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11/260. A diagnostic and therapeutic approach to paragangliomas of the larynx.

    BACKGROUND: Differentiating paragangliomas from moderately differentiated neuroendocrine carcinoma in the larynx is a difficult management problem. As the biological behavior of these 2 entities is different, we developed an algorithm for the preoperative diagnosis and treatment of this disease. DESIGN: The sample case from which the algorithm was developed consisted of a 69-year-old man who was transferred to us after tracheostomy and an attempt at biopsy for airway obstruction secondary to a vascular mass. biopsy resulted in substantial bleeding. Flexible laryngoscopy showed a vascular mass of the supraglottis. A computed tomographic scan showed 2 vascular masses at the carotid bifurcation and in the larynx. An arteriogram confirmed synchronous vascular tumors. RESULTS: The arteriogram showed the superior thyroid artery to be the major feeder vessel to this mass, a situation commonly seen in paragangliomas but not other neuroendocrine tumors. The presence of synchronous lesions and a vascular mass based on the superior thyroid artery helped differentiate paraganglioma from the other neuroendocrine tumors. As the biological behavior of paragangliomas is relatively benign, we performed a conservative supraglottic laryngectomy and excision of the carotid body tumor. Histologic diagnosis and immunohistochemical analysis confirmed the diagnosis of paraganglioma. CONCLUSIONS: The vascular nature of neuroendocrine tumors prevents preoperative pathological diagnosis. Radiologic features demonstrating a vascular mass with a dominant feeder vessel by the superior or inferior thyroid artery may help in the clinical diagnosis of paragangliomas of the larynx. Since paragangliomas are rarely malignant, a conservative surgical procedure should suffice. ( info)

12/260. Clear-cell odontogenic carcinoma with pulmonary metastases resembling pulmonary meningothelial-like nodules.

    Clear-cell odontogenic carcinoma (CCOC) is a rare neoplasm with malignant potential and unknown cytogenetic alterations. We describe the case of a 43-year-old woman who presented with an unusual odontogenic epithelial tumor. Histologically, the tumor was composed of clear-cell areas and exhibited a squamous pattern with little nuclear pleomorphism similar to benign squamous odontogenic tumor. Multiple small pulmonary nodules occurring 3 years after primary surgical treatment histologically closely resembled benign minute pulmonary meningothelial-like nodules (MPMN) with clear-cell features. comparative genomic hybridization (CGH) and immunohistochemistry, performed as diagnostic adjuncts, revealed in the odontogenic tumor and the pulmonary lesions a very similar pattern of chromosomal aberrations (loss of 9, gains of 14q, 19 and 20 in both, and additional loss of 6 in the odontogenic tumor) and the same pattern of expression (positive for cytokeratin 5, 6, 8, 19 and negative for cytokeratin 18, epithelial membrane antigen, and vimentin), differing from that of MPMN. These findings confirmed the final diagnosis of metastasizing CCOC with partial squamous differentiation, substantiated the unfavorable prognosis of the clear-cell component, and highlighted the diagnostic impact of CGH and immunohistochemistry for classification of these morphologically peculiar pulmonary CCOC metastases. ( info)

13/260. Multifocal malignant extra-adrenal paragangliomas of the Organ of Zuckerkandl and urinary bladder.

    A case of multifocal malignant extra-adrenal paragangliomas involving the Organ of Zuckerkandl and urinary bladder is presented. The role and order of imaging investigations of functioning extra-adrenal paraganglioma is discussed. ( info)

14/260. paraganglioma manifesting as shock: a case report.

    paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up. ( info)

15/260. MR findings of the spinal paraganglioma : report of three cases.

    Extraadrenal paragangliomas involving the spine is less common and usually takes the form of intradural compression of the cauda equina. The authors report three cases of spinal paragangliomas resulting in extradural spinal cord compression and their MR findings. The MR imaging revealed a well-demarcated extradural mass with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images compared to paravertebral muscles. After Gd-DTPA administration, heterogeneous and intense enhancement was found. Multiple punctate and serpiginous structures of signal void due to high-velocity flow were noted around and within the tumors on all sequences. In one case, the signal void structures were well corresponded with feeding arteries on angiography. These may be the characteristic findings of the extraadrenal paraganglioma involving the spine. ( info)

16/260. Tracheal paraganglioma: a diagnostic dilemma culminating in a complex airway management problem.

