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PubMed2004

[A case of gangliocytic paraganglioma in duodenum].

Duodenal gangliocytic paraganglioma derived from neural crest is a peculiar neuroendocrine tumor. It is incidentally found during radiographic studies or due to gastrointestinal hemorrhage caused by frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology consisting of endocrine cells, ganglion cells and spindle-shaped Schwann cells. We experienced a duodenal gangliocytic paraganglioma in a 40-year-old woman presenting with episodes of melena. Esophagogastroduodenoscopy revealed a submucosal ulcerated tumor in the second portion of duodenum and a biopsy confirmed gangliocytic paraganglioma. The tumor was enucleated through a duodenotomy. It's size was 5.5 X 3 X 1.5 cm sized and revealed positive cellular reaction for chromogranin, synaptophysin, neuron-specific enolase, and neurofilament by immunohistochemistry. The majority of the reported duodenal gangliocytic paraganglioma were of benign nature. Therefore, radical surgery or a lymph node dissection could be avoided if that disease was confirmed. However, thorough investigation for lymph node metastasis and postoperative follow-up are needed. We report the first case of a duodenal gangliocytic paraganglioma in Korea with a review of literature.

Adult↗

PubMed2004

[A case of vesical paraganglioma with intrapelvic multiplicity].

A 35-year-old woman, who had a past history of hypertension due to paraganglioma of the urinary bladder and in the pelvis, was referred to us 12 years after the initial diagnosis of paraganglioma. Before coming to us and during the follow-up after enucleation of myoma uteri, she was again diagnosed as having a bladder tumor by a gynecologist. Cystoscopy revealed a non-papillary broad-based tumor, measuring 2 cm in diameter, on the trigone of the urinary bladder. Magnetic resonance imaging also showed two solid tumors, each measuring 2 cm in diameter, on the bilateral peri-vesical sides in the pelvis. Endocrinologic examination disclosed increased levels of serum and urinary catecholamines. Histopathologic examination revealed that the bladder tumor specimen obtained by transurethral resection was paraganglioma. She underwent partial cystectomy and resection of these intrapelvic tumors via a retroperitoneal approach. These tumors were histopathologically diagnosed as multiple paragangliomas. She has been followed up for 10 months after operation without any evidence of recurrence. Herein, we report this rare case of vesical paraganglioma and present a brief review of the relevant literature.

Adult↗

PubMed2004

Hypopharyngeal paraganglioma: a case report.

Paragangliomas are neuroendocrine neoplasms that primarily afflict patients during the fourth and fifth decades of life. The majority of extra-adrenal paragangliomas arise in the head and neck region, notably from the carotid and aortic bodies, jugular bulb and tympanic plexus. Although one-fifth of all parapharyngeal neoplasms are paragangliomas, primary hypopharyngeal paraganglioma is relatively uncommon, only one other case having previously been documented by Filippin et al. (1989). We report a second case of hypopharyngeal paraganglioma in an 18-year-old male as seen in our center.

Adolescent↗

PubMedDate not supplied

Active succinate dehydrogenase (SDH) and lack of SDHD mutations in sporadic paragangliomas.

BACKGROUND: Paragangliomas are benign, slow-growing tumours of the head and neck region. The candidate gene for familial and some sporadic paragangliomas, SDHD (succinate dehydrogenase, subunit D), has been mapped to the PGL1 locus in 11q23.3. MATERIALS AND METHODS: Normal and tumour DNA of 17 patients with sporadic paragangliomas were analysed by sequencing (SDHD, SDHB and SDHC genes), fluorescence in situ hybridisation (FISH). In addition, loss of heterozygosity (LOH) and succinate dehydrogenase (SDH) enzyme activity assays were performed. RESULTS AND CONCLUSION: Only two patients from our collective showed SDH gene mutations, one in SDHD and one in SDHB, respectively. Moreover, SDH activity detected in 5/8 patients confirmed the fact that SDH inactivation is not a major event in sporadic paragangliomas. LOH and FISH analysis demonstrated a frequent loss of regions within chromosome 11, indicating that additional genes in 11q may play a role in tumour genesis of sporadic paragangliomas.

Carotid Body Tumor↗

PubMed1991

[Paraganglioma of the neck. Analysis of 32 operated cases].

