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PubMed1985

Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin.

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).

Adult↗

PubMed1982

Current management of paragangliomas.

Since 1948, 33 paragangliomas in 32 patients were seen and studied at Charity Hospital. Tumors of the head and neck comprised the bulk of recorded instances, with only five paragangliomas being noted below the diaphragm. The most frequent symptoms correlated closely with tumor location and were related to local mass effect. Angiography remains an important diagnostic study for tumors of the head and neck with computed tomography scanning becoming increasingly important in large paragangliomas of the jugular region. Paragangliomas of the retroperitoneal space can be successfully located by a computed tomography scan, although a new radionuclide scan may prove to be more specific in the future. Tumors of the urinary bladder and retroperitoneal space are commonly endocrine functional and have a greater metastatic potential than do paragangliomas in other locations. Over-all, total excision is possible with complete recovery in most instances. Prolonged follow-up study is recommended, however, as local recurrences and distant metastases may present years after the initial diagnosis.

Adult↗

PubMed1993

[Vagal paraganglioma].

Vagal paragangliomas are rare tumors of neural crest origin. This neoplasm is generally located at various points along the peripheral distribution of the vagus nerve, in the region of the jugular and nodose ganglia, just beneath the perineurium of the nerve, but may arise anywhere along the course of the vagus nerve. Histologically, vagal paragangliomas are similar to carotid body tumors, and occasionally can undergo malignant transformation and metastasize (10-19%) or infiltrate the base of the skull. Selective arteriography can distinguish vagal paraganglioma from carotid body tumor, because the first lies above carotid bifurcation without widening of the vessels. CT scanning and MR are useful for preoperative evaluation of the jugular foramen for neoplastic involvement. Surgical resection is the treatment of choice, but usually it isn't possible to exercise these lesions without sacrifice of the vagus nerve. A careful follow-up examination for the development of metastases and multicentric paragangliomas is necessary. Two cases of vagal paragangliomas one of them with cervical node metastases, are reported. Diagnostic and surgical features of these rare neoplasms are outlined.

Adult↗

PubMed1997

[Malignant paraganglioma of the posterior mediastinum with 27 years follow-up].

INTRODUCTION: A costovertebral paraganglioma with a long history of recurrences and metastases is reported. MATERIAL AND METHODS: The lesion is documented on histological ground and with immunocytochemistry and electron microscopy. RESULTS: The patient is a 42 year old man which presented with a 5 cm axillary mass and enlarged mediastinal lymph nodes. The former lesion has been interpreted as a metastasis of paraganglioma. The patient underwent surgery because of a costovertebral paraganglioma at the age of 19 and showed locoregional metastases and a recurrence over a period of 23 years. Twenty-seven years from removal of the primitive tumor, the patient is alive and well. All the neoplasms showed identical histological features as they were composed of neoplastic elements arranged in nests surrounded by dendritic cells. Neoplastic cells immunoreacted with anti-chromogranin antiserum whereas sustentacolar cells were positive with S-100 antiserum. DISCUSSION: The differential diagnosis between a multicentric paraganglioma a metastasis has been particularly taken into consideration. In addition, axillary tumor was distinguished from a metastatic renal cell carcinoma and melanoma. The literature regarding costovertebral paraganglioma has been also revised.

Adult↗

PubMed1999

Familial carotid body paragangliomas and sensorineural hearing-loss: a new syndrome.

BACKGROUND: Carotid body paragangliomas are rare tumors that are sometimes familial, the transmission of which is thought to be by genomic imprinting. We have treated a family who exhibited co-inheritance of carotid body paraganglioma and sensorineural hearing-loss, a relationship that has not been previously reported. METHODS: We studied a large Australian family who exhibited familial carotid body paragangliomas, many of whose members also suffered tinnitus or hearing-loss. This relationship was examined by reviewing the medical records of family members with confirmed tumors, carrying out neck ultrasonic scanning or computed tomography on their relatives to look for previously unrecognized tumors, and arranging audiometric testing. This information was used to characterize the type of hearing-loss present in this family and to construct a pedigree for the two traits. RESULTS: The hearing-loss observed in this family was sensorineural in character. Of 15 family members studied over four generations, eight were confirmed positive for both carotid body paraganglioma and sensorineural hearing-loss, two for the tumor only (one of whom did not have his hearing assessed) and one for hearing-loss alone. Four family members were negative for both traits. CONCLUSIONS: The pedigree provides evidence of a previously undescribed association between familial carotid body paraganglioma and sensorineural hearing-loss, a combination that appears to be co-inherited in this family.

Adult↗

PubMed1991

Familial multicentric paragangliomas in a child.

