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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↗

PubMed1987

[Pedunculated polypoid gangliocytic paraganglioma of the afferent jejunal loop of a Billroth II stomach].

A mobile pedunculated polypoid tumor was endoscopically removed from the afferent jejunal loop after gastrojejunostomy of a 54-year-old patient with anamnestic evidence of intestinal bleeding. Histologically epithelial carcinoid-like as well as mesenchymal paraganglioma- and ganglioneuroma-like patterns are mixed in varying portions, characteristic for gangliocytic paraganglioma. Immunohistochemically, serotonin, neuron-specific enolase, cytokeratin, vimentin S-100 protein and neurofilament were demonstrable. Gangliocytic paragangliomas are almost exclusively observed in the second portion of the duodenum, especially around the papilla Vateri and only two have previously been reported in the jejunum. The histogenesis of the tumors is unclear, but they may probably be either hamartomas, hyperplastic or neoplastic proliferations of so called endodermal-neuroectodermal complexes. Although gangliocytic paragangliomas contain a carcinoid-like component, they behave in a benign fashion, and metastases or recidives have not been noticed. Tumors with a pedicle may be endoscopically removed without complications.

Diagnosis, Differential↗

PubMed1990

Carotid body paragangliomas. A clinicopathologic and DNA analysis of 13 tumors.

The clinical and pathological features of 13 carotid body paragangliomas from 12 patients were examined and correlated with the DNA ploidy pattern as determined by image analysis. These tumors occurred in 7 women and 5 men aged 19 to 62 years (average, 42 years). All presented with a slowly enlarging, usually asymptomatic mass of 2 weeks' to 25 years' duration. Two patients were related and had a family history of paragangliomas. The tumors ranged from 2 to 6 cm. All contained scattered chief cells with pleomorphic nuclei, two exhibited mitoses, and three showed perineural and three vascular invasion. Follow-up was available in all 12 patients and ranged from 15 months to 28 years (average, 7.3 years). None of the tumors recurred locally, but one did metastasize to a single cervical lymph node that was apparent at the time of diagnosis. Of 13 carotid body paragangliomas examined for DNA, 4 were diploid, 3 diploid-tetraploid, 3 tetraploid, 2 aneuploid, and 1 polyploid. The only malignant tumor was polyploid. From these observations, we conclude that abnormalities in DNA content of carotid body paragangliomas are common and that tumor ploidy cannot be used to assess malignant potential. We also found no apparent relationship among nuclear pleomorphism, mitotic activity, perineural invasion, or vascular invasion and clinical behavior. Perineural and vascular invasion, however, were observed only in tumors with abnormal DNA histograms.

Adult↗

PubMed1992

Paraganglioma of the inferior laryngeal paraganglia. A case report.

Laryngeal paragangliomas are uncommon, usually benign neoplasms. The overwhelming majority of these tumors are supraglottic, arising from the superior laryngeal parasympathetic paraganglia. Few paragangliomas of the infraglottic region have been described. We describe the case of a 35-year-old man with a family history of carotid body tumors who presented with an infraglottic paraganglioma.

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↗

PubMed1986

Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure.

Thirty-one paragangliomas of the cauda equina region were studied (18 men and 13 women, ages 30-71 years [mean, 51 years]). Symptoms (1 day to 15 years in duration; mean, 48 months) included low back pain (87%), sensory/motor deficits (35%), urinary/fecal incontinence (13%), and paraplegia (6%). All patients studied had some myelographic block. Cerebrospinal fluid protein level ranged from 56 to 7000 mg/dl (mean, 1109 mg/dl). Most tumors were limited to the filum terminale, although one also involved the conus medullaris and two clearly arose from a caudal nerve root. All but one were entirely intradural. The tumor was totally excised in 26 cases; these patients remain disease-free. Of three patients whose tumors were excised subtotally, two received radiotherapy; the one non-radiated patient died of tumor-related complications. No autopsy was performed. One partially encapsulated tumor that had been subjected to biopsy and irradiation presented 1 year later with osseous invasion and retroperitoneal extension; 20 years after subtotal excision, this patient is alive but paraplegic. Morphologically, all tumors resembled paraganglioma at other sites. Cytologic atypia and mitotic activity generally were absent to mild. Fourteen (45%) cases showed ganglionic differentiation. All tumors tested were immunoreactive for neuron-specific enolase and neurofilament protein, and most showed somatostatin or serotonin reactivity. S-100 protein immunoreactivity was noted in sustentacular cells and, to a lesser extent, within chief cells and neurons. The authors conclude that paragangliomas are largely benign and encapsulated and respond to simple resection. When surgically feasible, gross total removal should be the goal of surgery. When subtotal resection is necessary or when local invasion leaves a question as to completeness of tumor removal, irradiation seems mandatory although far from guaranteeing prevention of recurrence. Biopsy alone is undesirable.

