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Infantile fibrosarcoma is a rare tumour in the paediatric age group. It occurs mainly in children below the age of 5 years. About 200 cases have been reported in the literature so far, very few of them in new-borns. We present here a case of infantile fibrosarcoma in a 27-day-old baby.
Infantile fibrosarcoma, an uncommon disease of childhood, generally occurs in an extremity and despite high local recurrence rates is usually curable with surgical excision alone. Two patients whose tumors were unresectable because of extensive retroperitoneal involvement are reported. Treatment with doxorubicin, vincristine, and cyclophosphamide resulted in complete clinical responses in both patients. They remain alive and tumor free 2.5 and 4 years from diagnosis. Chemotherapy as a single modality appears to be effective in controlling unresectable infantile fibrosarcoma.
Infantile fibrosarcoma (IF) has traditionally been treated with surgery, which may have considerable morbidity. Chemotherapy has been suggested in order to reduce the need for extensive surgery. Nine children with histologically confirmed IF who received chemotherapy are described. Six children were treated with chemotherapy initially, two following conservative surgery, and one following recurrence after surgery. All received vincristine (V) and actinomycin D (A), and six received additional drugs including ifosfamide (I), cyclophosphamide (C), adriamycin (Ad), etoposide (E), and cisplatinum (CDDP). Objective responses were achieved in eight: three responded completely (CR), two responded partially (PR), which allowed conservative surgery, one had stable disease, one had an initial PR, but subsequently had tumour recurrence 1 month after cessation of treatment, necessitating further surgery and chemotherapy, and one had an initial PR but died following local and regional metastases. One child had no response to chemotherapy but is alive with stable residual disease. Thus, five of nine children achieved a CR--three with chemotherapy alone. With the inclusion of chemotherapy as part of their treatment, five children, for whom curative surgery may have resulted in amputation, remain alive with limbs intact. Chemotherapy including V and A should be given to infants with fibrosarcoma in whom curative surgery would be mutilating.
Infantile fibrosarcoma (IFS) is a rare tumor most often affecting the extremities of infants and young children. Unlike its adult counterpart, IFS has a low potential for metastatic spread, and surgical extirpation alone has therefore resulted in an excellent prognosis. The amputation rate, however, exceeds 50%. The dramatic response in 2 recent cases to preoperative chemotherapy, given in an attempt to avoid amputation, prompted this report and a review of the literature.
Infantile fibrosarcoma is a rare soft tissue tumor, predominately affecting young infants. It grows rapidly and is locally infiltrative but rarely metastases. Complete surgical removal is usually curative but is impossible in some patients and would result in significant functional or cosmetic consequences in many others. Neoadjuvant chemotherapy will cause many tumors to shrink significantly, allowing less mutilating surgical resections to be performed--this is the current recommendation where immediate surgical removal cannot be accomplished without unacceptable morbidity. In contrast, there is no defined role for adjuvant chemotherapy or radiation following complete surgical resection. Although there is a significant risk of local recurrence, most of these can be successfully treated with further surgery and the overall survival rate exceeds 90%.
Infantile fibrosarcoma is a rare soft tissue malignant tumor, when it occurs, it is usually seen in the first year of life. The clinical course of infantile fibrosarcoma is more favorable and metastasis is rare compared with that in adulthood. While adult fibrosarcoma are common in the thigh, infantile fibrosarcoma affect chiefly the distal portions of the extremities. Standard treatment is primarily wide surgical excision. In this case report, we present our experience of an infantile fibrosarcoma of thigh with good clinical course 36 months after tumor resection and the usefulness of detecting the ETV6-NTRK3 gene fusion in differential diagnosis.
Infantile "fibrosarcoma" occurred in a newborn male infant and in a 2-month-old female infant. In both cases, the tumors grew rapidly and showed the histological features of malignancy. However, they were treated successfully with simple resections. Review of the literature shows that infantile fibrosarcoma has a relatively favorable prognosis. Simple local excision is the initial treatment of choice for lesions occurring in infants and children less than 5 years old.
