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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↗

PubMed2000

Expression analysis of RET and the GDNF/GFRalpha-1 and NTN/GFRalpha-2 ligand complexes in pheochromocytomas and paragangliomas.

Pheochromocytoma and its extra-adrenal counterpart paraganglioma are rare catecholamine producing tumors which usually occur sporadically but may also be a part of neuroendocrine tumor syndromes such as multiple endocrine neoplasia type 2A (MEN 2A). Activating mutations of the RET proto-oncogene which is the underlying cause of MEN 2A, is also seen in approximately 10% of sporadic pheochromocytomas. Glial cell line derived neurotrophic factor (GDNF) and neurturin (NTN) have been shown to function as independent ligands to RET, binding in a complex with the membrane-bound receptors GFRalpha-1 and GFRalpha-2 respectively. Here we have investigated the mRNA expression of RET and its ligand complexes, GDNF/GFRalpha-1 and NTN/GFRalpha-2, in a panel of pheochromocytomas and paragangliomas using mRNA in situ hybridization. RET expression was evident in normal adrenal medulla, and in 13/15 pheochromocytomas, including 5/5 MEN 2A associated tumors, but only in 1/10 paragangliomas. The frequent expression of RET in the pheochromocytomas suggest that this gene might be involved in the tumorigenesis. However, no expression of GDNF/GFRalpha-1 or NTN/GFRalpha-2 could be detected in any of the 25 tumors analyzed, suggesting that these ligand complexes are not important in the development of pheochromocytoma or paraganglioma.

Adolescent↗

PubMed2000

Paraganglioma of the tongue.

Paragangliomas are neuroendocrine tumors arising from extra-adrenal autonomic ganglia. We present what is to our knowledge the first immunohistochemically documented case of a paraganglioma of the tongue, a 2.5-cm benign tumor growing in the position of the foramen cecum. The patient was an elderly woman who presented with throat irritation. The histologic profile and ultrastructural appearance of this lesion were classic for paraganglioma, and cells stained strongly for neurosecretory granules by immunohistochemistry. Carcinoid, ectopic thyroid neoplasm, and other tumors that mimic paraganglioma were ruled out on immunohistochemical grounds. To our knowledge, paraganglia have not been previously documented in the tongue; this tumor may have arisen from a branch of cranial nerve VII or IX, or from an embryologic remnant of the thyroid or thyroid capsule.

Aged↗

PubMed1999

Management of incidentally discovered cervical paragangliomas: report of two cases and review of current issues.

Paragangliomas of the head and neck are uncommon neoplasms arising from the extra-adrenal paraganglia and include carotid body and glomus vagale tumors. These lesions may be discovered incidentally by imaging studies performed to evaluate carotid atherosclerotic occlusive disease. Incidental paragangliomas of the head and neck may be smaller than those discovered due to symptoms. Although surgical resection remains the definitive treatment for head and neck paragangliomas, important issues of management arise when such lesions are discovered. Two recent cases are reported. Epidemiology, pathophysiology, diagnostic evaluation, and issues of management of head and neck paragangliomas are discussed.

Aged↗

PubMed1999

Paraganglioma of the cauda equina. Case report with 33-month recurrence free follow-up and review of the literature.

Paraganglioma of the cauda equina is an unusual tumor and do not have the secretory properties of the same tumors arising outside the nervous system. In none of the few cases reported in literature a preoperative diagnosis was possible, and the surgical findings raised questions in the differential diagnosis with ependymomas. A rare case of paraganglioma of the cauda equina studied both pre- and postoperatively by MRI, and treated with subtotal excision combined with radiotherapy is described. Results and recurrence rates of the cases reported in literature are reviewed. Though MRI imaging has proven to be more sensitive than other radiological procedures, we stress the difficulties of preoperative diagnosis of paragangliomas in this site. The correct diagnosis of the paraganglioma of the cauda equina still relies on immunochemistry and electron microscopy. Total excision is often very difficult owing the tendency of these neoplasms to infiltrate cauda's roots. A 33-month recurrence free follow-up of our patient confirms that successful treatment is achieved by subtotal resection combined with radiotherapy.

Adult↗

PubMed2001

[Recurrence and pulmonary metastasis of extradural paraganglioma in thoracic vertebral canal: report of a case].

A 70-year-old female was operated on for extradural spinal cord tumor in 1982. Microscopic examination revealed the tumor as paraganglioma. Tumor recurred at paravertebral twice in 1985 and 1989, and they were also resected. In 1995, her chest X-ray film showed round tumor in the right upper field. Exploratory open lung biopsy was performed in 1996, and right upper lobectomy was performed according to for malignant lung tumor because intra-operative microscopic findings showed carcinoid or lung metastasis of paraganglioma. Chest wall tumor at paravertebral was resected at the same time. Postoperative microscopic examination revealed the tumors were same as operated paraganglioma. The 2nd thoracotomy was done in 1999, and two chest wall tumors and a pulmonary nodule in right S8 segment were resected. They were recurrence and pulmonary metastasis of paraganglioma. Now 18 years after initial operation, she is out of hospital in tumor free.

