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PubMed2006

[Paraganglioma of the head and neck--tumor control, functional results and quality of life].

BACKGROUND: Surgical therapy for paragangliomas (PG) of the head and neck is, due to the alternatives of radiation therapy and wait-and-scan strategy and because of postoperative morbidity, under ongoing discussion. MATERIAL AND METHODS: Between 1981 and 2004, 79 patients with 94 PG of the head and neck were treated at our department. These patients had follow-up examination within a clinical trial considering tumor control, functional results and for the first time neuropsychologically evaluated postoperative quality of life. Mean follow-up time was 65 months (1 to 228 months). RESULTS: Among the 94 PG there were 19 carotid body (GCP), 12 vagal nerve (GVP) and 63 jugular-tympanal paragangliomas (JTP). Of these, 87 tumors underwent surgery. In 68 patients (78.1 %), the tumor could be removed completely. In particular, complete resection of GCP was achieved in 100 %, of GVP in 90.9 %, of JTP type A in 100 %, of JTP type B in 83.3 %, of JTP type C in 66.6 % and of JTP type D in 61.5 %. During the follow-up period, residual or recurrent tumors were diagnosed in 17 patients (19.5 %). Six of the seven residual PG were observed by magnetic resonance tomography and did not show growth. One residual PG and 6 recurrencies were resected completely. One recurrent tumor was radiated and 3 others are under observation without showing growth tendencies. Two patients died postoperatively due to borderline operations of extended tumors. The quality of life after PG surgery showed a SIP of 4.8, which is comparably much better than after acoustic neuroma surgery (SIP 10.3). CONCLUSION: Whereas complete tumor resection of GCP and JTP types A and B is almost ever possible without cranial nerve palsies, surgery of GVP and advanced JTP causes often severe functional deficits. However, postoperative quality of life is mostly good. Nevertheless, advanced PG require an individualized therapeutic regime also including radiation and observation of tumor growth.

Adult↗

PubMed1989

[Juxtapapillary gangliocytic paraganglioma of the duodenum].

A juxtapapillary duodenal polyp (measuring 1.5 x 1.0 x 1.2 cm) was removed endoscopically in a 57-year-old man who had had recurrent intestinal bleedings. Histological examination revealed a typical duodenal gangliocytic paraganglioma with carcinoid and paraganglioma-like components, as well as neuroid portions with ganglion cells. Various neuroendocrine markers (neuron-specific enolase, chromogranin, protein-gene product 9.5) were demonstrated immunohistochemically. Tests for pancreatic polypeptide, substance P, enkephalin, cholecystokinin, glucagon and S-100 proteins, were all positive.

Ampulla of Vater↗

PubMed2000

Vagal paraganglioma: the Jefferson experience.

Vagal paraganglioma is a rare tumor of neural crest origin. Although the literature is in agreement with regard to epidemiology, diagnosis, and tumor biology, there is some controversy over treatment modalities for these patients. We performed a nonrandomized retrospective study in a large single-institution series of patients (n = 19) in whom vagal paraganglioma was diagnosed. General statistics included age, male/female ratio, tumor size, and duration of follow-up. Other variables such as signs and symptoms at presentation, family history, multicentricity, metastatic disease, and secretion of catecholamines were included. CT scan, MRI, and angiography were used in combination for diagnostic purposes as well as for treatment planning. Preoperative embolization was performed in 5 of the more recently treated patients. Current issues regarding the use of preoperative embolization and choice of surgical approach were analyzed. In this article the possibility and sequela of vagus nerve-sparing procedures will be presented. Operative complications and postoperative morbidity related to cranial neuropathies will be discussed. The rationale for performing adjunct procedures, including cricopharyngeal myotomy and vocal fold medialization, to facilitate the rehabilitation of patients with postoperative cranial nerve deficits will be given. Our findings and recommendations will be compared with currently accepted treatment protocols in conjunction with a review of the literature.

