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PubMedDate not supplied

Remarkable regression of malignant paraganglioma in the retroperitoneum and neck after chemotherapy: report of a case and a review of the literature.

The present communication describes a patient with paraganglioma found in the retroperitoneum and neck. She was treated with the combination chemotherapy employing cyclophosphamide, doxorubicin, and cisplatin (CAP therapy), and resulted in remarkable regression of the tumor in size. We reviewed the literature about the therapy of paraganglioma and the nature of retroperitoneal paraganglioma in relation with this case.

Aclarubicin↗

PubMed1999

Paraganglioma in the frontal skull base--case report.

A 56-year-old female presented with a paraganglioma in the left anterior cranial fossa who manifesting as persistent headache. Computed tomography and magnetic resonance imaging showed a solid, enhanced tumor with a cystic component located medially. The tumor was attached to the left frontal base and the sphenoid ridge. Angiography demonstrated a hypervascular tumor fed mainly by the left middle meningeal artery at the left sphenoid ridge. The preoperative diagnosis was meningioma of the left frontal base. The tumor was totally resected via a left frontotemporal craniotomy. Histological examination revealed the characteristic cellular arrangement of paraganglioma generally designated as the "Zellbaren pattern" on light microscopy. Only 10 patients with supratentorial paraganglioma have been reported, seven located in the parasellar area. The origin of the present tumor may have been the paraganglionic cells which strayed along the middle meningeal artery at differentiation.

Cerebral Angiography↗

PubMed2006

Extraadrenal paragangliomas of the body: imaging features.

OBJECTIVE: This article illustrates the anatomic distribution of the healthy paraganglion system and the imaging and pathologic features of various extraadrenal paragangliomas occurring in the head and neck, chest, and abdomen. CONCLUSION: Although paragangliomas can occur in a variety of anatomic locations, the majority are seen in relatively predictable regions of the body. Extraadrenal paragangliomas have nearly identical imaging features, including a homogeneous or heterogeneous hyperenhancing soft-tissue mass at CT, multiple areas of signal void interspersed with hyperintense foci (salt-and-pepper appearance) within tumor mass at MRI, and an intense tumor blush with enlarged feeding arteries at angiography.

Adult↗

PubMed1987

MR imaging of paragangliomas.

MR imaging of 15 paragangliomas in 10 patients was compared with CT of 13 of the lesions in eight patients. All lesions were confirmed with angiography. All lesions were detected by MR and CT with the exception of one small glomus tympanicum tumor that was seen only in retrospect with MR. CT better demonstrated subtle osseous changes of the skull base and the relation of the tumor to the middle ear structures. MR better demonstrated the relation of the tumor to the adjacent internal jugular vein and carotid artery. The paragangliomas had a characteristic MR appearance based on their vascularity. Serpiginous areas of signal void representing high vascular flow were interspersed among areas of high signal intensity caused by slowly flowing blood and tumor cells. This "salt-and-pepper" pattern was seen in all lesions greater than 2 cm in maximal dimension. MR was therefore able to accurately characterize the tumors as highly vascular. Multiplanar imaging and good tissue contrast and anatomic detail permitted display of the relations of these neoplasms to surrounding carotid sheath vessels and to intracranial structures better than did CT. In this experience, the MR appearance of paragangliomas was quite characteristic and differed markedly from meningiomas, neuromas, and metastatic disease of the skull base.

Carotid Body Tumor↗

PubMed1994

MR diagnosis of paraganglioma of the head and neck: value of contrast enhancement.

