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PubMed2005

Diagnosis and treatment of carotid body paraganglioma: 21 years of experience at a clinical center of Serbia.

BACKGROUND: The carotid body paraganglioma (chemodectoma) is a relatively rare neoplasm of obscure origin. These are usually benign and commonly present as asymptomatic cervical mass. PATIENTS AND METHODS: Records of 12 patients (9 female and 3 male) with carotid body tumors treated between 1982 and 2003, treated at our center were retrospectively reviewed. Data on classification, clinical presentation, and surgical treatment were extracted from the case records. Surgical complications and treatment outcome were noted and survival was calculated by actuarial method. The literature on carotid body paraganglioma was reviewed. RESULTS: The average age of the patients was 52 years (range 30-78 years). Eight of these cases presented as a large asymptomatic non-tender neck mass, and two each presented with dysphagia, and hoarseness of voice. As per Shamblin classification seven of tumors were type II and 5 were types III. In 7 cases subadventitial tumor excision was performed, while in 5 associated resection of both external and internal carotid arteries was carried out. The artery was repaired by end-to-end anastomosis in one case, with Dacron graft in one case, and with saphenous vein graft in 3 cases. There was no operative mortality. After a mean follow-up of 6.2 years (range 6 months to 20 years), there were no signs of tumor recurrence in any of the cases. CONCLUSIONS: Surgical excision is the treatment of choice for carotid body paragangliomas although radiation therapy is an option for patients who are not ideal candidates for surgery. For the tumors that are in intimate contact with carotid arteries, the treatment by vascular surgeon is recommended.

Journal Article↗

PubMed2003

Malignant paraganglioma with vertebral metastasis: case report.

A paraganglioma is a rare tumor, composed of chromaffin cells, groups of cells associated to the autonomous system. When the tumor occurs in the adrenal gland, it is called pheochromocitoma. The malignant paraganglioma is a very rare presentation; it is diagnosed by local recurrence after total resection of the primary mass, or findings of distant metastases. We present a case report of a 29-year-old woman with cervico-brachial pain. In 1995 she underwent a carotid body tumor resection. Magnetic resonance imaging (MRI), plain X-rays and computerized tomography scan revealed multiple lesions in C5, T5 and T12. She underwent a surgical procedure to correct the cervical lesion. The histological and immunohistochemical assays revealed a malignant paraganglioma. She received adjuvant radiotherapy, showing clinical improvement after treatment, presenting no symptoms after one year. The therapeutic approach is based on the total resection of the tumor. The treatment of distant metastases can be made with adjuvant measures such as conventional radiotherapy, I 131-MIBG, or chemotherapy, especially in malignant pheochromocitomas.

Adult↗

PubMedDate not supplied

Inferior laryngeal paraganglioma mimicking a primary thyroid tumor.

OBJECTIVE: To report an unusual case of inferior laryngeal paraganglioma that manifested as a thyroid neoplasm. METHODS: A case report is presented, including ultra-sonographic, surgical, histologic, and immunohistochemical findings, and diagnostic and therapeutic strategies are discussed. RESULTS: In a 33-year-old man with a mass in the left side of his neck, color Doppler ultrasonography revealed an extremely hypervascular lesion that appeared to arise in the left lobe of the thyroid gland. Fine-needle aspiration was noncontributory because of bloody samples, but core biopsy specimens suggested a nonthyroidal neoplasm. At surgical intervention, a 3.5-cm mass was found immediately posterior to the left thyroid lobe, not involving the thyroid capsule. Because the mass could not be dissected free from the thyroid, performance of a left thyroid lobectomy and isthmectomy was necessary. The pathology specimen was a paraganglioma. CONCLUSION: Paragangliomas may be sonographically similar to thyroid neoplasms and may be included in the differential diagnosis of a hypervascular thyroid mass.

Adult↗

PubMed1998

Composite Paraganglioma-Ganglioneuroma of the Urinary Bladder: A Clinicopathologic, Immunohistochemical, and UItrastructural Study of a Case and Review of the Literature.

