1/17. paraganglioma of the urinary bladder: A case report and review of the Japanese literature.We describe a patient with primary paraganglioma of the urinary bladder associated with typical clinical symptoms. Systolic blood pressure frequently increased to 300 mm Hg immediately after micturition. Levels of urinary vanillylmandelic acid excretion and serum norepinephrine were significantly elevated. The tumor was removed by partial cystectomy. Histological examination of the tumor revealed paraganglioma of the urinary bladder. Compared with a review of 53 similar cases reported in japan, the distribution of age, sex and tumor location in the bladder differed from those described worldwide.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
2/17. Nasopharyngeal gangliocytic paraganglioma.Gangliocytic paraganglioma (GP) is a rare neoplasm described almost exclusively in the gastrointestinal tract, especially the periampullary region. However, several examples have been reported at various sites, including the stomach, jejunum, and appendix. Herein we report a case of GP involving the nasopharynx. To our knowledge, this is the first report of GP at this site. A 44-year-old woman presented with headaches and symptoms of fullness and pressure related to mass effect. An initial endoscopic biopsy was followed by surgical excision of the nasopharyngeal mass. The triphasic tumor fulfilled the morphologic and immunohistochemical criteria for GP. The histogenesis of GP is uncertain, and the current belief is that it arises from the embryonic ventral pancreas. This concept is based largely on the location of most cases, which is along the embryologic migration route of the ventral pancreas, as well as the expression of pancreatic polypeptide by the tumor. The nasopharyngeal location of our case clearly refutes the pancreatic origin of GP. We propose that the tumor probably arises from totipotential adult stem cells, which in the right microenvironment differentiate along nonnative cell lineages.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
3/17. Lessons to be learned: a case study approach. paraganglioma of the urinary bladder.Primary paraganglioma arises infrequently in the urinary bladder. We present here the clinicopathological, immunohistochemical and ultrastructural findings in a 23-year-old Saudi female. She was hospitalised because of gross haematuria over the previous seven days. The intravenous urogram revealed a filling defect in the urinary bladder. cystoscopy disclosed a non-papillary tumour arising from the roof of the bladder neck. Transurethral resection was, accordingly, performed--but marked fluctuation in blood pressure was observed during the procedure. The histopathological findings of trabeculae and small nests of plump oval to spindle cells with a clear to acidophilic cytoplasm, forming an organoid pattern and demonstrating inconspicuous mitotic activity, were diagnostic of paraganglioma of the urinary bladder, the diagnosis was confirmed by means of immunohistochemical and ultrastructural studies. The endocrine markers chromogranin a and neuron-specific enolase were positive in chief cells; sustentacular cells at the periphery of neoplastic cell clusters were positive for S-100 protein. Neurosecretory granules were identified by electron microscopic examination. It is emphasised that, currently, there are no anatomico-pathological criteria to distinguish benign and malignant forms of the disease. Post-surgical recovery was uneventful in this case. recurrence and metastases, though infrequent, have been reported in the literature; therefore, radiation therapy may be an important adjunct--and long-term follow-up is mandatory.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
4/17. papilledema and intraspinal lumbar paraganglioma.Optic nervehead swelling is most frequently caused by ocular or intracranial lesions. The case presented here demonstrates that the spinal subarachnoid space must also be considered as a potential site for a lesion causing optic nervehead swelling. A 56-year-old man is presented with an intraspinal lumbar paraganglioma associated with increased cerebrospinal fluid protein, papilledema, transient obscurations of vision, and back pain. This may be the first reported case of a paraganglioma associated with optic nervehead swelling. magnetic resonance imaging of the lumbosacral region revealed the lesion noninvasively. The papilledema, transient obscurations of vision, and back pain resolved after resection of the tumor. The mechanisms are not defined for optic nervehead swelling in association with spinal tumors in general and paraganglioma in particular. The measured abnormal elevation of cerebrospinal fluid protein may have resulted in increased intracranial pressure and papilledema.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
5/17. hypotension in a woman with a metastatic dopamine-secreting carotid body tumor.OBJECTIVE: To describe a woman with metastatic carotid body tumor in whom hypotension occurred in the setting of exceedingly high plasma dopamine levels. methods: We present a case report and review the literature on the topic of dopamine-secreting paraganglioma or pheochromocytoma. RESULTS: A previously healthy 40-year-old Asian woman noted difficulty with swallowing and hoarseness. No neck mass was visible, and she had no symptoms of catecholamine excess and no family history of endocrine disorders or malignant disease. Indirect laryngoscopy revealed a paralyzed left vocal cord and a nonulcerating mass in the left parapharyngeal space. An initial needle biopsy was interpreted as undifferentiated carcinoma. After a second biopsy, this mass was diagnosed as a neuroendocrine tumor, consistent with paraganglioma. The patient underwent surgical resection and radiation therapy (total dose, 40 Gy), after which she remained asymptomatic for 11 years. Then loss of weight, fatigue, nausea, and hypotensive episodes (blood pressures as low as 70/35 mm Hg) prompted whole-body imaging with bone scans, computed tomography, and magnetic resonance imaging, which disclosed several lesions in the liver, lungs, and spine, suggestive of metastatic disease. The adrenal glands were unremarkable. A metaiodobenzylguanidine scan with use of (131)I was negative. liver biopsy of a hypodense lesion revealed a neuroendocrine tumor by histologic and immunohistochemical studies. Because of the patient's history, malignant paraganglioma was diagnosed. The tumor secreted predominantly dopamine at extraordinary levels (plasma concentration 27,942 pg/mL; normal, <30). The patient died before further treatment could be initiated. CONCLUSION: Carotid body tumors usually do not secrete catecholamines but frequently metastasize. During progression, these neuroendocrine tumors may become able to produce and secrete selected catecholamines such as dopamine. dopamine can lower the blood pressure rather than causing hypertension, even though hypertension is one of the main symptoms of a pheochromocytoma.- - - - - - - - - - ranking = 2keywords = pressure (Clic here for more details about this article) |
