Primary Tumors of Ribs

Primary Tumors of Ribs Frank E. Schmidt, M.D., and Max J. Trummer, Capt, MC, USN ABSTRACT An analysis of 50 consecutive patients with primary rib tumors operated on at the U.S. Naval Hospital, San Diego, is presented. Thirty-nine patients had benign and 11 had malignant tumors. Men predominated in the entire series, but a greater proportion of women had malignant tumors. The average age in the group with malignant lesions was 41 years compared with 31.3 in the benign group; however, the age range was wide, and extremes were noted in both groups. Fibrous dysplasia and osteochondroma were the most common benign lesions, and Ewing s sarcoma and metastatic lesions comprised the majority of malignancies. Only one chodrosarcoma was encountered. Clinical and roentgenographic findings proved to be the best guides by which to estimate malignancy, but errors in preoperative diagnosis were frequent enough for wide local excision to be recommended for all rib tumors. Large tumors of lower ribs, especially with a history of rapid growth, were more likely to be malignant. A history of pain was more common in benign lesions and was of little diagnostic help. Roentgenographic findings were of great value, especially in the more common benign lesions. The irregular and often mottled areas of bone destruction in Ewing s sarcoma were strongly suggestive of the correct diagnosis, as were the irregular areas of bone destruction seen in metastatic lesions. Wide excision offered good results in patients with benign tumors. While survival was low among patients with malignant neoplasms, 1 patient is alive without evidence of disease forty-two months after combined radical excision and cobalt 60 teletherapy for Ewing s sarcoma. Radical excision is recommended for chondrosarcoma and wide excision for myeloma and reticulum cell sarcoma. T umors of ribs, like tumors elsewhere, generally require operation for either excision or biopsy. They present problems, however, because excisions of the chest wall can profoundly affect respiratory function. Rib tumors are uncommon, and fewer than 500 cases have been reported in the medical literature. These probably represent about 10% of all bony tumors. Previous reports have emphasized the malignant nature of most rib tumors and their rather poor prognosis [14]. Most of these reports have come from clinics with a large referral practice. The experience presented here is that of a large military hospital which serves an essentially general population with a large proportion of young men. An analysis of this experience suggests that most rib tumors are benign and that wide excision is well tolerated and gives good results. From the Thoracic Surgery Service, U.S. Naval Hospital, San Diego, Calif. Supported in part by U.S. Public Health Service Cancer Clinical Research Center Grant 5P02CA 05A57. Presented at the Seventh Annual Meeting of The Society of Thoracic Surgeons, Dallas, Tex., Jan. 18-20, 1971. Address reprint requests to Dr. Schmidt, Department of Surgery, Tulane University School of Medicine, 1430 Tulane Ave., New Orleans, La. 70112. VOL. 13, NO. 3, MARCH, 1972 251

SCHMIDT AND TRUMMER Case Material The present series consists of 50 consecutive patients treated by operation at the Naval Hospital, San Diego, from 1943 to 1967. It comprises all patients who were found to have a rib lesion on clinical or roentgenographic examination and in whom no systemic or primary malignant origin was evident after thorough clinical and laboratory investigation. BENIGN TUMORS Thirty-nine patients had benign lesions (Table 1). They ranged in age from 2 to 55 years. One patient was Negro; the remainder were Caucasian. Six patients were female. Only 6 patients complained of pain or discomfort, and 2 of these also had a palpable mass (1 patient with a chondroma and 1 with a nonunited fracture). A total of 4 patients had a palpable tumor; only 1 of these, a patient with a chondroma, had noted an increase in the size of the mass. All the lesions were excised. Fibrous dysplasia was found in 13 patients, including 3 with bone cysts (Table 2). Ten patients had osteochondroma, and 4 were found to have nonunion of fractures. A chondroma was present in 3 patients, and an osteoma and an eosinophilic granuloma were each encountered in 2 patients. Five patients had single lesions consisting of xanthoma, osteomyelitis, chondromalacia, necrosis of bone, and hypertrophy of cartilage. Fibrous Dysplusiu. Fibrous dysplasia was the most common tumor en- TABLE 1. PRIMARY TUMORS OF RIBS, U.S. NAVAL HOSPITAL, SAN DIEGO, 1943-1967 Benign Malignant (39 patients) (11 patients) Age range (yr.) 2-55 (avg. 31.3) 5-75 (avg. 41) Sex 33 male, 6 female 8 male, 3 female Race 1 Negro, 38 Caucasian All Caucasian TABLE 2. PRIMARY BENIGN TUMORS OF RIBS, U.S. NAVAL HOSPITAL, SAN DIEGO. 1943-1967 (39 PATIENTS) Pathology Fi brous dysplasia 13 Osteochondroma 10 Nonunion of fracture 4 Chondroma 3 Osteoma 2 Eosinophilic granuloma 2 Xanthoma 1 Chondromalacia 1 Osteomyelitis 1 Necrosis of bone 1 Hypertrophy of cartilage 1 No. of Patients 252 THE ANNALS OF THORACIC SURGERY

