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Multifocal Extra-Adrenal Paraganglioma – Case Report

100%
Dobosz Ł., Dobrzycka M., Franczak P., Wieczorek J., Kobierska-Gulida G., Dobosz M.
Polish Journal of Surgery
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2015
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vol. 87
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issue 7
368-370
EN
Paraganglioma is a rare neoplasm originating from extra-adrenal pheochromocytes of the sympathetic and parasympathetic nervous system. It is usually benign and the treatment method of choice is a complete resection of the tumour. The authors present a case of 66-year-old female patient with a multifocal benign retroperitoneal paraganglioma, which was completely removed during surgery.
2
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Doppler ultrasound appearance of neck tumors

88%
Szymańska A., Szymański M., Gołąbek W., Drelich-Zbroja A., Jargiełło T.
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EN
Aim: The purpose of this study was the evaluation of the sonographic appearance of neck tumors and determining the features useful in differential diagnosis. Material and method: The studied group consisted of 57 patients: 16 patients with carotid body tumors, 9 patients with neurogenic tumors, 8 patients with venous anomalies, 12 patients with neck cysts, 6 patients with lipomas, 5 patients with extracranial carotid artery aneurysms and 1 with a laryngocele. Results: All carotid paragangliomas were located within the carotid bifurcation and demonstrated rich low-resistance vascular flow, with higher maximum velocity and lower flow resistance parameters registered in the ipsilateral external carotid artery. In 7 out of 9 cases, neurogenic tumors were homogeneous, and in the remaining 2 cases – heterogeneous. Four schwannomas were hypervascular or showed moderate vascularity, and the rest of neurogenic tumors were hypovascular or avascular, with symmetrical maximum velocity and resistance values of carotid blood flow. Apart from one branchial cleft cyst with multiple fine internal acoustic reflexes, all other neck cysts were anechoic and avascular, and presented with posterior acoustic enhancement. The laryngocele presented as a well-demarcated, hypoechoic, homogeneous lesion located in the immediate proximity of the larynx, without signs of internal vascular flow. Lipomas were well-demarcated, homogeneous, hypoechoic tumors with regular margins, without signs of internal vascular flow. Venous malformations presented as irregular, hypoechoic spaces with venous blood flow, easily compressed by the probe. Extracranial carotid artery aneurysms were hypoechoic, well-defined spaces, which presented with slow internal, turbulent flow on Doppler study, and showed continuity with the carotid artery. Conclusions: Doppler ultrasound allows to visualize features characteristic for certain neck tumors. Solid or cystic structure, echogenicity, localization, as well as internal flow signals and vascularity pattern create a combination of ultrasound findings helpful in the differential diagnosis of lesions such as paragangliomas, venous malformations, neurogenic tumors, aneurysms, cysts and laryngoceles.
PL
Cel pracy:Celem pracy była analiza obrazu ultrasonograficznego guzów szyi oraz ustalenie charakterystycznych cech umożliwiających różnicowanie poszczególnych patologii. Materiał i metody: W badanej grupie było 57 chorych: 16 z przyzwojakiem tętnicy szyjnej wspólnej, 9 z guzem neurogennym, 8 z malformacją żylną, 12 z torbielą szyi, 6 z tłuszczakiem, 5 z tętniakiem tętnicy szyjnej oraz 1 z laryngocele. Wyniki: Wszystkie przyzwojaki były położone w podziale tętnicy szyjnej wspólnej; prezentowały liczne naczynia o niskooporowym przepływie krwi oraz większe prędkości i niższe wartości oporu naczyniowego w tętnicy szyjnej zewnętrznej po stronie guza. Guzy neurogenne miały utkanie homogenne (7/9) lub niejednorodne (2/9). W czterech guzach schwannoma unaczynienie było bogate lub umiarkowane, u pozostałych chorych skąpe; wartości prędkości oraz współczynników oporu przepływu krwi w tętnicach szyjnych zewnętrznych były obustronnie porównywalne. Jedna torbiel boczna szyi zawierała drobne, rozproszone wewnętrzne odbicia, pozostałe zmiany były bezechowe, wszystkie ze wzmocnieniem akustycznym, bez sygnałów przepływu krwi. Laryngocele miało postać hipoechogenicznej, nieunaczynionej zmiany położonej w bezpośrednim sąsiedztwie krtani. Tłuszczaki były dobrze odgraniczonymi, hipoechogenicznymi guzami o regularnych obrysach i jednorodnej echostrukturze, bez cech przepływu krwi. Malformacje żylne miały postać hipoechogenicznych, nieregularnych przestrzeni, podatnych na ucisk, o żylnym spektrum przepływu krwi. Tętniaki tętnic szyjnych miały postać dobrze odgraniczonych, hipoechogenicznych przestrzeni w łączności z tętnicą szyjną, ze zwolnionym, turbulentnym przepływem krwi. Wnioski: Dopplerowskie badanie ultrasonograficzne wykazuje cechy charakterystyczne dla poszczególnych guzów szyi. Odróżnienie zmian litych od płynowych, echostruktura i lokalizacja, cechy przepływu krwi i typ unaczynienia tworzą kompozycje objawów pomocne w różnicowaniu zmian ogniskowych.
3
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Pheochromocytoma and paraganglioma – an overview

75%
Łubińska M., Sworczak K.
OncoReview
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2014
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vol. 4
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issue 1
A18-25
EN
Pheochromocytoma and paraganglioma derive from chromaffin tissue that secretes catecholamines, which determine the characteristic clinical picture of the disease. Serious and potentially fatal cardiovascular complications require rapid diagnosis and treatment. The available biochemical tests are recommended not only to patients with characteristic clinical symptoms, but also to all patients with incidentally identified adrenal tumours. There is a growing incidence of diagnosed germline mutations associated with familial pheochromocytoma and paraganglioma in tumours originally identified as the sporadic form, and therefore current recommendations ever more often indicate the need for genetic testing in all patients with diagnosed pheochromocytoma or paraganglioma. Surgical treatment, which is the treatment of choice, brings very good results in the case of benign tumours, and even the malignant ones, provided they are operational. Unfortunately, there is no sufficiently good and efficacious alternative treatment for patients with unresectable or metastatic malignant tumours.
4
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Carotid Body Tumor – radiological imaging and genetic assessment

75%
Berger G., Łukasiewicz A., Grinevych V., Tarasów E.
Polish Journal of Surgery
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2020
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vol. 92(6)
39-44
EN
Carotid Body Tumor i.e. Paraganglioma is a challenging entity from the point of view of multidisciplinary diagnosis. The main treatment option, i.e. surgery, yields intraoperative risk related to cranial nerve palsy and vascular morbidity. Bifurcation of the common carotid artery, especially at the carotid body, is the place were head and neck paraganglioma is most frequently seen, i.e. in 60% of cases [15]. Indeed, the knowledge of genetic germline SDH mutations, which cause deregulation of hypoxiainduced factors, allows for better understanding of the tumor nature. It is recommended to conduct selective neck dissection in regions IIA, IIB, III to exclude malignant transformation and metastasis, due to malignant potential of carotid body tumors, especially in case of SDHB mutation. SDHD mutation is the main cause of hereditary HNPGLs. Computed tomography (CT), magnetic resonance imaging (MRI) and angiography allow for thorough assessment of paraganglioma extension. In large tumors embolization of the supplying artery under guidance of angiography may be considered. In case of carotid body tumor, differential diagnosis should include: carotid artery aneurysm, lymphadenopathy, Schwannoma of the hypoglossal nerve or accessory thyroid gland.
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