Br. J. Cancer Br. J. Cancer
'."
S72-S75 (1992), XVIII, Suppi. XVIII, 66, Suppl. (1992), 66, S72-S75
©
Macmillan Press Ltd., 1992
Macmillan
Press
Ltd.,
1992
Spontaneous regression of neonatal fibrosarcoma N.P. Madden', R.D. Spicer', E.B. Allibone2 & I.J. Lewis3 Department of 'Paediatric Surgery, 2Pathology, and 3Paediatric Oncology, St Jame's University Hospital, Beckett Street, Leeds LS9 7TF, W. Yorks, UK. Summary We report a case of fibrosarcoma, which presented in a two week old boy. Excision was not performed because it would have required mutilating surgery. The tumour regressed and was impalpable by 7 months of age. The patient is tumour free at 4 years of age. This is the first reported case of spontaneous regression of a fibrosarcoma. The literature is reviewed. We conclude that the chances of metastasis are low, tumours are likely to respond to chemotherapy, and mutilating surgery is not appropriate initial treatment for infantile fibrosarcomas.
In 1962, Stout reviewed the existing literature and reported 23 new cases of fibrosarcoma in infants and children. He found a metastasis rate of 7.6%, and subsequent reports have confirmed this (Chung & Enzinger, 1976; Soule & Pritchard, 1977). In contrast, the five year survival rate for fibrosarcoma in adults is between 50% (Pritchard et al., 1974) and 64% (Iwasaki & Enjoji, 1979). The histological diagnosis of fibrosarcoma can be difficult, requiring differentiation from other mesenchymal malignancies and aggressive fibromatosis (Chung & Enzinger, 1976). Some authors report no difference in the microscopic appearance of fibrosarcoma in children and adults, whereas others (Chung & Enzinger, 1976; Iwasaki & Enjoji, 1979) suggest that in children the tumour cells appear less mature, lymphocytic infiltration is more marked, fascicular arrangement of cells is less apparent and vascular spaces are more prominent. However, there is general agreement that the microscopic appearances cannot be used to predict the biological behaviour of infantile fibromatosis or infantile fibrosarcoma. This distinction is somewhat academic since fibrosarcoma often shows a very benign natural history, whereas at least seven cases of infantile fibromatosis have been reported to metastasise (Shankwiler et al., 1989), and fibromatosis responds to chemotherapy (Mitrofanoff et al., 1988). We report a unique case of a fibrosarcoma presenting in a neonate, which resolved spontaneously. This stimulated us to carry out an exhaustive literature review of all adequately documented cases of fibrosarcoma presenting under the age of one year.
Case report A 2 week old boy presented with a lump on his left forearm which his parents said had appeared 'overnight'. On examination, there was a 4 cm diameter tumour involving the flexor muscles with normal overlying skin. Ultrasound showed a diffuse soft tissue mass, and there was no bony erosion on X-ray. The mass was thought to represent haemorrhage into an underlying haemangioma. At the age of four weeks the tumour was occupying the whole of the left forearm and an x-ray of the arm showed bony destruction of the left ulna (Figure 2a). Investigations including a CT scan, skeletal survey, bone scan, liver function tests and tumour markers (alpha-fetoprotein, betahuman chorionic gonadotrophin and neurone specific enolase) were all normal apart from a raised LDH (lactate dehydrogenase) 629 IU 1', (normal 160-430). The lesion
a
b
Figure 1 External appearance of left arm before treatment.
Correspondence: N.P. Madden, Department of Paediatric Surgery, Clarendon Wing, Leeds General Infirmary, Leeds LS2 9NS, UK.
Figure 2 a, Xray of left forearm at 4 weeks of age showing soft tissue tumour with bony erosion. b, Xray at 2 years of age: no soft tissue tumour and the erosion has healed.
