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PubMed2000

MR appearance of paraganglioma of the cauda equina. Case reports.

PURPOSE: To investigate the value of MR imaging for preoperative diagnosis of paraganglioma of the cauda equina. MATERIAL AND METHODS: A retrospective review of 2 cases of paraganglioma of the cauda equina examined with MR imaging was undertaken. Features assessed included the homogeneity of the lesions, presence or absence of serpiginous flow void and thin hypointense margins. RESULTS: In case 1, the tumor was hyperintense on the postcontrast examination and serpiginous flow void suggested vessels in the upper pole of the tumor. In case 2, the tumor was encapsulated by a thin hypointense margin on both T1- and T2-weighted images, which suggested hemosiderin. CONCLUSION: The MR appearance may be of great value in the preoperative diagnosis of paraganglioma of the cauda equina.

Cauda Equina↗

PubMed1999

Nuclear medicine therapy of pheochromocytoma and paraganglioma.

Pheochromocytomas and paragangliomas are rare catecholamine-producing tumors which arise from chromaffin tissue. When a pheochromocytoma/paraganglioma is suspected, biochemical confirmation is based on 24-hour urinary excretion rates of catecholamines and their metabolites (metanephrines, VMA, etc.). Following biochemical confirmation non invasive imaging techniques such as CT and/or MR of the abdomen and 123I-MIBG scintigraphy are performed to localize the tumor. 111In-octreotide may also be applied, mainly to localize head and neck chemodectomas. Malignant paragangliomas of either adrenal or extra-adrenal origin show a variable natural history: from a locally invasive indolent tumor to a highly aggressive malignancy. Surgery with complete resection or debulking of the primary tumor is the standard treatment. External radiotherapy and chemotherapy are usually scarcely effective. An alternative treatment is 131I-MIBG therapy which is performed with high specific activity 131I-MIBG. Usually a standardized dose ranging from 3.7 to 9.1 GBq of 131I-MIBG is administered by slow i.v. infusion. In advanced stage cases 131I-MIBG therapy aims at symptom palliation and tumor function reduction as well as at tumor arrest or tumor regression. In these cases MIBG therapy allows prolonged survival and good quality of life. In less advanced cases the purpose of MIBG therapy is to complement surgery and to achieve the total eradication of the tumor. Non functioning malignant paraganglioma can some time also concentrate MIBG and can be treated with high doses of the tracer. 131I-MIBG therapy is a safe treatment and is usually well tolerated by the patient (with rather low myelotoxicity).

3-Iodobenzylguanidine↗

PubMedDate not supplied

Paraganglioma of the bladder: report of a case.

Paragangliomas are uncommon tumours that comprise less than 0.1% of all primary bladder neoplasms. Approximately half of the patients present the clinical triad of hypertension, haematuria and attacks associated with micturition. About 10% of vesical paragangliomas behave malignantly. This may be manifested as local invasion or metastatic spread, particularly to lymph nodes and lung. The best therapy remains total excision and partial cystectomy is considered adequate treatment for most cases. We report a case of paraganglioma of the bladder with extramural appearance in a 39-year-old man. Clinical symptoms were hypertension, dysuria and a well-defined hypogastric mass. One year after complete removal of the tumour, the patient is asymptomatic and normotensive, with normal catecholamine and vanillylmandelic acid (VMA) levels.

Adult↗

PubMed1999

Malignant paraganglioma arising from the posterior mediastinum: a case report and review of the literature.

An unusual case of paraganglioma of posterior mediastinum occurred in a young adult with local recurrence and multiple distant metastasis. Because of its rarity, the determinants of prognosis factor between benign and malignant paraganglioma are uncertain. In this case, we investigated abnormalities of the p53 gene and ras gene mutations in tissues of primary and metastatic lesions. Neither abnormalities of p53 gene nor ras gene mutations were detected. The molecular approach is recommended as a means of clarifying the trend towards the malignancy of paraganglioma.

Adult↗

PubMed2000

[Retroperitoneal paraganglioma incidentally detected by health examination: a case report].

