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.
AllenP.W.: The fibromatosis: a clinicopathologic classification based on 140 cases. Part 2. Am.J. Surg. Pathol., 1: 305–321, 1977.
2.
ArgyleJ.C., TomlinsonG.E., StewartD., SchneiderN.R.: Ultrastructural, immunocytochemical, and cytogenetic characterization of a large congenital fibrosarcoma.Arch. Pathol. Lab. Med., 116: 972–975, 1992.
BrodersA.C., HargraveR., MeyerdingH.W.: Pathological features of soft tissue fibrosarcoma with special reference to the grading of its malignancy.Surg. Gynecol. Obstet., 69: 267–280, 1939.
CoffinC.M., DehnerL.P.: Fibroblastic-myofibroblastic tumors in children and adolescents: a clinicopathologic study of 108 examples in 103 patients.Pediatr. Pathol., 11: 559–588, 1991.
9.
CoffinC.M., JaszczW., O'SheaP.A., DehnerL.P.: So-called congenital-infantile fibrosarcoma: Does it exist and what is it? Pediatr.Pathol., 14: 133–150, 1994.
10.
DahlI., Save-SoderberghJ., AngervalL.: Fibrosarcoma in early infancy.Pathol. Eur., 8: 193–209, 1973.
11.
Dal CinP., BrockP., Casteels van DaeleM., De WeverI., Van DammeB., Van Der BergheH.: Cytogenetic characterization of congenital or infantile fibrosarcoma.Eur. J. Pediatr., 150: 579–581, 1991.
12.
DobsonL., DickeyL.B.: Spontaneous regression of malignant tumors. Reports of twelve-year spontaneous complete regression of an extensive fibrosarcoma, with speculations about regression and dormancy.Am. J. Surg., 92: 162–173, 1956.
EganM.J., RaafatF., CrockerJ., SmithK.: Nucleolar organiser regions in fibrous proliferations of childhood and infantile fibrosarcoma.J. Clin. Pathol., 41: 31–33, 1988.
15.
EnzingerF.M.: Fibrous tumors of infancy. In: Tumors of Bone and Soft Tissue. Eighth annual conference on Cancer, 1963, at the University of Texas, M.D. Anderson Hospital and Tumor Institute, Houston; pp. 375–395, Year Book Medical Publisher Inc., Chicago, 1965.
16.
EnzingerF.M., WeissS.W.: Fibrosarcoma. In: Soft Tissue Tumors.EnzingerF.M., WeissS.W. (Eds.), pp. 201–222, C.V. Mosby, St. Louis, 1988.
GardnerW.J., TurnerO.A.: Primary fibroblastic tumors of choroid plexus of lateral ventricles; clinicopathologic study of three cases.Surg. Gynecol. Obstet., 66: 804–809, 1938.
19.
GerbasiF.S., MargilethA.M., KiblerR.S.: Primary fibrosarcoma of lung in a young child: case treated by lobectomy and cobalt therapy.J. Pediatr., 54: 488–495, 1959.
20.
Gonzales-CrussiF.: Ultrastructure of congenital fibrosarcoma.Cancer, 26: 1289–1299, 1970.
21.
Gonzales-CrussiF., WiederholdM.D., Sotelo AvilaC.: Congenital fibrosarcoma: presence of a histiocytic component.Cancer, 46: 77–86, 1980.
22.
GormanP.A., MaloneM., PritchardJ., SheerD.: Deletion of part of the short arm of chromosome 17 in a congenital fibrosarcoma.Cancer Genet. Cytogenet., 48: 193–198, 1990.
HaysD.M., MirabalV.W., KarlanM.S., PatelH.R., LandingB.H.: Fibrosarcomas in infants and children.J. Pediatr. Surg., 5: 98–102, 1970.
28.
HorneC.H.W., SlavinG., McDonaldA.M.: Late recurrence of juvenile fibrosarcoma.Br. J. Surg., 55: 102–103, 1968.
29.
HudsonO.C.: Intrauterine fibrosarcoma of the foot.Am. J. Cancer, 26: 568–570, 1936.
30.
IwasakiH., EnjojiM.: Infantile and adult fibrosarcoma of the soft tissues.Acta Pathol. Jpm., 29: 377–388, 1979.
