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PubMed2006

[Malignant hereditary paraganglioma: problems raised by non-functional forms management].

Non-functional paraganglioma have not clinical or biological characteristics, so that the diagnostic is most of the time delayed and made on the occasion of advanced abdominal tumor or symptomatic metastasis management. Hereditary forms, notably those with SDHB mutation, seem to have a poor prognosis. On the other hand, and on the oposite to sporadic forms, they are the only ones to benefit from genetic testing which make possible, if positive, an earlier diagnostic, before apparition of symptoms, recurrence or metastasis. We report a case of non-functional malignant hereditary paraganglioma diagnosed belatedly and we will consider management problems raised by non-functional forms.

Abdominal Neoplasms↗

PubMed2006

Paraganglioma presenting as a vascular malformation: case report.

Paragangliomas are rare tumours that arise from extra-adrenal chromaffin cells or neuroectodermal chemoreceptor. They are high vascularised and can be functional, secreting catecholamines and other peptides. They occur anywhere from the skull base to the pelvic floor, within the paraganglion system. We present a case of paraganglioma with unusual location, atypical presentation and interesting vascular anatomy.

Adult↗

PubMed2005

A cardiac paraganglioma presenting with atypical chest pain.

Primary cardiac tumours are rare. The majority are benign and 75% are atrial myxomas. One of the more unusual benign tumours affecting the heart is a cardiac paraganglioma. A 56-year male was presented with a 6-month history of vague, left-sided chest pain, intermittent parathesia of the left arm and dyspnoea on bending. Echocardiography documented a large, highly vascular mass, attached to the intra-atrial septum. All investigations, haematological, biochemical, neuroendocrine tumour markers and urinary cathecholamine levels, were within normal limits. Macroscopically the tumour involved the whole of the atrial septum, roof of the left atrium and extended to surround the superior vena cava, excluding total resection. The feeding vessels were ligated in the hope of infarcting the remainder of the tumour. In over 2-years of follow-up, the patient remains clinically stable. To our knowledge this is the first reported case where vascular ligation has been used to control a cardiac paraganglioma.

Chest Pain↗

PubMedDate not supplied

Retroperitoneal paraganglioma: single-institution experience and review of the literature.

Paragangliomas are rare tumors arising from extra-adrenal chromaffin cells. We examined the clinical characteristics of all patients at our institution having paragangliomas resected from 1984 through 2005. Of 253 resections, 22 (9%) were retroperitoneal and were selected for further study. The ratio of males to females was 1.3:1, and the median age was 39 years. The average size, rate of metastasis (i.e., malignancy), and rate of function was 7.4 cm, 9.5%, and 57.1%, respectively. Tumors larger than 7 cm were more likely to require adjacent organ resection (P = 0.01). The overall 5-year survival was 73%. Survival was significantly worse after metastasis (P = 0.0023) but did not depend on the tumor diameter, the secreting function of the tumor, the status of surgical margins of resection, or status of the resected lymph nodes.

Digestive System Surgical Procedures↗

PubMed2006

Primary cutaneous paraganglioma of the scalp.

Paragangliomas are neuroendocrine tumors that arise in relation to the sympathetic or parasympathetic ganglia of the autonomic nervous system. Rarely, they are reported in unusual locations such as the urinary bladder, prostate, and uterus. Herein, we report a case of primary cutaneous paraganglioma of the scalp in a child. The tumor was studied histologically, immunohistochemically, and ultrastructurally. This case might represent an aberrant migration of neural crest cells in their descent toward the branchial arches. In addition, it highlights the complex interaction of the neural crest and branchial arches/clefts during embryogenesis.

Child↗

PubMed2005

Paraganglioma of the filum terminale.

Paraganglioma of the filum terminale is rare. The authors report a case of paraganglioma of the filum terminale presenting with paraparesis on a background of spastic quadriparesis. The clinical presentation, radiological findings and current literature are presented.

Biomarkers, Tumor↗

PubMed2005

Paraganglioma of the prostate in a young adult.

Extraadrenal pheochromocytomas, or paragangliomas, are rare and potentially malignant tumors that may occur in extraadrenal chromaffin tissue. The determination of malignant or benign represents the crucial point once the diagnosis has been established. We report a case of a 33-year-old man with an incidental paraganglioma involving the prostate. He underwent nerve-sparing radical retropubic prostatectomy and monolateral lymphadenectomy. Urinary continence was complete on postoperative day 5, and the patient had intercourse 2 weeks after surgery. The follow-up was at 24 months with no evidence of recurrent disease on clinical examination or body scan evaluation.

