Cases reported "Ganglioneuroma"

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1/84. Adrenal ganglioneuroma: a familial case.

    A 67-year-old woman was evaluated for asymptomatic microscopic hematuria. Intravenous urography and computed tomography demonstrated an adrenal mass, but the workup for a functional adrenal tumor was negative. The mass was surgically resected, with a histologic diagnosis of adrenal ganglioneuroma. No further treatment was necessary. After the diagnosis was made, the patient reported a family history positive for neuroblastic tumors. Two of her grandchildren had presented at early ages with a ganglioneuroblastoma and a third had presented with a ganglioneuroma. This presentation is unique because ganglioneuroma, especially that of adrenal origin, is rare in the adult population, and familial cases are extremely uncommon.
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2/84. diagnosis controversies for adrenal ganglioneuroma.

    The authors report a case of adrenal ganglioneuroma which was incidentally diagnosed performing preoperative examination for a sigmoid carcinoma. The authors took this finding as a starting point to underline the rarity of this condition and its chance discovery, this being due to the rare presence of signs and symptoms and its frequent association with other synchronous neoplasms; all this makes differential diagnosis rather difficult.
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3/84. Adrenal ganglioneuroma: report of five cases.

    Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system. The clinical presentation of most patients is asymptomatic, and most of these tumors are hormone silent. Usually the tumor is detected incidentally by abdominal imaging studies for unrelated reasons. Herein, we review our cases of adrenal ganglioneuroma over the past 20 years. All patients diagnosed with primary adrenal ganglioneuroma between 1979 and 1999 were reviewed. There were 5 cases of pathologically confirmed adrenal ganglioneuroma. All patients underwent surgical excision of the adrenal tumors. The mean age of the 5 patients (3 women and 2 men) was 27 years (range, 11 to 45 years). The average diameter of the tumor was 8.2 cm (range, 6 to 10 cm). The mean follow-up period was 33.6 months (range, 3 to 92 months). One case involved mesenteric lymph nodes. This patient had persistent hypertension following the operation, but no evidence of tumor recurrence was noted over the 7-year follow-up period. The prognosis was excellent after surgical excision in our series. awareness of this rare entity may add to the differential diagnoses of adrenal masses preoperatively.
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keywords = adrenal
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4/84. Autonomic dysfunction associated with multiple pelvic ganglioneuromas.

    We describe the case of a 59-year-old male in which several symptoms of autonomic dysfunction resulted associated with the presence of multiple ganglioneuromas in the adrenal glands and in paravertebral spaces. In a 2-year period, the patient developed sexual dysfunctions, micturitional disturbances and severe orthostatic hypotension and was erroneously diagnosed as having primary autonomic failure. However, the examination of all the autonomic functions showed that papillary function and cardiovascular reflexes were normal. CT scan of the abdomen revealed the presence of several small masses in the adrenal glands and along the lumbar sympathetic chain. Cytologic examination of the adrenal glands showed clusters of ganglionlike cells interspersed with schwann cells, leading to the diagnosis of ganglioneuromas. patients with signs and symptoms of autonomic dysfunction need an extensive clinical and laboratory examination of all the autonomic functions in order to exclude secondary causes of the symptomatology.
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keywords = adrenal
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5/84. Lhermitte-Duclos disease (Dysplastic gangliocytoma of the cerebellum).

    OBJECTIVE: Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos disease) is a rare hamartomatous lesion of the cerebellar cortex. The pathogenesis of the disease is still poorly understood. Lhermitte-Duclos disease was recently considered to be part of a multiple hamartoma-neoplasia syndrome (Cowden disease). We add two further cases to this rare entity. patients: A 24-year old woman presented with occipital headaches, blurred vision, diplopia and ataxia of gait. physical examination revealed turricephaly. The second patient was a 37-year old woman, who presented with progressive occipital headache with nausea and vomiting. physical examination revealed congenital facial asymmetry. Computed tomography and NMR-imaging, respectively demonstrated a space occupying mass of a cerebellar hemisphere in both cases. RESULTS: Suboccipital craniotomy and complete removal of the infratentorial tumour were performed in both patients. Histopathological findings clinched the diagnosis of Lhermitte-Duclos disease. Postoperative course was uneventful in the first and complicated by progressive occlusive hydrocephalus in the second patient, necessitating permanent surgical shunt drainage. Both patients were discharged free of complaints. CONCLUSIONS: Dysplastic cerebellar gangliocytoma is commonly associated with progressive mass effects in the posterior fossa and typically presents with headaches, cerebellar dysfunction, occlusive hydrocephalus and cranial nerve palsies. The disease usually manifests in young adults, but the age at presentation ranges from birth to the sixth decade. There is no sex predilection. NMR-imaging became a useful clue to the diagnosis within the last decade. Therapy consists of decompression of the posterior fossa by total surgical removal of the tumour mass.
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keywords = cortex
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6/84. A case of multiple endocrine neoplasia type 2b undiagnosed for many years despite its typical phenotype.

