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)

Three cases of secondary (therapy-related) hematologic malignant conditions were identified among 95 children as old as 18 years of age; the cases were diagnosed between 1984 and 1990 and consisted of acute lymphoblastic leukemia, acute myeloid leukemia (AML), and myelodysplastic syndrome (MDSs). They constituted 10% of all new cases of AML and MDS seen at the University Hospitals of Cleveland during this time and were not related to congenital factors. The primary malignant conditions were malignant thoracopulmonary tumor (Askin tumor), neuroblastoma, and Burkitt's lymphoma. The secondary hematologic disorders all showed a prominent monocytic component: acute monocytic leukemia, MDSs evolving to acute myelomonocytic leukemia, and chronic myelomonocytic leukemia. The mean interval between treatment for the primary malignant condition and the onset of secondary disease was 36 months. All had received cyclophosphamide and an epipodophyllotoxin for the primary tumor; two were treated with radiation therapy. Cytogenetic abnormalities included del(5), del(13), t(1;6), and t(9;11)(p22[symbol:see text]3). The survival time after the onset of secondary disease was short.
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PMID:Myelodysplastic syndrome and acute myeloid leukemia after treatment for solid tumors of childhood. 837 35

The Askin tumor is one of a group of small round cell tumors and is found mainly in the thoraco-pulmonary region of children and young adults. The treatment of the patient and the differentiation of this tumor from other small round cell tumors, especially Ewing's sarcoma and neuroblastoma by light microscopy, immunohistochemical methods, and electron microscopy is discussed.
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PMID:Diagnostic features and treatment of the Askin tumor--malignant small cell tumor of the thoraco-pulmonary region: a case report. 838 Dec 14

A case of thoracic neuroblastoma arising at the anterior mammillary line from the inner surface of the second and third right rib in a 17-month-old male child is reported. Primary surgery allowed excision of the mass. According to the histologic, histochemical, and molecular features of the tumor, all consistent with the diagnosis of neuroblastoma stage 2, the patient underwent adjuvant chemotherapy and, after 2 years, he is alive and disease free at follow-up. In view of different clinical and therapeutical approaches, only a strict adherence to histopathologic criteria allows differentiation of neuroblastoma, even in a very unusual anatomical site, from other small-cell, peripheral neoplasms presumably arising from the embryonic neural crest, such as Askin's tumor, neuroepithelioma, and ectomesenchymoma. The site of origin of these tumors is an important diagnostic clue but can be misleading: only careful microscopic evaluation and molecular analysis lead to incontrovertible diagnosis and yield correct clinical management.
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PMID:Ectopic neuroblastoma of the thoracic wall: a case report. 841 94

Peripheral neuroepitheliomas are rare malignant tumors of presumed neural crest origin, arising outside the central and sympathetic nervous system. We present herein a case of a young man with diffuse peripheral neuroepithelioma of the abdominal cavity. Although we twice attempted a debulking procedure on this patient, and treated him with alternating VIP-CAV combined chemotherapy, his disease showed a relentless course without responding to any treatment. The development of peripheral neuroepithelioma in the abdominal cavity is an extremely rare occurrence. These rare malignant tumors, of presumed neural crest origin, arise outside the central and sympathetic nervous systems (1). Other names for this tumor are: extracranial primitive neuroectodermal tumor (PNET), Askin tumor, peripheral or adult neuroblastoma (2,3,4). We present a case of a young man with diffuse development of this tumor in the abdominal cavity.
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PMID:Peripheral neuroepithelioma developing in the abdominal cavity. 892 Jul 88

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

The term primitive neuroectodermal tumor (PNET) names a group of malignant neoplasms of presumed neural crest origin; they are composed of round small cells, and may be centrally located or outside. The former are currently seen in childhood, mostly in reference to the posterior fossa, and include medulloblastoma and neuroblastoma. Outside of the central nervous system includes peripheral neuroectodermal tumor of the bone (PNET-B, Tefft's tumor), Askin's tumor, and even Ewing's sarcoma. We describe a case in a young adult, with extremely unusual spinal localization, in which histopathological and immunohistochemical analysis were done; also, a review of some features is presented clinical-histopathological issues concerning related.
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PMID:[Spinal primitive neuroectodermal tumor]. 1032 53

A peripheral primitive neuroectodermal tumor arising from the abdominopelvic cavity is reported in a 24-year-old young male without any previous remarkable pathology. He was admitted to our hospital with complaints of urinary symptoms (acute urinary retention) and mild intestinal occlusion that had been present for three months. Physical examination and CT scan revealed a pelvic mass occupying the entire pelvic cavity. The diagnostic workup included a CT-guided biopsy which defined the tumor as a sarcomatous type. Radical surgery was performed including tumor resection, pelvic exenteration (bladder and prostate gland) and urinary and fecal diversion. Adjuvant chemotherapy (VAIA) was delivered once the histology was confirmed. We reviewed the available literature focusing on the varied nomenclature of this tumor (peripheral neuroepithelioma, Askin's tumor, Ewing's extraosseous tumor, peripheral adult neuroblastoma, peripheral primitive extracranial neuroectodermal tumor (PPNET), the clinical features, the role of diagnostic imaging techniques, pathologic assessment and controversial therapeutic management. In addition, the prognosis and survival of this rare condition were analyzed.
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PMID:Peripheral primitive neuroectodermal tumor (PPNET) of pelvic origin: report of a case arising from an unusual location. 1140 Dec 7

