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PubMed2003

[Bladder paraganglioma (pheochromocytoma): report of a new case].

The authors report one case of vesical paraganglioma, not suspected in a 50-years-old female patient who presented gross hematuria as only symptom. The patient was not hypertensive. Radiologic and endoscopic investigations revealed a trigonal submucosal tumour. Complete transurethral resection of the tumor was performed with no intraoperative complications. The histopathological diagnosis indicated pheochromocytoma. Blood pressure was stable. There has been no recurrence for 18 months after transurethral resection.

Female↗

PubMed2001

Jugulotympanic paragangliomas.

Jugulotympanic paragangliomas are the most common tumors of the middle ear and temporal bone. Although these larger tumors can prove to be formidable, the advent of microscopic and skull base surgical techniques has greatly enhanced the ability to treat and manage these tumors.

Glomus Jugulare Tumor↗

PubMed2006

Tympanic paragangliomas: case reports.

Glomus tumors, also called paragangliomas, originate from nonchromaffin cells. The tumor is typically vascular and grows from capillary and pre-capillary vessels in-between epithelial cells. It is worth mentioning that the most common symptoms are pulsating tinnitus and hearing loss. Imaging studies (CT and MRI) are necessary for diagnosis. This paper shows five patients seen at the Hospital between 1995 and 2001 presenting glomus tympanicum. Women were most commonly affected, and the age ranged from 48 to 60 years (mean age of 50 years). The most common complaints were pulsating tinnitus and hearing loss. All patients were treated surgically.

Ear Neoplasms↗

PubMed1998

Paragangliomas of the head and neck region.

Three patients with paragangliomas of the head and neck region were referred to an oral and facial unit during a 16-month period. Because such lesions are rare, it was not initially considered in the differential diagnosis. This paper reviews these tumours and stresses the importance of including them in the differential diagnosis of a neck mass.

Aged↗

PubMed2001

Outcome of surgical treatment for carotid body paraganglioma.

BACKGROUND: The present study reviewed a 30-year experience of managing carotid body paraganglioma (CBP) and analysed clinical findings associated with perioperative morbidity. METHODS: Clinical records, radiological findings and pathological reports of all patients who presented with CBP between 1966 and 1997 were reviewed. There were 39 consecutive patients with 45 tumours and median follow-up was 10 years. The Shamblin classification was used to define complication rates and long-term surgical results. RESULTS: Preoperative information derived from magnetic resonance angiography (MRA) and colour Doppler imaging (CDI) was comparable to that from standard four-vessel arteriography. Forty-one CBPs were resected in 35 patients. Six patients had bilateral tumours and seven had multicentric tumours. The median duration of operation and blood loss were substantially higher for Shamblin type III tumours. All major vascular complications (four of 39 patients) and permanent neurological complications (three of 39) were observed in type III tumours. CONCLUSION: Surgical planning and prediction of perioperative complications can be obtained by staging derived from MRA and CDI. Severe complications occur predominantly in type III CBPs.

Adolescent↗

PubMed1993

[Risk of endocrine activation in interventions of paragangliomas in the head-neck area].

The intracytoplasmatic glycolytic enzyme neuron-specific enolase (NSE) can be found in cells of endocrine tissue and their derived tumours. The enzyme is not secreted but released if cells are destroyed, i.e. continuously in malignant tumours. The serum level of neuron-specific enolase (NSE) has been used as a marker for small cell carcinomas of the lung, glioblastomas and malignant phaeochromocytomas. In this investigation blood samples were taken prior to and following surgical manipulation of the tumour in 21 patients with paragangliomas of the head and neck region and 6 controls. 22 serum samples were obtained before angiography and two hours after angiography, 41 before surgery, during surgery and after surgery. The serum level of NSE was measured by NSE-RIA test (Diagnostics, Uppsala). In all tumour specimens NSE could be demonstrated by the immunohistological PAP method (Abb. 1). Only two patients had preoperatively elevated serum-NSE levels. An arterial venous shunt had been detected by angiography in one of these patients. No significant effect on NSE serum level could be produced in the majority of patients by an angiography with embolisation or surgical manipulation of the tumour (Fig. 1, 2). A reduction in serum NSE level was observed postoperatively in both cases mentioned before. None of the 6 controls showed any significant change in serum NSE level (Fig. 3). It can be concluded from this study that embolisation does not lead to direct or indirect cell destruction through ischaemia. Manipulation of the tumour does not destroy a significant number of cells.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed2001

Intracranial paraganglioma (glomus tumor) with orbital extension.

