Cases reported "Ganglioneuroblastoma"

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1/12. Results of surgery for a compound adrenal tumor consisting of pheochromocytoma and ganglioneuroblastoma in an adult: 5-year follow-up.

    A rare, compound adrenal tumor consisting of ganglioneuroblastoma and pheochromocytoma was completely resected in an adult woman. Most of the tumor was occupied by the ganglioneuroblastoma component. This ganglioneuroblastoma was an intermixed tumor, which is known to have a favorable prognosis in children. Based on the lack of spread, the resectability of the tumor, and the histology of the ganglioneuroblastoma, no adjuvant therapy was employed. There was no evidence of recurrence at the 5-year follow-up. This suggests that adjuvant therapy may not be necessary in these compound tumors.
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2/12. Adrenal ganglioneuroblastoma in pregnant woman: diagnosis with three-dimensional ultrasound.

    The diagnostic approach for evaluation of adrenal tumour in pregnant women is a problem. This article presents the multifactorial diagnosis of rare, incidentally detected massive adrenal tumour with 2D and 3D US. Grey-scale 2D and 3D US with tissue harmonic imaging showed inhomogeneous, encapsulated, solid tumour of the right adrenal region. Three-dimensional colour Doppler and power Doppler studies showed ample neovascularization in the tumour and the adrenal vein draining to the inferior vena cava similarly to angiographic studies performed in the past.
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3/12. ganglioneuroblastoma presenting as dilated cardiomyopathy.

    We report an unusual presentation of ganglioneuroblastoma with features of dilated cardiomyopathy in a 22 month old girl. She was admitted with cardiomegaly; during echocardiography a suspicious abdominal mass was detected by chance. Further imaging studies, including abdominal ultrasonography and spiral computed tomography, revealed a solid mass originating in the right adrenal gland. Metabolic studies and pathological findings were compatible with ganglioneuroblastoma. Following tumour removal and supportive therapy for cardiomyopathy, her clinical condition and laboratory findings improved. Although ganglioneuroblastoma with features of dilated cardiomyopathy is rare, because neurogenic tumours may be involved in its development, measurement of catecholamines in children with dilated cardiomyopathy is strongly recommended.
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4/12. ganglioneuroblastoma metastatic to the orbit.

    A 10-month-old girl presented with an extensive orbital and cranial metastatic lesion from an adrenal ganglioneuroblastoma. Treatment with chemotherapy alone resulted in complete regression of the tumors with over 7 years of follow-up. Good prognostic indicators included her young age at diagnosis, dna index of tumor cells of 1.4, and the histologic subtype of neuroblastic tumor. This is the first reported case of ganglioneuroblastoma metastatic to the orbit.
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5/12. adult-type ganglioneuroblastoma in the adrenal gland treated by a laparoscopic resection: report of a case.

    A case of ganglioneuroblastoma in the adrenal gland of a 50-year-old man is reported. The patient was incidentally found to have a nonfunctioning tumor in the right adrenal gland. The tumor, measuring 4.5 cm, was successfully removed using laparoscopy. Histologically, the tumor was diagnosed to be a ganglioneuroblastoma. Immunohistochemically, a few MIB-1-positive cells and no S-100 protein-positive cells were observed. There has been no evidence of recurrence for 2.5 years to date after the operation. Adrenal ganglioneuroblastoma is extremely rare in adults, and only seven such cases have been previously reported in the literature.
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6/12. Cushing's syndrome due to ectopic production of corticotropin-releasing hormone in an infant with ganglioneuroblastoma.

