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Soft tissue sarcoma in children
Soft Tissue Sarcoma in Children By M. SULAMAAAND C. MUELLER ROM THE SURGICAL point of view, sarcomatous growths of the subcutaneous tissue, fascia, and skeletal musculature appear to form a special group of malignant tumors in children. These so-called soft somatic tissue sarcomas have a relatively favorable prognosis compared with the poor outlook for patients with sarcomas of the internal organs, body cavities, or of bones and joints. This type of tumor is not common and it was not until Stout’s report in 1954r that the favorable prognosis of these growths was recognized. This was confirmed by Pack and ArieL2 who collected statistics of these cases in America. Since then, the surgical attitude toward these tumors has changed. As late as 1953 Fahey and Bollinger3 were arguing that in cases of soft tissue sarcoma of the extremity, amputation was unavoidable. Jensen et al,” however, achieved good results from mere local excision in 6 cases. Their publication was followed later by others with larger numbers of patients, but similar favorable results.fi,ti Hare and Cerny,7 collecting 200 cases, contributed a good deal to the thought that a less mutilating excision gave satisfactory results in cases of soft tissue sarcoma. Similar results were achieved in patients at the Children’s Clinic in Helsinki.* Fourteen cases have been followed for a sufficient period of time to make it worthwhile to describe the principles of treatment and the results that can be achieved.
F
CASE MATERIAL Between 1953 and 1966 14 cases of soft tissue sarcoma in children have been treated. A study of the age distribution shows that 6 of the 14 were under one year of age and it appears that neonates and infants are particularly susceptible. Seven patients were girls and 7 boys. In only 2 cases had the disease been present for any length of time (6 months and 2 years, respectively) before treatment was instituted. The other patients were brought for consultation almost as soon as the tumor was discovered. Three patients came for treatment of recurrent disease after operations had been carried out elsewhere. All the tumors were limited to the subcutaneous tissue, fascia, and skeletal musculature. In one child, metastases were observed in the regional lymph nodes (case 8 ) . In 2 children, who had been operated upon elsewhere, there were distant metastases. In one of these (case 4) the patient died after 6 months from lung
From the Department of Pediatric Surgery, University, Hospital Helsinki, Finland. M. SULAMAA, M.D.: Professor of Pediatric Surgery and Head of the Department of Pediatric Surgery, University Hospital, Helsinki. C. MILLER, M.D.: Consultant Surgeon, University Hospital, Helsinki. 520
JOURNAL OF PEDIATRICSURGERY,VOL. 4, No. 5 (OCTOBER), 1969
521
SOFT TISSUE SARCOMA
metastases. In the other case, pleural metastases were confirmed by thoracotomy (case 10). TREATMENT AND RESULTS
Operative treatment consisted of extensive excision of the tumor, including healthy surrounding tissue, regardless of the tissue defects that might be caused. Where regional lymph node involvement was suspected, these were removed, and in one case pleural metastases were excised at thoracotomy. On principle,
Fig. l.-Rhabdomyosarcoma in a newborn (case 1). A, Photo and B, x-ray of lower extremities. C, Histology of rhabdomyosarcoma (60X). D, (150X).
522
SULAMAA
Table CaSe
No.
Age
L-Fourteen Histologic DiagnoSis
Embryonal rhabdomyosarcoma Newborn Fibrosarcoma Newborn Fibrosarcoma Newborn? Sarcoma of uncertain histogenesis
Cases
of Soft Tissue Metastases
site
Sarcoma
Exci- Radiasion tion
AND
MijLLER
in Children
Chemotherapy
Recur- Follow-up (in ye.1 rence -
Dead
Newborn
1
2 3 4
3 months 3 months
5
6 7
2 years
8
2 years
Fibrosarcoma Sarcoma of uncertain histogenesis Sarcoma of uncertain histogenesis Myosarcoma
Right calf Left sole Right ankle Back Neck Umbilical region Left thigh Left trochanteric region
-
+* + +
-
-
3 9 4.5
+
+
+
0.5
+
-
-
6.5
_
+I:
-t
+
4.5
-
+
+
+
4
f (Lymph nodes)
+
+
+
1
-
-Ii-
f
+
6 6
+
-
7.5 4 5.5 16
+ (lung) -
2.5 years
Fibrosarcoma
10
4 years
Myosarcoma
Left pectoral region Left arm
11 12 13 14
9 years 10 years 12 years 13 years
Myxoliposarcoma Fibroliposarcoma Fibrosarcoma Fibrosarcoma
Sacral region Back Right calf Right thigh
9
(ple+ura) f _ + + +
+
+
* Disarticulation. t Came to treatment at 11 months of age. $ Incomplete excision.
