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At least 19 recordsLinked to original sources

PubMed1979

Paragangliomas of the head and neck region. A pathologic study of tumors from 71 patients.

The histopathology of 72 paragangliomas originating in the head and neck region of 71 patients is presented. There were 45 carotid body, 13 vagal body, eight jugulotympanic, and three nasal paragangliomas. In addition, two arose in the larynx and one in the area of the aortic arch. Tumors occurred in four unrelated families. The two most important histologic features leading to a diagnosis of paraganglioma were zellballen and the presence of cytoplasmic argyrophil granules in all cases in which staining with the Grimelius technique was carried out. Ultrastructural study of three carotid body and two vagal body paragangliomas revealed both light and dark chief cells. Tumor cells contained membrane bound, electron dense neurosecretory types of granules, which usually ranged in diameter from 120 to 200 nm. Follow-up information was available for 67 patients (94 per cent). Two of the three nasal paragangliomas, 50 per cent of the jugulotympanic paragangliomas, 17 per cent of the vagal body paragangliomas, and 10 per cent of those of the carotid body recurred locally following attempted surgical resection. All patients treated with radiation had persistent tumor. Four (9 per cent) of the carotid body paragangliomas were malignant, all four patients dying with widespread metastases. One vagal body paragangliomas metastasized to regional lymph nodes (the patient was alive and well at five years), and another caused death by direct intracranial extension. In contrast to the benign tumors, malignant paragangliomas tended to show foci of necrosis and vascular invasion. Mitotic figures, which usually were not identified in the benign cases, were seen in all malignant tumors.

Adult↗

PubMed1978

Primary tumors of the external and middle ear. II. A clinicopathologic study of 14 paragangliomas and three meningiomas.

Paragangliomas (chemodectomas, glomus jugulare tumors) represented 15% of all neoplasms of the ear in the period from 1964 to 1975 at the University of Minnesota, Minneapolis. Women in the fifth to sixthe decades of life were mainly affected, and hearing loss and tinnitus were the principal symptoms. Although most tumors had the typical histopathologic appearance, two neoplasms that were initially diagnosed as paragangliomas illustrated the problems in differential diagnosis. One of these latter two tumors had metastasized and was thought to represent a malignant paraganglioma. Retrospectively, both neoplasms were adenomatous tumors of the middle ear. For the remaining patients with typical jugular paragangliomas, surgery and/or irradiation therapy controlled the local tumor in 12 cases. Two of the three meningiomas occurring in the middle ear were preceded by or associated with an intracranial component. There were only four prior examples in the literature, excluding our one case, of primary meningiomas of the ear-mastoid region. Histologically, the features were virtually identical to the usual intracranial meningioma.

Adult↗

PubMed1978

[Biosynthesis of C19- and C21-steroids in tissue slices of paraganglioma and pheochromocytoma (author's transl)].

The biogenesis of C19- and C21-steroids has been studied in tissue slices of a paraganglioma, of a pheochromocytoma and of human adrenal cortex using radioactive steroids. Slices of paraganglioma as well as of pheochromocytoma metabolise 17-hydroxyprogesterone to cortisone, cortisol, 11-deoxycortisol and testosterone. The rate of formation of these steroids, however, by the two tumours is 8--15 times less than that in normal adrenal cortex tissue. After incubation of dehydroepiandrosterone with tissue slices of paraganglioma and pheochromocytoma, 7alpha-hydroxydehydroepiandrosterone, 11beta-hydroxy-4-androstene-3,17-dione and 4-androstene-3,17-dione were found as metabolites; testosterone was converted by both tissues to 4-androstene-3,17-dione. 17-Hydroxypregnenolone was converted to a small extent (1.7%) to dehydroepiandrosterone by slices of paranganglioma. These results show that enzymes of steroid biosynthesis (hydroxylases, oxidoreductases, delta4(-5)-isomerases, C17(-20)-desmolases) are present in both paraganglioma and pheochromocytoma.

Adrenal Cortex↗

PubMed1975

Nonchromaffin paraganglioma (chemodectoma) of thyroid region.

A case of a paraganglioma located over the thyroid cartilage in the midline is reported. The tumor had all the appearances of a carotid body tumor, but its odd location could not be explained on the basis of previously known areas where paraganglionic tissues have been found. Since a paraganglioma has also hitherto been described within the thyroid gland, clinicians and pathologists alike are cautioned that unusually located paragangliomas may be expected in the future.

Cell Nucleus↗

PubMed1976

Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association: ultrastructural study and review.

A case of a vagal body tumor with a solitary cervical node metastasis is described. There was a remarkable familial association: a biopsy-proven paraganglioma occurred in two members of the patient's family and there was a strong clinical suspicion of a paraganglioma in two others. In a review of the literature, eight patients with metastasizing vagal body tumors and eight patients in whom the vagal body tumor was a component of multicentric presentation of paragangliomas were found. Only one other patient in whom there was a familial occurrence involving a vagal body tumor has been reported. Ultrastructural study of the cervical node metastasis revealed the presence of light and dark chief cells containing scanty, membrane-bound, densecore, neurosecretory-type granules. Atypical granules were noted in a few of the dark cells. Nerve fibers, synaptic vesicles, and sustentacular cells, such as occur in normal paraganglionic tissue, were not observed in this tumor.