Primary Tumors of Ribs FIG. 1. Fibrous dysplasia of rib. The cortex is expanded and thinned, but the periosteal surface is typically smooth. No loculation is seen, but there is a homogeneous ground-glass appearance centrally. countered (Figs. 1, 2). It involved a single rib in each of 13 patients, all of whom were men. None of the patients had associated bony lesions of the skull or long bones, and 3 patients had lesions classified as bone cysts [71. The average age was 28.6 years, the youngest patient being 20 and the oldest, 55. The lower ribs (four through ten) were most often involved, and most of the lesions were located posteriorly. Only 1 patient complained of discomfort, and a mass was noted in 1 patient. One patient had a pathological fracture. All the lesions were excised widely, and there was one complication, a partial wound dehiscence. Osteochondroma. The average age of the 10 male patients with osteochondroma was 20.3 years. Only 3 patients were symptomatic. Two had a mass and 1 had discomfort on coughing. The tumors were in the upper four ribs in 7 patients and located anteriorly in 6. All were single lesions (Fig. 3), right-sided in 5 and left-sided in 5. FIG. 2. Fibrous dysplasia with expansion of rib contour. There is some erosion of the cortex, but it is smooth and there is new bone formation. Multilocular spaces are seen. VOL. 13, NO. 3, MARCH, 1972 253

SCHMIDT AND TRUMMER FIG. 3. Osteochondroma, right third rib. The tumor arises from the cortex of the rib and blends smoothly with the surrounding cortex at its periphery. A rim of calcification is evident. Irregular blotches of calcification are noted in the tumor. Its margins are distinct from the surrounding soft tissues. This is a typical lesion. Three of the 4 patients with nonunion of frac- Nonunion of Fracture. tures were women. The average age was 33.3 years. One of the 2 patients complaining of pain had a mass. The lesions were evenly distributed between upper and lower ribs and between anterior and posterior locations. Chondroma. Chondroma was found in 2 patients, 1 man and 1 woman aged 21 and 24, respectively. Both tumors were in the anterior portion of the third rib, 1 on the left and 1 on the right. A slow enlargement was described in each patient, having been known for 6 years in 1 and several months in the other. The patient with the most recent onset of symptoms had had discomfort in the area for 1 year. Osteoma. This lesion was present in 2 patients. The typical roentgenographic appearance is shown in Figure 4. A B FIG. 4. Osteoma, right fifth rib. Well-defined sclerosing margins with a central lucency as well as its projection from the body of the rib help identify this lesion. The adjacent rib is defwmed by the tumor. 254 THE ANNALS OF THORACIC SURGERY

~~ Primary Tumors of Ribs EosinophiZic GranuZoma. Eosinophilic granuloma was diagnosed in a 2-year-old boy and in a 28-year-old man. One tumor was located in the seventh rib laterally and the other in the eleventh rib posteriorly. The child presented with fever of unknown origin and the adult with lower chest pain of one month s duration. Both lesions were left-sided and presented on roentgenograms as osteolytic expanding lesions with thinned cortex. Neither patient had lesions of other bones or the skin [5]. MALIGNANT TUMORS Eleven patients ranging from 5 to 75 years of age had malignant lesions of the ribs (see Table 1). Three were women. Nine of the tumors were located on the right side. Ewing s Sarcoma. The 4 patients with Ewing s tumor comprised the largest group of primary malignant rib tumors (Table 3). All presented with a mass, but only 1 patient complained of pain. In each there was a history of rapid enlargement, the average duration being less than two months though 1 patient had had a mass for six months. Three lesions were located in the lower ribs (sixth, eighth, and eleventh), and 1 was present in the second rib. Operation was limited to biopsy in 1 patient who had diffuse infiltration of the retroperitoneal region and diaphragm from a tumor in the eleventh rib. Radiotherapy was given, but the patient died of his tumor in five months. Two patients had radical chest excisions. One died within a year of diffuse metastatic disease, and the other patient was lost to follow-up and is presumed dead. The fourth patient, a 5-year-old girl, had a radical chest wall resection that included excision of a wide portion of the diaphragm. She received cobalt 60 teletherapy after operation and is alive and well forty-two months after operation. Metastatic Carcinoma. Three patients presented with metastatic rib lesions. Excision was performed in each case, and tissue analysis demonstrated metastatic neoplasm. These patients were included in the series because they presented with no other evident disease and demonstrated only rib lesions. All 3 have died; primary tumors were found in only 2 patients, both of whom had pulmonary neoplasms. One of the neoplasms was undifferentiated, and the other was an adenocarcinoma. Myeloma. Two patients who presented with lytic lesions in the anterior portion of a lower rib (nine and ten) had wide excision. Both died with TABLE 3. PRIMARY MALIGNANT TUMORS OF RIBS, U.S. NAVAL HOSPITAL, SAN DIEGO, 1943-1967 (11 PATIENTS) Pathology No. of Patients Ewing s sarcoma 4 Metastatic disease 3 Plasma cell myeloma 2 Reticulum cell sarcoma 1 Chondrosarcoma 1 VOL. 3, NO. 3, MARCH, 1972 255

