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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The patients were infant male twins born by cesarean delivery following a healthy pregnancy at 36 weeks' gestation to unrelated parents. At 4 months of age, twin 2 presented with hepatomegaly and a right suprarenal mass. Resection of an adrenal tumor and a liver tumor biopsy were performed. Twin 1 had no symptoms at 4 months of age. Screening by abdominal ultrasonography showed multiple masses in the liver but no adrenal mass. Metaiodobenzylguanidine scintigraphy showed positive findings in multiple liver masses. A laparoscopic biopsy for a liver tumor was performed. All primary tumor and liver tumor specimens from twin 2 and the liver tumor of twin 1 had the same histologic classification of neuroblastoma and nearly identical genetic aberrations, including a chromosome gain or loss using array-comparative genomic hybridization. From these clinical and pathologic findings and genetic analyses, we strongly demonstrate the transplacental metastatic spread from twin 2 to twin 1. In the literature, 9 pairs of concordant twin neuroblastomas, including the current twin, have been presented; and the clinical findings of 5 twin pairs may represent placental metastases from one twin with congenital neuroblastoma to the other twin. This study is the first report presenting the possibility of twin-to-twin metastasis in monozygotic twins with neuroblastoma based on an analysis of the clinical features and genetic aberrations.
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PMID:Concordance for neuroblastoma in monozygotic twins: case report and review of the literature. 2112 36

A girl presented with a right adrenal mass, and multiple hepatic lesions and subcutaneous nodules 3 months after complete resection of left adrenal neuroblastoma in the neonatal period. She was treated with six cycles of chemotherapy and is well after 13 months' follow-up. This is the first case report of heterochronous bilateral adrenal stage 4S NB.
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PMID:Heterochronous bilateral adrenal neuroblastoma: stage 4S in early infancy following resection of stage I lesion in the neonatal period. 2200 5

Neuroblastoma, an embryonal tumour arising from the sympathetic nervous system, is the most common neonatal malignancy accounting for >20% of neonatal cancers. It may present as an antenatal adrenal mass or more commonly with localised or metastatic (4s/Ms) disease, which is usually low risk with a very good clinical outcome. Around 20% of neonatal neuroblastoma presents with spinal cord compression requiring prompt diagnosis and treatment with steroids and chemotherapy to relieve the cord compression. Patients with stage Ms disease without life- or organ-threatening symptoms or adverse genetic features (MYCN amplification or segmental chromosomal abnormalities) can be safely observed for spontaneous regression which may also occur with other localised neonatal neuroblastomas. Universal mass screening for neuroblastoma is not indicated but targeted screening of infants at risk of hereditary neuroblastoma with germline ALK or PHOX2B mutations is appropriate. Future studies will be aimed at observing more patients without adverse genetics or life-threatening features.
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PMID:Neonatal neuroblastoma. 2267 27

An adrenal mass in a newborn usually suggests a diagnosis of neuroblastoma or hemorrhage. A 6-week-old girl was referred for evaluation of a left adrenal mass. The initial findings were most consistent with an adrenal hemorrhage. On surveillance imaging, the lesion was characterized as a complex cystic mass. The patient subsequently developed lesions in the right adrenal gland and liver as well as multiple cutaneous lesions. Although the contralateral adrenal and hepatic lesions resolved spontaneously, the left adrenal and cutaneous lesions persisted. Subsequent imaging revealed that the mural components of the left adrenal mass had increased in size, and at age 13 months, a laparoscopic left adrenalectomy was performed. A benign adrenal adenoma with myxoid changes was reported on surgical pathology. This is the first reported case in a newborn.
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PMID:Novel report of an adrenal adenoma in a newborn. 2281 10

A case of fetal neuroblastoma of the right adrenal gland, with rapid development of hydrops fetalis due to catecholamine-induced cardiomyopathy, is reported. A fetus with a right suprarenal mass detected during ultrasonography at 32 weeks gestation progressively developed into hydrops fetalis by 35.2 weeks gestation. An emergent cesarean section was performed. At birth, the female neonate was hypertensive, with markedly elevated catecholamine levels; echocardiography showed poor contractility. Morphine, human atrial natriuretic peptide, milrinone, nitroprusside and dobutamine were initiated and her blood pressure was maintained within the normal range and her cardiac contractility improved 2 weeks after birth. Neuroblastoma cells were detected in the placenta, resulting in the right adrenal mass being diagnosed as a neuroblastoma. She was well, and the mass diminished in size within 4 months, without surgery. A fetus with suspected neuroblastoma, indicated by a suprarenal mass, should be managed with appropriate consideration of hydrops.
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PMID:A catecholamine-secreting neuroblastoma leading to hydrops fetalis. 2477 2