SPONTANEOUS REGRESSION OF NEONATAL FIBROSARCOMA
S73
was explored; it was arising in the forearm muscles, involving the neurovascular bundle and extending between the radius and ulna. Resection would have required amputation at the elbow so the tumour was biopsied only. Histologically (Figure 3) this was a highly cellular tumour of small spindle cells, arranged in fasciculi and diffusely infiltrated by small lymphocytes. There was a fine pattern of intercellular reticulin and a rich vasculature, which was regarded as belonging to the tumour itself, and not to infiltrated tissues, because the vessels were small, of uniform size and distributed uniformly through the tumour tissue. There was mild nuclear pleomorphism. Mitoses were comparatively few, on average 0.38 per x 400 high power field, with a maximum count of 2 in any field of 100 counted. At one edge of the biopsy, tumour was seen infiltrating between voluntary muscle bundles. No material was submitted for cytogenetic studies or electron microscopy. We elected to observe the tumour and give no other treatment. The tumour continued to enlarge at a rapid rate up to the age of six weeks, at a slower rate thereafter and then regressed; by the age of seven months the tumour was impalpable (Figure 4), though the left forearm remained hypertrophied compared with the right. At the age of four years, the child is well with no signs of disease (Figure 4) and normal function in the affected limb. Literature review We have reviewed 78 cases of fibrosarcoma reported in the literature in children under one year of age. The total number reported in the literature is greater than this, but we
a Figure 4 External appearance of left arm after regression.
Figure 3 a, Haematoxylin and eosin stain ( x 160): showing fascicles of uniform spindle cells with diffuse infiltrate of small lymphocytes. b, Reticulin stain ( x 160): showing intercellular collagen with many uniform intrinsic vessels.
have limited our analysis to cases with full documentation (Table I). Thirty-nine patients were male and 32 female. Sixty patients (77%) were under one month of age, of whom 30 were male. The limb was the commonest site (32 lower limb, 17 upper limb), 16 tumours were on the trunk and 13 on the head or neck. Age at presentation was not influenced by the site of the tumour. Sixty-two patients were treated by surgery alone, five by chemotherapy alone, four by chemotherapy plus surgery, two by radiotherapy alone and four by radiotherapy plus surgery. Surgical treatment was amputation of a limb in 15, local excision from the limb in 27 and local excision from other sites of the body in 20. One patient was biopsied only. Patients treated by amputation of a limb had no local recurrence, but a 7% metastasis rate. Local excision of a limb tumour resulted in a similar metastasis rate (4%) but a 46% local recurrence rate. Local excision from other sites resulted in a 20% recurrence rate and a 15% metastasis rate. Of the five patients treated with chemotherapy alone, one patient had a local recurrence and a late metastasis both of which were successfully treated. The four patients treated with surgery and chemotherapy had no local recurrences and no metastases. Of the two patients treated by radiotherapy alone, one had a late metastasis and of the four patients treated with radiotherapy and surgery, one had a local recurrence and one a late metastasis. The recurrence rate in patients presenting under the age of one month was 30% and under the age of one year 5% (this difference is not statistically significant). The overall recurrence rate was 24%. The metastasis rate was almost identical under one month and under one year with an overall metastasis rate of 10%. Figure 5 shows the age at treatment, emphasising that the
S74
N.P. MADDEN et al.
Table I References of origin of cases in review Number of cases Author 5 Balsaver et al. (1967) 9 8 other authors quoted by Balsaver et al. 5 Blocker et al. (1987) 8 8 other authors quoted by Blocker et al. 6 Chung & Enzinger (1976) 2 Delepine et al. (1986) 2 Drut (1986) 2 Gonzalez-Crussi et al. (1979) 3 Goslee et al. (1964) 2 Grier et al. (1985) 5 Hays et al. (1970) 3 Ninane et al. (1986) 2 Siegal & Horowitz (1978) 1 Sinclair-Smith et al. (1989) 13 Soule & Pritchard (1977) 1a Stout (1954) 4 Stout (1962) 3 Sulamaa & Moller (1969) 2 Wee et al. (1979) 78 Total aCase not included in the author's subsequent paper.
majority of patients presented in the first month of life and were treated in the first month of life. The rate of regression of our patient's tumour is shown on the same figure, illustrating that, in all but three patients, treatment was instituted too early to know whether spontaneous regression would have occurred within the same time scale as in our patient. Discussion
The difficulty of making a histological diagnosis of fibrosarcoma has been mentioned above. However, the microscopic appearances do not predict the clinical behaviour. Our case indicates that at least some of these cases may have benign natural history. It is possible that molecular genetics will provide the key to predicting the natural history. Unfortunately, our case presented before cytogenetics was a routine part of the examination of tumours in our unit, however recent work has demonstrated trisomies, which appear to be different
- 6 Tumour length ME Presented 1 m to 1 y - 5 Presented as neonate 4_ ,
30 -
-
25 20 -u,
a)
\3
-
(L 0
10
-
0
0 0
1
2
3
4
5
6
7
8
9 10 11 12
Age at treatment (months) Figure 5 Regression of the tumour (tumour length is shown in relation to the age at treatment of the cases reviewed.