SCHMIDT AND TRUMMER systemic disease within two years of their operation after cobalt 60 teletherapy and systemic chemotherapy had been given. Reticulum Cell Sarcoma. One patient, a woman aged 33, had partial rib excision for a reticulum cell sarcoma which presented as a painful mass. She received irradiation and later chemotherapy, but was lost to follow-up and is presumed dead. Chondrosarcoma. The single patient with chondrosarcoma was a 65- year-old man who had had excision of a tumor of the fourth rib anterolaterally. Grossly and microscopically the tumor was thought to be a chondroma, but a large recurrence developed at the operative site five years later. This was excised radically, and radiation therapy was given. After four years another recurrence was excised, and one year later another local excision was done. Another radical excision was done one year later with forequarter amputation. The patient died of inanition, and no postmortem examination was permitted. Comment Most of the patients in the present series had benign tumors, and the majority were asymptomatic. Several patients complained of discomfort, and a larger number noted a mass. The rib lesions were discovered on routine examination, most frequently on roentgenography. Fibrous dysplasia (including bone cyst) and osteochondroma accounted for more than half of the benign tumors. Wide excisions were done in all patients with good results. Only one complication, a wound dehiscence, was observed. No recurrences were found on follow-up examination. Only 11 of 50 patients had malignant neoplasms. The average age was 41, which is 10 years older than the average in patients with benign lesions. Two patients were less than 10 years old; 1 had a benign lesion and the other, Ewing s sarcoma. Although men predominated in both groups, the proportion of women was twice as great in the group with malignant tumors as in the group in which the tumors were benign. Six patients with malignant tumors had involvement of lower ribs, and 9 patients had a lesion located on the right side. Benign lesions were more evenly distributed between upper and lower ribs and equally divided between right and left sides. Pascuzzi, Dahlin, and Clagett [6] reported that of 126 rib tumors, 80 were right-sided. The higher incidence of malignant tumors in the lower ribs in the present series probably reflects the fact that only 1 patient with chondrosarcoma was encountered. In other large series this tumor is reported to be the most common malignant rib tumor; it comprised nearly half the malignant growths reported by Pascuzzi and colleagues [6]. A mass was present in a higher percentage of the malignant tumors, and 1 of these was painful. Roentgenographic examination was very helpful in diagnosis, and irregular or complete destruction of portions of ribs, some- 256 THE ANNALS OF THORACIC SURGERY

Primary Tumors of Ribs times with calcific mottling, was a reliable sign of malignancy. The absence of this sign in the I patient with myeloma and the 1 patient with chondrosarcoma, however, emphasizes the importance of wide local excision as the initial operative procedure. It is our practice to excise the entire rib along with the attached intercostal muscles, endothoracic fascia, and pleura. If there is a strong suspicion of malignancy, the adjacent ribs above and below are included in the resection. The poor results of therapy for malignant rib tumor reported by others are supported by analysis of the 11 such patients in this series. Several considerations are important, however. Myeloma and reticulum cell sarcoma are really systemic diseases, even when they present as solitary rib lesions. Excision establishes the diagnosis and frequently, by removing a painful solitary lesion, allows postponement of systemic chemotherapy; but it does not affect the overall course of the disease. The same is also true of single metastatic lesions with unknown primary sites. The one patient with chondrosarcoma who had a history of multiple excisions over a ten-year period suggests that cure might have been possible had an adequate excision been performed at the outset. Results in the 1 patient who has survived three and one-half years after radical resection of the chest wall and diaphragm followed by cobalt 60 teletherapy for Ewing s sarcoma seem to recommend this form of therapy. The remaining 3 patients with this neoplasm all died of their disease. The 1 surviving patient was 5 years of age at the time of diagnosis; the 3 who died were all in the third decade of life. References 1. Barrett, N. R. Primary tumors of rib. Br. J. Surg. 43:113, 1955. 2. Groff, D. B., 111, and Adkins, P. C. Chest wall tumors. Ann. Thorac. Surg. 4260, 1967. 3. Ochsner, A., Lucas, G., and McFarland, G. Tumors of the thoracic skeleton: Review of 134 cases. J. Thorac. Cardiovasc. Surg. 52:311, 1966. 4. O Neal, L. W., and Ackerman, L. V. Cartilaginous tumors of rib and sternum. J. Thorac. Surg. 21:21, 1951. 5. O Neal, L. W., Skrocak, S. J., and Casey, P. R. Eosinophilic granuloma of ribs. J. Thorac. Surg. 29:528, 1955. 6. Pascuzzi, C. A., Dahlin, D. C., and Clagett, 0. T. Primary tumors of the ribs and sternum. Surg. Gynecol. Obstet. 104:390, 1957. 7. Zimmer, J. F., Dahlin, D. C., Pugh, D. G., and Clagett, 0. T. Fibrous dysplasia of bone: Analysis of 15 cases of surgically verified costal fibrous dysplasia. J. Thorac. Surg. 31:488, 1956. VOL. 13, NO. 3, MARCH, 1972 257