1/23. Visualisation of a paraganglioma by technetium-99m-sestamibi scintigraphy.A 68-yr-old woman presented to our observation with multinodular goiter and a contiguous right laterocervical mass. In spite of ultrasound, technetium and iodine scan, CT and fine-needle biopsy, the precise origin of the mass remained uncertain. On additional multi-phase sestamibi scan, the neck region showed an early high uptake rapidly decreasing over time in the laterocervical mass, and a persistent inhomogeneous distribution in the thyroid gland. This behavior suggested that the laterocervical mass could derive from an anatomical structure other than the thyroid. Surgical exploration established the extrathyroid nature of the laterocervical mass and the histological examination confirmed that it was a typical paraganglioma. This finding is in keeping with a recent report of positive sestamibi uptake in a cervical paraganglioma, although our case showed a more rapid kinetic. This tumor should be therefore taken into consideration in the differential interpretation of focal sestamibi uptake.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/23. Metastatic paraganglioma causing spinal cord compression.We describe two cases of metastatic retroperitoneal paraganglioma associated with extradural spinal cord compression. Both occurred in young men; one being metastatic at presentation, the other becoming metastatic 19 years after attempted surgical resection. Despite a long, relatively stable natural history after diagnosis (10 and 19 years, respectively) both had an acceleration of their disease once extradural disease was detected. These cases illustrate the potentially aggressive nature of this disease, the need for long-term follow-up and the effectiveness of a variety of therapies for palliation, and also raise the possibility of "prophylactic" treatment to prevent spinal cord compression.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/23. Duodenal gangliocytic paraganglioma as a radiological moving defect.BACKGROUND: Gangliocytic paraganglioma of the duodenum is a peculiar neuroendocrine tumor, often revealed as an incidental radiographic finding or as a result of gastrointestinal hemorrhage, due to frequent ulceration of the overlying mucosa. Most lesions are pedunculated and submucosal with distinctive histology, consisting of endocrine, ganglion and spindle-shaped schwann cells. methods: A case of a duodenal gangliocytic paraganglioma is reported in a 50-year-old woman presenting with episodes of melena. Enteroclysis revealed a pedunculated tumor observed either in the second or third portion of the duodenum. Upper gastrointestinal endoscopy revealed a pedunculated ulcerated tumor which was subsequently confirmed by computed tomography. The tumor was simply excised through a duodenotomy. RESULTS: The size of the tumor was 4.5 x 3 x 1.5 cm. Immunohistochemical and ultrastructural features of the tumor classified it as a typical duodenal gangliocytic paraganglioma with positive cellular reaction for neuron-specific enolase, chromogranin, somatostatin, gastrin and S100. CONCLUSION: Forty-six months after surgery, the patient is well with no evidence of recurrence. The majority of the reported duodenal gangliocytic paragangliomas were of benign nature. However, the fact that in 4 isolated cases there was lymph node involvement indicates that thorough investigation for lymph node metastases is needed, as well as thorough postoperative follow-up.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/23. Duodenal periampullary gangliocytic paraganglioma: report of two cases with immunohistochemical and ultrastructural study.We report two cases of Gangliocytic paraganglioma (GP) of the ampulla of vater occurring in a 63-year-old and a 34-year-old individual. The patients were both admitted for a long history of intermittent gastrointestinal bleeding and abdominal discomfort, with no other symptoms. At endoscopy, the GP appeared as a polypoid, ulcerated mass in the ampullar region, measuring 2.5x1.8 and 2 cm, respectively. Microscopically, the tumors showed similar features and were composed of epithelial cells (more than 50%), spindle cells, and ganglion-like cells. The epithelial cells showed clear cytoplasm and formed nests (zellballen or paraganglioma-like groups), and less frequently, cords (carcinoid-like), extending to mucosa and submucosa. Ganglion cells were sparse, constantly associated with the spindle cells. Both epithelial and ganglion cells were synaptophysin, chromogranin a, and anti-neurofilament immunoreactive. The spindle cells were all S-100 positive. Ultrastructural studies revealed dark and light cells, rare elongated cellular processes, secretory granules, and fine fibrils resembling neurofilaments. The histogenesis of GP is still a matter of debate, however its neoplastic nature is supported by the occasionally reported malignant evolution.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/23. paraganglioma of the pituitary fossa: diagnosis and management.paraganglioma of the sellar area is extremely rare with only six cases having been reported in the literature. Surgical removal of these tumors is difficult, and the transsphenoidal approach usually results in limited resection. Most authors who published reports on this tumor recommended radiation therapy after partial removal of the tumor. However, considering the benign nature of these tumors, the risk of radiation-induced endocrine insufficiency and optic neuropathy and the lack of proven effectiveness of radiotherapy, its value remains controversial. We describe a 48-year-old woman with parasellar paraganglioma who presented with headaches, visual loss and oligomenorrhea. magnetic resonance imaging (MRI) showed an invasive tumor in the sellar and parasellar areas which extended to both cavernous sinuses and compressed the optic chiasm and the left internal carotid artery. Surgery by the transsphenoidal approach enabled only limited biopsy of the tumor. The patient was reoperated by an extended pterional approach which resulted in a subtotal removal of the tumor and adequate decompression of the adjacent structures. She received no adjuvant treatment during the 8-year postsurgical follow-up and remained in good health. A repeated MRI showed no change in the size of the residual tumor. Contrary to the therapeutic recommendations described in previous reports, we favor postoperative adjuvant therapy only if the symptoms or signs of cranial nerve compression persist following maximal tumor removal, or if there is evidence of subsequent growth of residual tumor.