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We present the CT and MR findings of an intrasellar and suprasellar paraganglioma in a 17-year-old girl. Paragangliomas in this location are rare. The lesion had several imaging features characteristic of paragangliomas including marked enhancement after contrast and multiple vascular flow voids.
The authors describe the magnetic resonance imaging (MRI), computed tomographic, ultrasonographic and angiographic appearance of a giant nonfunctioning retroperitoneal paraganglioma. To their knowledge, the MRI appearance of a nonfunctioning paraganglioma has not been previously described. The MRI characteristics of the nonfunctioning tumour were similar to those of a functioning paraganglioma and included hyperintensity in T2-weighted images and intense enhancement after intravenous administration of contrast agent.
The use of radiation therapy for the treatment of paragangliomas of the temporal bone remains controversial. Few studies exist that provide sufficient information on staging the long-term follow-up to clarify this issue. A retrospective study was undertaken of 38 patients with temporal bone paragangliomas treated with radiation therapy between 1956 and 1991. All patients received radiation therapy, either as a primary modality (14 patients), in combination treatment with surgery (13 patients), or as salvage therapy (11 patients). Mean disease stage (Fisch classification system) for each group was C2, B, and C1, respectively. The follow-up period ranged from 1 to 27 years (median 11.5 yr). Local control was achieved in 79% of the primary radiation therapy group, 100% of the combined treatment group, and 91% of the salvage therapy group. Complications resulting from radiation therapy were few and minor. This study demonstrates that the use of radiation therapy for temporal bone paragangliomas offers effective local control as a primary treatment modality, in combination with surgery, and as salvage therapy with few long-term complications.
OBJECTIVE: To describe the 123-I-MIBG scintigraphic, CT, MRI, operative and pathological findings in a case of malignant prostatic paraganglioma and to review the literature on this very rare tumor. EXPERIMENTAL DESIGN: Clinical imaging and pathological correlation of data in a referred patient. SETTING: Regional referral center and tertiary referral academic medical center. PATIENT: 17 year old man presenting with painless hematuria and a large prostatic mass. Interventions and measures. Renal ultrasound, transrectal ultrasound, ultrasound guided prostatic biopsy, pelvic CT and MRI, planar and SPECT 123-I-MIBG scintigraphy, and surgical exploration. RESULTS: The patient had a significant hydronephrosis of the left kidney and marked enlargement (120 ml) of the prostate gland by ultrasound. Ultrasound guided biopsies of the prostate and a left pelvic lymph node revealed a neuroendocrine tumor staining positive for chromogranin. CT and MRI revealed a large tumor of the prostate invading the seminal vesicles, bladder and rectum with extensive pelvic lymph node spread. The primary tumor and one of the nodes were shown to be 123-I-MIBG avid confirming the neuroendocrine nature of the tumor. The lesion was unresponsive to chemotherapy and unresectable at surgical exploration. CONCLUSIONS: To date there have only been 5 reports of prostatic paragangliomas. To our knowledge this is the first to have been studied by MIBG scintigraphy and like most paragangliomas it was MIBG-avid.
We report a case of medullary thyroid carcinoma with a paraganglioma-like pattern and melanin production. Macroscopically, a rectangular black area within a gray-white background was seen on the cut surface of the tumor. Histologically, the tumor was found to have a trabecular or nestlike architecture with many pigmented dendritic cells, resembling the "sustentacular cells" of paraganglioma, scattered among nonpigmented polygonal tumor cells. Fontana-Masson staining and bleaching with potassium permanganate and oxalic acid revealed that this pigment was melanin, which was also confirmed by electron microscopy. Immunohistochemically, pigmented dendritic cells were positive for calcitonin, as were the surrounding typical medullary thyroid carcinoma cells. These cells were also positive for S100 protein but not for HMB-45, compatible with sustentacular cells. These findings suggest that this tumor could be considered to bridge the gap between two variants of medullary thyroid carcinoma, the melanin-producing (pigmented) type and the paraganglioma-like type.
