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[Paraganglioma of the organs of Zuckerkandl. A case report (author's transl)].

Endocrine manifestations were absent in a patient presenting with pain in the left peri-umbilical region from a paraganglioma of the organs of Zuckerkandl. The authors describe the embryology of paragangliomas and review the published literature concerning the noradrenaline-secreting forms of these tumors. Only 86 references to those of Zuckerkandl, situated on the side of the aorta in relation to the inferior mesenteric artery, were found. Diagnosis of non-secreting forms is confirmed by arteriography which in this present case demonstrated three pedicles arising from the inferior mesenteric, celiac, and one of the lumbar arteries respectively. The only evidence of malignancy is the presence of metastases. Their diagnosis is therefore difficult but essential as one-third of the localisations of malignant pheochromocytomas (10 p. cent of pheochromocytomas) outside of the adrenals affect the organs of Zuckerkandl.

Angiography↗

[Gangliocytic paraganglioma of the duodenum].

A duodenal gangliocytic paraganglioma was found to be the cause of nonspecific abdominal complaints in a 68-year-old man with a known carcinoma of the rectum and the prostate. The duodenal gangliocytic paraganglioma is generally regarded as being benign. In this case it is shown to be an additional tumor in a patient with two pre-existent malignancies.

Aged↗

[Intracerebral extension of cervical paraganglioma].

A large paraganglioma invaded the neck and skull, with extension into the right supratentorial cerebral hemisphere through the calvarium. Immunohistological markers were found useful for the diagnosis. The patient died rapidly. Despite no evidence of metastasis, the usually benign paraganglioma can have a malignant course.

Brain Neoplasms↗

[Paraganglioma of the cauda equina. Clinical aspects and MRI. Apropos of a case].

The clinical and imaging features of a paraganglioma of the cauda equina are presented. A peridural anesthesia revealed the cauda equina compression syndrome in a 68 years old man who presented first with a urinary retention. Total surgical excision of a long round-shaped encapsulated tumor was uneventful. Macroscopically this lesion filled up the spinal canal and resembled to a meningioma. It was firmly attached to the filum terminale. Adjacent large arterialized vessels visible on MRI were spared. Although the diagnosis could be evoked pre-operatively on some suggestive patterns on the MRI, the pathological findings finally confirmed the diagnosis of paraganglioma. Along with the evidence of specific light microscopic features, the use of an immunocytochemical demonstration of synaptophysine in these tumors allows a confident diagnosis to be made.

Aged↗

[Rare association of a non-functional retroperitoneal paraganglioma and nephrotic syndrome].

Non functional retroperitoneal paragangliomas are rare: less than 50 cases in literature. They are usually asymptomatic and can attain reasonable dimensions. The association with nephrotic syndrome is exceptional. The authors report a clinical observation of non-functional paraganglioma in a 45 year old woman, with repeated surgical abstention. Literature was reviewed and therapeutic indications discussed.

Acute Kidney Injury↗

[Multiple paraganglioma].

Paragangliomas are tumors of the chromaffin system producing catecholamines and both the adrenal extra-adrenal type have a common origin at the neural crest, which has been supported by the similarities of the paraganglions both from a histological and functional perspective. Most of the extra-adrenal paragangliomas are bending and non-functional. In addition, they are usually confined to just one site. We present the case of a young patient with benign multiple paraglanglioma in two different locations, juxtahepatic and juxtacarotic (Chemodectoma) and, thus, far away from each other. The extra-abdominal location is very rare, with a frequency lower than 2%. In addition, one of paraganglions was functional and the other one was not. Initially, a surgical intervention was performed on the functional abdominal tumoration and latter, another intervention was performed at the cervical level, both with excellent results. We also highlight the good response to the postoperative therapy with Prazosin.

Abdominal Neoplasms↗

[Mediastinal functioning paraganglioma. A case report].

Functioning paraganglioma of the mediastinum is an uncommon tumor of the paraganglion system. We report a case of a 26 year-old woman with a 6 month history of hypertension refractory to therapy. Urinary catecholamine excretion and plasma noradrenaline concentration were elevated. Chest radiograph and computed tomography (CT) scan revealed high-density mass located on the posterior mediastinum. An mIBG scan showed uptake by the mediastinal tumor. At thoracotomy a 5 cm lesion was resected. Histological examination confirmed that it was a paraganglioma. Authors refer on the clinical finding and diagnostic procedures of this rare disease.

Adult↗

Irradiated paragangliomas of the head and neck: CT and MR appearance.

PURPOSE: To present the spectrum of CT and MR findings of glomus tumors of the head and neck successfully treated with radiation therapy. METHODS: The patient charts and all CT and MR studies of 24 patients (25 tumors) who had been successfully treated with radiation therapy were retrospectively reviewed. Eighteen patients had pre- and posttreatment imaging studies. Tumor size, internal morphology, enhancement pattern, visualization of flow voids, and bone erosion were evaluated before and after radiation therapy. Statistical evaluation of the presence of flows voids and tumor size was performed using the Fischer Exact Test. RESULTS: All patients had residual tumor after radiation therapy. Sixty-one percent of tumors demonstrated a reduction in size. Only one tumor with pretreatment bone destruction demonstrated healing of the bone. MR findings after radiation therapy included variable alteration in T2 signal, decreased heterogeneous enhancement, and a reduction in flow voids. There was a significant difference in the presence of flow voids based on tumor size. CONCLUSIONS: Successfully irradiated paragangliomas demonstrate residual masses, the presence of which does not by itself indicate treatment failure. Stabilization or reduction in size, decreased enhancement, diminished flow voids, and reduced T2 signal after radiation therapy are a result of therapy and are indicative of local control. Persistent bone demineralization and erosion without progression is commonly seen in successfully controlled tumors. Paragangliomas are relatively homogeneous in internal morphology except for areas of flow void. Flow voids are not a reliable criterion for diagnosis in lesions less than 2.5 cm.