Adult↗

PubMedDate not supplied

Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study.

We report two cases of Gangliocytic Paraganglioma (GP) of the ampulla of Vater occurring in a 63-year-old and a 34-year-old individual. The patients were both admitted for a long history of intermittent gastrointestinal bleeding and abdominal discomfort, with no other symptoms. At endoscopy, the GP appeared as a polypoid, ulcerated mass in the ampullar region, measuring 2.5x1.8 and 2 cm, respectively. Microscopically, the tumors showed similar features and were composed of epithelial cells (more than 50%), spindle cells, and ganglion-like cells. The epithelial cells showed clear cytoplasm and formed nests (zellballen or paraganglioma-like groups), and less frequently, cords (carcinoid-like), extending to mucosa and submucosa. Ganglion cells were sparse, constantly associated with the spindle cells. Both epithelial and ganglion cells were synaptophysin, chromogranin A, and anti-neurofilament immunoreactive. The spindle cells were all S-100 positive. Ultrastructural studies revealed dark and light cells, rare elongated cellular processes, secretory granules, and fine fibrils resembling neurofilaments. The histogenesis of GP is still a matter of debate, however its neoplastic nature is supported by the occasionally reported malignant evolution.

Adult↗

PubMed1998

Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: a review of 282 cases. French Association of Surgery (AFC), and The French Association of Endocrine Surgeons (AFCE).

OBJECTIVE: To find out the optimal strategy for the preoperative location of pheochromocytomas and paragangliomas. DESIGN: Retrospective study. PATIENTS: 282 patients operated on for histologically confirmed pheochromocytoma in France between 1980 and 1991, the past decade. MAIN OUTCOME MEASURES: The results of imaging procedures, i.e. computed tomography (CT), (131)I meta-iodobenzylguanidine scintigraphy (MIBG) and magnetic resonance imaging (MRI) were reviewed. RESULTS: Pheochromocytomas were sporadic in 206 (73%). They were unilateral in 189 (67%), bilateral in 54 (19%) and extra-adrenal in 39 (14%). Overall sensitivity of the studies was 89% for CT, 98% for MRI, and 81% for (131)I-MIBG. In unilateral adrenal lesions sensitivity were 100% for CT and MRI, and 88% for (131)I-MIBG; in bilateral lesions 66% for CT, 100% for MRI, and 62% for (131)I-MIBG; in extra-adrenal lesions 64% for CT, 88% for MRI, and 64% for (131)I-MIBG. CONCLUSION: The accuracy with which pheochromocytomas and paragangliomas can be visualized questions nowadays the routine use of abdominal approach. In selected cases of sporadic unilateral chromaffin tumours, a posterior, lateral, or even laparoscopic approach should be considered.

Abdominal Neoplasms↗

PubMed2005

In vivo detection of encapsulated intracardiac paraganglioma by delayed gadolinium enhancement magnetic resonance imaging.

Intracardiac paragangliomas are rare endocrine tumors that are usually benign and can be cured by surgical resection. Both invasive and encapsulated forms have been described and degree of invasion determines surgical complexity. We present in vivo detection of fibrotic encapsulation in a cardiac paraganglioma using delayed enhancement (DE) cardiac magnetic resonance imaging (CMRI), later confirmed on pathology. This finding improved presurgical risk assessment and helped guide management. Tumor necrosis was also easily identified. DE appears useful in the assessment of intracardiac tumor invasion.

Contrast Media↗

PubMed1998

Founder effect at PGL1 in hereditary head and neck paraganglioma families from the Netherlands.

PGL1, a gene responsible for hereditary paragangliomas of the head and neck, recently was mapped to a 2-cM interval on chromosome 11q22-q23, by linkage and haplotype-sharing analysis of a large multibranch Dutch family. We determined the disease-linked haplotype, as defined by 13 markers encompassing a large interval on 11q21-q23, in 10 additional families ascertained from the same geographical locale. Alleles were identical for six contiguous markers, spanning a genetic distance of 6 cM and containing PGL1. Despite this strong indication of a common ancestor, no kinships between the families could be demonstrated through genealogical surveys going back to 1800 a.d. We conclude that a single ancestral mutation is responsible for most, if not all, hereditary paragangliomas, in this region of The Netherlands, and that strong founder effects may exist at the PGL1 locus.