    A 41-year-old man presented to his general practitioner (GP) with a wheeze and dyspnoea on exercise. asthma was diagnosed and treatment with inhaled corticosteroid and a beta(2)-agonist commenced. Despite this, his condition deteriorated over three weeks culminating in stridor, requiring emergency admission to hospital. Nasendoscopy revealed a polypoidal lesion in the upper trachea, acting like a ball valve. A local anaesthetic tracheostomy, secured his airway. Subsequent direct laryngoscopy allowed avulsion of the lesion. Alternative methods of airway management are discussed. histology revealed a paraganglioma. The aetiology of paragangliomas is described and a literature review of tracheal paraganglioma is presented. Post-operative recovery was unremarkable. However, tumour recurrence occurred at nine months. A subsequent revision tracheostomy and laser resection has ensured disease-free survival, one year later. We recommend that acute onset wheeze, refractive to appropriate therapy, should be referred for urgent examination of the upper aerodigestive tract. ( info)

17/260. Nasal paraganglioma. A case report.

    Nasal paraganglioma. A case report. Nonchromaffin paragangliomas or chemodectomas arise in paraganglia distributed in various parts of the body. The jugular bulb, the vagal body and the bifurcation of the carotid artery are the most common sites of origin of paraganglioma in the head and neck region. Paragangliomas in the nose and paranasal sinuses are extremely rare and very few cases of definite paraganglioma arising primarily in the nose or paranasal sinuses have been reported. The paraganglioma is a slow-growing tumour that produces nasal obstruction, profuse epistaxis and facial swelling. Complete excision of the glomus tumour is normally curative. We report a case of nasal paraganglioma and discuss the diagnosis and therapy. ( info)

18/260. Typical and atypical Carney's triad presenting with malignant hypertension and papilledema.

    This report encourages clinicians to consider a diagnosis of Carney's triad in patients with multifocal gastric stromal sarcoma, extraadrenal paraganglioma (predominantly mediastinal), or pulmonary chondroma. The authors conducted a retrospective 20-year survey at the Hospital for Sick Children and identified two children with Carney's triad. One child, presenting atypically with papilledema and fundal hemorrhages from malignant hypertension and benign intracranial hypertension from chronic iron-deficiency anemia, is the second patient ever to date be described with the complete Carney's triad of neoplasms at diagnosis. Another child presented more typically with gastric stromal sarcoma and pulmonary chondroma without paraganglioma. Carney's triad is a rare differential diagnosis for "idiopathic" hypertension or iron-deficiency anemia from chronic gastrointestinal bleeding. If missed, patients with Carney's triad may have the debilitating physical and mental consequences of chronic iron deficiency and may die of untreated prolonged hypertension and metastatic leiomyosarcoma. ( info)

19/260. Clinics in diagnostic imaging (68). Intradural extramedullary spinal paraganglioma.

    Paragangliomas rarely present as spine tumours. The correct diagnosis is generally not suspected pre-operatively and initial imaging is often non-specific. A 36-year-old man with low back pain, and progressive leg numbness and weakness, was found to have an expansile intradural extramedullary spinal tumour on radiographs and magnetic resonance imaging. Surgery revealed a paraganglioma. The features of spinal paraganglioma and differential diagnosis of intradural extramedullary tumours are discussed. ( info)

20/260. Recurrent polytopic chromaffin paragangliomas in a 9-year-old boy resulting from a novel germline mutation in the von Hippel-Lindau gene.

    Pheochromocytomas are frequently associated with inherited cancer syndromes such as von hippel-lindau disease (VHL). Retinal angioma and hemangioblastomas of the central nervous system are hallmarks of VHL, but its clinical variety is remarkably broad. Pheochromocytomas as the sole or first manifestation of VHL are rare but have been observed. In this case report, the authors describe an unusual case of initial collapse, seizures, and hypertensive crisis in a child who later was found to have multiple extraadrenal pheochromocytomas. Molecular diagnostics revealed a novel point mutation in the VHL gene (VHL nt. 406 T-->G). Only 7 months after the first lesions had been removed, a new paraganglioma developed in the contralateral periadrenal region. When encountering pheochromocytomas in children, the clinician should be aware that an associated tumor syndrome might be present, and appropriate molecular screening should be initiated. Molecular genetics aid in the clinical decision-making and clinical management of individual patients with pheochromocytoma. ( info)
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