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Chondrosarcoma of the trachea
Chondrosarcoma of the trachea Report of a case and five-year follow-up M. Fallahnejad, M.D., D. Harrell, M.D., I. Tucker, M.D., * I. Forest, M.D., and W. S. Blakemore, M.D., Philadelphia, Pa.
A
review of the literature reveals only two reported cases of chondrosarcoma of the trachea.t> We are not aware of any previous report of surgical resection with follow-up results. Case report A 48-year-old woman was first seen at the Graduate Hospital of the University of Pennsylvania in March, 1967, for evaluation of bronchitis and asthma. Her symptoms began in December, 1965, with a deep, nonproductive cough and an audible wheeze. In October, 1966, she underwent a total abdominal hysterectomy. Postoperatively, she developed severe dyspnea and was placed on oxygen therapy. Subsequently she was referred to an allergist, and a course of medical therapy and desensitization was instituted. Her symptoms progressed with minimal response to treatment, and she experienced gradual development of dysphonia and weight loss. The dyspnea became disabling, and she spent most of her day in bed. Pertinent physical findings on admission included acute respiratory distress with labored breathing, intermittent stridor, and a deep, resonant cough (compression cough). Examination of the chest revealed inspiratory and expiratory bronchi throughout both lungs. Examination of the neck revealed a 5 by 3.5 em., soft, irregular, nontender mass in the suprasternal space which was intimately adherent to the trachea. It could be moved in the sagittal direction and with deglutition. There were no other significant physical From the Graduate Hospital, University of Pennsylvania. 19th and Lombard Streets, Philadelphia, Pa. 19146. Received for publication JUly 19, 1972. 'Hospital of the University of Pennsylvania, 34th and Spruce Streets, Philadelphia, Pa.
210
findings, and routine laboratory studies were entirely normal. A chest roentgenogram revealed normal lung fields, but the lateral view of the neck showed marked indentation of the anterior aspect of the trachea starting at the level of the first thoracic vertebra and extending 4 em. (Fig. I), with displacement of the trachea and esophagus. On direct laryngoscopy with a twin-lighted laryngoscope, an intrusion of the anterior wall of the trachea, which almost completely occluded the lumen, was noted just beyond the larynx. The lesion was approximately 3.5 em. in length and was covered with epithelium. It involved only the anterior wall; the posterior wall and the remainder of the trachea were normal. Uptake at the level of the third thoracic vertebra was below normal (I8 per cent). A thyroid scanogram showed that the gland was of normal size but had a 3 by 2 em., oval-shaped area in the right lower pole where uptake was spotty. In April, 1967, under general anesthesia, a transverse cervical incision which extended over the sternocleidomastoid muscles was made. The skin flap on the subplatysmal layer was elevated to the level of the thyroid bone, and the strap muscles were incised and retracted. The thyroid was normal. The tumor was seen to arise from the trachea. The thyroid isthmus was clamped and divided, and the trachea was exposed by dissection into the mediastinum. The tumor was 4 by 3 cm. and was soft and irregular with multiple lobulations. It extended from thyroid cartilage to the level of the sternal notch. A frozen-section biopsy of this mass showed an inflammatory change, which was subsequently seen to be necrotic cartilage. Initial finger dissection paratracheally into the mediastinum and around the carina provided good mobilization of the distal trachea. Division of the hyoid bone permitted the larynx to be mobilized.
Volume 65 Number 2
Chondrosarcoma of trachea
211
Februory, 1973
A number of heavy traction sutures were applied to the trachea cephalad and caudad to the lesion. A 9 em. segment of trachea (seven cartilaginous rings) was resected in a semicircumferential fashion. The posterior wall of the trachea was left intact. Then, by division of the hyoid bone, a laryngeal drop was accomplished. Attempts to approximate the tracheal edges by traction sutures left a small gap which was closed by flexion of the neck. An end-to-end closure was performed outside the mucosa with one layer of No. 30 wire sutures. A tracheostomy tube was inserted through the opening in the cricothyroid membrane, and the endotracheal tube was removed. Closure of the wound, with the insertion of a drain, was routine. A splint was applied in order to maintain the neck in maximal flexion. Postoperatively, the patient was kept in a high-humidity oxygen tent with a steam collar for the tracheostom y. She had an uneventful postoperative course. The course of her recovery was followed by endoscopic examination. The tracheostomy tube was removed before discharge at the fifth postoperative week. The bronchoscopic examination at this time showed an adequate tracheal lumen with some granulation of tissue at the suture line (Fig. 2). On pathologic examination, the lesion was composed of some cartilage rings and lobulated, pale brown, firm tissue which contained cartilaginous material. Microscopically, this proved to consist of a slightly myxomatous , lobulated growth containing cartilage cells irregularly distributed in a matrix in which there were varying degrees of differentiation. The growth clearly arose from the cartilage rings and extended into the tracheal mucosa. It was considered to be a chondrosarcoma (Fig. 3). After discharge, the patient was followed by periodic clinical evaluations, x-ray examinations, and bronchoscopy . For 5 years she has continued to do well with no recurrence or metastases and has been able to return to her normal work (Fig. 4).
