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PubMed2000

[Primary paraganglioma of the urinary bladder with high serum CA19-9 level: a case report].

A case of primary paraganglioma of the urinary bladder with a high serum CA19-9 level is reported. A 44-year-old woman visited our hospital with the chief complaint of lower abdominal pain. Magnetic resonance imaging (MRI) examination incidentally revealed a cystic bladder tumor. Cystoscopy disclosed a broad-based non-papillary tumor on the posterior wall of the urinary bladder. With the diagnosis of a bladder submucosal cystic tumor transurethral needle puncture and biopsy were performed. The solution sampled with puncture was bloody. The patient suddenly complained of headache and blood pressure was elevated to 215/120 mmHg when we held the tumor with a cold cup biopsy forceps. Catecholamine levels of the solution in the tumor were abnormally elevated. The serum CA19-9 level was also raised. Ten days later, she underwent partial cystectomy. Histological findings of the removed specimen showed primary paraganglioma of the urinary bladder. The serum CA19-9 level decreased to normal limits on the 28th postoperative day. Our experience suggests that the level of serum CA19-9 may serve as a useful index for observing the clinical course of a patient with this disease.

Adult↗

PubMed2000

Extraadrenal retroperitoneal paraganglioma causing malignant hypertension and permanent visual loss in an eleven-year-old girl.

We report a secretory paraganglioma in an eleven-year-old Trinidadian girl of East Indian descent who presented with malignant hypertension, proteinuria and modest elevation of urinary vanillylmandelic acid levels. The extra-adrenal mass was surgically removed without complications. The patient has been normotensive without evidence of recurrence of disease but with persistent visual sequel two years later. This case reinforces the observation that extraadrenal retroperitoneal paragangliomas can be functionally very active and early computed tomography or other imaging procedure is necessary for rapid noninvasive evaluation, timely surgery and prevention of permanent morbidity.

Child↗

PubMed2000

Vagal paraganglioma. Report of a case surgically treated and review of the literature.

The authors report the observation of one case of vagal paraganglioma occurred in a young woman. The tumor manifested itself as a left sub-mandibular tumescence; the very first diagnostic approach was achieved through echography, which showed a mass behind the internal carotid artery and compressing the internal jugular vein. The following examinations, represented by neck CT, NMR, angiography and fine needle aspiration initially directed towards the suspicion of chemodectoma. Only at operation, the anatomical situation of the tumor, which encapsulated the vagus nerve and the subsequent results of the hystological examination revealed the correct diagnosis of vagal paraganglioma.

Adult↗

PubMed2000

[Malignant paraganglioma of the uterus].

We report a malignant uterine paraganglioma in a 41-year-old woman who underwent a hysterectomy for meno-metrorrhagia. It was initially thought to be a leiomyoma in necrobiosis. The clinical outcome was characterized by an early regional recurrence (in the left Fallopian tube). Later, vertebral and lung metastasis occurred, leading to death 22 months after the initial diagnosis. Paragangliomas are uncommon neuroendocrine tumors, related to pheochromocytomas. They are mainly found in the para-aortic and retroperitoneal region, and less commonly in the pelvic area. Location in the uterus is extremely rare: 5 cases were previously reported and only one malignant.

Adult↗

PubMedDate not supplied

Multiple recurring paragangliomas: a problem of diagnosis and location. Case report and review of the literature.

RATIONALE: Paragangliomas constitute a particular diagnostic challenge because of the difficulties involved in identifying new lesions. CASE REPORT: The findings in the case of a young woman with multiple recurring paragangliomas which reappeared eleven years after successful surgical treatment are presented. DISCUSSION: This case illustrates the diagnostic difficulties of this pathologic entity and the lack of a technique of sufficient sensitivity to reveal the existence of one or more tumors with or without hormonal secretion, their location and rate of growth and the number of neoplasias to avoid as many surgical interventions as possible. Inasmuch as an ideal technique for diagnosis and localization of this type of tumor remains to be developed, non-invasive methods, particularly 131I MIBG scintigraphy, play a significant role in evaluation because of their high sensitivity for detecting chromaffin tissue, especially in extra-adrenal locations. CONCLUSION: Periodic long-term radionuclide scanning should be recommended for all asymptomatic patients given the risk of residual and/or reactivating tumoral tissue with benign or metastatic behavior.

