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Chest wall chondrosarcoma in youth
Chest wall chondrosarcoma in youth John A. Odom, Jr., M.D.,* Philadelphia, Pa., William E. DeMuth, Jr.,
M.D.,
Carlisle, Pa., and William S. Blakemore, M.D., Philadelphia, Pa.
Y^hondrosarcoma of the chest wall in the first two decades of life is rare. Indeed, malignant tumors of all types are very rarely encountered in the thoracic wall in this age group. Watkins and Gerard14 found only two malignant tumors of the chest wall in the experience gained during a period of 20 years at the Lahey Clinic. In 1957, Pascuzzi and associates8 reported one of the largest series ever recorded, 144 tumors of the ribs and sternum, and no patient under 25 years of age was encountered. Because of the infrequency with which chondrosarcoma of the thoracic wall is en countered in youth, our experience with such a tumor found in a boy 17 years of age is being reported. Case report D. R. (A-57955), a 17-year-old white boy, was admitted to the hospital on Aug. 6, 1964. He had noticed a mass on the left chest wall for approxi mately 1 year before he consulted a physician. The mass had increased only slightly in size and had never been painful, although he frequently bumped the mass which annoyed him. He had noticed no weight loss, dyspnea, limitation of activity, anorexia, or malaise. The past medical history of the patient was noncontributory. Physical examination. The blood pressure was 110/80 mm. Hg; pulse 64; respirations 20; tem-
From the Department of Surgery, Graduate Hospital of the University of Pennsylvania and the Harrison De partment of Surgical Research, Schools of Medicine, University of Pennsylvania, Philadelphia, Pa., and Carlisle Hospital, Carlisle, Pa. Received for publication June 3, 1965. ♦Clinical Fellow, American Cancer Society.
550
perature 97.4° F. A firm nontender mass, adherent to the left lower thoracic wall in the anterior axillary line, measured 2 by 1.5 by 1.0 inches. No skin discoloration was present. The lungs were clear to auscultation and percussion. The remain ing findings during the physical examination were within normal limits. Radiography. Roentgenograms of the chest (Fig. 1) revealed an amorphous diffuse calcification in a soft tissue mass between the anterior ends of the eighth and ninth ribs on the left side. On the basis of these findings a diagnosis of chondro sarcoma was made. Operation. An oblique incision over the mass was made and a small ellipse of skin was taken with the mass which was excised en bloc. The excision included at least 2 centimeters of grossly normal tissue on all sides of the tumor. The lesion was found to involve intercostal muscles, ribs, parietal pleura, and the adjacent diaphragm was adherent to it. The tumor extended about 5 cm. within the thoracic cavity. This deep involvement was unexpected as it was not detected on x-ray study. The mass was excised in one piece, with the adherent diaphragm taken with it and exposure of the peritoneal cavity. The 8 by 10 cm. defect in the diaphragm was closed with interrupted sutures of Mersilene. Marlex mesh was used to cover the defect in the rib cage which measured 15 by 20 cm. A large rubber tube drain was placed through a stab wound posterior to the defect and attached to suction, and a routine wound closure of sub cutaneous tissue and skin was completed. Course in hospital and follow-up. On the third postoperative day the chest tube was removed. The convalescence was uneventful and the patient was discharged on the fifth postoperative day. The wound healed primarily (Fig. 2). Four months later the patient was noted to have some motion in the defect on respiration. The chest wall defect was firm at this time, and the patient had no limitation of activity. On March 30, 1965, chest roentgenograms demonstrated no evidence of local recurrence or pulmonary me-
Volume 50 Number 4
Chest wall chondrosarcoma 5 5 1
October, 1965
Fig. 3. Gross specimen demonstrates involvement of ribs and thoracic wall. The involved diaphragm is not clearly demonstrated.
Fig. 1. Roentgenogram demonstrates calcification in tumor.
