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PubMed2004

Multiple pheochromocytomas and paragangliomas in a young patient carrying a SDHD gene mutation.

UNLABELLED: We report a 13-year-old boy with an atypical manifestation of a multilocular paraganglioma. Surgical treatment was not curative despite extirpation of a left-sided abdominal paraganglioma. After surgery, the boy experienced several hypertensive crises. Further investigations including dopamine-positron emission tomography demonstrated bilateral adrenal tumours, a further right-sided paravertebral tumour as well as bilateral cervical glomus tumours. Genetic testing revealed a germline mutation in the succinate dehydrogenase subunit D (SDHD) gene. CONCLUSION: The final diagnosis was familial pheochromocytoma/paraganglioma type 1 (OMIM 168000). Antihypertensive treatment was succesfull and improved the patient's quality of life.

Abdominal Neoplasms↗

PubMed2003

Altitude is a phenotypic modifier in hereditary paraganglioma type 1: evidence for an oxygen-sensing defect.

Hereditary paraganglioma type 1 (PGL1) is characterized by slow-growing and vascularized tumors that often develop in the carotid body (CB) and is caused by mutations in the gene for succinate dehydrogenase D ( SDHD) of mitochondrial complex II. The mechanisms of tumorigenesis and the factors affecting penetrance and expressivity are unknown. Because chronic hypoxic stimulation at high altitudes causes sporadic CB paragangliomas, it has been hypothesized that the SDHD gene product may be involved in oxygen sensing. On this background, we examined genotype-phenotype-environment relationships and tested whether higher altitudes adversely affect the phenotype in PGL1. An analysis of 58 subjects from 23 families revealed that nonsense/splicing mutation carriers developed symptoms 8.5 years earlier than missense mutation carriers ( P<0.012). We also found that subjects who were diagnosed with single tumors at their first clinical evaluation lived at lower average altitudes and were exposed to lower altitude-years than those with multiple tumors ( P<0.012). Pheochromocytomas developed in six subjects (approximately 10%), five of whom had nonsense mutations ( P=0.052). Subjects with pheochromocytomas also lived at higher average altitudes and were exposed to higher altitude-years than those without them ( P=0.026). To test whether altitude is also associated with the more frequent detection of germ-line founder mutations among sporadic cases in The Netherlands than in the USA ( P=0.00033), we calculated population-weighted elevations of the two countries. We found that the population-weighted elevations were approximately 260 m for the US and 2 m for the central-western Netherlands ( P~0), where three Dutch founder mutations were discovered. This finding suggests that low altitudes in The Netherlands reduce penetrance and relax the natural selection on SDHD mutations. Collectively, these data suggest that higher altitudes and nonsense/splicing mutations are associated with phenotypic severity in PGL1 and support the hypothesis that SDHD mutations impair oxygen sensing.

Adrenal Gland Neoplasms↗

PubMed2004

A functioning-desensitization paraganglioma which caused hemodynamic instability during tumoral resection: report of a case.

Paragangliomas are uncommon tumors arising from the neuroendocrine elements of the paraganglia. Their successful management is associated with many problems. We herein present the findings of a 22-year-old man in whom a paraganglioma was incidentally found and in which the clinical and previous operative behavior was functioning desensitization. As a result, preoperative medication was not performed; however, during the tumor resection the patient demonstrated hemodynamic instability.

Adult↗

PubMed2000

Intracerebral paraganglioma.

Paragangliomas of the CNS are relatively rare. Cases of location in the pineal and pituitary glands, cerebellopontine angle, cauda equina and filum terminale are known. In our neurosurgical unit a 42-year-old male patient with a history of vertigo and a generalized seizure underwent an operation for a fronto-temporal tumour. The histological diagnosis was paraganglioma.

Adult↗

PubMed2003

Paragangliomas of the parasellar region.

Parasellar paragangliomas are rare tumors. As far as we know, only ten cases are described in the literature. Their clinical, pathological, and radiological features and possible origin are discussed in this article and a review of the literature is given. Additionally, we report a new case of a 51-year-old woman with paraganglioma growing in the anterior, middle, and posterior cranial fossa with extended destruction of the skull base. The patient had been suffering from long-standing headaches and facial nerve paresis. Preoperatively, this tumor was suspected to be a meningioma.

Female↗

PubMed2003

Hyperostotic paraganglioma of occipitotemporal bone.