Adult↗

PubMed2000

[Diagnosis and treatment of temporal paragangliomas: an analysis of 21 cases treated in Bern between 1992 and 1999].

INTRODUCTION: Paragangliomas are usually benign tumours stemming from the neural crest which may have neuroendocrine activity. Because of hypervascularisation they usually lead to pulsatile tinnitus. Local expansion initially causes middle ear and later inner ear hearing loss, and cranial nerve dysfunction. PATIENTS/METHODS: Signs and symptoms, demographic data, tumour stages, treatments and outcomes were analysed for the 21 patients treated at our clinic between 1992 and 1999. Among 16 surgically treated patients 3 type A and 4 type B tumours were treated by mastoidectomy and middle ear reconstruction. For the 8 type C and one type D tumours, angiography was performed preoperatively, usually followed by selective embolisation of tumour vessels, in 7 cases with additional permanent occlusion of the internal carotid artery. These 9 tumours were removed through an infratemporal approach with partial petrosectomy, preservation of the inner ear and facial nerve rerouting, followed in 8 cases by middle ear occlusion. The patient with a type D2 tumour had a two-stage otological and neurosurgical operation. One patient with type C3 tumour only underwent radiation therapy. 4 patients have had no treatment so far: 2 had small type A tumours and one a type C3 tumour but contraindications for surgery. A last patient with a slowly growing type D2 tumour cannot be operated on due to general health problems. RESULTS: All patients with type A and B tumours had normal to subnormal postoperative hearing and normal facial nerve function. In one case with a type C tumour the middle ear reconstruction led to normal hearing thresholds. After rerouting of the facial nerve, transient palsy was noted in 6 out of 9 cases. In 3 patients a slight permanent impairment of facial nerve function (House-Brackman II) remained. 4 patients had some dysfunction of cranial nerves IX-XII postoperatively. After a follow-up of up to 8 years (mean 5 years) no recurrence was observed in the surgically treated patients. The patient treated by radiation had further tumour growth and another treatment is being planned. Among the 4 patients followed without treatment, no tumour growth has been observed up to now in 3 patients. DISCUSSION: Our study confirms that the well standardised conservative surgical treatment allows reliable removal of paragangliomas in most cases, and involves low morbidity.

Cranial Nerve Diseases↗

PubMed2001

Aortico-pulmonary paraganglioma associated with bilateral carotid body tumors. Diagnostic presentation and clinical implications.

A case of mediastinal paraganglioma in association with bilateral carotid body tumors is presented. Characteristic radiological findings included a hypointense signal in T1-weighted, a hyperintense signal in T2-weighted magnetic resonance (MR) images and a vascular enhancement pattern in dynamic contrast enhanced MR imaging. Thus, feeding vessels could be depicted noninvasively. The importance of family screening in affected individuals is stressed, as a hereditary form of the disease exists in which multiple paragangliomas are common.

Aorta, Thoracic↗

PubMed2001

Paraganglioma of the bile duct.

We report the case of a 28-year-old woman with a 3-month history of right upper quadrant abdominal pain. Medical history and physical examination were unremarkable. Abdominal ultrasonography showed a dilated gallbladder, suggestive of gallstones, without bile duct dilatation. Serum chemistry values were normal. Laparoscopic cholecystectomy was planned, and a mass encircling the common bile duct was found. The procedure was converted to an open operation, and cholecystectomy, mass excision, and hepaticojejunostomy were done. The specimen was interpreted as paraganglioma of the bile duct. There were no manifestations of catecholamine hypersecretion or signs of biliary obstruction. Six years later, the patient is asymptomatic without signs of recurrence. This is the third report of paraganglioma of the hepatic ducts. We describe the clinical and pathologic findings and therapeutic approach in this unusual case and review the literature.

