1/101. 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.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/101. Laryngeal paraganglioma in a five-year-old child--the youngest case ever recorded.A paraganglioma is a neuroendocrine neoplasm that originates from the paraganglion cells of the parasympathetic system. The average age of presentation is in the fifth decade. We report a case of laryngeal paraganglioma in a five-year-old child, the youngest case ever recorded. The features of paraganglioma which differentiate it from other tumours are also discussed.- - - - - - - - - - ranking = 27.078113658024keywords = endocrine (Clic here for more details about this article) |
3/101. pathology findings with acrylic implants.We report the pathological findings in cases of acrylic implants obtained by direct intratumoral injection of polymethyl-methacrylate (PMMA) and N-butyl-cyano-acrylate (NBCA). Direct intratumoral injection of acrylic implants was performed for a variety of primary and secondary bone lesions. These types of treatments have been used at our institution in the last 4 years for 40 vertebroplasty (PMMA) procedures and for nine bone lesions of other locations (PMMA, NBCA). Postmortem histology became available for 1 case of PMMA and for 5 cases with NBCA intratumoral acrylic implants. The pathological findings associated with PMMA and NBCA were evaluated and compared. PMMA exhibited a macroscopic and microscopic rim of tumor necrosis, 6 months after implantation. NBCA exhibited compressive effects on the nearby tumor tissue, however, without signs of significant necrosis outside the acrylic tumor cast. Tumor captured inside the acrylic cast showed extensive to near complete necrosis. Acrylic implants may lead to necrosis when injected directly in tumors. The necrotizing effect may extend beyond the limits of an implant in the case of PMMA. Such an extended effect of PMMA, when compared with NBCA, may be due to the variable toxicity of acrylic implants, including the different degrees of the exothermic reaction during polymerization.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
4/101. Sustentacular cells in sporadic paraganglioma-like medullary thyroid carcinoma: report of a case with diagnostic and histogenetic considerations.Sustentacular cells (SCs) are glial supporting cells of the fetal, adult normal, and neoplastic extra and adrenal human chromaffin cell lineage. SCs have also been identified in some cases of medullary thyroid carcinoma (MTC), raising both diagnostic and histogenetic problems. We report a rare case of a sporadic paraganglioma-like variant of MTC showing numerous S-100 protein and glial fibrillar acid protein (GFAP) positive SCs encircling neoplastic cells in a nesting (Zellballen) pattern similar to that observed in paragangliomas or pheochromocytomas. Although stromal amyloid deposits were only focally detected, diagnosis was immunohistochemically confirmed by immunoreactivity of the neoplastic cells for cytokeratin, CEA, calcitonin, chromogranin a, neuron-specific-enolase, and synapthopysin. As for the histogenesis of SCs, if we assume that MTC is a neural crest-derived tumor, it is likely that these cells reflect the ability of the common precursor cell to differentiate towards a sustentacular-type glial cell lineage in addition to the typical neuroendocrine one. This viewpoint is supported by the evidence that rare cases of MTC may contain neoplastic or supporting cells showing a multidirectional differentiation (usually neuroendocrine and melanocytic) that recapitulates the different cell lineages arising from the developing neural crests.- - - - - - - - - - ranking = 54.156227316048keywords = endocrine (Clic here for more details about this article) |
5/101. paraganglioma as a systemic syndrome: pitfalls and strategies.Tumours of the neuroendocrine system in the head and neck region are mostly paragangliomas of the glomus tympanicum or jugulare, or of the carotid body. The majority of these tumours are benign, and the coexistence of multiple paragangliomas seems to be rare. Pre-operative embolization and surgery are regarded as primary therapy for these tumours. The treatment regimen in any patient depends on age, general health, hearing status and the function of the lower cranial nerves. Several presentations are possible in which paragangliomas occur as systemic disease. 1. Paragangliomas may occur bilaterally, or, in rare cases, in multiple areas. Pre-operative bilateral angiography is of utmost importance. In case of multicentricity, it might be necessary to proceed without, or just with, unilateral surgery for preservation of adjacent structures. In surgery of jugular vein paraganglioma, we usually perform a modified transmastoidal and transcervical approach with preservation of middle-ear structures and the ossicles. As an alternative or supplement to surgery, radiotherapy or definitive embolization may be used in the treatment of paragangliomas. 