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Paraganglioma of vagal origin.

Vagal paragangliomas are anatomically specific tumors of neuroectodermal origin. The history, embryology, and anatomic classification of these tumors will be reviewed and our present experience with six patients with vagal paragangliomas summarized. All patients had vocal cord dysfunction; various other findings depended upon the extent of the tumor. We found that massive tumors extending rostral to the jugular fossa may be difficult or impossible to distinguish from jugulotympanic paragangliomas.

Adult↗

PubMed2005

Dual CD117 expression in gastrointestinal stromal tumor (GIST) and paraganglioma of Carney triad: a case report.

Carney triad is a rare syndrome, with only 20 complete cases reported. We report a 36-year-old white woman with complete Carney triad, including metastatic gastric stromal tumor (GIST), pulmonary chondroma, and nonfunctioning extra-adrenal paraganglioma. Immunohistochemistry was positive for CD34 and CD117 (c-kit) in the GIST, and positive for chromogranin and CD117 in the paraganglioma. Ultrastructural studies demonstrated skeinoid fibers in the GIST. To our knowledge, this is the 21st complete Carney triad case reported and the first report of dual expression CD117 in both GIST and paraganglioma, a finding with intriguing pathogenetic implications related to the organization of the autonomic nervous system.

Adult↗

PubMed2005

The SDH mutation database: an online resource for succinate dehydrogenase sequence variants involved in pheochromocytoma, paraganglioma and mitochondrial complex II deficiency.

BACKGROUND: The SDHA, SDHB, SDHC and SDHD genes encode the subunits of succinate dehydrogenase (succinate: ubiquinone oxidoreductase), a component of both the Krebs cycle and the mitochondrial respiratory chain. SDHA, a flavoprotein and SDHB, an iron-sulfur protein together constitute the catalytic domain, while SDHC and SDHD encode membrane anchors that allow the complex to participate in the respiratory chain as complex II. Germline mutations of SDHD and SDHB are a major cause of the hereditary forms of the tumors paraganglioma and pheochromocytoma. The largest subunit, SDHA, is mutated in patients with Leigh syndrome and late-onset optic atrophy, but has not as yet been identified as a factor in hereditary cancer. DESCRIPTION: The SDH mutation database is based on the recently described Leiden Open (source) Variation Database (LOVD) system. The variants currently described in the database were extracted from the published literature and in some cases annotated to conform to current mutation nomenclature. Researchers can also directly submit new sequence variants online. Since the identification of SDHD, SDHC, and SDHB as classic tumor suppressor genes in 2000 and 2001, studies from research groups around the world have identified a total of 120 variants. Here we introduce all reported paraganglioma and pheochromocytoma related sequence variations in these genes, in addition to all reported mutations of SDHA. The database is now accessible online. CONCLUSION: The SDH mutation database offers a valuable tool and resource for clinicians involved in the treatment of patients with paraganglioma-pheochromocytoma, clinical geneticists needing an overview of current knowledge, and geneticists and other researchers needing a solid foundation for further exploration of both these tumor syndromes and SDHA-related phenotypes.

Codon, Nonsense↗

PubMed2006

Changes in urinary total metanephrine excretion in recurrent and malignant pheochromocytomas and secreting paragangliomas.

We quantified urinary excretion of total metanephrines (metanephrine plus normetanephrine) (UETM) in 261 patients with pheochromocytoma (PH) or secreting paraganglioma, before primary tumor resection. We then determined UETM 2 weeks after primary tumor resection and once per year thereafter. The tumor was considered benign in 242 patients and malignant in 19 patients. Patients with malignant tumors had higher UETM and tumor diameters, lower plasma epinephrine concentrations, and were more likely to have secreting paragangliomas than patients with benign tumors. In the 215 patients with a single-benign primary tumor, preoperative UETM and tumor diameter were significantly correlated. Thirty-six patients subsequently developed recurrences or new tumors, which were malignant in 18 cases. All recurrent or new tumors could be detected before the onset of symptoms, on the basis of an increase in metanephrine concentration. We followed one patient with von Hippel-Lindau disease and a new tumor for 50 months before reoperation and found that UETM and tumor diameter were significantly correlated. In patients with malignant tumors, the logarithm of UETM was significantly correlated with time, suggesting an exponential increase in tumor activity and, presumably, tumor burden. Urinary metanephrine excretion is a marker of tumor activity and a surrogate of tumor burden in patients with pheochromocytoma or secreting paraganglioma. UETM excretion could be used to assess treatment response in malignant cases.

Adrenal Gland Neoplasms↗

PubMed1992

In vitro and in vivo detection of somatostatin receptors in pheochromocytomas and paragangliomas.