we avoided amputation of extremities. In a newborn girl, a rhabdomyosarcoma of the proximal calf had partially surrounded the nerves and blood vessels; a disarticulation of the knee joint was therefore performed (Fig. 1). Radiotherapy and, in more recent times chemotherapy, have been used in cases where it was suspected that tumor was not completely removed at surgery. Radiotherapy was used in the three cases with local recurrence. Two of our 14 patients died. One of them was a 2-year-old girl (case 8) who had been operated upon three times previously elsewhere. She was brought to us with a third recurrence in the trochanteric region and metastases in the inguinal lymph nodes. Extensive excision together with removal of the inguinal nodes, radiotherapy, and chemotherapy did not delay the advanced malignant process and she died within a year. The other patient, a boy, had had a tumor of the back since birth. He was not brought for treatment until the age of I1 months, with a history that the tumor had been growing for 2 months. A large fibrosarcoma was removed from the dorsal musculature. There were metastases in both lungs and in the mediastinum. In spite of x-ray and chemotherapy the patient died in 6 months. Twelve patients are living with no sign of recurrence of the tumor. Table 1 shows the periods of survival, which in 7 cases exceed 5 years, and in all exceed 3 years. The extensive excisions have, of course, left the patients with
SOFT TISSUE SARCOMA
523
some incapacities. Two patients are suffering from a growth disturbance of the lower extremities, and a third child has a weakness and awkwardness of the upper arm. In a fourth case, there is a severe deformity of the right foot as a result of excision (Fig. 2).
Fig. 2.-Fibrosarcoma in a newborn (case 3). A, Photo of lesion. B, Histology of fibrosarcoma (60X) showing herring bone arrangement of the growth (150X).
524
SULAMAA
AND MijLLER
DISCUSSION There is a favorable chance of cure by extensive surgical excision without resorting to amputation in soft tissue sarcoma. Chemotherapy and x-ray therapy were adopted only when there was recurrence or in those cases where tumor tissue was thought to be incompletely removed. Our case material shows that the prognosis could hardly be improved even if a more radical and more mutilating operation had been performed. This series is small compared with collective statistics in the literature, but these patients come from one pediatric clinic and the therapy therefore has been uniform. Recent surveys paint a considerably gloomier picture of the prognosis for soft tissue sarcoma, and emphasize the need for primary amputation” or at least a postoperative combination of radiotherapy and chemotherapy.lO Our experience has led us to avoid amputation and primary radiotherapy and chemotherapy. It is possible that a more accurate histologic classification would shed additional light on this problem. Lieberman et all1 have recently carried out a special histologic study of alveolar soft tissue sarcomas. In children, these tumors have a particularly favorable prognosis. Vinnik and Altman’” and Balsaver et all3 have also reported favorable clinical courses in newborns with fibrosarcoma. On the other hand, Lawrence et al.l* discuss the malignant nature of some embryonal rhabdomyosarcomas. We have only seen this type of tumor once. In that case, a disarticulation of the knee joint was performed because of hopeless local tissue involvement. Every method of treatment must, of course, be controlled by careful supervision and as our experience shows, even recurrent cases with metastases can often be treated with complete success. SUMMARY Fourteen cases of soft tissue sarcoma in children are reviewed. Twelve had a favorable outcome; there were 2 deaths, 6 months and one year respectively, after surgery. Five children have been followed-up for more than 3 years, and 7 for more than 5 years. Treatment consisted in extensive local excision of the tumor regardless of the tissue defects thus produced. Additional radiotherapy and chemotherapy have only been used in recurrent cases and in children with metastases as well as in those situations where the excision was considered to be incomplete. SUMMARIO
IN INTERLINGUA
Un serie de 14 cases de sarcoma de tissu molle in juveniles es revistate. Le resultato final esseva favorabile in 12. Le serie includeva 2 cases de morte, occurrente 6 menses e 1 anno post le operation, respectivemente. Cinque de1 juveniles ha essite sub observation catamnestic durante periodos de plus que 3 annqs e 7 durante periodos de plus que 5 annos. Le tractamento consisteva in le extense excision local de1 tumor, sin reguardo al magnitude de1 defectos tissular assi producite. Radiotherapia e chimotherapia additional esseva usate solo in cases recurrente e in juveniles con metastases e etiam in situationes in que il esseva suspicite que le excision esseva incomplete.