Between january 1965 and november 1990, 32 operations for neck paraganglioma were performed: 29 chemodectomas (carotid body tumors) and 3 paragangliomas of the vagus nerve. Seven subjects were affected with bilateral chemodectomas and one of them showed concurrent unilateral vagal paraganglioma. Two paragangliomas were malignant, with invasion of the latero-cervical lymphnodes revealed at operation. Four individuals came to observation from two different families, suggesting familiarity. Preoperative diagnosis was correctly made in 12 of 18 asymptomatic chemodectomas (66.6%), ten of whom observed during the last decade: angiography is the gold standard for diagnosis but CT scan, ultrasound and NMR imaging are going to earn the confidence of physicians for precise evaluation of latero-cervical masses. Surgery is to date the treatment of choice, and the results are dependent on the size of the tumor and the involvement of the neighbouring vascular, nervous and visceral structures. According to the majority of the literature, the 29 chemodectomas were classified in the three groups of Shamblin: I: 4 cases; II: 10; III: 15. Twenty out of the 24 transient or permanent postoperative complications took place in the third group: in five instances some procedures of internal carotid artery reconstruction were needed. Fourteen complications for chemodectomas and 2 for vagal paragangliomas affected the cranial nerves; three transient and one permanent ischemic central neurological deficits occurred in the group III chemodectomas. Not any operative mortality was registered in this series.

Adult↗

PubMed1990

[Primary paraganglioma of the urinary bladder: a report of two cases].

We report two cases of primary paraganglioma of the urinary bladder. Case 1. A 61-year-old man was hospitalized with the chief complaints of gross hematuria, dysuria and headache. The patient had a history of hypertension. Cystoscopy disclosed a nonpapillary, sessile tumor in the retrotrigonum of the bladder. An attack of paroxysmal hypertension was induced by bimanual palpation of the tumor, and paraganglioma was suspected. Partial cystectomy with staging pelvic lymphadenectomy was performed. Case 2. A 65-year-old man was hospitalized with the chief complaints of gross hematuria and urinary retention. The patient had no history of hypertension. Cystoscopy disclosed a nonpapillary tumor in the right lateral wall of the bladder. Transurethral resection was performed with no cardiovascular complication. Prostatic needle biopsy showed the histological evidence of prostatic adenocarcinoma. Radical retropubic prostatectomy with limited lymphadenectomy was performed. There was no histological evidence of lymph node involvement of paraganglioma or adenocarcinoma. The histological and biochemical examinations revealed a chromaffin positive, functioning and non-malignant tumor in Case 1, and a chromaffin negative, non-functioning and non-malignant tumor in Case 2. In total, 29 cases of primary paraganglioma of the urinary bladder have been so far reported in Japan. The tumor recurrence, multiple or metastases were recorded in 8 of 29 cases (27.6%), mainly in regional lymph node metastases. We recommend lymphadenectomy at the initial operation, irrespective of pathological finding of the primary paraganglioma of the urinary bladder.

Aged↗

PubMed1985

Duodenal gangliocytic paraganglioma. An immunohistochemical and ultrastructural study and a hypothesis concerning its origin.

We report the immunohistochemical and ultrastructural features of three duodenal gangliocytic paragangliomas and compare them with duodenal carcinoid, extra-adrenal paraganglioma, pheochromocytoma, and ganglioneuroma. The gangliocytic paraganglioma is characterized by polygonal or columnar epithelial cells, ganglion cells, and spindle cells. The epithelial cells stained for neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases; leu-enkephalin, molluskan cardioexcitatory peptide, and vasoactive intestinal peptide in two; and glucagon and insulin in one case each. The ganglion cells were positive for leu-enkephalin, neurofilament, neuron-specific enolase, pancreatic polypeptide, and somatostatin in three cases, and glucagon in one. The spindle cells stained for neurofilament, neuron-specific enolase, and S-100 protein. Although there was some overlap in immunoreactivity between the gangliocytic paraganglioma and the other tumors examined, our data indicate that the gangliocytic paraganglioma is a distinctive lesion. We propose that it is a hyperplastic or neoplastic proliferation of 1) endodermally derived epithelial cells originating from the ventral primordium of the pancreas, 2) neuroectodermal ganglion cells, and 3) neuroectodermal spindle cells (Schwann cells).

Adult↗

PubMed1982

Current management of paragangliomas.