Adult↗

Paraganglioma of the vagus nerve masquerading as a carotid body tumor.

A case of paraganglioma of the vagus nerve in a 69-year-old woman presented as a pulsatile neck mass of 20 years duration. Angiogram showed it to be a vascular tumor and suggested the diagnosis of carotid body tumor. At operation, the tumor was found to involve the vagus nerve, which needed to be resected. The relevant literature regarding paragangliomas of the vagus nerve is discussed.

Aged↗

Paraganglioma of the filum: MR appearance.

Paragangliomas are benign tumors that are thought to arise from the neuroectodermally derived paraganglionic cells of the sympathetic nervous system. We report the imaging findings of an unusual case of paraganglioma of the filum terminale.

Cauda Equina↗

[A case of benign paraganglioma arising in the middle mediastinum; 201Tl-SPECT for differentiation from malignant tumor].

A case of benign paraganglioma arising in the middle mediastinum was reported. 201Tl SPECT showed high accumulation in tumor on early images at 15 min and reduced on late images at 3 hours after infusion. The patient was a 57 year old female. In the contrast enhanced CT, 3 x 4 cm tumor with intensive enhancement was recognized at the right middle mediastinum. Under the radiological images, the tumor was surgically removed. The pathologic diagnosis was a low atypical nonfunctioning aortico-pulmonary paraganglioma. This report was suggested that 201Tl SPECT was useful for differential diagnosis of benign neurogenic mediastinal neoplasms.

Diagnosis, Differential↗

[Intra-abdominal paraganglioma: clinical, functional, structural, and immunohistochemical study of an exceptional case].

Paragangliomas are tumors, derived from paraganglionary system, able to synthesize and to liberate substances with neuroregulatory activity. They are rare, mainly in the neck located, difficult to anatomo-pathologic diagnose, and of poor prognosis. They may present as space-occupying lesions or as secondary syndromes due to the liberation of biologically active substances. Whenever it is possible, surgical excision is the treatment of choice. Nor chemotherapy, nor immunomodulators, like alpha-2b-interferon, have provided satisfactory results. A 57 year-old man, with a non-surgical paraganglioma characterized by abdominal mass associated with liver and bone metastasis, is presented. The rarity of the intra-abdominal presentation, the image study findings, the histopathological and immunohistochemical examinations, and the evolution after alpha-2b-interferon therapy are analyzed.

Humans↗

[Paraganglioma of the cauda equina].

The case of a 36 year old woman with a paraganglioma of the cauda equina was studied. This localization is rare and special emphasis is made concerning the peculiarities of presentation, aggravating factors of the clinical manifestations and neuroradiologic findings. A review of the 66 paragangliomas of the cauda equina published to date is carried out. This entity deals with a tumor in the adult, with slight male predominance and a clinical presentation not differing from that of other lesions occupying the space of the cauda equina. The most frequent initial symptom is low back pain with or without sciatic irradiation. Total resection of the tumor may cure the patient.

Adult↗

[A case of malignant paraganglioma].

Herein we report a case of paraganglioma. The metastatic lesion was incidentally found in a 64-year-old man by chest roentgenography. The primary tumor was extra-adrenal and was revealed to be malignant. The patient was normotensive without clinical symptoms despite extreme high serum and urinary dopamine levels. A venous injection of 5 mg metoclopramide elicited a significant increase of the blood pressure. This phenomenon disappeared after surgical excisions of the primary and metastatic lesions. The metoclopramide test is considered to be useful to diagnose asymptomatic paraganglioma.

Humans↗

[Lateral neck paraganglioma: diagnostic imaging and preoperative embolization].

Sixteen paragangliomas of the neck were reviewed. Lesions were found in the carotid body (n = 12), vagal nerve (n = 3) and larynx (n = 1). While paragangliomas were often misdiagnosed clinically, CT scanning defined the extent of the disease. MR was able to accurately characterize the tumors as highly vascular. Multiplanar imaging, exquisite tissue contrast and anatomic detail allowed better display of the relationship between these neoplasms and surrounding carotid sheath vessels and intracranial structures. MR constitutes the imaging modality of choice in carotid body tumor. Arteriography is necessary for definite diagnosis and presurgical embolization.

Adult↗

Catecholamine-secreting paraganglioma of the pterygopalatine fossa: case report.

The third reported case of a pheochromocytoma-like syndrome attributable to a catecholamine-secreting paraganglioma arising within the pterygopalatine fossa is documented. The 25-year-old, female subject was found to have raised levels of urinary catecholamines. At another center, a large vascular tumor was identified at lateral rhinotomy. Biopsy confirmed the diagnosis of paraganglioma. Tumor removal by a Fisch type-C approach to the sphenoid, parasellar region, and pterygopalatine fossa was complete and recovery uneventful.

Adult↗

[Laryngeal paraganglioma: a case report].

A case of laryngeal paraganglioma in a 25-year-old female is reported. The site of the tumor was the left aryepiglotic fold and ventricular band. The diagnosis was made by histology and immunohistochemistry medial thyrotomy was performed in order to remove a bilateral carotid paraganglioma. We analyzed 76 cases reported in the world literature, clinical, histological, prognostic, and therapeutic factors in laryngeal paraglioma.

Adult↗