the result of mediastinal tumors is variable: In benign tumors such as teratoma, congenital cyst, intrathoracic struma and neurogenic tumor, a complete resection causes a good result. Simultaneous thoracotomy and laminectomy should be performed in the Dumbbell type of neurogenic tumor. Since complete resection of the invasive thymoma promises a good prognosis, excision of all the tumor and invading tissues followed by radiation therapy is recommended in the treatment of thymoma. In pure seminoma resection and radiation therapy is the treatment of choice, while in non-seminomatous germ cell tumor, aggressive chemo- and radiation therapy should be performed, until tumor markers such as AFP and HCG become negative. The operation is to be followed afterwards. A mass screening test for neuroblastoma in infant facilitates an early detection of this disease and promises a good result by chemotherapy, radiation therapy, and surgery. There is still a possibility that the result of treatment of the malignant mediastinal tumors would be improved by exact diagnosis and intensive treatment.
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PMID:[Current status of surgery for mediastinal tumors]. 930 51

We report three cases of neuroblastoma arising within the thymus of elderly patients. All tumors consisted of primitive neuroblasts showing focal gangliocytic differentiation within nests of neuropil. All stained for neuroendocrine markers but were negative for cytokeratins and for the MIC2 gene product. One tumor was associated with the syndrome of inappropriate secretion of antidiuretic hormone, an endocrinopathy we found in three of five case reports of thymic neuroblastoma in adults. Immunohistochemical stains confirmed production of antidiuretic hormone by this tumor. One patient died of progressive disease, one patient is disease free at 18 months, and the other patient died of unrelated causes, a spectrum that reflects the variable clinical behavior others have reported. The possible histogenesis of these purely neural tumors includes malignant transformation of a mediastinal teratoma, aberrantly located sympathetic ganglia, neuroectodermal cells native to the normal thymus, and precursors of thymic epithelial cells that have differentiated along neural lines.
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PMID:Thymic neuroblastoma in adults: report of three cases with special emphasis on its association with the syndrome of inappropriate secretion of antidiuretic hormone. 935 92

One case of neuroblastoma arising in an adult immature testicular teratoma is described, with multiple systemic metastases, a partial response to intensive chemotherapy and a swift recurrence leading to death. Such instances of prevailing neuroblastoma with systemic metastases, have only seldomly been reported hitherto. Because of the teratoma and the focal presence of intratubular germ cell neoplasia of unclassified type, we think this tumor must be indeed of germ cell derivation.
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PMID:[Neuroblastoma arising in testicular teratoma]. 940 83

The class III beta-tubulin isotype is widely used as a neuronal marker in normal and neoplastic tissues. This isotype was, however, also immunodetected in certain tumours of non-neuronal origin such as squamous cell carcinoma. Using a newly described monoclonal antibody we compared the distribution of class III beta-tubulin in normal and neoplastic tissues. The TU-20 mouse monoclonal antibody was prepared against a conserved synthetic peptide from the C-terminus of the human class III beta-tubulin isotype, and its specificity was confirmed by immunoblotting, by competitive enzyme-linked immunosorbent assay and by immunofluorescence microscopy on cultured cells. In different cell lines of various origins the antibody reacted only with neuroblastoma Neuro-2a cells and with embryonal carcinoma P19 cells stimulated to neuronal differentiation by retinoic acid. Immunohistochemistry on formaldehyde-fixed paraffin-embedded normal human tissues revealed the presence of the class III beta-tubulin isotype in cell bodies and processes of neuronal cells in the peripheral and central nervous systems. In other tissues, this beta-tubulin isotype was not immunodetected. Class III beta-tubulin was found in all cases of ganglioneuroblastoma, ganglioneuroma, medulloblastoma, neuroblastoma, sympathoblastoma and in one case of teratoma. In contrast, no reactivity was detected in tumours of non-neuronal origin, including 32 cases of squamous cell carcinoma. The results indicate a specific TU-20 epitope expression exclusively in neuronal tissues. The antibody could thus be a useful tool for the probing of class III beta-tubulin functions in neurons as well as for immunohistochemical characterisation of tumours of neuronal origin.
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PMID:Expression of class III beta-tubulin in normal and neoplastic human tissues. 954 71

