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PubMed1980

Computed tomography of jugulotympanic paragangliomas.

Seven cases of jugulotympanic paragangliomas were studied with computed tomography (CT) and verified by angiography and surgery. All of five glomus jugulare tumors were detected and their extent accurately demonstrated by CT. Two small glomus tympanicum tumors were not convincingly demonstrated. Computed tomography is useful in investigating this neoplasm when it extends beyond the tympanic cavity or is associated with bone destruction.

Aged↗
PubMedDate not supplied

Duodenal gangliocytic paraganglioma occurring in adolescence.

A 15-year-old boy complained of abdominal pain and massive upper gastrointestinal hemorrhage. Endoscopic evaluation demonstrated an ulcerated, submucosal mass involving the ampulla. This was locally excised and found to show the characteristic histologic and ultrastructural features of a gangliocytic paraganglioma. Similar lesions have been reported in 29 patients with a mean age of 55 years (range, 32-80). Twenty-six patients complained of abdominal pain or gastrointestinal bleeding, symptoms that mimic those of peptic ulcer disease. These tumors are uniformly benign, nonencapsulated, and submucosal. They usually arise in the second portion of the duodenum and are treated by simple excision. This patient is the youngest reported, and his case is presented as an illustration of an unusual tumor that can masquerade clinically and radiologically as a duodenal ulcer. The importance of endoscopic evaluation is stressed.

Adolescent↗
PubMed2001

Proportion of heritable paraganglioma cases and associated clinical characteristics.

OBJECTIVE/HYPOTHESIS: To determine the heritable proportion of paraganglioma (PGL) and identify clinical features associated with heritable PGL. STUDY DESIGN: Patients diagnosed with head and neck PGLs, identified retrospectively through clinical otolaryngology practices and/or participation in previous PGL research studies, were given a medical and family history questionnaire. METHODS: Questionnaire information was used to classify participants as having "heritable" or "non-heritable" cases of PGL. Classification of the participants identified through otolaryngology clinics was used to estimate the heritable proportion of PGL. Statistical analysis was performed to identify significant differences in the clinical characteristics of the heritable versus non-heritable groups. RESULTS: Among the otolaryngology clinic population, 35% were classified as having heritable PGL. Individuals with heritable PGL were younger on average than those with non-heritable PGL. The majority of non-heritable participants were female, but there was an equal gender ratio among the heritable participants. Individuals diagnosed with a carotid body tumor (CBT) were 5.8 times more likely to be classified as heritable than those diagnosed with PGL at other anatomic locations. CONCLUSIONS: Approximately 35% of individuals who present to an otolaryngologist with a head and neck PGL have inherited a predisposition for this growth. Among individuals diagnosed with head and neck PGL, those diagnosed with CBT are 5.8 times more likely to have an inherited predisposition than those diagnosed with PGL at other anatomic locations.

Adult↗
PubMed1983

The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma: a five-year review.

Among 24 patients, 6 had gastric epithelioid leiomyosarcoma, pulmonary chondroma, and functioning extra-adrenal paraganglioma, and the remaining 18 had two of the three tumors. This unusual triad of tumors almost certainly constitutes a specific entity because the coincidental occurrence of the three tumors is improbable, because the tumor multicentricity in the organs or system affected is unusual, because the tumors appeared at an age when neoplasms are uncommon, and because of the predilection of the association for young women. If two of the neoplasms are present, a presumptive diagnosis of the "triad" can be made, particularly if age and sex factors are supportive. Because two components of the "triad" are potentially lethal, it is important that patients less than 35 years of age who have any one of the three tumors be examined periodically in search of the others.

Adolescent↗
PubMed1993

Treatment of malignant phaeochromocytoma, paraganglioma and carcinoid tumours with 131I-metaiodobenzylguanidine.

131I-metaiodobenzylguanidine (MIBG) has been used for the therapy of neural crest tumours. We report our experience with this agent in nine patients (malignant phaeochromocytoma 2; malignant paragangliomas 3; malignant carcinoid tumours 4). Six patients had soft tissue metastases alone while three had both soft tissue and bone metastases. The follow-up period ranged from 8 to 110 months, the number of doses ranged from one to seven with 3.1 to 11.1 GBq per administration and a cumulative activity from 4.8 to 40.1 GBq, while the tumour response was assessed according to World Health Organization (WHO) criteria. None of the patients showed complete tumour regression. Five patients showed complete symptomatic and three showed complete hormonal responses. Three patients with carcinoid tumours died. 131I-MIBG is thus a useful therapeutic modality which provides temporary palliation of these difficult tumours, but is rarely curative.

3-Iodobenzylguanidine↗
PubMed1980

Paraganglioma of the hepatic duct.

We have described what appears to be the first case of paraganglioma of the hepatic duct at the hilum of the liver. Though extremely rare, this tumor should be included in the differential diagnosis of tumor causing biliary obstruction.