Anastomosis, Roux-en-Y↗

PubMed2002

[Nonchromaffinic paraganglioma (chemodectoma) of tympanic cavity].

Nonchromaffinic paraganglioma derives from neuroectoderma. It is characterised by plenty vascularisation. Histopathologically it is a benign tumor, and doesn't give any metastasis. Although because of localisation, vascularisation and possibility of bone destruction is clinically malignant. In this paper we present three cases of paraganglioma localised in tympanic cavity treated surgically.

Adult↗

PubMed2002

Primary pulmonary paraganglioma.

We describe a 46-year-old man with a mass in the lower lobe of the right lung. The tumor was initially suspected to be a poorly differentiated adenocarcinoma after endobronchial biopsy. Subsequently, a right lower lobectomy was performed, and a paraganglioma was diagnosed histologically. Using whole-body CT scan and metaiodobenzylguanidine (MIBG) scintigraphy, we excluded the involvement of other organs. No evidence of recurrence or metastasis was found during the follow-up period of 3 years. Primary pulmonary paragangliomas are very uncommon tumors. Literature relevant to this disease entity is discussed.

3-Iodobenzylguanidine↗

PubMed2002

Intrapericardial paraganglioma.

We report a case of intrapericardial paraganglioma accidentally discovered during surgery for mixed mitral valve disease. The 6 cm tumor was located in the aorta-caval groove, adherent to the roof of the left atrium and compressing the superior vena cava. A biopsy of the mass established the histological diagnosis of a non pressor secreting paraganglioma. The tumor was resected 2 months after the original mitral valve surgery. The patient made an uneventful recovery and remains well 2 years after surgery.

Adult↗

PubMed2002

Twelve-year experience in the investigation and treatment of paragangliomas.

We report a 12-year laboratory experience in the diagnosis of 37 patients with phaeochromocytoma, one with malignant paraganglioma and one with ganglioneuroblastoma. Surgery was performed in eight different hospitals, which underlines the difficulty of ensuring uniformity of investigations. Of the 37 with phaeochromocytoma, 15 were in the right adrenal gland, 12 were on the left, seven were bilateral and three were extra adrenal. Twenty-three were male and 15 were female; the median age was 36 years. Four of five patients with von Hippel-Lindau disease had bilateral adrenal tumours and four had neurofibromatosis. None had multiple endocrine neoplasia. Using essential hypertensive patients as a reference population, diagnostic sensitivity was 80% for noradrenaline, 54.4% for adrenaline, 19.1% for dopamine, 93.3% for total normetanephrine, 33.3% for total metanephrine, 78.7% for 3-methoxy-4-hydroxymandelic acid (HMMA) and 23.5% for homovanillic acid (HVA). However, if each patient's results were taken as a package, the sensitivities were 100% for noradrenaline, 63.6% for adrenaline, 30.3% for dopamine and 89% for HMMA. Seven recent patients had gene analysis. There were six (15.4%) deaths due directly or indirectly to the paraganglioma. Postoperative blood pressures were normal in 74%. The decrement in catecholamine values post-surgery is shown. Urinary noradrenaline values fell by a mean of 94.5% and the use of urinary noradrenaline as a tumour marker is also demonstrated.

Adrenal Gland Neoplasms↗

PubMed2003

[Paraganglioma of the vagus. A case report and review of literature].

The vagal nerve paraganglioma is a rare tumor over all the head and neck tumours. It presents with a insidious clinic, appearing usually as a asymptomatic laterocervical mass of low growth pattern. We present a case of a 53 years old woman with a left of painless laterocervical mass that produced cough with the palpation. We pretend to review the most relevant aspects of the diagnosis and treatment of the paraganglioma and therefore include this pathology in the differential diagnosis of laterocervical tumor.

Cranial Nerve Neoplasms↗

PubMedDate not supplied

[Angiographic evaluation and embolization of cervico-cranial tumors -- juvenile paraganglioma and angiofibroma of the nasopharynx].