Adult↗

PubMed1979

Retroperitoneal paraganglioma and systemic amyloidosis: a case report.

The case of a 50-year-old woman with a retroperitoneal paraganglioma and nephrotic syndrome is presented. After the tumor was removed deposits of amyloid material were observed in the paraganglioma, kidneys, and liver. There was no thrombosis of the renal vein. The clinical picture (fever and nephrotic syndrome) was similar to that of patients with amyloidosis associated with neoplasms. The nephrotic syndrome disappeared 8 months after the operation. The patient has not had fever or nephrotic manifestations over the past 4 years and is asymptomatic at the present time. We believe this to be the first reported case of paraganglioma associated with amyloidosis. Nor have we found any references to clinical and biological remission of the nephrotic syndrome following surgical removal of the concomitant neoplasm.

Amyloidosis↗

PubMed1979

Nonchromaffin paraganglioma of the duodenum.

A benign nonchromaffin paraganglioma of the duodenum is described and compared with other reported cases. Duodenal location is extremely rare but the morphology, based on the optical microscopic pattern (Zellballen) and the ultrastructural appearance is, comparable with paragangliomas of other sites. The lack of nerve fibres and ganglion cells in this tumour, together with the absence of a positive chromaffin reaction permits us to classify it as a pure nonchromaffin paraganglioma. This finding constitutes indirect evidence of the probable existence of a paraganglion in the duodenal wall, a structure not yet demonstrated in the adult.

Duodenal Neoplasms↗

PubMed1979

Laryngeal paraganglioma. Case report with ultrastructural analysis and literature review.

Laryngeal paraganglioma is an infrequently reported tumor; only 16 examples have been recorded in the English literature. All but one laryngeal paraganglioma originated superiorly in the larynx; involvement of the ipsilateral aryepiglottic fold is common. Male patients predominate (11:5). The average age of patients at the time of diagnosis was 47 years, and symptoms had been present for an average duration of 5.8 years (range 6 months to 27 years). Attempted biopsy has resulted in significant hemorrhage in three cases. As illustrated by the present case, the Grimelius argyrophil stain is a useful diagnostic procedure. Electron microscopy confirmed the presence of neurosecretory granules with core diameters ranging from 110 to 140 nm. Surgical resection is the preferred treatment and has been possible in 14 cases; nine patients are alive and free of tumor for an average of 3 years. Compared to other head and neck paragangliomas, these have a more malignant course with a 25% mortality; tender subcutaneous metastases are commonly observed in these patients.

Adult↗

PubMed1976

Tissue culture, electron microscopic and enzyme histochemical investigations of extraadrenal paragangliomas.

Light and electromicroscopical as well as histochemical investigations were performed on three cases of extraadrenal paragangliomas. They were localized in the carotid body, tympanicum and cauda equina region. Tissue of two cases was cultivated in vitro in nutrient medium TCM 199. The tumours were classified as paragangliomas of the paraganglionic type with typical cell clusters, of the adenomatous and angiomatous type. The enzyme histochemistry showed a very high dehydrogenase activity. Ultrastructurally numerous typical osmiophilic granules could be observed in the cytoplasm of the tumour cells. In tissue culture only a minimal cellular proliferative activity could be detected. The few proliferating cell colonies showed mostly characteristics of epithelial tissue and sometimes a similar behaviour to cells of a ganglioneuroblastoma. The minimal proliferative activity in vitro is in good agreement with the proliferative behaviour of the extraadrenal paragangliomas in vivo.

Carboxylic Ester Hydrolases↗

PubMed1978

Mediastinal paragangliomas (aortic body tumor): a report of four cases and a review of the literature.

Four cases of paraganglioma originating in the supra-aortic or aortico-pulmonary bodies are described. A review of the reported examples of aortic body tumors and a study of these four cases indicate that there is a high incidence of aggressive tumor growth in the mediastinum, with resultant important morbidity or death in 16 of 35 cases. The morphologic features of aortic body tumors are identical to those of paragangliomas of other locations, and the occurrence of invasive growth and/or metastasis cannot be predicted on histologic grounds.

Adolescent↗

PubMed1979

Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature.

The light microscopic and ultrastructural features of an aortico-pulmonary paraganglioma (A-PP) are presented. The tumor was characterized by organoid clustering of neoplastic chief cells to form Zellballen. Argyrophilic granules were demonstrated within chief cell cytoplasm using a modified Grimelus technique. Ultrastructurally, three distinct cell types were present within the tumor: endothelial cells, pericytes and neoplastic chief cells. Membrane-bound neurosecretory granules were present and measured 100 to 2000 nm in diameter. "Light" and "dark" chief cells were less distinct than previously reported in other head and neck paragangliomas. Analysis of the 36 documented A-PP reported in the English literature reveals that the tumor has been either incompletely excised or has been considered unresectable in one-third of the cases. The reported surgical mortality is 9%, or approximately equal to the incidence of malignant behavior. The treatment of choice is surgical resection but when this is not possible, radiation may be a useful adjunct in therapy.

