Why the study?
Primary angiosarcoma within the sinus of Valsalva is uncommon with no previously documented instances, making diagnosis challenging due to the absence of apparent clinical manifestations.
Primary angiosarcoma can rarely arise within the sinus of Valsalva and should be considered as a potential underlying etiology for unexplained aneurysm formation or expansion.
Rare sinus of Valsalva angiosarcoma warrants consideration in unexplained aneurysms; leaves open standardized diagnostic and therapeutic pathways.
INTRODUCTION: Primary angiosarcoma of the aorta, particularly within the sinus of Valsalva, is uncommon, with no documented instances of primary angiosarcoma. The absence of apparent clinical manifestations in this severe condition makes it challenging to diagnose, often resulting in a poor prognosis. CASE PRESENTATION: A 60-year-old patient underwent procedures for fistula closure and coronary artery bypass grafting, which resulted in the rupture of an aneurysm within the sinus of Valsalva. Computed tomography examination 5 years after the procedure suggested no pathological abnormalities. Nevertheless, the patient required repeat surgery at 67 years due to the observed expansion of the sinus of Valsalva aneurysm noted during a clinical evaluation, prompted by elevated levels of inflammatory markers. Exploration of the residual aneurysmal locus within the sinus of Valsalva revealed an intraluminal thrombus devoid of any demonstrable hemodynamic access into the aneurysmal sac. Histopathological assessment of the aneurysmal wall confirmed a definitive diagnosis of primary angiosarcoma within the sinus of Valsalva. After surgery, the patient exhibited pyrexia. Magnetic resonance imaging substantiated multifocal osseous metastases, corroborated by histological analysis following a bone biopsy, confirming a diagnosis of angiosarcoma. Therefore, adjuvant chemotherapy with paclitaxel was initiated. After 1 year, a sustained state of disease stability was noted. DISCUSSION: In this case, the need for surgical intervention, based on an expanded sinus of Valsalva aneurysm, culminated in the unanticipated detection of primary angiosarcoma. CONCLUSION: Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation in cases where the etiology remains obscure in the early stages of therapeutic intervention.
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Morimoto et al. (2024) studied this question.
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