Cerebral ganglioneuroblastoma of adult onset: Two patients and a review of the literature

Schipper, Mirjam H; Duinen, Sjoerd G. van; Taphoorn, Martin J.B.; Kloet, Alfred; Walchenbach, R.; Wiggenraad, Ruud; Vecht, Charles J.

doi:10.1016/j.clineuro.2012.03.015

Cited by 17 publications

(20 citation statements)

References 37 publications

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“…The mainstay of treatment approaches includes chemotherapy, surgical resection, and radiotherapy [1,4,5,6,7]. However, neuroblastoma is remarkably heterogeneous.…”

Section: Introductionmentioning

confidence: 99%

The REST Gene Signature Predicts Drug Sensitivity in Neuroblastoma Cell Lines and Is Significantly Associated with Neuroblastoma Tumor Stage

Liang

Tong

Zhao

et al. 2014

IJMS

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Neuroblastoma is the most common and deadly solid tumor in children, and there is currently no effective treatment available for neuroblastoma patients. The repressor element-1 silencing transcription (REST) factor has been found to play important roles in the regulation of neural differentiation and tumorigenesis. Recently, a REST signature consisting of downstream targets of REST has been reported to have clinical relevance in both breast cancer and glioblastoma. However it remains unclear how the REST signature works in neuroblastoma. Publicly available datasets were mined and bioinformatic approaches were used to investigate the utility of the REST signature in neuroblastoma with both preclinical and real patient data. The REST signature was found to be associated with drug sensitivity in neuroblastoma cell lines. Further, neuroblastoma patients with enhanced REST activity are significantly associated with higher clinical stages. Loss of heterozygosity on chromosome 11q23, which occurs in a large subset of high-risk neuroblastomas, tends to be correlated with high REST activity, with marginal significance. In conclusion, the REST signature has important implications for targeted therapy, and it is a prognostic factor in neuroblastoma patients.

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“…The mainstay of treatment approaches includes chemotherapy, surgical resection, and radiotherapy [1,4,5,6,7]. However, neuroblastoma is remarkably heterogeneous.…”

Section: Introductionmentioning

confidence: 99%

The REST Gene Signature Predicts Drug Sensitivity in Neuroblastoma Cell Lines and Is Significantly Associated with Neuroblastoma Tumor Stage

Liang

Tong

Zhao

et al. 2014

IJMS

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“…Out of the 50 adult cases reviewed, 25 patients had dopamine and its metabolites (homovanillic acid (HVA) and vanillylmandelic acid (VMA)) levels available, of which 12 cases had normal levels, whilst the other 12 cases showed increased urinary values for HVA, VMA or both. 4,5 Except for a 24-h history of loin pain, our patient was completely asymptomatic. As she had raised urinary normetadrenaline and markedly raised urinary dopamine and 3-methoxytyramine, the differential diagnosis was either paraganglioma or phaeochromocytoma.…”

Section: Discussionmentioning

confidence: 79%

“…Surgical resection appears to be the only curative regimen, with the option of chemo-radiotherapy when the resection had been incomplete or tumour recurrence is seen. 4,10 In most cases, the metastases were seen locally and almost half did not show metastases. 4 The mean survival of adult patients is 13 months with better prognosis noted in those who had complete clearance at surgery.…”

Section: Discussionmentioning

confidence: 99%

First reported case of ganglioneuroblastoma in pregnancy and a review of the literature

et al. 2014

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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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“…The sites of origin of ganglioneuroblastoma include adrenal medulla, extra-adrenal retroperitoneum, posterior mediastinum, the neck, and pelvis. 1 More than 90% of all ganglioneuroblastomas are seen in children under 5 years old, and it is rare in adults. 2 Only 11 cases have been observed in the adult adrenal glands, and we report here on 1 more adult patient with adrenal ganglioneuroblastoma.…”

Section: Introductionmentioning

confidence: 99%

Adult adrenal ganglioneuroblastoma: A rare case report

Ding

Hou

et al. 2015

CUAJ

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Ganglioneuroblastoma is an uncommon malignant tumour, and it is extremely rare in adults. A 27-year-old woman was admitted to hospital complaining of commitment left loin pain for 7 months accompanied with fever for 1 day. Computed tomography (CT) scan shows a huge cystic solid mass among the rear of the pancreatic body and tail, inside of the spleen, and the top of the left kidney. Hormone examinations showed that the serum levels of glucocorticoid, aldosterone, norepinephrine, and epinephrine were normal. However, neuron-specific enolase (NSE) was significantly higher (289.46 ng/mL, normal level <16.3 ng/mL). Adrenal mass resection was scheduled. However, intraoperative separation was very difficult and adrenal tumour resection, resection of the pancreatic body and tail, left nephrectomy, and splenectomy were carried out. Pathological diagnosis was ganglioneuroblastoma.

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Cerebral ganglioneuroblastoma of adult onset: Two patients and a review of the literature

Cited by 17 publications

References 37 publications

The REST Gene Signature Predicts Drug Sensitivity in Neuroblastoma Cell Lines and Is Significantly Associated with Neuroblastoma Tumor Stage

The REST Gene Signature Predicts Drug Sensitivity in Neuroblastoma Cell Lines and Is Significantly Associated with Neuroblastoma Tumor Stage

First reported case of ganglioneuroblastoma in pregnancy and a review of the literature

Adult adrenal ganglioneuroblastoma: A rare case report

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