Adolescent↗

PubMed1979

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma.

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma was found in two young female Mayo Clinic patients, and two of these tumors occurred in another two. Review of the world literature disclosed nine patients who probably had two or more of the three neoplasms. We are also aware of two other patients with two of the three tumors. The association of gastric epitheloid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma may well constitute a syndrome because of 1) the improbability of the coincidental occurrence of this triad of tumors, 2) the multicentricity of the tumors in the organs or system affected, and 3) the development of the tumors at a young age. Because two of the 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 to search for the others.

Adolescent↗

PubMed1979

A nonfunctioning paraganglioma of vagus nerve: an ultrastructural study.

The clinical, histological, and ultrastructural aspects of a cervical paraganglioma of the vagus nerve, in a 66-year-old white man, have been discussed in detail. Ultrastructurally, the tumor chief cells contained characteristic membrane-bound and dense-cored neurosecretory granules which ranged in size from 85 millimicron to 190 millimicron. Unlike earlier ultrastructural reports, the present study showed the presence of sustentacular or supporting cells. These cells were smaller, darker, polymorphic, and were commonly located at the periphery of a single or group of chief cells. Furthermore, unlike earlier reports on vagal paragangliomas, nonmyelinated nerve fibers and an occasional axon were identified in the present fine structure study.

Aged↗

PubMed1978

Ultrastructure of malignant paraganglioma of organ of Zuckerkandl.

Electron microscopic study of a malignant paraganglioma of the organ of Zuckerkandl revealed similarities between the tumor and the normal paraganglia. The well-differentiated portion of the tumor recapitulated the structure of the basic functional units of the paraganglion. In addition there signs of anaplasia both at the histologic and ultrastructural level. Large neurosecretory granules were noted in some of the tumor cells, but most of the cells were agranular. Crystaloids resembling those seen in alveolar soft part sarcoma were also noted. The study supports the theory on the common origin and histogenesis of paragangliomas and alveolar soft part sarcomas.

Chromaffin System↗

PubMed1977

Paraganglioma of cauda equina.

After a brief introduction to the problem of the nature and localisation of sympathetic and parasympathetic paragangliomas a new case of paraganglioma of cauda equina is reported.

Adult↗

PubMedDate not supplied

Multiple simultaneous paragangliomas of the head and neck in association with multiple retroperitoneal pheochromocytomas.

A patient in whom multiple, familial, catecholamine-secreting head and neck paragangliomas and retroperitoneal pheochromocytomas were identified is reported. There were at least nine primary and possibly five recurrent neoplasms, the most reported in a single patient. In patients with family history of pheochromocytoma or paraganglioma or with multiple tumors, careful laboratory and angiographic studies are indicated to discover additional lesions.

Adult↗

PubMed2025

Identification of Prognostic Gene Signatures for Survival of Patients With Phaeochromocytoma, Paraganglioma, and Other Tumor Types.

BACKGROUND/AIM: Tumor treatments remain unsatisfactory, as many patients continue to die despite therapy. There is an urgent need for novel drug targets, particularly for rare tumors. In this study, we sought to identify genes with prognostic significance for survival in patients with phaeochromocytoma or paraganglioma. We also examined whether these genes are relevant in other tumor entities. PATIENTS AND METHODS: We mined the TCGA-based KM Plotter and studied 186 risk genes for phaeochromocytoma and paraganglioma. RESULTS: Using Kaplan-Meier statistics, we performed 3,163 calculations based on 7,489 tumor biopsies and identified a 2-gene signature for phaeochromocytoma/paraganglioma (AQP4, FAM84H). Since the 186 risk genes are not exclusively related to the development of phaeochromocytoma/paraganglioma alone, we also investigated their prognostic relevance in 17 other tumor types. A clustered 12-gene signature has been found common in four other tumor entities (liver hepatocellular carcinoma, renal clear cell carcinoma, renal papillary cell carcinoma, lung adenocarcinoma). This signature consisted of BUB1, BUB1B, CDK1, CENPA, CKAP2L, IQGAP3, MKI67, NDC80, PBK, RRM2, TOP2A, and TTK. CONCLUSION: Our analysis provides a basis for the development of a novel prognostic test to predict the survival time of patients.

Kaplan-Meier analysis↗

PubMedDate not supplied

[Non-chromaffin laryngeal paraganglioma. Clinical and electron microscopical study].

The authors describe the case of a 51 year-old woman presenting with a very large laryngeal paraganglioma in the aryepiglottic fold which had passed through the cricothyroid membrane and invaled the thyroid. A crico-hyoido-pexy enabled the tumour to by completely removed and anatomo-pathological examination showed degeneration. Under electron microscopy, the ultrastructure of the tumour was similar to that of chromaffin paragangliomas. It is three years since this patient was cured.

Female↗