Five cases of pediatric desmoid tumor: A case series

Arakawa Ayumu, Kawai Akira, Kobayashi Eisuke, Iwata Shintaro, Kawakubo Naonori, Kumamoto Tadashi, Ogawa Chitose

doi:10.11412/jspho.57.366

Introduction: Desmoid tumor (DT) is a very rare mesenchymal tumor and has local aggressive behavior with no distant disease. Although the wait-and-see strategy and tumor resection are first-line therapies, complete surgery is often challenging because of the invasion of the muscle and the fascia and a high tendency of local recurrence. To avoid cosmetic or functional sequelae, chemotherapy is selected as a treatment option.Method: Five cases of pediatric desmoid tumors treated with chemotherapy at the National Cancer Center Hospital were analyzed retrospectively.Result: The median patient age at diagnosis was 12.0 y (7.3–14.6 y), and the tumor involved the upper limb in three patients, the lower limb in one patient, and an intra-abdominal site in one patient. Methotrexate (MTX)+vinblastine (VBL) was given to four patients, pazopanib to three patient, and doxorubicin to one patient. One patient achieved partial response (PR), and in one patient, the disease was maintained stable over one year with MTX+VBL. A 14-year-old-girl with femoral DT was successfully treated with pazopanib for six months; after achieving good PR, tumor resection was performed, and her contracture improved considerably after treatment. A 12-year-old boy with a family history of Gardner syndrome had multiple intra-abdominal DTs, and his DT was resistant to MTX+VBL or pazopanib; he experienced repeated abscess formation or intra-abdominal rupture because of rapid tumor growth. He achieved PR with doxorubicin monotherapy.Discussion: In our experience, disease control was generally possible with chemotherapy in pediatric cases of DT. DT with Gardner syndrome showed resistance to chemotherapy, similar to previous reports.

Five cases of pediatric desmoid tumor: A case series

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