    OBJECTIVE: To report the first recognized case of Cushing's syndrome due to a corticotropin-releasing hormone (CRH)-secreting ganglioneuroblastoma, which was found in an 18-month-old boy with hypertensive encephalopathy. methods: The clinical, biochemical, and immunohistochemical characteristics of this rare syndrome are described, and the relevant literature is reviewed. RESULTS: An 18-month-old boy with a history of recent weight gain was admitted because of sudden onset of right fixed esotropia and left facial palsy after episodes of emesis. magnetic resonance imaging showed old left frontal lobe and right hypothalamic infarcts. The patient had generalized obesity, decelerated linear growth, hypertrichosis, hypertension (144/103 mm Hg), hypokalemia, and proteinuria. The 24-hour urinary excretion of free cortisol, catecholamines, and metanephrines was increased. The serum cortisol concentration after a 1-mg overnight dexamethasone suppression test (DST) was 53.7 mg/dL (normal, <5). The serum adrenocorticotropic hormone (ACTH) concentration was 7 pg/mL (normal, 10 to 60), and the CRH level was 439 pg/mL (normal, 24 to 40). An overnight high-dose DST (8 mg) failed to suppress serum cortisol; however, both cortisol and ACTH were responsive to ovine CRH stimulation. Despite discordant dynamic endocrine testing and negative somatostatin receptor scintigraphy, computed tomography showed a right 3.6- by 3.0-cm extra-adrenal retroperitoneal mass with central calcification extending 7 cm cephalocaudally. The patient underwent exploratory laparotomy, followed by chemotherapy. Findings on light microscopic and immunohistochemical examination of the retroperitoneal mass were consistent with a ganglioneuroblastoma that expressed CRH, pro-opiomelanocortin, and ACTH. CONCLUSION: The evaluation of Cushing's syndrome is one of the most complex endocrine challenges. In this case, it was due to ectopic production of CRH by a ganglioneuroblastoma. Because most CRH-producing tumors also secrete ACTH, the ectopic production may represent a paracrine phenomenon in addition to an endocrine phenomenon. The ectopic CRH may also indirectly provoke pituitary ACTH secretion. This dual mechanism may explain the resistance of the tumor to feedback inhibition and a CRH-stimulation response indistinguishable from that observed in pituitary-dependent Cushing's syndrome.
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7/12. A case report: adrenal ganglioneuroblastoma in a 59-year old man.

    ganglioneuroblastoma is a common type of tumor in children, but is rarely seen in adults. We present here a case of adrenal ganglioneuroblastoma in a 59-year old man.
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8/12. A rare ganglioneuroblastoma secreting dopamine and the value of its measurement in diagnosis and prognosis.

    A case is described of a patient with a ganglioneuroblastoma, initially located in the right adrenal, which produced an excess of dopamine (7646 and 7959 nmol/24 h), approximately two and a half times the upper limit of the normal daily urine output. The urinary excretion of noradrenaline, adrenaline and methylated derivatives was always within the normal reference ranges. The patient was generally well, with normal blood pressure and only mild flushes. Two years after surgical resection, recurrence was indicated by an increase in urinary dopamine (8507 nmol/24 h); it was located in the tumour bed and left side of the neck by CT and (123)I MIBG scans. The patient was treated with a high dose of (131)I MIBG, with subsequent reduction in dopamine production. This was repeated on four other occasions, the latest being in January 2005. The output of dopamine was thus used as a marker of tumour diagnosis and progression and it is recommended that the assay of dopamine be included in the screening of catecholamine-secreting tumours to avoid possible misdiagnosis.
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9/12. Gastric ganglioneuroblastoma: a rare finding in an infant with multifocal ganglioneuroblastoma.

    PURPOSE: This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement. PATIENT: The patient had a right cervical tumor, a left posterior mediastinal tumor, bilateral adrenal tumors, and bony and bone marrow metastases. The tumor cells were diploid and lacked N-myc gene amplification. The gastric involvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy. RESULTS: Our patient achieved only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currently has stable, unresectable disease with elevated catecholamines. CONCLUSIONS: Multifocal ganglioneuroblastomas may arise from either neuroblastic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. patients with multifocal ganglioneuroblastomas warrant meticulous radiographic and surgical evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.
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10/12. Primary adrenal ganglioneuroblastoma in an adult.

    We present here a case of adrenal incidentaloma in a 35-year-old male. Imaging technology including magnetic resonance imaging (MRI) clarified that the tumor was heterogeneous, and large in size (9x7 cm), and that it originated from the left adrenal gland. MRI scans on T1-weighted sequence showed the tumor with a low signal intensity followed by rapid contrast enhancement, whereas those on T2-weighted sequence showed markedly increased intensity. The tumor was surgically removed, and pathologic diagnosis was well-differentiated ganglioneuroblastoma. ganglioneuroblastoma in adults is extremely rare, with only about 30 cases documented in people over the age of 20 years.
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