The Children's Oncology Group (COG) study ANBL00P2 showed that expectant observation of patients younger than six months of age with perinatal neuroblastoma presenting as a small adrenal mass yields excellent overall survival and spares surgical resection to the majority of patients. We report a 5-year-old female who was initially diagnosed with a perinatal neuroblastoma. The patient was observed on COG study ANBL00P2. By nine months of age she had no ultrasonographic or biochemical evidence of disease. She presented four years later with abdominal pain and was found to have high-risk stage 4 MYCN amplified neuroblastoma.
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PMID:Relapsed perinatal neuroblastoma after expectant observation. 2517 38

A 20-month-old female presented with respiratory distress and a right adrenal mass extending into the inferior vena cava and right atrium. The mass was initially thought to be neuroblastoma. Pathology later revealed adrenocortical carcinoma. Inferior vena cava extension is far more common in adrenocortical carcinoma than neuroblastoma, and its presence should prompt clinical and laboratory evaluation for an adrenocortical tumor. The genetic findings in TP53 associated with this disease are discussed.
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PMID:Adrenocortical carcinoma with extension to the inferior vena cava and right atrium: 20-month-old girl with TP53 mutation. 2739 16

We report the imaging appearances of a case of pathologically proven, neonatal neuroblastoma 4S with diffuse hepatic metastatic involvement at presentation. Patient had an abnormal appearing liver both by ultrasound and MR. There was no evidence for associated adrenal tumor by imaging. Lack of an associated adrenal mass led to initial misinterpretation of diffuse hepatic accumulation of MIBG seen with radionuclide scintigraphy. To the best our knowledge, this is the first report of metastatic neonatal 4S neuroblastoma without an adrenal (or extra-adrenal) primary identified either on pre- or post-natal imaging.
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PMID:Neonatal neuroblastoma 4s with diffuse liver metastases (Pepper syndrome) without an adrenal/extraadrenal primary identified on imaging. 2987 90

A 15-month-old girl was initially referred for endocrine evaluation for clitoromegaly and subsequently found to have an adrenal mass that tracked along the paravertebral region and was associated with increased vascular markings along the skin. Neurologic examination was normal. Magnetic resonance imaging of the chest mass demonstrated a serpiginous lesion along the intercostal margins. Initial differential diagnosis included neuroblastoma, ganglioneuroblastoma, vascular lesion, or nerve sheath tumor. Biopsy was consistent with plexiform neurofibroma. Subsequent examination revealed features consistent with neurofibromatosis type 1. A review of clinical features of NF1 is provided in the case report.
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PMID:A 15-Month-Old Girl Presenting With Clitoromegaly and a Chest Mass. 2996 6

Neuroblastoma of the iris is an extremely rare clinical entity. An otherwise healthy 2-month-old male infant presented to the oncology clinic with a nodular whitish iris lesion in his right eye. The excisional tumor biopsy was consistent with a pathological diagnosis of neuroblastoma with differentiation and negative MYCN gene mutation. Further systemic evaluation revealed a right adrenal mass with no metastatic lesion. The biopsy of the adrenal lesion was also consistent with neuroblastoma. After four courses of chemotherapy, the adrenal mass was completely resected. The patient underwent two additional courses of postoperative chemotherapy and continued retinoic acid treatment. The patient is under regular follow-up with no evidence of recurrence 36 months after the initial diagnosis. This is the first case report to present a histopathological verification of neuroblastoma of the iris. The authors suggest that neonates and infants who are diagnosed as having neuroblastoma undergo an ophthalmologic examination after the initial diagnosis to investigate the true incidence of small iris lesions in neuroblastoma that may have been unrecognized. Neuroblastoma should be included in the differential diagnosis of amelanotic iris lesions in infants and young children. [J Pediatr Ophthalmol Strabismus. 2019;56:e12-e16.].
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PMID:Neuroblastoma of the Iris in Children. 3074 77


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