ePlasty: Vol. 20
PIK3CA-Related Overgrowth Syndrome (PROS) and Angiosarcoma: A Case Report
Brielle Weinstein, MD,a Evita Henderson-Jackson, MD,b C. Wayne Cruse, MD,a,c and Andrew S. Brohl, MDc,d

aDepartment of Plastic Surgery, University of South Florida, Tampa; bDepartments of Pathology; cCutaneous Oncology; and dSarcoma, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Fla


Correspondence: andrew.brohl@moffitt.org
Keywords: PIK3CA-related overgrowth syndrome, angiosarcoma, CLOVES, cutaneous oncology, cancer genetics

DESCRIPTION

A 73-year-old man presented with cutaneous angiosarcoma of the left temporal scalp. In addition, he had a constellation of clinical findings including hemihypertrophy, lipomatous overgrowth with truncal distribution, scoliosis, epidermal nevi, and later, with tissue identification of PIK3CA mutation, he was diagnosed with PIK3CA-related overgrowth syndrome (PROS), likely CLOVES syndrome.

QUESTIONS

1. What is CLOVES syndrome?

2. What is PIK3CA Related Overgrowth Syndrome (PROS) and which pathways can this gene affect?

3. Describe the clinical presentations of cutaneous angiosarcoma.

4. What are appropriate comprehensive oncologic treatment regimens for cutaneous angiosarcoma?