Aged↗

PubMed2003

Malignant nasal paraganglioma: a case report and review of the literature.

A rare case of malignant nasal paraganglioma is described. A 30 year old female patient presented with a one year history of bilateralnasal obstruction, nasal deformity and recurrent epistaxis. CT scan demonstrated an enhancing mass occupying both nasal cavities, right maxillary antrum and anterior ethmoid sinus. Histopathologic diagnosis was malignant paraganglioma. A total maxillectomy with excision of growth was performed. Post-operative radiotherapy and chemotherapy was given but patient expired before the completion of therapy. Nose being a rare site for paragangliomas, these lesions present a diagnostic challenge to histopathologists and clinicians alike. A review of the four previously described malignant nasal paragangliomas is also presented.

Adult↗

PubMed2004

Head and neck paragangliomas: improved tumor detection using contrast-enhanced 3D time-of-flight MR angiography as compared with fat-suppressed MR imaging techniques.

BACKGROUND AND PURPOSE: MR imaging techniques have proved their efficacy in imaging the head and neck region. In this study, we compared T1-weighted, dual T2-weighted, and fat-suppressed MR imaging and unenhanced and contrast-enhanced 3D time-of-flight MR angiography sequences for detection of head and neck paragangliomas. METHODS: Thirty-one patients with 70 paragangliomas were examined. Four combinations of MR images were reviewed by two neuroradiologists: T1-weighted and dual T2-weighted fast spin-echo images, T1- and T2-weighted fat-suppressed fast spin-echo images, T1-weighted and contrast-enhanced T1-weighted fat-suppressed spin-echo images, and unenhanced and contrast-enhanced 3D time-of-flight MR angiograms. The randomized examinations were independently evaluated for image quality, presence of tumor, tumor size, and intratumoral flow signal intensity. The standard of reference for presence of tumor was digital subtraction angiography. Data were analyzed by using the logistic regression method. RESULTS: Mean sensitivity, specificity, and negative predictive values, respectively, were assessed by the two observers to be as follows: for dual T2-weighted fast spin-echo, 74%/99%/86%; for T2-weighted fat-suppressed fast spin-echo, 70%/100%/85%; for contrast-enhanced T1-weighted fat-suppressed spin-echo, 73%/100%/86%; and for unenhanced and contrast-enhanced 3D time-of-flight MR angiography, 89%/99%/93%. Sensitivity was significantly better for unenhanced and contrast-enhanced 3D time-of-flight MR angiography (P =.000028). More intratumoral flow signal intensity was depicted with unenhanced and contrast-enhanced 3D time-of-flight MR angiography. CONCLUSION: A combination of unenhanced and contrast-enhanced 3D time-of-flight MR angiography is superior for detecting paragangliomas and should be added to a standard imaging protocol, especially for patients with familial paragangliomas because they are more susceptible to multicentric disease.

Adult↗

PubMed1996

Multicentric metachronous pulmonary and intravagal paraganglioma: a case report with immunohistochemical findings.

An unprecedented presentation of multicentric paraganglioma in a 48-year-old man is described. One of the paragangliomas, originally diagnosed as a carcinoid tumor, presented as a lung mass and was removed. Four years later, an intravagal paraganglioma was discovered. The lung and intravagal tumors had identical morphologic and immunoreactive characteristics. Both tumors consisted of chief cells (type 1) and sustentacular cells (type 2). The chief cells were immunoreactive with neuroendocrine markers (synaptophysin and chromogranin), but nonreactive with epithelial markers (CAM 5.2, high- and low-molecular-weight keratins, epithelial membrane antigen, and carcinoembryonic antigen). The sustentacular cells were positive for S100 protein. Although pulmonary carcinoids may mimic paragangliomas and occasionally contain sustentacular cells, the diagnosis was rejected because the tumor cells did not demonstrate reactivity with epithelial markers.

Biomarkers, Tumor↗

PubMed2005

Deletions and altered expression of the RIZ1 tumour suppressor gene in 1p36 in pheochromocytomas and abdominal paragangliomas.

Pheochromocytomas and abdominal paragangliomas are rare catecholamine-producing tumours arising from neural crest-derived chromaffin cells. Frequent deletions of several distinct regions on the short arm of chromosome 1 suggest their involvement in the tumourigenesis process. The RIZ1 tumour suppressor encoded by the RIZ gene in 1p36.21 represents an attractive candidate target for the distal 1p deletions in these tumours. A panel of 18 pheochromocytomas (14 benign, and 4 malignant) and 11 abdominal paragangliomas (4 benign, and 7 malignant) were characterised for somatic deletions and mRNA expression status of RIZ1 using loss of heterozygosity (LOH) analysis and real-time quantitative PCR, respectively. Furthermore, we evaluated the methylation status of the RIZ1 promoter utilising methylation-specific PCR (MSP). Intragenic LOH at the RIZ locus was detected in 10 of 16 informative cases (62%), including 8 of 12 pheochromocytomas (67%) and 2 of 4 paragangliomas (50%). RIZ1 mRNA appeared to be significantly under-expressed in the tumour samples compared to normal adrenal controls (mean 0.6 vs. 1.0, p<0.001). This was not associated with RIZ1 promoter methylation in any of the samples, indicating that promoter hypermethylation is unlikely to be the underlying cause of the frequent expressional silencing. The recurrent inactivation of the tumour suppressor RIZ1 suggests that this event may be a significant contributing factor to tumour development in pheochromocytomas and abdominal paragangliomas.

