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)

A case of meningeal carcinomatosis associated with cerebral metastases from an adrenal neuroblastoma is described. The clinical picture was ushered-in by bilateral sciatic pain in a 50 years old female and was followed by rapidly progressive sensory-motor deficits of the arms and legs, leading to flaccid quadriplegia associated with paralysis of cranial nerves and episodes of mental confusion. Death occurred 4 months alter, in cardiac failure. At autopsy, a bilateral tumor of the adrenal glands was found. No metastases were detected anywhere except in the central nervous system. Histology identified the tumor as a neuroblastoma; meningeal carcinomatosis, radicular infiltration by tumor cells and parenchimal metastases were found in the central nervous system. Neuroblastoma is typically a tumor of childhood, only 13% of them being found in adult's according to Russell and Rubinstein. Meningeal metastases from adrenal neuroblastoma have not hitherto been reported in the literature. In our opinion, the most likely mode of spread of tumor cells to the central nervous system was hematogenous because of the presence of small multiple intraparenchimal metastases; however, possible spread through the perineural lymphatics, as proposed by others, cannot be excluded, due to the prominent localization of tumor cells at spinal roots level. The main differential diagnostic problems (paraneoplastic neuropathy (Wyburn-Mason) and infectious subacute or chronic meningitis) are discussed. The authors stress the emportance of complete cerebro-spinal fluid examination including a careful search for tumor cells.
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PMID:[Meningeal carcinomatosis: clinical and anatomical study of a case of suprarenal neuroblastoma (author's transl)]. 6

A 21-day-old girl presented with a left subdiaphragmatic retroperitoneal mass discovered on maternal ultrasound at 23 weeks' gestation. The clinical impression was neuroblastoma, and preoperative workup showed no for metastatic disease. Histologically, the lesion proved to be an extralobar pulmonary sequestration. We present this case to remind pathologists of the occurrence of subdiaphragmatic pulmonary sequestration and its potential clinical confusion with other more familiar lesions that may occur in this location in a neonate.
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PMID:Subdiaphragmatic extralobar pulmonary sequestration. 202 27

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5

Ifosfamide was given to 61 patients with malignant solid tumors diagnosed before the age of 21 years. In this phase II study, all patients received 1.6 g/m2/day X 5 iv over 15 minutes followed by mesna at a dose of 400 mg/m2 iv at 15 minutes and 4 and 6 hours after ifosfamide. Responses were observed in five of 15 patients with osteosarcoma, two of ten with neuroblastoma, two of six with Wilms' tumor, two of five with rhabdomyosarcoma, four of eight with other soft tissue sarcomas, one of one with retinoblastoma, one of two with germ cell tumors, one of one with B-cell lymphoma, and one of one with a primitive neuroectodermal tumor. Fifty-nine of 61 patients had received prior alkylating agent therapy which included cyclophosphamide, cisplatin, mechlorethamine, melphalan, or dacarbazine. Fourteen of 19 responses developed in patients whose tumors were resistant to treatment with cyclophosphamide. A patient with malignant Schwannoma who had received no prior chemotherapy developed a complete response which lasted 12 months. A patient with brain metastases of osteosarcoma has had complete response for greater than 2 years. Complete response was also observed in a patient with B-cell lymphoma. Toxicity consisted of mild to moderate nausea and vomiting, transient reversible myelosuppression, occasional elevation of serum BUN or creatinine, and transient neurotoxicity characterized by somnolence, confusion, weakness, tremor, hallucinations, or seizures. We conclude that ifosfamide is an important alkylating agent without apparent complete cross-resistance with cyclophosphamide, and as such should be further investigated for determination of its activity in patients with pediatric neoplasms and considered for incorporation into phase II-III trials for certain tumors.
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PMID:Phase II trial of ifosfamide in children with malignant solid tumors. 310 34

Esthesioneuroblastoma is an uncommon tumor arising from the olfactory epithelium of the nasal vault. Differentiation from other tumors is often difficult, but techniques like immunocytochemistry and electron microscopy can help to distinguish a neuroblastoma from a malignant lymphoma with certainty. A patient is presented in whom difficulty was experienced in deciding on the diagnosis; the pathological data are presented to illustrate why confusion about the finite diagnosis can occur despite the use of these techniques. The initial diagnosis was a malignant lymphoma of the poorly differentiated lymphocytic type. The disease responded well to chemotherapy with cyclophosphamide, vincristine, procarbazine, and doxorubicin (COPA). When tumor recurred, rebiopsy of the small cell tumor was considered to be esthesioneuroblastoma, and the patient was treated with cyclophosphamide, vincristine, dacarbazine, and radiotherapy. The patient is now disease-free and has been without evidence of disease for more than 2.5 years; it is more than 5 years since the initial diagnosis of a small cell malignant neoplasm was made. This report illustrates the problems of diagnosis and treatment of this rare condition.
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PMID:Esthesioneuroblastoma or malignant lymphoma? A case report. 318 32

Qualitative aspects of protein synthesis in organelles and intact cultured cells of brain origin were compared to clarify the distinction between synaptosomal and mitochondrial protein synthesis. Brain mitochondria and synaptosomes were isolated either on a traditional Ficoll-sucrose gradient or by a new Percoll gradient procedure, and were incubated in an amino acid incorporation system containing [35S]methionine, then electrophoresed on gradient slab gels. Autoradiography of the gels revealed that in the presence of cycloheximide both mitochondria and synaptosomes synthesized at least 17 proteins in the 6,000-50,000 MW range, and that incubation with chloramphenicol reduced or eliminated these bands. With minor variation these patterns in the low-molecular-weight region also resembled patterns obtained from cycloheximide-inhibited rat liver mitochondria and intact brain cells (cultured glia, glioma, and neuroblastoma). In the higher molecular weight region of the gels (greater than 50,000) banding patterns were more complex and tended to differ between organelles and intact cells. These polypeptides probably reflect nonmitochondrial protein synthesis, and their variable response to inhibitors may account for confusion in the literature with regard to the effects of inhibitors of protein synthesis in brain mitochondria and synaptosomes.
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PMID:Comparison of protein synthesis in mitochondria, synaptosomes, and intact brain cells. 396 18