Peripheral primitive neuroectodermal tumor (PNET) and Ewing's sarcoma (ES) constitute a unique group of small round cell tumors in childhood and young adults that are characterized by the same chromosomal translocation t(11;22)(q24;q12). Recently, the expression of neurotrophin receptors has been found in various human tumors including PNET/ES, but the functional significance of these receptor expressions has not been documented in PNET/ES. In the present study, we investigated the biologic effects of trkA neurotrophin receptor activation by nerve growth factor (NGF) in a newly established Askin tumor cell line, JK-GMS, which constitutively expresses a high level of trkA. The activation of trkA induced differentiation and inhibited the growth of JK-GMS cells, which was characteristically associated with down-regulation of c-myc and N-myc mRNA expression. NGF did not exert significant changes in two different PNET/ES cell lines, CADO-ES1 and RD-ES, which did not express detectable levels of trkA. The biologic effects mediated by NGF were abrogated by treatment of the cells with K-252a, and the treatment with brain-derived neurotrophic factor did not affect the biologic behavior of JK-GMS cells, indicating that the effects are trkA specific. The results observed were quite similar to those of neuroblastoma cells, another childhood tumor of neural crest origin. Overall findings strongly suggest that the trkA-mediated signaling pathway plays a crucial role in controlling the basic biologic properties of JK-GMS cells.
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PMID:Activation of trkA induces differentiation and inhibits the growth of JK-GMS Askin tumor cells. 1185 May 35

Chest wall lesions in childhood include a wide range of pathologies. Benign lesions include lipoma, neurofibroma, lymphangioma, haemangioma and mesenchymal hamartoma. Malignant lesions include neuroblastoma, rhabdomyosarcoma, Ewings sarcoma, Askin tumour and primitive neuroectodermal tumours. Manifestations of systemic diseases such as leukaemia, lymphoma, Langerhans cell histocytosis and infections such as tuberculosis and actinomycosis may also cause chest wall lesions. The imaging characteristics of the above are reviewed but only a minority of lesions show diagnostic imaging characteristics. Most lesions require biopsy and histopathological examination for definitive diagnosis. The role of different imaging modalities is discussed, with an emphasis on magnetic resonance imaging for demonstrating lesion morphology and local spread, with computed tomography and nuclear medicine being used mainly to assess remote disease.
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PMID:Chest wall lesions. 1245 4

Peripheral primitive neuroectodermal tumour (PNET)/Ewing's sarcoma (ES) and neuroblastoma (NB) are related tumours of neural crest origin with primitive neural characteristics. Fibroblast growth factor 2 (FGF2) is a critical signalling molecule for primitive neural crest cells. The treatment of NB cells with FGF2 variably affects biological characteristics such as growth and differentiation, while in PNET/ES, FGF2 predominantly induces apoptosis. The JK-GMS Askin tumour cell line can be induced to differentiate upon treatment with nerve growth factor (NGF), indicating the integrity of the cellular machinery necessary for differentiation. The present study assesses whether FGF2 can induce differentiation in JK-GMS cells. JK-GMS cells expressed high-affinity FGF receptors (FGFRs), and treatment with FGF2 induced phosphorylation of FGFR1 together with activation of extracellular signal-regulated kinases (ERK1/ERK2) and c-Jun N-terminal kinase (JNK). Subsequent biological effects were growth inhibition, neuronal differentiation, and apoptosis, and these changes were associated with increased expression of neurofilaments, reduction of c-myc and bcl-2 expression, and activation of caspase 3. Treatment of the cells with a specific inhibitor of the MAPK/extracellular signal-regulated kinase (MEK)-1, PD98059, predominantly inhibited the effects of FGF2 on growth, differentiation, and apoptosis, while an inhibitor of JNK reduced apoptosis, indicating that the ERK1/2 and JNK pathways are critical components of FGF2-mediated effects in JK-GMS cells. Additional comparative analyses of FGF2-mediated effects in two ES cell lines (CADO-ES, RD-ES) and a PNET cell line (SK-N-MC) showed pronounced differentiation in SK-N-MC, but not in CADO-ES or RD-ES cells. This study demonstrates that FGF2 can induce neuronal differentiation of PNET including Askin tumour. These findings clearly indicate that the FGF2-mediated signalling pathway plays a critical role in controlling the major properties of PNET cells and may provide a potential therapeutic target for PNET.
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PMID:Fibroblast growth factor 2 induces differentiation and apoptosis of Askin tumour cells. 1469 27


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