Urinary bladder paragangliomas are rare. An 81-yr-old woman was admitted because of whole-stream painless hematuria. Investigations revealed a pedunculated bladder tumor Pathological examination showed a biphasic tumor, composite paraganglioma-ganglio neuroma. The two separate components were documented by both immunohistochemical and ultrastructural studies. A review of the English literature on urinary bladder paragangliomas showed that the present case is the first case with this unique feature documented in detail, and the patient is the oldest one being reported.

Journal Article↗

PubMed2003

[Paraganglioma of the latero-sellar area. Case report].

Paraganglioma of the sellar and latero-sellar area is extremely rare. We report a case of latero-sellar and suprasellar paraganglioma of a 58-year-old women which caused deterioration of visual acuity and left exophthalmia without endocrine dysfunction. Magnetic resonance imaging showed a large tumor in the sellar and parasellar area, which extended to the left cavernous sinus and infiltrated the left orbit, the ethmoid, the sphenoidal sinus and the left pterygomaxillary fossa. Surgery by transcranial, left frontotemporal approach, enabled subtotal removal. Definitive histologic examination revealed a paraganglioma with signs of anaplasis. We report our clinical findings and present a review of literature.

Adenocarcinoma, Clear Cell↗

PubMedDate not supplied

[Carotid paraganglioma: a case report].

Carotid paraganglioma are tumors arising from chemoreceptor paraganglioma cells. These cells have proved to have an embryologic origin from the neural crests. Due to their low incidence these tumors are not an habitually found in daily practices. We present a case of carotid body paraganglioma diagnosed by clinical exploration, CT scan, magnetic resonance imaging and magnetic resonance angiography which was successfully treated by surgery. The diagnostic and therapeutic difficulties present in these tumors are discussed.

Angiography↗

PubMed1993

[Malignant paragangliomas--the results of radiotherapy in 6 patients].

Between February 1984 and May 1989, six patients (four male, two female) with malignant paragangliomas of the carotid body (n = 4) and glomus jugulare (n = 2) were irradiated in the Department of Radiation Oncology at Essen University. All patients had macroscopic (residual) tumor at the start of treatment. Five out of six paragangliomas had to be considered malignant, since histological lymph node involvement or distant metastasis was confirmed. In the sixth case extra- and intracranial tumor growth was observed. Total doses between 40 and 55 Gy using cobalt 60, 10 or 15 MeV photons were administered. Radiation therapy was canceled at 30.6 Gy in one patient because of multiple distant metastasis. Three out of four patients, who received curative treatments, were locally controlled (25 to 91 months). A local recurrence in one patient was detected 18 months after irradiation; this patient died of distant metastasis 23 months after treatment. In one out of two palliatively irradiated patients, local control could be achieved until the patient died of distant metastasis twelve months after treatment. Grade III- or grade IV-treatment toxicity was not observed. Radiation therapy of malignant paragangliomas with doses between 45 and 54 Gy in five to six weeks is an effective treatment with low toxicity and should be preferred to surgery in glomus jugulare and advanced carotid body tumors.

Adult↗

PubMed1987

Intracranial catecholamine secreting paragangliomas.

Three intracranial catecholamine-secreting paragangliomas are described. They involved a glomus jugulare, a sphenopalatine ganglion and the clivus and upper cervical spine respectively. The extent of the tumours was shown by CT and MRI. They were all highly vascular with a substantial blood supply from systemic arteries which was subjected to particulate embolisation, followed in two cases by surgery. The importance of studying any tumour which could possibly be a paraganglioma for hormonal and, especially, for catecholamine secretion prior to any invasive procedure, including angiography and embolisation, is emphasised: all such procedures should be covered with catecholamine blocking agents and, in addition, carefully monitored with resuscitation facilities immediately available.

Adult↗

PubMed1990

Histopathology of benign versus malignant sympathoadrenal paragangliomas: clinicopathologic study of 120 cases including unusual histologic features.

