Cases reported "Paraganglioma"

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11/66. A case of primitive floor of the mouth paraganglioma in a child: an embryological theory unifying viscerocranium appended paragangliomas.

    BACKGROUND: Paragangliomas are unusual tumors in the head and neck originating from the paraganglia or glomus cells of neural crest origin. methods: We describe the first case of a primitive paraganglioma of the floor of the mouth presenting in childhood. RESULTS: Complete surgical removal was performed after embolization of the left lingual artery. There was no evidence of either persistent or recurrent disease 5 years after surgery. The embryologic and anatomic origins of head and neck paragangliomas are reviewed. CONCLUSIONS: An embryologic theory based on the common neural crest origin and migration pathways of both autonomic viscerocranium appended ganglias and paragangliomas is proposed that unifies the topographically heterogeneous group of viscerocranium-appended paragangliomas.
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12/66. Sympathetic paraganglioma as an unusual cause of Horner's syndrome.

    BACKGROUND: Paragangliomas are rare tumors arising from paraganglionic tissue of neural crest origin. They are present in any location where autonomic ganglia are found. The most common location in the head and neck is the carotid body, followed by the jugular bulb and vagus nerve. methods: A 30-year-old woman with a slowly growing left neck mass, aniscoria, and left eyelid ptosis was found to have a vascular tumor consistent with a paraganglioma arising near the left carotid bifurcation. After preoperative embolization, the patient underwent resection of the tumor. RESULTS: The tumor was found to be arising from the left sympathetic trunk and did not involve any other surrounding structures. Histopathologic analysis revealed the typical findings of a paraganglioma. CONCLUSIONS: Sympathetic paragangliomas are exceedingly rare tumors in the head and neck and should be considered in the differential diagnosis when clinical and radiographic evidence suggest a paraganglioma. The presentation is typically a slow-growing neck mass with the presence of an ipsilateral Horner's syndrome.
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13/66. Preoperative embolization in the management of a mediastinal paraganglioma.

    Parangliomas are rare and highly vascular tumors of neuroendocrine cell origin which are treated by complete surgical resection. Preoperative embolization to reduce perioperative bleeding complications, although described in paragangliomas of the neck and carotid body, has never before been described in the case of a mediastinal paraganglioma. The following is a presentation of such a case of mediastinal paraganglioma, in which embolization was used successfully before surgical resection.
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14/66. Lessons to be learned: a case study approach. paraganglioma of the urinary bladder.

    Primary paraganglioma arises infrequently in the urinary bladder. We present here the clinicopathological, immunohistochemical and ultrastructural findings in a 23-year-old Saudi female. She was hospitalised because of gross haematuria over the previous seven days. The intravenous urogram revealed a filling defect in the urinary bladder. cystoscopy disclosed a non-papillary tumour arising from the roof of the bladder neck. Transurethral resection was, accordingly, performed--but marked fluctuation in blood pressure was observed during the procedure. The histopathological findings of trabeculae and small nests of plump oval to spindle cells with a clear to acidophilic cytoplasm, forming an organoid pattern and demonstrating inconspicuous mitotic activity, were diagnostic of paraganglioma of the urinary bladder, the diagnosis was confirmed by means of immunohistochemical and ultrastructural studies. The endocrine markers chromogranin a and neuron-specific enolase were positive in chief cells; sustentacular cells at the periphery of neoplastic cell clusters were positive for S-100 protein. Neurosecretory granules were identified by electron microscopic examination. It is emphasised that, currently, there are no anatomico-pathological criteria to distinguish benign and malignant forms of the disease. Post-surgical recovery was uneventful in this case. recurrence and metastases, though infrequent, have been reported in the literature; therefore, radiation therapy may be an important adjunct--and long-term follow-up is mandatory.
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15/66. A case of pelvic malignant paraganglioma.