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/23. Sinonasal paraganglioma: endoscopic resection with a 4-year follow-up.BACKGROUND: Paragangliomas are uncommon neuroendocrine tumors. In the head and neck, they are most commonly seen at the carotid body, the temporal bone, or along the vagus nerve. Sinonasal paragangliomas are extremely rare. Most cases reported describe management by open surgical resection, with sublabial and/or transfacial approaches. methods: We present a case of a large sinonasal paraganglioma successfully managed by endoscopic resection. RESULTS: The patient has no evidence of recurrence after 4 years of follow-up. CONCLUSIONS: This case demonstrates that sinonasal paragangliomas might be amenable to endoscopic resection. However, the most important priority to consider when selecting a surgical technique is whether adequate excision with clear margins can be obtained. Furthermore, because of the aggressive nature of these tumors, regardless of the surgical approach, long-term follow-up is recommended.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/23. Emergency catheter probe endoscopic sonography (CP-EUS): an effective procedure in the assessment of a bleeding gangliocytic paraganglioma.Catheter probe endoscopic sonography is a relatively rapid and safe procedure, carried out during standard endoscopy, capable of distinguishing solid from cystic lesions and vascular from avascular masses. Herein we discuss the role of catheter probe endoscopic sonography in the emergency assessment of a patient with recent and severe bleeding from an ulcerated polyp, arising from the papilla. During the endoscopy, catheter probe endoscopic sonography showed the solid and submucosal nature of the lesion, suggesting its localised and benign nature and, most importantly, demonstrating the high risk of rebleeding from vascular structures communicating with the ulcer. Because of this finding three metallic endoclips were positioned. The lesion was not removed endoscopically because of its difficult position and the high risk of haemorrhage. The mass, removed through a duodenotomy, was shown to be a gangliocytic paraganglioma, an uncommon tumour, frequently resulting in surgery because of abdominal pain and gastrointestinal bleeding.- - - - - - - - - - ranking = 2keywords = nature (Clic here for more details about this article) |
8/23. Suprasellar and intrasellar paragangliomas.neoplasms of the sellar region are entities with a large differential diagnosis. Although paraganglionic cells have not been demonstrated in the pituitary or adjacent structures, the existence of sellar region paragangliomas is well-documented. To elucidate, in this area the nature of these unusual tumors is relatively difficult. Clinical history, physical examination, radiographic investigation as well as intraoperative gross observation are the same as those of sellar meningioma or pituitary adenoma. immunohistochemistry, using neuroendocrine markers and electron microscopy are the two definitive diagnostic methods to differentiate among these entities. The clinical management, the possible pathogenesis of the tumor, the importance of immunohistochemistry in making the diagnosis and the clinical outcome of these patients are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/23. paraganglioma of the vulva.A case of vulvar paraganglioma is reported. Following presentation with vulvar pain, a 1-cm nodule was excised from the labium minus of a 58-year-old woman. Histologically, the tumor was composed of nests of round eosinophilic cells with moderately pleomorphic nuclei, beneath an intact squamous epithelium. Ultrastructural studies indicated two cell types within the neoplasm: chief cells with numerous small neurosecretory granules and peripheral slender sustentacular cells. A reticulin stain confirmed the "zellballen" nature of the neoplasm, and the neoplastic cells showed moderate argyrophilia on a Grimelius stain. The immunoperoxidase stains for chromogranin and neuron-specific enolase were strongly positive in the neoplastic chief cells. Immunostaining using anti-S-100 antibody confirmed the finding of sustentacular cells by identifying many slender cellular processes among the chief cells. These light and electron microscopic findings are diagnostic of paraganglioma, an entity not previously reported in the vulva to our knowledge.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/23. The intriguing nature of gastric tumors in Carney's triad. Ultrastructural and immunohistochemical observations.The authors describe clinical and pathologic features present in an adolescent girl who had a gastric tumor and mediastinal mass. The latter was shown to be a paraganglioma, and the gastric neoplasm was classified as malignant "leiomyoblastoma," with the use of current histologic criteria. This tumor had metastasized to the liver but not to the lungs. Although the histologic criteria for leiomyoblastoma were fulfilled, no definite evidence of smooth-muscle cell differentiation was present ultrastructurally or by immunostaining methods. Gastric tumors that form part of "Carney's triad" are known to differ clinically and pathologically in important ways from smooth-muscle cell malignant neoplasms that are not part of this syndrome. Some have been classified as gastrointestinal autonomic nerve tumors, but the current study did not confirm this contention. The nature of gastric leiomyoblastomas in Carney's multitumoral association remains undecided.- - - - - - - - - - ranking = 5keywords = nature (Clic here for more details about this article) |
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