The clinical and pathologic features of 120 adrenal and extraadrenal paragangliomas were studied in an attempt to identify features which might predict malignant behavior. Clinical follow-up was obtained in 98 cases (82%); 64 tumors were clinically benign, and 34 were malignant as evidenced by regional or distant metastases and/or extensive local invasion. Thirty-two of the 34 malignant tumors (94%) were functionally active. Features noted more frequently in malignant tumors included male predominance (74%; P2 [two-sided P value] = .002), extraadrenal location (52%; P2 less than .0001), greater tumor weight (mean 383 g versus 73 g for nonmalignant tumors), confluent tumor necrosis, and the presence of vascular invasion and/or extensive local invasion. Intracytoplasmic hyaline globules were seen in 59% and 32% of benign and malignant tumors, respectively (P2 = .001). Logistic regression analysis of 16 nonhistologic and histologic parameters showed four of them to be most predictive of malignancy--extraadrenal location, coarse nodularity of the primary tumor, confluent tumor necrosis, and absence of hyaline globules. Most malignant paragangliomas had two or three of these features (71%), while 89% of benign tumors had only one (or none; P less than .0001). According to the statistical model developed, there was better than a 95% probability that more than 70% of tumors could be classified correctly on the basis of the four factors indicated. Although limitations still exist, results of this study provide some basis for evaluating malignant potential of these tumors.

Adolescent↗

PubMed1988

Histopathology, cytology and cytochemistry of pheochromocytomas and paragangliomas including chemodectomas.

The results of histopathological, histochemical and ultrastructural investigations on pheochromocytomas and paragangliomas have been reported. These results allowed the functional identification of the cell types composing many of such tumours. Moreover, comparison of these data with clinico-pathologic findings outlined the advantages and limits of cytologic studies for understanding the natural history of pheochromocytomas and paragangliomas and improving our diagnostic and prognostic criteria.

Adrenal Gland Neoplasms↗

PubMed1989

Catecholamine-secreting paraganglioma of glomus jugulare region.

A 43-year-old woman had clinical and biochemical evidence of a secreting paraganglioma of the glomus jugulare region. Catecholamine secretion was exacerbated during embolization of the tumor before surgery and resulted in a life-threatening vasomotor attack. Preoperatively, pharmacologic blockade of excessive catecholamine secretion with prazocin controlled her blood pressure, tachycardia, and symptoms. The tumor was resected and its catecholamine content measured. This case is reported to stress the importance of adequate preoperative assessment of patients with paragangliomas of the head and neck. The extreme rarity of catecholamine-secreting tumors of this region should not lead us to underestimate the morbidity and mortality of such patients undergoing surgery or any other invasive procedure whether the diagnosis is confirmed or only suspected.

Adult↗

PubMed1984

Paragangliomas of the temporal bone: high-resolution CT studies.

Seventeen patients with 15 surgically proved paragangliomas involving the temporal bone and parapharyngeal spaces were examined with high-resolution computed tomography (CT). Scans were obtained in multiple axial (0 degree, 30 degrees) and coronal (70 degrees, 105 degrees) section planes using 1.5-mm collimation and table incrementation. The purpose of this study was to demonstrate the utility of HRCT in assessing tumor extent and size, with particular emphasis on intrinsic temporal bone invasion. The most characteristic HRCT signs of paraganglioma include: jugular fossa expansion, soft-tissue mass in the hypotympanum and sinus tympani, permeation of the infracochlear surface, dural ballooning adjacent to the jugular fossa (indicative of epidural tumor), anterior displacement of parapharyngeal fat planes, and extension into the proximal aspect of the eustachian canal. It is concluded that HRCT is the single most efficacious examination to determine tumor extent, especially when intratemporal invasion has occurred, and to define other additional clinically unsuspected lesions.

Diagnosis, Differential↗

PubMed1993

Iodine-131-metaiodobenzylguanidine scintigraphy in preoperative and postoperative evaluation of paragangliomas: comparison with CT and MRI.

Iodine-131-metaiodobenzylguanidine (MIBG) scintigraphy, transmission computed tomography and magnetic resonance imaging were used to evaluate 36 patients with clinically suspected functioning paragangliomas. The patients were divided into two groups. In Group 1 (n = 21), studied before surgery, patients mainly had benign adrenal disease. In Group 2 (n = 15), studied after surgery, patients frequently had malignant or extra-adrenal tumors. In Group 1, transmission computed tomography and magnetic resonance imaging were more sensitive (100% for both) than MIBG scintigraphy (82%), which, however, was the most specific (100%). In Group 2, MIBG scintigraphy and magnetic resonance imaging were more sensitive (83% for both) than transmission computed tomography (75%), but MIBG was again the most specific (100%). Thus, all three were complementary modalities for localizing paragangliomas both preoperatively and postoperatively. MIBG imaging is indicated for both groups but it is especially recommended for postsurgical patients with recurrence because the disease is often malignant or extra-adrenal.

