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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↗

PubMed1983

Malignant catecholamine-secreting carotid body paraganglioma.

The second known case of a malignant catecholamine-secreting (DA)-secreting carotid body paraganglioma is presented. Dopamine synthesis and secretion can be increased in malignant tumors derived from neural crest cells. Whether this is true, in addition, for extra-adrenal paragangliomas is not yet clear. Malignant paragangliomas of the carotid body and larynx, although rare, frequently have been accompanied by increased catecholamine secretion. Malignant catecholamine-secreting carotid body paragangliomas are best treated by composite resection (internal carotid artery and neck dissection), with special attention being given to measures preventing severe hypertension and arrhythmias in the perioperative period.

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↗

PubMed1983

Paraganglioma of the cauda equina.

The authors present two cases of paraganglioma located in the cauda equina. Diagnosis was suspected by light microscopy and confirmed with the identification of characteristic neurosecretory vesicles. The presence of cytoplasmic filaments in both of our cases and of cilia in one case are very unusual findings in paraganglioma and seem to be characteristic of this location. The cauda equina is a extremely rare location for this type of tumor and, as far is known, only ten other cases have been reported. Paraganglioma should be considered as a diagnostic possibility in order not only to make a correct diagnosis, but also to prepare tissues for special stains and biological and electronmicroscopic studies.

Adult↗

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↗

PubMed1984

Combined paraganglioma and glioma of conus medullaris and cauda equina.

A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.

Adult↗

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↗

PubMed1991

[Therapy of a malignant sympathetic paraganglioma of the organ of Zuckerkandl--a case report].

We present a case report on a 35-year-old patient in whom a malignant sympathetic paraganglioma of the organ of Zuckerkandl was the cause of severe hypertension with excessive perspiration at night. Since curative surgery was not possible medical treatment was initiated. Interferon alfa 2b (Intron A, Essex Pharma) and the somatostatin-analogue SMS 201-995 (Sandostatin, Sandoz) had no effect on catecholamine production and progression of the tumor. Treatment with alpha-methyl-para-tyrosin (MPT, [Metyrosin], Demser, MSD) turned out to be an effective and well tolerable therapy in this patient with peritoneal carcinosis. Clinical and hormonal progression of the paraganglioma resumed only after two years of therapy, which constitutes the longest documented period of time of successful MPT treatment. The superior efficacy of MPT in our patient should encourage postoperative medical treatment with MPT in malignant pheochromocytoma or malignant paraganglioma, particularly when the tumor turns out to be resistent to alpha blocking drugs.

Adult↗

PubMed1981

Aorticopulmonary paraganglioma and gastric leiomyoblastoma in a young woman.

The 16th report of a patient with "Carney's triad" is presented. The triad consists of an extra-adrenal paraganglioma, gastric leiomyoblastomas and a pulmonary chondroma. The diagnosis is made by discovery of the presence of at least two of these individually rare tumors. The patient described as 15 year old girl who presented with a pericardial effusion caused by an invasive mediastinal paraganglioma. She was subsequently found to have multiple gastric leiomyoblastomas. The leiomyoblastomas have been resected. The paraganglioma was unresectable, and the patient underwent sequential radiation therapy and chemotherapy without response. Concomitant 5-fluorouracil chemotherapy with radiation resulted in an objective regression of tumor mass.

Adolescent↗

PubMed1984

Catecholamine-secreting paraganglioma of the interatrial septum.

A 29-year-old woman was found to have clinical and biochemical evidence of a functioning paraganglioma (pheochromocytoma) five years after the onset of symptoms. Despite extensive investigation of known potential sites of paragangliomas (including laparatomy), the suspected tumor eluded localization. Adrenergic blocking agents controlled her blood pressure but did not affect the other manifestations of catecholamine excess. Ischemic perforation of the colon and refractory gram-negative sepsis resulted in death. At autopsy, a large paraganglioma was found within the heart; the unique tumor location was an important factor in the fatal clinical course.

Adult↗

PubMed1989

Paragangliomas: assessment of prognosis by histologic, immunohistochemical, and ultrastructural techniques.

To predict clinical outcome, we studied 42 paragangliomas from 37 patients by routine histology, immunohistochemistry, and electron microscopy. A panel of antisera to neuron-specific enolase (NSE), chromogranin, and met-enkephalin was used to identify chief (type I) cells, and S-100 protein and glial fibrillary acid protein (GFAP) sustentacular (type II) cells. The intensity of staining of type I cells and the density of type II cells were assessed semiquantitatively (0 to 4+) in a total of 38 tumors. A total of 23 of 24 low-grade tumors (solitary, multiple, or associated with other neoplasms; 95.8%) contained type II cells immunoreactive with either S-100 protein or GFAP, and all were positive when S-100 protein and GFAP were used in combination. Five of the nine intermediate-grade (recurrent and/or locally aggressive) tumors were identified as glomus jugulare tumors (GJT). Three intermediate-grade GJTs were devoid of GFAP-reactive type II cells and four GJTs were negative for S-100 protein. Type II cells were identified in only one of five high-grade (malignant) paragangliomas and that tumor contained vanishingly rare cells that were weakly S-100 protein positive but GFAP negative. Sustentacular cell density and chief cell staining intensity were both inversely related to tumor grade. The most sensitive chief cell marker was NSE (92.1%), followed by chromogranin (84.2%). The least sensitive (73.0%) and specific marker was met-enkephalin. Combinations of NSE or chromogranin with met-enkephalin identified chief cells in all cases. Electron microscopy identified neurosecretory granule-containing chief cells, but was of less value in delineating sustentacular cells because of their scarcity and the absence of specific features. By comparison, immunohistochemistry was superior in identifying sustentacular cells. The use of an immunohistochemical panel, in addition to routine histology, can confirm the diagnosis of a paraganglioma and can give an indication of the likely prognosis for a patient.

Adolescent↗

PubMed1982

Functioning paraganglioma in the posterior mediastinum.

An unusual case of a functioning paraganglioma originating from the posterior mediastinum is reported. The main indications in the 21-year-old patient were hypertension and an abnormal roentgenogram of the chest. Blood and urine catecholamine assay confirmed the diagnosis of norepinephrine-secreting tumor, which was covered by the parietal pleura and attached to the sympathetic trunk. In the literature we could find reports of 25 patients with paraganglioma arising from the sympathetic trunk in the posterior mediastinum, 8 of whom had some symptoms and only 3 of whom were assayed for catecholamines. We diagnosed the present tumor as functioning aorticosympathetic paraganglioma according to the new classification and terminology suggested by Glenner and Grimley [1].

Adult↗