There has been an exponential growth in interest in purinoceptors since the potent effects of purines were first reported in 1929 and purinoceptors defined in 1978. A distinction between P1 (adenosine) and P2 (ATP/ADP) purinoceptors was recognized at that time and later, A1 and A2, as well as P2x and P2y subclasses of P1 and P2 purinoceptors were also defined. However, in recent years, many new subclasses have been claimed, particularly for the receptors to nucleotides, including P2t, P2z, P2u(n) and P2D, and there is some confusion now about how to incorporate additional discoveries concerning the responses of different tissues to purines. The studies beginning to appear defining the molecular structure of P2-purinoceptor subtypes are clearly going to be important in resolving this problem, as well as the introduction of new compounds that can discriminate pharmacologically between subtypes. Thus, in this review, on the basis of this new data and after a detailed analysis of the literature, we propose that: (1) P2X(ligand-gated) and P2Y(G-protein-coupled) purinoceptor families are established; (2) four subclasses of P2X-purinoceptor can be identified (P2X1-P2X4) to date; (3) the variously named P2-purinoceptors that are G-protein-coupled should be incorporated into numbered subclasses of the P2Y family. Thus: P2Y1 represents the recently cloned P2Y receptor (clone 803) from chick brain; P2Y2 represents the recently cloned P2u (or P2n) receptor from neuroblastoma, human epithelial and rat heart cells; P2Y3 represents the recently cloned P2Y receptor (clone 103) from chick brain that resembles the former P2t receptor; P2Y4-P2Y6 represent subclasses based on agonist potencies of newly synthesised analogues; P2Y7 represents the former P2D receptor for dinucleotides. This new framework for P2 purinoceptors would be fully consistent with what is emerging for the receptors to other major transmitters, such as acetylcholine, gamma-aminobutyric acid, glutamate and serotonin, where two main receptor families have been recognised, one mediating fast receptor responses directly linked to an ion channel, the other mediating slower responses through G-proteins. We fully expect discussion on the numbering of the different receptor subtypes within the P2X and P2Y families, but believe that this new way of defining receptors for nucleotides, based on agonist potency order, transduction mechanisms and molecular structure, will give a more ordered and logical approach to accommodating new findings. Moreover, based on the extensive literature analysis that led to this proposal, we suggest that the development of selective antagonists for the different P2-purinoceptor subtypes is now highly desirable, particularly for therapeutic purposes.
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PMID:Purinoceptors: are there families of P2X and P2Y purinoceptors? 772 57

Cervical neuroblastoma (CNB) is relatively rare, accounting for less than 5% of these tumors. Because it arises from the cervical sympathetic chain, complete resection will leave the child with Horner's syndrome in a high proportion of cases. Advances in technology have allowed for the development of diagnostic and imaging modalities more specific to the disease. One of these has been the advent of radiolabeled meta-iodobenzylguanidine (MIBG) to assess the primary tumor and focal metastatic involvement. This nuclide is also taken up by normal salivary-gland tissue; this may be altered, however, in the presence of sympathetic denervation. We present a case of a primary CNB associated with Horner's syndrome, which led to confusion in interpretation of the subsequent MIBG scan. We alert the reader to potential pitfalls in the use of this examination in this disease entity.
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PMID:Pitfalls in the interpretation of MIBG scans in cervical neuroblastoma. 1095 91

Carboplatin is currently recommended to be dosed according to renal function. In adults, dosing by the Calvert formula is based on evidence that carboplatin clearance closely parallels glomerular filtration rate. Several studies have attempted to validate the Calvert formula and its derivations in pediatrics, but no final consensus has been achieved. As a result, different versions of the original formula exist in the pediatric literature. Other factors may also contribute to confusion when applying the formula to young patients, including the manner in which renal function is measured and reported. We describe how misinterpretation of the Calvert formula resulted in carboplatin overdosing in 2 pediatric patients with high-risk neuroblastoma undergoing peripheral blood stem cell transplantation. Measures to avoid such errors have been instituted.
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PMID:Misinterpretation of a Calvert-derived formula leading to carboplatin overdose in two children. 1452 96

Cases of cerebral neuroblastoma or supratentorial primitive neuroectodermal tumor with malignant gliomatous components are relatively uncommon. Less frequent is the combination of these 2 elements with a mesenchymal component. This is a case report of a lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation occurring in a 48-year-old woman. She presented with headaches and confusion. A right parietal lobe mass was excised and subsequently recurred, requiring additional surgery 10 months later. The patient died 13 months after initial surgery. Histologic findings showed a proliferation of small rounded synaptophysin-positive neural cells consistent with neuroblastoma. These cells were arranged against a benign lipomatous background. The second resection consisted primarily of glioblastomatous-like tissue with intermixed lipomatous component. The glioblastoma component was marked by prominent cellularity, moderate nuclear pleomorphism, readily identifiable mitotic activity, vascular proliferative changes, and necrosis. The glioblastomatous component of the tumor demonstrated glial fibrillary acidic protein immunoreactivity. A Ki-67 labeling index of 18.9% was noted in the initial resection. The literature on similar-appearing lesions is reviewed.
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PMID:Lipomatous supratentorial primitive neuroectodermal tumor with glioblastomatous differentiation. 1911 80


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