Adult↗
PubMed2002

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.

Abdominal Neoplasms↗
PubMed2006

Cervical paraganglioma mimicking a parathyroid adenoma on Tc-99m sestamibi scintigraphy.

A 32-year-old man with a clinical and biochemical profile suggestive of primary hyperparathyroidism presented with a soft tissue mass on the left side of the neck. Multiphasic planar and SPECT Tc-99m sestamibi (MIBI) scintigraphy showed focally increased tracer concentration in the mass. Subsequent histopathology and immunostaining identified the mass as a parathyroid hormone-secreting cervical paraganglioma, indicating that this tumor should be considered in the differential interpretation of MIBI concentration in the neck.

Adenoma↗
PubMed2005

Partition of calibrated tris-acryl gelatin microspheres in the arterial vasculature of embolized nasopharyngeal angiofibromas and paragangliomas.

PURPOSE: To determine the location of calibrated tris-acryl gelatin microspheres (TGMs) in the arterial vasculature of nasopharyngeal angiofibromas (NAFs) and paragangliomas (PGs). MATERIALS AND METHODS: Forty-nine specimens (25 PGs and 24 NAFs) treated operatively after embolization with TGMs of various sizes (100-300 microm to 900-1200 microm) were stained with hematoxylin and eosin saffron and analyzed at an objective magnification of 10 or 20 with a micrometric eyepiece (magnification, x12.5). The diameter of occluded vessels, their localization (intra- or extratumoral), and the number and diameter of TGMs they contained were determined. RESULTS: Embolized vessels (N = 1125) were measured: 440 in PGs and 685 in NAFs. Vessels were 89% intratumoral and 11% extratumoral. The diameter of the occluded vessels increased significantly with the size range of TGMs used for embolization for each tumor type (P < .0001). Intratumoral occluded vessels were significantly smaller than extratumoral vessels (P < .0001). Distribution of TGMs within the vascular network (intratumoral or extratumoral location) were similar for NAFs and PGs. The intratumoral and extratumoral dissemination of TGMs was different when comparing 100-300-microm TGMs versus 500-700-microm TGMs (P = .0006) as well as 300-500-microm TGMs versus 500-700-microm TGMs (P = .0001). CONCLUSIONS: The size of the vessels occluded by TGMs and their intra- or extratumoral location directly depend on the size of the injected TGMs. The vessels located inside the tumors were smaller than those located outside the tumors. A threshold for the intratumoral penetration of TGMs in the vasculature can be proposed from these data. There was no evidence of different behavior of TGMs in NAFs versus PGs.

Acrylic Resins↗
PubMed1986

Naloxone provokes catecholamine release in phaeochromocytomas and paragangliomas.

The effects of low- (2 mg) and high-dose (10 mg) intravenous naloxone administration on circulating plasma catecholamines in four patients with proven and two patients with suspected phaeochromocytomas have been evaluated. In three out of four patients with confirmed phaeochromocytomas or paragangliomas, 10 mg naloxone resulted in a marked rise (290-575%) in circulating noradrenaline; despite combined alpha and beta-adrenoceptor blockade, in two out of four cases systolic and diastolic pressures rose whilst pulse rate fell. No response was seen to 2 mg naloxone in any patient. No humoral or pressor response was obtained in the fourth patient, who may have been secreting maximal levels of plasma noradrenaline at the time of study. In the two patients with suspected phaeochromocytoma, there was no response to naloxone: further investigations failed to reveal a source of abnormal catecholamine secretion in these patients. It is concluded that naloxone represents a new pharmacological agent which can provoke catecholamine release in patients with phaeochromocytomas; in such patients, endogenous opioids may regulate catecholamine release either from the tumour itself or from the enhanced peripheral catecholamine stores.

Adolescent↗
PubMed1984

Radiotherapy of paragangliomas.

From 1964 to 1980 28 patients with extensive paragangliomas were treated with radiation therapy. Nine patients had a carotid body tumour and 19 had a temporal bone tumour. During the 16 year period the radiation dose was gradually reduced from 60 Gy in 6 weeks to 40 Gy in 4 weeks. Continuous tumour control was achieved in all patients (follow-up 1.5-18 years) with no difference between glomus caroticum tumours and glomus tympanicum tumours, and complications were few, certainly when 40-50 Gy was given in 4-5 weeks.

Adolescent↗
PubMed2004

SDHB, SDHC, and SDHD mutation screen in sporadic and familial head and neck paragangliomas.

