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PubMed2004

Management of jugular paragangliomas: the Gruppo Otologico experience.

OBJECTIVE: The objective of this study was to review the outcome of surgical management in patients of jugular paragangliomas. STUDY DESIGN: We conducted a retrospective case review. SETTING: Tertiary care otology and skull base center. MATERIALS AND METHODS: Fifty-five patients with the diagnosis of a jugular paraganglioma (Fisch Class C and D Glomus Jugulare) were managed over a period of 15 years. All patients with adequate follow up and complete records (53 cases) were reviewed with emphasis on the results of surgical management and the factors influencing them. INTERVENTION: All 53 patients were managed with a view to surgically extirpate the tumor. The primary approach was the infratemporal fossa approach-Type A used in the majority of the patients. In eight cases, the procedure was staged owing to the presence of large intracranial extension. Three patients required additional procedures to ameliorate the after-effects of lower cranial nerve resection. RESULTS: Gross total tumor removal was achieved in 49 patients. There were five cases of recurrence. Coupled with the residual tumors in five patients, the surgical control achieved was 83%. There was no perioperative mortality. There were two cases of postoperative cerebrospinal fluid leak, both of which required surgical exploration and closure. The facial nerve was resected in seven patients. The overall preservation rate of clinically uninvolved lower cranial nerves was 75%. CONCLUSIONS: The low level of complications along with a high surgical control achieved makes surgery the primary mode of treatment in the vast majority of these tumors, regardless of the size and location.

Adolescent↗
PubMed2006

Lateral thyrotomy for excision of laryngeal paragangliomas.

Laryngeal paragangliomas, although rare, are lesions that warrant appropriate diagnosis and treatment secondary to their location and high risk of bleeding when violated. This article describes a method to workup patients with solid submucosal lesions of the larynx to diagnose a paraganglioma without a biopsy. When recognized preoperatively, a lateral approach to removal can be performed, decreasing the risk of significant bleeding and the need for tracheotomy or permanent laryngostoma. This article also adds two more cases to the reported literature of 75.

Aged↗
PubMed2005

Current trends in the diagnosis and management of head and neck paragangliomas.

PURPOSE OF REVIEW: The purpose of this review is to provide an overview of the diagnosis and management of head and neck paragangliomas. RECENT FINDINGS: The review will focus on three main areas of interest: genetics, newer imaging modalities, and management with radiation therapy. SUMMARY: The succinate dehydrogenase enzyme complex is important in the pathogenesis of paragangliomas. Magnetic resonance angiography and functional imaging have become very helpful in diagnosing these tumors. Surgery is still the mainstay of therapy, but conventional or stereotactic radiotherapy seems to be a very safe option in very large or inoperable tumors.

Carotid Body Tumor↗
PubMed2006

Sclerosing paraganglioma: report of 19 cases of an unusual variant of neuroendocrine tumor that may be mistaken for an aggressive malignant neoplasm.

Nineteen cases of a distinctive variant of paraganglioma characterized by extensive collagen deposition resulting in a pattern of growth that resembled an invasive malignant neoplasm are described. The patients were 3 men and 16 women, 32 to 69 years of age (mean, 50.5 years). The tumors were located in the carotid body region, parapharyngeal region, and mediastinum. Tumor size ranged from 2 to 6 cm in greatest diameter. Grossly, the tumors were described as rubbery to firm, tan-red, and with extensive areas of sclerosis. Histologic examination showed nests and cords of tumor cells separated by broad bands of fibrous tissue. The tumor cells ranged from round to polygonal with abundant cytoplasm to elongated spindle cells with scant cytoplasm. Nuclear cytomegaly was present focally enhancing the atypical appearance of the tumor cell population in 17 cases. Mitoses were sparse (<1 x 10 HPF), and there was no evidence of necrosis in any of the cases. Foci of vascular and perineural invasion were present in 2 and 4 cases, respectively. The most striking morphologic feature was the presence of irregular cords and bands of hyalinized fibrous tissue that compartmentalized the lesion into irregular nests, islands, or cords of tumor cells, imparting them with an infiltrative appearance. All the tumors showed positive immunostaining for chromogranin, synaptophysin, and monoclonal neuron specific enolase. S-100 protein stains identified a sustentacular cell network, whereas cytokeratin AE1/AE3 was negative in all cases. Clinical follow-up in 14 cases, ranging from 2 months to 20 years (mean follow-up, 6.6 years) showed evidence of local recurrence in 2 cases and the development of a separate tumor in the contralateral neck in 1 case. The remainder of patients were free of recurrence or metastasis following simple local excision. Because of the prominent sclerosis, a diagnosis of an invasive malignant neoplasm was initially considered in the majority of cases. Sclerosing paraganglioma should be included in the differential diagnosis of sclerosing lesions of the head and neck region and mediastinum. Appropriate immunohistochemical stains may be of aid for establishing the correct diagnosis.

Adult↗
PubMed2006

Fluorine-18 DOPA PET imaging of paraganglioma syndrome.

