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We present two cases of unusually large skull base paragangliomas. The first tumor was accompanied by marked bony destruction of the central skull base and multiple associated cysts. The second tumor arose along the petrous ridge, with a large intracranial component. The CT, MR imaging, angiographic, histologic, and electron microscopic findings of these unusual lesions are described.
The MR imaging features of a paraganglioma of the cauda equina with associated spinal cord cysts are presented. MR imaging showed the tumor to be isointense with the spinal cord on all pulse sequences and to enhance homogeneously. The intramedullary cysts had increased signal intensity on proton density- and T2-weighted images, and involved the cervical and thoracic regions.
On the basis of 7 cases of temporal bone paraganglioma diagnosis of this tumor is presented. Apart from aural symptoms cranial nerves palsy may appear. Computer tomography, magnetic resonance imaging and subtraction angiography are essential investigations.
A case of laryngeal paraganglioma in a 65-year-old male was treated by supraglotic laryngectomy. This controversial tumor is analyzed and guidelines are given for its clinical and histological (immunohistochemical) diagnosis and treatment.
Two cases with retroperitoneal tumours affecting young women who cannot undergo surgery were investigated by biopsy and needle sampling. One of these cases presented a mainly alveolar histologic pattern and was easily diagnosed by immunohistochemical means, as well as a chromaffin paraganglioma. We discussed different diagnoses and approaches in the literature.
Gangliocytic Paraganglioma of the duodenum is rare neuroendocrine tumor. This type of the tumor can cause bleeding into the gastrointestinal tract and epigastric pain in this region. It can be difficult to differentiate this tumor from the primary tumor of the head of the pancreas growing secondary to the duodenum. The authors describe one such misdiagnosed case treated finally successfully by hemipancreatodecodenectomy.
The authors report a case of 47 years old male who was treated for 1,5 year because of recurrent bleedings from upper GIT. Performed examinations showed the presence of retroperitoneal paraganglioma parasympaticum with multiple metastases in bones. A Zollinger-Ellison syndrome was suspected. The final correct diagnosis was established at last microscopically after surgical excision of the specimens.
A case of nonfunctional benign preaortic paraganglioma is reported. The patient, a woman aged 50, complained of vague mesogastric pain irradiated posteriorly, occasional nausea and vomiting, related to a tender pulsatile mesogastric mass fixed to the posterior wall of the abdomen. Preoperative diagnostic examinations showed a severe anemia, a severe mechanical impairment of renal function as shown at IVP, a doubt of aortic aneurism at echography, not confirmed by selective arteriography. The mass was removed radically. The diagnosis of nature was only microscopic. Since from 15 days after surgery IVP improved significantly. At three months follow-up the patient is well with no sign of recurrence.
A carotid body tumor is a paraganglioma of the carotid bifurcation. Histologic appearance does not correlate with the malignant potential of the lesion, and thus a reliable prognostic marker for these tumors is lacking. To determine whether flow cytometric analysis of paraffin block specimens by DNA index and synthetic phase fraction (SPF) would be of prognostic value, a retrospective chart review of 14 patients with carotid body tumors was performed. Three of 14 tumors were aneuploid and were the only tumors with SPF greater than 15%. One of 3 patients (33%) with an aneuploid tumor (SPF = 22%) developed a local recurrence; no patient with a diploid tumor developed a recurrence. Two of 3 (67%) patients with aneuploid tumors (SPF = 18%) but only 1 of 11 (9%) with a diploid tumor were symptomatic preoperatively (P = 0.03). DNA index and SPF may help select a subgroup of patients with more aggressive tumors who are at increased risk for recurrence and therefore require closer follow-up.
The genes responsible for hereditary paragangliomas (glomus tumors, MIM No. 168000) have been mapped to two distinct loci on the long arm of chromosome 11. Most of the informative families appear to be linked to the distal locus on chromosome 11q23 (PGL1), which has been previously confined to a 2-cM interval by haplotype analysis in an extended Dutch pedigree. To facilitate the identification of the PGL1 disease gene, we constructed an approximately 4-Mb ordered clone contig map of Sequence tagged sites, expressed sequence tags (ESTs), and known genes that spans the PGL1 critical region on chromosome 11q23. Among 29 new positional candidate ESTs, only two (EST100999 and EST241777) mapped within the PGL1 critical region. We further characterized the genomic organization of the promyelocytic leukemia zinc finger (PLZF) gene that maps within the PGL1 critical region and physically excluded the serotonin receptor type 3 (5HT3R) gene. Finally, we identified a common, silent, single-base substitution polymorphism in the 5HT3R gene and characterized the allele sets of two new highly polymorphic microsatellite repeats within the PGL1 critical region.
Two patients with cervical paragangliomas underwent positron emission tomography (PET) with 2-[18F]-fluoro-2-deoxy-D-glucose (FDG). There was marked tumor uptake and retention of FDG. Adjacent salivary gland accumulation of FDG was minimal, though quite prominent with meta-iodobenzylguanidine. FDG PET offers another potentially useful approach to functional imaging of these uncommon tumors, independent of the presence of specific amine uptake mechanisms or cell surface receptors required by other scintigraphic techniques.