Fig. 2. Photograph which shows area of excision in left anterolateral chest wall. tastasis, and the patient was clinically well and free of any evidence of residual neoplasm. No respiratory paradox was present. Pathology. The gross specimen (Fig. 3) con sisted of the tumor mass, portions of four ribs with their costochondral junctions, and the portion of excised diaphragm. The over-all dimensions of the mass were 12 by 11 by 10 cm. The major portion of the soft tissue mass projected beneath the parietal pleura and the leaf of the diaphragm. This portion extended 8 cm. into the chest cavity
Fig. 4. Photomicrograph which demonstrates char acteristic features of chondrosarcoma (xllO). from its wall. It bulged between and separated the two most central parts of the adjacent ribs. The tu mor was firm and cut as if cartilaginous. It was colorless, glistening, encapsulated, and contained some areas of hemorrhagic necrosis. Focal areas of calification and some areas of ossification were present. It did not appear to invade bone.
Journal of
5 52
Odom,
DeMuth,
Blakemore
Thoracic and Cardiovascular Surgery
Table I
Author
Age of onset and sex
Location
Symptoms
Duration of Symptom (yr.) 2
Phemister9 1930
18 M
Ribs 2, 3, 4, 5, 6 & 7, right Slight pain, tumor anterior
Janes4 1941
16 M
Ribs 9 and 10, left posterior Dry cough, pleural pain, shortness of breath
O'Neal and Ackerman6 1951
11 M
Manubrium
Tumor
Polk et al.30 1961
5 M
Rib, 4th left lateral
Pneumonia, tumor noted on x-ray study
9
Aletti and Balbo1 1963
18 M
Rib, 6th posterior axillary line
Unknown
\Vi
Salvini12 1963
11 M
Ribs 4, 5 and 6, left sterum Tumor costal junction
Safar« 1964 Odom, DeMuth, & Blakemore 1965
3 Mo. Ribs, right upper lateral chest M 16 M
Ribs 8 and 9, left anterior axillary line
Microscopic. Sections of chest wall tumor mass reveals the neoplastic cell to be cartilaginous. In some areas a typical hyaline pattern prevails, but in others the nucleus forms crescent and fibrillary shapes. There is also some blending with spindleshaped connective tissue fibers. Many sections across tumor-intercostal muscle junctions reveal in vasion of the fascia in but one instance. The calcified areas are characteristic of reaction to hemorrhage, rather than a neoplastic process. There is no extension into adjacent costal bones (Fig. 4). Diagnosis. Chondrosarcoma of chest wall. Discussion
One of the earliest series of primary tu mors of the ribs and sternum was reported by Paget in 1897.7 From the literature and his own experience he collected 33 cases, 24 of which were malignant. Hedblom2 re ported large series of malignant tumors of the thoracic wall in 1921 and 1933. In the latter series he collected 291 such cases from the literature and his personal experi ence, but he did not present specific cases in detail. At that time chondrosarcomas
3 da. 12
Unknown
Unknown
4
Tumor, pain
1
were usually classified in a group with osteogenic sarcomas. This lack of distinction tends to invalidate some early reports in which microscopical documentation is lack ing. In 1930, Phemister9 stressed the im portance of separating these dissimilar le sions as pathological entities. Lichtensten"' and Jaffe3 later described the detailed char acteristics of chondrosarcoma which are ac cepted by most pathologists today. Lack of information in classification in earlier litera ture and insufficient documentation in some reports make a thorough review of the literature impossible. These obstacles not withstanding, we have made an attempt to tabulate (Table I) those instances of chon drosarcoma in childhood and adolescence where little doubt exists concerning the na ture of the lesion. Including our own, 8 such patients have been recorded. They range in age from 3 months to 18 years. All are males. The long delays in diagnosis, up to 12 years in one instance, probably stem from the fact that these tumors
Volume 50
Chest wall chondrosarcoma
Number 4
553
October, 1965
Treatment
Local recurrence
Metastasis Lung
Survival after treatment
Survival after Cause of onset of death symptoms
9 mo.
2 yr. 9 mo. Metastasis to lung
6 wk.
6!/2 wk.
Only large external mass removed
None
Biopsy only
Not applicable Unknown
1. 2. 3. 4.
Yes
16 yr. To spine 5 mo. (paraplegic, 50 mo. before death)
1. Block resection of 4th rib 2. 20 radiological treatments
None
None
Infiltration of lung and diaphragm; inoperable
Unknown
Local extension Discharged on Unknown 25th day
1. Biopsy 2. Cobalt therapy
Not applicable Unknown
7 mo.
Surgical resection
None
None
4 yr.
8 yr.