Fig. 1. Lateral view of the neck with abduction of the shoulder shows marked indentat ion of the trachea (arrow).
FIg. Z. A photograph obtained through a bronchoscope shows open bronchial lumen with granulation of tissue at the suture line.
Discussion
Malignant tumors of the trachea are rare. The material of the Mayo Clinic" revealed only 53 cases of primary malignancy of the trachea. There are 2 previously reported cases of chondrosarcoma of the trachea; in the latter instance, the patient was treated conservatively with palliative local endotracheal resection." In general a chondrosarcoma is a slowgrowing and confined lesion,' which makes surgical treatment an ideal therapeutic approach. Circular resection of the trachea was
described by Kuester' and von Eiselsberg" in the 1800's; however, the procedure was not fully recognized until many years later, when pioneer work by Belsey,' Crafoord," Griffith," Clagett, to and Jackson» established the foundation of surgical management of tracheal lesions. Except in rare instances, the experiences with extensive resection and replacement of the trachea have been discouraging. Various attempts to utilize free grafts with autologous supporting structures," synthetic ma-
2I2
Fallahnejad et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 3. A, Tumor arising from a tracheal ring. B, Another field shows variation in morphology. (A and B, hematoxylin and eosin; original magnification x60.)
Fig. 4. Posteroanterior chest x-ray film, obtained in April, 1972, after 5 years, shows normal bronchopulmonary configuration.
terial,"- 14 or biological substitutes have not yielded predictable results. Belsey,' in his classic contribution, describes four essential characteristics of a reconstructed airway: (l) lateral rigidity, (2) longitudinal elasticity and flexibility, (3) an adequate and airtight lumen, and (4) an uninterrupted lining of ciliated columnar epithelium. At the present time, end-to-end anastomosis of the trachea is the only procedure that will adequately fulfill these criteria. Anatomic limitations and technical problems with regard to an adequate airway and blood supply and the avoidance of tension on the anastomotic line make major resection extremely difficult. The experimental and clinical work of Grillo" and Dedo-" has contributed significantly to the possibility of segmental resection and anastomosis of rather long segments of the trachea. Cardiopulmonary bypass combined with a sternal-splitting incision or thoracotomy, which has
Volume 65 Number 2 Februory, 1973
been utilized successfully," 18 is a helpful modality when necessary. In the present case we were fully prepared to utilize any of these modalities but found the following techniques most helpful: (I) blunt dissection of the trachea and upper bronchi from a generous collar incision; (2) laryngeal drop by means of division of the hyoid bone, as practiced by one of the authors (J. T.) with good results for many years; (3) a tracheostomy at the cricothyroid membrane; and (4) the additional length obtained by neck flexion. The last is a useful functional additive which has not been considered in studies done on cadavers. Finally, regarding the diagnostic standpoint, early recognition of these lesions would obviate many of the difficulties encountered in extensive resection. Jackson's'! aphorism "all is not asthma that wheezes" bears remembrance.
Summary This report gives the case history of a patient who had a successful resection of a chondrosarcoma of the trachea 5 years ago. Technical aspects of the procedure and follow-up results are presented. REFERENCES Moersh, J. J., Clagett, O. T., and Ellis, F. H., Jr.: Tumors of the Trachea, Med. Clin. North Am. 38: 1091, 1954. 2 Daniels, A. C., Conner, G. H., and Straus, F. H.: Primary Chondrosarcoma of the Tracheobronchial Tree: Report of a Case and Brief Review, Arch. Pathol. 84: 615, 1967. 3 Houston, H. E., Payne, W. S., Harrison, E., Jr., and Olsen, A. M.: Primary Cancers of the Trachea, Arch. Surg. 99: 132, 1969. 4 Goethals, P. L., Dahlin, D. C., and Devine, K. D.: Cartilaginous Tumors of the Larynx, Surg. Gynecol, Obstet. 117: 77, 1963.