from the chromosome abnormalities observed in adult fibrosarcomas (Mandahl et al., 1989; Speleman et al., 1989). We strongly urge that clinicians treating patients with these rare tumours should ensure that tumour tissue is made available for full biological evaluation including cytogenetics, ploidy and DNA studies. Information thus obtained should then be pooled by organisations such as the United Kingdom Children's Cancer Study Group (UKCCSG) and the International Society of Paediatric Oncology (SIOP) so that it may be correlated with clinical behaviour. In 1962, Stout wrote 'If, after biopsy or incomplete excision, the tumour proves to be a fibrosarcoma, one should not sacrifice extremeties without making every attempt to achieve a cure by less dramatic measures. The fact that the chances of metastasis are so remote in a given case should influence the surgeon's judgement in planning treatment.' Furthermore, there is now growing evidence of the effectiveness of chemotherapy in treating fibrosarcomas in infants (Delepine et al., 1986; Grier et al., 1985; Ninane et al., 1986). We conclude that mutilating surgery is not appropriate for infantile fibrosarcoma. If simple excision is impossible, we advocate a period of active observation. If the tumour does not regress, chemotherapy can be used to reduce tumour bulk, and thus the magnitude of excisional surgery. Chemotherapy can also be used to treat local recurrences and metastases.
References BALSAVER, A.M., BUTLER, J.J. & MARTIN, R.G. (1967). Congenital fibrosarcoma. Cancer, 10, 1607.
IWASAKI, H. & ENJOJI, M. (1979). Infantile and adult fibrosarcomas of the soft tissues. Acta Path. Jap., 29, 377.
BERNADO, L., ADMELLA, C., LUCAYA, J., SANCHEZ DE TOLEDO, J.
MANDAHL, N., HEIM, S., RYDHOLM, A., WILLEN, H. & MITELMAN,
& BOSCH, J. (1987). Infantile fibrosarcoma of femur. Pediatr. Pathol., 7, 201. BLOCKER, S., KOENIG, J. & TERNBERG, J. (1987). Congenital fibrosarcoma. J. Pediatr. Surg., 22, 665. CHUNG, E.B. & ENZINGER, F.M. (1976). Infantile fibrosarcoma. Cancer, 38, 729.
F. (1989). Nonrandom chromosome aberrations ( + 8, + 11, + 17, + 20) in infantile fibrosarcoma. Cancer Genet. Cytogenet., 40, 137. MITROFANOFF, P., VANNIER, J.P., BACHY, B., HEMET, J., BOILLOT,
B. & BORDE, J. (1988). Fibromatoses de l'enfant: Regression sous chimiotherapie prolongee. Chir. Pediatr., 29, 325.
DELEPINE, N., CORNILLE, H., DESBOIS, J.C., DELEPINE, G. &
NINANE, J., GOSSEYE, S., PANTEON, E., CLAUS, D., ROMBOUTS,
VOISIN, M.C. (1986). Complete response of congenital fibrosarcoma to chemotherapy. Lancet, 2, 1453. DRUT, R. (1986). Congenital fibrosarcoma: a congenital fibrous histiocytoma. Pediatr. Pathol., 6, 403.
J.-J. & CORNU, G. (1986). Congenital fibrosarcoma preoperative chemotherapy and conservative surgery. Cancer, 58, 1400. PRITCHARD, D.J., SOULE, E.H., TATLOR, W.F. & IVINS, J.C. (1974). Fibrosarcoma - a clinicopathological and statistical study of 199 tumors of the soft tissues of the extremeties and trunk. Cancer, 33, 88. SHANKWILER, R.A., ATHEY, P.A. & LAMKI, N. (1989). Aggressive infantile fibromatosis. Pulmonary metastases documented by plain film and computed tomography. Clin. Imaging, 29, 325. SIEGAL, A. & HOROWITZ, A. (1978). Aggressive fibromatosis (infantile fibrosarcoma). Clin. Pediatr., 17, 517. SINCLAIR-SMITH, C.C., EMMS, M. & MILLS, A.E. (1989). Intracisternal paracrystalline serpigenous inclusions: a marker of disordered fibroblastic proliferation. Ultrastruct. Pathol., 13, 443.