    We report the case of a 24-yr-old man with a typical phenotype of multiple endocrine neoplasia type 2b (MEN 2B). The patient had previously undergone minor surgery to remove multiple tumors on the lip, but he had no further examinations. MEN 2B was suspected owing to characteristic multiple ganglioneuromatosis when the patient presented with a goiter associated with high levels of plasma calcitonin and CEA. Aspiration biopsy cytology revealed medullary thyroid carcinoma (MTC), and abdominal computed tomography and nuclear scanning with metaiodobenzylguanidine revealed bilateral adrenomedullary tumors. Adrenomedullary function tests showed high levels of serum and urinary fractionated catecholamines, and genetic analysis showed a point mutation in the codon 918 (M918T) of the RET gene. The patient was diagnosed with MEN 2B and underwent right adrenalectomy and total thyroidectomy. No distant metastasis of the MTC was noted although MEN 2B had remained undiagnosed since the ganglioneuromatosis was first noticed. MEN 2B is a rare hereditary disorder, but the occurrence of characteristic ganglioneuromatosis was quite helpful in making the diagnosis.
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7/84. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.

    We report a 56-year-old man in whom an olfactory neuroblastoma with epithelial and endocrine differentiation transformed into a mature ganglioneuroma after chemoradiotherapy. The tumor arising from the sphenoidal and maxillary sinuses showed rapid growth into the frontal lobe and metastasis to the cervical lymph nodes. The patient showed signs of a syndrome of inappropriate secretion of antidiuretic hormone (SIADH). A radical craniofacial resection of the primary tumor was performed after 16 Gy of local irradiation and systemic chemotherapy. Three months after the operation, the patient died of mediastinal metastasis. The biopsy before chemoradiotherapy showed a neuroblastoma with Homer-Wright rosettes, fibrillary matrix, Flexner-Wintersteiner rosettes and antidiuretic hormone production. After chemoradiotherapy, the histology changed to that of a ganglioneuroma consisting of large ganglion cells and schwann cells without immature neuroblastoma components. Although transformation to ganglioneuroma in an adrenal neuroblastoma is common, an olfactory neuroblastoma showing ganglioneuronal maturation after chemoradiotherapy has not been reported. The pluripotent progenitor cells of the olfactory neurons may be the origin and their existence explains why various neoplasms with neuronal and epithelial differentiation arise from the olfactory mucosa.
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keywords = adrenal
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8/84. Fluorodeoxyglucose and methionine uptake in Lhermitte-Duclos disease: case report.

    OBJECTIVE AND IMPORTANCE: Lhermitte-Duclos disease (LDD) represents a focally indolent dysplastic growth of the cerebellar cortex. The 106 cases reported previously in the literature show an extremely slow evolution, and the operative indications and techniques for this disease entity are still controversial. The authors present the first case of LDD studied with positron emission tomography using the labeled tracers [(18)F]2-fluoro-2-deoxy-d-glucose ([(18)F]FDG) and (11)C-labeled methionine ([(11)C]Met) to study the glucose and protein metabolism of the lesion. CLINICAL PRESENTATION: A 40-year-old woman suddenly became unconscious then completely recovered 5 minutes later. magnetic resonance imaging of her brain showed a well-delineated 10 x 5-cm abnormal area with enlarged cerebellar folia, which led to the diagnosis of LDD. TECHNIQUE: On positron emission tomographic scans, [(18)F]FDG and [(11)C]Met uptake in the normal cerebral and cerebellar cortex appeared higher than normal, reaching levels found in patients with primary malignant brain tumors. Moreover, the uptake of both tracers was heterogeneous, in contrast to the homogeneous uptake visualized on magnetic resonance imaging scans. The areas of greatest [(11)C]Met and [(18)F]FDG uptake were discordant. Some areas of greater than normal [(18)F]FDG uptake corresponded to areas of moderate or low [(11)C]Met uptake. Because of the important mass effect in the posterior fossa, total surgical resection was performed. A histological examination confirmed the diagnosis of LDD. CONCLUSION: This first reported metabolic study of LDD supports the view that LDD is an active and evolving disease. These data should prompt reevaluation of the indications for surgery in patients with this disease as well as the timing of surgery.
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ranking = 0.0030946060981619
keywords = cortex
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9/84. myelolipoma associated with adrenal ganglioneuroma.

    Adrenal myelolipomas are rare, benign mesenchymal tumors composed of mature adipose tissue and hematopoietic cells in varying proportions. Although the majority of cases occur as isolated adrenal lesions, myelolipomas have been described in association with various adrenal pathologic conditions. These conditions include enzyme deficiencies and hyperplastic and neoplastic lesions of the adrenal cortex, with perhaps endocrine dysfunction as a common feature. ganglioneuroma is a benign tumor of the sympathetic nervous system that rarely produces symptoms of endocrine dysfunction. We report an unusual case of myelolipoma associated with ganglioneuroma of the adrenal medulla. The histogenesis of myelolipoma remains speculative. However, the close proximity to adrenal cortical cells within the stroma of ganglioneuroma suggests that the hormonal microenvironment may have played a role in the development of the myelolipoma.
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ranking = 2.2515473030491
keywords = adrenal, cortex
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10/84. An unusual etiology of hypertension in a 5-year-old boy.

    Ganglioneuromas are rare benign tumors of neural crest origin, arising from ganglia of the sympathetic nervous system and adrenal medulla. These masses are usually detected during the first 2 decades of life and are generally discovered incidentally. We present a 5-year-old boy with sickle beta-thalassemia whose hypertension is caused by a perihilar ganglioneuroma encasing the right renal artery and distorting the right renal vein. The tumor was resected and the child's blood pressure subsequently normalized.
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keywords = adrenal
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