A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review
A 50-year-old Omani woman presented to the Outpatient Clinic, Royal Hospital, Oman with right upper abdominal pain and backache that had lasted 10 days. She had no palpitation, sweating, or hypertension (blood pressure 122/78mmHg). The patient’s history revealed that she had a similar incidence of a...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
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Oman Medical Specialty Board
2015-09-01
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| Series: | Oman Medical Journal |
| Subjects: | |
| Online Access: | http://www.omjournal.org/fultext_PDF.aspx?DetailsID=699&type=fultext |
| _version_ | 1817976153148751872 |
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| author | Waad-Allah S. Mula-Abed Riyaz Ahmed Fatima A. Ramadhan Manal K. Al-Kindi Noor B. Al-Busaidi Hilal N. Al-Muslahi Mohammad A. Al-Lamki |
| author_facet | Waad-Allah S. Mula-Abed Riyaz Ahmed Fatima A. Ramadhan Manal K. Al-Kindi Noor B. Al-Busaidi Hilal N. Al-Muslahi Mohammad A. Al-Lamki |
| author_sort | Waad-Allah S. Mula-Abed |
| collection | DOAJ |
| description | A 50-year-old Omani woman presented to the Outpatient Clinic, Royal Hospital, Oman with right upper abdominal pain and backache that had lasted 10 days. She had no palpitation, sweating, or hypertension (blood pressure 122/78mmHg). The patient’s history revealed that she had a similar incidence of abdominal pain two months prior, which was a "dull ache" in nature and somewhat associated with headache. The pain was relieved using a mild analgesic drug. Abdominal ultrasonography showed a right adrenal mass, and both computed tomography and magnetic resonance imaging of the adrenal glands confirmed a right adrenal mass consistent with adrenal pheochromocytoma. However, clinical biochemistry tests revealed normal levels of plasma catecholamines (dopamine, norepinephrine, and epinephrine) and metanephrine, which are unusual findings in adrenal pheochromocytoma. Meanwhile, the patient had markedly raised plasma normetanephrine (10-fold) which, together with the normal metanephrine, constitutes a metabolic profile that is compatible with extra-adrenal pheochromocytoma. The patient also had markedly raised chromogranin A (16-fold), consistent with the presence of a neuroendocrine tumor. Laparoscopic right adrenalectomy was done and the adrenal tumor was excised and retrieved in total. Histopathology and immunohistochemistry confirmed the diagnosis of adrenal pheochromocytoma; the tumor cells being positive for chromogranin, synaptophysin, and S-100 protein. Following surgery, the patient did well and showed full recovery at follow-up after three months. Molecular genetic testing showed no pathogenic mutation in pheochromocytoma genes: MAX, SDHA, SDHAF2, SDHB, SDHC, SDHD, VHL, and PRKAR1A. A review of the literature was conducted to identify the pathophysiology and any previous reports of such case. To our knowledge, this is the first report in Oman of the extremely rare entity of pheochromocytoma with an unusual clinical and biochemical scenario. |
| first_indexed | 2024-04-13T21:59:38Z |
| format | Article |
| id | doaj.art-e1661c87b96a4bab9a65c420210303d8 |
| institution | Directory Open Access Journal |
| issn | 1999-768X 2070-5204 |
| language | English |
| last_indexed | 2024-04-13T21:59:38Z |
| publishDate | 2015-09-01 |
| publisher | Oman Medical Specialty Board |
| record_format | Article |
| series | Oman Medical Journal |
| spelling | doaj.art-e1661c87b96a4bab9a65c420210303d82022-12-22T02:28:09ZengOman Medical Specialty BoardOman Medical Journal1999-768X2070-52042015-09-0130538239010.5001/omj.2015.76A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature ReviewWaad-Allah S. Mula-Abed0Riyaz Ahmed1Fatima A. Ramadhan2Manal K. Al-Kindi3Noor B. Al-Busaidi4Hilal N. Al-Muslahi5Mohammad A. Al-Lamki6Department of Chemical Pathology, Royal Hospital, Muscat, OmanDepartment of Endocrine Surgery, Royal Hospital, Muscat, OmanDepartment of Histopathology, Royal Hospital, Muscat, OmanDepartment of Chemical Pathology, Royal Hospital, Muscat, OmanNational Diabetes and Endocrine Centre, Royal Hospital, Muscat, OmanNational Diabetes and Endocrine Centre, Royal Hospital, Muscat, OmanMedical Endo-Metabolic Unit, Royal Hospital, Muscat, OmanA 50-year-old Omani woman presented to the Outpatient Clinic, Royal Hospital, Oman with right upper abdominal pain and backache that had lasted 10 days. She had no palpitation, sweating, or hypertension (blood pressure 122/78mmHg). The patient’s history revealed that she had a similar incidence of abdominal pain two months prior, which was a "dull ache" in nature and somewhat associated with headache. The pain was relieved using a mild analgesic drug. Abdominal ultrasonography showed a right adrenal mass, and both computed tomography and magnetic resonance imaging of the adrenal glands confirmed a right adrenal mass consistent with adrenal pheochromocytoma. However, clinical biochemistry tests revealed normal levels of plasma catecholamines (dopamine, norepinephrine, and epinephrine) and metanephrine, which are unusual findings in adrenal pheochromocytoma. Meanwhile, the patient had markedly raised plasma normetanephrine (10-fold) which, together with the normal metanephrine, constitutes a metabolic profile that is compatible with extra-adrenal pheochromocytoma. The patient also had markedly raised chromogranin A (16-fold), consistent with the presence of a neuroendocrine tumor. Laparoscopic right adrenalectomy was done and the adrenal tumor was excised and retrieved in total. Histopathology and immunohistochemistry confirmed the diagnosis of adrenal pheochromocytoma; the tumor cells being positive for chromogranin, synaptophysin, and S-100 protein. Following surgery, the patient did well and showed full recovery at follow-up after three months. Molecular genetic testing showed no pathogenic mutation in pheochromocytoma genes: MAX, SDHA, SDHAF2, SDHB, SDHC, SDHD, VHL, and PRKAR1A. A review of the literature was conducted to identify the pathophysiology and any previous reports of such case. To our knowledge, this is the first report in Oman of the extremely rare entity of pheochromocytoma with an unusual clinical and biochemical scenario.http://www.omjournal.org/fultext_PDF.aspx?DetailsID=699&type=fultextAdrenal glandsAdrenalectomyCatecholaminesChromogranin AMetanephrineNormetanephrinePheochromocytoma |
| spellingShingle | Waad-Allah S. Mula-Abed Riyaz Ahmed Fatima A. Ramadhan Manal K. Al-Kindi Noor B. Al-Busaidi Hilal N. Al-Muslahi Mohammad A. Al-Lamki A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review Oman Medical Journal Adrenal glands Adrenalectomy Catecholamines Chromogranin A Metanephrine Normetanephrine Pheochromocytoma |
| title | A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review |
| title_full | A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review |
| title_fullStr | A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review |
| title_full_unstemmed | A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review |
| title_short | A Rare Case of Adrenal Pheochromocytoma with Unusual Clinical and Biochemical Presentation: A Case Report and Literature Review |
| title_sort | rare case of adrenal pheochromocytoma with unusual clinical and biochemical presentation a case report and literature review |
| topic | Adrenal glands Adrenalectomy Catecholamines Chromogranin A Metanephrine Normetanephrine Pheochromocytoma |
| url | http://www.omjournal.org/fultext_PDF.aspx?DetailsID=699&type=fultext |
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