Pancreatic paraganglioma with draining vessels.

A pancreatic paraganglioma is a rare neoplasm that is difficult to distinguish from a pancreatic neuroendocrine tumour. Here we present a case of pancreatic paraganglioma that was surgically resected following preoperative diagnosis of a pancreatic neuroendocrine tumour. Careful evaluation of the endoscopic ultrasonography findings revealed abundant draining vessels, which could have led to a correct preoperative diagnosis of pancreatic paraganglioma.

Contemporary surgical management of cardiac paragangliomas.

BACKGROUND: Cardiac paragangliomas are an extremely rare subset of chromaffin cell tumors that develop from neural crest cells. METHODS: Between March 2004 and October 2010, 7 male patients from our two institutions who underwent surgical resection of cardiac paraganglioma were retrospectively reviewed. RESULTS: In 5 patients, paragangliomas originated from the roof of the left atrium, and in 2 patients, they originated from the aortic root. Hospital mortality was 14%. CONCLUSIONS: Complete surgical resection remains the mainstay of therapy and can be curative, but carries a significant risk of intraoperative bleeding and usually requires cardiopulmonary bypass and often complex resection techniques, including cardiac autotransplantation.

Paraganglioma of the cauda equina: a highly vascular tumour.

Paragangliomas uncommonly occur in the cauda equina region. This type of tumour may be misdiagnosed pre-operatively as a neurinoma or an ependymoma. Paragangliomas are highly vascular tumours. We report three patients with paraganglioma of the cauda equina. The first patient had many dilated vessels resembling an arteriovenous malformation, which were seen during surgery. The second patient had a post-operative haemorrhage without any residual tumour. The third patient had a selective angiogram with dense tumour staining.

Non-functional paraganglioma of the posterior mediastinum.

Mediastinal paraganglioma is a rare and slow growing neurogenic tumor. Here, we describe a 49-year-old woman with a non-functional posterior mediastinal paraganglioma. Video-assisted thoracoscopic surgery for tumor excision failed due to massive bleeding. The tumor was excised successfully by lateral thoracotomy with bipolar electrocautery 1 week after the first operation. Mediastinal paraganglioma remains a surgical challenge due to its hypervascular character and firm adhesion to adjacent mediastinal structure. Since the non-functional posterior mediastinal paraganglioma is often diagnosed after operation, it should be regarded as a differential diagnosis of mediastinal mass, especially if surgeons experience unexpected massive bleeding during operation.

Intraoperative monitoring of intraarterial paraganglioma embolization by indocyaningreen fluorescence angiography.

Preoperative arterial embolization (AE) of paraganglioma (PG) is widely used to diminish intraoperative blood loss. Thereby conditions for a resection of the tumor shall be improved and risks for facial, vagal or hypoglossus nerve injuries are reduced. The vascularization of jugular and tympanic PGs is particularly complex due to collaterals with the vertebral and internal carotid arteries. Thus AE is often not complete and intraoperative blood loss may still be considerable. The postinterventional perfusion is of interest for the surgeon. We evaluated the arterial perfusion after AE using indocyaningreen (ICG) angiography. Six patients with PG, two carotid PGs, two jugular PGs, one vagal PG and one tympanic PG underwent surgery 1 day after AE. After tumor was exposed, ICG was intravenously applied followed by fluorescence angiography. Residual perfusion was assessed on the video clip and the perfusion index was automatically calculated by the IC-CALC software. This index was compared with the radiologist's assessment of arteriographic control after AE. Two of the six patients showed only marginal residual perfusion. These were patients with carotid PGs. The patient with the vagal PG showed 20%, the patients with jugular PGs 80 and 60% and the patient with the tympanic PG had 70% residual blood flow. The preoperative AE is rarely complete in PGs of the petrous bone. Intraoperative fluorescence angiography is a reliable procedure to evaluate the efficiency of preoperative embolization and can help the surgeon to estimate intraoperative bleeding favouring risks.

Intrapericardial paraganglioma directly irrigated by the right coronary artery.

We present a case of a nonfunctioning intrapericardial paraganglioma that presented as a typical chest pain in a 51-year-old woman. The tumor was initially diagnosed on coronary angiography where it had direct irrigation from the right coronary artery. Further computed tomography and magnetic resonance imaging scans showed significant compression of the superior vena cava by the tumor. This was excised through median sternotomy and extracorporeal circulation. Histopathological examination of the mass was characteristic of a paraganglioma.

