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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↗

PubMed1986

Melanotic paraganglioma of the uterus.

Two cases of melanotic paraganglioma of the uterus are reported. Interpreted as a pigmented paraganglioma, both lesions were an incidental finding in uteri removed for unrelated benign conditions. Microscopically, both tumors were circumscribed, and composed of nests of large round or angulated polygonal cells with abundant clear or granular pale, eosinophilic cytoplasm. Large amounts of coarse intracytoplasmic melanin pigment were present in many cells, whereas other cells were completely devoid of pigment. Both lesions contained psammoma bodies. Electron microscopy showed abundant intracellular melanosomes, including many compound melanosomes and few premelanosomes. A lamellar whorled structure was the dominant infrastructure on which melanin pigment was deposited in many cells. The absence of microvilli or dendritic cell processes and basal lamina, as well as the lack of S-100 protein, pointed against a Schwannian or melanocytic differentiation. The presence of abundant pigment and only rare neuroendocrine-like secretory granules in the cytoplasm of a few cells indicated divergence from classic paraganglioma. Architecturally, this lesion is similar to a paraganglioma; but, functionally, instead of neuroendocrine granules, the tumor cells produce melanin pigment.

Adult↗

PubMed1989

Nasal and nasopharyngeal paraganglioma.

Paragangliomas of the nasal cavity and nasopharynx should be classified according to the presumed origin from the nasal, jugulotympanic, vagal, or ciliary paraganglion because of the substantial differences in clinical behavior and operative management. This paper presents the ninth published case of a primary nasal paraganglioma and reviews the previously reported cases. Primary nasal paragangliomas are generally localized, although the potential for local invasion is well documented. Malignancy or functional activity has not been described. Complete excision is curative. Jugulotympanic, vagal, and ciliary paragangliomas with extension into the nasopharynx or nasal cavity are typically more advanced at the time of diagnosis and require complete evaluation by computed tomography and arteriography to determine the tumor extent. The operative approach is dependant on the extent of tumor invasion. Irradiation and tumor embolization have been useful for unresectable or partially excised tumors.

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↗

PubMed1992

MRI screening of kindred at risk of developing paragangliomas: support for genomic imprinting in hereditary glomus tumours.

Paragangliomas of the head and neck (glomus tumours) can occur in a hereditary pattern and may be hormonally active as well as being associated with paragangliomas elsewhere. A number of these tumours may be present without symptoms. To detect the presence of subclinical paragangliomas we screened 83 members of a family at risk of developing hereditary paragangliomas using whole body MRI and urinary catecholamine testing. In eight previously diagnosed members, eight known glomus tumours of which one functioning, and two unknown glomus tumours and one unknown pheochromocytoma were present. Six unsuspected members showed ten glomus tumours and one pheochromocytoma. It has been suggested that the manifestation of hereditary glomus tumours is determined by the sex of the transmitting parent. There were no tumours in the descendants of female gene carriers. Comparing the likelihood of inheritance with genomic imprinting versus inheritance without genomic imprinting we found an odds ratio of 23375 in favour of genomic imprinting.

Carotid Body↗

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↗

PubMed1986

Demonstration of S-100 protein in sustentacular cells of phaeochromocytomas and paragangliomas.

Eighteen phaeochromocytomas, including both sporadic and familial cases, four cervical paragangliomas, two jugular paragangliomas, and one abdominal paraganglioma were examined immunohistochemically for the presence of S-100 protein. Positive staining in cells morphologically similar to the sustentacular cells of normal paraganglia and adrenal medulla were found in all paragangliomas and in the benign and aggressively growing phaeochromocytomas. In the two malignant tumours no positive reaction was demonstrated. In one tumour the sustentacular cells were shown to contain glial fibrillary acidic protein further supporting their Schwann cell relationship. The number of S-100 positive cells varied considerably. They demonstrated a spindle celled or elongated configuration with long slender processes. The nature of the sustentacular cell proliferation, neoplastic versus reactive, is discussed.

Adolescent↗

PubMed1991

Immunocytochemistry of paragangliomas--value of staining for S-100 protein and glial fibrillary acid protein in diagnosis and prognosis.

Surgical specimens of 65 adrenal and 27 extra-adrenal paragangliomas, the latter comprising 11 carotid body, five jugulotympanic, one aorticopulmonary, eight aorticosympathetic and two visceral autonomic tumours, were examined immunocytochemically for the presence of glial fibrillary acid protein (GFAP) and S-100 protein. Six adrenal and four extra-adrenal (one parasympathetic and three sympathetic) neoplasms pursued a malignant clinical course. S-100 staining of sustentacular (type 2) cells was seen in both adrenal (48/65) and extra-adrenal (23/27) lesions, the 10 malignant tumours being entirely devoid of S-100 protein positive cells. GFAP positivity of type 2 cells was seen in only 16 of the extra-adrenal tumours, all of these lesions belonging to the group of benign parasympathetic paragangliomas. The presence of S-100 positive type 2 cells may thus help to exclude malignancy in individual paraganglioma cases, while GFAP positivity of such cells renders possible the correct typing of benign parasympathetic paragangliomas.