A survey is given of the clinical picture, diagnosis and management of foramen jugulare paragangliomas (JFP), based on reports of 175 cases from the literature and personal experience of 26 cases. Special emphasis is given to the operative techniques. Improvements of diagnostic possibilities as well as operative techniques, including the pre-operative occlusion of tumour-feeding arteries by endovascular methods have markedly reduced the operative mortality to a rate between 0 and 5 percent. The rate of incomplete removal of C and D tumours dropped to about 15%. But very large and extensive tumours remain a real challenge. Teamwork between neuroradiologists, ENT-surgeons and neurosurgeons is mandatory.
Reports on the association of papillary thyroid carcinoma with paraganglionic or desmoid tumors have appeared infrequently. The former setting usually affects middle-aged females; the latter is typical of familial adenomatous polyposis. We report the case of a 69-yr-old man in whom two abdominal masses had been instrumentally detected following an access of abdominal pain. Save for a moderate hypertension, he was asymptomatic and an impalpable thyroid nodule was detected by ultrasonography. A high urinary noradrenaline output and cytology of the masses raised the suspicion of pheochromocytoma. At laparotomy, an adrenal pheochromocytoma and a paracaval paraganglioma were excised. Subsequently, hemithyroidectomy was performed, and histopathology revealed papillary microcarcinoma. A nodule of desmoid tumor was also removed from the abdominal wall. An analysis of RET, APC, and TP53 gene mutations, and of RET and NTRK1 gene rearrangements, yielded negative results. No in vitro transforming activity was detected in the tumor DNA when assayed in transfection experiments. The lack of a consistent family history also made unlikely the possibility of identifying the putative germline defect by linkage analyses. Should this unusual aggregation of tumors represent a new entity, a number of genetic alterations have now been excluded.
The management of cervical paragangliomas (PGs) depends on their specific type and their relation to adjacent vessels. The purpose of this study was to evaluate the potential of magnetic resonance imaging (MRI) and color duplex sonography (CDS) to classify PGs according to topography and vascularization. Sixteen patients harboring 22 PGs were studied retrospectively. With digital subtraction angiography as reference, the topographic relation of the tumors to the carotid arteries and the internal jugular vein and the patterns of vascularization were assessed. On MRI and CDS the typical feature of 15 carotid PGs was splaying of the carotid bifurcation, with the external carotid displaced anteriorly and the internal carotid artery and internal jugular vein located posteriorly. In five vagal PGs both modalities showed unidirectional anterior displacement of the external and internal carotid arteries. Two jugular PGs were found to extend within the lumen of the internal jugular vein. CDS completely depicted carotid body tumors but failed to delineate the high cervical portion of vagal and jugular PGs. MRI allowed us to assess the entire extent of all PGs. Nineteen lesions showed flow voids corresponding to abundant flow signal on CDS: three carotid body tumors appeared hypovascular on CDS and MRI. On CDS, intratumoral flow was directed cranially in carotid and inferiorly in vagal and jugular PGs. CDS and MRI are suitable for classification of cervical PGs as carotid, vagal or jugular PGs based on the topographic relation to the carotid arteries and internal jugular vein. Visualization of the intrinsic tumor vasculature proved an additional distinguishing criterion on CDS.
Two patients with carotid body paraganglioma developed bone metastases 3 and 6 years respectively after surgical excision of the primary tumors. Plain radiographs showed ill-defined metastatic lesions. Scintigram using radiolabeled metaiodobenzylguanidine, an analogue of noradrenaline that is taken up by neurosecretary granules, showed an abnormal accumulation in the corresponding metastatic lesion. Histologically, nests of epithelioid cells with clear cytoplasm and pyknotic nuclei and abundant collagen fibers were observed within destroyed trabeculae. Treatment including external radiation and surgery provided pain relief and early local disease control.
Carotid body tumors constitute the third commonest group of parapharyngeal tumors. Newer diagnostic modalities such as computed tomography scan, magnetic resonance imaging, and Doppler ultrasound are commonly used for diagnosing lesions at this site. However, simple technique of fine-needle aspiration (FNA) cytology is rarely performed in apprehension of complication of hemorrhage. Present case report is of carotid body paraganglioma diagnosed on FNA without any complications and thus providing definite preoperative diagnosis to the surgeon.
We report a case of a 29-year-old male, who during workup of hypertension was found to have a malignant primary paraganglioma of the heart. The tumor arose from the site of the aortopulmonary window and right ventricular outflow tract (RVOT) and was removed with the aid of cardiopulmonary bypass. Reconstruction of the RVOT and pulmonary valve was necessary because of involvement by the tumor. The surgical course was uncomplicated, with normalization of catecholamine secretion and blood pressure.
Carotid body paragangliomas (CBP) are relatively rare tumors arising from the carotid body paraganglion. Clinical signs and images are necessary to establish the diagnosis. Surgery is the preferred method of treatment, paying special attention to the carotid artery. Nine patients with carotid body tumors were identified, three of them with complicated diagnosis. One patient is treated with radiotherapy. Out of the eight surgical patients, three preserved the whole arterial structure, the external carotid artery is resected in two patients, the carotid artery is replaced (safen graft, gore tex prosthesis) in other two patients, and the common carotid was tied in the remaining patient. Our objective is to show the decisions taken in the patients from our casuistic, comparing them to the literature data. We conclude that the diagnosis may be deceptive, and that the attitude forward the carotid is variable, always being prepared to do arterial replacement techniques.