SOFT
TISSUE
525
SARCOMA
REFERENCES 1. Stout, A. P.: Juvenile fibromatoses. Cancer 7:9.53, 1954. 2. Pack, G. T. and Ariel, J. M.: Sarcomas of the soft somatic tissues in infants and children, Surg. Gynec. Obstet. 98:675, 1954. 3. Fahey, J. J. and Bollinger, J. A.: Congenital sarcoma of the foot. Case report and review of literature. Amer. J. Dis. Child. 86:23, 1953. 4. Jensen, A. R., Martin, L. W., and Longino, L. A.: Digital neurofibrosarcoma in infancy. J. Pediat. 51:566, 1957. S. Kauffmann, S. L. and Stout, A. P.: Lipoblastic tumors of children. Cancer 12: 912, 1959. 6. Shafer. A. I).: Sarcoma of the leg in a newborn infant. J. Pediat. 59:97, 1960. 7. Hare, H. F. and Cerny, M. J.: Soft tissue sarcoma. A review of 200 cases. Cancer 16:1332, 1963. 8. Moller, C.: Weichteilsarkome im Sauglings-und Kindesalter. Zschr. Kinderchirurgie 3 : 164. 1966.
9. Brennhovd, J. D.: The treatment of soft tissue sarcomas-a plea for a more urgent and aggressive approach. Acta Chir. Stand. 131:438, 1966. 10. Yonemoto, R. H., Byron. R. L. Jr. and Melville, L. J.: Combined irradiation, intra-arterial chemotherapy, and surgery for the treatment of sarcomas of the extremities. Surgery 61:355, 1967. 11. Lieberman, P. H., Foote, F. W., Stewart, F. W., and Berg, J. W.: Alveolar softpart sarcoma. J. Amer. Med. Ass. 198:1047, 1966. 12. Vinnik, M. and Altman, D. H.: Congenital malignant tumors. Cancer 19:967. 1966. 13. Balsaver, A. M., Butler, J. J., and Martin, R. G.: Congenital fibrosarcoma. Cancer 20: 1607, 1967. 14. Lawrence, W. Jr., Jegge, G.. and Foote, F. W., Jr.: Embryonal rhabdomyosarcoma: A clinicopathological study. Cancer 17:361, 1964.
F
CASE MATERIAL Between 1953 and 1966 14 cases of soft tissue sarcoma in children have been treated. A study of the age distribution shows that 6 of the 14 were under one year of age and it appears that neonates and infants are particularly susceptible. Seven patients were girls and 7 boys. In only 2 cases had the disease been present for any length of time (6 months and 2 years, respectively) before treatment was instituted. The other patients were brought for consultation almost as soon as the tumor was discovered. Three patients came for treatment of recurrent disease after operations had been carried out elsewhere. All the tumors were limited to the subcutaneous tissue, fascia, and skeletal musculature. In one child, metastases were observed in the regional lymph nodes (case 8 ) . In 2 children, who had been operated upon elsewhere, there were distant metastases. In one of these (case 4) the patient died after 6 months from lung
From the Department of Pediatric Surgery, University, Hospital Helsinki, Finland. M. SULAMAA, M.D.: Professor of Pediatric Surgery and Head of the Department of Pediatric Surgery, University Hospital, Helsinki. C. MILLER, M.D.: Consultant Surgeon, University Hospital, Helsinki. 520
JOURNAL OF PEDIATRICSURGERY,VOL. 4, No. 5 (OCTOBER), 1969
521
SOFT TISSUE SARCOMA
metastases. In the other case, pleural metastases were confirmed by thoracotomy (case 10). TREATMENT AND RESULTS
Operative treatment consisted of extensive excision of the tumor, including healthy surrounding tissue, regardless of the tissue defects that might be caused. Where regional lymph node involvement was suspected, these were removed, and in one case pleural metastases were excised at thoracotomy. On principle,
Fig. l.-Rhabdomyosarcoma in a newborn (case 1). A, Photo and B, x-ray of lower extremities. C, Histology of rhabdomyosarcoma (60X). D, (150X).
