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Radiographic imaging plays an important role in the diagnosis and treatment of paragangliomas. Diagnosis and treatment should be performed as a team effort, with all the involved disciplines working together to provide the best possible individualized work-up and treatment plan for the patient.
Paragangliomas can be challenging to even the most experienced surgeon. Injuries to the carotid artery are not uncommon. To avoid injury, a careful preoperative assessment of the size and extent of the tumor and its relation to the internal carotid artery, external carotid artery, and common carotid artery is important for planning the procedure. For more extensive tumors, it is often helpful to have a multidisciplinary team available to handle all potential issues and complications.
Paragangliomas are vasculature in nature and are surrounded by vital neurovascular structures. The extirpation of these lesions requires careful preoperative evaluation, meticulous surgical technique, and the aid of experienced skull base surgical and rehabilitative teams. When surgery is performed in this way, complications can be minimized, and the function of the upper aerodigestive tract can be protected.
BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.
Nuclear "inclusions" similar to those seen in a variety of normal and neoplastic cells are commonly found in pheochromocytomas and paragangliomas. Rather than representing areas of nuclear or nucleolar degeneration, the "inclusions" of these tumor cells represent deep invaginations of the cytoplasm into the nucleus. The great frequency of nuclear "inclusions" in pheochromocytomas is a reflection of the high degree of nuclear angulation and lobulation commonly seen in these tumors.
Ten cases of duodenal paraganglioma were studied by conventional histologic and immunocytochemical techniques at both light and electron microscopic levels. Histologically, mixtures of epithelial, ganglion, and spindle cells were seen. In all of the cases immunoreactivity for neuron-specific enolase (NSE) and protein gene product (PGP) 9.5 was seen in each component. Pancreatic polypeptide immunoreactivity was detected in eight cases, mainly in epithelial cells. Somatostatin immunoreactivity was present in epithelial and ganglion cells in nine cases. In seven cases immunoreactivity for neurofilaments, a marker for neurons, was seen in ganglion and spindle cells. However, immunoreactivity for chromogranin, a protein found in endocrine storage granules, was found in only two cases, and the staining was confined to well-granulated epithelial cells. The spindle cells were immunostained only for neuronal markers, NSE and neurofilaments, and the glial marker S-100 protein.
BACKGROUND: Metastasis of a paraganglioma (PRG) to the calvarium is very rare. In this paper, the case of a 25-year-old male with metastasis of a PRG to the frontoparietal bone is described. CASE DESCRIPTION: The patient presented with bulging on the left side of the head, headache, and weight loss. Magnetic resonance imaging (MRI) revealed a mass lesion in the left frontoparietal region that had destroyed both the external and internal table of the bone, extending under the skin and above the dura mater. After a frontoparietal craniotomy the tumor was removed totally. Histopathological examination revealed the "Zellballen," which are pathognomonic for a PRG. Systemic examination and radiological investigation revealed no primary tumor source. CONCLUSION: Metastasis of a PRG to the calvarium is possible; radical removal of the tumor will provide a cure.
A nonchromaffin paraganglioma was found in the periglandular connective tissue of the glandula suprarenalis of a sheep-dog bastard and characterized by histological and immunohistochemical techniques. Insulin-, glucagon- and gastrin/cholecystokinin-like material was revealed within the cells of the tumour. Possible functional consequences of these findings are discussed.
A 66-Year-old man presented with diplopia, headache and dizziness. Magnetic resonance imaging showed a right intrasphenoidal paracavernous mass. A biopsy was performed by endonasal route in the lesion which appeared to be part of the hypophyseal fossa. Histological examination revealed a highly cellular tumoral proliferation, with a variable architecture, of which chief cells were immunoreactive for neuroendocrine markers and spindle-shaped cells positive for PS 100. Morphological features were consistent with the diagnosis of paraganglioma. Our observation is the first case of this rare tumor reported in the French literature.