We report a case of non-functioning retroperitoneal paraganglioma detected incidentally by health examination in a 60-year-old female without any clinical manifestation. She was admitted to our hospital for the purpose of evaluation of a renal mass detected by ultrasound sonography. Computed tomography, magnetic resonance imaging and angiography revealed the heterogeneously-enhanced 10 x 10 cm mass in the left retroperitoneal space without any distant metastasis. It was suspected to be a renal cell carcinoma. All laboratory data, including those from endocrinological examinations, were within normal ranges. Radical nephrectomy was performed to resect en bloc the mass and the left kidney. The pathological and immunohistochemical examinations of the mass which was completely separated from the kidney and the adrenal gland, showed characteristic features of paraganglioma with vascular and capsular invasion, with a final clinical diagnosis of the pathologically-malignant non-functioning paraganglioma occurring between the left kidney and the adrenal gland.

Female↗

PubMed2001

[Significance of CT findings and catecholamine determination in peripheral blood of asymptomatic pheochromocytoma and paraganglioma].

The purpose of this study was to assess the CT findings and significance of hormone determination in the peripheral blood of asymptomatic patients with pheochromocytoma and paraganglioma. CT findings in 29 patients with surgically proven pheochromocytoma(n = 19) and paraganglioma(n = 10) were reviewed. Nine patients(31%) were symptomatic and 20 (69%) were asymptomatic. Tumor size ranged from 39 mm to 114 mm(mean: 60 mm) in symptomatic patients and 11 mm to 100 mm(mean: 50 mm) in asymptomatic ones. Of the 9 symptomatic patients and 18 asymptomatic patients, a homogeneous solid pattern was seen in 4 and 4, mixed pattern in 2 and 6, and massive necrotic pattern in 3 and 8 patients, respectively, on CT scans. The CT attenuation values in symptomatic cases ranged from 30 HU to 50 HU(mean: 41 HU) on precontrast CT scans and 60 HU to 111 HU(mean: 77 HU) on postcontrast CT scans, while those in asymptomatic cases ranged from 15 HU to 48 HU(mean: 33 HU) on precontrast CT scans and 66 HU to 133 HU(mean: 95 HU) on postcontrast CT scans. There were no statistically significant differences in tumor size, homogeneity, or CT attenuation values between symptomatic and asymptomatic patients. All symptomatic patients and 17(89%) of 19 asymptomatic cases showed elevated levels of catecholamine (epinephrine) or norepinephrine in the peripheral blood. Our study showed that the CT findings in asymptomatic patients were similar to those in symptomatic patients, and 89% of asymptomatic patients showed elevation of catecholamine in the peripheral blood. Determination of catecholamine level in the peripheral blood is recommended for preoperative diagnosis in patients suspected of having asymptomatic pheochromocytoma or paraganglioma on CT scans.

Adrenal Gland Neoplasms↗

PubMed2001

Paraganglioma manifesting as shock: a case report.

Paraganglioma is a rare neuroendocrine tumor in children that rarely manifests as shock. We describe the case of a 12-year-old girl with paraganglioma who developed impaired cardiac function, pulmonary edema, and shock at the time of admission. Her blood pressure stabilized after intravenous normal saline rescue and dopamine treatment. However, hypertension was noted thereafter. After a series of examinations, paraganglioma was diagnosed and excision of the tumor was performed. After surgery, blood pressure stabilized and her cardiac function had fully recovered at 4 months' follow-up.

Child↗

PubMed2001

Assignment of PGL3 to chromosome 1 (q21-q23) in a family with autosomal dominant non-chromaffin paraganglioma.

We performed a whole genome scan in a family with maternally transmitted paraganglioma (PGL3). The family included five patients with histologically proven paraganglioma and one patient with imaging findings consistent with a paraganglioma. In addition, there were 33 clinically unaffected family members. Of these eight could be examined by magnetic resonance imaging. Our investigations indicate that PGL3 is located in 1q21-q23 for several reasons: 1) two-point linkage analysis yielded the highest LOD score of 2.25 at 1q21-q23 (marker D1S2675); 2) haplotype analysis was most consistent for 1q21-q23 markers; and 3) the locus was excluded from more than 97% of the genome using a total of 381 highly polymorphic markers.

Chromosomes, Human, Pair 1↗

PubMedDate not supplied

[Paraganglioma of the cauda equina with papillary pattern and cytokeratin expression: 2 potential sources of diagnostic error. Report of a case and review of the literature].