Adrenal Gland Neoplasms↗

PubMed1987

Paraganglioma of the cauda equina: a case report.

This case report describes a paraganglioma of the cauda equina in a 63 year old woman. Very few examples of paraganglioma have been recorded in this site. Histologically these neoplasms may have considerable similarity with ependymoma, the most common neoplasm of the lower spinal cord, and the diagnosis can be easily missed unless special techniques are employed. The clinical and pathological data obtained from this and the other reported examples suggests that paragangliomas of the cauda equina are benign, slowly growing neoplasms. In contrast to ependymomas and to paragangliomas elsewhere, they are well circumscribed, amenable to complete resection and have an excellent prognosis.

Aged↗

PubMed1991

Paraganglioma of the larynx. A critical review of the literature.

The clinicopathologic features of two cases of paragangliomas of the larynx are presented along with immunohistochemical, ultrastructural and DNA image analysis. The world literature is also critically reviewed in order to develop a more accurate clinicopathologic profile of the tumor. The results indicate that paragangliomas of the larynx occur in patients averaging 47 years of age and are 3 times more common in women. Most (82%) arise from the supraglottic larynx and manifest clinically as hoarseness. They are typically benign and rarely functional. Almost all alleged malignant paragangliomas of the larynx are in reality atypical carcinoids that have been misdiagnosed. Features that distinguish paragangliomas from other similar tumors are discussed.

Adult↗

PubMed1992

Laryngeal paraganglioma and pregnancy.

Paraganglioma is an uncommon tumor of the larynx. We present a 30-year-old woman with a paraganglioma which took a heretofore not described clinical course. The first clinical sign was persistent hoarseness, when she was 26 weeks pregnant. A histological examination of a piece of the reddish, racemose tumor removed during microlaryngoscopy revealed only some slight nonspecific inflammatory changes. The patient remained free of symptoms following this operative procedure until 3 years later when she presented with a recurrence of hoarseness and a laryngeal tumor and was 25 weeks pregnant. Histology showed a laryngeal paraganglioma in the same supraglottic localization as before. Histological studies failed to confirm the obvious suspicion that the episodic growth of the paraganglioma may have been induced by estrogen or gestagen. To date, 6 years after the removal of the tumor, there has been no recurrence.

Adult↗

PubMed1986

Multiple endocrine neoplasia with Cushing's syndrome due to paraganglioma producing corticotropin-releasing factor and adrenocorticotropin.

A male patient with corticotropin-releasing factor (CRF) and adrenocorticotropin (ACTH)-producing syndrome is described. Soon after being referred to us the patient developed pneumonia, anaemia, oedema and respiratory distress, and died on the 24th day after admission. Autopsy and histology revealed that he had a rare type of multiple endocrine neoplasia (type 1 + paraganglioma) with a mediastinal paraganglioma, parathyroidal hyperplasia, pancreatic islet cell adenoma, duodenal multiple carcinoid tumours and adrenocortical nodular hyperplasia. It was not possible to examine the pituitary. The paraganglioma contained a large amount of immunoreactive (IR)-CRF (606 ng/g wet weight), IR-ACTH (59.4 ng/g wet weight), IR-human proopiomelanocortin n-terminal (1-76) peptide (hNT, 156.8 ng/g wet weight) and IR-beta-lipotropin (beta-LPH, 146.9 ng/g wet weight). The major IR-ACTH, beta-LPH and IR-hNT were eluted at ACTH-(1-39), beta-LPH and hNT marker positions, respectively. Big ACTH was not detected. IR-CRF eluted at the human CRF marker position on Sephadex G-75 chromatography and high performance liquid chromatography (HPLC). The IR-CRF fraction from the HPLC showed CRF bioactivity which paralleled that of synthetic human CRF in monolayer cultured rat anterior pituitary cells. Our results suggest that not only ACTH but CRF produced by the paraganglioma was responsible for the patient's Cushing's syndrome.

Adrenocorticotropic Hormone↗

PubMedDate not supplied

Paraganglioma of the cauda equina: an ultrastructural and immunohistochemical study of two cases.

The ultrastructural and immunohistochemical features of 2 paragangliomas arising in the cauda equina are described. In both cases the tumor cells were arranged in small nests or cords and contained characteristic neurosecretory granules, lamellar stacks of rough endoplasmic reticulum (RER), and some well-developed Golgi apparatuses in their cytoplasm. The cells varied in electron density; the darker cells, occasionally resembling sustentacular cells, were probably dehydrated light cells because they contained a few neurosecretory granules. Sustentacular cells were difficult to identify by electron microscopy, but irregularly distributed S-100 protein and glial fibrillary acidic protein (GFAP) were found in these cells by immunostaining. Many tumor cells contained abundant neurofilaments. Curiously, a few cytokeratin-positive cells were found in 1 case. On microscopic examination, a small area of ganglioneuroma was found associated with the paraganglioma in 1 case. Ganglionic differentiation was concluded to be frequent in paragangliomas of the cauda equina region as in duodenal paragangliomas.