Chondrosarcoma of trachea
2 13
5 Kuester, E.: Vorstellung eines Patienten bei welchen des halbe kenlkopf extirpirt worden ist, Verhandl. Dtsch. Gesellsch. Chir. 13: 95, 1884. 6 Von Eiselsberg, A.: Zur Resection und Naht der Trachea, Dtsch. Med. Wochenschtr. 22: 343, 1896. 7 Belsey, R.: Resection and Reconstruction of the Intrathoracic Trachea, Br. J. Surg, 38: 200, 1950. 8 Crafoord, C., and Eindgren, A. G. H.: Mucous and Salivary Gland Tumors in the Bronchi and Trachea, Formerly Generally Called Nomata, Acta Chir. Scand. 92: 481, 1945. 9 Griffith, J. L.: Fracture of the Bronchus, Thorax 4: 105, 1949. 10 Clagett, O. T., and Moersh, H. J.: Intrathoracic Tracheal Tumors: Development of Surgical Technics for Their Removal, Ann. Surg. 136: 520, 1952. 11 Jackson, C., and Jackson, C. L.: Disease of the Nose, Throat and Ear, Philadelphia, 1959, W. B. Saunders Company. 12 Fonkalsrud, E. W., and Plested, W. G.: Tracheobronchial Reconstruction With Autologous Periosteum, J. THORAC. CARDIOVASC. SURG. 52: 666, 1966. 13 Ekestrom, S., and Ense, C.: Teflon Prosthesis in Tracheal Defect in Man, Acta Chir. Scand. 245: 71, 1959 (Suppl.). 14 Graziano, J.: Prosthetic Replacement of Trachea, Ann. Thorac. Surg. 4: I, 1967. 15 Grillo, H. C., Dignan, E. F., and Miura, T.: Extensive Resection and Reconstruction of Mediastinal Trachea Without Prosthesis or Graft: An Anatomical Study in Man, J. THoRAC. CARDIOVASC. SURG. 48: 741, 1964. 16 Dedo, H. H., and Fishman, N. H.: Laryngeal Release and Sleeve Resection for Tracheal Stenosis, Ann. Otol. Rhinol. Laryngol. 78: 285, 1969. 17 Woods, F. M., Neptune, W. B., and Palatchi, A.: Resection of Carina and Main Stem Bronchus With the Use of Extracorporeal Circulation, N. Engl. J. Med. 264: 492, 1961. 18 Mathey, J., Binet, J. P., and Galey, J. J.: Tracheal and Tracheobronchial Resections, J. THORAC. CARDIOVASC. SURG. 51: 1, 1966.
A
review of the literature reveals only two reported cases of chondrosarcoma of the trachea.t> We are not aware of any previous report of surgical resection with follow-up results. Case report A 48-year-old woman was first seen at the Graduate Hospital of the University of Pennsylvania in March, 1967, for evaluation of bronchitis and asthma. Her symptoms began in December, 1965, with a deep, nonproductive cough and an audible wheeze. In October, 1966, she underwent a total abdominal hysterectomy. Postoperatively, she developed severe dyspnea and was placed on oxygen therapy. Subsequently she was referred to an allergist, and a course of medical therapy and desensitization was instituted. Her symptoms progressed with minimal response to treatment, and she experienced gradual development of dysphonia and weight loss. The dyspnea became disabling, and she spent most of her day in bed. Pertinent physical findings on admission included acute respiratory distress with labored breathing, intermittent stridor, and a deep, resonant cough (compression cough). Examination of the chest revealed inspiratory and expiratory bronchi throughout both lungs. Examination of the neck revealed a 5 by 3.5 em., soft, irregular, nontender mass in the suprasternal space which was intimately adherent to the trachea. It could be moved in the sagittal direction and with deglutition. There were no other significant physical From the Graduate Hospital, University of Pennsylvania. 19th and Lombard Streets, Philadelphia, Pa. 19146. Received for publication JUly 19, 1972. 'Hospital of the University of Pennsylvania, 34th and Spruce Streets, Philadelphia, Pa.