Alive
Surgical resection
None
None
8 mo.
lVi yr.
Alive
Removed with osteotone Removed piecemeal 1 yr. later Nodule removed 5 yr. later Removed from sternum & muscle
do not usually cause pain or disability. Valid statistical information cannot be obtained from experience with this small group of patients. The much more extensive literature on chondrosarcoma in all age groups in all locations would indicate that wide surgical excision is preferred to all other methods of treatment. Though locally aggressive, chondrosarcoma metastasizes late, and, consequently, cures after re-exci sion are not rare. Enormous tumors, often thought initially to be beyond cure, have been resected with apparent cure. Despite this favorable feature in the group of 8 pa tients tabulated, optimal therapy was ap plied only three times. Although follow-up is much too short to determine the final out come in these three instances, no recurrence has been noted up to the time of writing. There have undoubtedly been occasions where radical excision of large segments of the chest wall were not carried out because of fear of the respiratory embarrassment to be created. Chest wall defects have been
6 yr.
Unknown
28 yr. 5 mo.
Unknown
15 yr.
Alive
Unknown
Unknown Unknown
managed in a variety of ways. Extensive consideration of this problem is beyond the purview of this discussion, suffice to say that prosthetic substances have appreciably sim plified the problem. We used Marlex mesh, the synthetic plastic popularized by Usher,13 and a growing experience with this sub stance would indicate that it is well tolerated by the host, withstands stress well, and be comes incorporated into connective tissue with a minimal of reaction. Irradiation has not been very successful in the treatment of chondrosarcoma, and it should not be employed as a primary method of treatment where the possibility of cure exists. We have no knowledge of extensive use of chemotherapeutic agents in the treatment of advanced chondrosarcoma. Summary Chondrosarcoma of the chest wall is rarely encountered in youth. Experience with a 17-year-old boy having this lesion is recorded. Seven additional instances are tab-
Journal of 5 5 4
Oàom, DeMuth,
Blakemore
Thoracic and Cardiovascular Surgery
ulated. Wide excision is the preferred treat ment. REFERENCES 1 Aletti, L., and Balbo, G . : Studio anatomoclinico di tredici casi di tumor: Castilagirrei della parete thoracica, Cancro 16: 465-495, 1963. 2 Hedblom, C. A.: Tumors of the Boney Chest Wall, Ann. Surg. 98: 528, 1933. 3 Jafïe, H. L.: Tumors and Tumorous Conditions of the Bones and Joints, Philadelphia, 1958, Lea & Febiger, pp. 314-340. 4 Janes, R . M.: Tumors of the Thoracic Cage, A m . J. Surg. 54: 127-138, 1941. 5 Lichtensten, L.: Bone Tumors, ed. 2, St. Louis, 1959, The C. V. Mosby Co., pp. 163-190. 6 O'Neal, L. W., and Ackerman, L. V.: Carti laginous Tumors of Ribs and Sternum, J. THORACIC SURC. 2 1 : 7 1 , 1951.
7 Paget, S.: Surgery of the Chest, New York, Tret, 1897.
8 Pascuzzi, C. A., Dahlin, D . C , and Clagett, O. T.: Primary Tumors of the Ribs and Sternum, Surg., Gynec. & Obst. 104: 390-400, 1957. 9 Phemister, D . B.: Chondrosarcoma of Bone, Surg., Gynec. & Obst. 50: 216-233, 1930. 10 Polk, J. W., Bailey, A. H., Gregoriades, D. G., and Buckingham, W. W.: Malignant Lesions of the Chest Wall, Missouri Med. 58: 217, 1961. 11 Safar, J.: Chondrosarcoma of the Chest Wall in a 3-Month-Old Infant With Prolonged Sur vival, Cesk. Pediat. X I X 5: 422-424, 1964. 12 Sal vin i, E.: I tumori primitivi delle coste; Radiol. Med. 49: 721-756, 1963. 13 Usher, F . C , Fries, J. G., Oschner, J. L., and Tuttle, L . L. D., Jr.: Marlex Mesh, A New Plastic Mesh for Replacing Tissue Defects: Clinical Studies, A. M. A. Arch. Surg. 78: 138, 1959. 14 Watkins, E., Jr., and Gerard, F . P.: Malignant Tumors Involving the Chest Wall, J. of THORACIC
1960.
