If you don't remember your password, you can reset it by entering your email address and clicking the Reset Password button. You will then receive an email that contains a secure link for resetting your password
If the address matches a valid account an email will be sent to __email__ with instructions for resetting your password
To the Editor: Retiform hemangioendothelioma is a low grade angiosarcoma of unknown etiology that was first described in 1994. A Medline search using the keyword “retiform hemangioendothelioma” yielded 15 reports totaling 33 patients, none of whom were treated with Mohs micrographic surgery (MMS).
We report a case of retiform hemangioendothelioma treated with MMS.
An 11-year-old otherwise healthy girl was evaluated by our dermatologic surgeon for a rapidly enlarging and intermittently painful soft tissue swelling of her left distal fourth finger. The swelling initially developed after trauma 4 months before presentation. Two previous resections had been performed; both had positive margins. Two months after the second resection, she reported recurrent soft tissue swelling of her finger. The physical examination revealed a nontender enlarged distal finger pad with associated hyperhidrosis (Fig 1, A). Range of motion was intact. Histopathology reports from her previous excisions described an unusual vascular proliferation consistent with a low grade angiosarcoma. Radiographic and magnetic resonance imaging (MRI) studies revealed a 7 mm × 5 mm area of soft tissue thickening without involvement of the distal phalanx. Upon review of her clinical course and previous histopathology, a diagnosis of retiform hemangioendothelioma was made.
In an attempt to avoid amputation of the finger because of persistently positive and poorly defined tissue margins, MMS was performed under general anesthesia. After two Mohs' layers, the peripheral margins were clear. The deep margin, abutting bone, remained positive (Fig 2, A). Partial amputation of the distal phalanx just proximal to the last Mohs' layer was performed by a hand surgeon using a bone shear followed by flap repair (Fig 1, B). Histopathology revealed superficial erosion of the bony phalanx by tumor (Fig 2, B). Five months postoperatively, there was no evidence of clinical recurrence.
Retiform hemangioendothelioma is a locally aggressive, frequently asymptomatic vascular tumor. Hyperhidrosis is a previously unreported symptom. The majority of tumors are limited to the dermis and subcutaneous tissue with rare reports of bony infiltration and two reports of regional lymph node metastasis.
No cases of distant metastasis, transformation to an aggressive vascular tumor, or tumor-related death have been observed.
Surgery with clear margins has been considered the treatment of choice. Unfortunately, the infiltrative growth pattern of retiform hemangioendotheliomas can lead to difficulty in achieving tumor-free margins. In addition, the failure of routine frozen sectioning to visualize the entire surgical margin may result in false reassurance and insufficient resection. Both of these factors likely contribute to high recurrence rates. The ability to visualize the complete surgical margin at the time of the procedure makes MMS an ideal treatment for retiform hemangioendothelioma. While osseous invasion necessitated a partial amputation in this case, the use of MMS spared amputation at the distal interphalangeal joint and resulted in preservation of cosmesis and full function of the finger.
Retiform hemangioendothelioma: a case report and review of the literature.