UNLABELLED: Paragangliomas are unusual neuroendocrine tumours, rare in the cauda equina and filum terminale. Due to their low frequency, no classical patterns and dual immunophenotype, may be misinterpreted as others neoplasms more frequent in this site. It is our aim to report a case of paraganglioma of the cauda equina and filum terminale (PGCCFT), standing out it's histological-immunohistochemical pattern, differential diagnosis, prognostic and treatment. We reviewed the clinical presentation, NMR features and morphological aspect of one PGCCFT, diagnosed in the ABC Medical Center's Surgical Pathology Department of Mexico City. Report of the case: 26 years old man, who had been suffering a low lumbar pain for one year before he came to our hospital. The NRM showed heterointense lesion in the level of first and second vertebra, suggesting ependymoma. The tumor was totally resected. Histologically it was papillary, the chief cells have stained with chromogranin, synaptophysin, and cytokeratin. The few sustentaculars cells were S100 positive. The patient have haven a favorable course. CONCLUSIONS: The certainty and differential diagnosis of paragangliomas of the cauda equina and filum terminale needs correlation between histological pattern and immunophenotype, the last must be considered in the peculiar context of this anatomic region.

Adult↗

PubMed2001

Clinics in diagnostic imaging (68). Intradural extramedullary spinal paraganglioma.

Paragangliomas rarely present as spine tumours. The correct diagnosis is generally not suspected pre-operatively and initial imaging is often non-specific. A 36-year-old man with low back pain, and progressive leg numbness and weakness, was found to have an expansile intradural extramedullary spinal tumour on radiographs and magnetic resonance imaging. Surgery revealed a paraganglioma. The features of spinal paraganglioma and differential diagnosis of intradural extramedullary tumours are discussed.

Adult↗

PubMed2002

[Non-secreting, extra-adrenal retroperitoneal paragangliomas].

OBJECTIVE: Two cases of extra-adrenal non-secretory retroperitoneal paraganglioma in male patients are presented. The first case had intermittent vascular claudication in lower limbs and the second one was an incidental finding during staging of a lymphoma. METHODS: Both cases were studied using imaging techniques (US, CT and MRI) and histological and immunohistochemical analyses. RESULTS: The imaging techniques demonstrated solid homogeneous retroperitoneal masses intimately adhered to the abdominal aorta in the first case and located in the left upper hemiabdomen (in front of the left kidney) in the second one. Histological analysis showed a proliferation of spindle cells with cuboid cytoplasm arranged in solid nests, with typical immunohistochemical findings of paraganglioma. CONCLUSIONS: Extra-adrenal retroperitoneal paraganglioma are rare tumors, particularly the non-secretory variant. The only reliable criteria for malignancy are extensive capsular invasion and distant metastases.

Aged↗

PubMed2002

[Secreting paraganglioma of the organ of Zuckerkandl. A case report].

We report a case of a secreting retroperitoneal paraganglioma which developed on the organ of Zuckerkandl. Paraganglioma is a rare tumor of the paraganglioma system arising from undifferentiated cells of the neural crest. The originality of our observation comes from the secreting character of the tumor and the malignity proved by the local spreading to the wall of the inferior vena cava and the metastasis. The probable existence of a double primary localisation is also exceptional.

3-Iodobenzylguanidine↗

PubMed2003

Multiple paragangliomas of the head and neck.

Multiple paragangliomas of the head and neck are rare conditions. The incidence of multiple paragangliomas is reported to the approximately 10% of the total patients, but in familial cases it increases up to 35-50%. In the head and neck region, the most common association is represented by bilateral carotid body tumors or by carotid body tumor associated with tympanic-jugular glomus. The presence of three synchronous glomus tumors is really rare, as well as association with vagal glomus and carotid body. In this paper the authors present a patient affected ipsilaterally by a carotid body tumor and vagal paraganglioma, focusing on the diagnostic options offered by imaging techniques (CT and MRI).

Carotid Arteries↗

PubMed1992

[A case of subclavian paraganglioma in the superior mediastinum extending to the neck].