We report four cases of sinonasal teratocarcinosarcoma (SNTCS), a rare malignant tumor that displays combined features of an immature or malignant teratoma and a carcinosarcoma. The patients, three men and one woman, were all adults ranging in age from 21 to 69 years who presented with nasal obstruction and epistaxis. The tumors were typically composed of round cells and short spindle cells with neuroectodermal/rosette-like structures. Also seen were foci of fetal-like squamous epithelium, glandular epithelium, immature mesenchyme, immature cartilage, and neuronal differentiation. Immunohistochemistry performed in three cases showed expression of vimentin, CD99 (MIC2), and neuron-specific enolase in most cells, and focal expression of cytokeratin, epithelial membrane antigen, alpha fetoprotein, glial fibrillary acidic protein, chromogranin, and synaptophysin. The tumors were consistently negative for beta human chorionic gonadotrophin, neurofilament protein, and leukocyte common antigen. The entities considered in the differential diagnosis were poorly differentiated carcinomas, sarcomas, and olfactory neuroblastoma. We suggest that these neoplasms arise from a primitive cell capable of organized divergent differentiation.
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PMID:Teratocarcinosarcoma of the paranasal sinuses: a clinicopathologic and immunohistochemical study. 967 Aug 29

A case of congenital thyroid teratoma with nodal spread is reported. Primary surgery was performed on a female infant on the 6th day of life. The thyroid mass was removed in toto, and an adjacent 1.2 cm lymph node was also removed. Histology showed solid and cystic teratoma with a variety of elements including prominent neurological tissue that was neuroblastoma-like in places. Residual compressed non-neoplastic thyroid tissue was identified in the subcapsular plane. The lymph node was largely replaced by neuroglial tissue that was cellular in some areas and showed intrasinusoidal growth and some mitotic activity. Recurrent cervical lymphadenopathy gradually developed, commencing a few months after surgery. Excision of cervical nodes was undertaken at 9 months of age. About 13 nodes up to 2 cm in diameter were excised. Most of the specimens consisted of reactive lymph nodes, but in three of the smaller nodes, there were subcapsular and sinusoidal masses of focally cellular neuroglial tissue, again with occasional mitoses. This tissue stained strongly for glial fibrillary acidic protein, in addition to expressing neural markers. The lymph node "deposits" were interpreted as "displaced" lesional tissue rather than metastases in the usual, aggressive sense. The girl remains well at 5 years of age.
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PMID:Congenital thyroid teratoma: a case with persistent neuroglial involvement of cervical lymph nodes. 1046 96

We describe our clinical experience of eight cases of secondary cardiac tumor. The pathology of the tumors were lymphoma (three), Wilms' tumor (two), malignant teratoma (one), neuroblastoma (one), and pleuropulmonary blastoma (one). Metastatic sites were the right atrium in Wilms' tumor and neuroblastoma, the left atrium in pleuropulmonary blastoma and malignant teratoma, and multiple sites in lymphoma. Primary masses in the mediastinum extended directly to the heart (three lymphoma, malignant teratoma, pleuropulmonary blastoma). Wilms' tumor and neuroblastoma showed cardiac metastases through the inferior vena cava. Many cases revealed vague abnormal cardiovascular findings (symptoms in six; physical signs in five). In five cases surgery was performed to relieve the possible obstruction to flow and to identify the pathology (lymphoma in three, Wilms' tumor in one, and malignant teratoma in one). Chemotherapy prior to operation resulted in the disappearance of the intracardiac masses in each case of Wilms' tumor and pleuropulmonary blastoma. All three patients with lymphoma died immediately after operation. Four died of multiple metastases or Pneumocystis pneumonia several months after operation. This study indicates that suspicion of a secondary cardiac tumor is crucial to early diagnosis. Because of the poor postoperative outcome, surgery for secondary cardiac tumors should be done cautiously only in cases with definite hemodynamic decompensation.
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PMID:Secondary cardiac tumor in children. 1055 85


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