Cytogenetic analysis of an infantile fibrosarcoma showed the presence of a t(12;13) and numerical changes of chromosomes 15 and 20. Until now only non-random gain or loss of total chromosomes as well as one case with a deletion at 17p have been reported for this kind of tumor. This report represents the first cytogenetic description of an aggressive infantile fibrosarcoma with a translocation.
Congenital-infantile, fibrosarcoma is a rare tumour presenting at birth or in the neonatal period. Few such tumours have been reported, and imaging details in particular are scant. The authors describe two neonates with congenital-infantile fibrosarcoma, the first case involving the right thigh and extending into the pelvis, and the second involving the calf and the ankle. In both cases magnetic resonance imaging (MRI) demonstrated well-demarcated, low-signal-intensity soft-tissue masses with t1-weighting and inhomogeneous, high-signal-intensity masses with T2-weighting. MRI was superior to other imaging modalities in the assessment of soft-tissue involvement and proved especially useful in planning therapy and monitoring chemotherapeutic response.
Congenital infantile fibrosarcoma, a rare malignant tumor of childhood, may present as a highly vascularized mass that is clinically difficult to distinguish from a hemangioma. When ulcerated, significant hemorrhage, anemia, and thrombocytopenia may occur in children with these lesions. This report describes three infants with ulcerated congenital infantile fibrosarcomas of the hand. As appropriate medical and surgical management hinges on timely and appropriate diagnosis, we review the clinical manifestations of these lesions.
Three patients with a predominantly fibrosarcomatous, desmoplastic, and fascicular spindle-cell sarcoma, resembling that of infantile fibrosarcoma but with ultrastructural and immunocytochemical evidence of rhabdomyoblastic differentiation, are described. The tumors were compared light and electron microscopically and immunocytochemically with six classical cases of infantile fibrosarcoma of the desmoplastic type. The three tumors occurred in two girls and one boy who were between 1 and 3 years of age. Ultrastructurally, the tumor cells primarily demonstrated fibroblastic and myofibroblastic features, but there were also tumor cells with sarcomere-like structures. The spindle-shaped, fibroblast-like tumor cells expressed vimentin, desmin, and smooth muscle-specific and sarcomere-specific actins. In one tumor peculiar intracytoplasmic inclusions were observed by light and electron microscopy. The immunocytochemical analysis indicated that these bodies contained all types of thin and intermediate filaments. In short-term cell cultures the tumor cells of two examined tumors revealed immunoreactivity for vimentin, smooth muscle-specific actin, and desmin. Cytogenetically, both tumors displayed a similar chromosomal pattern. One tumor contained two abnormal clones with monosomy 19 and monosomy 22, respectively, and the other tumor revealed a single clone with monosomy 19. In addition, both tumors contained clones with a normal karyotype. Two of the patients with metastatic spread died within 2 years of the primary operation. The third patient is alive with a local recurrence. The three tumors described here deviate from infantile fibrosarcoma in terms of their clinical, ultrastructural, immunocytochemical, and cytogenetic characteristics. The descriptive term "infantile rhabdomyofibrosarcoma" is suggested. The present investigation calls for detailed studies of tumors diagnosed as infantile fibrosarcomas that have metastasized and followed a fatal course.
Congenital infantile fibrosarcoma (CIFS) is a rare soft-tissue malignancy most commonly involving the extremities. Metastases are rare; however, local recurrence is common. Because the tumor is so rare and clinical experience is lacking, both diagnosis and treatment are difficult. The complex planning and implementation of neonatal and oncologic care require ongoing collaboration between both nursing specialties. This case study discusses the pathophysiology, diagnosis, and management of an infant with CIFS, with emphasis on the nursing care of the patient and family.
