Unresectable Retiform Hemangioendothelioma Treated with External Beam Radiation Therapy and Chemotherapy: A Case Report and Review of the Literature

Abstract

Retiform hemangioendothelioma (RH) is an infrequently encountered vascular neoplasm of intermediate or borderline malignancy. Treatment of RH is controversial. We present a case of a 44-year-old Asian male presenting with an unresectable RH of the pelvis. The patient was treated with concurrent low-dose Cisplatin and External beam Radiation (4140cGy in 180cGy per fraction). This is the first report of a clinical complete response and a long-term local control of this rare tumor. This has significant clinical implication, since it gives the first evidence of treatment of this rare tumor using concurrent low-dose chemotherapy and radiation.

Unusual multiple cutaneous retiform hemangioendothelioma on forearm and neck misdiagnosed as angiosarcoma with metastasis

“…Due to its high incidence of local recurrence, the treatment of choice for RH is a wide surgical excision with histopathological tumor-free margins and long-term follow-up is essential [1,11,[13][14][15]. Radiotherapy has been reported to be successfully administered as adjuvant treatment for local as well as regional nodal RH recurrences [16,19]. Recently, low-dose cisplatin and moderate radiotherapy has also been performed on a patient with unresectable RH [16].…”

“…Radiotherapy has been reported to be successfully administered as adjuvant treatment for local as well as regional nodal RH recurrences [16,19]. Recently, low-dose cisplatin and moderate radiotherapy has also been performed on a patient with unresectable RH [16]. But the efficacy of chemotherapy on recurred RH or RH with lymph node metastasis is still unclear.…”

“…RH does not appear to have any particular predilection site, but most of the reports were in the distal extremities, particularly the lower limbs. Nevertheless, the scalp, skull, and penis are involved in some cases [2,10,14,16]. RH with multiple lesions has seldom been described.…”

Retiform hemangioendothelioma in the infratemporal fossa and buccal area: a case report and literature review

“…To date, 29 papers and 48 RH cases have been reported, including this case. (Table 1)12345678910111213141516171819202122232425262728 There has been a female predominance (female:male, 32:16), and the patients were predominantly young and middle-aged adults in 2005. However, in this review, patients under 20 years represented nearly 30% of the sample, and patients over 50 years accounted for nearly 20%.…”

“…Five were successfully treated, and one patient died but the diagnosis was considered classic angiosarcoma9. In addition, Hirsh et al10 first reported successful treatment of RH with low-dose cisplatin and moderate radiotherapy without surgical resection. Use of surgical treatment and adjuvant therapies should be carefully considered to avoid unnecessary interventions and potential severe complications.…”