A 25-year-old male presented with off-and-on vertigo of 10-year duration. He had left-sided cerebellar signs, left vocal cord paresis, and minimal left-sided hearing impairment. Computed tomography of the head revealed significant hyperostosis of squamous occipital, mastoid, and petrous temporal bone with no adjacent soft tissue mass. On excision, it turned out to be paraganglioma. Paraganglioma as a pure bony mass is not reported in the literature. The site of origin of such a tumor could not be ascertained, even on generous screening. The tumor remained nonsecretory on clinical and biochemical investigations.

Adult↗

PubMed1985

Metastatic intravagal paraganglioma. Case report and review of the literature.

Intravagal paragangliomas are rare tumors of neural crest origin. These tumors are usually benign with few reports of metastases or aggressive behavior. One case of intravagal paraganglioma metastatic to a regional lymph node is described and the diagnosis and management of these tumors are discussed. The reports of 14 other cases with regional or distal metastases are reviewed.

Adult↗

PubMed1992

Paraganglioma of the posterior mediastinum: value of magnetic resonance imaging.

A case of paraganglioma arising in the posterior mediastinum in a 29-year-old man diagnosed by magnetic resonance imaging is reported. Excision of mediastinal paraganglioma is often hazardous because of its rich vascular supply and tendency to involve surrounding structures. Magnetic resonance imaging is valuable for the preoperative diagnosis of this vascular tumor as well as for determination of its resectability and appropriate surgical procedure.

Adult↗

PubMed1991

Paraganglioma of the urinary bladder: immunohistochemical, ultrastructural, and DNA flow cytometric studies.

Primary paraganglioma arises infrequently in the urinary bladder. We present the clinicopathologic, immunohistochemical, ultrastructural, and DNA flow cytometric findings in three cases (one man and two women). Ages at diagnosis were 19, 35, and 45 years. One female presented with paroxysmal headaches and hypertension that followed urination; the remaining two patients presented with hematuria. Immunohistochemical studies revealed positive reactivity for chromogranin (three patients), met-enkephalin (three), leu-enkephalin (three), vasoactive intestinal polypeptide (two), serotonin (one), and S-100 protein (one; sustentacular cells only). Neurosecretory granules were identified in all cases; in the patient with hypertension, the granules were small with eccentric cores similar to those of adrenal pheochromocytomas. A nondiploid DNA flow cytometric pattern was present in all three patients, an aneuploid pattern was present in two, and a tetraploid pattern was present in one. After diagnosis, one patient was alive without progression at 7 years, one died of an uncertain cause at 5 years, and one suffered multiple recurrences over a 24-year period before developing metastatic disease. While the presence of aneuploidy has been shown to be a predictor of malignant behavior in adrenal pheochromocytomas, our study illustrates that DNA ploidy cannot be used as a diagnostic criterion for malignancy in urinary bladder paraganglioma.

Adult↗

PubMed1984

Hemibase syndrome: an unusual presentation of intracranial paraganglioma.

A case of paraganglioma of parasellar origin in a 7-year-old girl is described. She presented with hemibase syndrome with involvement of a majority of the left cranial nerves. Three times in the past 2 years she had recurrent ophthalmoplegia with complete recovery. She was treated surgically and with postoperative radiotherapy. The site of origin of this paraganglioma as well as the clinical presentation are uncommon features in this case.

Child↗

PubMed1978

Ultrastructure of malignant paraganglioma of organ of Zuckerkandl.

Electron microscopic study of a malignant paraganglioma of the organ of Zuckerkandl revealed similarities between the tumor and the normal paraganglia. The well-differentiated portion of the tumor recapitulated the structure of the basic functional units of the paraganglion. In addition there signs of anaplasia both at the histologic and ultrastructural level. Large neurosecretory granules were noted in some of the tumor cells, but most of the cells were agranular. Crystaloids resembling those seen in alveolar soft part sarcoma were also noted. The study supports the theory on the common origin and histogenesis of paragangliomas and alveolar soft part sarcomas.

Chromaffin System↗

PubMed1981

Malignant paraganglioma of retroperitoneum. Light, electron microscopic and ultrasonographic study.

A malignant retroperitoneal nonchromaffin paraganglioma in a thirty-four-year-old man was studied by light and electron microscopy. Histologically, the tumor cells displayed a tendency to surround granular, eosinophilic intercellular material and to form nests and pseudo-acini. Ultrastructurally, the tumor was composed of moderately well-differentiated epithelial cells intermixed with sparse sustentacular cells. An organoid pattern, reminiscent of the functional anatomic unit of nonchromaffin paraganglia, was seen occasionally. Epithelial cells formed pseudo-acini around dilated microvillous processes. These morphologic features are consistent with the neurocrestal origin of paragangliomas. The patient died ten months after presentation despite an initial favorable response to irradiation.

