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PubMed1987

Observations on the function of normal adrenomedullary tissue in patients with phaeochromocytomas and other paragangliomas.

123I-MIBG is a norepinephrine analogue used for imaging phaeochromocytomas and other paragangliomas. In this study 7 normal volunteers and 31 subjects with high or borderline high plasma catecholamine and urinary metanephrine and VMA levels were investigated. In the normals the uptake by the adrenal medulla was in the range of 0.01%-0.21% of the injected dose at 22 h. In 22 subjects there was no evidence for any paraganglioma, with the uptake by the adrenal medulla in the range of 0.01%-0.22% of the injected dose at 22 h. In nine patients there was evidence of a paraganglioma on imaging which was confirmed on surgery. The uptake by the tumours was in the order of 0.001%-0.14% of the injected dose per gram of tumour tissue. In all nine cases the non-tumorous adrenal medulla could be visualized; the uptake in the adrenals was in the range of 0.01%-0.21% of the injected dose. This study shows that in the presence of elevated plasma catecholamines of tumour origin the unaffected adrenal medullary tissue retains the capacity for 123I-MIBG uptake. In the two cases also studied by adrenal venous sampling, catecholamine release was also within the normal range.

3-Iodobenzylguanidine↗

PubMed1979

Nonchromaffin paraganglioma of the duodenum.

A benign nonchromaffin paraganglioma of the duodenum is described and compared with other reported cases. Duodenal location is extremely rare but the morphology, based on the optical microscopic pattern (Zellballen) and the ultrastructural appearance is, comparable with paragangliomas of other sites. The lack of nerve fibres and ganglion cells in this tumour, together with the absence of a positive chromaffin reaction permits us to classify it as a pure nonchromaffin paraganglioma. This finding constitutes indirect evidence of the probable existence of a paraganglion in the duodenal wall, a structure not yet demonstrated in the adult.

Duodenal Neoplasms↗

PubMed1991

[Therapy of a malignant sympathetic paraganglioma of the organ of Zuckerkandl--a case report].

We present a case report on a 35-year-old patient in whom a malignant sympathetic paraganglioma of the organ of Zuckerkandl was the cause of severe hypertension with excessive perspiration at night. Since curative surgery was not possible medical treatment was initiated. Interferon alfa 2b (Intron A, Essex Pharma) and the somatostatin-analogue SMS 201-995 (Sandostatin, Sandoz) had no effect on catecholamine production and progression of the tumor. Treatment with alpha-methyl-para-tyrosin (MPT, [Metyrosin], Demser, MSD) turned out to be an effective and well tolerable therapy in this patient with peritoneal carcinosis. Clinical and hormonal progression of the paraganglioma resumed only after two years of therapy, which constitutes the longest documented period of time of successful MPT treatment. The superior efficacy of MPT in our patient should encourage postoperative medical treatment with MPT in malignant pheochromocytoma or malignant paraganglioma, particularly when the tumor turns out to be resistent to alpha blocking drugs.

Adult↗

PubMed1999

An extremely large solitary primary paraganglioma of the lung: report of a case.

We present herein the case of a 38-year-old woman found to have an extremely large solitary primary paraganglioma of the lung. The patient presented with chest pain on exertion and a mass was discovered in the left lower lobe of the lung by chest X-rays and computed tomography (CT). As no other neoplasms were detected elsewhere, a left lower lobectomy was performed. The patient has remained well without any evidence of recurrence for 5 years since her operation. The tumor, measuring 13 x 12 x 7 cm, was composed of ovoid cells (Zellballen), which were positive for Fontana-Masson and Grimelius stains, and sustentacular cells. Immunohistochemically, the ovoid cells were positive for neuron-specific enolase, S-100, CAM5.2, Leu7, and chromogranin A, and negative for carcinoembryonic antigen and epithelial membrane antigen. The sustentacular cells were positive for S-100 protein and CAM5.2, and negative for glial fibrillary acid protein. Therefore, the tumor was diagnosed as a paraganglioma. The tumor from our patient is the largest of the 17 solitary primary pulmonary paragangliomas reported thus far in the English-language literature.

Adult↗

PubMed1996

Duodenal gangliocytic paraganglioma presenting as an ampullary tumor.