INTRODUCTION: The goal of pre-operative embolization is tumoral desvascularization, with decrease of intraoperative hemorrhage and shortening of operation time and, consequently, decrease operative morbility. The authors review their experience in angiographic evaluation and preoperative embolization of paragangliomas and juvenile nasopharyngeal angiofibroma (JNA). PATIENTS AND METHODS/RESULTS: There were reviewed the angiographic characteristics of 22 patients with paragangliomas, most of them jugular and/or tympanic, 10 of them embolized with PVA. There were angiographically evaluated 6 JNA, 5 of which were embolized with PVA. There weren't any neurological complications during the procedures, diagnostic angiography and embolization. In the cases submitted to endovascular treatment, there was a significative preoperative desvascularization rate, with subjective improving of intraoperative conditions in terms of blood loss. CONCLUSION: A detailed angiographic characterization of the tumor allows the evaluation of embolization risks, due to the presence of anastomoses or arterial feeding of important structures. Tumor preoperative embolization must be done in a restricted group of hypervascular tumors, in a multidisciplinary approach with the surgical team and must be a safe procedure, without carrying additional morbility to the surgery.

Adult↗

PubMedDate not supplied

Primary cardiac paraganglioma. Case report and review of surgically treated cases.

A 58 year old man with an infiltrating non-resectable left atrial paraganglioma was diagnosed by sternotomy and open biopsy. The patient remains symptom free 16 months after the operation. No evidence of an increase in the size of the tumour has been detected by echocardiography. The literature regarding surgically treated patients with cardiac paragangliomas is reviewed.

Heart Atria↗

PubMed2003

Multidisciplinary management of paragangliomas of the head and neck, Part 1.

Paragangliomas most commonly occur in the carotid body, jugulotympanic area, and vagus nerve but have also been reported in other areas of the head and neck. These tumors are highly vascular and characteristically have early blood vessel and neural involvement, making their treatment particularly challenging. Surgery has traditionally been the preferred method of treatment, especially in light of recent advances in technique. However, compared to radiation therapy, it can result in a higher incidence of cranial nerve dysfunction. Radiation therapy has the advantage of avoiding the increased morbidity of surgery while offering an equal possibility of cure. Part 1 of this two-part article focuses on techniques for diagnosing paraganglioma and the indications for and use of surgery as primary treatment. The complications commonly associated with surgery are reviewed, and strategies for rehabilitation of affected patients are presented.

Head and Neck Neoplasms↗

PubMed1992

[Cervical branchiomeric paraganglioma. Report of 21 cases].

Over the last 24 years the authors operated 21 branchiomeric paragangliomas in 18 patients. They emphasize the difficulty of making the diagnosis and the importance of completing a thorough work-up before deciding surgery. Adequate imaging is particularly important (neck sonography, computed tomography, arteriography) to avoid making the diagnosis by exploratory neck dissection and to assess the vascular risk. Biological studies have regained importance, especially in the context of complex neuro-cristopathy. Treatment is essentially surgical and must be considered with prudence in patients with bilateral lesions. The surgical removal of branchiomeric paragangliomas necessitates preparation of a small saphenous vein bypass in case it is not possible to avoid sacrificing the internal carotid artery.

Adult↗

PubMed1992

[The dynamics of the cerebral circulation during the surgical treatment of paragangliomas of the neck].

Clinical and rheoencephalographic studies ot 17 cases of paraganglioma of the neck revealed insufficient collateral blood circulation after compression of the common carotid artery on the affected side. In such cases, surgery carries a risk of brain ischemia. Improvement of collateral blood circulation was achieved by rheoencephalographically controlled compression of the common carotid artery performed daily with increasing duration. A patient was considered eligible for surgery if no signs of brain hemisphere ischemia were apparent following a 40% or less decrease in rheoencephalographic anacrotism amplitude from baseline. Common or internal carotid arteries were resected in 6 out of 17 cases of neck paraganglioma dissection. Adequate preoperative preparation prevented cerebral ischemia development in the postoperative period.

Adult↗