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At least 1,045 records · Page 58Linked to original sources
PubMed2001

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.

Adult↗
PubMed2003

Duodenal gangliocytic paraganglioma with lymph node metastasis: a case report and review of the literature.

A case of duodenal gangliocytic paraganglioma (DGP) in a 67-year-old woman is presented. The DGP arose in the second part of the duodenum. Although most of the reported cases of DGP are considered benign, in the present case, we found regional lymph nodes containing metastatic tumor. Previous reports have documented metastases containing only epithelioid cells. The current case demonstrates metastatic tumor in regional lymph nodes containing all 3 of the DGP components (spindle cells, ganglion-like cells, and epithelioid cells).

Aged↗
PubMed1999

[A case of paraganglioma of the urinary bladder].

A 50-year-old male was referred to our department because of dysuria. On cystoscopy, a submucosal bladder tumor was seen at the posterior wall of the urinary bladder. Transurethral resection was performed with no intraoperative complications. Histopathological diagnosis of paraganglioma was confirmed by immunohistochemical staining.

Humans↗
PubMed1999

[Intraoperative diagnosis of a mediastinal paraganglioma: Anesthetic management].

A 45-year-old woman with a posterior mediastinal tumor underwent right thoracotomy for resection, developing hypertension and difficult-to-control tachycardia while the tumor was being manipulated. A catecholamine-secreting tumor was suspected, the operation halted, and the patient prepared for surgery at a later time. The tumor was a mediastinal paraganglioma and the final outcome was satisfactory. Risk related to anesthesia is high in such patients, with perioperative mortality ranging from 40 to 85%. Correct diagnosis and appropriate preoperative drug preparation with adrenergic receptor blockers appreciably decreases morbidity and mortality related to surgery. We discuss the effect of labetalol on such tumors and describe our observations.

Catecholamines↗
PubMed1998

[Duodenal gangliocytic paraganglioma: intraoperative ultrasound evaluation].

Case report. A case of obstructive jaundice due to a preoperative unrecognized rare periampullary tumor, histologically demonstrated to be a gangliocytic paraganglioma of duodenum associated with Von Recklinghausen's disease, is herein reported. Intraoperative ultrasonography guided identification and evaluation of the obstructing lesion, suggesting in Whipple's procedure the most suitable treatment. The clinical case and ultrasonographic and Color Doppler findings are in detail described.

Ampulla of Vater↗
PubMedDate not supplied

Paraganglioma of the cauda equina: MR findings.

The MR findings of a case with paraganglioma of the cauda equina are presented. T2-weighted images showed the tumor to have a hyposignal rim and serpiginous flow voids, suggesting vessels capping the tumor. Contrast-enhanced MR images highlighted the tumor and tumor vessels more clearly. Knowledge of MR findings in this unusual disorder may aid in diagnosis.

Adult↗
PubMed1999

The antiangiogenic agent linomide inhibits the growth rate of von Hippel-Lindau paraganglioma xenografts to mice.

The aim of this study was to ascertain the potential usefulness of the antiangiogenic compound linomide for treatment of von Hippel-Lindau (VHL)-related tumors. Paraganglioma tissue fragments obtained at surgery from a VHL type 2a patient were transplanted s.c. to male BALB/c nu/nu (nude) mice: (a) 2-3-mm fragments for "prevention" experiments; and (b) 2-3-mm fragments allowed to grow to 1 cm for "intervention" studies. Both groups received either 0.5 mg/ml linomide in drinking water or acidified water and were followed until tumor diameter reached 3 cm or for 4 weeks. In both the prevention and intervention experiments, a significant diminution of tumor size and weight was observed in the drug-treated animals. In vivo nuclear magnetic resonance analysis of tumor blood flow in linomide-treated animals showed localization of blood vessels almost exclusively to the periphery of the poorly vascularized tumors with a significant reduction of both vascular functionality and vasodilation. Histological examination of tumors from linomide-treated animals revealed marked avascularity. Treated animals also displayed a 2.4-fold reduction of tumor vascular endothelial growth factor mRNA levels. Taken together, our data indicate that in VHL disease, therapy directed at inhibition of constitutively expressed VEGF induction of angiogenesis by VHL tumors may constitute an effective medical treatment.

Administration, Oral↗
PubMed2000

Extra-adrenal pheochromocytoma (paraganglioma).

The authors describe the case of a 19-year-old female patient with an abdominal paraaortic extra-adrenal pheochromocytoma (paraganglioma), presenting arterial hypertension. The predominant catecholamine produced by the tumor was norepinephrine (4110 pg/ml; normal < 450 pg/ml). 131I metaiodobenzylguanidine (131I-MIBG), computed tomography and magnetic resonance imaging allowed location and characterization of the tumor. Histologically the tumor (weight = 34.2 g; 5.8 x 4 x 3 cm) was a typical pheochromocytoma.

Abdominal Neoplasms↗
PubMedDate not supplied

Thyroid paraganglioma: report of a case and review of the literature.

A case of thyroid paraganglioma is reported. Immunohistochemically the tumor showed negativity for thyroglobulin and calcitonin and positivity for chromogranin A and S-100. Protein-positive sustentacular cells were demonstrated. The authors discuss the previous literature on these tumors.

Biopsy, Needle↗
PubMed2000

[Acute gastrointestinal bleeding caused by a rare, benign tumor of the duodenum (gangliocytic paraganglioma)].

