A rare angiosarcoma: retiform haemangioendothelioma.

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Retiform haemangioendothelioma is a rare, low-grade angiosarcoma.
• First described in 1994, it presents as a vascular neoplasm with challenging histopathological diagnosis.
• It has a high rate of local recurrence but a low rate of metastasis.

Observation:

• An 18-year-old male presented with a recurrent, ulcerating lesion on his left ear (pinna).
• This case is the third reported instance of lymph node metastasis in retiform haemangioendothelioma.
• Thirty-one cases have been documented to date.

Findings:

• Histopathological diagnosis can be difficult, appearing intermediate between haemangioma and angiosarcoma.
• This patient's presentation included lymph node metastasis, a rare occurrence for this tumor.
• The tumor's appearance was consistent with retiform haemangioendothelioma.

Implications:

• Surgical excision is the primary treatment for retiform haemangioendothelioma.
• Adjuvant radiotherapy is recommended for cases with large tumor size, local recurrence, and lymph node metastasis.
• This case underscores the need for vigilance in diagnosing and managing this rare vascular tumor.