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PubMed1999

PGL3, a third, not maternally imprinted locus in autosomal dominant paraganglioma.

Paragangliomas (glomus tumors) are slowly growing, mostly benign tumors of the parasympathetic ganglia which most frequently occur in the head and neck region. Between 10% and 50% of cases are familial and follow an autosomal dominant mode of inheritance. The trait is maternally imprinted and exclusively transmitted through the paternal line. To date, two loci have been implicated in this disorder: one at 11q23 (PGL1), the other one at 11q13 (PGL2). We have analyzed a large German family with hereditary paraganglioma, but no evidence of maternal imprinting. By linkage analysis with markers flanking both PGL1 and PGL2, we demonstrate that the trait does not segregate with either of the loci at 11q. Our findings show that a third locus, PGL3, can be involved in hereditary paraganglioma.

Aged↗

PubMed2005

Paraganglioma of the cerebellum: case report and review of the literature.

Intracerebral paragangliomas are tumors with a low incidence. Only 13 cases have been reported in the literature to date. This type of tumor shows characteristic histopathological features but unclear histogenesis. Owing to its low incidence, little knowledge exists concerning the best therapeutic strategy. We report on a 55-year-old male patient with the diagnosis of a paraganglioma of the cerebellum. We compare our patient's case to similar cases reported in the literature, describe the typical course of the disease, and discuss therapeutic options. No relapse of disease was observed in our patient within 2 years of diagnosis. Surgery and adjuvant conventionally fractionated external beam radiotherapy seem to be well tolerated and feasible. A review of patients reported in the literature and a comparison to our patient reveals a good prognosis for intracerebral paragangliomas. The optimal therapeutic strategy with regard to the benefit of adjuvant radiotherapy after total or subtotal resection has yet to be defined.

Adolescent↗

PubMed2005

Cerebellopontine angle paraganglioma - report of a case and review of literature.

Majority of the cerebellopontine angle (CPA) tumors are acoustic neuromas, while bulk of the non-acoustic tumors are formed by meningiomas and epidermoid cysts. Primary paraganglioma is a rare tumor in this location, with only two such cases having been reported in the literature, till date. Recently, a case has been described wherein a paraganglioma was apparently arising as a primary lesion in the cerebellar hemisphere. We report another case of an intracranial paraganglioma of the CPA in a 40-year-old female, which did not have any vascular attachment but had focal cerebellar extension.

Adult↗

PubMed2007

Malignant non-functional paraganglioma of the bladder presenting with azotemia.

Paragangliomas of the urinary bladder are rare tumors representing less than 1% of bladder tumors and are usually benign. Malignant paragangliomas are uncommon and are defined by their clinical behavior rather than the histologic features. We describe a patient with recurrent nonfunctioning paraganglioma of bladder presenting with hematuria and obstructive uropathy due to involvement of ureteroileal anastomoses. Treatment consisted of excision of recurrent lesion involving both lower ureters with the revision of ureteroileal anastomoses. Histology confirmed the presence of nests of tumor cells with abundant eosinophilic cytoplasm. Immunohistochemically the tumor cells were strongly positive for chromogranin A.

Adult↗

PubMed2004

Paraganglioma of the posterior mediastinum diagnosed by immunohistochemical staining.

Paraganglioma of the mediastinum are rare neoplasms. To date, no definitive morphologic criteria exist that correlate with the clinical outcome of these tumors. We have encountered a case of paraganglioma in which biological behavior was assessed by immunohistochemical staining to determine whether supplementary postoperative treatment was needed. A 28-year-old man came to our hospital because of an abnormal shadow on a radiogram of the chest. He had no symptoms. Hematological findings were unremarkable. Diagnostic imaging suggested a neurogenic tumor. Surgical resection was performed in September, 2002. A typical nesting pattern (Zellballen) and positivity for chromogranin on immunohistochemical staining were evident, indicating neuroendocrine characteristics, and paraganglioma was diagnosed. Tissue specimens indicated an MIB-1-labeling index of 1.3% on MIB-1 staining, and a relatively well maintained distribution of S-100 protein-positive sustentacular cells, which were suggestive of a benign tumor. The patient did not receive any supplementary therapy postoperatively but was given careful follow-ups.

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↗

PubMed1994

Anterior and middle mediastinum paraganglioma: complete resection is the treatment of choice.

