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)

Malignant ectomesenchymoma (MEM) is a rare soft tissue tumour believed to arise from a pluripotential migratory neural crest cell and composed of both a mesenchymal element (most often rhabdomyosarcoma) and a neuroectodermal element (often neuroblastoma). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. We report the first case of an orbital MEM, with a review of the 17 cases previously reported from other sites.
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PMID:Primary malignant ectomesenchymoma of the orbit. 162 16

Malignant ectomesenchymoma is a rare soft tissue tumor of the childhood believed to arise from a remnant of pluripotential migratory neural crest cell (ectomesenchym) and composed of both a mesenchymal element (most often embryonal rhabdomyosarcoma) and a neuroectodermal element (ganglioneuroma, schwanomma neuroblastoma or melanocytic cells). Reported sites of origin are the abdomen, perineum or scrotum, the extremities, the middle ear, nasopharynx, face, and neck. Herein we report a new case of an orbital ectomesenchymoma studied by means of histochemistry and immunohistochemistry in order to increase the morphologic and histogenetic knowledge of this peculiar tumor and its significance concerning the differential diagnosis.
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PMID:Primary malignant ectomesenchymoma of the orbit. 906 87

We report a case of neuroblastoma in a patient who had no involvement of the spermatic cord at diagnosis but who developed spermatic cord metastasis 2 months later. The metastasis appeared on sonography as a hypoechoic, highly vascular, fusiform, hard, 14x10x7 mm mass located in the right inguinal canal and extending into the scrotum. The diagnosis of spermatic cord metastasis was confirmed by resection and histopathologic examination. We recommend that the scrotum and spermatic cord be evaluated by high-resolution sonography in children with neuroblastoma, both at the time of diagnosis and during follow-up.
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PMID:Neuroblastoma with spermatic cord metastasis in a child: sonographic findings. 1035 94

Clinically, a neuroblastoma presents mostly as an abdominal mass. Within the tumor, bleeding can be present, sometimes extending in to its surroundings. This case report describes a neuroblastoma, presenting as scrotal hematoma in a newborn boy, which initially raised the suspicion of a torsion testis. In this patient, the bleeding descended through the subcutaneous and muscular tissue into the scrotum. In addition, anemia and jaundice were marked features. This clinical manifestation of a neuroblastoma has not been reported previously. Ultrasonography is advised as the first diagnostic step to exclude retroperitoneal origin of the bleeding. Magnetic resonance imaging and meta-iodobenzyl guanidine scanning were helpful tools in the final diagnostic workup.
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PMID:Scrotal hematoma, anemia, and jaundice as manifestations of adrenal neuroblastoma in a newborn. 1062 73

Bovine seminal ribonuclease (BS-RNase) is an antitumoral active enzyme exhibiting specific antitumoral action against a number of different cancer cell lines. However, its systemic use is limited by its pharmacokinetic properties and antigenicity. Therefore, it was conjugated to polyethylene glycol (PEG) chains to overcome these problems. Measurement of aspermatogenic effects of the preparation after s.c. injection and injection into the scrotum was chosen as a model for the distribution of the enzyme in the body mediated by the linkage to PEG chains. Additionally, the antigenicity of BS-RNase coupled to PEG chains (BS-RNase-PEG) was compared to that of free BS-RNase, as antigenicity is known to be one of the main obstacles in the use of protein-based drugs. BS-RNase-PEG caused aspermatogenic effects after systemic administration to mice in very low concentrations at which free BS-RNase is not effective. Moreover, BS-RNase possessed a very low antigenicity as long as it was coupled to the PEG chains. In order to investigate the antitumoral efficacy of BS-RNase-PEG in vivo, preliminary experiments on the effect of the conjugate on neuroblastoma growth in mice were performed in a UKF-NB-3 xeno-transplantate model, demonstrating a drastically increased anti-tumoral activity of the conjugate compared to the free enzyme.
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PMID:Coupling of the antitumoral enzyme bovine seminal ribonuclease to polyethylene glycol chains increases its systemic efficacy in mice. 1190 7

We report the fourth example of a patient with germline partial trisomy of 2p21-pter and congenital neuroblastoma. The male infant had a dysmorphic facial expression and presented with congenital heart disease, supernumerary nipples, hypospadias, shawl scrotum, hemilateral persistent hyperplastic primary vitreous, and neuroblastoma. His germline karyotype of 46,XY,der(8)t(2;8)(p21;p23.2) was inherited from a maternal-balanced translocation, which indicates that the proto-oncogene MYCN region of 2p24.3 is tripicated in germline cells. A cytogenetic study of the biopsied tumor cells did not show MYCN amplification, but the DNA index was 2.4 and histologic fluorescent in situ hybridization analysis indicated somatic mutation with near-pentaploidy of the tumor cells. This could be an alternative mechanism of MYCN activation in the process of the tumorigenesis of neuroblastoma.
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PMID:Congenital neuroblastoma in a patient with partial trisomy of 2p. 1679 7

Retroperitoneal hemorrhage is known to cause bruising of flanks (Turner's sign), umbilicus (Cullen's sign), pubis and groin (Stabler's sign), upper thigh (Fox's sign), or scrotum (Bryant's sign). Spontaneous adrenal hemorrhage is the most common cause of nontraumatic parietal ecchymosis sign in neonates. Only 20 such neonatal cases have been reported in the literature. Because all of them were of benign etiology, conservative management is generally recommended in the presence of this sign. We report the sign for the first time in a case of spontaneously ruptured adrenal neuroblastoma of newborn. Perinatal physicians need to be aware of this unusual presentation of neonatal neuroblastoma.
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PMID:Stabler's sign revisited in a spontaneously ruptured neuroblastoma of the newborn. 1805 35