522
SULAMAA
Table CaSe
No.
Age
L-Fourteen Histologic DiagnoSis
Embryonal rhabdomyosarcoma Newborn Fibrosarcoma Newborn Fibrosarcoma Newborn? Sarcoma of uncertain histogenesis
Cases
of Soft Tissue Metastases
site
Sarcoma
Exci- Radiasion tion
AND
MijLLER
in Children
Chemotherapy
Recur- Follow-up (in ye.1 rence -
Dead
Newborn
1
2 3 4
3 months 3 months
5
6 7
2 years
8
2 years
Fibrosarcoma Sarcoma of uncertain histogenesis Sarcoma of uncertain histogenesis Myosarcoma
Right calf Left sole Right ankle Back Neck Umbilical region Left thigh Left trochanteric region
-
+* + +
-
-
3 9 4.5
+
+
+
0.5
+
-
-
6.5
_
+I:
-t
+
4.5
-
+
+
+
4
f (Lymph nodes)
+
+
+
1
-
-Ii-
f
+
6 6
+
-
7.5 4 5.5 16
+ (lung) -
2.5 years
Fibrosarcoma
10
4 years
Myosarcoma
Left pectoral region Left arm
11 12 13 14
9 years 10 years 12 years 13 years
Myxoliposarcoma Fibroliposarcoma Fibrosarcoma Fibrosarcoma
Sacral region Back Right calf Right thigh
9
(ple+ura) f _ + + +
+
+
* Disarticulation. t Came to treatment at 11 months of age. $ Incomplete excision.
we avoided amputation of extremities. In a newborn girl, a rhabdomyosarcoma of the proximal calf had partially surrounded the nerves and blood vessels; a disarticulation of the knee joint was therefore performed (Fig. 1). Radiotherapy and, in more recent times chemotherapy, have been used in cases where it was suspected that tumor was not completely removed at surgery. Radiotherapy was used in the three cases with local recurrence. Two of our 14 patients died. One of them was a 2-year-old girl (case 8) who had been operated upon three times previously elsewhere. She was brought to us with a third recurrence in the trochanteric region and metastases in the inguinal lymph nodes. Extensive excision together with removal of the inguinal nodes, radiotherapy, and chemotherapy did not delay the advanced malignant process and she died within a year. The other patient, a boy, had had a tumor of the back since birth. He was not brought for treatment until the age of I1 months, with a history that the tumor had been growing for 2 months. A large fibrosarcoma was removed from the dorsal musculature. There were metastases in both lungs and in the mediastinum. In spite of x-ray and chemotherapy the patient died in 6 months. Twelve patients are living with no sign of recurrence of the tumor. Table 1 shows the periods of survival, which in 7 cases exceed 5 years, and in all exceed 3 years. The extensive excisions have, of course, left the patients with
SOFT TISSUE SARCOMA
523
some incapacities. Two patients are suffering from a growth disturbance of the lower extremities, and a third child has a weakness and awkwardness of the upper arm. In a fourth case, there is a severe deformity of the right foot as a result of excision (Fig. 2).
Fig. 2.-Fibrosarcoma in a newborn (case 3). A, Photo of lesion. B, Histology of fibrosarcoma (60X) showing herring bone arrangement of the growth (150X).
