Primary angiosarcoma arising in the sinus of Valsalva: A case report

TL;DR

A rare case of primary angiosarcoma in the sinus of Valsalva was diagnosed after surgical intervention revealed an unusual aneurysm.

Contribution

This is the first documented case of primary angiosarcoma arising in the sinus of Valsalva.

Findings

Primary angiosarcoma in the sinus of Valsalva is extremely rare and difficult to diagnose early.

Surgical exploration of an aneurysm led to the unexpected discovery of angiosarcoma.

Abstract

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.

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.

In this case, the need for surgical intervention, based on an expanded sinus of Valsalva aneurysm, culminated in the unanticipated detection of primary angiosarcoma.

Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation in cases where the etiology remains obscure in the early stages of therapeutic intervention.

•Primary aortic angiosarcoma arising in the sinus of Valsalva is uncommon.•It exacerbates the challenge of early diagnosis and worsens the prognostic outlook.•Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation.

Primary aortic angiosarcoma arising in the sinus of Valsalva is uncommon.

It exacerbates the challenge of early diagnosis and worsens the prognostic outlook.

Neoplastic etiologies may plausibly underlie the pathogenesis of aneurysm formation.

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