A 52-year-old female was admitted to our hospital with left anterior chest pain. On physical examination, an elastic hard mass sized in 3.0 x 2.5 cm was palpable at the left suprasternal area. Chest X-ray film showed a large mass shadow in the cervico-thoracic region, and CT films revealed a well-defined mass in the superior mediastinum which was heterogeneously enhanced by contrast medium. Operation was performed under the preoperative diagnosis of thymic tumor extending upwards. At operation, the tumor was located between the trachea and the left subclavian artery in the superior mediastinum displacing these structures and extending to the neck. Resected specimen which was 5.5 x 3.5 x 3.0 cm in size and 42 gm in weight was well encapsulated and highly vascularized. On microscopic findings, the tumor was finally diagnosed as paraganglioma. According to the classification of Glenner and Grimley, it was thought that the tumor originated from subclavian paraganglion in the superior mediastinum. Subclavian paraganglioma had rarely been reported in the literatures. As subclavian paraganglioma, our patient was the first reported case in Japan.

Female↗

PubMed1992

Papilledema and intraspinal lumbar paraganglioma.

Optic nervehead swelling is most frequently caused by ocular or intracranial lesions. The case presented here demonstrates that the spinal subarachnoid space must also be considered as a potential site for a lesion causing optic nervehead swelling. A 56-year-old man is presented with an intraspinal lumbar paraganglioma associated with increased cerebrospinal fluid protein, papilledema, transient obscurations of vision, and back pain. This may be the first reported case of a paraganglioma associated with optic nervehead swelling. Magnetic resonance imaging of the lumbosacral region revealed the lesion noninvasively. The papilledema, transient obscurations of vision, and back pain resolved after resection of the tumor. The mechanisms are not defined for optic nervehead swelling in association with spinal tumors in general and paraganglioma in particular. The measured abnormal elevation of cerebrospinal fluid protein may have resulted in increased intracranial pressure and papilledema.

Back Pain↗

PubMed1992

[Carotid artery obstruction caused by vagal paraganglioma].

A 72 year-old-man presented with right hemiparesis and global aphasia related to a large infarct in the left middle cerebral artery territory. A left carotid angiography revealed internal carotid artery occlusion and vascular blush just below the base of the skull. The blush was supplied by branches from the external carotid artery. Cervical CT-scan showed a large tumor centered by the left internal carotid artery. At cervical surgery, the internal carotid artery was compressed and infiltrated by the tumoral process: a benign paraganglioma. The site below the base of the skull and the connection between tumor and vagal nerve suggested a vagal paraganglioma. To our knowledge, this is the first case of cerebral infarction and internal carotid artery occlusion revealing a vagal paraganglioma.

Aged↗

PubMed2003

Paraganglioma of the urinary bladder--a case report.

Paragangliomas of the urinary bladder are extremely rare tumors accounting for less than 1% of all bladder tumors. Males and females are affected roughly equally with an average age of 41 years. Hypertension and headache during or immediately after voiding in association with intermittent hematuria is virtually diagnostic of urinary bladder paragangliomas. A high index of clinical suspicion is required to diagnose these tumors. We present a case of a urinary bladder paraganglioma because of its rarity.

Adult↗

PubMedDate not supplied

[Paraganglioma of the common right iliac artery. Case report and review of the literature].

UNLABELLED: Paraganglioma is a neoplasm derived from the neural crest of the neuroendocrine system. Head and neck account for its more frequent localizations. Parasympathetic paraganglioma have been encountered at cauda equina and iliac vessels. Its clinical course is unpredictable (mitosis, pleomorphism, and even vascular invasion), as various authors affirm that it apparently depends on quantity of protein S-100 contained as structural support. The present paper is a report of a patient who underwent an appendectomy. During abdominal surgery, surgeons discovered a retroperitoneal tumor located above iliac artery that included the characteristic Zellballen cellular arrangement. CASE REPORT: A 44-year-old male patient with abdominal pain diagnosed with acute appendicitis was surgically operated on at the ISSSTE General Hospital Darío Fernández Fierro in Mexico, City. During surgery, a retroperitoneal mass was found located above the common right iliac artery. It was completely resected. Microscopic study established characteristics of paraganglioma. Patient outcome was good. Scanning for other tumor sites was negative.

Adult↗