GONZALEZ-CRUSSI, F., WIEDERHOLD, M.D. & SOTELO-AVILA, C.
(1979). Congenital fibrosarcoma, presence of a histiocytic component. Cancer, 46, 77. GOSLEE, L., CLERMONT, V., BERNSTEIN, J. & WOOLEY, P.V. (1964).
Superficial connective tumors in early infancy. J. Pediatr., 65, 377. GRIER, H.E., PEREZ-ATAYDE, A.R. & WEINSTEIN, H.J. (1985).
Chemotherapy for inoperable infantile fibrosarcoma. Cancer, 56, 1507. HAYS, D.M., MIRABAL, V.Q., KARLAN, M.S., PATEL, H.R. & LAN-
DING, B.H. (1970). Fibrosarcomas in infants and children. J. Pediatr. Surg., 5, 176.
SPONTANEOUS REGRESSION OF NEONATAL FIBROSARCOMA SOULE, E.H. & PRITCHARD, D.J. (1977). Fibrosarcoma in infants and children: a review of 110 cases. Cancer, 40, 1711. SPELEMAN, F., DAL-CIN, P., DE POTTER, K., LAUREYS, G., ROELS,
H.J. & LEROY, J. (1989). Cytogenetic investigation of a case of congental fibrosarcoma. Cancer Genet. Cytogenet., 39, 21. STOUT, A.P. (1954). Juvenile fibromatoses. Cancer, 7, 953.
S75
STOUT, A.P. (1962). Fibrosarcoma in infants and children. Cancer, 15, 1028. SULAMAA, M. & MOLLER, M. (1969). Soft tissue sarcoma in children. J. Pediatr. Surg., 4, 520. WEE, A., PHO, R.W.H. & ONG, L.B. (1979). Infantile fibrosarcoma report of cases. Arch. Pathol. Lab. Med., 103, 236.
'."
S72-S75 (1992), XVIII, Suppi. XVIII, 66, Suppl. (1992), 66, S72-S75
©
Macmillan Press Ltd., 1992
Macmillan
Press
Ltd.,
1992
Spontaneous regression of neonatal fibrosarcoma N.P. Madden', R.D. Spicer', E.B. Allibone2 & I.J. Lewis3 Department of 'Paediatric Surgery, 2Pathology, and 3Paediatric Oncology, St Jame's University Hospital, Beckett Street, Leeds LS9 7TF, W. Yorks, UK. Summary We report a case of fibrosarcoma, which presented in a two week old boy. Excision was not performed because it would have required mutilating surgery. The tumour regressed and was impalpable by 7 months of age. The patient is tumour free at 4 years of age. This is the first reported case of spontaneous regression of a fibrosarcoma. The literature is reviewed. We conclude that the chances of metastasis are low, tumours are likely to respond to chemotherapy, and mutilating surgery is not appropriate initial treatment for infantile fibrosarcomas.
In 1962, Stout reviewed the existing literature and reported 23 new cases of fibrosarcoma in infants and children. He found a metastasis rate of 7.6%, and subsequent reports have confirmed this (Chung & Enzinger, 1976; Soule & Pritchard, 1977). In contrast, the five year survival rate for fibrosarcoma in adults is between 50% (Pritchard et al., 1974) and 64% (Iwasaki & Enjoji, 1979). The histological diagnosis of fibrosarcoma can be difficult, requiring differentiation from other mesenchymal malignancies and aggressive fibromatosis (Chung & Enzinger, 1976). Some authors report no difference in the microscopic appearance of fibrosarcoma in children and adults, whereas others (Chung & Enzinger, 1976; Iwasaki & Enjoji, 1979) suggest that in children the tumour cells appear less mature, lymphocytic infiltration is more marked, fascicular arrangement of cells is less apparent and vascular spaces are more prominent. However, there is general agreement that the microscopic appearances cannot be used to predict the biological behaviour of infantile fibromatosis or infantile fibrosarcoma. This distinction is somewhat academic since fibrosarcoma often shows a very benign natural history, whereas at least seven cases of infantile fibromatosis have been reported to metastasise (Shankwiler et al., 1989), and fibromatosis responds to chemotherapy (Mitrofanoff et al., 1988). We report a unique case of a fibrosarcoma presenting in a neonate, which resolved spontaneously. This stimulated us to carry out an exhaustive literature review of all adequately documented cases of fibrosarcoma presenting under the age of one year.