A 12-year-old boy was admitted after recently having had a resection performed of an extraadrenal retroperitoneal paraganglioma and left adrenalectomy for pheochromocytoma. Despite these procedures, the blood pressure and urinary noradrenalin were elevated. Screening with fluorine-18 DOPA PET demonstrated increased tracer uptake in the right adrenal gland, in a second abdominal lesion, which was prevertebral, and 2 cervical hot spots near the carotid bifurcation, one on each side of the neck. The patient carries a mutation of the gene Succinate dehydrogenasis subunits D (SDHD) and is thus classified with the paraganglioma syndrome type 1.

Abdominal Neoplasms↗
PubMed2006

Laparoscopic resection of urinary bladder paraganglioma: a case report.

The case report of a 55-year-old woman with an incidentally diagnosed urinary paraganglioma of the bladder is presented. The patient had no irritative voiding symptoms, hematuria or hypertension. She was diagnosed to have an immobile solid mass on the left side neighboring the lower segment of the uterus. Transvaginal ultrasonography revealed a well-limited ovoid mass with solid and cystic areas adjacent to the urinary bladder and the uterus. Tumor markers were within normal range. Laparoscopy was performed for the treatment of the mass and complete excision of the cyst was accomplished. Frozen section was performed revealing a benign cystic structure but the identification of the origin was left to definitive histopathological examination which showed paraganglioma of the bladder. Immunohistochemically, the tumor cells were strongly positive for chromogranin A and synaptophysin and there was focal positiveness for neuron specific enolase although vimentin and cytokeratin were negative.

Female↗
PubMed2005

The effect of calcium channel blockers on outcome following the surgical treatment of phaeochromocytomas and paragangliomas.

The peri-operative management of patients undergoing surgery for phaeochromocytoma or paraganglioma with calcium channel blockers (CCB) and their impact on postoperative morbidity and mortality were studied. The medical records of 105 patients undergoing surgery between 1991 and 2002 were analysed retrospectively. In all patients, the calcium channel blocker nicardipine was used for the peri-operative management of haemodynamic changes. Sixty-five patients (61.9%) showed transient intra-operative hypertension. Systolic blood pressure (SBP) > 220 mmHg and SBP > 180 mmHg for > 10 consecutive minutes was observed in 14 (13%) and four patients (2.8%), respectively. SBP < 80 mmHg for > 10 consecutive minutes occurred in 13 patients (12.3%). Eleven patients (10.4%) developed postoperative complications and three patients died (2.8%). The median (range) ICU and hospital length of stay were, respectively, 1 (0-7) day and 10 (2-35) days. The sole use of calcium channel blockers for the peri-operative management of phaeochromocytoma and paraganglioma resection does not prevent all haemodynamic changes; however, its use was associated with a low morbidity and mortality.

Adolescent↗
PubMed2004

K40E: a novel succinate dehydrogenase (SDH)B mutation causing familial phaeochromocytoma and paraganglioma.

OBJECTIVE: Germline mutations in succinate dehydrogenase (SDH)B, SDHC and SDHD, encoding three of the four subunits of mitochondrial complex II, have been implicated in the tumourigenesis of familial paragangliomas and phaeochromocytomas. Twenty-three SDHB mutations have been identified to date. PATIENTS: We present a novel missense SDHB exon 2 mutation (c.118 A > G; K40E) identified in an Australian family. The proband was diagnosed with phaeochromocytoma at an early age following an unexpected hypertensive crisis and was found to be SDHB mutation-positive. Subsequent genetic screening of 26 family members has identified 17 mutation-positive relatives. In addition to the proband, four mutation positive relatives were found to have clinical symptoms or a lesion and/or catecholamine excess after the identification of the mutation led to further evaluation. Both the proband and an uncle have required surgical removal of a tumour. CONCLUSIONS: This family indicates the importance of germline screening of first-degree relatives when a patient presents with an apparently sporadic extra adrenal phaeochromocytoma at a young age or whenever a patient with a nonsecretory paraganglioma is found.

Adrenal Gland Neoplasms↗
PubMed1997

Fine needle aspiration (FNA) cytology of extra-adrenal paragangliomas.

We studied nine histologically proved cases of extra-adrenal paragangliomas. Of these, eight cases were correctly diagnosed preoperatively by FNA cytology. Salient cytological features included clusters and discretely scattered cells, with occasional acinar configuration. The cells showed moderate to abundant slightly basophilic cytoplasm with ill-defined margins and round to oval nuclei with mild to marked nuclear pleomorphism. Fine reddish intracytoplasmic granules were also noted (six cases). Cytological features along with clinical data are quite diagnostic of paragangliomas. A correct preoperative cytological diagnosis helps to prevent undue surgical complications.

Adult↗
PubMed1986

Carney's triad: gastric leiomyosarcoma, pulmonary chondroma and extra-adrenal paraganglioma in young females.

The case of a 13-year-old girl with a gastric leiomyosarcoma and a pulmonary osteochondroma is presented. The association of these two tumours and extra-adrenal paraganglioma has been described as a triad by Carney. The patient is free of recurrence of the gastric tumour with no evidence of paraganglioma 10 months after the operation. To our knowledge this is the first case of the triad reported in the UK.

