Primary Rhabdomyosarcoma

Transcription

1 CASE REPORTS Primary Rhabdomyosarcoma of the Bronchus Graham Fallon, M.D., Medad Schiller, M.D., and James W. Kilman, M.D. ABSTRACT A 6-year-old child with an unresectable rhabdomyosarcoma of the right main treated by irradiation and chemotherapy has survived 33 months following tissue diagnosis. Only 10 patients with pulmonary rhabdomyosarcoma have been reported previously. Early surgical management of the tumor appears to be the most effective means of therapy; the role of chemotherapy is not yet established. P rimary rhabdomyosarcoma of the is a rare pathological entity. The purpose of this presentation is to review the available literature and report the first known occurrence of this tumor type in the pediatric age group. A 6-year-old girl was referred to Children s Hospital in Columbus in September, 1967, with a radiologically documented atelectatic right upper lobe. A regimen of antibiotics and postural drainage had been initiated by the referring physician two weeks before admission, but the child s roentgenographic findings had remained unchanged. The history disclosed no previous evidence of chest pain, hemoptysis, weight loss, or dyspnea. Physical examination revealed a low-grade fever and decreased breath sounds over the right upper lobe. Bronchography (Fig. 1) demonstrated severe bronchiectatic changes involving the right lower lobe bronchi and absent filling of the right upper and middle lobes. Marked stenosis of the right main, extending from its origin down to the level of the intermediate, suggested a neoplastic process. Bronchoscopy confirmed the presence of a large, highly vascular neoplasm encroaching upon the lumen of the right From the Division of Thoracic and Cardiovascular Surgery, Department of Surgery, Ohio State University Hospitals, and Columbus Children s Hospital, Columbus, Ohio. Accepted for publication Nov. 30, Address reprint requests to Dr. Kilman, Division of Thoracic Surgery, Ohio State University Hospitals, 410 W. Tenth Ave., Columbus, Ohio THE ANNALS OF THORACIC SURGERY

2 CASE REPORT: Rhabdomyosarcoma of the Bronchus FIG. 1. Bronchogram demonstrating severe bronchiectatic changes involving the right lower lobe bronchi and invasion of the right main. main and projecting up to the margin of the carina. A biopsy was obtained, and histological examination of this tissue revealed embryonal rhabdom yosarcoma. A systematic clinical and radiological investigation failed to demonstrate a primary lesion outside the pulmonary system, and thoracotomy was subsequently performed with the hope that an excisional procedure would be possible. At operation a mass was found which projected from the right main into the right upper lobe. It was associated with multiple tumor nodules involving all segments of the right lung and was presumed to be metastatic. Palpable perihilar lymph nodes were present along the left main and beneath the carina. The tumor was thought to be unresectable, and the chest was closed. Postoperatively, the child received 3,500 R to the mediastinum and right upper lobe and was placed on a periodic regimen of actinomycin D and vincristine. Her condition has remained generally stable during the 33 months following tissue diagnosis, despite continued bronchoscopic and bronchographic evidence of tumor invasion. Path o 1 ogical Findings The surgical specimens obtained over a period of 16 months were fragments of soft tissue, either gray-white or dark red and hemorrhagic. Microscopically, respiratory epithelium was present on the surface, with underlying tumor cells. Papillations and projections were apparent, indicating the bot- VOL. 12, NO. 6, DECEMBER,

3 FALLON, SCHILLER, AND KlLMAN 2 3 FIG. 2. The tumor at high magnification, with respiratory epithelium at top, showing morphology of neoplastic cells, mitoses, and nuclear fragmentation. (H&E; X 730, before 45% reduction.) FIG. 3. Oil immersion photomicrograph of the tumor, demonstrating cytoplasmic crossstriations. (H&E; X 1830, before 45% reduction.) ryoid nature of the lesion. The tumor immediately underlying the epithelium was rather loose and myxoid. In deeper strata, however, the neoplasm was more densely populated with nuclei that were blunt, spindle-shaped, and dispersed in intertwining bundles (Fig. 2). Mitotic figures and nuclear fragments were numerous. Occasionally, cross-striations were seen in the cytoplasm of tumor cells (Fig. 3). A few multinucleated giant cells (not shown) were present in some areas. The background tissue consisted of a few connective tissue fibers but predominantly of cytoplasmic processes. The findings were believed to be compatible with an embryonal rhabdomyosarcoma of the. Comment Only 10 acceptably documented cases of primary rhabdomyosarcoma of the lung have appeared in the available world literature. An analysis of the information available from these and the present report is presented in the Table. Rhabdomyosarcomas are classified as embryonal, alveolar, and pleo- 652 THE ANNALS OF THORACIC SURGERY