OBJECTIVE: To report a case of paraganglioma localized in the organ of Zuckerkandl and to discuss its diagnosis, treatment and outcome. METHODS/RESULTS: A 37-year-old female presented with a periumbilical mass and pain that radiated to the lumbar region for the past three months. Abdominal US and CT disclosed a well vascularized mass lying adjacent to the aorta, 1 cm from its bifurcation. At laparotomy, an 8 x 10 cm tumor was discovered at the aortic bifurcation that displaced the left ureter and included the inferior mesenteric artery. Pathological analysis of the surgical specimen revealed a paraganglioma. CONCLUSION: Extra-adrenal paraganglioma should be taken into account in the differential diagnosis of retroperitoneal masses, particularly those adjoining the abdominal aorta.
We report a case of paraganglioma of the cauda equina and review the MRI features in 11 previously published cases with MRI description. On T1-weighted images, paraganglioma is isointense relative to spinal cord and shows moderate to intense enhancement after Gd-DTPA application, usually with heterogeneous texture. On T2-weighted images, different findings were found. MRI features do not allow to distinguish paraganglioma of the cauda equina from majority of other tumors in this region.
A rare case of a paraganglioma of the filum terminale is reported. The patient presented with headache and papilledema. Magnetic resonance imaging demonstrated enlarged ventricles of the brain and a well-demarcated intradural spine tumor. After resection of the tumor, the papilledema and headaches resolved, and the ventricles returned to normal size. Pathologic examination revealed a paraganglioma of the filum terminale. We present this rare case and review the literature of spinal paragangliomas as well as the association of spinal tumors with hydrocephalus.
BACKGROUND: A case of a renin-producing paraganglioma of adrenal origin with metastases to the retroperitoneal area, paravaginal area, and the ovary is reported with immunohistochemical findings indicating expression of multiple neuropeptide immunoreactivities. The patient was 23 years old at the time of diagnosis, and died from metastatic spread of the tumor 7 years later. METHODS: Tumor tissue was examined by light microscopy, indirect immunohistochemistry, and electron microscopy. RESULTS: The tumor tissue investigated contained several cells exhibiting opioid peptide-like immunoreactivities (i.e., enkephalin and dynorphin-like immunoreactivity [LI]). A lower number of cells displayed neuropeptide Y-, galanin-, somatostatin-, neurotensin-, substance P-, peptide histidine-isoleucine-, cholecystokinin-, renin-, and calbindin-LI. CONCLUSION: To the authors' knowledge, galanin, dynorphin, peptide histidine-isoleucine, cholecystokinin, and calbindin have not been reported previously to occur in paraganglioma, and renin has been reported to occur very rarely. A review of recent literature suggest that enkephalin-, neuropeptide Y-, and somatostatin-like immunoreactivities may be useful as diagnostic markers for paragangliomas.
BACKGROUND: Paragangliomas of the carotid bifurcation are rare and usually benign neoplasms arising from the carotid body chemoreceptors. The aim of this retrospective study was to report long-term results of carotid bifurcation paragangliomas treated in a single institution over a period of 20 years. The following variables are described: age, sex, localization, diagnostic workup, operative procedure, complications, proportion and characteristics of malignant tumors, and cure rates. METHODS: Thirty-six patients,10 men and 26 women, were treated. Twenty-three patients had tumors on the right side, 10 patients had tumors on the left side, and three patients had bilateral tumors. All patients were symptomatic. The most common complaint was a neck mass that had been present for 1 to 144 months. All patients had diagnostic procedures done before treatment. The most widely used was ultrasonography (26 patients). Angiography was performed in 14 patients. RESULTS: All patients underwent complete tumor resection. In three cases, resection of the internal carotid was necessary. In all patients who underwent internal carotid resection, immediate reconstruction was performed. No patients had postoperative strokes. Five patients had postoperative nerve palsies. During the follow-up period of 4 months to 12 years, no patients had local recurrence develop. Four patients had distant metastasis. CONCLUSION: The complete removal of paragangliomas of the carotid bifurcation is effective with acceptable morbidity. All recurrences of the malignant tumors were distant metastasis.