210
findings, and routine laboratory studies were entirely normal. A chest roentgenogram revealed normal lung fields, but the lateral view of the neck showed marked indentation of the anterior aspect of the trachea starting at the level of the first thoracic vertebra and extending 4 em. (Fig. I), with displacement of the trachea and esophagus. On direct laryngoscopy with a twin-lighted laryngoscope, an intrusion of the anterior wall of the trachea, which almost completely occluded the lumen, was noted just beyond the larynx. The lesion was approximately 3.5 em. in length and was covered with epithelium. It involved only the anterior wall; the posterior wall and the remainder of the trachea were normal. Uptake at the level of the third thoracic vertebra was below normal (I8 per cent). A thyroid scanogram showed that the gland was of normal size but had a 3 by 2 em., oval-shaped area in the right lower pole where uptake was spotty. In April, 1967, under general anesthesia, a transverse cervical incision which extended over the sternocleidomastoid muscles was made. The skin flap on the subplatysmal layer was elevated to the level of the thyroid bone, and the strap muscles were incised and retracted. The thyroid was normal. The tumor was seen to arise from the trachea. The thyroid isthmus was clamped and divided, and the trachea was exposed by dissection into the mediastinum. The tumor was 4 by 3 cm. and was soft and irregular with multiple lobulations. It extended from thyroid cartilage to the level of the sternal notch. A frozen-section biopsy of this mass showed an inflammatory change, which was subsequently seen to be necrotic cartilage. Initial finger dissection paratracheally into the mediastinum and around the carina provided good mobilization of the distal trachea. Division of the hyoid bone permitted the larynx to be mobilized.
Volume 65 Number 2
Chondrosarcoma of trachea
211
Februory, 1973
A number of heavy traction sutures were applied to the trachea cephalad and caudad to the lesion. A 9 em. segment of trachea (seven cartilaginous rings) was resected in a semicircumferential fashion. The posterior wall of the trachea was left intact. Then, by division of the hyoid bone, a laryngeal drop was accomplished. Attempts to approximate the tracheal edges by traction sutures left a small gap which was closed by flexion of the neck. An end-to-end closure was performed outside the mucosa with one layer of No. 30 wire sutures. A tracheostomy tube was inserted through the opening in the cricothyroid membrane, and the endotracheal tube was removed. Closure of the wound, with the insertion of a drain, was routine. A splint was applied in order to maintain the neck in maximal flexion. Postoperatively, the patient was kept in a high-humidity oxygen tent with a steam collar for the tracheostom y. She had an uneventful postoperative course. The course of her recovery was followed by endoscopic examination. The tracheostomy tube was removed before discharge at the fifth postoperative week. The bronchoscopic examination at this time showed an adequate tracheal lumen with some granulation of tissue at the suture line (Fig. 2). On pathologic examination, the lesion was composed of some cartilage rings and lobulated, pale brown, firm tissue which contained cartilaginous material. Microscopically, this proved to consist of a slightly myxomatous , lobulated growth containing cartilage cells irregularly distributed in a matrix in which there were varying degrees of differentiation. The growth clearly arose from the cartilage rings and extended into the tracheal mucosa. It was considered to be a chondrosarcoma (Fig. 3). After discharge, the patient was followed by periodic clinical evaluations, x-ray examinations, and bronchoscopy . For 5 years she has continued to do well with no recurrence or metastases and has been able to return to her normal work (Fig. 4).
Fig. 1. Lateral view of the neck with abduction of the shoulder shows marked indentat ion of the trachea (arrow).
FIg. Z. A photograph obtained through a bronchoscope shows open bronchial lumen with granulation of tissue at the suture line.
Discussion
Malignant tumors of the trachea are rare. The material of the Mayo Clinic" revealed only 53 cases of primary malignancy of the trachea. There are 2 previously reported cases of chondrosarcoma of the trachea; in the latter instance, the patient was treated conservatively with palliative local endotracheal resection." In general a chondrosarcoma is a slowgrowing and confined lesion,' which makes surgical treatment an ideal therapeutic approach. Circular resection of the trachea was
described by Kuester' and von Eiselsberg" in the 1800's; however, the procedure was not fully recognized until many years later, when pioneer work by Belsey,' Crafoord," Griffith," Clagett, to and Jackson» established the foundation of surgical management of tracheal lesions. Except in rare instances, the experiences with extensive resection and replacement of the trachea have been discouraging. Various attempts to utilize free grafts with autologous supporting structures," synthetic ma-
2I2
Fallahnejad et al.
The Journal of Thoracic and Cardiovascular Surgery
Fig. 3. A, Tumor arising from a tracheal ring. B, Another field shows variation in morphology. (A and B, hematoxylin and eosin; original magnification x60.)