Adolescent↗
PubMed1986

Alveolar soft part sarcoma, granular cell tumor, and paraganglioma. An immunohistochemical comparative study.

Five cases of alveolar soft part sarcoma, 5 cases of granular cell tumor, and 6 cases of paraganglioma were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, desmin, and glial fibrillary acidic protein (GFAP], actin, myoglobin, and nervous tissue markers (S-100 protein, neuron-specific enolase, and Leu-7). In alveolar soft part sarcomas, some of the tumor cells were positive for desmin, but negative for nervous tissue markers. The tumor cells of granular cell tumors were stained with anti-S-100 protein antibody, but not with anti-neuron-specific enolase antibody. In contrast, the tumor cells of paragangliomas were positive for neuron-specific enolase, but not for S-100 protein except for stellate cells surrounding the tumor cell nests. This immunohistochemical approach was valuable for the differential diagnosis of these three tumors. Furthermore, the complete absence of cytokeratin in all of the tumor cells may be helpful in distinguishing these three tumors from metastatic carcinoma in soft tissue. The histogenesis of alveolar soft part sarcoma is a matter of controversy. The result that besides desmin actin was also demonstrated in some of the tumor cells may support the myogenic origin of this tumor.

Adult↗
PubMed1982

Paraganglioma of the falciform ligament: a case report.

A single case of an extra-adrenal paraganglioma removed at laparotomy from the falciform ligament of a 16 year-old girl is reported. Whilst extra-adrenal paragangliomata have been frequently documented in specific sites in relation to the vascular axis, the occurrence of these tumours in other areas is rare. This is the first report of a paraganglioma occurring within the falciform ligament in the midline in apposition to the anterior abdominal wall.

Adolescent↗
PubMed1993

Paraganglioma of the urinary bladder: an immunohistochemical study and report of an unusual association with intestinal carcinoid.

Two cases of paraganglioma of the urinary bladder are reported. Their immunohistochemical profiles and the clinical features are compared with other cases in the literature. The three pan-endocrine markers (neuron-specific enolase, synaptophysin and chromogrannin) were positive in both cases. Positivity to other neuropeptides (including the present two cases and those in literature) includes adrenocorticotropic hormone (three out of five cases), calcitonin (two out of nine cases), gastrin (two out of six cases), glial fibrillary acidic protein (one out of five cases), glucagon (two out of six cases), serotonin (five out of nine cases), and somatostatin (four out of eight cases). A previously unmentioned association between paraganglioma of the urinary bladder and carcinoid in the gastrointestinal tract is noted in one of the present cases. This peculiar association highlights the importance of multiplicity of tumours of the neuroendocrine system other than the classical multiple endocrine neoplasia syndromes.

Adult↗
PubMed1994

An intraocular paraganglioma?

A tumour of the iris with the morphological and immunohistochemical characteristics of a paraganglioma is presented. Neither this type of tumour, nor the specialized nerve cells giving rise to the tumour, have previously been reported in the eye. A successful local resection was performed. The diagnosis and concept of paragangliomas are discussed briefly.

Adult↗
PubMed1992

Orbital paraganglioma: case report and review of the literature.

Paragangliomas of the orbit are extremely rare. A case of an orbital paraganglioma, including the first magnetic resonance imaging description of this tumour is described here. The patient underwent surgery with gross total removal of the tumour and relief of his initial chief complaint of visual blurring. The differential diagnosis and therapeutic options for the management of this tumour are discussed.

Humans↗
PubMed1995

Gastrointestinal haemorrhage from a jejunal gangliocytic paraganglioma.

A case of jejunal gangliocytic paraganglioma is reported in a 54 year old woman who presented with brisk melaena. The tumour was not encapsulated, involved the mucosa and submucosa, and was composed of epithelial nests, spindle cells, and ganglion cells. These cells were mixed, giving carcinoid-like, paraganglioma-like, and ganglioneuroma-like patterns in different areas of the tumour. The lesion was excised locally and recovery was uneventful. Only four previous cases have been reported at this site.

Female↗
PubMed1987

Pheochromocytoma and paraganglioma: comparison of MR imaging with CT and I-131 MIBG scintigraphy.

To ascertain the magnetic resonance (MR) imaging characteristics of pheochromocytomas and paragangliomas and to compare MR with computed tomography (CT) and iodine-131 metaiodobenzylguanidine (I-131 MIBG), 19 patients (18 with pheochromocytomas, one with a paraganglioma) were studied. The 18 patients with pheochromocytomas had had positive findings with I-131 MIBG scintigraphy. Abdominal pheochromocytomas were generally hypointense compared with normal liver on T1-weighted MR images and extremely hyperintense on T2-weighted MR images. MR imaging was preferable to CT in the evaluation of primary pheochromocytomas due to superior tissue characterization, particularly in the patient with hypertension and borderline catecholamine levels. For patients with recurrent or metastatic disease, the data suggest that I-131 MIBG scintigraphy is the examination of choice.

3-Iodobenzylguanidine↗