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PubMed2000

Metastatic paraganglioma causing spinal cord compression.

We describe two cases of metastatic retroperitoneal paraganglioma associated with extradural spinal cord compression. Both occurred in young men; one being metastatic at presentation, the other becoming metastatic 19 years after attempted surgical resection. Despite a long, relatively stable natural history after diagnosis (10 and 19 years, respectively) both had an acceleration of their disease once extradural disease was detected. These cases illustrate the potentially aggressive nature of this disease, the need for long-term follow-up and the effectiveness of a variety of therapies for palliation, and also raise the possibility of "prophylactic" treatment to prevent spinal cord compression.

Adolescent↗
PubMed1985

Carotid body paraganglioma with metastases.

The diagnosis of a carotid body paraganglioma with metastases led us to a critical review of the literature of 106 cases with metastasis reported between 1893 and 1980. The percentage of tumors with metastasis is in the order of 11.5% to 13%. There is no proven histological criterion from which to assert or infer their malignancy.

Aged↗
PubMed1988

Dopamine in paragangliomas of the glomus jugulare.

Glomus jugulare tumors have the ability to synthesize, store, and secrete biogenic amines. Although the majority of these tumors remain endocrinologically silent, on rare occasions they present either as a pheochromocytoma or with a carcinoid syndrome. We report a 20-year-old male with two intracranial tumors: an intrasellar neoplasm and a glomus jugulare tumor. Catecholamine catabolites in the urine were not increased, and blood pressure was always normal. The pituitary tumor was an adenoma, immunostaining positive for prolactin. The second patient, a 29-year-old hypertensive male, with a glomus jugulare tumor, had increased vanillylmandelic-acid excretion. In both cases, the paraganglioma tumor cells contained numerous dense-core vesicles (125 to 380 nm in diameter) in electron microscopy, and showed intense fluorescence by the sucrose-potassium phosphate-glyoxylic acid method. Using high-performance liquid chromatography and microspectrofluorometry we were able to establish the presence of large amounts of dopamine in the cytoplasm of the tumor cells.

Adenoma↗
PubMed2001

Neurofibromatosis-1 and ampullary gangliocytic paraganglioma causing biliary and pancreatic obstruction.

Gangliocytic paragangliomas (GPs) are exceedingly rare tumors that arise in close proximity of the papilla of Vater. Nevertheless, jaundice is an uncommon presenting symptom, reported in only 3 of 125 cases described in the literature to date, with gastrointestinal bleeding being more common. Association between GPs and neurofibromatosis 1 (NF-1), described in two patients, may be more than casual, being the frequency in normal population 1:3,000. We report an additional case of this association, presenting for the first time with simultaneous obstruction of the common biliary duct, and of the main pancreatic duct. Previous cases of GPs with jaundice and/or NF-1 are reviewed, and updating of the histogenesis presented.

Ampulla of Vater↗
PubMed2026

Biomarkers of metastatic disease in pheochromocytoma and paraganglioma.

Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors with variable metastatic potential. While metastatic disease occurs in approximately 10-20% of cases, its prediction remains a major clinical challenge, as no histological system has been universally validated to reliably identify aggressive tumors at diagnosis. This review aims to provide a comprehensive and updated overview of current and emerging biomarkers of metastatic risk in PPGL, encompassing histopathological scoring systems, genetic and molecular markers, biochemical phenotyping, liquid biopsy approaches, and imaging-based biomarkers. Among established markers, germline SDHB mutation status, loss of SDHB expression by immunohistochemistry, elevated plasma 3-methoxytyramine, and histopathological scoring systems, such as GAPP and COPPS, represent the most clinically validated tools for risk stratification. Emerging biomarkers - including somatic alterations in ATRX and TERT, genomic instability indices, tumor immune microenvironment characterization, circulating tumor DNA, and oncometabolite quantification - show promise in refining prognostic assessment but require prospective validation before routine clinical implementation. Accurate risk stratification in PPGL demands a multiparametric and dynamic approach, integrating clinical, genetic, biochemical, and molecular parameters. Future progress will depend on large prospective international cohorts, standardized biomarker platforms, and biomarker-driven clinical trial designs to translate emerging molecular knowledge into improved patient outcomes.

SDHB↗
PubMed1992

Paraganglioma associated with hypoglycemia.

A 63-year-old female had frequent attacks of palpitations with sweating and occasional loss of consciousness for 7 years before admission. These symptoms and documented hypoglycemia (blood glucose: 46 mg/dl) suggested insulinoma, but computed tomography demonstrated a retroperitoneal tumor which was diagnosed as a paraganglioma by histological examination of the resected specimen.

Adrenal Glands↗
PubMed1997

Basilar invagination associated with an intercarotid paraganglioma--case report.

A 24-year-old female presented with basilar invagination and kyphoscoliosis of the cervical spine associated with a large intercarotid paraganglioma. She had suffered from pharyngeal discomfort from the age of 9 years due to the tumor. The tumor had originated from the right carotid body and extended in the parapharyngeal space compressing the upper cervical spine. Presumably the slowly growing tumor had caused the kyphoscoliosis and disturbed osseous development of the occipito-atlanto-axial complex, resulting in anterior basilar invagination, hypoplasia of the clivus, and aplasia of the posterior arch of the atlas.

Adult↗
PubMed1989

Paragangliomas of the jugular bulb and carotid body: MR imaging with short sequences and Gd-DTPA enhancement.