31.
KroeseM.C.S., RutgersD.H., WilsI.S., Van UnnikJ.A.M., RohollP.J.M.: The relevance of the DNA index and proliferation rate in the grading of benign and malignant soft tissue tumors.Cancer, 65: 1782–1788, 1990.
32.
KynastonJ.A., MalcomA.J., CraftA.W., DeviesS.M., JonesP.H., KingD.J.: Chemotherapy in the management of infantile fibrosarcoma.Med. Pediatr. Oncol., 21: 488–493, 1993.
33.
MandhalN., HeimS., RydholmA., WillenH., MitelmanF.: Non random chromosome aberrations (+8, +11, +17, +20) in infantile fibrosarcoma.Cancer Genet., Cytogenet., 40: 137–139, 1989.
34.
MartellJ.R.Jr., BusnardoM.S., BarjaR.H.: Congenital fibrosarcoma of the forearm.J. Bone Joint Surg., 68: 620–623, 1986.
35.
MeisJ.M., EnzingerF.M.: Inflammatory fibrosarcoma of the mesentery and retroperitoneum.Am. J. Surg. Pathol., 15: 1146–1156, 1991.
PritchardD.J., SouleE.H., TaylorW.F., IvinsJ.C.: Fibrosarcoma. A clinicopathologic study of 199 tumors of the soft tissues of the extremities and trunk.Cancer, 33: 888–897, 1974.
38.
PuzeyM.S., InnesD.F., Van Der ElstC.W.: Infantile fibrosarcoma presenting as shoulder dystocia. A case report.S. Afr. Med. J., 76: 73–74, 1989.
39.
RobinsonW., CrawfordA.H.: Infantile fibrosarcoma. Report of a case with long-term follow-up.J. Bone Joint Surg., 72A: 292–294, 1990.
40.
RootmanJ., CarvounisE.P., DolmanC.L., DimmickJ.E.: Congenital fibrosarcoma metastatic to the choroid.Am. J. Ophthalmol., 87: 632–638, 1979.
41.
RosenbergH., StenbackW., SpjutH.: The fibromatosis of infancy and childhood.Perspect. Pediatr. Pathol., 4: 269–348, 1978.
ScottS.M., ReimanH.M., PritchardD.J., IlstrupD.M.: Soft tissue fibrosarcoma: a clinicopathologic study of 132 cases.Cancer, 64: 925–931, 1989.
44.
SherburnE.W., TeyaK., BoteroL.M., ShawA.: Pancreaticoduodenectomy in the treatment of congenital fibrosarcoma of the duodenum.J. Pediatr. Surg., 10: 801–806, 1975.
45.
SchoefieldD.E., FletcherJ.A., GrierH.E., YunisE.J.: Fibrosarcoma in infants and children. Applications of new techniques.Am. J. Surg. Pathol., 18: 14–24, 1994.
46.
SiegalA., HorowitzA.: Aggressive fibromatosis-infantile fibrosarcoma. Difficulty of diagnostic and prognostic evaluation.Clin. Pediatr., 17: 517–520, 1978.
47.
SouleE.H., PritchardD.J.: Fibrosarcoma in infants and children. A review of 110 cases.Cancer, 40: 1711–1721, 1977.
48.
SpelemanF., Dal CinP., De PotterK., LaureysG., RoelsH.J., LeroyJ., Van Den BergheH.: Cytogenetic investigation of a case of congenital fibrosarcoma.Cancer Genet. Cytogenet., 39: 21–24, 1989.
StoutA.P.: Fibrosarcoma in infants and children.Cancer, 15: 1028–1040, 1962.
51.
SwainR.E., SessionsD.G., OguraJ.H.: Fibrosarcoma of the head and neck. A clinical analysis of forty case.Ann. Otol. Rhinol. Laryngol., 83: 439–444, 1974.
52.
TeyssierJ.R., FerreD.: Frequent clonal chromosomal changes in human non-malignant tumors.Int. J. Cancer, 44: 828–832, 1989.
53.
TsuneyoshiM., DaimaruY., EnjojiM.: Malignant hemangiopericytoma and other sarcomas with hemangiopericytoma-like pattern.Pathol. Res. Pract., 178: 446–453, 1984.
