A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective
Introduction: Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population. Methods: We identified patients with positive histopathological confirmations of PCC and PGL who we...
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| Format: | Article |
| Language: | English |
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Wolters Kluwer – Medknow Publications
2022-11-01
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| Series: | Singapore Medical Journal |
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| Online Access: | https://journals.lww.com/10.11622/smedj.2021135 |
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| author | Yingshan Lee Leon Yuan Rui Tan Shaikh Abdul Kader Kamaldeen Abdul Shakoor |
| author_facet | Yingshan Lee Leon Yuan Rui Tan Shaikh Abdul Kader Kamaldeen Abdul Shakoor |
| author_sort | Yingshan Lee |
| collection | DOAJ |
| description | Introduction:
Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population.
Methods:
We identified patients with positive histopathological confirmations of PCC and PGL who were treated at a tertiary Singapore hospital between January 2000 and December 2015. The results were analysed for clinical presentations, treatment and long-term outcomes.
Results:
A total of 27 cases (20 PCC, 7 PGL) were identified over a 15-year period. One case of PGL developed bilateral disease on follow-up. There were 17 male and 10 female patients with a median age of 57 (range 24–77) years. A positive family history was uncommon and present in only 3.7% of patients. Uniquely, the top three presenting symptoms were abdominal discomfort, palpitations and diaphoresis. Despite adequate preoperative preparation, intraoperative haemodynamic instability occurred in 70.4% and early postoperative hypotension occurred in 11.1% of patients. After surgery, hypertension was resolved in 41.2% (7/17) and diabetes mellitus in 60% (3/5). Disease recurrence was reported in 22.2% and distant metastases in 14.8%. At the end of the follow-up period (median 35 [range 3–148] months), 70.4% were still alive.
Conclusion:
PCC and PGL can present with a wide range of symptoms. Intraoperative haemodynamic instability was frequent despite good preoperative preparation. Disease recurrences and metastasis occurred in up to one-fifth of the patients. Genetic screening should be offered to patients with PCC and PGL. |
| format | Article |
| id | doaj-art-1e7c04d3eaa740a98d76d452317e78c7 |
| institution | Kabale University |
| issn | 0037-5675 2737-5935 |
| language | English |
| publishDate | 2022-11-01 |
| publisher | Wolters Kluwer – Medknow Publications |
| record_format | Article |
| series | Singapore Medical Journal |
| spelling | doaj-art-1e7c04d3eaa740a98d76d452317e78c72025-02-10T05:47:16ZengWolters Kluwer – Medknow PublicationsSingapore Medical Journal0037-56752737-59352022-11-01631164965810.11622/smedj.2021135A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspectiveYingshan LeeLeon Yuan Rui TanShaikh Abdul Kader Kamaldeen Abdul ShakoorIntroduction: Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population. Methods: We identified patients with positive histopathological confirmations of PCC and PGL who were treated at a tertiary Singapore hospital between January 2000 and December 2015. The results were analysed for clinical presentations, treatment and long-term outcomes. Results: A total of 27 cases (20 PCC, 7 PGL) were identified over a 15-year period. One case of PGL developed bilateral disease on follow-up. There were 17 male and 10 female patients with a median age of 57 (range 24–77) years. A positive family history was uncommon and present in only 3.7% of patients. Uniquely, the top three presenting symptoms were abdominal discomfort, palpitations and diaphoresis. Despite adequate preoperative preparation, intraoperative haemodynamic instability occurred in 70.4% and early postoperative hypotension occurred in 11.1% of patients. After surgery, hypertension was resolved in 41.2% (7/17) and diabetes mellitus in 60% (3/5). Disease recurrence was reported in 22.2% and distant metastases in 14.8%. At the end of the follow-up period (median 35 [range 3–148] months), 70.4% were still alive. Conclusion: PCC and PGL can present with a wide range of symptoms. Intraoperative haemodynamic instability was frequent despite good preoperative preparation. Disease recurrences and metastasis occurred in up to one-fifth of the patients. Genetic screening should be offered to patients with PCC and PGL.https://journals.lww.com/10.11622/smedj.2021135adrenal incidentalomaneuroendocrine carcinomaparagangliomapheochromocytoma |
| spellingShingle | Yingshan Lee Leon Yuan Rui Tan Shaikh Abdul Kader Kamaldeen Abdul Shakoor A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective Singapore Medical Journal adrenal incidentaloma neuroendocrine carcinoma paraganglioma pheochromocytoma |
| title | A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective |
| title_full | A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective |
| title_fullStr | A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective |
| title_full_unstemmed | A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective |
| title_short | A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective |
| title_sort | 15 year pheochromocytoma and paraganglioma experience in a single centre a singapore perspective |
| topic | adrenal incidentaloma neuroendocrine carcinoma paraganglioma pheochromocytoma |
| url | https://journals.lww.com/10.11622/smedj.2021135 |
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