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)

We report a case of a 12-month-old girl with stage 3 neuroblastoma composed of 2 distinct clones in the adrenal primary tumor. One clone showed neuroblastoma, poorly differentiated subtype with a low mitosis-karyorrhexis index (favorable histology), and the other was neuroblastoma, poorly differentiated subtype with a high mitosis-karyorrhexis index (unfavorable histology), according to the International Neuroblastoma Pathology Classification. Fluorescent-labeled in-situ hybridization using the formalin-fixed, paraffin-embedded material showed that the MYCN oncogene was amplified in the latter clone but not in the former clone. Lymph nodes from ipsilateral and contralateral sides contained metastatic neuroblastoma of the latter clone. It is well documented that tumors in the Ganglioneuroblastoma, nodular (composite, Schwannian stroma-rich/stroma-dominant and stroma-poor) category are composite and composed of multiple clones. To our knowledge, however, this is the 1st case report of composite tumor with biologically favorable and unfavorable clones in the Neuroblastoma (Schwannian stroma-poor) category.
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PMID:A case of composite neuroblastoma composed of histologically and biologically distinct clones. 1753 87

Ganglioneuroblastoma is a rare tumor variant of neuroblastoma. Only five cases have been observed in the adult brain, and we report here on two more adult patients with cerebral ganglioneuroblastoma. Additionally, a review was carried out on all 50 published adult cases with ganglioneuroblastoma, located in the adrenal gland (9), mediastinum (8), retroperitoneal area (7), the brain parenchyma (7), or the spinal cord (3). Median age at onset was 39 years, and 52% of patients were female. For extracranial locations, treatment usually consisted of surgery followed by radiotherapy and adjuvant chemotherapy. Of the cases with cerebral involvement only one patient did not receive any treatment. The other six patients underwent surgical resection and radiation therapy, in four cases followed by chemotherapy with temozolomide. The median survival of cerebral ganglioneuroblastomas was 14 months and did not differ from the whole group of ganglioneuroblastomas (12 months). For cerebral ganglioneuroblastoma, the preferred regimen would seem to be neurosurgical removal, followed by chemoradiotherapy including temozolomide.
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PMID:Cerebral ganglioneuroblastoma of adult onset: two patients and a review of the literature. 2251 May 1

Ganglioneuroblastoma (GNB) is a cancer of the peripheral sympathetic nervous system and represents the 30% of cases of Neuroblastoma. When originates from thoracic ganglia it may appear very late, with severe symptoms like respiratory distress or neurological dysfunctions. We present an incidental diagnosis of thoracic GNB, discovered by a chest radiography during a recurrent wheezing unresponsive to bronchodilators. The early diagnosis allowed a precocious treatment probably improving the outcome.
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PMID:Incidental diagnosis of thoracic ganglioneuroblastoma in a 3-year-old female with wheezing. 2297 59

The most common malignant neurogenic tumors in children are neuroblastic tumors, classified as ganglioneuroblastoma or neuroblastoma. Ganglioneuroblastomas usually occur at the sympathetic ganglia in the mediastinum, whereas neuroblastomas occur in the abdominal cavity. We describe a case of large posterior mediastinal ganglioneuroblastoma extending from the aortic arch to the left renal hilum in a 17-year-old boy. Despite chemotherapy, post-treatment computed tomography showed disease progression. The patient underwent a thoracolaparotomy incision and excision of the tumor. These malignant mediastinal tumors can potentially grow to a very large size. If alternative treatment has failed, resection can be accomplished with relative safety.
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PMID:Giant thoracoabdominal ganglioneuroblastoma in a 17-year-old patient. 2488 35

Ganglioneuroblastoma is a variant of neuroblastoma tumours with mature ganglion cell differentiation which occurs commonly in cervical, mediastinal and retroperitoneal locations. Approximately 90% of ganglioneuroblastomas are seen in children younger than five years old. There are 50 adult cases of ganglioneuroblastomas reported to date. Our patient is the 51st case and she is the first to be diagnosed in pregnancy. Our patient's tumour site was the thoraco-abdominal retroperitoneal space adjacent to her kidney. This tumour is generally diagnosed incidentally or by compression presentations, i.e. pain or neurological symptoms. Our patient's ultrasound scan appearances triggered suspicion of an atypical mass after presenting with loin pain. Caesarean section with tumour removal in the same session is preferred if tumour is small and localized. However in this case, there was uncertainty regarding the extent of the tumour. Therefore, the patient underwent thoracotomy after delivery to excise the thoraco-abdominal tumour, which extended from the level of the T9 to L2 vertebrae.
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PMID:First reported case of ganglioneuroblastoma in pregnancy and a review of the literature. 2751 39

Chronic abdominal pain is a very common complaint in the population of children and adolescents. In most cases, the usual cause are functional gastrointestinal disorders. However, in a few percent of children, the reason for persistent chronic stomach pain are organic diseases occurring in the gastrointestinal tract, as well as parenteral diseases, including uro-genital tract abnormalities, inflammation of the lower respiratory tract and cancer processes. Among organic causes, in addition to those commonly encountered, such as: intolerances and food allergies, gastroesophageal reflux disease, chronic gastritis or duodenitis, or urinary tract infections, the diagnosis should also include very rare causes, for example, neoplastic diseases, among them tumors of the abdominal cavity. In the case described in the present article, a 6-year-old girl with chronic abdominal pain, symptoms of gastro-oesophageal reflux and constipation, and previously diagnosed food allergy and lactose intolerance, was referred for widening the diagnosics due to the occurrence of alarm symptoms. The nodule revealed in the chest X-ray, in CT scan, turned out to be a paravertebral tumor with the specific features of neuroblastoma. After a macroscopically complete tumor resection based on the result of histopathological examination, the diagnosis of ganglineuroblastoma was established. The presence of alarm symptoms in anamnesis and physical examination in children with abdominal pain suggests a higher probability of the organic origin of the disease and should always lead to extended diagnostics. Ganglioneuroblastoma is a very rare disease, in most cases is located primarily in the abdominal cavity, and the most common associated symptom is abdominal pain.
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PMID:[Ganglioneuroblastoma in a child with chronic abdominal pain - a case report]. 3063 34

Ganglioneuroblastoma, neuroblastoma, and ganglioneuroma (GN) are the tumors that arise from the neural crest cells. Of these, GN has the most benign origin without metastatic potential. The most common sites of their origin are the posterior mediastinum and retroperitoneum. Although the imaging studies, including CT, are available to detect these tumors, the definitive diagnosis can only be made by histological examination. We present a case of a 40-year-old woman with a retroperitoneal GN causing longstanding, gradually increasing, uncontrolled abdominal pain due to its pressure effect on the pancreas, duodenum, and right kidney with the displacement of the inferior vena cava. An exploratory laparotomy was performed, and the mass was removed. Histopathology confirmed the benign nature of the mass (a GN). These tumors are rarely malignant and mostly asymptomatic. However, in our case, abdominal pain was affecting the patient's life. After a discussion with the patient, an elective surgical procedure was performed, and the patient was symptom-free postoperatively and able to resume her regular routine.
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PMID:Retroperitoneal Ganglioneuroma in a Patient Presenting With Vague Abdominal Pain. 3278 73


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