PURPOSE: To describe the clinical features of an intracranial glomus tumor with orbital extension. METHODS: Case report. RESULTS: A 15-year-old girl was examined for a large, vascular intracranial mass extending into the orbit. Attempted orbital biopsy was aborted for fear of uncontrollable hemorrhage. Tumor embolization and partial resection was performed. Histology was consistent with paraganglioma (glomus tumor). CONCLUSION: The orbital surgeon should include glomus tumor in the differential diagnosis of vascular orbital lesions. Their vascularity and hemorrhagic potential should be considered when approaching these lesions surgically.

Adolescent↗

PubMed2004

Transpharyngeal ultrasound detection of carotid body paraganglioma.

We report a patient in whom a right carotid body paraganglioma was delineated using a transpharyngeal approach during a transesophageal echocardiographic (TEE) study. Color Doppler-guided pulsed-Doppler interrogation of the right internal carotid artery in the tumor region, revealed high maximum systolic and maximum diastolic velocities of 1.5 m/s and 1.0 m/s, respectively, suggestive of tumor compression.

Carotid Artery, Internal↗

PubMed1987

A functioning intrapericardial paraganglioma (pheochromocytoma).

A patient with a functioning intrapericardial paraganglioma (pheochromocytoma) that was located at the root of the aorta overlying the right coronary artery and adherent to the right ventricular wall is reported. The tumour was successfully removed under total cardiopulmonary bypass without inducing cardiac arrest.

Female↗

PubMed1996

First experiences with genetic counselling based on predictive DNA diagnosis in hereditary glomus tumours (paragangliomas).

Hereditary glomus tumour (MIM 168,000) or paraganglioma (PGL) is a slowly progressive disorder causing benign tumour growth predominantly in the head and neck region. Though benign in nature the tumours can lead to severe morbidity. Inheritance of PGL is autosomal dominant and is strongly modified by genomic imprinting; only a paternally transmitted PGL gene leads to symptoms. A gene for PGL has recently been mapped to 11q22.3-q23. Genetic counselling on the basis of DNA linkage diagnosis was offered in an extended Dutch pedigree. Thirty-two subjects opted for further counselling, of whom 20 applied for DNA testing and participated in a standardised protocol. Sixteen cases had presymptomatic testing (paternal allele); four of these appeared to have the at risk haplotype and in two of them a glomus tumour was subsequently detected on MRI. In one case linkage results were inconclusive (recombination) and one person did not want to learn his test result. Four cases had testing for carrier status (maternal allele) of which one appeared to be a carrier. Our data show that genetic counselling gains significant accuracy when based on parent of origin, sex of the counsellee, and DNA linkage diagnosis. Moreover, a normal DNA result may prevent unnecessary worry and investigations, while an established presymptomatic diagnosis will guide adequate clinical management. The psychological impact of counselling and predictive DNA testing is unclear as yet. Further investigations into the natural history of PGL in gene carriers and into the psychological impact of DNA testing is desirable.

DNA, Neoplasm↗

PubMed1993

Bilateral carotid body paraganglioma and central alveolar hypoventilation.

A 62-year-old woman with a bilateral carotid body paraganglioma presented, 2 years after the removal of the right one, with signs of right-heart failure. Hypoxemia, hypercapnia, polycythemia and pulmonary hypertension with normal ventilatory capacity were found. Central alveolar hypoventilation was diagnosed on the basis of absence of ventilatory response and sensation to provoked hypercapnia, prolonged breath-holding time and correction of hypercapnia by voluntary hyperventilation.

Carotid Body Tumor↗

PubMed2004

Jugulotympanic paraganglioma (glomus tumour) presenting with recurrent epistaxis.

A case is presented where a left jugulotympanic paraganglioma (JTP) extended to the nasopharynx and the patient presented with recurrent epistaxis. Although initial biopsy of an aural polyp had been suggestive of the diagnosis several years previously, the diagnosis was not confirmed until the patient presented with recurrent epistaxis and severe anaemia. To the best of our knowledge, this is the first case reported of such a presentation of JTP.

Ear Neoplasms↗

PubMedDate not supplied

[Pheochromocytoma and paraganglioma].

