Haemangioendothelioma in an Infant’s Wrist

Abstract

A rare case of an epitheloid haemangioendothelioma presenting as an aggressive soft tissue mass in the wrist of an infant is presented. The management of the lesion presents particular difficulties because of its diffuse involvement of surrounding muscles, tendons and neurovascular bundle and the importance of preserving hand function. The epitheloid haemangioendothelioma rarely occurs extravascularly with less than 15 such cases reported in the literature.

Epitheloid hemangioendothelioma of the subcutaneous tissues of a finger

“…EHE of the soft tissues of the hand usually present as a slowly growing infiltrative mass (similar to our case)567 or it may have a scar like appearance within the nail bed 4. Most hand surgeons have treated these soft tissue tumors with excision preserving the adherent vital structures 567. No postoperative radiotherapy was used and all patients did not have local recurrence or metastasis 567.…”

“…Most hand surgeons have treated these soft tissue tumors with excision preserving the adherent vital structures 567. No postoperative radiotherapy was used and all patients did not have local recurrence or metastasis 567. Our case is an important addition to the existing body of literature because we document mitotic figures/pleomorphism and positive resection margins and yet no local recurrence or metastases were noted.…”

“…Intravascular EHE of the hand are very rare and are treated by excision of the involved segment of the vessel and revascularization of the involved digits (if required) 9. EHE of the soft tissues of the hand usually present as a slowly growing infiltrative mass (similar to our case)567 or it may have a scar like appearance within the nail bed 4. Most hand surgeons have treated these soft tissue tumors with excision preserving the adherent vital structures 567.…”

“…Outside the hand, EHE are known to have a local recurrence rate of 13%, a metastasis rate of 31% and a mortality rate of 13% 11. A review of the literature of EHE of the hand345678910 showed the rare occurrence of local recurrence8 and zero rates of metastasis and mortality. It is well known that the malignant potential of visceral EHE is higher than EHE of the lower limbs 11.…”

“…The behavior of EHE is intermediate between the other two types and there is no consensus about the management of these tumors in hand. In hand, management varied from amputation34 to en-block or wide excision 5678. Some authors have also used adjuvant radiotherapy following excision of EHE of the hand 9.…”