Case report A 2 week old boy presented with a lump on his left forearm which his parents said had appeared 'overnight'. On examination, there was a 4 cm diameter tumour involving the flexor muscles with normal overlying skin. Ultrasound showed a diffuse soft tissue mass, and there was no bony erosion on X-ray. The mass was thought to represent haemorrhage into an underlying haemangioma. At the age of four weeks the tumour was occupying the whole of the left forearm and an x-ray of the arm showed bony destruction of the left ulna (Figure 2a). Investigations including a CT scan, skeletal survey, bone scan, liver function tests and tumour markers (alpha-fetoprotein, betahuman chorionic gonadotrophin and neurone specific enolase) were all normal apart from a raised LDH (lactate dehydrogenase) 629 IU 1', (normal 160-430). The lesion
a
b
Figure 1 External appearance of left arm before treatment.
Correspondence: N.P. Madden, Department of Paediatric Surgery, Clarendon Wing, Leeds General Infirmary, Leeds LS2 9NS, UK.
Figure 2 a, Xray of left forearm at 4 weeks of age showing soft tissue tumour with bony erosion. b, Xray at 2 years of age: no soft tissue tumour and the erosion has healed.
SPONTANEOUS REGRESSION OF NEONATAL FIBROSARCOMA
S73
was explored; it was arising in the forearm muscles, involving the neurovascular bundle and extending between the radius and ulna. Resection would have required amputation at the elbow so the tumour was biopsied only. Histologically (Figure 3) this was a highly cellular tumour of small spindle cells, arranged in fasciculi and diffusely infiltrated by small lymphocytes. There was a fine pattern of intercellular reticulin and a rich vasculature, which was regarded as belonging to the tumour itself, and not to infiltrated tissues, because the vessels were small, of uniform size and distributed uniformly through the tumour tissue. There was mild nuclear pleomorphism. Mitoses were comparatively few, on average 0.38 per x 400 high power field, with a maximum count of 2 in any field of 100 counted. At one edge of the biopsy, tumour was seen infiltrating between voluntary muscle bundles. No material was submitted for cytogenetic studies or electron microscopy. We elected to observe the tumour and give no other treatment. The tumour continued to enlarge at a rapid rate up to the age of six weeks, at a slower rate thereafter and then regressed; by the age of seven months the tumour was impalpable (Figure 4), though the left forearm remained hypertrophied compared with the right. At the age of four years, the child is well with no signs of disease (Figure 4) and normal function in the affected limb. Literature review We have reviewed 78 cases of fibrosarcoma reported in the literature in children under one year of age. The total number reported in the literature is greater than this, but we
a Figure 4 External appearance of left arm after regression.
Figure 3 a, Haematoxylin and eosin stain ( x 160): showing fascicles of uniform spindle cells with diffuse infiltrate of small lymphocytes. b, Reticulin stain ( x 160): showing intercellular collagen with many uniform intrinsic vessels.
have limited our analysis to cases with full documentation (Table I). Thirty-nine patients were male and 32 female. Sixty patients (77%) were under one month of age, of whom 30 were male. The limb was the commonest site (32 lower limb, 17 upper limb), 16 tumours were on the trunk and 13 on the head or neck. Age at presentation was not influenced by the site of the tumour. Sixty-two patients were treated by surgery alone, five by chemotherapy alone, four by chemotherapy plus surgery, two by radiotherapy alone and four by radiotherapy plus surgery. Surgical treatment was amputation of a limb in 15, local excision from the limb in 27 and local excision from other sites of the body in 20. One patient was biopsied only. Patients treated by amputation of a limb had no local recurrence, but a 7% metastasis rate. Local excision of a limb tumour resulted in a similar metastasis rate (4%) but a 46% local recurrence rate. Local excision from other sites resulted in a 20% recurrence rate and a 15% metastasis rate. Of the five patients treated with chemotherapy alone, one patient had a local recurrence and a late metastasis both of which were successfully treated. The four patients treated with surgery and chemotherapy had no local recurrences and no metastases. Of the two patients treated by radiotherapy alone, one had a late metastasis and of the four patients treated with radiotherapy and surgery, one had a local recurrence and one a late metastasis. The recurrence rate in patients presenting under the age of one month was 30% and under the age of one year 5% (this difference is not statistically significant). The overall recurrence rate was 24%. The metastasis rate was almost identical under one month and under one year with an overall metastasis rate of 10%. Figure 5 shows the age at treatment, emphasising that the
S74
N.P. MADDEN et al.