4 p - N z 0 Q1 U R M El p SUMMARY OF DATA FROM REPORTED CASES OF PRIMARY RHABDOMYOSARCOMA OF THE LUNG Age (yrj Author & Sex Patient s Postmortem Location of Tumor Survival Examination Treatment (mo.) Performed McDonald 52, M Right lung 5 Yes & Heather El01 Forbes [41 Gordon & Boss 151 Kos teleck y & Stolz [81 Maschio [91 Bignardi [l] Drennan & McCormack [31 Steele 1111 Conquest & associates [21 Fallon & associates 68, M 20, F 48, M 64, M 60, F 56, M 68, M 53, M 55, M 6, F Left upper lobe Right main Left upper lobe Right lung Right lower lobe Left lung Left lower lobe Left lower lobe Right upper lobe Right main Lobectomy, radiation therapy Left pneumonectomy Left lower lobectomy Left pneumonectomy Wedge resection Expl. thoracotomy, actinomycin D, vincristine, radiation therapy 3.5 Yes 24 Yes 23 (1 1 postop.) Yes 12 Yes 3 Yes 10 (6 postop.) No 36 postop Alive at No Yes...

5 FALLON, SCHILLER, AND KILMAN morphic. The histological criteria distinguishing these three tumor types have been discussed by Stout [12] and by Horn and Enterline [71. The embryonal rhabdomyosarcoma occurring in our patient is typical of the tumor in general, which arises most commonly in the head, neck, or genitourinary system of young children. When such lesions occur beneath an epithelial surface, they may assume the gross appearance of a botryoid sarcoma. They are believed to arise from myoblastic differentiation of primitive mesenchymal cells present in the bronchial walls and pulmonary interstitial tissue. The age of the previously reported patients with pulmonary rhabdomyosarcoma varies from 20 to 68 years, with a mean age of There is a significant male predominance. The mean duration of the tumor from onset to death was 29.5 months, ranging from 3 to 152 months. If 1 unusually long surviving patient is disregarded, the mean survival was 14.0 months. Surgical ablation of the tumor was attempted in half the patients, resulting in the longest survival (152 months) and a mean postoperative survival of 47.2 months compared with a nonoperative survival of 9.5 months. The patient reported here is the youngest in the literature and the only patient to have received chemotherapy. Grosfeld and his associates 161 have recently described the Columbus Children s Hospital experience with chemotherapy of rhabdomyosarcoma. Despite the unresectable nature of the lesion, the child s clinical course has remained satisfactory, and metastatic involvement outside the right lung has not been demonstrated. The efficacy of chemotherapy has yet to be established. References 1. Bignardi, P. Su un caso di rabdomiosarcoma del polmone. Arch. Ztal. Anat. Zstol. Pat. 20:367, Conquest, H. F., Thornton, J. L., Massie, J. R., and Coxe, J. W., 111. Primary pulmonary rhabdomyosarcoma. Ann. Szirg. 161 :688, Drennan, J. M., and McCormack, R. J. M. Primary rhabdomyosarcoma of the lung. J. Palh. Bact. 79:147, Forbes, G. B. Rhabdomyosarcoma of. J. Path. Bact. 70:427, Gordon, L. Z., and Boss, H. Primary rhabdomyosarcoma of the lung: Report of a case. Cancer 8:588, Grosfeld, J. L., Clatworthy, H. W., Jr., and Newton, W. A., Jr. Combined therapy in childhood rhabdomyosarcoma: An analysis of 42 cases. J. Pediat. Surg. 4:637, Horn, R. C., Jr., and Enterline, H. T. Rhabdomyosarcoma: A clinicopathological study and classification of 39 cases. Cancer 11:181, Kostelecky, A., and Stolz, J. Lungenrhabdomyosarkom. Zbl. Chir. 81 :473, Maschio, C. Rabdomiosarcoma del polmone. Riv. Anat. Pat. Oncol. 11: 1161, McDonald, S., Jr., and Heather, J. C. Neoplastic invasion of the pulmonary veins and left auricle. J. Path. Bact. 48:533, Steele, J. D. Personal communication, Stout, A. P. Tumors of the Soft Tissue. In Atlas of Tumor Pathology, Sect. 11, Fax. 5. Washington, D.C.: Armed Forces Institute of Pathology, THE ANNALS OF THORACIC SURGERY