Fifty-one adrenal pheochromocytomas and 14 paragangliomas were evaluated for somatostatin (SRIH) receptor content with in vitro autoradiography on tissue sections from surgically removed tumors, using iodinated 125I[Tyr]3 octreotide as radioligand. Thirty-seven of 51 pheochromocytomas were SRIH receptor positive (73%), as well as 13 of 14 paragangliomas (93%). These SRIH receptors were of high affinity, pharmacologically specific for SRIH and localized on the tumor tissue. Using in vivo imaging techniques with radiolabeled SRIH analogs, paragangliomas could be visualized in five patients, as well as pheochromocytomas in two of three patients. All tumors tested subsequently in vitro (n = 7) were shown to contain SRIH receptors. A majority of pheochromocytomas were also shown to have a high tumoral SRIH content as measured by immunohistochemical techniques. Detection of SRIH messenger RNA in pheochromocytomas by in situ hybridization indicated that the SRIH was produced in the tumors. A weak inverse correlation was observed between SRIH receptor status and tumoral SRIH content, suggesting that SRIH receptors may be down-regulated by high levels of endogenous SRIH in some tumors. There was no correlation between the SRIH receptor status and sex, age, tumor size, benign vs. malignant tumor, or urinary metanephrine excretion. These tumors were also analyzed for allelic losses on various chromosomes and showed significant loss of heterozygosity (LOH) on chromosomes 1p, 3p, 17p, and 22q. All eight tumors with LOH on chromosome 1p were SRIH receptor positive (100%), whereas only 6 of 11 tumors without LOH on 1p (55%) were SRIH receptor positive, suggesting a correlation between LOH on 1p and SRIH receptor positive status. SRIH receptors thus represent a consistent pathobiochemical marker for most of these adrenal and extra-adrenal tumors. In addition, these receptors may be of potential interest for the in vivo localization of these tumors.

Adrenal Gland Neoplasms↗

PubMed2003

Functioning paraganglioma of the thoracic spine: case report.

OBJECTIVE AND IMPORTANCE: Paragangliomas of the thoracic spine are rare. Previously described cases involved nonfunctioning tumors. This report documents the diagnosis and surgical treatment for a patient who presented with a functioning thoracic paraganglioma. CLINICAL PRESENTATION: A 53-year-old woman presented with a 10-month history of headaches, facial flushing, and palpitations associated with hypertension. Urinary catecholamine levels were markedly elevated. Magnetic resonance imaging and m-[(123)I]iodobenzylguanidine scans demonstrated an extradural tumor located within the T12 vertebra, with a significant paraspinal component. The neurological examination revealed mild hypesthesia in the right T12 dermatome. INTERVENTION: The patient underwent resection of the tumor after alpha-adrenergic receptor blockade. Grossly complete excision was achieved without neurological complications. Postoperatively, the patient was normotensive and exhibited catecholamine levels within the normal range. CONCLUSION: Functioning paragangliomas of the thoracic spine are rare lesions that are difficult to treat. Successful treatment requires careful surgical planning and expert pharmacological manipulation.

Adrenergic alpha-Antagonists↗

PubMed2005

The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma.

Phaeochromocytoma or paraganglioma that exclusively secretes dopamine is very rare. This case illustrates its atypical presentation and the importance of interpretative reporting for urine catecholamines leading to the diagnosis and subsequent management of a patient with this condition. We report a 71-year-old Chinese woman with a large dopamine-secreting paraganglioma. She presented with low back pain for six months. On examination, a right abdominal mass was palpable incidentally. Her blood pressure was normal throughout. Serial 24-h urine collections for catecholamines showed enormous elevation of urine dopamine excretion to 80.7 micromol/day (normotensive:<2.6 m mol/day). However, the daily excretions of urine adrenaline and noradrenaline, as well as their metabolites were within their respective reference intervals. Good communication between chemical pathologists and physicians prompted the arrangement of the whole body 131I-meta-iodobenzylguanidine (MIBG) scintigraphy, which showed a large signal in the right upper quadrant of the abdomen corresponding to a large extra-adrenal tumour detected by both ultrasonography and computerized tomography (CT) of the abdomen. Histological section of the tumour tissue revealed paraganglioma, which stained positive for chromogranin and neuron-specific enolase. After four months, the patient presented with chest symptoms and CT of the thorax revealed multiple nodules. Lung metastases were suspected. However, follow-up urine catechola- mine and dopamine excretions were again within their respective normotensive reference intervals. A second MIBG scintigraphy was performed, but no specific uptake at either the thorax or the abdomen could be demonstrated. Fine-needle aspiration cytology using the thoracoscopic technique was performed and immunochemical staining of the biopsy specimen showed the presence of non- small-cell carcinoma of the lung.

3-Iodobenzylguanidine↗

PubMed2000

Neck mass caused by an intraluminal jugular paraganglioma.