Chromosome Mapping↗

PubMed2005

Paraganglioma-like medullary thyroid carcinoma: a rare entity.

OBJECTIVE: The wide variety of rare histologic variants of medullary thyroid carcinoma (MTC) may make the differential diagnosis difficult. Pathologic examination of the resected specimen will not always be able to confirm the diagnosis, leaving the surgeon with an uncertainty as to what type of resection is best for the patient. The following report describes the case of a 58-year-old man with the rare diagnosis of paraganglioma-like MTC. METHODS: The patient presented with markedly elevated calcitonin (CT) and carcinoembryonic antigen (CEA) levels. A 1-cm tumor was detected in the right lobe of the thyroid. Based on the clinical diagnosis of MTC, we performed a total thyroidectomy with lymphadenectomy of the central and right lateral compartments. Paraffin sections of the resected specimen were stained with hematoxylin and eosin (H&E) and immunohistochemically characterized using antibodies to CT, CEA, chromogranin A, thyroglobulin, synaptophysin, sustentacular cells (SCs), low- and high-molecular cytokeratins (CK 5/6, 7, 18, 20), epidermal growth factor-receptor (EGFR), thyroid transcription factor-1 (TTF-1), bcl-2, Melan A, C-kit, neuron-specific enolase (NSE), and galectin-3. The patient's blood and tumor tissue were examined for mutations in the RET-protooncogene. RESULTS: H&E staining of both frozen and permanent sections was unable to differentiate benign from malignant tissue. Typical morphologic characteristics for MTC were completely absent. Only the additional finding of positivity for synaptophysin and numerous SC cells visible in-between neoplastic cells made the diagnosis of paraganglioma-like MTC possible. Sequencing of the RET proto-oncogene revealed no mutations. CONCLUSIONS: There are subgroups of MTC that present clinically similar to classic MTC, but in which missing typical morphologic characteristics make histopathology diagnosis difficult. In these cases, diagnosis, operative decisions, and follow-up strategies should be based on preoperative biochemical markers, imaging findings, and clinical parameters in accordance to the guidelines for classic MTC.

Carcinoma, Medullary↗

PubMed1982

Multiple endocrine neoplasia. Pituitary adenoma, multicentric papillary thyroid carcinoma, bilateral carotid body paraganglioma, parathyroid hyperplasia, gastric leiomyoma, and systemic amyloidosis.

Multiple endocrine neoplasia in a 70-year-old woman are described. The findings include a nonfunctioning pituitary adenoma, a multicentric papillary thyroid carcinoma, bilateral carotid body paragangliomas, parathyroid hyperplasia, gastric leiomyoma and systemic amyloidosis. A study of the kindred revealed that two family members, a daughter and a granddaughter, have clinical and radiographic evidence of pituitary tumors and bilateral carotid body paragangliomas, suggesting that this may represent a genetically determined syndrome inherited as an autosomal dominant.

Adenoma↗

PubMed1986

Duodenal gangliocytic paraganglioma. Clinicopathologic and immunocytochemical study of 11 cases.

The clinicopathologic features of 11 cases (8 in men) of duodenal gangliocytic paraganglioma are presented. The patients averaged 56 years of age; none showed evidence of phakomatosis. Ten tumors occurred in the second portion of the duodenum, and one arose in the third portion. All tumors were polypoid, and half presented with gastrointestinal bleeding. The neoplasms were composed of paraganglioma and carcinoid-like elements, neurons, and Schwann as well as sustentacular cells. All tumors behaved in a benign fashion after local resection or snare polypectomy; long-term follow-up (1-25 years; mean, 8.3 years) showed no recurrence in any case. Immunocytochemical examination demonstrated the presence of somatostatin, serotonin, and human pancreatic polypeptide within endocrine cells and neurons.