Fig. 4. Posteroanterior chest x-ray film, obtained in April, 1972, after 5 years, shows normal bronchopulmonary configuration.
terial,"- 14 or biological substitutes have not yielded predictable results. Belsey,' in his classic contribution, describes four essential characteristics of a reconstructed airway: (l) lateral rigidity, (2) longitudinal elasticity and flexibility, (3) an adequate and airtight lumen, and (4) an uninterrupted lining of ciliated columnar epithelium. At the present time, end-to-end anastomosis of the trachea is the only procedure that will adequately fulfill these criteria. Anatomic limitations and technical problems with regard to an adequate airway and blood supply and the avoidance of tension on the anastomotic line make major resection extremely difficult. The experimental and clinical work of Grillo" and Dedo-" has contributed significantly to the possibility of segmental resection and anastomosis of rather long segments of the trachea. Cardiopulmonary bypass combined with a sternal-splitting incision or thoracotomy, which has
Volume 65 Number 2 Februory, 1973
been utilized successfully," 18 is a helpful modality when necessary. In the present case we were fully prepared to utilize any of these modalities but found the following techniques most helpful: (I) blunt dissection of the trachea and upper bronchi from a generous collar incision; (2) laryngeal drop by means of division of the hyoid bone, as practiced by one of the authors (J. T.) with good results for many years; (3) a tracheostomy at the cricothyroid membrane; and (4) the additional length obtained by neck flexion. The last is a useful functional additive which has not been considered in studies done on cadavers. Finally, regarding the diagnostic standpoint, early recognition of these lesions would obviate many of the difficulties encountered in extensive resection. Jackson's'! aphorism "all is not asthma that wheezes" bears remembrance.
Summary This report gives the case history of a patient who had a successful resection of a chondrosarcoma of the trachea 5 years ago. Technical aspects of the procedure and follow-up results are presented. REFERENCES Moersh, J. J., Clagett, O. T., and Ellis, F. H., Jr.: Tumors of the Trachea, Med. Clin. North Am. 38: 1091, 1954. 2 Daniels, A. C., Conner, G. H., and Straus, F. H.: Primary Chondrosarcoma of the Tracheobronchial Tree: Report of a Case and Brief Review, Arch. Pathol. 84: 615, 1967. 3 Houston, H. E., Payne, W. S., Harrison, E., Jr., and Olsen, A. M.: Primary Cancers of the Trachea, Arch. Surg. 99: 132, 1969. 4 Goethals, P. L., Dahlin, D. C., and Devine, K. D.: Cartilaginous Tumors of the Larynx, Surg. Gynecol, Obstet. 117: 77, 1963.
Chondrosarcoma of trachea
2 13
5 Kuester, E.: Vorstellung eines Patienten bei welchen des halbe kenlkopf extirpirt worden ist, Verhandl. Dtsch. Gesellsch. Chir. 13: 95, 1884. 6 Von Eiselsberg, A.: Zur Resection und Naht der Trachea, Dtsch. Med. Wochenschtr. 22: 343, 1896. 7 Belsey, R.: Resection and Reconstruction of the Intrathoracic Trachea, Br. J. Surg, 38: 200, 1950. 8 Crafoord, C., and Eindgren, A. G. H.: Mucous and Salivary Gland Tumors in the Bronchi and Trachea, Formerly Generally Called Nomata, Acta Chir. Scand. 92: 481, 1945. 9 Griffith, J. L.: Fracture of the Bronchus, Thorax 4: 105, 1949. 10 Clagett, O. T., and Moersh, H. J.: Intrathoracic Tracheal Tumors: Development of Surgical Technics for Their Removal, Ann. Surg. 136: 520, 1952. 11 Jackson, C., and Jackson, C. L.: Disease of the Nose, Throat and Ear, Philadelphia, 1959, W. B. Saunders Company. 12 Fonkalsrud, E. W., and Plested, W. G.: Tracheobronchial Reconstruction With Autologous Periosteum, J. THORAC. CARDIOVASC. SURG. 52: 666, 1966. 13 Ekestrom, S., and Ense, C.: Teflon Prosthesis in Tracheal Defect in Man, Acta Chir. Scand. 245: 71, 1959 (Suppl.). 14 Graziano, J.: Prosthetic Replacement of Trachea, Ann. Thorac. Surg. 4: I, 1967. 15 Grillo, H. C., Dignan, E. F., and Miura, T.: Extensive Resection and Reconstruction of Mediastinal Trachea Without Prosthesis or Graft: An Anatomical Study in Man, J. THoRAC. CARDIOVASC. SURG. 48: 741, 1964. 16 Dedo, H. H., and Fishman, N. H.: Laryngeal Release and Sleeve Resection for Tracheal Stenosis, Ann. Otol. Rhinol. Laryngol. 78: 285, 1969. 17 Woods, F. M., Neptune, W. B., and Palatchi, A.: Resection of Carina and Main Stem Bronchus With the Use of Extracorporeal Circulation, N. Engl. J. Med. 264: 492, 1961. 18 Mathey, J., Binet, J. P., and Galey, J. J.: Tracheal and Tracheobronchial Resections, J. THORAC. CARDIOVASC. SURG. 51: 1, 1966.