A novel mutation in the SDHD gene responsible for familial paraganglioma. Medical and psychological implications.

Familial paragangliomas/pheochromocytomas are dominantly inherited disorders characterized by the development of highly vascularized tumors of the head and neck, derived from non-chromaffin cells of the extra-adrenal paraganglia, and tumors with endocrine activity, derived from chromaffin cells, usually located in the adrenal medulla and pre- and para-vertebral thoracoabdominal regions. Germline inactivating heterozygous mutations in one of the genes encoding for succinate dehydrogenase subunits B, C or D (SDHB, SDHC or SDHD) are responsible for hereditary paragangliomas (PGLs), accounting for nearly 70% of familial cases. Particularly in the SDHD gene, different types of mutations have been found, nevertheless, alterations other than point mutations and deletion leading to missense/nonsense/splicing mutations are extremely rare. Here we report a family with multiple cases of PGL which co-segregates with a novel SDHD gene mutation predictable to give rise to an abnormal gene product (CybS). The identification of the molecular event responsible for PGL in our family made genetic counseling particularly useful for younger first degree relatives at risk to develop this late-onset disease.

Management of cervical paragangliomas: review of a 15-year experience.

BACKGROUND AND AIMS: Cervical paragangliomas are highly vascular neoplasms and should be considered in the evaluation of all lateral neck masses. The aim of this study is to review an institutional experience in the management of these tumors. MATERIALS AND METHODS: Thirteen patients with 14 paragangliomas were treated in our institution during a period of 15 years. There were eight women (61.5%) and five men (38.5%) with a mean age of 41.3+/-15 years. A painless lateral neck mass was the main finding in 69.2% of patients. There was no evidence of a functional tumor. Carotid angiography was performed in all patients to define the vascular anatomy of the lesion. The 78.6% of paragangliomas underwent selective embolization of the major feeding arteries. Surgical resection followed within the next 48 h. RESULTS: The majority of the lesions were paragangliomas of the carotid bifurcation (85.7%), while one patient was diagnosed with a jugular and one with a vagal paraganglioma. In one patient, bilateral paragangliomas in the carotid bifurcation were detected. There was no evidence of malignancy in any case. Preoperative embolization has proven successful in reducing tumor vascularity. Vascular reconstruction was necessary in one patient. The main postoperative complication was transient cranial nerve deficit in seven (53.8%) patients, and a permanent Horner's syndrome was documented in one patient. No stroke occurred. The jugular paraganglioma was treated with irradiation due to skull base extension with significant symptomatic relief. CONCLUSION: Combined therapeutic approach with preoperative selective embolization followed by surgical resection by an experienced team offers a safe and effective method for complete excision of the tumors with a reduced morbidity rate.

Intratumoral injection of cyanoacrylate glue in head and neck paragangliomas.

BACKGROUND AND PURPOSE: Substantial intraoperative bleeding during surgical removal of head and neck paragangliomas may be a major problem in the management of these highly vascularized tumors. Traditional preoperative embolization via a transarterial approach has proved beneficial but is often limited by complex vascular anatomy and unfavorable locations. We report our experience with the preoperative devascularization of head and neck paragangliomas by using direct puncture and an intralesional injection of cyanoacrylate. METHODS: We retrospectively analyzed nine consecutive patients with head and neck paragangliomas who were referred for preoperative devascularization. Three patients were treated for carotid-body tumors; two for vagal lesions; and four, for jugular paragangliomas. Direct puncture of the lesion was performed by using roadmap fluoroscopic guidance. Acrylic glue was injected by using continuous biplane fluoroscopy. All patients underwent postembolization control angiography and immediate postoperative CT scanning. RESULTS: Angiograms showed that complete devascularization was achieved in all cervical glomus tumors, whereas subtotal devascularization was achieved in jugular paragangliomas. In this latter location, the injection of acrylic glue was limited by the potential risk of reflux into normal brain territory via feeders from the internal carotid or vertebral artery. The tumors were surgically removed and histologically examined. No technical or clinical complications related to the embolization procedure occurred. CONCLUSION: Percutaneous puncture of paragangliomas in the head and neck region and their preoperative devascularization by intralesional injection of acrylic glue is a feasible, safe, and effective technique.

