Pediatr Blood Cancer 2010;54:618–620 BRIEF REPORT Right Foot Congenital Infantile Fibrosarcoma Treated Only With Chemotherapy Hacı Ahmet Demir, MD, MSc, 1 * Canan Akyu ¨z, MD, 1 Ali Varan, MD, 1 Fatma Bilge Ergen, MD, 2 and Mu ¨ nevver Bu ¨yu ¨ kpamukc ¸u, MD 1 INTRODUCTION Congenital infantile ﬁbrosarcoma (CIF) is a rare tumor in childhood, but is the most common soft tissue sarcoma in children <1 year of age. CIF is classiﬁed as a non-rhabdomyosarcoma soft tissue sarcoma [1]. It is mostly seen under the age of 5 years [2]. Conventional treatment of this tumor requires surgical resection, which frequently necessitates amputation. Neoadjuvant and/or adjuvant chemotherapy has been suggested for reducing surgery- related morbidity. We report a case of extremity CIF that was successfully treated using only chemotherapy, to emphasize importance of considering chemotherapy before surgery. Case Report A 9-day-old full-term male was diagnosed with a mass involving the right foot. The lesion was detected by prenatal ultrasonography at the 30th week of gestation. On clinical examination, he was 53 cm in length (in 75–100 percentiles), weighed 3,500 g (in 25–50 percentiles), and appeared active and healthy. Complete blood count, hepatic and renal function tests were normal. The baby had a large, ﬁrm vascular-appearing mass (10  6  4 cm 3 ) with small ulcerated areas on the surface (Fig. 1A), as well as a 4  1 cm 2 mass in the right inguinal region. The tips of the ﬁve toes were at the lower pole of the mass. Lateral radiograms showed a large, soft tissue mass in the right foot and the tarsal, metatarsal, and phalangeal bones were not visible. MRI revealed an 8  6 cm 2 soft tissue mass which demonstrated low signal intensity in T1 weighted images and heterogeneous enhancement after the administration of the contrast (Fig. 1B,C). The mass itself could not be differentiated from tarsal and metatarsal bones because of a lack of fatty signal within bones at the time of presentation. Doppler ultrasonography revealed a highly vascularized mass. Thoracic computed tomography was normal. Incisional biopsies of the mass and inguinal lymph node were performed. Biopsy specimens from both the mass and inguinal lymph node revealed a neoplastic process consisting of pleomorphic cells dispersed by collagen ﬁbers. Stains were positive for vimentin, SMA, and MyoD1, and negative for S-100, CD34, and desmin. The proliferation index of the tumor was estimated as 40% on staining for Ki-67 antigen. A diagnosis of CIF was made. The patient was put on a course of VAC chemotherapy with vincristine, 0.06 mg/kg/day, maximum dose, 2 mg/day, on day 1; actinomycin D, 10 mcg/kg/day, 5 days and cyclophosphamide, 5 mg/kg/day, 5 days, with intervals of 4 weeks. After the second cycle of chemotherapy, veno-occlusive disease (VOD)- like syndrome developed due to actinomycin D. The dose of actinomycin D was then decreased to 50% of the initial dose. After the third cycle of chemotherapy, VOD reoccurred, and cyclo- phosphamide and actinomycin D were given for 3 days instead of 5 days. At the end of the ninth cycle of chemotherapy, the inguinal mass had disappeared and a signiﬁcant shrinkage of the primary mass (Fig. 2A) was detected. The tarsal, metatarsal, and phalangeal bones were became visible on X-rays (Fig. 2B). Follow-up MRI demonstrated about 95% regression of the mass (Fig. 2C,D). A biopsy specimen revealed no viable tumor cells. At the end of a follow-up period of 32 months, the patient was disease-free and could walk normally. DISCUSSION CIF is rare and comprises about 13% of ﬁbroblastic– myoﬁbro- blastic tumors in children and adolescents, and 12% of soft tissue cancers in children younger than 1 year [3]. Approximately 50% of these tumors are present at birth, the remainder developing during the ﬁrst year of life [4]. A male predilection is noted in most series [4], but a study by Cecchetto et al. [5] reported that 15/25 cases were female. The extremities are affected in 70% of cases, followed by the trunk, the head, and neck regions [6 – 8]. In addition to these regions, CIF can also originate in the retroperitoneum, colon, abdomen, pelvis, pericardium, or thoracic wall [5,9–13]. The clinical course of CIF in children is variable and differs from that of ﬁbrosarcoma in adulthood [14]. Distant metastases are reported in around 8% of pediatric cases [8]. One of the features of the presented case was the destruction of all tarsal, metatarsal, and phalangeal bones; and that the foot would have appeared like a mass-ball had we not discriminated the tips of the toes at the lower part of the mass. The other noteworthy feature of the presented case was the presence of metastasis in the inguinal lymph nodes. The lungs are the most frequent site for metastasis [6,15]. Other metastatic sites included Congenital infantile ﬁbrosarcoma (CIF) is a rare tumor in childhood. The 5-year survival rate for CIFs is high and has been reported between 84% and 93%, but limb-amputation/ disarticulation is still a major problem. We report the case of a male newborn with a mass in his right foot. X-ray and MRI revealed a mass destroying all tarsal, metatarsal, and phalangeal bones. The patient was treated only with VAC chemotherapy and is able to walk normally. Pediatr Blood Cancer 2010;54:618– 620. ß 2009 Wiley-Liss, Inc. Key words: chemotherapy; children; congenital infantile ﬁbrosarcoma; foot; surgery ß 2009 Wiley-Liss, Inc. DOI 10.1002/pbc.22389 Published online 8 December 2009 in Wiley InterScience (www.interscience.wiley.com) —————— 1 Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey; 2 Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey Conﬂict of Interest: Nothing to declare. *Correspondence to: Hacı Ahmet Demir, Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, 06100 Ankara, Turkey. E-mail: hdemir@hacettepe.edu.tr Received 24 May 2009; Accepted 29 October 2009