A Case of Paraganglioma with Cyanotic Congenital Heart Disease
Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently dev...
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| Format: | Article |
| Language: | English |
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Interna Publishing
2021-03-01
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| Series: | Acta Medica Indonesiana |
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| Online Access: | https://actamedindones.org/index.php/ijim/article/view/1065 |
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| author | Norasyikin A Wahab Beh Hui Chien Mohd Rahman Omar Aini Ab Aziz Norlaila Mustafa Norlela Sukor Nor Azmi Kamaruddin |
| author_facet | Norasyikin A Wahab Beh Hui Chien Mohd Rahman Omar Aini Ab Aziz Norlaila Mustafa Norlela Sukor Nor Azmi Kamaruddin |
| author_sort | Norasyikin A Wahab |
| collection | DOAJ |
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Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life.
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| format | Article |
| id | doaj-art-d6cc3b31d2bd45209e49e6f7b13e8706 |
| institution | DOAJ |
| issn | 0125-9326 2338-2732 |
| language | English |
| publishDate | 2021-03-01 |
| publisher | Interna Publishing |
| record_format | Article |
| series | Acta Medica Indonesiana |
| spelling | doaj-art-d6cc3b31d2bd45209e49e6f7b13e87062025-08-20T02:50:48ZengInterna PublishingActa Medica Indonesiana0125-93262338-27322021-03-01531422A Case of Paraganglioma with Cyanotic Congenital Heart DiseaseNorasyikin A Wahab0Beh Hui Chien1Mohd Rahman Omar2Aini Ab Aziz3Norlaila Mustafa4Norlela Sukor5Nor Azmi Kamaruddin6Department of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaUniversiti Sains Islam MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, MalaysiaDepartment of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia Co-occurrence of cyanotic congenital heart disease (CCHD) and phaeochromocytoma (PCC) and paraganglioma (PGL) are rare, although some cases have been reported. We report a case of left paraganglioma in a 20-year-old lady with an underlying CCHD who underwent palliative Glenn shunt, subsequently developed polycythaemia and cavernous sinus thrombosis presented with palpitation, sweating, headache and hypertension of 3-months duration at the age of 17. The abdominal CT scan revealed an enhancing left paraaortic mass measuring 5.2 cm x 4.4 cm x 3.8 cm. A 24-hour urine catecholamine demonstrated raised noradrenaline level to six times upper limit of normal and hence diagnosis of left sympathetic (sPGL) was made. In view of the delayed diagnosis and significant morbidity associated with her condition, surgical treatment is no longer an option. Therefore, vigilant screening and early treatment of PCC-PGL in patients with CCHD are crucial in order to avoid significant morbidity and ensure a good quality of life. https://actamedindones.org/index.php/ijim/article/view/1065cyanotic congenital heart diseaseparagangliomapheochromocytomahypoxia |
| spellingShingle | Norasyikin A Wahab Beh Hui Chien Mohd Rahman Omar Aini Ab Aziz Norlaila Mustafa Norlela Sukor Nor Azmi Kamaruddin A Case of Paraganglioma with Cyanotic Congenital Heart Disease Acta Medica Indonesiana cyanotic congenital heart disease paraganglioma pheochromocytoma hypoxia |
| title | A Case of Paraganglioma with Cyanotic Congenital Heart Disease |
| title_full | A Case of Paraganglioma with Cyanotic Congenital Heart Disease |
| title_fullStr | A Case of Paraganglioma with Cyanotic Congenital Heart Disease |
| title_full_unstemmed | A Case of Paraganglioma with Cyanotic Congenital Heart Disease |
| title_short | A Case of Paraganglioma with Cyanotic Congenital Heart Disease |
| title_sort | case of paraganglioma with cyanotic congenital heart disease |
| topic | cyanotic congenital heart disease paraganglioma pheochromocytoma hypoxia |
| url | https://actamedindones.org/index.php/ijim/article/view/1065 |
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