Paragangliomas can develop in a number of head and neck sites, the most common being the carotid body paragangliomas and glomus jugulare tumours. This is a case of a paraganglioma confined entirely within the lumen of the jugular vein.

Female↗

PubMed2001

Tracheal paraganglioma: a diagnostic dilemma culminating in a complex airway management problem.

A 41-year-old man presented to his general practitioner (GP) with a wheeze and dyspnoea on exercise. Asthma was diagnosed and treatment with inhaled corticosteroid and a beta(2)-agonist commenced. Despite this, his condition deteriorated over three weeks culminating in stridor, requiring emergency admission to hospital. Nasendoscopy revealed a polypoidal lesion in the upper trachea, acting like a ball valve. A local anaesthetic tracheostomy, secured his airway. Subsequent direct laryngoscopy allowed avulsion of the lesion. Alternative methods of airway management are discussed. Histology revealed a paraganglioma. The aetiology of paragangliomas is described and a literature review of tracheal paraganglioma is presented. Post-operative recovery was unremarkable. However, tumour recurrence occurred at nine months. A subsequent revision tracheostomy and laser resection has ensured disease-free survival, one year later. We recommend that acute onset wheeze, refractive to appropriate therapy, should be referred for urgent examination of the upper aerodigestive tract.

Acute Disease↗

PubMed2003

Pigmented vagal paraganglioma.

Paragangliomas are uncommon and those arising from the vagal trunk are rarer. Pigmented extra-adrenal paragangliomas are still rarer and reported sites of occurrence are the uterus, spine, retroperitoneum, bladder, mediastinum and orbit. The presence of abundant pigment in a cervical paraganglion has not been reported previously. We report one such unusual case of pigmented vagal paraganglioma that arose from the vagal trunk below the nodose ganglion, had massive central necrosis and showed hypovascularity on angiography. The unusual features and difficulties in the diagnosis of such cases are discussed.

Humans↗

PubMed1988

Laryngeal paraganglioma: update on diagnosis and treatment.

Sixty-one cases of laryngeal paraganglioma have been reported in the world literature. Histologic and clinical similarities between this neuroendocrine neoplasm and other organoid laryngeal tumors have led to uncertainty regarding the malignant potential and prognosis of this entity. Immunohistochemical assays have identified biogenic amines and hormonal peptides in these tumors which may prove useful as diagnostic markers. Computer tomography (CT) scanning demonstrates an enhancing lesion and can determine the location and extent of the tumor. The generally small size and constant blood supply of the laryngeal paraganglioma make preoperative angiography unnecessary. Accordingly, intraoperative ligation of the arterial blood supply negates the need for embolization. Partial laryngectomy remains the mainstay of treatment. A case is reported of a patient with a paraganglioma of the larynx who was unsuccessfully treated by laser excision and required surgical resection.

Adolescent↗

PubMed1988

The histopathological and clinical features of irradiated and nonirradiated temporal paragangliomas.

This study was undertaken to determine the effects of radiation on temporal paragangliomas. The histological features were assessed in a blind fashion from 20 surgical specimens obtained from 20 patients, half of whom received radiotherapy. All patients had progressive temporal paragangliomas and were selected on a random basis for this study. The derived data clearly identify the unpredictable response of these tumors to radiation and supports our contention that surgery is the preferred form of treatment for temporal paragangliomas even after radiation therapy. Analysis of clinical histories reveals that previous radiation therapy is associated with a greater operative blood loss and a longer mean postoperative hospital stay due to delayed healing. Despite this, there was no mortality or serious morbidity from surgery.

Adult↗

PubMed2001

Differential diagnosis of pheochromocytomas and paragangliomas.

Paragangliomas are of two types, sympathetic and parasympathetic, depending on the type of paraganglion in which they arise. The term pheochromocytoma is reserved for tumors arising in the adrenal medulla. These tumors are usually fairly easy to diagnose. However, several areas are the subject of debate, including the identification of malignant potential, the diagnosis of medullary hyperplasia, and the recognition of composite tumors. Some histologic features can cause problems in differential diagnosis. Paragangliomas may have spindle cell morphology or contain pigment, requiring distinction from mesenchymal tumors and melanoma, respectively. Extensive degenerative change in pheochromocytomas may mimic adrenal cortical tumors. This short review addresses the diagnosis of pheochromocytomas and paragangliomas and discusses useful approaches in the aforementioned problem areas.

Adrenal Gland Neoplasms↗

PubMed2005

Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors.