PURPOSE: Three cases of malignant carotid body paragangliomas with nodal metastases are reported. PATIENTS AND METHODS: Between 1985 and 1994, 3 female patients (51 to 65 years of age) were referred for postoperative radiotherapy after complete (2) or incomplete (1) surgical excision of a malignant carotid paraganglioma (Shamblin III). Preoperative angiographic embolization of the tumor-supplying arteries was performed in all cases. In 2 patients resection of the internal carotid artery and reconstruction by saphenous vein graft was necessary. Continuous course radiotherapy of the tumor bed (50 to 56 Gy/2 Gy) and regional lymph nodes (50 Gy) using photon beams was delivered in 2 patients. The third patient having had incomplete resection cancelled radiotherapy after 4 Gy. RESULTS: Within an observation time of 110 and 119 months no evidence of recurrence was obtained in both patients irradiated postoperatively. The third patient died of progressive disease. Twelve months after the withdrawn irradiation she presented with a tumor progression into the brain and an ulcerated mass on the right side of the neck and was irradiated consecutively for palliation (Figures 1a to 1f). In none of the patients severe acute or late radiation-induced complications were observed. CONCLUSION: In patients with malignant paraganglioma moderate dose postoperative radiotherapy of the tumor bed and regional lymph nodes is well tolerated. It seems to be effective to prolong local control after surgery, to eradicate microscopic lymphatic disease and eventually to postpone further spreading.
A paraganglioma is a rare tumor composed of chromaffin cells. Malignant paraganglioma is a very rare presentation diagnosed by local recurrence after total resection of the primary mass or findings of distant metastasis. We report a case of a 30-year-old man who had undergone a carotid body tumor resection 10 years before and who presented to us with recurrence of the tumor associated with lymph node, vertebra and skull metastasis. The diagnosis of malignant paraganglioma was based on loco-regional and distant metastasis.
The present study reviews in concise form the past 12 years of our clinical experience with paragangliomas of the carotid body. Every aspect of the anatomical, histological and biological and biological behaviour of paragangliomas of the carotid body has been recorded in order to be able to define better surgical management and the clinical prognosis. In addition to the conventional histological methods of investigation we also applied immunohistochemistry and made use of electron microscopy. Eighteen tumours of the carotid body in 12 female and four male patients with a mean age of 54 years (34 to 70 years) were treated surgically. Angiography of the aortic arch and supra-aortic branches was undertaken diagnostically in every case. In three patients computer tomography and magnetic resonance imaging demonstrated spreading of the tumour up to the base of the skull and pre-operative embolization of the arteries supplying the tumour was performed to decrease tumour vascularity. In four cases (22%) resection of the internal carotid artery and its reconstruction by a saphenous vein graft was required in order to achieve the therapeutic aim of a complete extirpation of tumourous tissue and preserving the vascular system of the internal carotid artery. In three cases (16%) there was histological evidence of a metastasizing process to the local lymph nodes of the neck and, due to this criterion of malignancy, 50-60 Gy of radiation was applied to the site of the tumour after surgery. Four patients experienced a transient neurological deficit in the supply area of the hypoglossal and the recurrent nerves. In one case, the vagus nerve could not be retained. In an average follow-up of 102 months we recorded one case of local recurrence 13 months after the initial resection and radiation treatment applied to a malignant paraganglioma. All the other patients were doing well without evidence of recurrent disease.
In 1982, Fisch described his results for the surgical treatment of 74 paragangliomas of the temporal bone, 5 years after his description of the infratemporal fossa approaches (types A and B). This study reviews the subsequent experience of the Department of Otolaryngology-Head and Neck Surgery of the University of Zürich with more than 136 surgically treated cases of paraganglioma of the temporal bone and discusses our current therapy 20 years after the initial description. One hundred nineteen (90%) of the patients had advanced tumors (Fisch class C or C+D), and 81 (68%) had intracranial extension. Total tumor excision was possible in 109 (82%) patients. Subtotal excision was performed in 22 (17%) patients, 21 of whom had intradural tumor invasion. In these cases, the resection was limited not by actual tumor size but by the degree of intracranial intradural tumor extension. Partial tumor excision was undertaken in only 1 patient with a C4De2Di2 tumor. The success rate in preservation of function of the lower cranial nerves was encouraging. Of the 69 patients whose facial nerve status was followed post-operatively, 81% maintained Fisch grade 76 to 100% (House-Brackman grades I and II). Analysis of follow-up data ranging from 2 to 11 years demonstrated 98% disease-free survival when total tumor extirpation was possible. In the patients who underwent subtotal or partial surgical resection there has been no subsequent tumor growth detected by either clinical or neuroradiological evaluation. We have confirmed after more than 20 years of experience that the infratemporal fossa approaches are a safe, highly effective means of surgical management of paragangliomas of the temporal bone, allowing eradication or arrest of disease with minimal morbidity. Limited intradural surgical resection in cases of very extensive tumors can greatly benefit patients for whom complete excision is not an option.