Paraganglioma of the mediastinum is described to be an indolent and slow-growing tumor. After a patient presented to our center, we reviewed the world literature to evaluate the prognosis of this tumor. This review showed that paragangliomas are locally invasive and have a high local recurrence rate (44/79 or 55.7%) with a true metastatic capacity (21/79 or 26.6%). The overall survival is 62.0% (49/79), but only 36.7% (29/79) of patients could be considered as free of disease, with survival time of 98.2 +/- 11.7 months (mean +/- standard error). The survival with a complete resection is 84.6% (125.7 +/- 18.7 months) versus 50.0% (71.5 +/- 13.8 months) for patients with a biopsy or a partial excision and adjuvant treatment (p < 0.01). We acknowledge the limitation of this retrospective study, but a prospective trial is not possible because of the rarity of the tumor. We want to emphasize that paraganglioma of the anterior and middle mediastinum is an aggressive tumor, and complete surgical resection, using cardiopulmonary bypass if necessary, is highly recommended.

Aged↗

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↗

PubMed1996

Paraganglioma of the thoracic spinal cord.

Paraganglioma arising in the thoracic extradural space is an uncommon tumor, with only four cases previously reported. The authors review the clinical and pathological features of thoracic paraganglioma and compare them to the more common paraganglioma of the cauda equina.

Adult↗

PubMed2004

[Paragangliomas: clinical and secretory profile. Result of 39 cases].

THIS RETROSPECTIVE STUDY AIMS: To define a clinical and secretory profile of paragangliomas extra-adrenal chromaffin tumors. METHODS: From 1971 throughout 2002, 39 paragangliomas have been observed in 38 patients (22 male, 16 female, average age 41,2 years). RESULTS: Four were located above the diaphragm, 35 were sub-phrenic (6 of the organ of Zuckerkandl), 32 secreted catecholamines, 23 were hypertensive (with only one without hypersecretion of catecholamines). Among 29 (131)I-metaiodobenzylguanidine scans (MIBG) reviewed, 20 tumors took up the radiopharmaceutical. The treatment was surgical in 35 cases with addition of external radiotherapy and MIBG in one case each; two patients died before any treatment. Two patients with persistent disease after surgery were successfully treated by surgery or MIBG. Histologically, 20 were malignant and 17 were seemingly benign. All exclusive dopamine secreting paragangliomas were malignant. Six patients relapsed two of which for a tumor initially classified as benign. The treatment of recurrences was surgical, by MIBG or by external radiotherapy. Nine patients had a family history of chromaffin tumor(s). The genetic survey made in five of these nine patients was positive in all cases.

3-Iodobenzylguanidine↗

PubMed2005

Paraganglioma with ganglioneuromatous component located in the posterior mediastinum.

A 4-cm paravertebral mediastinal tumor was resected in a 70-year-old male patient treated for hypertension. The tumor displayed both paraganglioma and ganglioneuroma areas that were in equal proportion and often merged one into the other. Paraganglioma areas contained synaptophysin and chromogranin-positive chief cells and PS100-positive sustentacular cells. Ganglioneuroma areas contained neurofilament-positive mature ganglion cells and PS100-positive Schwann cells. Such pheochromocytoma-ganglioneuroma has not been previously reported in the mediastinum and appears as the adrenal and aorticosympathetic counterpart of gangliocytic paraganglioma described in other anatomic sites.

Aged↗

PubMed2006

Long-term results of irradiation for paraganglioma.

PURPOSE: The management of paragangliomas is controversial. Observation, surgery, external-beam radiotherapy (EBRT), and stereotactic radiosurgery (SRS) may, alone or in combination, be appropriate, depending on the size and extent of the tumor, previous treatment, and patient age, general health, and neurologic condition. Few data exist regarding long-term tumor control and late effects after EBRT or SRS. METHODS AND MATERIALS: We performed a retrospective review of all patients treated with EBRT or SRS for paraganglioma at our institution between 1967 and 1994. The endpoints of the study were tumor control and late complications. RESULTS: The 33 patients in this study had a median follow-up of 13 years (range, 4 months to 36 years). The 10-year tumor control rate was 92% (95% confidence interval, 75-98%). At the last follow-up visit, no patient had developed a radiation-induced malignancy. CONCLUSION: External-beam RT and SRS are safe and effective for enlarging and/or symptomatic paragangliomas. The risk of developing a delayed radiation-induced malignancy after EBRT or SRS is low. This risk must be weighed against the significant immediate and permanent risk of cranial nerve deficits if the tumor is untreated or is surgically resected. This risk must also be weighed against the immediate but low risk of surgical mortality.