Abdominal Neoplasms↗

PubMed2005

Laparoscopic resection of periaortic paragangliomas.

Paragangliomas are extra-adrenal pheochromocytomas that are often located in the retroperitoneum along the aorta. Although laparoscopic resection is recommended for adrenal pheochromocytomas, few laparoscopic resections for paragangliomas have been reported. We describe the presentation and imaging findings of four patients with periarotic paraganglioma. Laparoscopic resection was successfully performed in three of the four patients; the fourth patient required conversion to an open procedure. This report adds to a growing number of cases documenting that laparoscopic resection of paragangliomas is safe and feasible.

Adult↗

PubMed2005

Laryngeal paraganglioma: an endoscopic diode-laser-assisted surgical approach: a case report.

Laryngeal paraganglioma is a rare vascular neuroendocrine benign tumour. Surgery is the standard treatment. The pre-operative gold standard is Magnetic Resonance Imaging with Gadolinum contrast. Computed tomography scans and angiography are also useful in making the diagnosis of paraganglioma before surgical intervention. A case of a large supraglottic paraganglioma is described. Complete excision of the tumour was achieved using microlaryngoscopical-laser surgical resection. No severe complications were observed. The patient was discharged from hospital without any complaints on the fourth post-operative day and she was disease-free 36 months after surgery. The effectiveness of this conservative endoscopic surgical procedure compared to the external approaches is discussed and it can be concluded that the endoscopic diode-laser resection of even extended laryngeal paraganglioma provides a good operative field and is a safe and effective technique.

Angiography↗

PubMed2001

[Diagnosis and treatment of paragangliomas in the central nerve system].

OBJECTIVE: To explore the diagnosis and treatment of paragangliomas in the central nerve system. METHODS: 17 patients with paragangliomas in the central nerve system over a 20-year period (1976-1999) were analyzed retrospectively. They included 11 patients with glomus jugular tumors, 1 with carotid body tumor, 1 with supersellar paraganglioma, 2 with spinal involvement, and 2 with superficial hemisphere involvement. RESULTS: Neoplasm was completely resected in 11 patients, subtotally in 3, and partial in 3. The postoperative mortality was 1/17. One patient remained severe disability, yet the others had excellent long-time outcome. CONCLUSIONS: Both the characteristic clinical presentation and neuroradiological manifestation were useful for preoperative diagnosis. Total resection is ideal for paragangliomas. Preoperative embolization should be used routinely to control intraoperative bleeding, and postoperative radiation-therapy would be valuable to control the recurrence of the remaining tumor.

Adult↗

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↗

PubMedDate not supplied

Extra-adrenal nonfunctional retroperitoneal paraganglioma: case report and review of the literature.

An extra-adrenal paraganglioma is a rare tumor derived from the chromaffin cells of sympathetic ganglia. This report documents a rare case of nonfunctional extra-adrenal paraganglioma in a 20-year-old woman with persistent flank pain. Ultrasonography and computed tomography revealed a solid mass with calcification in center, measuring 7 x 6 x 6 cm, and localized in the left upper para-aortal retroperitoneal region. Absence of typical clinical symptoms made a correct preoperative diagnosis unlikely. Tumor was successfully removed through a midline laparotomy incision, and pathological analysis of the surgical specimen revealed a paraganglioma. After 12 months, the patient is still in a good health, asymptomatic, and without evidence of tumor recurrence. This case emphasizes the necessity to include extra-adrenal paraganglioma in the differential diagnosis and management of retroperitoneal tumors, despite its rarity.

Adult↗

PubMed2006

[Paraganglioma of the vagus nerve].

Paragangliomas of the vagus nerve are uncommon vascular benign neoplasms of neuroectodermic origin. Initial clinical manifestation is usually as an asymptomatic cervical mass, although sometimes may cause lower cranial nerve palsies. These paragangliomas seldom associate to high levels of circulating catecholamines. Diagnosis is based on the clinics aided by imaging, where CT and MRI play an important role. Angiography is not only diagnostic, but it also allows preoperative embolization of the mass. Most accepted treatment is surgical removal, even though some paragangliomas are suitable for radiation therapy in very specific patients. In this paper we describe a new case of paraganglioma of the vagus nerve in a cervical location, with hypertensive episodes and high catecholamine-levels. The authors review the literature describing the clinical presentation, the diagnosis and the treatment of this rare lesion.

Head and Neck Neoplasms↗

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↗