Table I References of origin of cases in review Number of cases Author 5 Balsaver et al. (1967) 9 8 other authors quoted by Balsaver et al. 5 Blocker et al. (1987) 8 8 other authors quoted by Blocker et al. 6 Chung & Enzinger (1976) 2 Delepine et al. (1986) 2 Drut (1986) 2 Gonzalez-Crussi et al. (1979) 3 Goslee et al. (1964) 2 Grier et al. (1985) 5 Hays et al. (1970) 3 Ninane et al. (1986) 2 Siegal & Horowitz (1978) 1 Sinclair-Smith et al. (1989) 13 Soule & Pritchard (1977) 1a Stout (1954) 4 Stout (1962) 3 Sulamaa & Moller (1969) 2 Wee et al. (1979) 78 Total aCase not included in the author's subsequent paper.
majority of patients presented in the first month of life and were treated in the first month of life. The rate of regression of our patient's tumour is shown on the same figure, illustrating that, in all but three patients, treatment was instituted too early to know whether spontaneous regression would have occurred within the same time scale as in our patient. Discussion
The difficulty of making a histological diagnosis of fibrosarcoma has been mentioned above. However, the microscopic appearances do not predict the clinical behaviour. Our case indicates that at least some of these cases may have benign natural history. It is possible that molecular genetics will provide the key to predicting the natural history. Unfortunately, our case presented before cytogenetics was a routine part of the examination of tumours in our unit, however recent work has demonstrated trisomies, which appear to be different
- 6 Tumour length ME Presented 1 m to 1 y - 5 Presented as neonate 4_ ,
30 -
-
25 20 -u,
a)
\3
-
(L 0
10
-
0
0 0
1
2
3
4
5
6
7
8
9 10 11 12
Age at treatment (months) Figure 5 Regression of the tumour (tumour length is shown in relation to the age at treatment of the cases reviewed.
from the chromosome abnormalities observed in adult fibrosarcomas (Mandahl et al., 1989; Speleman et al., 1989). We strongly urge that clinicians treating patients with these rare tumours should ensure that tumour tissue is made available for full biological evaluation including cytogenetics, ploidy and DNA studies. Information thus obtained should then be pooled by organisations such as the United Kingdom Children's Cancer Study Group (UKCCSG) and the International Society of Paediatric Oncology (SIOP) so that it may be correlated with clinical behaviour. In 1962, Stout wrote 'If, after biopsy or incomplete excision, the tumour proves to be a fibrosarcoma, one should not sacrifice extremeties without making every attempt to achieve a cure by less dramatic measures. The fact that the chances of metastasis are so remote in a given case should influence the surgeon's judgement in planning treatment.' Furthermore, there is now growing evidence of the effectiveness of chemotherapy in treating fibrosarcomas in infants (Delepine et al., 1986; Grier et al., 1985; Ninane et al., 1986). We conclude that mutilating surgery is not appropriate for infantile fibrosarcoma. If simple excision is impossible, we advocate a period of active observation. If the tumour does not regress, chemotherapy can be used to reduce tumour bulk, and thus the magnitude of excisional surgery. Chemotherapy can also be used to treat local recurrences and metastases.
References BALSAVER, A.M., BUTLER, J.J. & MARTIN, R.G. (1967). Congenital fibrosarcoma. Cancer, 10, 1607.
IWASAKI, H. & ENJOJI, M. (1979). Infantile and adult fibrosarcomas of the soft tissues. Acta Path. Jap., 29, 377.
BERNADO, L., ADMELLA, C., LUCAYA, J., SANCHEZ DE TOLEDO, J.