Adolescent↗

PubMed2004

Posterior mediastinal paraganglioma with bilateral adrenal pheochromocytoma.

Paraganglioma and adrenal pheochromocytoma are tumors of common origin arising from chromaffin cells. However, it is extremely rare to find mediastinal paraganglioma simultaneously with bilateral adrenal pheochromocytoma. We report a 53-year-old man who was diagnosed with posterior mediastinal paraganglioma and bilateral adrenal pheochromocytoma and who underwent successful excision of the posterior mediastinal mass and bilateral total adrenalectomy.

Adrenal Gland Neoplasms↗

PubMed2005

[Tympanic paragangliomas: analysis of a nine cases].

OBJECTIVE: The purpose of this work was to evaluate the diagnostic strategy and outcome of patients treated for tympanic paraganglioma. PATIENTS AND METHODS: Nine patients presented with tympanic paraganglioma were treated in our unit from 1996 to 2003. There were eight women and one man, mean age 65 years. Surgery was performed in all nine patients. This retrospective analysis focused on revealing signs, diagnostic tools, surgical procedure for tumor resection, and short-, mid-, and long-term functional outcome. RESULTS: The surgical procedure was well tolerated by all patients. There were no cases of postoperative mastoiditis. None of the patients experienced postoperative dizziness. Total tumor removal was achieved in seven patients. In one patient, incomplete tumor resection was decided in order to preserve facial motion. All patients but one were relieved from their tinnitus in the early postoperative period. In four patients, preoperative hearing levels were worsened by the surgical procedure, essentially through an alteration of preoperative conductive hearing loss. CONCLUSION: MRI is highly contributive to the diagnosis of tympanic paraganglioma, which should be evoked in patients with pulsatile tinnitus. Diagnosis should be established as early as possible to lower the postoperative morbidity. While surgery can provide cure and relieve tinnitus, there is a high risk of auditory deterioration.

Adult↗

PubMed2005

[Head and neck paragangliomas in Senegal. About 8 cases].

UNLABELLED: Paragangliomas are uncommon neoplasms with rare occurrence in the head and neck area. OBJECTIVE: The purpose of this study was to report the experience of management of these rare tumors by a team of Ear, Nose, and Throat (ENT) surgeons working in the context of a sub-Saharan country. MATERIAL AND METHODS: We conducted a retrospective study in the Ear, Nose and throat Department of a Dakar university hospital. The study concerned 8 patients with a highly vascular tumor located in the neck and temporal bone. The preoperative investigations were computed tomography scanning (CT scan) using contrast injection in 88% and Doppler ultrasonography for the patients with a neck mass. All the patients underwent surgery except one. In 2 cases, the management with a team of vascular surgeons was necessary. Histological examination of the tumor was realized in all cases. RESULTS: The tumor was located in the neck in 5 cases (62,5%) with a carotid body tumor in three patients (37,5%) and vagal location in two (25%). The temporal bone was involved in three patients with a tympano-jugular location (37,5%). All the neck masses were operated on via a unique cervical approach. The tympano-jugular tumors were treated by radical mastoidectomy in two patients. In the third patient with a tympano-jugular tumor, with important involvement of the temporal bone, only the neck extension was operated. The histological diagnosis of paraganglioma was confirmed in all patients. Radiation therapy was delivered in two patients (25%), to complete surgery in the event of extensive temporal bone tumor and exclusive in one case of an inoperable vagal tumor. The outcomes, marked early death in one patient (14%), were good in the short and mid term for the others patients. CONCLUSION: With the improvement of technological infrastructures, particularly with developing modern imaging, we have better knowledge of paragangliomas in the head and neck area, in our experience in a developing country. However, therapeutic approaches are still limited by modest humans and material resources.

Adolescent↗

PubMed2001

Preoperative embolization in the management of a mediastinal paraganglioma.

Parangliomas are rare and highly vascular tumors of neuroendocrine cell origin which are treated by complete surgical resection. Preoperative embolization to reduce perioperative bleeding complications, although described in paragangliomas of the neck and carotid body, has never before been described in the case of a mediastinal paraganglioma. The following is a presentation of such a case of mediastinal paraganglioma, in which embolization was used successfully before surgical resection.

Adult↗

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↗