A 12-year-old girl presented with bilateral carotid-body paragangliomas and a unilateral jugular paraganglioma. The tumours were surgically removed. This is a rare combination of tumours in any patient and previously unreported in a child of this age. Her father died of a cerebellar astrocytoma and her mother underwent surgical removal of a large mediastinal paraganglioma. The association of astrocytoma with familial paragangliomas has never been documented. The literature on the epidemiology and inheritance pattern of familial paragangliomas is reviewed. The need for thorough pre-operative evaluation of the patient and close follow-up of family members is stressed.

Carotid Body Tumor↗

PubMed1998

[Jugulotympanic paraganglioma: therapy concepts under development].

BACKGROUND: The operative treatment and radiation therapy of jugulotympanic paragangliomas (JTP) are still a matter of controversial discussion. In spite of various improvements during the last 50 years, selecting the appropriate treatment modality (surgery, radiation, or observation) is still a challenge. PATIENTS: During a 16-year period, 44 patients with 45 JTP (10 at level A/B and 35 at level C/D according to Fisch) were seen at the ENT-department in Fulda. Forty-one cases were treated surgically. RESULTS: Complete resection was possible for level A/B in 100% of the patients (n = 10). Residual tumor was demonstrated for level C in 23% of the patients (5/22) and for level D in 40% (4/10) with a median follow-up time of 69 months. In two cases residual tumor was treated by radiation. Six patients with residual paraganglioma tissue were maintained under observation without any evidence of tumor progression (median follow-up time 39 months). We report one death after the attempt to resect a large residual paraganglioma that had already caused brain stem compression. A sufficient duraplasty could not be achieved following radiation therapy. CONCLUSIONS: Complete tumor resection of jugulotympanic paragangliomas of levels A and B is often possible without injury to the cranial nerves. Extensive tumors present difficulties in complete tumor resection and increase the risk of cranial nerve injuries. Advanced paragangliomas therefore require an individualized therapeutic regime including surgery, radiation therapy, and observation of tumor growth.

Adult↗

PubMed1997

Image cytometric nuclear DNA quantitation of paragangliomas in tissue sections. Prognostic significance.

OBJECTIVE: Image cytometric quantitation of nuclear DNA of paragangliomas may provide prognostic information that cannot be obtained from histopathologic study. Flow cytometry has demonstrated DNA aneuploid tumors to have a higher risk of progression than diploid neoplasms. STUDY DESIGN: DNA ploidy of 56 paragangliomas was assessed by image cytometry of 5-micron, Feulgen-stained, formalin-fixed, paraffin-embedded tissue sections. RESULTS: Thirty-three (59%) paragangliomas were diploid and 23 (41%) aneuploid. Of the 30 adrenal pheochromocytomas, 15 (50%) were diploid. Thirteen (93%) of the 14 carotid body tumors were diploid. Five of seven (71%) glomus jugulare tumors and two of five (40%) extraadrenal paragangliomas were aneuploid. During a mean follow-up of 57 months (range, 1 month to 36 years) of 44 patients with 47 paragangliomas, 33 (75%) were alive and without disease; 7 (16%), including 1 glomus jugulare, 2 carotid body and 4 pheochromocytoma patients, developed recurrences/metastases. By multivariate analysis, image cytometric DNA ploidy was predictive of disease-free survival for adrenal pheochromocytomas. No significant differences in overall survival, disease-free survival or recurrence/metastasis rate were noted between other diploid and aneuploid tumors. CONCLUSION: Aneuploidy suggests a risk of early recurrence for adrenal pheochromocytomas.

Adrenal Gland Neoplasms↗

PubMed1988

Decreased expression of neuropeptides in malignant paragangliomas: an immunohistochemical study.

Paraffin-embedded sections of 99 human adrenal and extraadrenal paragangliomas were analyzed by the indirect immunoperoxidase technique for the presence of neuron-specific enolase (NSE) and 10 neuropeptides. Each showed diffuse staining for NSE. Most tumors were positive for [Leu5]-enkephalin (76 per cent), [Met5]-enkephalin (75 per cent), somatostatin (67 per cent), and pancreatic polypeptide (51 per cent), followed by vasoactive intestinal polypeptide (VIP) (43 per cent), substance P (31 per cent), ACTH (28 per cent), calcitonin (23 per cent), bombesin (15 per cent), and neurotensin (12 per cent). The neuropeptides paralleled to a large extent those normally found in the sympathetic nervous system. Clinically malignant paragangliomas (n = 25) with proven regional or distant metastases expressed considerably fewer neuropeptides, although the spectrum of those seen remained similar. Malignant paragangliomas contained an average of two neuropeptides per tumor, in contrast to five for the benign tumors (P less than 0.05). Logistic regression analysis of staining results revealed that the paucity of enkephalins, somatostatin, pancreatic polypeptide, and VIP along with the patient's sex was predictive of clinical malignancy. Our results show a definite relationship between expression of neuropeptides and the biologic behavior of these paragangliomas.

