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[Non-chromaffin laryngeal paraganglioma. Clinical and electron microscopical study].

The authors describe the case of a 51 year-old woman presenting with a very large laryngeal paraganglioma in the aryepiglottic fold which had passed through the cricothyroid membrane and invaled the thyroid. A crico-hyoido-pexy enabled the tumour to by completely removed and anatomo-pathological examination showed degeneration. Under electron microscopy, the ultrastructure of the tumour was similar to that of chromaffin paragangliomas. It is three years since this patient was cured.

Female↗

Paraganglioma of the cauda equina. A case report and review of the literature.

A case of paraganglioma of the cauda equina is reported. The patient was a 55-year-old Japanese woman who complained of lower back pain and gradual weakening of the left lower extremity, she was diagnosed as having a spinal cord tumor, and the tumor was removed surgically. Histologically, the tumor was encapsulated, and consisted of solid nests of large, polyhedral epithelioid cells with abundant eosinophilic cytoplasm. The nests were separated from each other by a thin fibrovascular stroma. Grimelius staining revealed neurosecretory granules in the cytoplasm of the tumor cells. This was further confirmed by electron microscopic observation. The tumor cells were immunohistochemically positive for neuron-specific enolase (NSE), but negative for glial fibrillary acidic protein (GFAP). On the basis of the histologic, immunohistochemical and electron microscopic features of the tumor cells, the tumor was diagnosed as paraganglioma. The cauda equina is a rare location for this type of tumor, and only a limited number of cases have been reported.

Cauda Equina↗

Suburethral paraganglioma.

Masses presenting beneath the urethra are not uncommon. Besides ureteral diverticula, which account for the vast majority of these masses, a multiplicity of heterogeneous benign and malignant tumors have been encountered in this region. To the best of our knowledge, we report the first case of a neuroendocrine tumor, a paraganglioma, discovered in the anatomical compartment between the urethra and vagina. When evaluating a patient with a suburethral mass, it is important to consider paraganglioma as part of the differential diagnosis because unwitting surgical removal of a functional catecholamine-secreting tumor may precipitate a severe hypotensive episode and/or death.

Adult↗

Vagal paraganglioma (2 case reports).

Paragangliomas are rare tumours of the head and neck and are a diagnostic challenge. Two cases of paragangliomas arising from the vagus are being reported. In the first case, an angiogram raised the possibility of intravagal tumour and exploration followed by biopsy confirmed the diagnosis. In the second case, it was a diagnostic surprise where exploration was done on a clinical suspicion of parotid swelling and on the table, the swelling was found to arise from the vagus nerve pushing the parotid gland laterally.

Adult↗

[2 cases of sympathetic paraganglioma of the urinary bladder].

Two cases of symphathetic paraganglioma of the urinary bladder are reported. Symppathetic- or parasymphatetic origin of intramural paraganlia or paragangliomas can be determined by demonstration of praesynaptic myelinated fibers. This method gives more reliable results than formaldehyd-induction-method carried out on freezed sections or the Gömöri's chromaffin reaction which often appears to be false-negative.

Chromaffin Granules↗

[Familial paragangliomas of the neck: integrated imaging and the planning of family screening].

The authors have studied a case of familial paraganglioma of the neck with integrated diagnostic methods: sonography (US) and MR imaging. They verified, through personal experience and literature review, the utility of familial diagnostic screening and of clinico-diagnostic screening planning. The main aim was to detect the elements which could help plan the screening, thus improving the early diagnosis of paragangliomas.

Adult↗

Malignant jugulotympanic paraganglioma.

Jugulotympanic paragangliomas are neoplasms that arise in the jugular bulb (glomus jugulare tumor) or temporal bone (glomus tympanicum tumor). They are histologically similar to carotid body tumors; both are derived from extra-adrenal paraganglia. Of only 20 metastases reported, five had biopsy-proved bone involvement. We present a review of the literature of metastatic jugulotympanic paragangliomas and report a case that recurred locally with regional metastases 10 years after initial presentation and metastasized to spine 13 years later. Histologic data are reviewed, and potential treatment regimens are contrasted.

Adult↗

[A case of pulmonary paraganglioma].

A pulmonary paraganglioma that was discovered incidentally as a solitary coin lesion on abdominal CT was successfully resected. Though this type of paraganglioma is extremely rare, it needs to be differentiated from certain other pulmonary neoplasms.

Aged↗

[Functioning extra-adrenal paragangliomas].

The diagnosis of extra-adrenal paraganglioma requires the recognition of catecholamine hypertension and the site of the lesion. I-131-MIBG scintigraphy, CAT scan and angiography may localize primitive and metastatic lesions. Six cases of extra-adrenal paraganglioma (5 abdominal, 1 thoracic) surgically treated with complete resolution of the hypertensive state are presented here.

Abdominal Neoplasms↗

[Intraspinal paraganglioma. Apropos of 3 cases with a review of the literature].

The authors present three cases of paraganglioma located in the cauda equina. In all cases pre-operative diagnosis was neurinoma of the cauda equina and only the histology permitted correct identification. All were intradural extramedullary tumors and in two cases the presenting and dominant symptom was low back pain and sciatica. The remaining third case experimented a paraparesis with urinary and fecal incontinence. The follow-up (range of 4 months-4 years) after total excision was uneventful in two cases and showed in the third case with preoperative paraparesis, some persistent urinary incontinence (follow-up: 3 years) with good motor recovery. The light microscopic features were a Zellballen pattern of cells containing argyrophil granules. Electron microscopy was not performed. The most likely theory regarding the embryogenesis of cauda equina paragangliomas is that they arise from pre-existing paraganglia, possibly of the visceral-autonomic group. The prognosis after complete excision appears to be good. The literature is thoroughly reviewed.

