Rosai-Dorfman Disease, Presenting as a Right Atrial Mass with Involvement of the Tricuspid Valve in a 54-Year-Old Woman.

BACKGROUND Sinus histiocytosis with massive lymphadenopathy (SHML), Rosai-Dorfman disease, or Rosai-Dorfman-Destombes disease (RDD), is a rare non-Langerhans cell of unknown etiology. This report is of a case of isolated SHML, or Rosai-Dorfman disease, presenting as a right atrial mass with involvement of the tricuspid valve in a 54-year-old woman. This case shows the challenges of diagnosing this condition in the heart and the challenges of treating this rare disease with the limited information on the efficacy of the treatment modalities. CASE REPORT A 54-year-old Asian woman presented to the Emergency Department with chest and right upper quadrant pain. Transthoracic echocardiogram and computed tomography angiography showed a right atrium mass at the level of the tricuspid valve, causing moderate-severe regurgitation. Partial tumor debulking with biopsies later showed pink-yellow soft tissue, with histopathology showing histiocytes demonstrating emperipolesis. The tumor was positive for CD68 and S100 and negative for CD1a, consistent with Rosai-Dorfman disease. Subsequently the patient received targeted therapy with cobimetinib, without worsening cardiac function or disease progression. CONCLUSIONS This case highlights the challenging histopathological diagnosis of SHML, or Rosai-Dorfman disease, particularly in non-lymphoid tissue, such as the heart. Obtaining tissue for diagnosis can be challenging in this organ. Treatment is challenging when the mass cannot be extracted completely, like in our case, because other forms of therapies are not well studied and warrant further investigation, such as cobimetinib, which is a MEK pathway inhibitor approved in 2022 by the US Food and Drug Administration for histiocytic neoplasms.

Cardiac Sarcoidosis Presented With Hiccups: A Case Report and Literature Review.

Sarcoidosis is a multisystem disorder of unknown etiology commonly associated with hilar lymphadenopathy and granulomas. Cardiac involvement is less common; however, sarcoidosis is a known cause of restrictive cardiomyopathy. It typically presents as new-onset arrhythmias or heart failure, although cases of sudden cardiac death have been reported. We present a case of a 56-year-old male with a known history of pulmonary sarcoidosis, not on active treatment, who presented to the emergency department with a week of continuous hiccups every few seconds associated with non-exertional dyspnea. An initial computed tomography (CT) scan of the chest showed multiple stellate-like ground-glass opacities and the progression of bronchiectasis. Troponins were negative. On the initial electrocardiogram (EKG), he was found to be in atrial flutter and was admitted to the medical floor. Cardiology was consulted for suspected cardiac sarcoidosis, and they recommended transfer to the tertiary care center for further evaluation. Upon arrival, the patient underwent catheter ablation for atrial flutter and returned to sinus rhythm after the procedure. The initial nuclear scan with gallium was not suggestive of cardiac sarcoidosis. However, subsequent cardiac magnetic resonance imaging (MRI) showed cardiac involvement. Due to the high risk of arrhythmias, the patient was scheduled for implantable cardioverter defibrillator placement before discharge. The patient was given oral prednisone. The patient was discharged in stable condition, and interrogation of the device found it well functioning, and no significant arrhythmias were noted. Presentation of cardiac sarcoidosis can be variable, and any should be considered in any patient with a known history of sarcoidosis who presents with atypical symptoms above the diaphragm, such as hiccups or with new-onset arrhythmias.

Cardiac Arrest as the Initial Presentation of Undiagnosed Kawasaki Disease: A Case Report and Literature Review.

Kawasaki Disease (KD) is a self-limited acute vasculitis that mainly affects medium-sized arteries in childhood, with the coronary arteries being one of the main targets. A well-known complication is a coronary aneurysm with myocardial ischemia. We report the case of a 29-year-old female with an insignificant past medical history who presented with sudden cardiac arrest. Labs were significant for elevated troponin, consistent with non-ST elevation myocardial infarction, given diffuse ST depression on the electrocardiogram. The patient underwent a coronary angiogram that revealed diffuse coronary artery disease with multiple ulcerations, aneurysms, and occlusions consistent with KD, despite denying prior history. Cardiac arrest may be the initial presentation of undiagnosed KD and should be considered as one of the differential diagnoses.

Hypoglycemia as a Cause of Reversible Recurrent Chorea in a Diabetic Uremic Patient: A Rare Presentation.

Chorea is characterized by involuntary, fidgety, dance-like movements caused by basal ganglia lesions. It has frequently been reported with hyperglycemia in diabetic patients, but not in association with hypoglycemia. We report on a diabetic male on hemodialysis who developed recurrent, acute, reversible choreiform movements associated with repeat episodes of hypoglycemia. Imaging was able to capture brain lesions corresponding to the acute episodes and the resolution of lesions between acute episodes.

Reverse Takotsubo Cardiomyopathy in a Critically Ill Patient in the ICU: A Case Report With Literature Review.

Takotsubo, or stress cardiomyopathy (SC), is described as a transient systolic dysfunction of the apical segments of the left ventricle mainly triggered by emotional or physical stress resembling the presentation of an acute coronary syndrome in the absence of obstructive coronary artery disease. Reverse Takotsubo SC is a rare variant of SC that presents with basal ballooning instead of apical ballooning seen in classic SC. We present a case of a 74-year-old male who was admitted to the ICU with septic shock. Laboratory test results showed elevated troponin. An echocardiogram showed reduced cardiac contractility and relative hypokinesis of the basal and mid segments compared to the apical segments, consistent with reverse Takotsubo SC, which recovered after 10 days. It can happen in critically ill patients in the ICU secondary to severe sepsis and could contribute to hemodynamic worsening, affecting the final clinical outcomes.