A Newborn with Infantile Fibrosarcoma of Foot

Journal of Perinatology (2010) 30,63–65 r 2010 Nature Publishing Group All rights reserved. 0743-8346/10 $32 www.nature.com/jp PERINATAL/NEONATAL CASE PRESENTATION A newborn with infantile ﬁbrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery

C Akyu¨z1, N Sari2,I˙ Vargel3, G Gedikoglu4, M Haliloglu5 and M Bu¨yu¨kpamukc¸u1 1Department of Pediatric Oncology, Institute of Oncology, Hacettepe University, Ankara, Turkey; 2Department of Pediatric Oncology, Ankara Oncology Research and Education Hospital, Ankara, Turkey; 3Department of Plastic and Reconstructive Surgery, Faculty of Medicine, Kirikkale University, Kirikkale, Turkey; 4Department of Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey and 5Department of Radiology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

examination, he was a normal healthy baby, 50 cm in length, Infantile fibrosarcoma represents less than 1% of all childhood cancers, but 2.8 kg in weight and a mass around the right foot 8 Â 8 cm in size it is the most common soft-tissue sarcoma in those under 1 year of age. We with a necrotic tissue that was hard and immobile. The overlying report an infant with congenital infantile fibrosarcoma diagnosed as skin was purple in color. The mobility of fingers was normal. The hemangiopericytoma. He was treated with chemotherapy and extremity- hematological and biochemical investigations, chest X-ray, and sparing surgery. Amputation was avoided. abdominal and cranial sonogram were normal. Hypercalcemia Journal of Perinatology (2010) 30, 63–65; doi:10.1038/jp.2009.92 (12 mg per 100 ml) with normal phosphate, alkaline phosphatase, Keywords: infantile fibrosarcoma; hemangiopericytoma; neonate; parathyroid hormone, vitamin D and protein/albumin level was treatment present. Magnetic resonance imaging and conventional angiography revealed the presence of a homogenous mass that represents hemangioma supplied by anterior and posterior tibial arteries and peroneal artery (Figures 1 and 2). With the diagnosis Introduction of hemangioma, a high dose of methyl prednisolone (30 mg kgÀ1) Fibrosarcoma is a mesenchymal tumor of adult and pediatric age was started, and after 10 days, dosage was decreased gradually. groups, representing 10 to 12% of all soft-tissue sarcomas. It represents Calcitonin was started because of hypercalcemia. With this less than 1% of all childhood cancers, but it is the most common treatment, the mass softened and the color lightened but the size of soft-tissue sarcoma in those under 1 year of age. In more than the lesion was stable. Tumor excision was impossible because of one-thirdofpatients,itispresentatbirthandasecondpeakof the involvement of the main arteries of the foot, and incisional incidence occurs between 10 and 15 years of life.1 Owing to its different biopsy was performed. It was reported as infantile behavior, fibrosarcoma during the first year of life must be considered hemangiopericytoma. Interferon a-2a at a dose of 3 million unit a separate entity from the identical lesions seen in adults. The tumor per m2 body surface area, subcutaneously thrice a week, was started may be observed at birth, in infancy or in childhood, and has been because of its antiangiogenic activity. After biopsy, the size of the called congenital or infantile fibrosarcoma. It is locally aggressive, with mass increased rapidly because of hematoma, which was also araremetastaticspreadandagoodrateofsurvival.2 shown by control magnetic resonance imaging (Figure 3). Hemangiopericytoma is also very rare in childhood and comprises Interferon a-2a treatment did not cause any decrease in size, two different clinical entities: the adult type and the infantile type hence chemotherapy (vincristine 0.06 mg kgÀ1, actinomycin-D occurring in the first year of age. The infantile type is locally invasive 10 gamma kgÀ1, cyclophosphamide 5 mg kgÀ1) was started. At the with rare metastasis, and prognosis is better than that of the adult type.3 end of two cycles of chemotherapy, the tumor had decreased in size, the level of calcium decreased but venoocclusive disease was diagnosed with pancytopenia, abnormal liver enzymes and Case coagulation tests, hepatosplenomegaly and ascites. With intensive A full-term male newborn was presented to our pediatric oncology supportive treatment, the clinical findings of the patient were department with a mass on the right foot. On physical resolved. He was operated upon and the tumor was excised gross totally without amputation, and was revealed as infantile Correspondence: Dr N Sari, Pediatric Oncologist, Department of Pediatric Oncology, Ankara fibrosarcoma by histopathological examination. Postoperative Oncology Research and Education Hospital, 06520 Ankara, Turkey. E-mail: [email protected] magnetic resonance imaging revealed a residual mass on the heel. Received 4 March 2009; accepted 27 April 2009 The third cycle of the same chemotherapy protocol was Congenital infantile fibrosarcoma C Akyu¨z et al 64

Figure 1 (a) T1-weighted sagittal image shows soft tissue mass plantar and dorsal side of the right foot. (b) Contrast-enhanced T1-weighted sagittal image shows a homogenous enhancement of the mass. administered and again it caused a venoocclusive disease. The chemotherapy protocol was changed to vincristine 0.06 mg kgÀ1, ifosfamide 60 mg kgÀ1 and adriamycin 0.7 mg kgÀ1. With this treatment, the residual mass disappeared and no major complication was observed. At the end of the ﬁrst year of diagnosis, treatment was stopped while patient was in complete remission. This patient is still alive after 7 years of diagnosis with normal appearance and a functioning foot.

Discussion Infantile fibrosarcoma is histologically similar to those seen in adults. Its clinical course is more favorable with a rare metastatic spread.2 The local recurrence rate is high, up to 43%, and recurrence may occur as late as 15 to 31 years after the initial operation.4 Wide local excision is the primary form of treatment, but in some cases, as in our case, it is impossible to remove lesion because of the anatomic extent of these tumors without disrupting peripheral vessels and nerves. Sometimes, the adjacent bone also Figure 2 Angiography (early arterial phase) shows vascularized mass supplied has to be excised.5 In larger series of patients with infantile by anterior tibial artery, posterior tibial artery and peroneal artery. fibrosarcoma, the primary and secondary amputation rate is approximately 50%. In irresectable tumors, in relapses and after incomplete surgery, chemotherapy, and in older children, The most commonly used chemotherapy regimen consisted of radiotherapy is an adjuvant therapeutic tool. With preoperative vincristine, actinomycin-D and cyclophosphamide (VAC). Rarely chemotherapy, first successes appeared in the 1980s. were ifosfamide and adriamycin used. The most common short- Kurkchubasche et al. reviewed the literature and reported the term side effect of this regimen was myelosuppression.6 patients receiving chemotherapy by adding their two cases. In 50% Hemangiopericytoma is a soft-tissue neoplasm most commonly of patients, chemotherapy influenced the subsequent surgery. In seen in adults, with 5 to 10% of cases occurring in children. In only one patient was amputation performed because of recurrence. children older than 1 year, it behaves in a manner similar to adult

Journal of Perinatology Congenital infantile ﬁbrosarcoma C Akyu¨z et al 65

hemangiopericytoma is both a pathological entity and a morphological pattern.10 In summary, our case was treated successfully with chemotherapy and extremity-sparing surgery. Amputation and radiotherapy were avoided. On performing incisional biopsy, the lesion was first diagnosed as hemangiopericytoma because of its morphological pattern. The specimen may be insufficient to conclude a diagnosis of infantile fibrosarcoma. In newborns with infantile fibrosarcoma, neoadjuvant chemotherapy should be tried before extensive surgery or amputation.

Conﬂict of interest The authors declare no conﬂict of interest.

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