Epithelioid hemangioendothelioma after radiotherapy for congenital hemangioma: a case report

Abstract

A rare case of post-radiation epithelioid hemangioendothelioma arising in a congenital hemangioma of the lower leg 18 years after radiotherapy is reported. At 7 years of age, the patient underwent partial resection of a subcutaneous congenital hemangioma with intramuscular extension of the left lower leg, and at 10 years of age, the patient was given radiotherapy with a total dose of 30 Gy for recurrence. At 28 years of age, the patient noticed a large tumor mass with intractable pain at the antero-lateral aspect of the proximal lower leg, and she presented to our hospital. Since microscopic examination of a biopsy specimen showed epithelioid hemangioendothelioma, disarticulation at the knee joint was performed. Although malignant change in a benign vascular tumor is extremely rare, irradiation can induce malignant transformation of a hemangioma.

Case of epithelioid hemangioendothelioma occurring in the postradiation setting for breast cancer

“…16,17 It is important to note that neither of these EHEs were confirmed to be fusion positive by molecular testing. 16,17 Interestingly, signatures of ionizing radiation have been described in tumors with clear associations with radiotherapy, including increased small deletions (some of which affecting TP53 with hotspots at codons 135 and 237), increased balanced inversions (thought to result from chromosomal structural damage), and a gene expression signature suggestive of oxidative stress. [18][19][20][21] Review of the literature regarding EHE and associations with breast site and cancer revealed two cases of extra mammary EHE in patients with history of breast cancer and one report of EHE in the breast in an otherwise healthy 30-year-old patient, none of which were confirmed with molecular testing.…”

“…12,15 A review of the literature regarding EHE following radiotherapy revealed two reported cases: one in the treatment of congenital hemangioma and a second in the treatment of cervical cancer. 16,17 These presented as tumors of the extremity and terminal ileum at 18-and 22-year intervals, respectively. 16,17 It is important to note that neither of these EHEs were confirmed to be fusion positive by molecular testing.…”

“…16,17 These presented as tumors of the extremity and terminal ileum at 18-and 22-year intervals, respectively. 16,17 It is important to note that neither of these EHEs were confirmed to be fusion positive by molecular testing. 16,17 Interestingly, signatures of ionizing radiation have been described in tumors with clear associations with radiotherapy, including increased small deletions (some of which affecting TP53 with hotspots at codons 135 and 237), increased balanced inversions (thought to result from chromosomal structural damage), and a gene expression signature suggestive of oxidative stress.…”