Pembrolizumab as an Off-Label Treatment of Facial Angiosarcoma: A Case Report

May 2026 | Volume 25 | Issue 5 | 9348 | Copyright © May 2026


Published online April 29, 2026

Alexander R. Gomez-Lara BA BSa, Ritika Khandpur MDb

aKaiser Permanente Bernard J. Tyson School of Medicine, Pasadena, CA
bDepartment of Dermatology, Southern California Permanente Medical Group, Downey, CA

Abstract
Summary of Case: We report an atypical case of cutaneous angiosarcoma presenting as unilateral nasal swelling in an elderly male. The lesion was unresponsive to topical and antibiotic therapy. Diagnosis was confirmed after surgical excision, and disease control was ultimately achieved through radiation and off-label use of pembrolizumab.
Patient Info: A 79-year-old male with a history of squamous cell carcinoma presented with a 1–2 month history of erythematous, scaly swelling of the left nasal tip and ala. Family history was notable for nonmelanoma skin cancer.
Diagnosis: Initial differential diagnoses included contact dermatitis, rosacea, and rhinophyma. A shave biopsy revealed actinic damage but no malignancy. Following persistent symptoms and referral to plastic surgery, debulking surgery was performed. Histopathology confirmed cutaneous angiosarcoma.
Treatment: The patient declined wide local excision and instead underwent a 4-week course of hypofractionated radiation. Subsequent imaging showed regional lymph node recurrence. He declined biopsy and surgery, and was started on off-label pembrolizumab immunotherapy for disease control.
Outcome: Following immunotherapy, the patient experienced sustained remission with no clinical or radiographic evidence of disease four years post-treatment.
Discussion: Cutaneous angiosarcoma is a rare, aggressive malignancy with a poor prognosis. This case illustrates the importance of considering angiosarcoma in atypical facial lesions and demonstrates disease control using a non-traditional, patient-centered treatment strategy. Immunotherapy with pembrolizumab may offer a promising option in select patients, particularly those with contraindications to extensive surgery.

INTRODUCTION

Cutaneous angiosarcoma is a rare, aggressive malignant neoplasm of vascular endothelial cell origin with a poor prognosis, due to its rapid growth and propensity for local recurrence and distant metastasis.1,2 The typical presentation of cutaneous angiosarcoma involves a progressive, bruise-like lesion on the head or neck.2 In most cases, the scalp is the predominant site affected, while other less commonly affected areas include the trunk and extremities. Cutaneous angiosarcoma, despite its poor prognosis, is extremely rare accounting for less than 1% of all sarcomas.3 The incidence rate is approximately 3.3 cases per 1,000,000 person-years, with higher prevalence in those over 70 years of age.3 As Wagner et al (2024) highlight, the increase in cases within a 20-year period emphasizes the need for early detection and treatment. Current treatment for cutaneous angiosarcoma includes a multimodal approach due to its aggressive nature and high recurrence rate. Treatment plans usually include wide local excision, radiation therapy, and chemotherapy.4

We present an atypical case of facial angiosarcoma arising as an asymmetric, non-purpuric swelling of the left nasal tip and ala, treated unconventionally with debulking surgery, radiation therapy, and off-label pembrolizumab immunotherapy. These treatment paradigms aligned with the patient’s goals as they navigated concerns of wide local excision due to cosmesis and quality of life. The patient is doing well four years later and will resume surgery or radiation if needed for any recurrence.

Case Presentation
A 79-year-old male with a history of squamous cell carcinoma presented to the dermatology department with a chief concern of "swelling" on the left side of the nose for the past 1-2 months. He reported his symptoms on the left side of the nose as "red", "swollen", "somewhat scaly", and "not itchy". The patient has a family history of nonmelanoma skin cancer. The dermatologic exam revealed an erythematous, ill-circumscribed nodule at the left nasal tip, eliminating the left alar groove, positive for