Pheochromocytoma and paraganglioma are neoplasias of the chromaffin cells that manifest themselves in some 40-60% of cases by symptoms of episodic freeing of catecholamines. They are the cause of stable and malign HTA in some 0.1-1% of cases and must be discounted in a study of the same. They most frequently affect males and their incidence increases with age. The symptomatic episodes are basically characterised by high blood pressure, migraines, sweating and palpitations, although they can have other multiple manifestations and even take a silent form in upto 40-50% of cases. They are frequently associated (10%) with multiple endocrine neoplasias and other neuroectodermic syndromes. For their diagnosis it is essential to measure the catecholamines and their metabolites, both plasmatic and urinary, and to locate the tumour by means of image tests, especially magnetic resonance. In the handling of these tumours it must be born in mind that the carrying out of physical maneuvers on the tumour and the employment of drugs that free catecholamines can induce hypertensive crises. The treatment chosen is always surgical, curative in cases of benign pheochromocytoma or producing a reduction of the tumourous mass and associated symptoms in cases of malign pheochromocytoma (10%); in this latter case treatment is complemented with chemotherapy or drugs that control the tumourous symptoms. Untreated pheochromocytoma can be a mortal disease, hence the importance of its detection and early treatment.

English Abstract↗

PubMed1992

Malignant paraganglioma of the glomus jugulare: a case report.

Jugular paragangliomas are noted for their tendency to recur, but frank malignant behaviour is rare. We report a case in which a patient harbouring a recurrent tumour of the glomus jugulare re-presented with metastatic spread to mediastinal lymph nodes. Review of the 16 reported cases provides information which can be a basis for management of the malignant form of this tumour.

Female↗

PubMedDate not supplied

A carotid body and glomus jugulare paraganglioma secreting norepinephrine.

Extra-adrenal paragangliomas (PGOMAs) are rare and can occur anywhere along the sympathetic chain including the carotid body, glomus jugulare, vagal bodies, ganglium tympanicum, larynx, ciliary bodies, organs of Zuckerkandl, urinary bladder, and other locations. These are microscopically identical. The embryological origin is from neural crest cells. One to three percent are reported to be functioning, predominantly secreting norepinephrine. Diagnosis of a functioning PGOMA can be delayed, even when symptoms of catecholamine hypersecretion are present. Furthermore, diagnostic testing and surgery have a significant morbidity if the diagnosis is not considered in advance, as illustrated by the following cases. PGOMAs can be sporadic or familial with an autosomal dominant pattern with variable penetrance. They can be multicentric and associated with other endocrine gland tumors. Benign and malignant PGOMAs have been described, with malignancy being defined by lymph node metastasis. Few cases of functioning glomus jugulare and carotid body tumors have been reported in the medical literature.

Journal Article↗

PubMed2000

[Bilateral carotid paraganglioma].

We report an unusual case of bilateral paraganglioma of the carotid. The tumor had been neglected for a long time and increased in volume during pregnancy, inducing compression and requiring surgical treatment. We focus on the therapeutic strategy: devascularization followed by percutaneous sclerosis before total surgical removal in a two-step procedure.

Adult↗

PubMed2001

Prognostic value of DNA flow cytometry in sympathoadrenal paragangliomas.

OBJECTIVE: To determine whether ploidy patterns are related to prognosis in sympathoadrenal paragangliomas (SAP) using flow cytometry. STUDY DESIGN: DNA flow cytometric analysis of formalin-fixed, paraffin-embedded tumor samples from 36 patients with SAP was performed. Eight cases fulfilled at least one of the following malignancy criteria: (1) extensive invasion of adjacent structures (5 cases), (2) local recurrence (3 cases), or (3) metastases (4 cases). RESULTS: Of the 36 tumors, 22 (61%) showed nondiploid patterns (12 aneuploid, 10 tetraploid). All diploid tumors were benign, while all malignant cases showed nondiploid patterns (P = .0131). The differences between diploid and aneuploid tumors and between diploid and tetraploid tumors, with regard to the malignancy of the disease, were statistically significant (P = .03311 and .01976, respectively). Only one malignant tumor had a DNA index < 1.75 (P = .00259). CONCLUSION: Anomalous DNA ploidy patterns are frequent in SAP, without necessarily implying malignancy. However, diploid DNA content may be a marker of a good prognosis. The likelihood of malignancy is greater in the tetraploid and peritetraploid range.

Adolescent↗

PubMed2001

[Diagnostic image (63). Paraganglioma].

A 33-year-old woman was examined for a non-tender mass in the neck. Imaging findings were consistent with paraganglioma. The diagnosis was confirmed at surgery.

Adult↗