In this paper, we describe the clinical course of a 61-year-old female patient with paraganglioma in the head and neck region. Computed tomographic scan (CT), magnetic resonance imaging (MRI), ultrasound scan (US) and arteriogram findings initially led us to suspect that this tumor originated in the vagal nerve. In particular, a color Doppler US enabled an easy diagnosis of hypervascular tumor. We removed this surgically, but the tumor was easy to peel from the vagal nerve and carotid bifurcation. The distal side of the tumor was under the digastric muscle and running into the hypoglossal nerve. The intraoperative findings were highly suggestive of localization at the hypoglossal nerve, although Xth and XIIth cranial nerve palsies have remained.
Approximately 20 cases of malignant vagal paragangliomas (MVP)have been reported in English literature. Malignancy is based on the presence of metastases. A careful preoperative evaluation is necessary to detect multicentricity and/or significant production of catecholamines. A new case of MVP treated with embolization and surgery is presented and the literature discussed. It is concluded, that preoperative embolization followed by radical surgical resection is a rational treatment of patients with unilateral MVP.
We have applied a new method of preoperative embolization to an intravagal and to carotid body paraganglioma, using estrogen dissolved in absolute alcohol and polyvinyl alcohol particles, which diffusely embolizes vessels from capillaries to main feeders. Total resection of the tumors after embolization was successively performed without postoperative complications; total blood loss was 205 and 130 ml, respectively, and the surgical time was approximately 3 hours in both cases. The technique, characteristics, and advantages of this method are discussed.
Dosimetry and therapeutic application of [(131)I]-Tyr3-octreotide were evaluated in three patients with metastatic paraganglioma and carcinoid tumor. The in vitro stability of [(131)I]-Tyr3-octreotide was verified. Tumor uptake and residence time were between 0.02 and 0.1% and 0.5 to 9.8 h, respectively. The calculated tumor radiation doses were between 0.105 and 0.696 mGy.MBq(-1). No intolerance or adverse effects were observed after the therapeutic doses (3.3-6.6 GBq). A partial tumor response was obtained in one patient and no response occurred in two patients.
A 26-year-old man presented with left leg pain and progressive paraparesis. Imaging revealed a large intradural tumour compressing the cauda equina. The lesion was radically resected and histological analysis revealed it to be a paraganglioma. The clinical features of this rare tumour are described with a review of the literature.
A case of non-chromaffin paraganglioma of the nasopharynx is presented. Previously reported cases are discussed and classified, and theories of origin are reviewed in the light of new evidence suggesting a common origin for this rare tumour and juvenile angiofibroma of the nasopharynx.
Von Haller, in 1743, first described the carotid body and was so impressed with the richness of its innervation that he called it 'ganglion minutum', thinking it to be a sympathetic ganglion. Valentine, in 1840, saw a peculiar structure in the tympanic canaliculus in which a nerve cell was present and called it 'gangliolum tympanicum'. Almost 100 years later, in 1941, Stacey Guild reported the presence of some structures near the wall of the jugular bulb and described them as 'glomus jugulare'. Following this report, Rosenwaser (1945) and Otani were able to make a correct classification of these tumours in the middle ear. There have been innumerable reports of them and various authors have called them by various names. We report here a case of paraganglioma which was localized entirely to the tympanic membrane without involving any other part of the middle-ear cleft.
A case of metastatic laryngeal paraganglioma is presented. We have reviewed the current world literature on this entity and thereby identified two apparently distinct types of this rare lesion (type I and type II). The histopathological features are unhelpful in discriminating these two types and this is instead based on clinico-epidemiological differences. The latter are annotated and discussed with respect to prognosis and management. A true estimate of the frequence of type II (potentially malignant) awaits the utilization of the recent improvements in diagnostic techniques.
A rare case of paraganglioma of the external auditory canal is presented. It was treated by excision and no recurrence has been noted after 18 months of follow up.