Adult↗

Paraganglioma of the spermatic cord. Report of a case with immunohistochemical and ultrastructural studies.

We describe a case of paraganglioma arising in the spermatic cord, which is an extremely rare location. Immunohistochemical studies characterized two types of cells: (1) polygonal cells expressing neuron-specific enolase, chromogranin A, and synaptophysin and (2) S100 protein-positive sustentacular cells. Electron microscopy revealed that within the cytoplasm of the polygonal cells, there were electron-dense granules whose morphological appearance was consistent with that of neurosecretory granules. Paraganglioma of the spermatic cord may originate from embryonic chromaffin cells that have followed the testis into the scrotum.

Adolescent↗

Gangliocytic paraganglioma of the duodenum.

In spite of possessing microscopic features of invasiveness, the clinical behaviour of duodenal gangliocytic paraganglioma is benign. The operation of choice for removal of the tumour is local excision. This conservative approach was used in the case of a 70-year-old white women who presented with occult gastrointestinal bleeding. The unusual histologic features of this woman's tumour are discussed. Although the tumour appears to be aggressive no recurrence has been reported to date following adequate local excision of a duodenal gangliocytic paraganglioma.

Aged↗

Head and neck paragangliomas: a clinicopathologic study with DNA flow cytometric analysis.

A total of 11 head and neck paragangliomas were the subject of pathologic study, including histologic, immunohistochemical, and DNA flow cytometric analyses. We cannot absolutely predict aggressive clinical behavior using histologic parameters alone, but we can use such parameters to segregate patients into low-risk and high-risk groups. Several trends were observed in the current study. Tumors with higher S-phase fractions, G2/M fractions, or aneuploid cell populations tended to behave "aggressively." The presence of sustentacular cells in the primary tumors cannot be used as an absolute indicator of tumor metastatic potential, as two metastatic paragangliomas in this study contained sustentacular cells in both the primary and metastatic lesions. DNA ploidy status cannot be used as an absolute prognostic parameter as the two metastatic tumors were composed of diploid primary and metastatic lesions. The three tumors with aneuploid cell populations showed "aggressive" histologic and clinical features, but the length of the follow-up period for these cases is too limited to draw any conclusions. Although no absolute criteria can be used at present to gauge aggressiveness, close follow-up of these patients is essential, especially if pathologic findings suggest an "aggressive" course (ie, "malignant" histology, higher S-phase fractions, G2/M fractions, aneuploid cell populations, or decreased sustentacular cell density).

Adult↗

[Gangliocytic paraganglioma of the duodenum. Case report with immunocytochemical characterization].

Described in this paper are histological, electron-microscopic, and immunocytochemical findings recorded from a duodenal gangliocytic paraganglioma in a 21-year old man. The sessile polypoid tumor consisted of epithelial cells, neuroid spindle cells, and gangliocytic elements. Neurosecretory granules were detected by electron-microscopy in the epithelial cells. The neuroid spindle cells exhibited the ultrastructural feature of Schwann cells. The tumor was immunocytochemically characterized by the S-100-reactivity of the spindle cells and numerous PP-reactive epithelial cells. Gangliocytic paragangliomas of the duodenum are of benign behaviour and should be removed by local excision.

Adult↗

Gastric paraganglioma: an immunohistological and ultrastructural case study.

This is a report of the immunohistochemical and ultrastructural features of a case of paraganglioma of the stomach in a 61-year-old woman who presented with melaena. The typical 'Zellballen' arrangement of epithelial cells with granular cytoplasm was present and the tumour was highly vascular. The polygonal epithelial cells stained strongly for neuron specific enolase and the intervening elongated sustentacular or support cells were positive for S100 protein and vimentin. Many dense core granules were present within the epithelial cells while the elongated support cells contained plentiful diffuse intermediate filaments. No hybrid cells or ganglion cells were present in the tumour. This is the sixth recorded case of gastric paraganglioma and the first to be studied in detail by immunostaining and electron microscopy.

Female↗

[Computed tomographic diagnosis of paraganglioma of the base of the skull].

On the basis of examination of 30 patients with the diagnosis of paragangliomas of the base of the skull the following main computed tomographic signs of this group of tumors were revealed: destruction of bone structures of the base of the posterior and middle cranial fossae, erosion and widening of the jugular foramen; diminished pneumatization of the air cells of the mastoid process; extracranial simultaneous with intracranial spreading of the tumor. A classification of paragangliomas is suggested.

Adult↗

[Paraganglioma of the head and neck. Familial forms].

The clinical, epidemiological and genetic aspects of familial paragangliomas of the head and neck are discussed in relation to 6 recent cases from two families which accounted for 2 and 4 patients respectively. Tumors of the carotid body are the most frequent. Bilaterality and association with other paragangliomas are suggestive of a hereditary disorder and should lead to investigation of the family. The establishment of a family tree will define those subjects at risk in whom systemic screening should be performed. The recent advances obtained using new imaging techniques in the diagnosis, evaluation of extension and treatment should enable treatment to be instituted at an earlier stage and thus reduce functional sequelae.

Adult↗