Mutations within three genes, SDHB, SDHC, and SDHD, encoding distinct subunits of a hetero-oligomeric protein known as the mitochondrial complex II, a component of the mitochondrial electron transport chain and the Krebs cycle have been implicated in the pathogenesis of hereditary paraganglioma (PGL). This study describes a mutation screen of SDHB, SDHC, and SDHD in blood and tumor samples of 14 sporadic and three familial cases of head and neck PGL (HNP). Germline mutations in SDHB and SDHD were identified in two of the three affected individuals with familial HNP. The SDHB mutation was a novel 3 base pair, in-frame deletion of AGC at nucleotide 583-585 encoding serine (delS195). The SDHD mutation was a C to T transition within codon 81 causing substitution of proline with leucine (P81L). In contrast to familial cases, no germline or somatic mutations were identified in the 14 sporadic cases of HNP. The presence of mutations within SDHB and SDHD in two of the three samples of familial PGLs and absence of mutations in sporadic cases is consistent with the significant contribution of these genes to familial but not sporadic PGL. The etiology of sporadic PGL remains to be elucidated.

Amino Acid Sequence↗
PubMed1996

Immunohistochemical characterization of a case of duodenal gangliocytic paraganglioma.

A case of duodenal gangliocytic paraganglioma (DGP) arising in the ampulla of Vater of an 84 year old male is reported. The present case shows the typical histological features with a mixture of epitheliold, ganglion-like and spindle cells. Immunohistochemically, the spindle cells were positive with antibodies to neuron specific enorse, S-100 protein and neurofilament (NF), but negative for anti-glial fibrillary acidic protein (GFAP), synaptophysin and myelin basic protein (MBP). Both anti-Leu-7 and -chromogranin A antibodies gave positive reactions with a fine granular pattern in the epithelioid cells. Most ganglion-like cells showed the cytoplasmic immunoreaction with GFAP and a few with NF and synaptophysin. In these three tumor components, no reactivity was detected in the antibodies to the neurohormonal peptides tested. The present data are fundamentally in keeping with those previously reported, but add the immunohistochemical positivity of ganglion-like cells for GFAP, suggesting different neuroglial differentiation.

Aged↗
PubMed2005

Composite paraganglioma-ganglioneuroma of the urinary bladder.

Presented herein is the case of a 73-year-old man, complaining of dysuria, who had a composite paraganglioma-ganglioneuroma of the urinary bladder (CPGUB). At cystoscopy a submucosal tumor was found in the urinary bladder and resected after transurethral biopsy. The levels of serum catecholamine and 24 h urinary excretion of catecholamine and vanillylmandelic acid were elevated. Grossly, the resected tumor, measuring 4 x 3 x 2.5 cm, had a brownish cut surface with no necrosis and hemorrhage. Histologically, the tumor had alternating cellular and fibrous areas. The cellular areas consisted of polygonal cells, arranged in well-defined nests (Zellballen) and positive for Grimelius staining, with abundant amphophilic to acidophilic cytoplasm, occasionally containing eosinophilic hyaline globules and brown pigments. Although the nuclei of several polygonal cells were bizarre, mitoses and vascular invasion were not found. Fibrous areas consisted of spindle cells, resembling Schwann cell, admixed with ganglionic cells. To the authors' knowledge, only four cases of CPGUB have been reported in the English-language literature. Detailed reported cases and the present case showed no malignant features, such as extra-bladder infiltration and metastasis, and no recurrence in the short length of follow up. Accumulation of long-term follow-up cases may provide valuable prognostic information on this composite tumor.

Aged↗
PubMed1980

Non-chromaffin, non-functioning retroperitoneal paragangliomas: a report of three additional cases with a review of the literature.

A review of the literature has revealed only 24 cases of retroperitoneal non-functional paraganglioma recorded up to the present date. This report presents three additional cases, in two of which metastasis had occurred at the time of surgery. The clinical presentation and the biological behaviour of these tumours are reviewed in detail, as also are recommendations regarding therapy.

Adult↗
PubMed1989

Malignant paraganglioma causing bilateral pleural effusions.

A male patient presented with dyspnea due to a large left pleural effusion, and pleural biopsy revealed a malignant paraganglioma. Raised urinary catecholamine levels confirmed a functioning tumour. Aggressive local spread occurred which did not respond to cytotoxic chemotherapy. The primary site of the tumour was most likely the aorticosympathetic chain.

Aged↗
PubMed2006

An apparently sporadic paraganglioma with an SDHB gene germline mutation presenting at age 68 years.

Paragangliomas (PGLs) are rare tumours arising from parasympathetic-associated paraganglia (particularly of the head and neck) or from sympathetic-associated paraganglia such as in the adrenal medulla when they are termed phaeochromocytomas and at extra-adrenal sites in the abdomen and thorax. Recent reports have found frequent germline mutations of VHL, RET, SDHB or SDHD not only in familial cases but also in apparently sporadic cases of phaeochromocytoma. These germline mutations are particularly likely to be found if multifocal disease is present or if the phaeochromocytoma or PGL occurs at a young age. We report a germline splice site mutation in SDHB in a patient presenting with an incidental, apparently sporadic, abdominal sympathetic PGL at 68 years of age.

Abdominal Neoplasms↗