OBJECTIVE: Contrast enhancement with gadopentetate dimeglumine has been advocated to increase the efficacy of MR imaging for paragangliomas of the head and neck. However, contrast media are expensive, time-consuming to use, and involve minimal but not negligible risks. The purpose of this study was to determine if the use of contrast material is warranted in patients undergoing MR imaging for the diagnosis of paragangliomas of the head and neck. MATERIALS AND METHODS: Unenhanced MR images were compared with images obtained after administration of gadopentetate dimeglumine in 23 healthy subjects and 37 patients who had a total of 71 tumors. Three combinations of sequences were reviewed independently and in a random order by four observers who had no clinical information. Combination A comprised enhanced and unenhanced T1-weighted sequences, combination B comprised unenhanced T1- and T2-weighted sequences, and combination C was a combination of all sequences. A four-point scale of certainty was used. CT, scintigraphic, angiographic, and surgicopathologic findings were used as the standard of reference. Results were subjected to alternative free-response receiver-operating-characteristic (AFROC) scoring and statistical analysis. RESULTS: The mean areas under the AFROC curve for combinations A, B, and C were 0.761, 0.856, and 0.827, respectively. Mean sensitivity/specificity values after dichotomizing the scoring results were 0.73/0.94, 0.79/0.95, and 0.78/0.94 for combinations A, B, and C, respectively. The performance of combinations B and C did not differ markedly, but both combinations were significantly better than combination A. In a relatively large percentage (36%) of small postoperative tumor residues not detected on unenhanced images, however, gadopentetate dimeglumine allowed detection. CONCLUSION: The results of this study indicate that, in general, the use of gadopentetate dimeglumine is not necessary for the detection of head and neck paragangliomas. The addition of contrast-enhanced imaging does not increase the sensitivity or specificity compared with imaging without enhancement. Only when searching for small postoperative tumor residues is the addition of gadopentetate dimeglumine warranted.

Adult↗

PubMed1985

Paragangliomas of the head and neck: ultrastructural and immunohistochemical analysis.

Eighteen head and neck paragangliomas were studied by light microscopy and light microscopic immunohistochemistry by the peroxidase technique for the presence of NSE (neuron-specific enolase), serotonin, and a battery of neuropeptides. Seven of these tumors were also studied by electron microscopy. All 18 cases demonstrated immunostaining for NSE; 10 of the 11 carotid body tumors had immunostaining for multiple hormones. Considering all 18 cases, the most frequently demonstrated hormonal substances were in order: serotonin, leu-enkephalin, gastrin, substance P, vasoactive intestinal polypeptide (VIP), somatostatin, bombesin, calcitonin, and alpha MSH. In several tumors, adjacent-step sections stained for different hormonal substances strongly suggested reactivity for more than one hormone in given tumor cells. By electron microscopy, all 7 cases studied displayed considerable heterogeneity of the neurosecretory granules with respect to size, shape, and electron density. This demonstrated that branchiomeric paragangliomas are capable of producing a spectrum of neuropeptides in addition to their known amine content. The presence of immunoreactive serotonin in most of these neoplasms was confirmed. In addition to these findings, neurofibrils within the substance of carotid body paragangliomas demonstrated immunoreactivity for somatostatin and a gastrinlike neuropeptide. The significance of the neuropeptides in these neoplasms and their possible presence and role in normal and hyperplastic paraganglia remain to be defined.

Carotid Body Tumor↗

PubMed1986

Crystals in a jugulotympanic paraganglioma.

The morphologic features of a jugulotympanic paraganglioma are reported. The tumor showed the usual histology of paragangliomas. No sustentacular cells were identified. In the tumorous chief cells there were typical neurosecretory dense-core granules 60-180 nm in diameter. Granules averaging 400 nm in diameter were also observed, sometimes with a regular rhomboid core or crystallized content. Rhomboid crystals were seen in the cytoplasm in membrane-bound spaces and in telolysosomes. The crystals had a substructure consisting of alternating light and dark lines with a periodocity of 5-10 nm. Such crystals had not been reported previously in paragangliomas. The possible origin of the crystals is discussed.

Crystallization↗

PubMed1990

Paraganglioma of the cauda equina. Report of three cases.

The authors report the clinical, radiological, and pathological findings in three cases of paraganglioma of the cauda equina. In one case, magnetic resonance imaging and neurochemical study results are described. No specific identifying features were encountered either clinically or radiologically that were helpful in making a distinction between this and other more common tumors at this site such as ependymoma or neurofibroma. At surgery, these neoplasms were well-circumscribed red fleshy tumors. Histological examination of one paraganglioma showed a superficial resemblance to ependymoma, and this may be particularly true on initial assessment by frozen section or smear. The use of electron microscopy and immunohistochemical demonstration of synaptophysin in these tumors allowed a confident diagnosis to be made. Neurochemical assessment in one case showed very high levels of serotonin and a turnover of dopamine similar to that of human cerebral cortex. Paraganglioma of the cauda equina is an uncommon tumor with just over 50 cases reported in the world literature. The clinical course of these tumors is benign and they should be completely removed at surgery to prevent later recurrence.

