J Korean Assoc Pediatr Surg.  2017 Dec;23(2):19-23. 10.13029/jkaps.2017.23.2.19.

Soft Tissue Hemangioendothelioma in Children

• Youn JK
• Jung DH
• Kim SH
• Jung SE
• Park KW
• Kim HY

• KMID: 2398571
• DOI: http://doi.org/10.13029/jkaps.2017.23.2.19

Abstract

PURPOSE
Soft tissue hemangioendothelioma (STHE) is a rare vascular tumor, which has a similar prognosis to borderline malignancy. The disease is poorly understood in pediatric cases because of its low incidence; therefore, we investigated treatment strategies for STHE in children.
METHODS
We retrospectively analyzed 8 patients with STHE, who were pathologically confirmed between January 1995 and June 2015. The median duration of follow-up was 72 months.
RESULTS
Five were male and the median age at the time of surgery was 1.2 years. Six patients presented with a palpable mass, and 2 patients experienced facial paralysis. The median tumor size was 4.0 cm. The following tumor locations were observed head (2 patients), neck (2 patients), chest wall (1 patient), sacrococcyx (1 patient), upper limb (1 patient), and lower limb (1 patient). The patients underwent either microscopic complete resection (R0) (3 patients), macroscopic complete resection (R1) (2 patients), or macroscopic incomplete resection (R2) (3 patients). After histopathological examination, 6 patients were diagnosed with kaposiform hemangioendothelioma (HE), one with retiform HE, and one with epithelioid HE. Postoperative sequelae occurred as gait disturbance, hearing impairment, and vocal cord palsy. Tumor recurrence or regrowth occurred in 4 patients. These patients underwent reoperation and IFN therapy; however, in the patient with epithelioid HE, metastasis to the scalp occurred after these therapies. The patient with the head tumor who underwent R2 resection, underwent resection three more times, but died 11 months after the first surgery.
CONCLUSION
When treating STHE in children, R0 resection should be first considered, but recurrence and metastasis should be monitored depending on the size, pathology, and location of the lesion. When major sequelae are expected, function-preserving surgery could be considered, depending on tumor location, size, and nearby organs.

Keyword

Hemangioendothelioma; Soft tissue neoplasms

MeSH Terms

Child*
Facial Paralysis
Follow-Up Studies
Gait
Head
Hearing Loss
Hemangioendothelioma*
Humans
Incidence
Lower Extremity
Male
Neck
Neoplasm Metastasis
Pathology
Prognosis
Recurrence
Reoperation
Retrospective Studies
Scalp
Soft Tissue Neoplasms
Thoracic Wall
Upper Extremity
Vocal Cord Paralysis