CONCLUSION: We report a case of a duodenal gangliocytic paraganglioma that had an unusual clinical presentation and atypical immunohistochemical features. BACKGROUND: Duodenal gangliocytic paragangliomas are rare tumors of the gastrointestinal tract with approx 70 cases reported in the literature. The most common presentation is with gastrointestinal bleeding, whereas obstructive jaundice is very uncommon. Immunohistochemistry typically shows positive epithelial cell staining for somatostatin, pancreatic polypeptide, and other neuroendocrine markers. METHODS: A 56-yr-old man presented with intermittent episodes of epigastric pain, vomiting, and obstructive jaundice. Upper gastrointestinal endoscopy revealed a pedunculated ampullary tumor that was subsequently confirmed by computed tomography. At operation, there was marked secondary obstructive chronic pancreatitis in the remaining pancreas necessitating a pylorus-preserving total pancreatectomy. RESULTS: Histology confirmed typical features of a duodenal gangliocytic paraganglioma, but immunohistochemistry demonstrated negative staining for somatostatin and other polypeptides, which is in contrast to most other reported cases. Two years after surgery, the patient remains alive and well on medical treatment with no evidence of recurrent tumor.

Ampulla of Vater↗

PubMed2004

[Paraganglioma in the area of the head and neck. A review of molecular genetic research].

Paragangliomas of the head and neck region are usually benign tumors that develop from chemoreceptors of paraganglionic origin in the majority of patients. These receptors play an important role in sensing and regulation of the blood CO(2) level. Genetic alterations in the mitochondrial enzyme complex II (SDH), which is involved in respiratory chain and citric acid cycle reactions, have been shown to lead to sporadic as well as familial cases of these tumors. The gene encoding the subunit SDHD shows mutations in up to 50% of these cases. In addition, loss of heterozygosity (LOH) was demonstrated in these tumor samples and has been shown to be connected with oncogenesis of paragangliomas. Thus, SDHD is the first known tumor suppressor gene encoding a mitochondrial protein. In this article we summarize the current state of knowledge concerning the development of paragangliomas.

Cell Transformation, Neoplastic↗

PubMed2004

[Paragangliomas in the head-/neck region. I: Classification and diagnosis].

Paragangliomas of the head and neck occur sporadically or are hereditary. The hereditary phenotype characteristically occurs between the 2nd and 3rd decade of life; the sporadic phenotype beyond the 4th decade. The hereditary phenotype "paraganglioma syndrome type 1" frequently shows multilocular tumor manifestations and rarely cases of maltransformation. Therefore, paragangliomas should be considered a systemic disease. For diagnosis, an interdisciplinary, step by step procedure is mandatory comprising genetic screening, whole body medical check-up including catecholamine metabolism, and optionally an 18F-Dopa-PET and localised imaging procedures.

Diagnosis, Differential↗

PubMed1999

Paragangliomas of the spinal canal.

We report the clinical MRI and histopathological features of five consecutive cases of spinal paraganglioma. Three intradural tumours were found in the typical location (two at the L4, one at the S2 level); one intradural extramedullary tumour arose at an unusual level, from the ventral C2 root, and one extradural tumour growing along the L5 nerve root sheath had an aggressive growth pattern with early, local paraspinal recurrence and, eventually, intradural metastatic spread. This type of growth pattern has not been described previously. Paragangliomas of the spinal canal are more common than previously thought and can be located anywhere along the spine, although the lumbosacral level is the most common. Their appearance on MRI can not distinguish them from other tumours in the spinal canal. Even though paragangliomas in general are benign and slowly growing their growth pattern can vary and be more aggressive, to the point of metastatic spread.

Adult↗

PubMedDate not supplied

Malignant paraganglioma draining into the main portal vein.

Abdominal paraganglioma is a relatively rare tumor, and there have been no previous reports describing its contrast-enhanced ultrasonographic findings. We report a case of a lesion of unknown origin incidentally detected by ultrasound in the right upper abdomen. The lesion was markedly hypervascular and contrast-enhanced ultrasonogram showed it to be communicating with the portal vein. This communication was then confirmed by angiogram. Such an arteriovenous communication through paraganglioma has been recently reported, and a diagnosis of a paraganglioma should be considered when contrast-enhanced ultrasonogram shows a solid mass of unknown origin draining directly into the venous system.

Abdominal Neoplasms↗

PubMed1997

Magnetic resonance imaging findings of a nonfunctional mediastinal paraganglioma with an unusual presentation.