PURPOSE: To retrospectively evaluate the treatment and outcome of patients diagnosed with infantile fibrosarcoma at the Dana-Farber Cancer Institute and Children's Hospital, Boston. PATIENTS AND METHODS: Between 1982 and 1998, a total of 11 infants were diagnosed pathologically with infantile fibrosarcoma. A retrospective chart review was conducted to determine the extent of surgical therapy and chemotherapy required for a favorable clinical outcome. Cytogenetic findings were reviewed and archived tumor specimens were analyzed, when available, for the presence of the TEL/TRKC fusion gene. RESULTS: Three patients had primary surgical resection with negative pathologic margins and have been lost to follow-up. Two patients received chemotherapy only after limited biopsy or subtotal resection and are alive with no evidence of disease 8 and 18 years from diagnosis. Four patients had limited biopsies followed by chemotherapy with delayed resection. One of these four patients had negative margins and received no further chemotherapy. The other three of these patients had positive microscopic margins; two of them received postoperative chemotherapy while the third did not. All four are currently alive with no evidence of disease. Two patients had progressive disease within 7 and 10 months of diagnosis while on chemotherapy after subtotal resections. One of these two patients is dead of disease; the other is alive after palliative radiotherapy. Seven patients had archived or frozen tissue available for molecular analysis. All seven had evidence of TEL gene rearrangement; six exhibited the TEL/TRKC fusion. Six patients had characteristic trisomies previously reported to be associated with infantile fibrosarcoma. CONCLUSIONS: Previously reported series of treatment outcomes in infantile fibrosarcoma have been limited to very few patients due to the rare occurrence of this tumor. In our experience, initial chemotherapy combined with surgery has been successful for most cases. When disease progression occurred, it was within one year of diagnosis. There was no development of distant metastases in the patients with progressive disease. The role of additional chemotherapy for microscopic margins after local control is not clear. We found a high incidence of the TEL/TRKC fusion gene, confirming its utility in diagnosis. We propose a uniform approach to treatment to gather clinical and biologic information about this rare and curable disease.
Congenital infantile fibrosarcoma is a rare soft-tissue malignant tumor that usually presents as a mass involving the arm or leg. We report a case of congenital fibrosarcoma on the right hand of a newborn infant. The patient was a 12-day-old male. From the time of delivery, he had a red tumor on his dorsal right hand. The tumor was initially associated with bleeding within a hemangioma and compressed. The course was observed. It appeared that the tumor might be malignant because it increased in size to 10 x 8 x 3 cm, and ulceration and bleeding appeared on the surface after 10 days. A biopsy suggested a fibrosarcoma. The tumor was resected 32 days after birth at the narrow margin in consideration of preservation of function. However, a recurrence was observed under the graft after 2 weeks and another resection was performed. Because the tumor had passed through the interosseous muscle and invaded the palm of the hand, the middle finger, ring finger, and little finger had to be amputated. One year and 7 months postoperatively, no recurrence or metastasis has been observed.
Congenital-infantile fibrosarcoma (CIFS) is a cellular, mitotically active neoplasm with a paradoxically limited biologic potential in most cases. Its phenotype and proliferative features have been incompletely explored with inconclusive results. We studied the clinical, pathologic, immunohistochemical, and flow cytometric features of 26 cases (16 males, 10 females; 92% of cases detected within the first year life; 11 on extremities, 10 on the trunk, 5 in the head and neck). All displayed interlacing fascicles of spindle cells with focal necrosis, mitoses, and a focal hemangiopericytomatous vascular pattern. Immunohistochemically, 22 of 22 cases were reactive for vimentin. Other markers were present in a minority of cases. Flow cytometry of formalin-fixed, paraffin-embedded tissue in 10 cases demonstrated moderate to high proliferation activity and diploid DNA content in nine cases. Follow-up of all 26 patients revealed 20 patients alive and well, 15 without evidence of recurrence, and 5 with a small residual mass. Six patients had died of tumor, none had distant metastases. Patients with tumors in the head and neck or deep truncal soft tissues, including mesentery, had a poor prognosis because of local extension. CIFS is a clinically and morphologically homogeneous condition with considerable immunophenotypic diversity. Diploid DNA content in the majority of cases suggests that it may not be a fully expressed sarcoma. The clinicopathologic features are sufficiently distinctive to permit recognition and warrant conservative initial treatment in most cases. "Fibrosarcoma" is a term of convenience rather than of nosologic certainty.
