Unusual case report of malignant pheochromocytoma presenting with STEMI

Abstract

Background: Pheochromocytomas (PHEOs) are a group of tumours that leads to multiple symptoms and can induce hypercoagulability and promote thrombosis. Pheochromocytomas may also present without elevated serum and urinary markers. We aimed to provide tips and tricks for the diagnostic and therapeutic management of an unusual case of PHEOs.

Case summary: Thirty-four-year-old woman with the unremarkable medical history presented with epigastric pain and dyspnoea. Electrocardiogram showed ST-segment elevation in the inferior limb leads. She underwent an emergency coronary angiogram, which showed a high thrombus burden in the distal right coronary artery. A subsequent echocardiogram demonstrated a 31 × 33 mm right atrial mass adhering to the inferior vena cava and abdominal computed tomography (CT) scan revealed a 113 × 85 mm necrotic mass in the left adrenal bed, with tumour thrombus extending proximally to the confluence of hepatic veins immediately inferior to the right atrium and distally to iliac vein bifurcation. Blood parameters, thrombophilia panel, vanillylmandelic acid, 5 hydroxy indole acetic acid, and homovanillic acid levels were normal. Tissue sampling confirmed the diagnosis of PHEOs. The surgical procedure was not planned due to the presence of metastatic foci on imaging, including positron emission tomography (PET)-CT. Anticoagulation with rivaroxaban and treatment with ¹⁷⁷Lu-DOTATATE-based peptide receptor radionuclide therapy (PRRT) was initiated.

Discussion: The coexistence of arterial and venous thrombosis is extremely rare in patients with PHEOs. Multidisciplinary approaches are required for the care of such patients. Catecholamines likely contributed to the development of thrombosis in our patient. Early recognition of PHEOs is the key point to ameliorate clinical outcomes.

Keywords: Acute coronary syndrome; Case reports; Pheochromocytoma; Pulmonary embolisms.

Figure 1

(A) Coronary angiogram showed high thrombus burden in the distal right coronary artery (Thrombolysis In Myocardial Infarction Grade 0 flow). (B) Thrombolysis In Myocardial Infarction Grade 3 flow was achieved in control angiography. (C) All other segments of the epicardial coronary arteries (Thrombolysis In Myocardial Infarction Grade 3 flow) were normal.

Figure 2

(A and B) An echocardiogram showed a 31 × 33 mm right atrial mass adhering to the inferior vena cava.

Figure 3

(A and B) Acute near-occlusive embolism in the left main pulmonary artery. Embolus was massive, involving bilateral pulmonary arteries, starting at the interlobar artery on the right side. There was evidence of right heart strain with a right to left ventricle ratio of 1.4. Hypodense peripheral consolidations with some ground glass changes in both lung bases were compatible with pulmonary infarctions. (C) Enhancing large round mass in the left adrenal bed inseparable from the left kidney and with spoke-wheel appearance and central necrosis. Also noted is an expansile occlusive thrombus involving the inferior vena cava. (D) Thrombus extended to the iliac bifurcation and bilateral common iliac veins. It was unclear if the thrombus extended beyond the level of common iliac veins due to potential contrast mixing artefacts.

Figure 4

(A–D) High power (×400) view, haematoxylin–eosin staining, mass in the kidney, tru-cut biopsy. (A) Tumour cells with large, abundant, fine granular cytoplasm, (B) large, polygonal, nuclear pseudoinclusions, (C) granular, amphiphilic cytoplasm, pleomorphic nucleus, and marked nucleolus, and (D) round-oval nucleus, prominent nucleolus, (A–D) atypical mitosis (arrowhead).

Figure 5

(A–D) Immunohistochemical study. (A) Diffuse strong positive immune reaction with vimentin antibody (×200), (B)- diffuse strong positive immune reaction with CD56 antibody (×200), (C) diffuse positive immune reaction with synaptophysin antibody (×200), and (D) suspected positive immunoreaction with MelanA antibody (×100).