Adolescent↗

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↗

PubMed1996

Preoperative embolization of paragangliomas and angiofibromas. Measurement of intratumoral arteriovenous shunts.

OBJECTIVE: To determine if technetium Tc 99m-labeled macroaggregated albumin selective angioscintigraphy enables estimation of microparticle-arteriovenous shunt in branchial paragangliomas and nasopharyngeal angiofibromas. DESIGN: Technetium Tc 99m-labeled macroaggregated albumin particles with calibrated diameters between 25 and 50 microns were administered during selective catheterization of the feeding arteries in 4 branchial paragangliomas (glomus tumors) and 4 nasopharyngeal angiofibromas. Preliminary measures of the activity of the tumor region and of the lungs were obtained in the angiography suite using a hand-held detector. Without changing the position of the microcatheter, definitive embolization was performed. After endovascular treatment, scintigraphy was performed and the pulmonary shunt index was calculated with the use of a gamma camera and a dedicated computer program. RESULTS: The pulmonary shunt index varied between 0% and 36% for branchial paragangliomas (glomus tumors) and between 82% and 95% for angiofibromas. CONCLUSIONS: Our results provide new evidence that the incidence of microparticle-arteriovenous shunt in nasopharyngeal angiofibromas is much higher than in paragangliomas.

Adolescent↗

PubMed2000

Paraganglioma of the urinary bladder: can biologic potential be predicted?

BACKGROUND: Paraganglioma of the urinary bladder is rarely encountered and its biologic behavior is uncertain. The authors sought to determine the prognostic factors that would predict patient outcome. METHODS: The Mayo Clinic experience over 53 years with paraganglioma of the bladder was reviewed. All histologic slides from 16 patients were reviewed by the authors. Eight cases were examined immunohistochemically with cytokeratin (AE1/3, cytokeratin 7, and cytokeratin 20), vimentin, S-100 protein, neuroendocrine markers (chromogranin, synaptophysin, and neuron specific enolase), p53 protein, and MIB-1. DNA ploidy was determined by digital image analysis in formalin fixed, paraffin embedded tissue. The mean follow-up was 6.3 years (range, 0.4-16.4 years). RESULTS: Paraganglioma usually occurred in young adult women (mean age, 45 years; range, 16-74 years). The male-to-female ratio was 1 to 3. The common symptoms and signs were hypertension and hematuria. The tumors were usually located intramurally in the lateral and posterior wall of the bladder and were multifocal in 3 cases (18%). Seven patients were treated by transurethral resection, eight by partial cystectomy, and one by radical cystectomy. T classification was T1 (1 patient), T2 (9 patients), T3 (2 patients), and T4b (4 patients). At the time of diagnosis, one patient had distant metastasis and one had regional lymph node metastasis. One patient developed metastasis 1 year after diagnosis and died of the disease 1.5 years later. None of the patients with T1 or T2 tumors had recurrence or tumor progression. All tumors were aneuploid. The mean MIB-1 labeling index was 1.5% (range, 0.03-7.0%). The tumor cells displayed immunoreactivity for S-100 protein and neuroendocrine markers and were negative for p53 (except 1 case) and cytokeratin. CONCLUSIONS: Paraganglioma of the urinary bladder occurs mostly in young adult women. Patients with tumor of advanced classification (>/=T3) are at risk of recurrence, metastasis, and dying of the disease, whereas patients in this study with T1 or T2 disease had favorable outcomes after complete tumor resection.

Adolescent↗

PubMed1997

Subglottic paraganglioma.

Paragangliomas are uncommon neuroendocrine tumors. In the head and neck region they are most commonly associated with the carotid body, vagus nerve, jugulotympanic paraganglia, and occasionally the superior and inferior laryngeal paraganglia. Laryngeal paragangliomas and subglottic paragangliomas are rare. There have been nine reported cases in the English literature of subglottic paragangliomas. We present a case of this unusual lesion and discuss histologic characteristics and surgical treatment.

Female↗

PubMed2000

Paragangliomas-- a decade of clinical experience.