3-Iodobenzylguanidine↗

PubMed1983

Laryngeal paraganglioma. Report of two tumors with immunohistochemical and ultrastructural analysis.

Paraganglionic tumors of the larynx are uncommon neoplasms and their secretory products have rarely been elucidated. We saw two patients whose laryngeal paragangliomas were examined with an electron microscope and by the use of immunocytochemical techniques. Both tumors manifested positive immunoreactivity against calcitonin and vasoactive intestinal polypeptide. One of the paragangliomas was immunoreactive against carcinoembryonic antigen and pursued a biologically malignant course. Our observations suggest the coexistence of amines and hormonal polypeptides in cells of paraganglionic tumors and demonstrate the multihormonal nature of these tumors.

Aged↗

PubMed1987

Paraganglioma of the temporal bone in infancy. A congenital lesion?

Jugulotympanic glomus tumors have been called many things, but "paraganglioma of the temporal bone" is perhaps a more suitable term. This tumor is generally described as benign with a predilection for middle-aged women. Herein we report the case of an 11-month-old female infant with this condition. The extensiveness of the tumor with intracranial involvement at presentation suggests that the lesion might have been present for some time and raises the question of it being congenital in origin. We emphasize the overall importance of including paraganglioma of the temporal bone in the differential diagnosis of a "bleeding polyp" of the ear.

Female↗

PubMed1988

Paragangliomas of the head and neck.

The medical records of 64 patients with paraganglioma of the head and neck seen between 1954 and 1984 at the University of Texas M.D. Anderson Hospital, Houston, were reviewed. Lesions were found in the carotid body (n = 24), jugulotympanic area (n = 37), vagal nerve (n = 4), and larynx (n = 1). A hereditary pattern was identified in six patients (9%). Metastases developed and caused the death of two patients (8%). No functional secreting tumors were identified. Paragangliomas are often misdiagnosed, both clinically and pathologically. Angiography is the definitive clinical study for diagnosis and treatment planning. Computed tomographic scanning further defines extent of the disease. Lesions in the neck are effectively treated by surgery. Jugulotympanic tumors can be successfully managed by surgery, radiation therapy, or a combination of both depending on the size and extent of the tumor. Secondary treatment of residual disease can be as effective as primary treatment.

Adult↗

PubMed1992

Nucleolar organizer regions in paragangliomas of the head and neck.

The clinical behavior of head-and-neck paragangliomas cannot be accurately predicted using standard histologic criteria. Immunohistochemical profiles have proved to be prognostically helpful; however, other independent indicators of prognosis are needed. Tissue markers of proliferative activity include argyrophilic nucleolar organizer regions (AgNOR), which are proteins specifically associated with loops of transcriptionally active ribosomal DNA. Fifteen paragangliomas of the head and neck were divided into solitary nonrecurrent (n = 8), recurrent or locally invasive (n = 4), and multiple (n = 1), or malignant (n = 2) groups. The mean AgNOR count per cell was statistically different between the solitary nonrecurrent and the combined other poorer prognosis groups, suggesting that it may be useful as an independent indicator of biologic behavior. The wide variation in AgNOR counts within groups and the overlap of counts between groups limit, however, the predictive value of this technique for individual tumors.

Adult↗

PubMedDate not supplied

Color Doppler imaging of paragangliomas in the neck.

PURPOSE: In this study, we describe the color Doppler imaging findings in carotid body tumors and vagal body tumors. METHODS: B-mode and color Doppler imaging were performed on 17 patients who had a total of 25 previously diagnosed paragangliomas (14 carotid body tumors and 11 vagal body tumors). RESULTS: Nineteen of 25 tumors were depicted. Five small vagal body tumors in the region of the nodose ganglion and 1 carotid body tumor could not be depicted. With B-mode imaging, paragangliomas appeared as well-defined, solid, hypoechoic masses. With color Doppler imaging, hypervascularity with a low-resistance flow pattern was demonstrated in all but 1 of the 19 tumors. CONCLUSIONS: The use of color Doppler imaging in the workup of an ambiguous neck mass is advocated.

Adult↗