Twenty-six patients with glomus jugulare (16), glomus tympanicum (three), or carotid glomus (seven) tumors were examined with contrast-enhanced CT scans and MR scans without and with Gd-DTPA. MR and CT scans had similar sensitivities, but the enhanced MR scans were diagnostically more specific than either CT or nonenhanced MR. Dynamic MR scanning permitted measurement of the degree of Gd-DTPA enhancement over time. We recommend contrast-enhanced MR with short sequences and a dynamic approach in patients with suspected carotid, tympanic, and jugular paragangliomas.

Carotid Body Tumor↗
PubMedDate not supplied

Crystalloid structures in retroperitoneal paragangliomas: a light and electron microscopic study.

Samples from three clinically functional retroperitoneal paragangliomas were studied by light and electron microscopy. The tumors exhibited a Zellballen pattern histologically, and ultrastructurally all three neoplasms consisted of cells containing catecholamine granules. Prominent cytoplasmic crystalloids were present in all cases. The crystalloids were identified in routine histologic sections, demonstrated eosinophilia, and stained with periodic acid-Schiff, Giemsa, phloxine-tartrazine, and azan stains. Ultrastructurally the crystalloids were osmiophilic, often appeared as slender needles, were membrane bound, and demonstrated a periodicity of 9 nm. The crystalloids, unlike the catecholamine granules, were negative for catecholamine fluorescence. X-ray microanalysis, however, revealed the selective presence of chromium in both catecholamine granules and crystalloids.

Adult↗
PubMed1991

Paraganglioma of the cauda equina: case report and review of the literature.

A rare paraganglioma of the cauda equina is reported. The clinical, radiological and histological features of this neoplasm are described. Diagnosis was resolved by the absence of glial fibrillary acid protein and electron microscopic evidence of neurosecretory features. Relevant literature is reviewed.

Adult↗
PubMed1991

Cerebello-pontine angle paraganglioma simulating an acoustic neurinoma.

An unusual case of paraganglioma arising from the internal auditory meatus and growing into the cerebello-pontine angle is reported. Clinical features, pre-operative radiological appearances and intra-operative findings were indistinguishable from those of an acoustic neurinoma. However, the paraganglionic nature of the tumour was confirmed by the electron microscopic evidence of neurosecretory features.

Adult↗
PubMedDate not supplied

Paraganglioma of the vagina: the first report of a rare tumor in a child.

Paraganglioma (extra-adrenal pheochromocytoma) is a rare tumor, particularly in childhood. Those in the female genital tract are exceptionally rare, with only 9 cases reported in detail since 1926. All were seen in adults and only two arose in the vagina. This study examined the incidence of this tumor in the childhood population of the West Midlands region of the United Kingdom since 1957 and found 4 cases: 2 abdominal, 1 para-aortic, and 1 carotid body. A recent additional case is reported in the vagina of a child aged 11 years, who presented with heavy vaginal bleeding in the absence of hypertension. Initial diagnosis suggested rhabdomyosarcoma and near complete excision was carried out. Since this is the first such case to be described in a child, the outcome can only be assumed. Although histopathological examination suggested the benign nature of the tumor, implying that surgical excision was sufficient treatment, close follow-up is recommended.

Adolescent↗
PubMed1990

Resection of high-cervical paraganglioma with cervical-to-petrous internal carotid artery saphenous vein bypass. Report of two cases.

Two patients with large high-cervical paragangliomas were treated with radical resection and placement of a cervical-to-petrous internal carotid artery saphenous vein bypass. The high-cervical and infratemporal segments of the internal carotid artery engulfed within the tumor were resected and successfully replaced with a saphenous vein interposition graft. The postoperative course was uneventful in both cases and follow-up angiography revealed satisfactory reconstruction of the high-cervical and infratemporal skull base carotid artery. The operative technique, indications, and potential future applications of this newly developed skull base bypass procedure are discussed.

Adult↗
PubMed1999

Gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma (Carney Triad): natural history, adrenocortical component, and possible familial occurrence.

OBJECTIVE: To investigate the natural history of the triad of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma, a rare syndrome of unknown cause primarily affecting young women. METHODS: Mayo Clinic records, the world literature, and the author's files were searched for patients with all or 2 of the 3 tumors. RESULTS: Seventy-nine patients, 67 women and 12 men, were identified, 17 (22%) with the 3 tumors and 62 (78%) with 2 tumors. Forty-two (53%) had gastric and pulmonary tumors, the most common combination. The longest interval between detection of the first and second components was 26 years (mean, 8.4 years; median, 6 years). Follow-up ranged from 1 year to 49 years (mean, 20.6 years; median, 20 years). Sixty-four patients (81%) were alive, 19 (24%) apparently free of disease and 45 (57%) with residual or metastatic tumors. Thirty-two patients (41%) had had 1 or more local recurrences of the gastric sarcoma; the longest interval to first recurrence was 36 years. Twenty-one survivors (27%) had hepatic metastatic gastric sarcoma with follow-up of 1 year to 25 years (mean, 9.3 years; median, 7 years). Fifteen patients (19%) were dead, 10 (13%) of whom died of the disorder. Ten patients (13%) had nonfunctioning adrenocortical tumors. Two patients each had a sibling with 1 component of the triad. CONCLUSIONS: The triad is a chronic, persistent, and indolent disease. Benign adrenocortical tumors are a component of the condition. The disorder may be familial.

Adolescent↗
PubMedDate not supplied

[Duodenal gangliocytic paraganglioma].

We present the case of an 85 year old male who was admitted to hospital with abdominal pain and jaundice. Different explorations were performed for this reason, with a mass observed in his 2nd duodenal portion. Histological study showed that it was a duodenal gangliocytic paraganglioma. The clinical characteristics of this infrequent tumour are described and we review the diagnosis and treatment.

Aged↗