Aged↗

PubMedDate not supplied

Cushing's syndrome due to ectopic ACTH production by a nasal paraganglioma.

A patient is described with Cushingoid features and a tumor of the paranasal sinuses. Laboratory studies revealed hyperglycemia, hypokalemia, alkalosis, hypercortisolemia, and elevated serum ACTH levels. The cortisol level increased briskly in response to parenteral ACTH, but was only partially suppressed by dexamethasone. Microscopically, the tumor was found to be a paraganglioma containing many neurosecretory granules.. Treatment with methyrapone, o,p'DDD, and irradiation of the tumor resulted in a lasting remission. It is concluded that this unique patient suffered from Cushing's syndrome due to ectopic ACTH production by a paraganglioma of the paranasal sinuses.

ACTH Syndrome, Ectopic↗

PubMed2002

Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma: an immunohistochemical and in situ hybridization study.

We describe a case of primary nonfunctioning paraganglioma that, unlike any other previously reported case, was strictly confined to the liver and must therefore have arisen on liver parenchyma. An asymptomatic 46-year-old man was referred to us for laparotomy and a right hemihepatectomy after a preoperative diagnosis of fibrolamellar hepatocellular carcinoma, based on a fine-needle biopsy. An 8-cm resiliently firm, pale gray nodule with a large central area of fibrosis and a thin fibrous capsule was resected. The polygonal eosinophilic tumor cells containing round nuclei lacking nucleoli were arranged in small nests set in a vascularly rich stroma. At immunohistochemistry neoplastic cells were strongly positive for chromogranin A, neuron-specific enolase, synaptophysin, and IGF-II protein; they were negative for keratin, S-100 protein, CD10, vimentin, and smooth muscle actin. In situ hybridization confirmed that, as in other sites, liver paraganglioma can express IGF-II gene. Conversely (and unlike hepatocellular carcinomas), the neoplastic cells did not express albumin mRNA, which was detected only in surrounding hepatocytes. The clinical course was benign and the patient is well and free of neoplastic disease 9 years after surgery. Knowledge of the entity should avoid possible confusion with hepatocellular carcinoma, especially of the fibrolamellar variety.

Carcinoma, Hepatocellular↗

PubMed1987

Gastric autonomic nerve (GAN) tumor and extra-adrenal paraganglioma in Carney's triad. A common origin.

Carney's Triad comprises a triad of neoplasms: gastric stromal tumor, extra-adrenal paraganglioma (usually functional), and pulmonary chondroma. At least two of these are needed for the presumptive diagnosis of the Triad. This report presents a patient who had resected a gastric tumor and nonfunctional extra-adrenal paraganglioma. The gastric tumor resembled a gastric leiomyosarcoma by light microscopy, but electron microscopy revealed it to be a gastric autonomic nerve (GAN) tumor. Based on this evidence it appears that both the gastric lesions and the paragangliomata of Carney's Triad are tumors of the autonomic nervous system. Thus, the Triad may be a disorder of the autonomic nervous system rather than a multiple endocrine neoplasia syndrome or multiple hamartoma syndrome.

Adolescent↗

PubMed1992

Recent advances in parathyroid tumors and paragangliomas.

The management of parathyroid tumors has advanced mainly in the area of imaging with ultrasonography and nuclear scanning. Surgery remains the preferred treatment for parathyroid tumors and paragangliomas. Information about genetic aspects of parathyroid tumors has increased, but the rarity of parathyroid tumors and paragangliomas has limited the pace of advancement in knowledge about their biology and clinical aspects.

Humans↗

PubMed2000

Paraganglioma of the temporal bone: role of magnetic resonance imaging versus computed tomography.