Paragangliomas (glomus tumors) comprise 15% of all neoplasms at the skull base. Despite extensive growth, these tumors usually do not secrete active biogenic substances into the circulation in sufficient quantities to produce symptoms. When they do secrete large amounts of catecholamines, they will cause symptoms that mimic a pheochromocytoma. The still confusing nomenclature of paragangliomas is reviewed, and the clinical work-up, surgical treatment, and follow-up of five patients with catecholamine-secreting paragangliomas of temporal bone (3), infratemporal fossa (1), and nasopharynx (1) are presented and discussed.
To determine if recent trends in evaluation and therapy have contributed to the successful surgical management of carotid body paragangliomas, we reviewed our experience over the past decade. Nineteen carotid body paragangliomas were identified in 17 patients. Eleven patients underwent complete, preoperative embolization of their afferent arteries with one complication. Calculated carotid body paragangliomas surface areas did not differ between the embolized 64.6 +/- 43.3 cm2 and nonembolized 63.0 +/- 57.9 cm2 lesions. Intraoperative blood loss was lower (p = 0.02) in the patients treated with embolization (372 +/- 213 ml) compared with their cohorts (609 +/- 564 ml). However, the operative times were equivalent 4.1 hours versus 4.5 hours in both groups. Intraoperative electroencephalographic (EEG) monitoring was used in 10 patients; in one patient the EEG indicated intraoperative thrombosis of the carotid artery, which was successfully treated by thrombectomy without complications. Two patients required carotid bifurcation resection and vascular reconstruction to remove the entire tumor; a late stroke manifested by contralateral hand weakness developed in one of these patients. The incidence of cranial nerve injury was low at 16%, with one transient ramus mandibularis paresis and two instances of vocal cord dysfunction. Two additional patients had a postoperative Horner's syndrome. We conclude that by diminishing intraoperative blood loss through complete and careful preoperative embolization and use of intraoperative EEG monitoring along with careful surgical technique, the complications associated with this challenging operation are facilitated and diminished.
The avascular paraganglioma described in this article appears to be the second such tumor reported in the international literature and the first to be reported in the tympanojugular region. Despite a highly suggestive history and clinical appearance, the tumor showed no signs of vascularization on radiologic studies. The pathologic postoperative study confirmed the diagnosis of paraganglioma with extensive stromal fibrosclerosis and without the typical well-vascularized thin fibrous septa. In the authors' opinion, this observation is notable because of the difficulties encountered in the correct diagnostic interpretation of an avascular mass in the tympanojugular region. In such cases, the possibility of a paraganglioma should always be considered.
OBJECTIVE: To review the cytologic features and potential pitfalls of pheochromocytoma and retroperitoneal paraganglioma and to evaluate complications of the aspiration procedure and the diagnostic utility of immunocytochemistry. STUDY DESIGN: We reviewed 15 cytologic specimens from 12 patients with 13 tumors (1 bilateral case). Ten were adrenal (pheochromocytomas) and 3 extraadrenal paragangliomas. Eleven specimens were from fine needle aspiration (FNA) procedures that were performed in collaboration with radiologists using 23-25-gauge needles. In 3 patients the cytologic material was obtained during intraoperative diagnosis. Immunocytochemistry was performed on alcohol-fixed smears. RESULTS: Two aspirates were hypocellular, while the remainder were cellular. Cells were distributed singly or formed discohesive groups. When present, cytoplasm was abundant and ill defined. Most cells had an eccentric nucleus and plasmacytoid morphology. Nuclear pleomorphism, binucleation and multinucletaion, naked nuclei and intranuclear preudoinclusions were common findings. In 2 cases a lipid background was seen focally. Evident cytoplasmic immunoexpression of synaptophysin or chromogranin was detected in the 10 cases analyzed. One patient developed a hypertensive episode during the FNA procedure. It was controlled medically without complications. CONCLUSION: When adequate cytologic material is present, the recognition of pheochromocytoma and extraadrenal paraganglioma is possible. Together with morphology, immunocytochemical studies allow a specific preoperative diagnosis. Scarce material can be a source of diagnostic errors. FNA of pheochromocytomas is not necessarily contraindicated. When analytic data are not diagnostic, FNA may follow. Aspiration must be performed in an area equipped with the therapeutic tools necessary to control a pheochromocytoma crisis.