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

Fanconi's anemia (FA) is an autosomal recessive inherited syndrome with a predisposition to malignancy. The association between FA and solid pediatric tumors is extremely rare. The authors report a rare case of VACTERL syndrome associated with FA and multiple solid pediatric tumors occurring in a very young girl. This child had numerous congenital anomalies (horseshoe kidney, cerebella hypoplasia, microcephaly, sacral agenesis) and esophageal atresia, which was repaired in neonatal period. Such association led quickly to the diagnosis of FA. At age of 11 months, she developed simultaneously a renal tumor in a horseshoe kidney and bilateral adrenal tumors. The left adrenal mass was removed, and partial nephrectomy was performed. Histological analysis concluded to adrenal neuroblastoma and nephroblastoma. We also evaluated the c-kit expression in these tumors to propose a therapeutic alternative to chemotherapy by oral agent STI-571 (Gleevec; Novartis, East Hanover, NJ). Strong cytoplasmic immunostaining of c-kit was found in both tumors. Unfortunately, she quickly developed a posterior cerebellar fossa tumor and died 1 month later. This clinical situation is very rare but suggests that young patients with FA and solid pediatric tumors may belong to a particular subgroup of FA. Further studies are necessary to test if STI-571 treatment could be efficient in such patients with pediatric tumors.
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PMID:Bilateral adrenal neuroblastoma and nephroblastoma occurring synchronously in a child with Fanconi's anemia and VACTERL syndrome. 1641 81

Neuroblastoma of the adrenal gland is an extremely rare tumour in adulthood although it is one of the most common malignancies in childhood. In this report, we present a 52-year-old man who had a left adrenal mass on preoperative imaging. On laboratory, slightly elevated catecholamine metabolites were detected in the urine that was collected over 24 hours. He was operated and the mass was resected in en-block manner along with the regional lymph nodes. The histopathological examination of the specimen revealed the diagnosis of neuroblastoma. He had no metastatic disease at the time of diagnosis and received chemotherapy after the operation. However, the prognosis was poor and he died 10 months after the operation. Although neuroblastoma of adrenal gland is rare in adulthood, it should be considered in the differential diagnosis for patients with adrenal masses.
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PMID:An adult adrenal neuroblastoma: a case report. 1643 86

Adrenal hemorrhages are not uncommon in newborns, but they may occur before birth. The incidence of adrenal hemorrhages based on extensive necropsy has been estimated as about 1.7 per 1000 births. With the increasing use of ultrasonography, prenatal recognition of fetal adrenal hemorrhages has become more common in recent years. We report an echogenic abdominal mass, which was found at 36 weeks of gestation using real-time ultrasound, and was enlarged during the 2-week follow-up period. No trauma history was noted during the pregnancy. Since the mass was close to the kidney, we performed power Doppler imaging to localize the lesion and tried to make a differential diagnosis. The left adrenal mass was posterior to the stomach and was without pulsatile blood flow inside. After birth, sonography confirmed a solid homogenous adrenal mass in the newborn. Seven days after birth, the persistent adrenal echogenic mass without resolution by computerized tomography led to a diagnosis of neuroblastoma. The mass was removed by surgery and adrenal hemorrhage diagnosed. The outcome of the adrenal hemorrhage was excellent. The differential diagnosis in unilateral adrenal mass is difficult from the images of 2-D ultrasound since similar pictures may occur among them. In this case, accurate localization of an adrenal mass is feasible using power Doppler imaging (PDI) before birth by determining its blood supply from the middle suprarenal artery. At the same time, fetal adrenal hemorrhages can be demonstrated as an avascular mass, which is the major difference from adrenal tumors. In conclusion, PDI offered more information to localize the adrenal glands from other lesions by separating the blood supply. In addition, the avascular adrenal mass favors the prenatal diagnosis of adrenal hemorrhage.
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PMID:Real-time and power Doppler imaging of fetal adrenal hemorrhage. 1651 20

Opsoclonus-myoclonus, a rare paraneoplastic syndrome that may occur in patients with neuroblastoma, is thought to be a humorally mediated immune reaction to malignant cells that cross-react with autoantigens. This report describes the case of an occult neuroblastoma diagnosed in a 4-year-old female 2 years after presentation of opsoclonus-myoclonus. Although no mass was evident on previous imaging at an interval of 10 months, a computed tomographic scan 4 months after rituximab treatment and 20 months after presentation revealed a new left adrenal mass. Although neuroblastomas can be identified months after presentation of opsoclonus-myoclonus without treatment with rituximab, this report describes one of the longest intervals using up-to-date imaging techniques. Therefore the case raises two concerns: (1) whether the same immune process that causes opsoclonus-myoclonus may suppress neuroblastomas, and (2) whether immunosuppressive therapy with rituximab may inhibit the immune reaction to occult neuroblastomas in patients with unexplained opsoclonus-myoclonus.
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PMID:Neuroblastoma found in a 4-year-old after rituximab therapy for opsoclonus-myoclonus. 1693 63