Adult↗

PubMed2003

[Gangliocytic paraganglioma of the pancreas with bone metastasis].

We report the case of a 50-years-old man who underwent sequential curative resection for gangliocytic paraganglioma of the pancreas with metachronous sternal metastasis. Gangliocytic paraganglioma are mainly located on the duodenum but several localizations have been reported, including on the digestive tract. Locoregional lymph node metastases are possible and prove their malignant potential but, to our knowledge, this observation is the first documented case of distant metastasis. Their usually good prognosis supports complete resection of these tumors.

Bone Neoplasms↗

PubMed2005

Orbital paraganglioma: report of a rare case.

CASE REPORT: Paragangliomas of the orbit are extremely rare tumors. We report a well-documented case of orbital paraganglioma in a 45-year-old man who presented with proptosis and decreased vision. The patient underwent complete excision of the tumor with preservation of orbital structures. There was total regression of symptoms after surgery and vision was well maintained. COMMENTS: Radiological and pathological features are described and differential diagnoses of this entity are discussed.

Biomarkers, Tumor↗

PubMed1985

A case of nonfunctioning paraganglioma arising from the retroperitoneum: angiographic and scintigraphic features.

We report a case of a nonfunctioning nonchromaffin paraganglioma arising from organ of Zuckerkandl. Angiography manifested a cartwheel-shaped image in the arterial phase and a characteristic tortoise shell-shaped lobular structure with homogeneous opacification in the venous phase. Just after administration of 99mtechnetium-E-HIDA scintigraphy disclosed a globular hot density below the left kidney. These findings may represent characteristic signs of paragangliomas.

Adult↗

PubMed2001

Head and neck paragangliomas: physiology and biochemistry.

Paragangliomas of the head and neck are derivatives of neural crest cells, comprising part of the diffuse neuroendocrine system. Indeed, paragangliomas encompass a unique subset of tumors of the head and neck. Their biochemistry and physiology are similar to other neuroendocrine tumors unlike tumors based on location. This article discusses their distinct biologic attributes.

Biochemical Phenomena↗

PubMed2001

Paragangliomas: classification, pathology, and differential diagnosis.

This article discusses the paraganglion system and extra-adrenal paragangliomas. In particular, the clinicopathologic, immunohistochemical, and ultrastructural features of paragangliomas and neuroendocrine neoplasms of the larynx are presented with a discussion of the differential diagnosis.

Biopsy, Needle↗

PubMed2001

Treatment of paragangliomas with radiation therapy.

Paragangliomas of the head and neck may be treated successfully with surgery, radiation therapy, or stereotactic radiosurgery. The choice of treatment depends on the location and extent of the tumor, the presence of multiple tumors, the age and health of the patient, and the preference of the patient and attending physician. This article reviews the role of radiation therapy in the treatment of patients with paragangliomas of the head and neck.

Female↗

PubMed2003

Iodine -131 metaiodobenzylguanidine is an effective treatment for malignant pheochromocytoma and paraganglioma.

INTRODUCTION: Iodine 131-meta-iodobenzylguanidine ((131)I-MIBG) has been applied to the palliative treatment of metastatic pheochromocytoma in small studies. We report our institutional experience for the treatment of metastatic pheochromocytoma and paraganglioma. METHODS: We performed a retrospective review of 33 patients with metastatic pheochromocytoma (n=22) and paraganglioma (n=11) treated at our institution with (131)I-MIBG over a 10-year period. RESULTS: Patients received a mean dose of 388+/-131 mCi (131)I-MIBG. Median survival after treatment was 4.7 years. Most patients experienced a symptomatic response leading to an improved survival (4.7 years vs 1.8 years, P<.01). Patients with a measurable hormone response demonstrated an increased survival in comparison to those with no response (4.7 years vs 2.6 years, P=.01). Patients who received a high dose (>500 mCi) as their initial therapy also had improved survival (3.8 years vs 2.8 years, P=.02). CONCLUSION: These data support (131)I-MIBG treatment for select patients with metastatic pheochromocytoma. In our experience, prolonged survival was best predicted by symptomatic and hormone response to (131)I-MIBG treatment. An initial dose of 500 mCi may be optimal. The benefit of (131)I-MIBG treatment for metastatic pheochromocytoma must also be weighed against its side effects.

3-Iodobenzylguanidine↗

PubMed1977

Paragangliomas of the neck: clinical and pathologic analysis of 116 cases.

