Hemangiopericytoma in pediatric ages

Abstract

BACKGROUND Hemangiopericytoma (HPC) is very uncommon in childhood and comprises two different clinical entities, the adult type and the infantile type, occurring in the first year of age. We report on a series of 27 pediatric patients treated from 1978 to 1999 by the Italian and German Soft Tissue Sarcoma Cooperative Group. METHODS Seven patients had infantile HPC; complete resection was achieved in the tumors of five patients and chemotherapy was given to four patients. Twenty children had adult type HPC; nine received complete tumor resection (four patients at diagnosis and five at delayed surgery). Post‐operative radiotherapy was administered to 15 patients, chemotherapy to 19. RESULTS Six of seven patients with infantile HPC were alive in first remission; one patient died of disease. Chemotherapy achieved an objective response in four of four patients. Among the adult type HPC cases, 5‐year event free survival was 64% (median follow‐up 125 months); 12 patients were alive in first remission, eight patients relapsed and died of disease. Seven of 10 evaluable patients showed good response to chemotherapy. Statistically significant differences in outcome were observed in relation to Intergroup Rhabdomyosarcoma Study grouping, size, local invasiveness, and gender. CONCLUSIONS Infantile HPC is a unique entity probably related to infantile myofibroblastic lesions and characterized by a high response to chemotherapy, which is required in case of unresectable, life‐threatening tumors. In children over 1 year of age, HPC behaves like its adult counterpart; complete surgical resection remains the mainstay of treatment, but chemotherapy and radiotherapy seem effective and are recommended in all patients with incomplete tumor resection and/or locally invasive, large tumors. Cancer 2001;92:2692–8. © 2001 American Cancer Society.

Preoperative Chemotherapy to Salvage the Hand in a Case of Giant Infantile Hemangiopericytoma

“…6,7 I-HPC is found most often in the head and neck and extremities, 9 although tumors may arise anywhere throughout the body. 4,8,18 Although there are a few descriptions of arm or forearm tumors in children, there have been only 2 previously documented cases of hand I-HPC in the past 50 years. 6,10 I-HPC overall has an extremely low recurrence rate and very low metastatic potential.…”

“…16,19,20 However, I-HPC may certainly be malignant and even fatal, with an 80% 5-year survival in one case series. 8,18 Although larger size at diagnosis and increased rate of growth may be indicators of a more aggressive tumor, it is difficult to predict malignant potential. 9,19,21 Overall, patients with I-HPC appear to have a good long-term prognosis with com- plete surgical excision with or without adjuvant chemotherapy.…”

“…9,19,21 Overall, patients with I-HPC appear to have a good long-term prognosis with com- plete surgical excision with or without adjuvant chemotherapy. 1,7,8 Surgical excision is the treatment of choice for either A-HPC or I-HPC. 18 Given the rarity of this tumor, there is no standard margin for resection, although microscopically or macroscopically clear margins have been used interchangeably with "complete" resection in the literature.…”

“…18 Given the rarity of this tumor, there is no standard margin for resection, although microscopically or macroscopically clear margins have been used interchangeably with "complete" resection in the literature. [7][8][9] In both adults and children, HPC is a vascular tumor, and preoperative embolization has been advocated as a means of reducing intraoperative hemorrhage. 14 A-HPC is poorly chemoresponsive, and radiotherapy has been used to prevent local recurrence or as adjuvant therapy after incomplete resection.…”

“…I-HPC, accounting for only 5% to 10% of cases, occurs most often in patients less than one year old. 1,6 -8 These tumors have been described in the head and neck, 8,9 trunk and extremities, 1,7,8 lung, 7 and liver, 8 with 4 previous case reports of HPC limited to the hand. 6,10 -12 Preoperative chemotherapy has been used to permit excision of otherwise unresectable HPC in other anatomic locations 7-9 ; we present a case of a giant I-HPC of the hand and use of chemotherapy to shrink the tumor preoperatively to salvage the hand.…”