& CARDIOVAS.
SURG.
39: 117-129,
M.D.,
Carlisle, Pa., and William S. Blakemore, M.D., Philadelphia, Pa.
Y^hondrosarcoma of the chest wall in the first two decades of life is rare. Indeed, malignant tumors of all types are very rarely encountered in the thoracic wall in this age group. Watkins and Gerard14 found only two malignant tumors of the chest wall in the experience gained during a period of 20 years at the Lahey Clinic. In 1957, Pascuzzi and associates8 reported one of the largest series ever recorded, 144 tumors of the ribs and sternum, and no patient under 25 years of age was encountered. Because of the infrequency with which chondrosarcoma of the thoracic wall is en countered in youth, our experience with such a tumor found in a boy 17 years of age is being reported. Case report D. R. (A-57955), a 17-year-old white boy, was admitted to the hospital on Aug. 6, 1964. He had noticed a mass on the left chest wall for approxi mately 1 year before he consulted a physician. The mass had increased only slightly in size and had never been painful, although he frequently bumped the mass which annoyed him. He had noticed no weight loss, dyspnea, limitation of activity, anorexia, or malaise. The past medical history of the patient was noncontributory. Physical examination. The blood pressure was 110/80 mm. Hg; pulse 64; respirations 20; tem-
From the Department of Surgery, Graduate Hospital of the University of Pennsylvania and the Harrison De partment of Surgical Research, Schools of Medicine, University of Pennsylvania, Philadelphia, Pa., and Carlisle Hospital, Carlisle, Pa. Received for publication June 3, 1965. ♦Clinical Fellow, American Cancer Society.
550
perature 97.4° F. A firm nontender mass, adherent to the left lower thoracic wall in the anterior axillary line, measured 2 by 1.5 by 1.0 inches. No skin discoloration was present. The lungs were clear to auscultation and percussion. The remain ing findings during the physical examination were within normal limits. Radiography. Roentgenograms of the chest (Fig. 1) revealed an amorphous diffuse calcification in a soft tissue mass between the anterior ends of the eighth and ninth ribs on the left side. On the basis of these findings a diagnosis of chondro sarcoma was made. Operation. An oblique incision over the mass was made and a small ellipse of skin was taken with the mass which was excised en bloc. The excision included at least 2 centimeters of grossly normal tissue on all sides of the tumor. The lesion was found to involve intercostal muscles, ribs, parietal pleura, and the adjacent diaphragm was adherent to it. The tumor extended about 5 cm. within the thoracic cavity. This deep involvement was unexpected as it was not detected on x-ray study. The mass was excised in one piece, with the adherent diaphragm taken with it and exposure of the peritoneal cavity. The 8 by 10 cm. defect in the diaphragm was closed with interrupted sutures of Mersilene. Marlex mesh was used to cover the defect in the rib cage which measured 15 by 20 cm. A large rubber tube drain was placed through a stab wound posterior to the defect and attached to suction, and a routine wound closure of sub cutaneous tissue and skin was completed. Course in hospital and follow-up. On the third postoperative day the chest tube was removed. The convalescence was uneventful and the patient was discharged on the fifth postoperative day. The wound healed primarily (Fig. 2). Four months later the patient was noted to have some motion in the defect on respiration. The chest wall defect was firm at this time, and the patient had no limitation of activity. On March 30, 1965, chest roentgenograms demonstrated no evidence of local recurrence or pulmonary me-
Volume 50 Number 4
Chest wall chondrosarcoma 5 5 1
October, 1965
Fig. 3. Gross specimen demonstrates involvement of ribs and thoracic wall. The involved diaphragm is not clearly demonstrated.
Fig. 1. Roentgenogram demonstrates calcification in tumor.
Fig. 2. Photograph which shows area of excision in left anterolateral chest wall. tastasis, and the patient was clinically well and free of any evidence of residual neoplasm. No respiratory paradox was present. Pathology. The gross specimen (Fig. 3) con sisted of the tumor mass, portions of four ribs with their costochondral junctions, and the portion of excised diaphragm. The over-all dimensions of the mass were 12 by 11 by 10 cm. The major portion of the soft tissue mass projected beneath the parietal pleura and the leaf of the diaphragm. This portion extended 8 cm. into the chest cavity
Fig. 4. Photomicrograph which demonstrates char acteristic features of chondrosarcoma (xllO). from its wall. It bulged between and separated the two most central parts of the adjacent ribs. The tu mor was firm and cut as if cartilaginous. It was colorless, glistening, encapsulated, and contained some areas of hemorrhagic necrosis. Focal areas of calification and some areas of ossification were present. It did not appear to invade bone.