BACKGROUND: Congenital-infantile fibrosarcoma is a rare tumor of the pediatric age. It involves subjects under 5 years of age, and more than 200 cases have been reported in the literature. METHODS: The authors present the clinicopathologic findings of 2 cases and review the literature. RESULTS: Of our 2 patients, the first was a 2-years and 6-months-old female and the second a newborn male. The female presented a tumor in the retroperitoneum without recurrences or metastasis after 17 months, and the male on left foor with a recurrence after 3 months. Histologically, the tumors were mainly composed of spindle-shaped cells. Immunohistochemically, in both cases, neoplastic cells were positive for vimentin; focal positivity for muscular specific actin was present in the tumor of the female. Ultrastructurally, tumors were composed of mesenchymal cells with fibroblastic and myofibroblastic features. Flow cytometric analysis of the retroperitoneal tumor showed an aneuploid population of neoplastic cells. CONCLUSIONS: Congenital-infantile fibrosarcoma should be considered a borderline tumor; its biologic behavior is better than that of adult fibrosarcoma. Histologic diagnosis is not easy; the microscopic picture may be confused with fibromatosis or with malignant mesenchymal neoplasms. Only a follow-up of many years can confirm the benignancy or malignancy of any individual tumor, even though clinico-pathologic features may be distinctive enough to permit its recognition.
We report 5 cases of infantile fibrosarcoma (4 boys and 1 girl) whose average age was 5, 7 months (range 0 days to 14 months). The tumor was congenital in 4 cases. All tumors presented in the extremities (forearm, hand, thigh: 1 case, lower leg: 2 cases). Treatment was based on surgery (3 cases: amputation, 2 cases: local excision) with a favorable course in all cases, even those with marginal excision (follow-up ranging from 5 to 21 years). The lesions were characterized by dense monotonous cells growing in a fascicular pattern, with small necrotic areas and scattered lymphocytes. The mitotic index was high (average 8/10 high-power fields). ETV6-NK3 chimeric RNA was detected by reverse transcriptase polymerase chain reaction in two cases out four cases from paraffin-embedded tissue blocks. The infantile fibrosarcoma is a good prognosis tumor characterized by particular histological features and ETV6-NK3 gene fusion.
BACKGROUND: Large congenital neoplasms of the extremities may be associated with coagulopathies and significant hemorrhage in the neonatal period. At times, the differences between coagulation derangements can be very subtle, leading to errors in diagnosis. Infants with vascular lesions and coagulopathies are often found to have the Kasabach-Merritt phenomenon, which is a platelet-trapping coagulopathy. However, other neoplasms or vascular malformations can be accompanied by disseminated intravascular coagulation. It is important to obtain accurate diagnoses of the neoplasm and the coagulopathy because the treatments of similar-appearing tumors and coagulopathies can be markedly different. METHODS: The authors report the case of a newborn with a congenital tumor of the left hand that was accompanied by a coagulopathy that caused significant bleeding. RESULTS: A presumption was made by the neonatal critical care physicians and hematologists that the infant had a kaposiform hemangioendothelioma along with the Kasabach-Merritt phenomenon. However, steroid treatment did not reduce the size of the mass or correct the coagulopathy. Only after obtaining consultation with a hand surgeon and a tissue diagnosis was it learned that the patient had an infantile fibrosarcoma that was accompanied by disseminated intravascular coagulation. Limb-sparing resection of the lesion along with chemotherapy markedly improved the patient's condition. CONCLUSIONS: Large congenital neoplasms presenting with attendant bleeding diatheses must be rapidly and accurately diagnosed with both a biopsy-proven tissue diagnosis and a hematologic characterization of the nature of the coagulopathy. The differential diagnosis of a vascular-appearing mass in the extremity can be subtle, and presumptive diagnosis, as occurred in this case, can lead to incorrect or delayed treatment. Specifically, kaposiform hemangioendothelioma must be differentiated from infantile fibrosarcoma. The principles of infantile fibrosarcoma treatment are limb-sparing resection and chemotherapy.