    We report a rare case of pelvic malignant paraganglioma that was treated with surgery, combination chemotherapy and radiation. A 47-year-old man was diagnosed with pelvic malignant paraganglioma that had metastasised to the thoracic vertebrae. The pelvic mass, which was 6 cm in size, was on the posterior side of the bladder and had invaded the prostate, seminal vesicle and bladder neck. We resected the intrapelvic tumor and lymph nodes using cystoprostatectomy. Metastases to bilateral obturator lymph nodes and the right internal iliac lymph node were shown by pathology. Adjuvant therapies included six courses of the combination chemotherapy (cyclophosphamide, vincristine and dacarbazine), and 12 courses of VP-16 therapy. radiation therapy was done for metastasis of the thoracic vertebrae. Local recurrence, progression of bone metastasis and new metastasis have not been detected since these treatments. The patient has been clinically stable during 20 months of follow-up. Chemotherapy of cyclophosphamide, vincristine and dacarbazine and VP-16 with radiation appears to be effective in treating advanced malignant paraganglioma.
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16/66. Malignant paraganglioma of the urinary bladder. A case report.

    paraganglioma (pheochromocytoma) is probably the most fascinating of all tumors as it can present with a wide range of clinical manifestations. paraganglioma of the urinary bladder is one of the rare tumors and constitute less than 10% of all bladder tumors. The common presentation of paraganglioma of the urinary bladder is painless haematuria, headache, palpitation and anxiety. Malignancy is uncommon and no histological feature is characteristic of malignancy. Invasion of adjacent organs or metastasis to the lymph nodes is the only criteria to suggest malignancy. In the presence of proven metastasis radical cystectomy with pelvic lymphadenectomy is recommended. In the present case the tumor was arising from anterior wall of the bladder away from the bladder neck and that in a large capacity bladder it was possible to get a wide margin for partial cystectomy along with bilateral pelvic lymphadenectomy without violating the oncological principles. At a follow up of two years there is no evidence of recurrence of tumor.
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17/66. Rare localization of paraganglioma in head and neck.

    In this paper, we describe the clinical course of a 61-year-old female patient with paraganglioma in the head and neck region. Computed tomographic scan (CT), magnetic resonance imaging (MRI), ultrasound scan (US) and arteriogram findings initially led us to suspect that this tumor originated in the vagal nerve. In particular, a color Doppler US enabled an easy diagnosis of hypervascular tumor. We removed this surgically, but the tumor was easy to peel from the vagal nerve and carotid bifurcation. The distal side of the tumor was under the digastric muscle and running into the hypoglossal nerve. The intraoperative findings were highly suggestive of localization at the hypoglossal nerve, although Xth and XIIth cranial nerve palsies have remained.
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18/66. Paraganglionomas in the neck.

    Three paraganglionomas in the neck investigated by angiography are presented. The value of angiography in these tumours is stressed, and one case showed distinctive radiological features hitherto undescribed which probably constitute a basis for the definitive preoperative diagnosis of malignant lesions.
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19/66. Multiple paragangliomas of the head and neck.

    Multiple paragangliomas of the head and neck are rare conditions. The incidence of multiple paragangliomas is reported to the approximately 10% of the total patients, but in familial cases it increases up to 35-50%. In the head and neck region, the most common association is represented by bilateral carotid body tumors or by carotid body tumor associated with tympanic-jugular glomus. The presence of three synchronous glomus tumors is really rare, as well as association with vagal glomus and carotid body. In this paper the authors present a patient affected ipsilaterally by a carotid body tumor and vagal paraganglioma, focusing on the diagnostic options offered by imaging techniques (CT and MRI).
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20/66. Hereditary paraganglioma due to the SDHD M1I mutation in a second Chinese family: a founder effect?

    OBJECTIVES/HYPOTHESIS: Hereditary paraganglioma is a rare condition that is inherited in an autosomal-dominant fashion. Four distinct loci have been associated with hereditary paraganglioma, including the SDHD, SDHC, and SDHB genes and a locus at 11q13. The SDHD, SDHC, and SDHB genes code for subunits of succinate dehydrogenase, which forms part of the mitochondrial respiratory chain. SDHD mutations are widely distributed along the gene with no apparent hot spots, although a founder effect has been described in the Dutch population. methods: Following a prior report of the SDHD M1I mutation in an Australian Chinese family, a second Chinese family with the same mutation is reported. The proband developed bilateral head and neck paragangliomas at age 34 years and a functioning adrenal pheochromocytoma and two extra-adrenal abdominal paragangliomas 7 years later. His brother had unilateral head and neck paraganglioma at age 39 years. Given the multicentricity of the proband's tumor and the familial clustering of paragangliomas, a clinical diagnosis of hereditary paraganglioma was made, and the proband was tested for a mutation in the SDHD gene. RESULTS: The proband was found to be heterozygous for the SDHD MII mutation that removes the start codon, and his brother subsequently tested positive for the same mutation. The family is not related to the Australian Chinese family. CONCLUSION: The finding suggests the possibility of a founder effect in the Chinese population and warrants further investigation.
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