A case of histologically proven mediastinal paraganglioma presenting with metastatic spread to supraclavicular lymph nodes is presented. Mediastinal paragangliomas are extremely rare tumors and their CT and MR imaging features have not been well documented in the radiologic literature. The T 1-weighted and T 2-weighted spin-echo MR findings of a locally invasive mediastinal paraganglioma with metastasis to the supraclavicular lymph nodes are described and the literature is briefly reviewed.

Adult↗

PubMed2003

Transventricular paraganglioma of the larynx.

Laryngeal paragangliomas are rare neuroendocrine tumours. Most laryngeal paragangliomas are supraglottic in location, with only 3% reported to be glottic in location. We present a rare case of transventricular paraganglioma with fixation of the hemilarynx that presented a diagnostic and therapeutic challenge.

Female↗

PubMed1998

Expression of insulin-like growth factor (IGF)-II in human prostate, breast, bladder, and paraganglioma tumors.

Insulin-like growth factors (IGFs) are potent mitogens for a variety of cancer cells in vitro. A paracrine/autocrine role of IGF-II in the growth of breast and prostate cancer cells has been suggested. Information on cell-type-specific IGF-II expression in vivo in the breast and prostate is, however, limited. Thus, cell types expressing IGF-II mRNA and protein in tumors were identified by in situ hybridization and immunohistochemistry. Of 36 prostate, 17 breast, and 10 bladder cancers, and 9 paraganglioma tissues examined, IGF-II was expressed in more than 50% of prostate, breast, and bladder tumors, and in 100% of paraganglioma tumors. Expression levels of IGF-II were highest in the paraganglioma and bladder followed by prostate and breast tumors. In all the tumors expressing IGF-II, both mRNA and protein were localized to malignant cells, expression in the stroma being minimal. Since previous studies had indicated that an incompletely processed form of 15-kDa IGF-II exhibited higher mitogenic potency than the completely processed 7.5-kDa IGF-II form, the quantity and size of IGF-II proteins expressed in these tumors were analyzed by Western immunoblotting. Greater expression of 15-kDa IGF-II relative to the 7.5-kDa IGF-II form was clearly demonstrated in all six prostate cancers and in half of the two breast and four bladder cancers examined. The results are consistent with the hypothesis that the 15-kDa form of IGF-II expressed in cancerous cells contributes to autocrine cancer cell growth in vivo.

Breast Neoplasms↗

PubMed2001

Laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl.

We describe the successful laparoscopic resection of a functional paraganglioma in the organ of Zuckerkandl. A 47-year-old man with hypertension and diabetes mellitus was found to have an abdominal mass beside the aorta. The tumor was diagnosed as a functional paraganglioma by diagnostic imaging and biochemical tests. We then performed a transperitoneal laparoscopic resection for removal. After freeing the left ureter, resecting the inferior mesenteric artery, and dividing the small blood vessels, the tumor was isolated and found to be preserved in its capsule. It was retrieved in a bag through an enlarged incision. The operation time was 450 min and blood loss was 410 ml. The postoperative course was uneventful and there has been no local recurrence or distant metastasis during the 18-month follow-up period. Laparoscopic resection of functional extraadrenal paragangliomas is technically feasible and safe if adequate pre- and intraoperative medical management and a careful, steady surgical technique are used.

Abdominal Neoplasms↗

PubMed2004

Duodenal gangliocytic paraganglioma treated with endoscopic hemostasis and resection.

Gangliocytic paragangliomas are exceedingly rare tumors that arise in close proximity to the papilla of Vater. There are few reports of the endoscopic resection of duodenal gangliocytic paraganglioma. A 61-year-old woman was admitted with a complaint of melena. Endoscopic examination revealed a pedunculated submucosal tumor with erosion in the third portion of the duodenum. Hemostasis, using a gold probe, was performed. Nine days later, we successfully resected the tumor, using endoscopic polypectomy. To determine the depth of tumor invasion, endoscopic ultrasonography was used. The size of the tumor was 3.0 x 2.5 x 1.0 cm. A total of 25 cases of duodenal gangliocytic paraganglioma have been reported in Japan. Generally, this tumor is considered benign. However, resection was performed in many patients because preoperative diagnosis was impossible. In Japan, no previous studies have reported using endoscopic hemostasis, to our knowledge. Our patient is the fourth in Japan to be treated by endoscopic resection. We report on our patient, with a review of the literature.

