Aortico‐pulmonary paraganglioma.
            <i>Report of a case with ultrastructural study and review of the literature</i>

Lack, Ernest E.; Stillinger, Richard A.; Colvin, Donald B.; Groves, Robert M.; Burnette, Douglas G.

doi:10.1002/1097-0142(197901)43:1<269::aid-cncr2820430139>3.0.co;2-#

Cited by 47 publications

(6 citation statements)

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“…A careful preoperative assessment of the tumor location is crucial and determines the surgical approach. Incomplete resection and high perioperative mortality (5.3% to 9%) have been reported [ 8 , 24 ]. Using ECG gated CT in patients with suspected PGL located intracardially or intrapericardially significantly improves the image quality [ 22 ].…”

Section: Discussionmentioning

confidence: 99%

“…Paragangliomas located in the medial mediastinum arise from paraganglia in the region of the cardiac plexus [ 7 ]. The PGLs localized in the proximity of the ductus arteriosus, the main pulmonary artery and its bifurcation and the adjacent aortic arch are termed “aortic body tumors, aorticopulmonary or aortopulmonary paragangliomas” [ 8 ]. Aortopulmonary paragangliomas are usually asymptomatic [ 9 ].…”

Section: Introductionmentioning

confidence: 99%

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Mediastinal paragangliomas related to SDHx gene mutations

Michałowska¹,

Ćwikła²,

Prejbisz³

et al. 2016

kitp

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IntroductionParagangliomas (PGLs) related to hereditary syndromes are rare mediastinal tumors. Paragangliomas are caused by mutations in genes encoding subunits of succinate dehydrogenase enzyme (SDH).AimTo evaluate clinical, anatomical and functional characteristics of mediastinal paragangliomas related to SDHx gene mutations.Material and methodsRetrospective analysis of 75 patients with confirmed SDHx gene mutations (24 patients with SDHB, 5 SDHC, 46 with SDHD mutations) was performed. Patients underwent evaluation using computed tomography (CT), somatostatin receptor scintigraphy (SRS) (99mTc-[HYNIC,Tyr3]-octreotide), 123I mIBG scintigraphy and urinary excretion of total methoxycatecholamines.ResultsOut of 75 patients, 16 (21%) patients (1 SDHB, 15 SDHD mutations) had 17 PGLs localized in the mediastinum. Fourteen PGLs were localized in the middle mediastinum (intrapericardial) and 3 PGLs in the posterior mediastinum. The median diameter of paragangliomas measured on the axial slice was 24.3 mm (interquartile range (IQR): 14.7–36.6), and the median volume was 2.78 ml (IQR: 0.87–16.16). Twelve out of 16 patients (75%) underwent SRS, and 11 of them (92.3%) had pathological uptake of the radiotracer. Eleven (68.75%) out of 16 patients underwent 123 I mIBG, with only 3 positive results. Symptoms of catecholamine excretion were observed in 3 patients with PGLs localized in the posterior mediastinum. All PGLs were benign except in 1 patient with the SDHB mutation and PGL detected in the posterior mediastinum, who had a metastatic disease.ConclusionsMost mediastinal paragangliomas were related to SDHD gene mutations. They were asymptomatic, localized in the medial mediastinum, intrapericardially.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Mediastinal paragangliomas related to SDHx gene mutations

Michałowska¹,

Ćwikła²,

Prejbisz³

et al. 2016

kitp

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“…This over-lap in morphology is even more striking in gangliocytic PG of the small bowel where mixtures of paragangliomatous and carcinoid-islet cell patterns are found (Reed, Daroca & Harkin 1977). This 1978, Gallivan et al 1979, Justrabo et al 1980, Schaefer et al 1980, Hordijk et al 1981, Wetmore et al 1981, Sneige et al 1983) and of paragangliomas in other locations (Grimley & Glenner 1967, Glenner & Grimley 1974, Kahn 1976, Gullotta & Helpap 1976, Damjanov et al 1978, Lack et al 1979b, Robertson & Cooney 1980, Hull et al 1982. Sustentacular cells were present in one case of LP (Sneige et al.…”

Section: Discussionmentioning

confidence: 99%

Laryngeal neuroendocrine tumour with features of a paraganglioma, intracytoplasmic lumina and acinar formation

Spagnolo

Paradinas

1985

Histopathology

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Laryngeal neuroendocrine tumour with features of a paraganglioma, intracytoplasmic lumina and acinar formation A supraglottic laryngeal neuroendocrine tumour in a 71-year-old female is presented. In addition to the typical features of extra-adrenal paragangliomas it showed unusual, numerous intracytoplasmic lumina and occasional true acini with accumulation of alcian blue and PAS positive secretory product in them which led originally to an erroneous diagnosis of metastatic adenocarcinoma. Ultrastructurally, the tumour was composed of light and dark chief cells containing varying numbers of dense-core secretory granules. Intracytoplasmic lumina and true acini contained microvilli with glycocalyceal material and varying amounts of membranous and amorphous material, possibly secreted via small, smooth-surfaced cytoplasmic vesicles but typical much granules were not seen. These features have not been previously described in laryngeal paragangliomas but are seen in endocrine tumours of other sites and have been used as an argument in favour of an endodermal rather than ectodermal origin for endocrine cells in those sites. Laryngeal paragangliomas are often malignant and the presence of spasmodic pain appears to be the most reliable indication of possible malignancy, histological criteria being as yet poorly defined.

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“…Lack et a/., reported the case of a 15-year-old girl with an aorticopulmonary paraganglioma detected on routine chest X-ray. 4 The authors reviewed 36 cases of paraganglioma at this site and found that 50%1 were detected on routine chest X-ray in asymptomatic patients. Symptomatic patients presented with thoracolumbar sympathetic chain).…”

Section: Case Reportmentioning

confidence: 99%