Ganglioneuromas, which are benign tumors of sympathetic nervous system, are rare to arise in adrenal gland. They are usually clinically silent and detected during work-up for other unrelated conditions. Currently, histopathology is the only tool to diagnose ganglioneuroma and to differentiate it from ganglioneuroblastoma and neuroblastoma. We discuss the case of a 40-year-old female found to have a right adrenal mass during investigation of dull ache in right upper abdomen. Clinico-radiological assessment was non-confirmatory on the nature of the mass, and excision was done. Histological examination showed the mass to be a ganglioneuroma arising in the adrenal gland. In conclusion, ganglioneuroma occurs rarely in adrenal gland and pre-operative diagnosis is difficult since the symptoms are usually non-specific. Histological examination is the mainstay of diagnosis and should be thorough to exclude neuroblastomatous foci, portending a worse outcome.
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PMID:Ganglioneuroma of the adrenal gland: a rare case. 1830 53

Blueberry Muffin baby is a rare neonatal skin disorder. Many causes are known, examples are congenital infections, hemolysis and tumors. We report on a newborn presenting with Blueberry Muffin syndrome and an adrenal mass which lead to the diagnosis of neuroblastoma. Actually, it corresponded to an acute monoblastic leukaemia with an adrenal localization and a cerebrospinal fluid involvement. Leukaemia should always be considered in such patients, even in the absence of blasts on white blood cells count and bone marrow examination, as in this patient. This observation was also unusual due to spontaneous remission. The patient is in complete remission at 1 year follow-up.
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PMID:[Blueberry Muffin Baby and spontaneous remission of neonatal leukaemia]. 1859 69

A newborn with a prenatally detected adrenal mass underwent complete resection of a stage 1 favorable histology neuroblastoma (NB) without MYC-N amplification. Two months later, the infant presented with a local recurrence and multiple hepatic metastases. Close follow-up without therapy was adopted for stage 4s NB. Enlarging tumor lesions were seen until the child was 8 months old, followed by later decrease in size. At 36 months of follow-up, the child is alive and disease-free. We describe this case of NB and its abnormally short evolution from stage 1 to stage 4s, despite initial surgery. Its spontaneous regression may help us understand the natural history of congenital NB.
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PMID:Congenital stage 1 neuroblastoma evolved into stage 4s. 1912 91

The characterisation of adrenal lesions is a common radiological dilemma. Incidental adrenal lesions are commonly detected with computed tomography (CT), and lesion characterisation is critical. The prevalence of incidental adrenal lesions has been reported to be 2.3% at autopsy and 0.5-2% with abdominal CT. Such lesions are likely to be seen with increasing frequency given the expanding use of radiological imaging in clinical practice. Although the majority of adrenal lesions are benign, in patients with an extra-adrenal primary cancer the probability of an adrenal mass being a metastasis is 52%. Unfortunately, there may be significant overlap between the imaging appearances of benign lesions such as lipid-poor adenomas and malignant lesions, particularly metastases and small adrenal carcinomas. This review highlights recent advances in radiological imaging of adrenal lesions and we discuss the relative merits of CT and magnetic resonance imaging to aid the identification of benign and malignant adrenal lesions and their roles, in combination with biochemical and clinical data, in recognizing common pathologies such as adrenal adenoma, phaeochromocytoma, carcinoma and metastases. We also discuss the radiological characteristics of rarer adrenal lesions including lymphoma, neuroblastic tumours (neuroblastoma, ganglioneuroblastoma, and ganglioneuroma), lipomatous tumours (myelolipoma, angiolipoma, teratoma, lipoma and liposarcoma), in addition to hemangioma, hemangiosarcoma and leiomyosarcoma.
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PMID:Radiological localizing techniques in adrenal tumors. 1947 Dec 40

A case of neonatal bilateral adrenal mass causing severe respiratory distress and requiring an emergency debulking surgical procedure is reported. Histopathology revealed a cystic neuroblastoma stroma poor, poorly differentiated, without MYCN amplification and 1p deletion. During postoperative follow-up, a progression to stage 4s was observed, characterized by liver involvement. According to good prognostic indexes, no further treatment was administered. Both adrenal masses and hepatic nodules showed progressive decrease in size, till complete disappearance. The authors encourage a multidisciplinary approach to develop the best patient-related strategy for cystic neuroblastoma, thus reducing complications rate.
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PMID:Life-threatening bilateral adrenal cystic neuroblastoma in an infant. 1988 60

A 32-month-old female child with a history of stage IV favorable biology neuroblastoma with a 123-I MIBG (metaiodobenzylguanidine scan) avid adrenal mass, with retroperitoneal nodal extension and bony metastasis, was in complete remission after intense multimodal therapy. Seventeen months after diagnosis a surveillance 123-I MIBG scan showed abnormal tracer uptake in the midzone of the right thorax. Chest x-ray and CT scan confirmed right upper lobe consolidation corresponding to the tracer uptake. Chest x-ray after antibiotics showed resolution of the abnormality. 123-I MIBG scan 3 months later showed no abnormal uptake. False-positive MIBG uptake in pneumonia has not been previously reported.
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PMID:False-positive MIBG uptake in pneumonia in a patient with stage IV neuroblastoma. 2070 60


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