Journal of
5 52
Odom,
DeMuth,
Blakemore
Thoracic and Cardiovascular Surgery
Table I
Author
Age of onset and sex
Location
Symptoms
Duration of Symptom (yr.) 2
Phemister9 1930
18 M
Ribs 2, 3, 4, 5, 6 & 7, right Slight pain, tumor anterior
Janes4 1941
16 M
Ribs 9 and 10, left posterior Dry cough, pleural pain, shortness of breath
O'Neal and Ackerman6 1951
11 M
Manubrium
Tumor
Polk et al.30 1961
5 M
Rib, 4th left lateral
Pneumonia, tumor noted on x-ray study
9
Aletti and Balbo1 1963
18 M
Rib, 6th posterior axillary line
Unknown
\Vi
Salvini12 1963
11 M
Ribs 4, 5 and 6, left sterum Tumor costal junction
Safar« 1964 Odom, DeMuth, & Blakemore 1965
3 Mo. Ribs, right upper lateral chest M 16 M
Ribs 8 and 9, left anterior axillary line
Microscopic. Sections of chest wall tumor mass reveals the neoplastic cell to be cartilaginous. In some areas a typical hyaline pattern prevails, but in others the nucleus forms crescent and fibrillary shapes. There is also some blending with spindleshaped connective tissue fibers. Many sections across tumor-intercostal muscle junctions reveal in vasion of the fascia in but one instance. The calcified areas are characteristic of reaction to hemorrhage, rather than a neoplastic process. There is no extension into adjacent costal bones (Fig. 4). Diagnosis. Chondrosarcoma of chest wall. Discussion
One of the earliest series of primary tu mors of the ribs and sternum was reported by Paget in 1897.7 From the literature and his own experience he collected 33 cases, 24 of which were malignant. Hedblom2 re ported large series of malignant tumors of the thoracic wall in 1921 and 1933. In the latter series he collected 291 such cases from the literature and his personal experi ence, but he did not present specific cases in detail. At that time chondrosarcomas
3 da. 12
Unknown
Unknown
4
Tumor, pain
1
were usually classified in a group with osteogenic sarcomas. This lack of distinction tends to invalidate some early reports in which microscopical documentation is lack ing. In 1930, Phemister9 stressed the im portance of separating these dissimilar le sions as pathological entities. Lichtensten"' and Jaffe3 later described the detailed char acteristics of chondrosarcoma which are ac cepted by most pathologists today. Lack of information in classification in earlier litera ture and insufficient documentation in some reports make a thorough review of the literature impossible. These obstacles not withstanding, we have made an attempt to tabulate (Table I) those instances of chon drosarcoma in childhood and adolescence where little doubt exists concerning the na ture of the lesion. Including our own, 8 such patients have been recorded. They range in age from 3 months to 18 years. All are males. The long delays in diagnosis, up to 12 years in one instance, probably stem from the fact that these tumors
Volume 50
Chest wall chondrosarcoma
Number 4
553
October, 1965
Treatment
Local recurrence
Metastasis Lung
Survival after treatment
Survival after Cause of onset of death symptoms
9 mo.
2 yr. 9 mo. Metastasis to lung
6 wk.
6!/2 wk.
Only large external mass removed
None
Biopsy only
Not applicable Unknown
1. 2. 3. 4.
Yes
16 yr. To spine 5 mo. (paraplegic, 50 mo. before death)
1. Block resection of 4th rib 2. 20 radiological treatments
None
None
Infiltration of lung and diaphragm; inoperable
Unknown
Local extension Discharged on Unknown 25th day
1. Biopsy 2. Cobalt therapy
Not applicable Unknown
7 mo.
Surgical resection
None
None
4 yr.
8 yr.
Alive
Surgical resection
None
None
8 mo.
lVi yr.