Adult↗

PubMed1991

[Immunohistochemical study of 6 multiple familial cervical paragangliomas with lymph node metastasis in one case].

Cervical localizations of extra-adrenal paragangliomas are infrequently malignant, especially in their familial forms. An immunohistochemical study was performed on 6 cervical paragangliomas which were detected in 3 sisters at the age of 20 20. One had a lymph node metastasis. This study confirmed the diagnosis of paraganglioma with endocrine chief cells and supratentacular cells. It also enabled the hormonal contents of these cervical paraganglioma to be determined. In addition to catecholamine, these tumors may (like pheochromocytomas) produce serotonin and one or more other peptides.

Adult↗

PubMed1991

[A case report of mediastinal paraganglioma and a review of the literature in Japan].

Paraganglioma is a tumor arising from the extra-adrenal paraganglion system and is rarely observed in the mediastinum. The authors experienced a case of nonfunctioning paraganglioma of the upper posterior mediastinum. The patient was a 40-year-old female in whom abnormal shadows were noted in chest X-ray films taken in March, 1989. She was examined at our hospital with a diagnosis of mediastinal tumor in June of the same year. CT of the chest revealed a tumor in the upper posterior mediastinum and it was shown to be hypervascular by a DSA study. Urinary hormone levels were normal. The tumor was resected in May, 1990. The tumor, measuring 6.8 X 4.0 X 3.5 cm, was encapsulated, appeared dark red and had relatively clear borders. A histopathologic diagnosis of paraganglioma was made. The patient remains disease-free at present, 6 months after operation. Problems concerning diagnosis and treatment of mediastinal paraganglioma were evaluated with a review of cases reported in Japan.

Adult↗

PubMed1990

[A case of malignant aortico-pulmonary paraganglioma].

A 64-year-old male was admitted to our hospital suffering from dysphagia and dyspnea. The chest X-ray films and chest CT films revealed huge tumor in his upper-anterior mediastinum. The filling defect at lower rim of the left brachiocephalic vein on venography indicated malignant potential of the tumor. There was no abnormal value in blood and urine examinations except CA 19-9 which was 31.8 U/ml. The tumor was resected with bilateral pleura, pericardium and left brachiocephalic vein. Macroscopically the specimen accompanied with a lot of bleeding spots was soft and yellow and has no capsule. The size of tumor was 15 X 13 X 8 cm and its weight was 865 gm. No tumor recurrence was found until present time: two years after surgical therapy followed by adjuvant radiation therapy (60 Gray). Though histological findings of anterior part of the tumor were consisted of "Zellballen" cells and bleedings, the posterior part had cell atypia and invasion into vessels. These cells were slightly positive on Keratin stain and on NSE stain. Typical findings of paraganglioma were also indicated by electron microscopy: those findings were more chief cells, fewer sustentacular cells, abundant mitochondria and few dense-cored granules which are thought to secrete hormonal substances. Judging from these clinical and pathological results, this tumor was diagnosed nonfunctional aortico-pulmonary paraganglioma. Mediastinal paraganglioma is a very rare tumor. There is no report of malignant aortico-pulmonary paraganglioma in Japan after Glenner and Grimelys' study concerning extra-adrenal paraganglion system.

Aorta↗

PubMed1990

Nonfunctioning paraganglioma of the liver, gallbladder and common bile duct.

Paragangliomas are rare extra-adrenal neoplasms of neural crest origin. Although most of these lesions are of retroperitoneal origin, paragangliomas have been reported at a host of remote sites, including the urinary bladder, larynx, orbit and lung. In our review of the literature, we discovered only solitary case reports of such neoplasms involving the gallbladder and hepatic duct. We report a case of paraganglioma simultaneously involving the liver, gallbladder and common bile duct, as well as celiac and portal lymph nodes. Our findings add paraganglioma to the list of differential diagnoses for hypervascular portacaval space lesions. Furthermore, the radiographic appearance of this unusual tumor may be sufficiently distinctive to suggest the correct histologic diagnosis preoperatively.

Cholangiopancreatography, Endoscopic Retrograde↗

PubMed1987

Localization and treatment of familial malignant nonfunctional paraganglioma with iodine-131 MIBG: report of two cases.

Two cases of familial, malignant, nonfunctional paraganglioma are reported. Uptake of iodine-131 metaiodobenzylguanidine ([131I]MIBG) by the tumors and metastases was demonstrated. In the first case, with multicentric and locally invasive disease, [131I]MIBG correctly localized a right carotid body paraganglioma which had been missed arteriographically. In the second case, with widespread, symptomatic metastatic disease, a therapeutic dose of [131I]MIBG produced palliation of bone pain after the failure of radio- and chemotherapy. Uptake of [131I]MIBG by paragangliomas does not correlate with catecholamine secretory activity. Iodine-131 MIBG should be considered as a therapeutic option in unresectable, malignant paragangliomas which take up this radiopharmaceutical.

3-Iodobenzylguanidine↗