BACKGROUND AND OBJECTIVES: This study is a review of 14 patients with paragangliomas between 1986 and 1996. The purpose was to determine the sites of origin, clinical manifestations and analyze the benefits of different treatment modalities. METHODS: There were 20 tumors in 14 patients. Three (21.0%) of the patients had familial history. There were 7 (50%) females and 7 (50%) males. Anatomically 14 (70%) tumors were in head and neck, 5 (25%) were in the retroperitoneum, one (5%) was in the heart. Of the head and neck tumors 9 (64.25%) were in the carotid body, 3 (21.42%) were found in the vagus, and 2(14.33%) were found in the middle ear. The tumor found in the heart was in the atrial septum. The clinical behavior of paragangliomas is determined by cellular characteristics, secreting capabilities and tumor location. The symptoms and signs depend on the site of origin and the stage at which it presents. The clinically functioning tumors were 3 (17%) in our experience and they typically present with uncontrolled hypertension. The carotid body and mediastinal tumors usually manifested as asymptomatic masses. The intravagal tumors presented with paresis of the nerve. Malignancy rarely occurs and is defined by the existence of metastasis rather than by histology. In our series 2 (10%) of the patients presented with metastasis to lymph nodes, and the vertebrae. The diagnoses in our patients were established by CT and MRI scanning. Angiography was performed in 5 patients with carotid body tumor, two of whom underwent therapeutic embolization to reduce the tumor size. The mainstay of treatment was surgical removal, though radiation has been advocated for patients who cannot undergo surgery. RESULTS: All patients underwent successful surgical resection of the tumor after appropriate preoperative preparation. Late mortality occurred in two (12.5%) patients at 3 and 5 years from unrelated etiology. Four (25%) patients were lost to follow-up. Three (18.7%) patients developed new primaries, two of them at two years and one after 8 years. One (6%) patient developed recurrent paraganglioma after remaining disease free for 20 years. CONCLUSION: In conclusion, paragangliomas are rare with multicentricity being more common in patients with familial history. The malignant potential of the tumor is determined by metastasis as there are no characteristic cellular change. Aggressive surgery is mandatory to obtain disease free survival with low morbidity and mortality. Recurrences can also be successfully operated with low morbidity.

Adolescent↗

PubMed1982

Cyanotic congenital heart disease with malignant paraganglioma.

A malignant paraganglioma of the subclavian (supra-aortic) area and organ of Zuckerkandl with metastases to the liver and pancreas was discovered at autopsy in a 22-year-old man with known transposition of the great arteries. Light microscopy showed the typical "Zellballen" pattern and Grimelius stain showed intracytoplasmic argyrophilic granules, which appeared ultrastructurally as electron dense granules. Review of the literature disclosed 59 previously reported cases of hypoxia associated with endocrine tumors. The case presented is believed to be the first example of a subclavian paraganglioma associated with hypoxemia. One previous case of a paraganglioma arising in the organ of Zuckerkandl that occurred in a hypoxic state has been reported. The possibility of cyanotic congenital heart disease with chronic long-standing hypoxia, predisposing to the development of paraganglioma with malignant transformation is presented.

Adult↗

PubMed1984

Paraganglioma of the duodenum in association with congenital neurofibromatosis. Possible relationship.

A case of a duodenal paraganglioma in a patient with von Recklinghausen's disease is presented, along with a review of the common clinical features of the 26 cases of duodenal paraganglioma and related neurogenic tumors reported in the literature. There was no reported evidence of recurrence or metastasis. Local excision was the most commonly employed method of treatment. It is proposed that duodenal paraganglioma with its admixed components and variable histologic features may represent hamartomatous hyperplasia of the paraganglia and the supporting neuroid tissue in this area. This would explain the benign behavior of these tumors as compared with carcinoids. Its association, in this case, with neurofibromatosis supports the neural crest origin of duodenal paragangliomas.

Cytoplasmic Granules↗

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↗

PubMed2002

National Cancer Data Base report on malignant paragangliomas of the head and neck.

BACKGROUND: Malignant paragangliomas of the head and neck are rare, with previous reports limited to nine or fewer patients. The current review included 59 cases extracted from the National Cancer Data Base that were diagnosed between 1985-1996. METHODS: The primary criterion for inclusion in the current study was verified metastatic spread from a paraganglioma of the head and neck. Patterns of presentation and treatment as well as clinically relevant associations were demonstrated in contingency tables. Relative survival was used for analysis of outcome. RESULTS: The average patient age at presentation was 44 years, and gender distribution was equivalent. Metastases were confined to regional lymph nodes in the majority of cases (68.6%), with carotid body tumors found to have an even higher rate of regional confinement (93.8%). Surgery was the most common treatment (76.3%). The use of adjuvant irradiation for regionally confined disease increased across time, from 27% (1985-1990) to 46% (1991-1996). The 5-year relative survival rate was 59.5% (76.8% for regionally confined carcinoma and 11.8% for distant metastasis). Among patients who were followed until death, those treated with adjuvant irradiation had a longer median survival (45 months) compared with those patients who were treated with surgery alone (12 months). CONCLUSIONS: Malignant paraganglioma represents metastatic spread of a tumor type that, when restricted to the site of origin, is considered benign. Metastases from malignant paragangliomas of the head and neck usually are regionally confined. The primary management of a recognized malignancy should be directed toward complete surgical removal of the primary tumor and regional lymph nodes. Postoperative irradiation may be beneficial in slowing the progression of residual disease.

Adult↗