Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. He was treated with chemotherapy and extremity-sparing surgery. Amputation was avoided.
Fibrosarcoma is a mesenchymal tumor of adult and pediatric age groups, representing 10 to 12% of all soft-tissue sarcomas. It represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. In more than one-third of patients, it is present at birth and a second peak of incidence occurs between 10 and 15 years of life.1 Owing to its different behavior, fibrosarcoma during the first year of life must be considered a separate entity from the identical lesions seen in adults. The tumor may be observed at birth, in infancy or in childhood, and has been called congenital or infantile fibrosarcoma. It is locally aggressive, with a rare metastatic spread and a good rate of survival.2
Hemangiopericytoma is also very rare in childhood and comprises two different clinical entities: the adult type and the infantile type occurring in the first year of age. The infantile type is locally invasive with rare metastasis, and prognosis is better than that of the adult type.3
A full-term male newborn was presented to our pediatric oncology department with a mass on the right foot. On physical examination, he was a normal healthy baby, 50 cm in length, 2.8 kg in weight and a mass around the right foot 8 × 8 cm in size with a necrotic tissue that was hard and immobile. The overlying skin was purple in color. The mobility of fingers was normal. The hematological and biochemical investigations, chest X-ray, and abdominal and cranial sonogram were normal. Hypercalcemia (12 mg per 100 ml) with normal phosphate, alkaline phosphatase, parathyroid hormone, vitamin D and protein/albumin level was 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 of hemangioma, a high dose of methyl prednisolone (30 mg kg−1) was started, and after 10 days, dosage was decreased gradually. Calcitonin was started because of hypercalcemia. With this treatment, the mass softened and the color lightened but the size of the lesion was stable. Tumor excision was impossible because of the involvement of the main arteries of the foot, and incisional biopsy was performed. It was reported as infantile hemangiopericytoma. Interferon α-2a at a dose of 3 million unit per m2 body surface area, subcutaneously thrice a week, was started because of its antiangiogenic activity. After biopsy, the size of the mass increased rapidly because of hematoma, which was also shown by control magnetic resonance imaging (Figure 3). Interferon α-2a treatment did not cause any decrease in size, hence chemotherapy (vincristine 0.06 mg kg−1, actinomycin-D 10 gamma kg−1, cyclophosphamide 5 mg kg−1) was started. At the 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 coagulation tests, hepatosplenomegaly and ascites. With intensive supportive treatment, the clinical findings of the patient were resolved. He was operated upon and the tumor was excised gross totally without amputation, and was revealed as infantile fibrosarcoma by histopathological examination. Postoperative magnetic resonance imaging revealed a residual mass on the heel. The third cycle of the same chemotherapy protocol was administered and again it caused a venoocclusive disease. The chemotherapy protocol was changed to vincristine 0.06 mg kg−1, ifosfamide 60 mg kg−1 and adriamycin 0.7 mg kg−1. 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.
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 has to be excised.5 In larger series of patients with infantile fibrosarcoma, the primary and secondary amputation rate is approximately 50%. In irresectable tumors, in relapses and after incomplete surgery, chemotherapy, and in older children, radiotherapy is an adjuvant therapeutic tool. With preoperative chemotherapy, first successes appeared in the 1980s. Kurkchubasche et al. reviewed the literature and reported the patients receiving chemotherapy by adding their two cases. In 50% of patients, chemotherapy influenced the subsequent surgery. In only one patient was amputation performed because of recurrence. The most commonly used chemotherapy regimen consisted of vincristine, actinomycin-D and cyclophosphamide (VAC). Rarely were ifosfamide and adriamycin used. The most common short-term side effect of this regimen was myelosuppression.6
Hemangiopericytoma is a soft-tissue neoplasm most commonly seen in adults, with 5 to 10% of cases occurring in children. In children older than 1 year, it behaves in a manner similar to adult hemangiopericytoma,7 and 30 to 50% of it occurs in the extremities.8 Infantile hemangiopericytoma is histologically identical to adult type but has a more benign clinical course. Spontaneous regression is also reported.9 It was first described by Stout and Murray 50 years ago but still has uncertainty and disagreement among pathologists. Hemangiopericytomatous growth pattern, defined as a monomorphic population of compact polygonal or fusiform cells, and branching stromal vascular pattern with a ‘staghorn’ configuration may be observed in ‘true’ hemangiopericytoma, synovial sarcoma, mesenchymal chondrosarcoma, infantile fibrosarcoma, malignant fibrous histiosarcoma, malignant peripheral nerve sheath tumor, leiomyosarcoma, endometrial stromal sarcoma, solitary fibrous tumor, myofibroma, malign mesothelioma, tymoma, sarcomatoid carcinoma and malignant melanoma. It is thought that 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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Akyüz, C., Sari, N., Vargel, İ. et al. A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery. J Perinatol 30, 63–65 (2010). https://doi.org/10.1038/jp.2009.92
- infantile fibrosarcoma