Duodenal Neoplasms↗

PubMed2002

Paragangliomas of the temporal bone: results of different treatment modalities in 53 patients.

BACKGROUND: The authors retrospectively compared the results of three different treatment modalities (surgery, conventional radiotherapy and gamma knife radiosurgery) in patients with paragangliomas of the temporal bone, in order to determine the optimal current treatment concept. METHOD: Between 1978 and August 2001, 53 patients (12 men and 41 women; mean age, 58.3 years; range, 17 to 84 years) with paragangliomas of the temporal bone were treated at the neurosurgery and ENT departments of the University of Vienna. According to the Fisch classification, 6 patients had class B tumours, 20 had class C, and 27 patients had class D tumours. Thirty-two patients (mean age, 57.0 years; 6 B, 14 C, 12 D) underwent surgery. In 17 cases the tumour was embolised prior to surgery. Nine patients (mean age, 73.9 years; 6 C, 3 D) received primary radiotherapy (median total dose, 46.8 Gy). Six patients (mean age, 73.5 years; 6 D) underwent primary radiosurgery (median centre dose 24, Gy) and 6 patients (6 D) admitted from other departments with recurrent tumours adjuvant radiosurgery (median centre dose, 25.5 Gy). FINDINGS: In 20 of the surgical cases (62.5%) complete tumour resection was achieved and the patients required no further treatment over a mean follow-up period of 9.1 years. Of the 12 patients with incomplete tumour resection, 9 (5 C, 4 D) received postoperative adjuvant radiotherapy and three patients (3 D) adjuvant radiosurgery. In 15 (83.4%) of the 18 patients who underwent radiotherapy the tumours showed no signs of progression and the patients remained clinically unchanged over a mean period of 9.4 years. Three patients (16,6%) experienced progression of their tumour within an average period of 2.8 years. In the 15 patients who underwent primary radiosurgery, an objective 100% tumour control rate with no evidence of progression of disease was observed. INTERPRETATION: The results indicate that the most effective current treatment option for patients with paragangliomas of the temporal bone is a single-stage radical tumour resection, performed in advanced tumours as an interdisciplinary neuro-otosurgical procedure. For subtotally resected or non-resectable tumours, gamma knife radiosurgery has proved to be a safe and effective treatment modality.

Adolescent↗

PubMed2004

Nasopharynx paraganglioma with extension in the clivus.

Paraganglioma is a rare benign tumor arising from the sympathetic nervous system. Here we describe an exceptional case of a paraganglioma located in the nasopharynx with an extension through the clivus up to the dura. Atypically, no contact with any major vessels was found. A radical resection of the mass was performed by an anterior transmaxillary approach through a Le Fort I osteotomy. One year follow up reveals no signs of local or distant recurrence. No cosmetic changes can be observed after the surgery and nasal and masticatory functions are unmodified. We review the clinical presentation, workup of paraganglioma, as well as the surgical approaches to the clivus.

Adult↗

PubMed2005

Management of delayed intracranial and intraspinal metastases of intradural spinal paragangliomas.

The preferential site of extra-adrenal paragangliomas is the head and neck region. However intradural spinal paragangliomas are commonly described and are considered as benign entities. We report the case of a paraganglioma of the cauda equina followed after complete surgical removal by intracranial and intraspinal cerebrospinal fluid metastases. Seven years after the first operation, a cystic cerebellar lesion was successfully treated by surgery. During a long follow-up, four locations in the posterior fossa, a lumbar recurrence and metastatic nodules at T6 and S1-S2 level were also discovered. Radiotherapy stopped the lesions' growth and allowed improvement of the neurological status. Through a review and analysis of the literature, we discuss the management of this unusual evolution.

Adolescent↗

PubMed1999

Paraganglioma of the cauda equina region--report of two cases and review of the literature.

A paraganglioma of cauda equina region is extremely rare and except for secreting tumour, the pre-operative diagnosis of paraganglioma is very difficult. Two cases of non-functional paragangliomas of the cauda equina region are reported, one was attached to the filum terminale and the other to a rootlet looking very much like a vascular neurinoma. Both were successfully removed by surgery. An extensive review of the literature permits one to find 77 other cases. The clinical, radiological, pathological (ultrastructural and immunohistochemical) features and surgical findings of all theses cases are discussed. Surgery remains the treatment of choice. No effect of radiotherapy on recurrence prevention has ever been demonstrated.

Adult↗