Since 1948, 33 paragangliomas in 32 patients were seen and studied at Charity Hospital. Tumors of the head and neck comprised the bulk of recorded instances, with only five paragangliomas being noted below the diaphragm. The most frequent symptoms correlated closely with tumor location and were related to local mass effect. Angiography remains an important diagnostic study for tumors of the head and neck with computed tomography scanning becoming increasingly important in large paragangliomas of the jugular region. Paragangliomas of the retroperitoneal space can be successfully located by a computed tomography scan, although a new radionuclide scan may prove to be more specific in the future. Tumors of the urinary bladder and retroperitoneal space are commonly endocrine functional and have a greater metastatic potential than do paragangliomas in other locations. Over-all, total excision is possible with complete recovery in most instances. Prolonged follow-up study is recommended, however, as local recurrences and distant metastases may present years after the initial diagnosis.

Adult↗

PubMed1993

[Vagal paraganglioma].

Vagal paragangliomas are rare tumors of neural crest origin. This neoplasm is generally located at various points along the peripheral distribution of the vagus nerve, in the region of the jugular and nodose ganglia, just beneath the perineurium of the nerve, but may arise anywhere along the course of the vagus nerve. Histologically, vagal paragangliomas are similar to carotid body tumors, and occasionally can undergo malignant transformation and metastasize (10-19%) or infiltrate the base of the skull. Selective arteriography can distinguish vagal paraganglioma from carotid body tumor, because the first lies above carotid bifurcation without widening of the vessels. CT scanning and MR are useful for preoperative evaluation of the jugular foramen for neoplastic involvement. Surgical resection is the treatment of choice, but usually it isn't possible to exercise these lesions without sacrifice of the vagus nerve. A careful follow-up examination for the development of metastases and multicentric paragangliomas is necessary. Two cases of vagal paragangliomas one of them with cervical node metastases, are reported. Diagnostic and surgical features of these rare neoplasms are outlined.

Adult↗

PubMed1997

[Malignant paraganglioma of the posterior mediastinum with 27 years follow-up].

INTRODUCTION: A costovertebral paraganglioma with a long history of recurrences and metastases is reported. MATERIAL AND METHODS: The lesion is documented on histological ground and with immunocytochemistry and electron microscopy. RESULTS: The patient is a 42 year old man which presented with a 5 cm axillary mass and enlarged mediastinal lymph nodes. The former lesion has been interpreted as a metastasis of paraganglioma. The patient underwent surgery because of a costovertebral paraganglioma at the age of 19 and showed locoregional metastases and a recurrence over a period of 23 years. Twenty-seven years from removal of the primitive tumor, the patient is alive and well. All the neoplasms showed identical histological features as they were composed of neoplastic elements arranged in nests surrounded by dendritic cells. Neoplastic cells immunoreacted with anti-chromogranin antiserum whereas sustentacolar cells were positive with S-100 antiserum. DISCUSSION: The differential diagnosis between a multicentric paraganglioma a metastasis has been particularly taken into consideration. In addition, axillary tumor was distinguished from a metastatic renal cell carcinoma and melanoma. The literature regarding costovertebral paraganglioma has been also revised.

Adult↗

PubMed1999

Familial carotid body paragangliomas and sensorineural hearing-loss: a new syndrome.

BACKGROUND: Carotid body paragangliomas are rare tumors that are sometimes familial, the transmission of which is thought to be by genomic imprinting. We have treated a family who exhibited co-inheritance of carotid body paraganglioma and sensorineural hearing-loss, a relationship that has not been previously reported. METHODS: We studied a large Australian family who exhibited familial carotid body paragangliomas, many of whose members also suffered tinnitus or hearing-loss. This relationship was examined by reviewing the medical records of family members with confirmed tumors, carrying out neck ultrasonic scanning or computed tomography on their relatives to look for previously unrecognized tumors, and arranging audiometric testing. This information was used to characterize the type of hearing-loss present in this family and to construct a pedigree for the two traits. RESULTS: The hearing-loss observed in this family was sensorineural in character. Of 15 family members studied over four generations, eight were confirmed positive for both carotid body paraganglioma and sensorineural hearing-loss, two for the tumor only (one of whom did not have his hearing assessed) and one for hearing-loss alone. Four family members were negative for both traits. CONCLUSIONS: The pedigree provides evidence of a previously undescribed association between familial carotid body paraganglioma and sensorineural hearing-loss, a combination that appears to be co-inherited in this family.