Paragangliomas, also known as glomus tumors or chemodectomas, are tumors arising from chemoreceptor tissue (paraganglia), which are neural crest in origin and found in higher concentration along the glossopharyngeal and vagal cranial nerve. Three types of paragangliomas are related with the temporal bone: glomus tympanicum, glomus jugulare, and glomus vagale. The role of computed tomography (CT) and magnetic resonance imaging (MRI) in diagnosing these types of tumors is discussed, along with the importance of arteriography in aiding the diagnosis and treatment. This article discusses the choice between CT and MR based on clinical symptoms and tumor location, and illustrates the newest CT, MR, and angiography applications. A brief discussion on treatment options is given.

Angiography↗

PubMed1991

Paraganglioma of the vulva.

A case of vulvar paraganglioma is reported. Following presentation with vulvar pain, a 1-cm nodule was excised from the labium minus of a 58-year-old woman. Histologically, the tumor was composed of nests of round eosinophilic cells with moderately pleomorphic nuclei, beneath an intact squamous epithelium. Ultrastructural studies indicated two cell types within the neoplasm: chief cells with numerous small neurosecretory granules and peripheral slender sustentacular cells. A reticulin stain confirmed the "zellballen" nature of the neoplasm, and the neoplastic cells showed moderate argyrophilia on a Grimelius stain. The immunoperoxidase stains for chromogranin and neuron-specific enolase were strongly positive in the neoplastic chief cells. Immunostaining using anti-S-100 antibody confirmed the finding of sustentacular cells by identifying many slender cellular processes among the chief cells. These light and electron microscopic findings are diagnostic of paraganglioma, an entity not previously reported in the vulva to our knowledge.

Cell Nucleus↗

PubMed1991

Spinal cord compression from a thoracic paraganglioma: case report.

A 34-year-old man with a 4-month history of midthoracic back pain sought treatment for a recent onset of lower extremity paresthesia and stiffness. A myelogram and computed tomographic myelogram disclosed an extradural block at the level of the 8th thoracic vertebral body with involvement of the pedicles, lamina, and spinous process. A posterior decompression of the spinal cord with subtotal resection of a highly vascular tumor was performed. The tumor was identified as a paraganglioma. In a second stage, the remainder of the tumor was embolized preoperatively, and gross total excision and sequential stabilization of the spine with a Luque rectangle and sublaminar wires were performed. The patient has been symptom free and without signs of a recurrence in the spine for over 13 months. A large abdominal paraganglioma was recently resected from its probable origin from the adventitia of the abdominal aorta.

Abdominal Neoplasms↗

PubMed1989

Pre- and post-treatment distribution pattern of 123I-MIBG in patients with phaeochromocytomas and paragangliomas.

123I-metaiodobenzylguanidine (MIBG) localizes in adrenergic neurones. A comparison of % washout of MIBG, during two time intervals, from various organs was carried out in seven normal volunteers and 11 patients with phaeochromocytoma and/or paraganglioma pre- and post-treatment using MIBG. % washout of MIBG during the time intervals of 30 min to 4 h were as follows: heart, controls 26.1 +/- 3.8 v. patients 51.5 +/- 9.1, (p less than 0.001), lungs, controls 36.5 +/- 5.3 v. patients 47.3 +/- 5.8, (p less than 0.05), liver, controls 26.5 +/- 5.7 v. patients 53.4 +/- 12.0 (p less than 0.01). Following treatment the % washout of MIBG from these tissues between 30 min and 4 h were similar in both groups of subjects. This observation coincided with the normalization of plasma catecholamine levels in most of the treatment group. There was no significant difference between the treatment and control groups, in the % washout of MIBG from these tissues during the 4 h and 22 h time intervals either before or after treatment. We conclude that quantitative scintigraphy 30 min to 4 h after MIBG administration is a useful technique to evaluate the pathophysiological effects of high circulating catecholamines in patients with phaeochromocytomas and/or paragangliomas.

3-Iodobenzylguanidine↗