FROM ACUTE CORONARY SYNDROME TO MALIGNANT PHEOCHROMOCYTOMA: A TWIST OF FATE

Objective: Malignant pheochromocytomas are endocrine tumors which develop in the chromaffin tissue. The diagnosis of malignancy is based on the volume of the tumor, the invasion of neighboring organs and the presence of metastases. Design and method: A 47-year-old woman presented with a clinical picture of ACS – NSTEMI but with a suggestive history of adrenergic crises (diaphoresis, nausea, palpitations and high blood pressure – with short course). The angiography showed permeable coronary arteries. The echocardiography was normal. The biochemical work-up revealed significant elevations in plasma and the 24-hour urine metanephrines and catecholamines. The CT scan of abdomen and pelvis showed a highly vascularized 9.3/7.6/11 cm right adrenal mass (which was not individualized) with necrosis areas, without cleavage plane with the liver and creating a mass effect on the surrounding organs. The abdominal scan later on discloses a hypoechogenous mass inside the inferior vena cava suggestive of thrombus. She was given antihypertensive and anticoagulant treatment, doxazosin and subsequently carvedilol, prior to surgery. Results: Excision of the tumor mass is thus performed, the patient having a favorable postoperative evolution, with the sampling of a nodular formation of increased dimensions 10/10/6 cm, delimited by a capsule, with an inhomogeneous appearance, with areas of necrosis, hemorrhagic areas and cystic areas. Microscopically, diffuse cell growth, with increased density, tumor necrosis, increased mitotic activity, atypical mitoses and marked nuclear pleomorphism are detected - aspects that allow the achievement of PASS scores and APP scores - with highly suggestive results for malignant pheochromocytoma. To complete the diagnostic scores, IHC tests are needed to determine the proliferation factors and SDHB mutation testing – with results currently in progress. Conclusions: The particularities of the case identified by us are the following: significant EKG changes despite the structurally normal heart, malignant pheochromocytoma with atypical manifestation - ACS type II, early diagnosis before the appearance of metastases or loco-regional invasion in patients with impressively large tumors.

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Publication Details

Journal
Journal of Hypertension
Published
2025-05-01
DOI
https://doi.org/10.1097/01.hjh.0001117812.94480.d0
Primary Topic
Adrenal and Paraganglionic Tumors
Type
article
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article

FROM ACUTE CORONARY SYNDROME TO MALIGNANT PHEOCHROMOCYTOMA: A TWIST OF FATE

Daniela Gheorghe, Diana Ciobanu, Mihai Radu, Alice Elena Munteanu
Journal of Hypertension
Adrenal and Paraganglionic Tumors
article

FROM ACUTE CORONARY SYNDROME TO MALIGNANT PHEOCHROMOCYTOMA: A TWIST OF FATE

Daniela Gheorghe, Diana Ciobanu, Mihai Radu, Alice Elena Munteanu
article en

Abstract

Objective: Malignant pheochromocytomas are endocrine tumors which develop in the chromaffin tissue. The diagnosis of malignancy is based on the volume of the tumor, the invasion of neighboring organs and the presence of metastases. Design and method: A 47-year-old woman presented with a clinical picture of ACS – NSTEMI but with a suggestive history of adrenergic crises (diaphoresis, nausea, palpitations and high blood pressure – with short course). The angiography showed permeable coronary arteries. The echocardiography was normal. The biochemical work-up revealed significant elevations in plasma and the 24-hour urine metanephrines and catecholamines. The CT scan of abdomen and pelvis showed a highly vascularized 9.3/7.6/11 cm right adrenal mass (which was not individualized) with necrosis areas, without cleavage plane with the liver and creating a mass effect on the surrounding organs. The abdominal scan later on discloses a hypoechogenous mass inside the inferior vena cava suggestive of thrombus. She was given antihypertensive and anticoagulant treatment, doxazosin and subsequently carvedilol, prior to surgery. Results: Excision of the tumor mass is thus performed, the patient having a favorable postoperative evolution, with the sampling of a nodular formation of increased dimensions 10/10/6 cm, delimited by a capsule, with an inhomogeneous appearance, with areas of necrosis, hemorrhagic areas and cystic areas. Microscopically, diffuse cell growth, with increased density, tumor necrosis, increased mitotic activity, atypical mitoses and marked nuclear pleomorphism are detected - aspects that allow the achievement of PASS scores and APP scores - with highly suggestive results for malignant pheochromocytoma. To complete the diagnostic scores, IHC tests are needed to determine the proliferation factors and SDHB mutation testing – with results currently in progress. Conclusions: The particularities of the case identified by us are the following: significant EKG changes despite the structurally normal heart, malignant pheochromocytoma with atypical manifestation - ACS type II, early diagnosis before the appearance of metastases or loco-regional invasion in patients with impressively large tumors.

Journal of HypertensionVol. 43(Suppl 1)
Romanian Society of Nephrology (RO)
Good health and well-being
Openalex Percentile: Top 80%
Adrenal and Paraganglionic Tumors
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