Adult↗

PubMed1991

Familial multicentric paragangliomas in a child.

A 12-year-old girl presented with bilateral carotid-body paragangliomas and a unilateral jugular paraganglioma. The tumours were surgically removed. This is a rare combination of tumours in any patient and previously unreported in a child of this age. Her father died of a cerebellar astrocytoma and her mother underwent surgical removal of a large mediastinal paraganglioma. The association of astrocytoma with familial paragangliomas has never been documented. The literature on the epidemiology and inheritance pattern of familial paragangliomas is reviewed. The need for thorough pre-operative evaluation of the patient and close follow-up of family members is stressed.

Carotid Body Tumor↗

PubMed2003

Does Catecholamine Secretion from Head and Neck Paragangliomas Respond to Radiotherapy? Case Report and Literature Review.

We report a 38-year-old woman with a malignant catecholamine-secreting vagal paraganglioma. In the preceding year she had intermittent severe frontoparietal headaches. While she was receiving radiotherapy (35 Gy in 15 fractions) she developed palpitations, which steadily worsened over the following three years. A repeat CT scan showed no change in the size of the tumor. Urinary catecholamines were elevated and a MIBG scan showed increased uptake in the region of the vagal paraganglioma. She underwent near total resection of her tumor via a Fisch type C approach. Pathological examination showed a paraganglioma with metastasis involving a cervical lymph node. The palpitations and headaches resolved completely after surgery. This report and other published case reports suggest that catecholamine secretion from head and neck paragangliomas does not appear to respond to radiotherapy. Patients with cardiovascular symptoms from catecholamine-secreting paragangliomas are best managed surgically.

Case Reports↗

PubMed1998

[Jugulotympanic paraganglioma: therapy concepts under development].

BACKGROUND: The operative treatment and radiation therapy of jugulotympanic paragangliomas (JTP) are still a matter of controversial discussion. In spite of various improvements during the last 50 years, selecting the appropriate treatment modality (surgery, radiation, or observation) is still a challenge. PATIENTS: During a 16-year period, 44 patients with 45 JTP (10 at level A/B and 35 at level C/D according to Fisch) were seen at the ENT-department in Fulda. Forty-one cases were treated surgically. RESULTS: Complete resection was possible for level A/B in 100% of the patients (n = 10). Residual tumor was demonstrated for level C in 23% of the patients (5/22) and for level D in 40% (4/10) with a median follow-up time of 69 months. In two cases residual tumor was treated by radiation. Six patients with residual paraganglioma tissue were maintained under observation without any evidence of tumor progression (median follow-up time 39 months). We report one death after the attempt to resect a large residual paraganglioma that had already caused brain stem compression. A sufficient duraplasty could not be achieved following radiation therapy. CONCLUSIONS: Complete tumor resection of jugulotympanic paragangliomas of levels A and B is often possible without injury to the cranial nerves. Extensive tumors present difficulties in complete tumor resection and increase the risk of cranial nerve injuries. Advanced paragangliomas therefore require an individualized therapeutic regime including surgery, radiation therapy, and observation of tumor growth.

Adult↗

PubMed2003

PET scan assessment of chemotherapy response in metastatic paraganglioma.

Paragangliomas are indolent tumors that arise from the chief cells of the paraganglia in the head and neck, mediastinum, and retroperitoneal regions. Less than 10% of paragangliomas metastasize. Paragangliomas are known to regress slowly and usually partially after radiation therapy, which has been attributed to the development of fibrosis within the abundant vascular elements of the tumor. Positron emission tomography (PET) scanning was used to monitor a 33-year-old woman with recurrent paraganglioma of the carotid body with lung and bone metastases before and after chemotherapy with cyclophosphamide, doxorubicin (Adriamycin), and dacarbazine. The patient derived clinical benefit from chemotherapy, with marked improvement of her systemic and respiratory symptoms, improvement of cancer-related anemia, and normalization of chromogranin A levels. A response was demonstrated on PET scan with decreased [18F] fluoro-2-deoxy-d-glucose uptake after chemotherapy, but no significant changes were detected on serial computed tomography (CT) scans. The patient has remained free of disease progression 24 months after chemotherapy completion. It is suggested that metabolic imaging with PET scans is superior to anatomical imaging with CT scans for the monitoring of patients with paragangliomas.

Adult↗

PubMed2005

Vascular endothelial growth factor expression, vascularization and proliferation in paragangliomas.

