4 - Cardiac Rosai-Dorfman Disease: tissue characteristics by Cardiac Magnetic Resonance

Thursday, February 13, 2020

6:40 PM – 6:50 PM

Location: Canary 2

Description of Clinical Presentation: A 44-year-old female patient sought medical attention due to sudden onset of palpitation. She had previous diagnoses of Rosai-Dorfman Disease (RDD) with extranodal infiltration in the kidneys, antiphospholipid antibody syndrome and hypothyroidism. She underwent high-dose corticosteroid treatment for RDD during a year with a satisfactory response.
The admission ECG showed ST elevation in D1, aVL and V2 leads with elevated cardiac enzymes. Referred to emergency coronary catheterization, which showed no coronary artery stenosis.
Transthoracic echocardiogram revealed rounded echogenic image inside the right atrium, close to the interatrial septum, measuring 32mm. She was sent to cardiac magnetic resonance (CMR) for clarification of mass etiology, which showed two cardiac masses with same tissue characteristic. One mass was located in the right atrium, non-pedunculated, with little mobility and regular borders. A second mass was located intramurally and infiltrating the basal anterior left ventricular wall.
On the Heart Team decision, the possibility of biopsy was denied due to the high risk of the procedure, and clinical treatment with corticosteroids was chosen.

Diagnostic Techniques and Their Most Important Findings: CMR images in short and long-axes planes were acquired using cine-MRI (SSFP) for evaluation of ventricular function, T1 and T2-weighed Fast Spin-echo, first-pass perfusion and Late Gadolinium Enhancement (LGE) for tissue characterization.
Figure 1. Long-axis 4-chamber view showing right atrial mass, with hyperenhancement in LGE images (panel A) and isointense signal to myocardium in SSFP (panel B).
Figure 2. Short-axis view showing basal anterior intramyocardial mass, with isointense signal to myocardium in SSFP (panel A), hyperintese in T2w-FSE (panel B) and hyperenhancement in LGE images (panel C).

Learning Points from this

Case: RDD is a rare disease with a prevalence of 1:200000, and consists of sinus histiocytosis with massive lymphadenopathy. It is a benign and self-limiting disease of unknown etiology (1). Classical manifestations include cervical lymphadenopathy usually accompanied by neutrophilia leukocytosis, and polyclonal hypergammaglobulinemia (2). The morphological profile is an inflammatory infiltrate with lymphocytes, plasma cells and large histiocytes, which is the hallmark of the disease. Extranodal involvement occurs in approximately 25-43% of cases, especially skin. Cardiac involvement (cRDD) is extremely rare with < 0.1% of the cases (3). The RDD management depends on the extent and sites of involvement (4). Surgical resection is indicated for localized disease, with or without adjuvant radiotherapy. In case of systemic and/or extensive extranodal disease, first line therapy is corticosteroids. The present case aimed to emphasize the rarity of cRDD as well as to highlight the CMR tissue characteristics of cRDD and the value of CMR for guiding treatment strategies.

Primary Author(s)

CS

CAMILA G. SILVEIRA, MD

HCOR

Disclosure: Disclosure information not submitted.

Co-Author(s)

BA

BRUNO A. AZEVEDO, MD, PhD

HCOR

Disclosure: Disclosure information not submitted.

Presenting Author(s)

Paula Costa, MD

Cardiologist
HCOR

Disclosure: Disclosure information not submitted.

Co-Author(s)

PB

Pedro Barbosa, MD

Cardiologist
HCOR

Disclosure: Disclosure information not submitted.
FR

FERNANDA RÊGO, MD

HCOR

Disclosure: Disclosure information not submitted.
LL

LARA LEITE, MD

HCOR

Disclosure: Disclosure information not submitted.
TM

TIAGO A. MAGALHÃES, MD, PhD

HCOR

Disclosure: Disclosure information not submitted.
JB

JULIANA BELLO, MD

HCOR

Disclosure: Disclosure information not submitted.
VM

VALÉRIA MOREIRA, MD

HCOR

Disclosure: Disclosure information not submitted.
CP

CARLOS EDUARDO PRAZERES, MD

HCOR

Disclosure: Disclosure information not submitted.
AC

Adriano Carneiro, MD

Cardiologist
HCOR

Disclosure: Disclosure information not submitted.
Carlos E. Rochitte, MD, PhD, FSCMR

Academic Coordinator of Cardiovascular MRI and CT at Heart Institute (InCor), University of São Paulo Medical School Director of Cardiovascular MRI and CT at Heart Hospital, Hospital do Coração, HCOR
Heart Institute (InCor), University of São Paulo Medical School and Heart Hospital, Hospital do Coração, HCOR

Disclosure: I do not have any relevant financial / non-financial relationships with any proprietary interests.