Investigators Find DLBCL When Probing Apparent Intramural Hematoma

A patient with a history of intramural hematoma (IMH) who complained of chest pain turned out to have a rare case of aorta-involved diffuse large B-cell lymphoma (DLBCL), according to a new report in the journal JACC: Case Reports.

The patient, a 73-year-old man, had a history of hypothyroidism and hypertension, explained corresponding author Elizabeth Jean-Marie, MD, MS, of University Hospitals Cleveland Medical Center. Three months before the episode reported in the case report, he had been diagnosed with aortic IMH and underwent thoracic endovascular aortic repair (TEVAR), during which a Bolton Relay thoracic stent was placed. The patient was discharged with no apparent issues.

When he returned to the hospital 3 months later, the patient said he had experienced chest pain while doing yard work, after which he also developed flank pain. He tried to rest and take over-the-counter medications. When those did not work, he went to the emergency department.

When doctors initially evaluated the patient, he reported having lost 9 points in the previous 2 months. Other than that, his vital signs and oxygen saturation were normal.

Due to his recent TEVAR procedure, Jean-Marie and colleagues said they initially suspected a complication like a stent graft infection, type 1 endoleak, or other cardiac issues. When the patient was given a computed tomography (CT) angiogram, it showed a periaortic fluid collection stretching from the level of the T8 to T11 vertebral bodies. There were no signs of infection, and his blood cultures were negative for leukocytosis or other signs of inflammation, the authors said.

Next, the patient was sent for positron emission tomography, which showed “a hypermetabolic periaortic bulky mass surrounding the majority of the adjacent aorta, engulfing the adjacent spine at the T8 through T11 vertebral bodies and extending into the spinal canal.”

A biopsy and pathological exam allowed his physicians to diagnose DLBCL. The patient was referred to the oncology department where he underwent 6 cycles of chemotherapy. After 6 months, he had metabolic resolution of the mass, and there was no evidence of hypermetabolic extranodal disease or distal lymphomatous involvement, the authors said.

Jean-Marie and colleagues noted that acute aortic syndromes are life-threatening and require swift action. Absent aortic rupture, they said patients typically are hypertensive and tachycardic, and usually report pain of the chest, back, abdomen or lower extremities.

“In the correct clinical context, healthcare providers should maintain a high index of suspicion for acute aortic syndromes and initiate the appropriate work-up, which usually includes electrocardiogram-gated high-resolution CT or magnetic resonance angiography,” they said.

In this case, imaging showed no signs of an endoleak or infection, prompting investigators to look at other potential causes of the periaortic fluid collection.

“On careful review of his historical records, it was noted that the fluid collection had grown considerably over the intervening months, and it demonstrated evidence of direct extension into surrounding tissue as it grew posteriorly toward the spine,” the authors said.

The change led to their decision to use PET imaging, they said.

The investigators said the case shows DLBCL is an important consideration in patients with persistent periaortic fluid collections.

“Once DLBCL is identified, it is vital to have prompt initiation of chemotherapy of this clinically aggressive malignant disease,” they concluded.

Reference

Jean-Marie E, Gillombardo CB, Baeza C, Hoit BD. Diffuse large B-cell lymphoma mimicking intramural hematoma of the thoracic aorta. JACC Case Rep. 2023;15:101858. doi:10.1016/j.jaccas.2023.101858