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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↗

PubMed1994

Further localization of the gene for hereditary paragangliomas and evidence for linkage in unrelated families.

Paragangliomas of the head and neck are slow-growing tumors that rarely show malignant progression. Familial transmission has been described, consistent with an autosomal dominant gene that is maternally imprinted. Clinical manifestations of hereditary paraganglioma 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 or female gene carrier until subsequent transmittance of the gene through a male gene carrier. Recently, we assigned the gene responsible for paragangliomas (PGL) to chromosome 11q23-qter by linkage in a single large Dutch kindred. We now report confirmation of this localization in five unrelated Dutch families with hereditary paragangliomas. On the basis of segregation of haplotypes in the available family material, we localize the PGL locus between markers STMY and CD3D on chromosome 11q22.3-q23.

Chromosomes, Human, Pair 11↗

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↗

PubMedDate not supplied

Pheochromocytoma and paraganglioma in children: a report of 24 cases of the French Society of Pediatric Oncology.

Pheochromocytoma and paraganglioma of childhood are rare neuroendocrine tumors. Urinary catecholamine measurements, metaiobenzylguanidine (MIBG) scanning, computed tomographic scanning, and magnetic resonance imaging have greatly facilitated diagnosis. Prognosis after surgical resection is excellent. In this retrospective series collected from French oncology centers, the risk of tumor progression was studied in order to assess prognostic factors and the optimal diagnostic and therapeutic management. Medical records of 24 children with paraganglioma were reviewed. This tumor occurred at a median age of 12.5 years and in most cases was revealed by arterial hypertension. The diagnosis was made by the demonstration of urinary excretion of catecholamines and their metabolites. Six patients had bilateral adrenal pheochromocytomas; two patients had extra-adrenal paragangliomas. In eight patients, the paraganglioma occurred as a familial disease. Surgical excision was the only therapeutic procedure. With a follow-up of 5.2 years, 14 of the patients are still in first complete remission and 6 have developed metastases or shown tumor progression. Despite a high long-term survival rate, the risk of malignancy and of multifocal involvement is of concern and is associated with a significant rate of late events. The outcome depends on adequacy of tumor resection and must be serially assessed.

Adolescent↗

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↗

PubMedDate not supplied

[Paraganglioma (chemodectoma) of the larynx. Presentation of 2 cases].

Two rare cases of laryngeal paraganglioma are reported. The first patient was a 14-year-old girl, that had a new laterocervical paraganglioma, 4 years after the excision of a laryngeal tumor. The other patient was a 33-year-old man. In both the cases, the diagnosis was made on histopathological ground and the tumors displayed the typical histological pattern of paragangliomas: epithelioid tumor cell arranged in nests (Zell-ballen) in a vascular stroma. Neoplastic cells were found to contain argyrophil granules and chromogranin. Laryngeal paragangliomas reported in the literature are 73, they generally are supraglottic, show no sex predilection and prefer the V-VII decades of age.

Adolescent↗

PubMed1989

A review of the histology, ultrastructure, immunohistology, and molecular biology of extra-adrenal paragangliomas.

This review summarizes our understanding of extra-adrenal paragangliomas, a subject that has evolved considerably during the past several years. Our object was to review the anatomical, histologic, and biological features of normal and neoplastic glands, with emphasis on immunohistologic studies, and briefly discuss the potential application of nucleic acid hybridization. Since it is difficult to predict clinical outcome for patients with paragangliomas, we have emphasized the differences between benign and malignant paragangliomas, concentrating on recent results obtained using immunohistologic techniques. These studies have emphasized the critical importance of the identification, by immunohistologic means, of two distinct cell populations, chief cells (type I) and sustentacular cells (type II). The relationship between these two cell populations, stable in normal glands and benign tumors, is progressively lost in tumors of increasing degrees of malignancy, sustentacular cells being absent from the most progressively metastasizing paragangliomas.

Adrenal Gland Neoplasms↗

PubMed1989

[Laryngeal paraganglioma].

A case is reported of laryngeal paraganglioma observed among 49,959 patients. A comparison is made of the characteristics of paragangliomas in general to those of laryngeal paraganglioma, which are more common in males; the case presented is that of a woman. Microscopic dissection failed to reveal the paraganglia in the trajectories of the superior and inferior laryngeal nerves, but they were found in histological sections of these nerves and their images were equal to those of the tumor studied. The trajectory of these nerves was investigated in preparations of the Embrioteca de Bellaterra (Prof. Doménech Mateu) and the clumps of paraganglioma cells from which these tumors proceed have been found; we emphasize the concept of neural crest pathology to which they pertain.

Adult↗

PubMedDate not supplied

[Paragangliomas of the chiasma and sellar area].

The article describes five cases of rare cerebral tumors, paragangliomas of the chiasma-sellar area. Their clinical course characteristically begins with the appearance of endocrine disorders manifested by progressive hypophyseal insufficiency. Analysis of the clinical and X-ray signs of paragangliomas of the chiasma-sellar region show these to be similar to adenomas of the hypophysis. A specific feature of paragangliomas of the chiasma-sellar area is their rich vascularization, which is demonstrated by angiography and verified in all cases during operation. The rich vascularization of paragangliomas of the chiasma-sellar area calls for their rapid and complete removal so as to reduce the loss of blood and provide reliable hemostasis.

Adenoma↗

PubMed1988

A comparison of paraganglioma, carcinoid tumor, and small-cell carcinoma of the larynx.

Laryngeal paraganglioma, carcinoid tumor, and small-cell carcinoma are rare. Histologically they are similar to analogous tumors in other locations but may be difficult to identify in small biopsy specimens. We compared the light microscopic, histochemical, immunohistochemical, and electron microscopic features of two laryngeal paragangliomas, one carcinoid tumor, and six small-cell carcinomas. The paraganglioma chief cells stained with Grimelius stain and for chromogranin and neuron-specific enolase. The carcinoid tumor cells stained with Grimelius stain and for chromogranin, serotonin, neuron-specific enolase, and keratin. The small-cell carcinoma cells stained for keratin and neuron-specific enolase. The patients with paragangliomas and carcinoid tumor remain healthy through 20 months of follow-up. Four of the patients with small-cell carcinomas have died. Distinction between these tumors is warranted by differing histologic appearances, staining characteristics, and biologic behavior.

Adult↗

PubMedDate not supplied

Cervical paraganglioma with intranuclear vacuoles in a fine needle aspirate.

In a 56-year-old man who presented with a cervical mass, a fine needle aspiration specimen was suggestive of a papillary thyroid adenocarcinoma because of the presence of prominent intranuclear vacuoles in rare cells. Operation and subsequent examination of the mass removed from the bifurcation of the carotid artery clearly identified a paraganglioma. This case emphasizes the following points: aspirates of cervical paragangliomas are difficult to interpret, especially when scanty; intranuclear vacuoles are found in cells of a variety of cervical lesions, including paragangliomas, and should not in themselves define the diagnosis of papillary thyroid adenocarcinoma; and aspiration of cervical paragangliomas is probably contraindicated (but may be done unwittingly from time to time).

Biopsy, Needle↗