MANDAHL, N., HEIM, S., RYDHOLM, A., WILLEN, H. & MITELMAN,
& BOSCH, J. (1987). Infantile fibrosarcoma of femur. Pediatr. Pathol., 7, 201. BLOCKER, S., KOENIG, J. & TERNBERG, J. (1987). Congenital fibrosarcoma. J. Pediatr. Surg., 22, 665. CHUNG, E.B. & ENZINGER, F.M. (1976). Infantile fibrosarcoma. Cancer, 38, 729.
F. (1989). Nonrandom chromosome aberrations ( + 8, + 11, + 17, + 20) in infantile fibrosarcoma. Cancer Genet. Cytogenet., 40, 137. MITROFANOFF, P., VANNIER, J.P., BACHY, B., HEMET, J., BOILLOT,
B. & BORDE, J. (1988). Fibromatoses de l'enfant: Regression sous chimiotherapie prolongee. Chir. Pediatr., 29, 325.
DELEPINE, N., CORNILLE, H., DESBOIS, J.C., DELEPINE, G. &
NINANE, J., GOSSEYE, S., PANTEON, E., CLAUS, D., ROMBOUTS,
VOISIN, M.C. (1986). Complete response of congenital fibrosarcoma to chemotherapy. Lancet, 2, 1453. DRUT, R. (1986). Congenital fibrosarcoma: a congenital fibrous histiocytoma. Pediatr. Pathol., 6, 403.
J.-J. & CORNU, G. (1986). Congenital fibrosarcoma preoperative chemotherapy and conservative surgery. Cancer, 58, 1400. PRITCHARD, D.J., SOULE, E.H., TATLOR, W.F. & IVINS, J.C. (1974). Fibrosarcoma - a clinicopathological and statistical study of 199 tumors of the soft tissues of the extremeties and trunk. Cancer, 33, 88. SHANKWILER, R.A., ATHEY, P.A. & LAMKI, N. (1989). Aggressive infantile fibromatosis. Pulmonary metastases documented by plain film and computed tomography. Clin. Imaging, 29, 325. SIEGAL, A. & HOROWITZ, A. (1978). Aggressive fibromatosis (infantile fibrosarcoma). Clin. Pediatr., 17, 517. SINCLAIR-SMITH, C.C., EMMS, M. & MILLS, A.E. (1989). Intracisternal paracrystalline serpigenous inclusions: a marker of disordered fibroblastic proliferation. Ultrastruct. Pathol., 13, 443.
GONZALEZ-CRUSSI, F., WIEDERHOLD, M.D. & SOTELO-AVILA, C.
(1979). Congenital fibrosarcoma, presence of a histiocytic component. Cancer, 46, 77. GOSLEE, L., CLERMONT, V., BERNSTEIN, J. & WOOLEY, P.V. (1964).
Superficial connective tumors in early infancy. J. Pediatr., 65, 377. GRIER, H.E., PEREZ-ATAYDE, A.R. & WEINSTEIN, H.J. (1985).
Chemotherapy for inoperable infantile fibrosarcoma. Cancer, 56, 1507. HAYS, D.M., MIRABAL, V.Q., KARLAN, M.S., PATEL, H.R. & LAN-
DING, B.H. (1970). Fibrosarcomas in infants and children. J. Pediatr. Surg., 5, 176.
SPONTANEOUS REGRESSION OF NEONATAL FIBROSARCOMA SOULE, E.H. & PRITCHARD, D.J. (1977). Fibrosarcoma in infants and children: a review of 110 cases. Cancer, 40, 1711. SPELEMAN, F., DAL-CIN, P., DE POTTER, K., LAUREYS, G., ROELS,
H.J. & LEROY, J. (1989). Cytogenetic investigation of a case of congental fibrosarcoma. Cancer Genet. Cytogenet., 39, 21. STOUT, A.P. (1954). Juvenile fibromatoses. Cancer, 7, 953.
S75
STOUT, A.P. (1962). Fibrosarcoma in infants and children. Cancer, 15, 1028. SULAMAA, M. & MOLLER, M. (1969). Soft tissue sarcoma in children. J. Pediatr. Surg., 4, 520. WEE, A., PHO, R.W.H. & ONG, L.B. (1979). Infantile fibrosarcoma report of cases. Arch. Pathol. Lab. Med., 103, 236.