2. Paragangliomas may occur as multiple endocrine neoplasia (men) syndrome combined with medullary thyroid gland carcinoma, and, facultatively, pheochromocytoma. In these cases, endocrinological examination and magnetic resonance imaging (MRI) of the adrenal region, the thorax and the neck are required for an adequate therapeutic strategy. As men may be inherited, family history should be evaluated. 3. Paragangliomas can became malignant and metastasize. Thus, cervical lymph node metastases or distant metastases may occur. We recommend the removal of all ipsilateral lymph nodes and their histological examination.- - - - - - - - - - ranking = 54.156227316048keywords = endocrine (Clic here for more details about this article) |
6/101. Calvarial metastasis of a paraganglioma. Case report and review of the literature.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.- - - - - - - - - - ranking = 0.66666666666667keywords = bone (Clic here for more details about this article) |
7/101. paraganglioma of the tongue.Paragangliomas are neuroendocrine tumors arising from extra-adrenal autonomic ganglia. We present what is to our knowledge the first immunohistochemically documented case of a paraganglioma of the tongue, a 2.5-cm benign tumor growing in the position of the foramen cecum. The patient was an elderly woman who presented with throat irritation. The histologic profile and ultrastructural appearance of this lesion were classic for paraganglioma, and cells stained strongly for neurosecretory granules by immunohistochemistry. Carcinoid, ectopic thyroid neoplasm, and other tumors that mimic paraganglioma were ruled out on immunohistochemical grounds. To our knowledge, paraganglia have not been previously documented in the tongue; this tumor may have arisen from a branch of cranial nerve VII or IX, or from an embryologic remnant of the thyroid or thyroid capsule.- - - - - - - - - - ranking = 27.078113658024keywords = endocrine (Clic here for more details about this article) |
8/101. Surgical resection of jugulare foramen tumors by juxtacondylar approach without facial nerve transposition.PRESENTATION: Jugulare foramen tumors (JFT) remain a difficult challenge especially in the forms extending extradurally and invading the petrous bone. In the standard technique, facial nerve function is placed at risks because of its transposition. We report on 31 extradural (N = 11) or intra-extra dural (N = 20) JFT resected surgically using the juxtacondylar approach alone or in combination with the infratemporal approach and without facial nerve transposition. RESULTS: The juxtacondylar approach permits the opening of the JF on its posteroinferior aspect and thus reduces the extent of petrous bone drilling. In tumors strictly located in the JF (N = 11), no petrous bone drilling is necessary and the facial nerve is never exposed. In tumors extending into the petrous bone (N = 20), the facial nerve was never transposed and moreover was kept in its bony canal in 15 cases. In only 5 cases, was the fallopian canal opened as the tumor was invading its bony wall. Similarly hearing function, when pre-operatively intact was always preserved and a better preservation of the lower cranial nerves could be achieved. Whatever the tumoral extent along the petrosal carotid artery, a radical resection could be realized in 30 cases. CONCLUSION: Therefore, we consider the juxtacondylar approach a useful adjunct to increase the possibilities of resection of JFT; it allows a better preservation of the neurovascular structures, especially the facial nerve which is kept in place and moreover kept in its bony canal when it is not invaded by the tumor.- - - - - - - - - - ranking = 1.3333333333333keywords = bone (Clic here for more details about this article) |
9/101. Malignant retroperitoneal paraganglioma: case report and review of treatment options.A case of retroperitoneal paraganglioma metastasizing to bone is presented. This is followed by a literature review of treatment options, including external beam radiotherapy, chemotherapy and 131I-metaiodobenzylguanidine.- - - - - - - - - - ranking = 0.33333333333333keywords = bone (Clic here for more details about this article) |
10/101. 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 = 54.156227316048keywords = endocrine (Clic here for more details about this article) |
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