Adult↗

PubMed1991

Cervical paraganglioma with subsequent intracranial and intraspinal metastases. Case report.

Paragangliomas, tumors derived from the extra-adrenal paraganglion system, have commonly been found in the retroperitoneum, abdomen, mediastinum, skull base, and neck. Rare intraspinal cases have included involvement of the cauda equina and filum terminale, and a recent case has been reported of thoracic cord paraganglioma with metastasis to the cauda equina. The authors present the case of a patient with a cervical paraganglioma who underwent subtotal resection followed by postoperative irradiation; she subsequently developed multiple intracranial and intraspinal metastases 7 months following the first decompression procedure.

Adult↗

PubMed1995

Symptomatic cerebellar metastasis and late local recurrence of a cauda equina paraganglioma. Case report.

This case report contains a description of a 61-year-old patient who presented with a progressive truncal ataxia 22 years after complete removal of a small paraganglioma of the cauda equina. Magnetic resonance imaging of the neuraxis revealed a large cystic lesion in the cerebellar midline, three small cortical-to-subcortical nodular tumors in the posterior fossa, and local recurrences of the paraganglioma of the cauda equina. Pathological examination showed the cerebellar midline lesion to be a paraganglioma, most likely a metastasis from the cauda equina localization.

Adult↗

PubMed2001

MR findings of the spinal paraganglioma : report of three cases.

Extraadrenal paragangliomas involving the spine is less common and usually takes the form of intradural compression of the cauda equina. The authors report three cases of spinal paragangliomas resulting in extradural spinal cord compression and their MR findings. The MR imaging revealed a well-demarcated extradural mass with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images compared to paravertebral muscles. After Gd-DTPA administration, heterogeneous and intense enhancement was found. Multiple punctate and serpiginous structures of signal void due to high-velocity flow were noted around and within the tumors on all sequences. In one case, the signal void structures were well corresponded with feeding arteries on angiography. These may be the characteristic findings of the extraadrenal paraganglioma involving the spine.

Adult↗

PubMed2001

A case of hemorrhagic gallbladder paraganglioma causing acute cholecystitis.

Gallbladder paraganglioma is a very rare tumor and so far only a few cases have been reported. Most of these were asymptomatic and were found incidentally during operation. Recently, we experienced a gallbladder paraganglioma that gave rise to hemorrhage, which in turn caused acute cholecystitis. Our case involved a 45 year-old female patient complaining of an intermittent right upper abdominal pain. After a preoperative evaluation, cholecystectomy and lymphadenectomy were performed under the impression of gallbladder cancer with acute cholecystitis. Postoperative pathologic examination revealed a hemorrhagic gallbladder paraganglioma accompanied by acute cholecystitis. Immunohistochemical staining of the chief cells for neuron specific enolase, chromogranin and synaptophysin were positive. Sustentacular cells also stained positively for S100 protein.

Acute Disease↗

PubMed2000

A case of Cushing's syndrome in ACTH-secreting mediastinal paraganglioma.

Paragangliomas are unusual neuroendocrine cell tumors arising from paraganglia, of which ACTH-secreting cases in the mediastinum are extremely rare. A 51-year-old woman was admitted for generalized edema and weakness which began 5 months ago. Chest X-ray and CT scan revealed a tumor mass in the anterior mediastinum. The plasma cortisol and ACTH levels were very high. Other sources secreting ACTH, except mediastinal mass, were not found. Surgical excision of mediastinal mass and left supraclavicular lymph node was performed. The postoperative microscopic finding and immunohistochemical staining revealed organoid tumor cell nests (zellballen) and S-100 protein positive sustentacular cells which are characteristics of paraganglioma. This was thus a case of Cushing's syndrome resulting from ectopic ACTH production in anterior mediastinal paraganglioma.

Adrenocorticotropic Hormone↗

PubMedDate not supplied

Ectopic adrenocorticotropic hormone hypersecretion due to a primary pulmonary paraganglioma.

