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Bilateral diffuse tumorous pseudoangiomatous stromal hyperplasia treated with bilateral mastectomy in a 40-year-old woman.

ABSTRACT: Pseudoangiomatous stromal hyperplasia (PASH) is a rare benign breast disease characterized by breast stromal proliferation mimicking low grade angiosarcoma. PASH is frequently detected as an incidental component coexisting with other breast lesions. However, it can also present as a single localized mass and is typically unilateral. Mammographic and sonographic findings are nonspecific and indistinguishable from those of benign lesions. We report an unusual case of PASH presenting with diffuse enlargement of the bilateral breasts in a 40-year-old woman treated with bilateral mastectomy.

SUBMITTER: Ko SY

PROVIDER: S-EPMC6156700 | biostudies-literature | 2018 Dec

REPOSITORIES: biostudies-literature

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Bilateral diffuse tumorous pseudoangiomatous stromal hyperplasia treated with bilateral mastectomy in a 40-year-old woman.

Ko Su Yeon SY Koh Myeong Ju MJ

Radiology case reports 20180925 6

Pseudoangiomatous stromal hyperplasia (PASH) is a rare benign breast disease characterized by breast stromal proliferation mimicking low grade angiosarcoma. PASH is frequently detected as an incidental component coexisting with other breast lesions. However, it can also present as a single localized mass and is typically unilateral. Mammographic and sonographic findings are nonspecific and indistinguishable from those of benign lesions. We report an unusual case of PASH presenting with diffuse e ...[more]

PMID: 30263085

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Project description:BACKGROUND:Endomyocardial fibrosis (EMF) represents the most common cause of restrictive cardiomyopathy worldwide. Despite a high prevalence in tropical regions, it occasionally occurs in patients who have never visited these areas. While researches have proposed various possible triggers for EMF, etiology and pathogenesis remain largely unknown. Diagnosis is based on patient history, heart failure symptoms, and echocardiographic signs of restrictive ventricular filling, atrioventricular valve regurgitation and frequently apical thrombus. Following is a case report of an Austrian patient with EMF who eventually had to undergo a heart transplant. This case report strives to promote awareness for this in non-tropical areas uncommon but nevertheless detrimental disease. CASE PRESENTATION:A 40-year-old woman was presented at our emergency department with chest pain and fever up to 38.1° Celsius. Plasma troponin-T levels and inflammatory markers were slightly elevated, but the echocardiogram was without pathological findings. The patient was hospitalized on the suspicion of acute myocarditis and discharged soon after improvement. Eight months later, she was presented again with chest pain and symptoms of heart failure. The echocardiogram showed normal systolic left ventricular (LV) function with LV wall thickening and severe restrictive mitral regurgitation as well as aortic and tricuspid regurgitation. Coronary angiogram was normal but right heart catheterization showed pulmonary hypertension due to left heart disease. Further diagnostic workup with cardiac magnetic resonance imaging revealed subendocardial late enhancement and apical thrombus formation in the left ventricle compatible with the diagnosis of EMF. A comprehensive diagnostic workup showed no evidence of infection, systemic immunologic or hematological disease, in particular hypereosinophilic syndrome. After a multidisciplinary consideration of several therapeutic options, the patient was listed for heart transplantation. On the waiting list, she deteriorated rapidly due to progressive heart failure and finally underwent a heart transplantation. Histological examination confirmed the diagnosis of EMF. Six years after her heart transplantation, the patient was presented in an excellent clinical condition. CONCLUSIONS:Even in non-tropical regions, the diagnosis of EMF should always be considered in restrictive cardiomyopathy. Knowledge of the distinct phenotype of EMF facilitates diagnosis, but comprehensive workup and therapeutic management remain challenging and require a multidisciplinary approach.

Dataset Information

Bilateral diffuse tumorous pseudoangiomatous stromal hyperplasia treated with bilateral mastectomy in a 40-year-old woman.

Publications

Bilateral diffuse tumorous pseudoangiomatous stromal hyperplasia treated with bilateral mastectomy in a 40-year-old woman.

Similar Datasets

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