[Histological variants of parasympathetic paragangliomas (chemodectomas) of the neck and morphological variants of their malignancy]. / Gistologicheskie varianty parasimpaticheskikh paragangliom (khemodektom) shei i morfologicheskie kriterii ikh zlokachestvennosti.

Histotopograms of 28 chemodectomas have been studied. Principal microscopic features of malignancy are as follows: structural atypia (complete in solid or fibrous variants) or partial (alveolar chemodectoma) loss of alveolarity as the most important sign of organotypia; another sign of malignancy is cell cataplasia (cell and nuclear polymorphism, change in nuclear-cytoplasmic relationship in the direction of its increase, nuclear hyperchromatism, appearance of large ungly hyperploid nuclei, mitotic activity of tumour cells in one case. Incomplete angiogenesis characteristic for malignant tumours was also detected. Invasive growth in the majority of chemodectomas in the form of partial growth through the walls of great vessels and ubiquitous growth through the walls of small vessels were present in most chemodectomas. Thus, there is a basis to claim that the chemodectomas studied are malignant tumours although they are characterized by a slow (years) growth and slow progression.

Color Doppler imaging of paragangliomas in the neck.

PURPOSE: In this study, we describe the color Doppler imaging findings in carotid body tumors and vagal body tumors. METHODS: B-mode and color Doppler imaging were performed on 17 patients who had a total of 25 previously diagnosed paragangliomas (14 carotid body tumors and 11 vagal body tumors). RESULTS: Nineteen of 25 tumors were depicted. Five small vagal body tumors in the region of the nodose ganglion and 1 carotid body tumor could not be depicted. With B-mode imaging, paragangliomas appeared as well-defined, solid, hypoechoic masses. With color Doppler imaging, hypervascularity with a low-resistance flow pattern was demonstrated in all but 1 of the 19 tumors. CONCLUSIONS: The use of color Doppler imaging in the workup of an ambiguous neck mass is advocated.

Tumor angiogenesis in pheochromocytomas and paragangliomas.

BACKGROUND: Angiogenesis correlates with growth and likely metastases in several tumors. To determine whether it has a similar role in pheochromocytomas, immunohistochemical staining of factor VIII was done on the tumor tissue of 42 patients. METHODS: Formalin-fixed, paraffin-embedded tissue was obtained from 29 women and 13 men with 24 primary adrenal and 18 extraadrenal pheochromocytomas. Patients were divided into two groups. Group 1 included 32 patients with benign pheochromocytomas, and group 2 included 10 patients with malignant tumors evidenced by capsular or vascular invasion (six), liver metastases (three), or periaortic lymph node metastases (one). Blood vessels highlighted by factor VIII staining of endothelial cells with labeled streptavidin-biotin were counted under light microscopy. Mean vessel count within a 10 mm2 micrometer disk was calculated under x100, x200, and x400 magnification fields. RESULTS: There were no significant differences in patient age or clinical symptoms between the groups. The mean tumor size in group 2 of 8.8 +/- 5.3 cm was larger than the mean of 4.8 +/- 2.8 cm in group 1 (p < 0.005). The mean counts of vessels in the x100, x200, and x400 magnification fields were 102 +/- 48, 40 +/- 18, and 19 +/- 9 in group 1, and 203 +/- 77, 73 +/- 28, and 37 +/- 15 in group 2. The number of blood vessels in group 2 was significantly higher than in group 1 (p < 0.001) in each studied field. CONCLUSIONS: In this study the number of tumor blood vessels correlated with the invasive behavior of pheochromocytomas. Tumor angiogenesis may be useful in determining the likelihood of malignant behavior in pheochromocytomas.

[Lateral neck paraganglioma: diagnostic imaging and preoperative embolization]. / Les paragangliomes latéro-cervicaux: imagerie diagnostique et embolisation pré-opératoire.

Sixteen paragangliomas of the neck were reviewed. Lesions were found in the carotid body (n = 12), vagal nerve (n = 3) and larynx (n = 1). While paragangliomas were often misdiagnosed clinically, CT scanning defined the extent of the disease. MR was able to accurately characterize the tumors as highly vascular. Multiplanar imaging, exquisite tissue contrast and anatomic detail allowed better display of the relationship between these neoplasms and surrounding carotid sheath vessels and intracranial structures. MR constitutes the imaging modality of choice in carotid body tumor. Arteriography is necessary for definite diagnosis and presurgical embolization.