Pheochromocytomas and extra-adrenal sympathetic paragangliomas show varied histological patterns, and it is difficult to diagnose malignancy or predict the clinical course using current histological criteria. In the present study, we reviewed 146 sympathetic paragangliomas including 116 adrenal (102 unilateral, 14 bilateral) and 30 extra-adrenal tumors including 38 metastatic tumors. We developed a scoring scale according to the following six factors: histological pattern, cellularity, coagulation necrosis, vascular/capsular invasion, Ki-67 immunoreactivity, and types of catecholamine produced. The tumors were classified as well (WD), moderately (MD), and poorly differentiated (PD) types according to their scores. The frequency of these tumor types were 113 WD (77%), 27 MD (19%), and 6 PD (4%). Metastasis was observed in 15 of 113 WD (13%), 17 of 27 MD (63%), and all 6 PD (100%). Five-year survivals of patients with metastases were 92% with WD, 69% with MD, 0% with PD. Respective 10-yr survivals were 83%, 38%, and 0%. Differences between groups were statistically significant. The data show that using this grading scoring system for sympathetic paragangliomas correlates with both metastatic potential and patient survival.

Adrenal Gland Neoplasms↗

PubMed2005

Malignant paraganglioma of the urinary bladder: Immunohistochemical study of prognostic indicators.

Using various immunohistochemical markers, the objective of our study was to assess whether correlation exists between growth potential of paraganglioma (pheochromocytoma) cells and formation of metastasis. The patient was a 28-yr-old man who presented with intermittent episodes of gross hematuria due to a mass in the urinary bladder. He had no constitutional symptoms to suggest paraganglioma. Histologic, immunohistochemical, and electron microscopic investigation of the surgically removed tissue proved that the tumor was a malignant paraganglioma with metastases in the regional lymph nodes. The immunohistochemical tests were not supportive of high cell proliferation index, indicating that metastases can develop in the absence of rapid multiplication of the tumor cells. Abnormalities in vascular architecture and marked expression of VEGF in the tumor cells may be regarded as prognostic signs to predict the formation of metastases.

Adult↗

PubMed2001

A case of adrenocortical tumor coexisted with paragangliomas.

We report a case of adrenocortical tumor that coexisted with paragangliomas. A 35-year-old woman was admitted to the hospital because of left upper abdominal pain. A palm-sized mass was palpated at left upper quadrant. Hormonal studies revealed the features of pheochromocytoma. An emergency operation was performed because hemorrhage of the tumor was suspected. A 10 cm diameter ruptured mass was found in the left adrenal area and other tumors were also noted adjacent to inferior vena cava (IVC). The pathologic report revealed that the adrenal mass was an adrenocortical tumor with hemorrhagic necrosis and that the tumors adjacent to IVC were paragangliomas. This was the first case of adrenocortical tumor with paragangliomas in our Medline search result, hence we report the case with a review of the literature.

Adrenal Cortex Neoplasms↗

PubMed2004

A thyroid nodule revealing a paraganglioma in a patient with a new germline mutation in the succinate dehydrogenase B gene.

A 32-year-old asymptomatic female was diagnosed with an isolated thyroid nodule of 2.5 cm diameter. Fine needle aspiration suggested a medullary thyroid carcinoma. Consequently, a total thyroidectomy was performed. The nodule stained positive for chromogranin A, neurone-specific enolase and synaptophysin, but not for calcitonin. Finally, pathological analysis showed a thyroid paraganglioma. Although the tumour appeared to be sporadic in a patient with no personal or familial history of paraganglioma and/or pheochromocytoma, we have identified a new mutation (392delC) of the succinate dehydrogenase-B (SDHB) gene in the genomic DNA extracted from the leukocytes of the patient. That mutation induced a shift in the reading frame of the gene creating a premature stop codon (P131fsX135) which was predicted to result in a truncated SDHB protein of 135 amino acids. This report highlights the difficulties of this unexpected diagnosis of hereditary thyroid paraganglioma. It also discusses the clinical involvements in terms of familial screening and the necessary follow-up of the patient.

Adult↗

PubMed1987

Cervical catecholamine-secreting paraganglioma in the pterygopalatine fossa.

A 25-year-old man was admitted for examination to determine the cause of hypertension. High levels of noradrenaline in plasma and urine were seen, suggesting that the patient had an adrenal or extra-adrenal pheochromocytoma. However, whole-body scintigraphy using the isotope of [131I] meta-iodo-benzylguanidine (131I-MIBG) failed to find the presence of a catecholamine-secreting tumor. The highest level of noradrenaline was detected in plasma obtained from the left jugular vein after selective venous collections through femoral catheterization. Both the computed tomography and cerebral angiography showed a hen-egg-sized tumor located in the left pterygopalatine. After surgical removal of the tumor, high blood pressure and the levels of noradrenaline in plasma and urine were significantly decreased. Histopathological diagnosis was paraganglioma (catecholamine-secreting paraganglioma). The patient with cervical catecholamine-secreting paraganglioma is the first case where the tumor was isolated and located in the pterygopalatine fossa.

3-Iodobenzylguanidine↗