6/17. Primary pulmonary paraganglioma: report of a functioning case with immunohistochemical and ultrastructural study.We describe a case of primary pulmonary paraganglioma, a tumor that has not been reported in sufficient detail in previous literature. The patient was a 55-year-old woman with hypertension accompanied by an elevated serum norepinephrine level (2651 pg/mL; normal 100-450 pg/mL). Computed tomography revealed a well-circumscribed solid mass, 3.5 cm in diameter, located in the lower lobe of the left lung. In the lobectomy specimen, the tumor had invaded the B8 bronchus and hilar lymph nodes with microscopic metastasis to the mediastinal nodes. The tumor showed histologic, immunohistochemical, and ultrastructural features of paraganglioma: argyrophilic cells arranged in a nesting (Zellballen) or anastomosing trabecular pattern within an arcuate vascular network. Neoplastic chief cells positive for neuroendocrine markers (CD56, synaptophysin, chromogranin a) were surrounded by sustentacular cells positive for S-100 protein. Neurofilament protein was positively stained, but cytokeratins were totally negative. On electron microscopy, chief cells possessed abundant dense core granules with an eccentric halo ("norepinephrine-type" granules). The patient's blood pressure began to decline soon after the resection, and her serum norepinephrine promptly returned to almost normal. On the basis of our experience, our case is a bona fide primary pulmonary paraganglioma, a tumor heretofore subject to considerable skepticism.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
7/17. cauda equina paraganglioma presenting with intracranial hypertension: case report and review of the literature.An unusual case of intradural paragangliomas in the cauda equina region in a 29-year-old male is presented. The patient presented with signs and symptoms of raised intracranial pressure. The symptoms resolved after tumor resection.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
8/17. The importance of the interpretation of urine catecholamines is essential for the diagnosis and management of patient with dopamine-secreting paraganglioma.Phaeochromocytoma or paraganglioma that exclusively secretes dopamine is very rare. This case illustrates its atypical presentation and the importance of interpretative reporting for urine catecholamines leading to the diagnosis and subsequent management of a patient with this condition. We report a 71-year-old Chinese woman with a large dopamine-secreting paraganglioma. She presented with low back pain for six months. On examination, a right abdominal mass was palpable incidentally. Her blood pressure was normal throughout. Serial 24-h urine collections for catecholamines showed enormous elevation of urine dopamine excretion to 80.7 micromol/day (normotensive:<2.6 m mol/day). However, the daily excretions of urine adrenaline and noradrenaline, as well as their metabolites were within their respective reference intervals. Good communication between chemical pathologists and physicians prompted the arrangement of the whole body 131I-meta-iodobenzylguanidine (MIBG) scintigraphy, which showed a large signal in the right upper quadrant of the abdomen corresponding to a large extra-adrenal tumour detected by both ultrasonography and computerized tomography (CT) of the abdomen. Histological section of the tumour tissue revealed paraganglioma, which stained positive for chromogranin and neuron-specific enolase. After four months, the patient presented with chest symptoms and CT of the thorax revealed multiple nodules. lung metastases were suspected. However, follow-up urine catechola- mine and dopamine excretions were again within their respective normotensive reference intervals. A second MIBG scintigraphy was performed, but no specific uptake at either the thorax or the abdomen could be demonstrated. Fine-needle aspiration cytology using the thoracoscopic technique was performed and immunochemical staining of the biopsy specimen showed the presence of non- small-cell carcinoma of the lung.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
9/17. fluorine-18 DOPA PET imaging of paraganglioma syndrome.A 12-year-old boy was admitted after recently having had a resection performed of an extraadrenal retroperitoneal paraganglioma and left adrenalectomy for pheochromocytoma. Despite these procedures, the blood pressure and urinary noradrenalin were elevated. Screening with fluorine-18 DOPA PET demonstrated increased tracer uptake in the right adrenal gland, in a second abdominal lesion, which was prevertebral, and 2 cervical hot spots near the carotid bifurcation, one on each side of the neck. The patient carries a mutation of the gene Succinate dehydrogenasis subunits D (SDHD) and is thus classified with the paraganglioma syndrome type 1.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
10/17. Robot-assisted laparoscopic resection of a large paraganglioma: a case report.A 19-year-old female patient presented with headache, nausea, hypertension, visual impairment of the left eye and exertion-related complaints of palpitations since 1 year. Fundoscopy showed severe hypertensive retinopathy grade IV. A paraganglioma in the left para-aortic region was diagnosed by urinary screening and magnetic resonance imaging scanning. The tumor was resected by a robot-assisted laparoscopic procedure. The surgical operating time was 250 minutes and the blood loss was 150 mL. After surgery the blood pressure normalized and antihypertensive drugs were no longer required. Oral intake was resumed on the first postoperative day and the hospital stay was 3 days. Final pathology examination revealed a paraganglioma with pheochromocytomal features of 7x5.1x3.5 cm. Reports on laparoscopic resection of large paragangliomas are scarce. This case has demonstrated that the procedure is feasible and associated with rapid recovery from surgery. The surgical team experienced explicit support by the robot in the dissection of this highly vascularized tumor.- - - - - - - - - - ranking = 1keywords = pressure (Clic here for more details about this article) |
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