Alive
Removed with osteotone Removed piecemeal 1 yr. later Nodule removed 5 yr. later Removed from sternum & muscle
do not usually cause pain or disability. Valid statistical information cannot be obtained from experience with this small group of patients. The much more extensive literature on chondrosarcoma in all age groups in all locations would indicate that wide surgical excision is preferred to all other methods of treatment. Though locally aggressive, chondrosarcoma metastasizes late, and, consequently, cures after re-exci sion are not rare. Enormous tumors, often thought initially to be beyond cure, have been resected with apparent cure. Despite this favorable feature in the group of 8 pa tients tabulated, optimal therapy was ap plied only three times. Although follow-up is much too short to determine the final out come in these three instances, no recurrence has been noted up to the time of writing. There have undoubtedly been occasions where radical excision of large segments of the chest wall were not carried out because of fear of the respiratory embarrassment to be created. Chest wall defects have been
6 yr.
Unknown
28 yr. 5 mo.
Unknown
15 yr.
Alive
Unknown
Unknown Unknown
managed in a variety of ways. Extensive consideration of this problem is beyond the purview of this discussion, suffice to say that prosthetic substances have appreciably sim plified the problem. We used Marlex mesh, the synthetic plastic popularized by Usher,13 and a growing experience with this sub stance would indicate that it is well tolerated by the host, withstands stress well, and be comes incorporated into connective tissue with a minimal of reaction. Irradiation has not been very successful in the treatment of chondrosarcoma, and it should not be employed as a primary method of treatment where the possibility of cure exists. We have no knowledge of extensive use of chemotherapeutic agents in the treatment of advanced chondrosarcoma. Summary Chondrosarcoma of the chest wall is rarely encountered in youth. Experience with a 17-year-old boy having this lesion is recorded. Seven additional instances are tab-
Journal of 5 5 4
Oàom, DeMuth,
Blakemore
Thoracic and Cardiovascular Surgery
ulated. Wide excision is the preferred treat ment. REFERENCES 1 Aletti, L., and Balbo, G . : Studio anatomoclinico di tredici casi di tumor: Castilagirrei della parete thoracica, Cancro 16: 465-495, 1963. 2 Hedblom, C. A.: Tumors of the Boney Chest Wall, Ann. Surg. 98: 528, 1933. 3 Jafïe, H. L.: Tumors and Tumorous Conditions of the Bones and Joints, Philadelphia, 1958, Lea & Febiger, pp. 314-340. 4 Janes, R . M.: Tumors of the Thoracic Cage, A m . J. Surg. 54: 127-138, 1941. 5 Lichtensten, L.: Bone Tumors, ed. 2, St. Louis, 1959, The C. V. Mosby Co., pp. 163-190. 6 O'Neal, L. W., and Ackerman, L. V.: Carti laginous Tumors of Ribs and Sternum, J. THORACIC SURC. 2 1 : 7 1 , 1951.
7 Paget, S.: Surgery of the Chest, New York, Tret, 1897.
8 Pascuzzi, C. A., Dahlin, D . C , and Clagett, O. T.: Primary Tumors of the Ribs and Sternum, Surg., Gynec. & Obst. 104: 390-400, 1957. 9 Phemister, D . B.: Chondrosarcoma of Bone, Surg., Gynec. & Obst. 50: 216-233, 1930. 10 Polk, J. W., Bailey, A. H., Gregoriades, D. G., and Buckingham, W. W.: Malignant Lesions of the Chest Wall, Missouri Med. 58: 217, 1961. 11 Safar, J.: Chondrosarcoma of the Chest Wall in a 3-Month-Old Infant With Prolonged Sur vival, Cesk. Pediat. X I X 5: 422-424, 1964. 12 Sal vin i, E.: I tumori primitivi delle coste; Radiol. Med. 49: 721-756, 1963. 13 Usher, F . C , Fries, J. G., Oschner, J. L., and Tuttle, L . L. D., Jr.: Marlex Mesh, A New Plastic Mesh for Replacing Tissue Defects: Clinical Studies, A. M. A. Arch. Surg. 78: 138, 1959. 14 Watkins, E., Jr., and Gerard, F . P.: Malignant Tumors Involving the Chest Wall, J. of THORACIC
1960.
& CARDIOVAS.
SURG.
39: 117-129,