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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↗

PubMed1977

Paragangliomas of the head and neck region: a clinical study of 69 patients.

Over a 38-year-period 73 paragangliomas from the head and neck region were seen at Memorial Hospital. These occurred in 69 patients. There were 44 carotid body, 13 vagal body, eight jugulo-tympanic, and three nasal paragangliomas. In addition, one each arose in the orbit, larynx, and area of the aortic arch. Sixty-two patients were surgically treated while three received irradiation only and four were observed but not treated. Follow-up was obtained for 94% of the patients. There were six postoperative deaths, five of which occurred before 1945. Of those treated surgically, 37 had no evidence of recurrent tumor while the paragangliomas recurred locally in 11. All patients in the radiation and untreated groups had persistent tumor. Malignant behavior with death due to tumor was observed in five cases (four carotid body and one vagal body paraganglioma). Using modern techniques carotid body and vagal body paragangliomas can usually be managed by surgery alone. Other paragangliomas of the head and neck due to their anatomic location are sometimes best treated by a combination of surgery and radiation or only by radiation therapy.

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↗

PubMed1977

Gangliocytic paraganglioma.

Six cases of upper intestinal gangliocytic paraganglioma, five in the duodenum and one in the jejunum, are reported. Three histologic patterns, each comparable to patterns in paraganglioma, ganglioneuroma, or carcinoid-islet cell tumor, are mixed in varying proportions. The complex histologic patterns encountered within these tumors reflect the differentiation of pluripotent cells. Although the parent cell is not identified in this study, gangliocytic paragangliomas may arise from cells which normally reside in the mucosal crypts and are presumably derived from cells originating in the neural crest. Three of the six tumors contain stromal amyloid. In one case, studied by electron microscopy, dense-cored cytoplasmic vesicles almost fill some of the tumor cells. Gangliocytic paragangliomas share the features of stromal amyloid and dense-cored vesicles with other neurocrine tumors.

Adult↗

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↗

PubMed1977

Malignant vagal paraganglioma: report of a case and review of literature.

A case of malignant vagal paraganglioma is presented. The literature on the subject is briefly reviewed. This report brings to 48 the number of vagal paragangliomas now in the medical literature. Eight of these cases are found to be acceptable examples of malignant paraganglioma with evidence of lymph-node or distant metastases, and pertinent findings from these cases are summarized. Salient features of other cases showing extensive local or vascular invasion are also reviewed.

Adult↗

PubMed1992

A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter.

Paragangliomas of the head and neck are slow growing tumors which rarely show malignant progression. Familial transmission has been described consistent with an autosomal dominant mode of inheritance. Clinical manifestations of hereditary paragangliomas are determined by the sex of the transmitting parent. All affected individuals have inherited the disease gene from their father, expression of the phenotype is not observed in the offspring of an affected female until subsequent transmittance of the gene through a male carrier. This finding strongly suggests that genomic imprinting is involved. We report the results of a linkage study on a large Dutch pedigree with hereditary paragangliomas. Highly significant evidence for genetic linkage to chromosome 11q23-qter with the anonymous DNA marker D11S147 was detected with a peak lod score of 6.0 at a recombination fraction theta = 0.0. Likelihood calculations yielded an odds ratio of 2.7 x 10(6) in favor of genomic imprinting versus the absence of genomic imprinting.

Chromosome Mapping↗

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↗

PubMed1977

Mediastinal paraganglioma causing spinal cord compression.

An invasive paraganglioma of the posterior mediastinum caused spinal cord compression in a 31 year old women. Electron microscopic examination of the paraganglioma invading the epidural space revealed numerous dense-cored granules in the cytoplasm of the tumour cells. We are reporting this case to present the ultrastructure of mediastinal paraganglioma, and to call attention to an unusual cause of spinal cord compression.

Adult↗

PubMed1992

[A case of subclavian paraganglioma in the superior mediastinum extending to the neck].

A 52-year-old female was admitted to our hospital with left anterior chest pain. On physical examination, an elastic hard mass sized in 3.0 x 2.5 cm was palpable at the left suprasternal area. Chest X-ray film showed a large mass shadow in the cervico-thoracic region, and CT films revealed a well-defined mass in the superior mediastinum which was heterogeneously enhanced by contrast medium. Operation was performed under the preoperative diagnosis of thymic tumor extending upwards. At operation, the tumor was located between the trachea and the left subclavian artery in the superior mediastinum displacing these structures and extending to the neck. Resected specimen which was 5.5 x 3.5 x 3.0 cm in size and 42 gm in weight was well encapsulated and highly vascularized. On microscopic findings, the tumor was finally diagnosed as paraganglioma. According to the classification of Glenner and Grimley, it was thought that the tumor originated from subclavian paraganglion in the superior mediastinum. Subclavian paraganglioma had rarely been reported in the literatures. As subclavian paraganglioma, our patient was the first reported case in Japan.

Female↗

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

[Paraganglioma of the posterior mediastinum (author's transl)].

The authors describe one case of paraganglioma of the posterior mediastinum successfully removed after being discovered accidentally in a young male patient who showed no symptoms attributable to the tumor. The latter was a round mass, about 6 cm in diameter, richly vascular, located in the right costomediastinal gutter. Histologically it was a functionally silent paraganglioma originating from the intrathoracic aortosympathetic paraganglia, these being part of the very numerous groups of the paragangliar system distributed segmentally into metamers, particularly in the adrenal medulla. According to the more recent views, chemodectoma or paraganglioma (chromaffine or non-chromaffine, functionally active or silent) and pheochromocytoma (functionally active or silent, adrenal or extra-adrenal) constitute a group of tumors sharing the same tissue matrix, namely paragangliar cells.

Adult↗

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↗