OBJECTIVE/HYPOTHESIS: Paragangliomas are heavily vascularized tumors, and the expression of VEGF (vascular endothelial growth factor) has been reported. The aim of our study was to extend the available database of VEGF expression in paraganglioma, to add correlated data concerning vessel density and proliferative activity, and to draw conclusions concerning the mechanisms resulting in tumor vascularization and growth. STUDY DESIGN: Semiquantitative histopathologic examination of paraganglioma specimens obtained from surgical cases. METHODS: Paraffin-embedded paragangliomas were analyzed by immunohistochemistry. Fourteen consecutive samples were hybridized with VEGF-, CD31- and Ki67-specific antibodies, and visualized by diaminobenzidine staining. Vessel density was determined by counting CD31-positive vessels and proliferation by quantification of Ki67-positive cells. RESULTS: Ten out of 14 samples were positive for VEGF. In this group, vessel density was up to 5 times as high and proliferative activity was about twice as high as in the VEGF-negative group. CONCLUSIONS: We observed higher CD31 and Ki67 counts in VEGF-positive tumors, but statistical significance could not be assessed due to low sample numbers. These data might suggest a contribution of VEGF secreted by paragangliomas to tumor vascularization and possibly proliferation. The clinical impact of VEGF expression analysis has to be proven in future studies.

Adult↗

PubMed1997

Image cytometric nuclear DNA quantitation of paragangliomas in tissue sections. Prognostic significance.

OBJECTIVE: Image cytometric quantitation of nuclear DNA of paragangliomas may provide prognostic information that cannot be obtained from histopathologic study. Flow cytometry has demonstrated DNA aneuploid tumors to have a higher risk of progression than diploid neoplasms. STUDY DESIGN: DNA ploidy of 56 paragangliomas was assessed by image cytometry of 5-micron, Feulgen-stained, formalin-fixed, paraffin-embedded tissue sections. RESULTS: Thirty-three (59%) paragangliomas were diploid and 23 (41%) aneuploid. Of the 30 adrenal pheochromocytomas, 15 (50%) were diploid. Thirteen (93%) of the 14 carotid body tumors were diploid. Five of seven (71%) glomus jugulare tumors and two of five (40%) extraadrenal paragangliomas were aneuploid. During a mean follow-up of 57 months (range, 1 month to 36 years) of 44 patients with 47 paragangliomas, 33 (75%) were alive and without disease; 7 (16%), including 1 glomus jugulare, 2 carotid body and 4 pheochromocytoma patients, developed recurrences/metastases. By multivariate analysis, image cytometric DNA ploidy was predictive of disease-free survival for adrenal pheochromocytomas. No significant differences in overall survival, disease-free survival or recurrence/metastasis rate were noted between other diploid and aneuploid tumors. CONCLUSION: Aneuploidy suggests a risk of early recurrence for adrenal pheochromocytomas.

Adrenal Gland Neoplasms↗

PubMed1988

Decreased expression of neuropeptides in malignant paragangliomas: an immunohistochemical study.

Paraffin-embedded sections of 99 human adrenal and extraadrenal paragangliomas were analyzed by the indirect immunoperoxidase technique for the presence of neuron-specific enolase (NSE) and 10 neuropeptides. Each showed diffuse staining for NSE. Most tumors were positive for [Leu5]-enkephalin (76 per cent), [Met5]-enkephalin (75 per cent), somatostatin (67 per cent), and pancreatic polypeptide (51 per cent), followed by vasoactive intestinal polypeptide (VIP) (43 per cent), substance P (31 per cent), ACTH (28 per cent), calcitonin (23 per cent), bombesin (15 per cent), and neurotensin (12 per cent). The neuropeptides paralleled to a large extent those normally found in the sympathetic nervous system. Clinically malignant paragangliomas (n = 25) with proven regional or distant metastases expressed considerably fewer neuropeptides, although the spectrum of those seen remained similar. Malignant paragangliomas contained an average of two neuropeptides per tumor, in contrast to five for the benign tumors (P less than 0.05). Logistic regression analysis of staining results revealed that the paucity of enkephalins, somatostatin, pancreatic polypeptide, and VIP along with the patient's sex was predictive of clinical malignancy. Our results show a definite relationship between expression of neuropeptides and the biologic behavior of these paragangliomas.

Adolescent↗