524
SULAMAA
AND MijLLER
DISCUSSION There is a favorable chance of cure by extensive surgical excision without resorting to amputation in soft tissue sarcoma. Chemotherapy and x-ray therapy were adopted only when there was recurrence or in those cases where tumor tissue was thought to be incompletely removed. Our case material shows that the prognosis could hardly be improved even if a more radical and more mutilating operation had been performed. This series is small compared with collective statistics in the literature, but these patients come from one pediatric clinic and the therapy therefore has been uniform. Recent surveys paint a considerably gloomier picture of the prognosis for soft tissue sarcoma, and emphasize the need for primary amputation” or at least a postoperative combination of radiotherapy and chemotherapy.lO Our experience has led us to avoid amputation and primary radiotherapy and chemotherapy. It is possible that a more accurate histologic classification would shed additional light on this problem. Lieberman et all1 have recently carried out a special histologic study of alveolar soft tissue sarcomas. In children, these tumors have a particularly favorable prognosis. Vinnik and Altman’” and Balsaver et all3 have also reported favorable clinical courses in newborns with fibrosarcoma. On the other hand, Lawrence et al.l* discuss the malignant nature of some embryonal rhabdomyosarcomas. We have only seen this type of tumor once. In that case, a disarticulation of the knee joint was performed because of hopeless local tissue involvement. Every method of treatment must, of course, be controlled by careful supervision and as our experience shows, even recurrent cases with metastases can often be treated with complete success. SUMMARY Fourteen cases of soft tissue sarcoma in children are reviewed. Twelve had a favorable outcome; there were 2 deaths, 6 months and one year respectively, after surgery. Five children have been followed-up for more than 3 years, and 7 for more than 5 years. Treatment consisted in extensive local excision of the tumor regardless of the tissue defects thus produced. Additional radiotherapy and chemotherapy have only been used in recurrent cases and in children with metastases as well as in those situations where the excision was considered to be incomplete. SUMMARIO
IN INTERLINGUA
Un serie de 14 cases de sarcoma de tissu molle in juveniles es revistate. Le resultato final esseva favorabile in 12. Le serie includeva 2 cases de morte, occurrente 6 menses e 1 anno post le operation, respectivemente. Cinque de1 juveniles ha essite sub observation catamnestic durante periodos de plus que 3 annqs e 7 durante periodos de plus que 5 annos. Le tractamento consisteva in le extense excision local de1 tumor, sin reguardo al magnitude de1 defectos tissular assi producite. Radiotherapia e chimotherapia additional esseva usate solo in cases recurrente e in juveniles con metastases e etiam in situationes in que il esseva suspicite que le excision esseva incomplete.
SOFT
TISSUE
525
SARCOMA
REFERENCES 1. Stout, A. P.: Juvenile fibromatoses. Cancer 7:9.53, 1954. 2. Pack, G. T. and Ariel, J. M.: Sarcomas of the soft somatic tissues in infants and children, Surg. Gynec. Obstet. 98:675, 1954. 3. Fahey, J. J. and Bollinger, J. A.: Congenital sarcoma of the foot. Case report and review of literature. Amer. J. Dis. Child. 86:23, 1953. 4. Jensen, A. R., Martin, L. W., and Longino, L. A.: Digital neurofibrosarcoma in infancy. J. Pediat. 51:566, 1957. S. Kauffmann, S. L. and Stout, A. P.: Lipoblastic tumors of children. Cancer 12: 912, 1959. 6. Shafer. A. I).: Sarcoma of the leg in a newborn infant. J. Pediat. 59:97, 1960. 7. Hare, H. F. and Cerny, M. J.: Soft tissue sarcoma. A review of 200 cases. Cancer 16:1332, 1963. 8. Moller, C.: Weichteilsarkome im Sauglings-und Kindesalter. Zschr. Kinderchirurgie 3 : 164. 1966.
9. Brennhovd, J. D.: The treatment of soft tissue sarcomas-a plea for a more urgent and aggressive approach. Acta Chir. Stand. 131:438, 1966. 10. Yonemoto, R. H., Byron. R. L. Jr. and Melville, L. J.: Combined irradiation, intra-arterial chemotherapy, and surgery for the treatment of sarcomas of the extremities. Surgery 61:355, 1967. 11. Lieberman, P. H., Foote, F. W., Stewart, F. W., and Berg, J. W.: Alveolar softpart sarcoma. J. Amer. Med. Ass. 198:1047, 1966. 12. Vinnik, M. and Altman, D. H.: Congenital malignant tumors. Cancer 19:967. 1966. 13. Balsaver, A. M., Butler, J. J., and Martin, R. G.: Congenital fibrosarcoma. Cancer 20: 1607, 1967. 14. Lawrence, W. Jr., Jegge, G.. and Foote, F. W., Jr.: Embryonal rhabdomyosarcoma: A clinicopathological study. Cancer 17:361, 1964.