Neck tumors developing from the paraganglion system are best called paragangliomas. They may be described further as to site of origin and as to whether they are functioning or nonfunctioning. Review was made of 116 cases in which operation for paraganglioma was performed at the Mayo Clinic. The diagnosis depends upon demonstration of a lateral neck mass, elimination of other causes, and a tumor blush on angiography. Screening tests should be done to determine whether the tumor is producing epinephrine. Surgical management should be selective, depending upon the location and involvement of surrounding structures, particularly the carotid artery system. A conservative approach is indicated for those patients whose tumor surrounds the carotid vessels, because the risk of complications is greater than the risk of leaving the tumor in the neck. Conservatism is also indicated for patients in the older age group, because of the slow growth of these tumors and the low incidence of malignancy.

Adolescent↗

PubMed2003

G2M arrest, blocked apoptosis, and low growth fraction may explain indolent behavior of head and neck paragangliomas.

Head and neck paragangliomas are characterized by unusually slow growth and a strong hereditary component, which is associated with inactivating mutations in subunits of complex II of the mitochondrial respiratory chain. It is unclear how mutations induce tumorigenesis and lead to the indolent clinical behavior that often plays a prominent role in treatment strategies. To better understand the natural course of the tumors, we studied a number of growth-related parameters in 42 hereditary and sporadic paragangliomas. Computerized image analysis showed that the fraction of Ki-67-positive cells was generally below 1%, in accordance with the slow growth. Weak or negative immunohistochemical staining indicated wild-type TP53 status, whereas p-21(waf) expression was heterogeneous. Most tumors showed strong expression of Bcl-x(L), and no apoptotic cells could be detected with the terminal deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick-end labeling assay. Flow cytometry showed abnormal DNA content profiles in 52% of the tumors, including overt aneuploidy as well as G(2)/M arrest or tetraploidization. These results fit into a model in which a stress-activated cell cycle checkpoint at the G(2) to M transition and inhibition of apoptosis permit the expansion of only a minor fraction of cycling cells with high likelihood of polyploidization.

Adult↗

PubMed1998

Paragangliomas of the head and neck region show complete loss of heterozygosity at 11q22-q23 in chief cells and the flow-sorted DNA aneuploid fraction.

Nonchromaffin paragangliomas of the head and neck region, also known as glomus tumors, are usually benign neoplasms consisting of clusters of chief cells surrounded by sustentacular cells arranged in so-called 'Zellballen.' Most of the patients have a familial background. In a previous study, examining all chromosome arms, we found loss of heterozygosity (LOH) predominantly at the chromosome 11q22-q23 region, where the disease causing gene PGL1 has been located by linkage analysis. However, all tumors showed only partial loss of allele signal intensities, and it was not clear whether this represented allelic imbalance or cellular heterogeneity. In the current study, we have performed LOH analysis for the 11q22-q23 region on DNA-aneuploid tumor cells, enriched by flow sorting, and on purified chief cell fractions obtained by single-cell microdissection. Complete LOH was found for two markers (D11S560 and CD3D) in the flow-sorted aneuploid fractions, whereas no LOH was found in the diploid fractions of three tumors. The microdissected chief cells from two of these tumors also showed complete LOH for both markers, indicating that the chief cells are clonal proliferated tumor cells. These results indicate that the PGL1 gene is likely to be a tumor suppressor gene, which is inactivated according to the two-hit model of Knudson. Furthermore, it shows that chief cells are a major if not the sole neoplastic component of paragangliomas.

Chromosomes, Human, Pair 11↗

PubMed1989

Orbital nonchromaffin paraganglioma. A case report and review of the literature.

Nonchromaffin paraganglioma (NCP), also called glomus body tumor or chemodectoma, is rarely found in the orbit. The behavior of orbital nonchromaffin paraganglioma may potentially be more aggressive than in other head and neck locations. Diagnosis depends on electron microscopic demonstration of membrane-bound neurosecretory granules. Results of histopathologic study show a well-circumscribed lesion without a true capsule with alveolar or organoid arrangements of epithelioid cells within a reticulin framework with thin-walled blood vessels. Cells are polygonal with round or oval nuclei containing rare mitotic figures and pale-staining cytoplasm. Differential diagnosis includes alveolar soft-part sarcoma, alveolar rhabdomyosarcoma, neuroblastoma, carcinoid, and granular cell tumor. Of 29 previously reported cases of orbital NCP, 16 have been reclassified as alveolar soft-part sarcoma. The authors report a patient with an electron microscopically established orbital NCP, with the history of a contralateral glomus jugulare tumor irradiated 14 years previously.

Adult↗