Diagnosis and treatment of massive gastrointestinal bleeding is sometimes very difficult problem in a surgical unit. Authors present a case of a 40 years old female with an atypical source of upper gastrointestinal bleeding. The origin of the bleeding was detected only by intraoperative enteroscopy. The source of the bleeding was a very rare benign tumour of the duodenum (Gangliocytic paraganglioma) which involved the papilla of Vater. During the operation resection of the pedunculated tumour was carried out, with choledochal--and Wirsungoplastyc, associated with external drainage of these ducts. According to the literature in preoperative diagnosis of this rare tumour endoscopy, angiography, and EUS are very useful. In case of malignancy, metastases radical operation-pancreatoduodenectomy--is indicated.

Acute Disease↗
PubMed2001

[Non-functional retroperitoneal paraganglioma].

Non functional retroperitoneal paragangliomas are rare tumors: less than 50 cases are reported in the literature. They are usually asymptomatic and can attain big dimensions. The authors report a case diagnosed by histological examination of the operative specimen. Malignant tumors more frequent than the benign tumors present with a regional extension and delayed metastases. The treatment is completed by surgical excision. Radiotherapy and chemotherapy can be used for palliation of symptoms.

Adult↗
PubMed1975

Catecholamine-secreting paraganglioma. The problem of classification.

Sudden catecholamine release, associated congestive heart failure, acute injury changes on ECG, and elevation of cardiac enzyme levels were observed after biopsy of a mediastinal mass. Five months later, a normal ECG and coronary arteriogram were recorded. Initial severe avoid unexpected catecholamine release, we recommend that all paragangliomas be evaluated for catecholaime production.

Adrenal Gland Neoplasms↗
PubMed2001

[Paraganglioma associated with gastric schwannosarcoma].

The authors report the case of a 45-year-old patient who presented with a left latero-aortic paraganglioma associated with gastric schwannosarcoma. Treatment consisted of resection of the chromaffin tumour and the gastric tumour, with a favourable course at last follow-up. The authors review the literature on this subject.

Humans↗
PubMed2001

[Acute cardiac failure revealing a paraganglioma].

Therapy of cardiomyopathy is usually symptomatic. So, the etiology is often only superficially investigated. However, because of their curability, rare causes must be sought for. We report a case of acute cardiac failure in a young female patient with severe hypertension of recent onset. Urinary catecholamines analysis and medical imaging demonstrated a paraaortic paraganglioma. After coelioscopic resection, cardiac function recovered.

Acute Disease↗
PubMed2002

Frequent germ-line succinate dehydrogenase subunit D gene mutations in patients with apparently sporadic parasympathetic paraganglioma.

PURPOSE: Recently, familial paraganglioma (PGL) was shown to be caused bymutations in the gene encoding succinate dehydrogenase subunit D (SDHD). However, the prevalence of SDHD mutations in apparently sporadic PGL is unknown. We studied the frequency and spectrum of germ-line and somatic SDHD mutations in patients with parasympathetic PGL. EXPERIMENTAL DESIGH: We studied 57 unselected patients who developed parasympathetic PGLs (n = 105 tumors) and who were treated between 1987 and 1999 at the Erasmus MC (Rotterdam, the Netherlands). Thirty-eight (67%) of these patients (n = 51 tumors) lacked a family history of parasympathetic PGL. We used conformation-dependent gel electrophoresis and sequence determination analysis of germ-line and tumor DNA to identify SDHD mutations. We compared the clinical and molecular characteristics of sporadic and hereditary PGLs. RESULTS: Three different SDHD germ-line mutations were identified in 32 of the 57 (56%) patients. These included 19 of 19 (100%) patients with familial PGL and also 13 of 38 (34%) patients with apparently sporadic PGL. All three mutations were characterized as missense mutations (D92Y, L95P, and L139P) in highly conserved regions of the SDHD gene and were not observed in 200 control alleles. No somatic mutations were found. CONCLUSIONS: Germ-line mutations of the SDHD gene are present in a significant number of patients with apparently sporadic parasympathetic PGL. Somatic SDHD mutations do not play a significant role in the sporadic form of this tumor. Genetic testing for SDHD germ-line mutations should be considered for every patient presenting with this tumor, even if a personal or family history of PGL is absent, to allow appropriate clinical management.

Adult↗
PubMed2000

[Paraganglioma associated with renal artery stenosis: unusual cause of secondary arterial hypertension. A case report and review of the literature].

Secreting paraganglioma is a rare cause of secondary HT and corresponds to an extra-adrenal phaeochromocytoma. This case reports the consecutive coexistence, with a free interval of 10 years, of dysplastic renovascular HT and abdominal paranganglioma in the organ of Zuckerkandl. This is a rare association which requires investigation of a common genetic predisposition (neural crest disease) and emphasizes the importance of long-term surveillance of these patients.

Adult↗
PubMed2003

[Paraganglioma and pregnancy].

A paraganglioma is an extra-adrenal pheochromocytoma, rarely observed during pregnancy. Maternal prognosis depends on early diagnosis and multidisciplinary management prior to tumor resection. The tumor is localized by ultrasonography or MRI. During the post-partum, and if MRI is not available, CT is often sufficient. We report a paragangliona in a patient with intrauterine fetal demise at 27 weeks gestation diagnosed by ultrasonography and computed tomography, who underwent surgical resection.

Adult↗