OBJECTIVE: To present a rare case of primary paraganglioma of the lung, which caused the syndrome of ectopic adrenocorticotropic hormone (ACTH) hypersecretion. METHODS: The clinical, biochemical, and imaging findings in this case are described, and the pathologic features of the resected tumor tissue are depicted. In addition, the related literature on paragangliomas is reviewed. RESULTS: In a 39-year-old woman with hypertension, weight gain, and easy bruising, laboratory studies showed hypercortisolemia, high plasma ACTH levels, and suppression of cortisol by high-dose dexamethasone. An indium-labeled octreotide whole-body scan disclosed a tumor in the left lower lung field. Thoracoscopic wedge resection of the pulmonary mass was performed. Postoperative microscopic findings and immunohistochemical stains revealed nests of rounded and polyhedral cells and S-100 protein-positive sustentacular (supporting) cells, charac- CONCLUSION: This unusual case of Cushing's syndrome was attributable to an ACTH-secreting primary pulmonary paraganglioma.

Adrenocorticotropic Hormone↗

PubMed1999

Intrasellar paraganglioma presenting as nonfunctioning pituitary adenoma.

Paragangliomas arising in the sellar region are rare. We report a case of intrasellar paraganglioma of a 54-year-old man who presented with gradually decreasing visual acuity. Physical examination revealed bitemporal hemianopsia with no apparent signs of endocrinologic dysfunction. Magnetic resonance imaging revealed a large sellar mass believed to be a nonfunctioning pituitary adenoma. The tumor was removed transsphenoidally and submitted for histologic examination. The morphologic features, based on histologic, immunocytochemical, and electron microscopic analyses, were consistent with the diagnosis of paraganglioma. The diagnostic morphologic features and the immunocytochemical profile of the tumor are reported.

Adenoma↗

PubMed2001

Pigmented cardiac paraganglioma.

A pigmented left atrial paraganglioma was found at autopsy in a 40-year-old black man who died unexpectedly. The cause of death was ascribed to coronary artery disease. The atrial mass was sharply demarcated and polypoid, measured 4 cm in greatest dimension, and had a cut surface that revealed dark red-brown soft tumor tissue. Histopathologically, the neoplasm exhibited a classic organoid clustering of cells (zellballen) with a prominent capillary network. The chief cells contained a brown-black pigment with histochemical characteristics of melanin. We report a case of pigmented cardiac paraganglioma because of its rarity. To our knowledge, no mention has been made of the presence of pigment in previously reported cases of cardiac paragangliomas.

Adult↗

PubMed1998

[Two cases of retroperitoneal functioning paraganglioma].

We report two cases of retroperitoneal functioning paraganglioma. Case 1: A 52-year-old male patient was admitted to our hospital for further examination of pulsating abdominal pain with chest pain and headache. Overfist-size smooth-surfaced hard mass was palpated at the right upper abdominal quadrant. Catecholamine levels in serum and urine showed marked elevation. Computed tomographic (CT) scan, magnetic resonance imaging (MRI) and angiography revealed a large tumor between the right kidney and aorta. He underwent surgical removal of the tumor and histological examination revealed paraganglioma. His chief complaints disappeared postoperatively. Case 2: A 68-year-old female patient was admitted to our hospital for control of diabetes. An abnormal mass was shown ultrasonically at the left renal region by routine screening examination. A marked increase of noradrenaline in serum and urine was observed. Abdominal CT scan, MRI and angiography revealed a retroperitoneal tumor which was located adjoining to the lower pole of the left kidney. The tumor was removed transabdominally. Histopathological studies showed paraganglioma. After the operation her blood sugar and noradrenaline levels decreased to the normal range.

Aged↗

PubMed1999

Bone metastases from a paraganglioma. A review of five cases.

Paragangliomas are infrequent, usually benign tumors developed from neuroectoderm cells. The neck is the most common location, although some cases arise within the abdominal cavity, usually in the retroperitoneal space. We report five cases with bone metastases. In three patients, convincing evidence was obtained that the primary was in the retroperitoneal space. Clinical manifestations of metastatic bone disease occurred up to 17 years after the diagnosis of paraganglioma. Useful data were obtained from plain radiographs, magnetic resonance imaging, serum and urine catecholamine assays, and above all meta 123iodobenzylguanidine scintigraphy. Histologic and immunohistochemical studies of the lesion yielded the definite diagnosis. Surgery and radiation therapy are the two mainstays of therapy. Although rare, metastatic forms of paraganglioma should be borne in mind. This diagnosis should be entertained in patients with bone lesions and recent-onset arterial hypertension, irrespective of whether they report a history of surgery for a tumor, and even if this tumor was removed many years earlier and labeled benign.

Adult↗