Adult↗

PubMed1991

Paraganglioma of urethra.

Paraganglioma of the urethra is a rare tumor; only 4 cases have been reported previously. We report a fifth case of urethral paraganglioma and review the literature.

Aged↗

PubMedDate not supplied

Vagal paragangliomas: a report of nine cases.

Vagal paragangliomas are rare tumors of neural crest origin: fewer than 175 cases have been reported in the English literature. This slow-growing tumor occurs most often at the base of the skull in the parapharyngeal space, but may arise anywhere along the course of the vagus nerve and its branches. Nine clinical presentations and the surgical outcome in seven patients with vagal paragangliomas treated at our institution are reported. A review of the literature, emphasizing evaluation and treatment of these potentially fatal neoplasms, is discussed. Surgical removal is the treatment of choice with vagal tumors. Complications related to the ablation or injury of cranial nerves IX, X, XI, and XII are commonly seen. Surgical techniques that may prevent injury to these vital neural structures are outlined. The postoperative treatment of patients with impaired deglutition secondary to multiple cranial neuropathies is discussed.

Adult↗

PubMed1994

MRI of orofacial tumors and paragangliomas with 2D GE sequences: indications and optimal sequence parameters.

The aim of this paper is to determine to what extent and in which cases 2D gradient echo (2D GE) sequences can be applied alternatively or additionally to spin echo (SE) sequences for improved diagnostic evaluation. Imaging with SE sequences is the most frequently used MR technique in the assessment of ear, nose and throat (ENT) tumors. In literature there are only a few reports on the contrast behaviour of 2D GE sequences using different sequence parameters and their application in ENT tumors. This paper set out to establish the most suitable sequence type and sequence parameters. Measurements were performed with a Magnetom SP 63 MR system (Siemens) with a field strength of 1.5 T, using head and Helmholtz coils. One-hundred twenty-eight volunteers and 369 patients were examined with 2D GE sequences. In order to find the best MR technique for the examination of orofacial tumors and paragangliomas, FLASH, FISP and PSIF sequences with different sequence parameters (TR, TE, flip angle, bandwidth) were applied. The results of these examinations confirmed the superiority of 2D GE sequences over SE sequences. In conclusion, contrast enhanced or unenhanced T1 weighted SE sequences should be replaced by 2D GE FLASH 70 degrees in the examination of orofacial tumors. In the case of paragangliomas of the jugular bulb and the parapharyngeal space T1- and T2-weighted SE sequences should be replaced by 2D GE FLASH 40 degrees.

Head and Neck Neoplasms↗

PubMedDate not supplied

Case report: CT of a duodenal paraganglioma.

Paraganglioma is a neoplasm of neuroectodermal origin that occurs rarely in the bowel. This case report illustrates the previously undescribed CT appearance of a duodenal paraganglioma. An intensely enhancing mass in the region of the pancreatic head was found. The intense enhancement ruled out adenocarcinoma and focal pancreatitis. An islet cell tumor of the pancreas was the major differential diagnostic consideration.

Diagnosis, Differential↗

PubMedDate not supplied

Nonfunctional paraganglioma of the liver.

Paragangliomas occurring in the liver are rare. We report a patient with a history of nonfunctional paragangliomas in the head and neck who developed liver metastases years later. We discuss findings on cross-sectional imaging modalities, including magnetic resonance imaging (MRI), which demonstrate the hypervascular nature of the liver masses.

Adult↗

PubMedDate not supplied

Paraganglioma presenting as chronic otitis media with cholesteatoma: pitfalls and strategies.

The coexistence of paraganglioma with cholesteatoma is a very rare clinical disorder. The clinical presentations are nonspecific. There may be radiological characteristics of either cholesteatoma or paraganglioma in the middle ear area, but the diagnosis of the coexistence of the 2 is usually made only postoperatively. Here is such a case that is made more interesting not only because it initially presented with conductive hearing loss but also because the clinical picture mimicked chronic otitis media. The patient underwent postauricular tympanomastoidectomy with extended facial recess approach to remove the tumor. No evidence of recurrence and complications were noted.

Cholesteatoma, Middle Ear↗