Clinically Suspected Eyelid Conjunctival Metastasis from Cutaneous Angiosarcoma Responding to Palliative Radiotherapy: A Case Report

Abstract

Introduction

Cutaneous angiosarcoma is a rare and highly aggressive vascular malignancy that commonly arises on the scalp and face of elderly individuals. It frequently metastasizes to visceral organs, whereas ocular involvement is exceedingly uncommon.

Case Presentation

We report a case of clinically suspected eyelid conjunctival metastasis from cutaneous angiosarcoma in a man in his 60s. The primary tumor involved the left scalp and cheek and was treated with intensity-modulated radiotherapy combined with weekly paclitaxel for 7 months. Although an initial response was observed, the disease progressed with distant metastases to the lungs, liver, and brain. During the terminal stage, a rapidly enlarging hemorrhagic lesion developed on the conjunctiva of the right eyelid, outside the previously irradiated field. Because of the patient’s poor general condition, histopathological confirmation was not feasible. Based on the clinical course and characteristic appearance, the lesion was clinically diagnosed as conjunctival metastasis. Short-course palliative radiotherapy achieved effective hemostasis and symptomatic relief.

Conclusion

This case highlights an unusual metastatic presentation of cutaneous angiosarcoma and supports the role of palliative radiotherapy in managing symptomatic ocular lesions.

Introduction

Cutaneous angiosarcoma is a rare and highly aggressive vascular malignancy that predominantly affects the scalp and face of elderly patients and is associated with a poor prognosis due to frequent recurrence and early hematogenous spread [1]. Multimodal treatment strategies have been developed, and taxane-based chemoradiotherapy has been shown to be superior to conventional surgery and radiotherapy alone in terms of disease control [2]. In addition, recent therapeutic advances, including immune checkpoint inhibitors, have expanded treatment options; however, outcomes in advanced disease remain unsatisfactory [3].

Case Report

A Japanese man in his 60s presented with a violaceous plaque and nodular lesions on the scalp and face (Fig. 1a, b). Histopathological examination of a skin biopsy revealed irregular, anastomosing vascular channels lined by atypical endothelial cells, confirming the diagnosis of cutaneous angiosarcoma (Fig. 2). The primary lesion involved the left scalp and cheek without extension to the contralateral eyelid. The patient was treated with intensity-modulated radiation therapy to the scalp and face (total dose, 70 Gy) combined with paclitaxel chemotherapy. Paclitaxel was administered weekly at 50–100 mg/m² for 6 consecutive weeks followed by a 2-week rest period, and this cycle was repeated over 7 months. An initial therapeutic response was observed; however, local recurrence developed on the left cheek 8 months after the initiation of treatment. Subsequently, distant metastases to the lungs, liver, and brain were detected. During the terminal stage of the disease, the patient developed a rapidly enlarging, hemorrhagic lesion involving the conjunctiva of the right eyelid (Fig. 1c, d). The lesion was friable and bled easily with minimal contact, causing marked discomfort. The palpebral lesion developed outside the previously irradiated field. Because of the patient’s poor general condition and disseminated disease, a biopsy of the conjunctival lesion was not performed. Based on the characteristic clinical appearance, rapid progression, and known history of metastatic angiosarcoma, the lesion was clinically diagnosed as eyelid conjunctival metastasis. Palliative external beam radiotherapy using a 4-MV photon beam at a total dose of 12 Gy was administered, which achieved effective hemostasis of the conjunctival lesion. Head computed tomography showed no evidence of intraocular invasion. The patient died 1 month after the appearance of the conjunctival metastasis.

Fig. 1.

Clinical findings. a, b The primary cutaneous angiosarcoma involved the left scalp and cheek without extension to the right periorbital region. b The biopsy site is indicated by an arrow. c, d Eight months after treatment initiation, local recurrence developed on the left cheek (c), followed by a rapidly enlarging hemorrhagic lesion of the right palpebral conjunctiva (d).

Fig. 2.

Histopathological findings of the primary cutaneous angiosarcoma. a Hematoxylin and eosin staining showed irregular, anastomosing vascular channels lined by atypical endothelial cells. Tumor cells were positive for CD31 (b) and D2-40 (c). The scale bars are 100 µm.

Discussion

Angiosarcoma typically metastasizes via the hematogenous route, most commonly to the lungs, liver, brain, and bones. Ocular involvement is extremely rare. Conjunctival metastases from internal malignancies, such as breast and lung cancer, have been described in small case series, highlighting the rarity of this presentation [4]. Furthermore, a retrospective study demonstrated that palliative radiotherapy is effective for symptom relief and local tumor control in patients with orbital and ocular metastases [5]. In the present case, short-course radiotherapy successfully controlled bleeding from the conjunctival lesion and contributed to symptomatic palliation during the terminal stage of disease. Although histopathological confirmation was not feasible because of the patient’s general condition, the lesion’s rapid progression, hemorrhagic nature, occurrence outside the irradiated field, and coexistence of systemic metastases strongly suggested metastatic angiosarcoma. To our knowledge, conjunctival metastasis from cutaneous angiosarcoma has not been previously well documented in the literature. This case highlights the potential for angiosarcoma to metastasize to unusual mucocutaneous sites and supports the role of palliative radiotherapy in managing symptomatic ocular metastases.

Statement of Ethics

Written informed consent was obtained from the patient’s next of kin for publication of the details of their medical case and any accompanying images. Ethical approval was not required for this single anonymized case report in accordance with local and national guidelines. The CARE Checklist for this case report is provided as online Supplementary Material (for all online suppl. material, see https://doi.org/10.1159/000553629).

Conflict of Interest Statement

The authors declare no conflicts of interest.

Funding Sources

No funding was received for this study.

Author Contributions

Teruki Yanagi: substantial contributions to the conception, review and editing, final approval of the version to be published, and agreement to be accountable for all aspects of the work. Riku Shirato: design of the work, drafting the work, review and editing, final approval of the version to be published, and agreement to be accountable for all aspects of the work. Mami Kudo and Wataru Yamagata: design of the work, review and editing, final approval of the version to be published, and agreement to be accountable for all aspects of the work.

Funding Statement

No funding was received for this study.

Data Availability Statement

All data generated or analyzed during this study are included in this article and its online supplementary material files. Further inquiries can be directed to the corresponding author.