Adult↗

PubMed1987

Extraadrenal paragangliomas. An immunocytochemical and ultrastructural report.

Although the majority of extraadrenal paragangliomas are nonfunctional, some of these tumors are associated with hormone production and clinical symptoms, notably hypertension. The authors have investigated 22 paragangliomas, five of which were diagnosed as clinically functional in a light microscopic immunocytochemical and electron microscopic study (nine cases). Histologically, all the paragangliomas exhibited similar features, with a "Zellballen" pattern of polygonal cells. All 22 cases were strongly immunoreactive to protein gene product 9.5 (PGP 9.5) antisera and moderately reactive to antineuron-specific enolase (NSE) sera. Ten cases (five functional) were focally immunoreactive to antichromogranin sera. Seven cases (four functional) were immunoreactive to neuropeptide Y and enkephalin antisera, and six (five functional) to tyrosine hydroxylase antisera. The clinically functional tumors expressed at least two of the antigens, enkephalin, neuropeptide Y, or tyrosine hydroxylase, whereas none of the 17 nonfunctional possessed more than one of these. Electron microscopic study revealed cells from all the nine cases studied to contain secretory granules. Granule sizes ranged from 100 to 280 nm and the morphologic examination of the secretory granules generally showed a dense core with a membrane-bound halo of variable size. Secretory granules were observed in the five functional cases and these were larger (220-280 nm) than those seen in the nonfunctional tumor cells (100-180 nm). Also, tumor cells from the functional cases contained numerous dilated mitochondrial profiles.

Adult↗

PubMed1991

An unusual organoid tumor. Alveolar soft part sarcoma or paraganglioma?

The histogenesis of alveolar soft part sarcoma (ASPS) is a subject of continued debate. Although many recent reports suggest a muscle origin, others advocate a neuroendocrine derivation. A tumor in the chest wall of a 16-year-old woman was diagnosed and treated as ASPS. The light microscopic, electron microscopic, and immunohistochemical findings showed features of both ASPS and paraganglioma. In addition, this lesion was positive for antibody to glucagon, a characteristic of neither ASPS nor paraganglioma, although seen in a few gangliocytic paragangliomas. This case demonstrates the need for continued inquiry into the histogenesis of ASPS.

Adolescent↗

PubMed1987

Observations on the function of normal adrenomedullary tissue in patients with phaeochromocytomas and other paragangliomas.

123I-MIBG is a norepinephrine analogue used for imaging phaeochromocytomas and other paragangliomas. In this study 7 normal volunteers and 31 subjects with high or borderline high plasma catecholamine and urinary metanephrine and VMA levels were investigated. In the normals the uptake by the adrenal medulla was in the range of 0.01%-0.21% of the injected dose at 22 h. In 22 subjects there was no evidence for any paraganglioma, with the uptake by the adrenal medulla in the range of 0.01%-0.22% of the injected dose at 22 h. In nine patients there was evidence of a paraganglioma on imaging which was confirmed on surgery. The uptake by the tumours was in the order of 0.001%-0.14% of the injected dose per gram of tumour tissue. In all nine cases the non-tumorous adrenal medulla could be visualized; the uptake in the adrenals was in the range of 0.01%-0.21% of the injected dose. This study shows that in the presence of elevated plasma catecholamines of tumour origin the unaffected adrenal medullary tissue retains the capacity for 123I-MIBG uptake. In the two cases also studied by adrenal venous sampling, catecholamine release was also within the normal range.

3-Iodobenzylguanidine↗

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↗