Primary Chest Wall Tumors

ORIGINAL ARTICLES

Primary Chest Wall Tumors Sabaratnam Sabanathan, F.R.C.S., F.R.C.S. (Ed), Fayek D. Salama, F.R.C.S., W. Ellis Morgan, F.R.C.S., and John A. Harvey, M.B.,Ch.B. ABSTRACT A retrospective study of 53 primary chest wall tumors, 26 benign and 27 malignant, was carried out to review their clinical radiological and pathological features. Forty-nine of the 53 lesions presented in the ribs and the remaining 4, in the sternum. The overall 5-year survival for patients with primary malignant neoplasms of the chest wall was 33.3%, and the 10-year survival was 18.5%. All of the deaths were disease related. All of the patients with benign tumors were treated by excision without recurrence or death. Distinction between benign and malignant chest wall tumors was not possible using radiographic criteria unless cortical destruction and involvement of soft tissues were visualized. On the basis of our analysis, we believe that all tumors of the chest wall should be considered malignant until proven otherwise and that wide excision should be carried out. This is necessary not only to obtain an adequate diagnosis but also to provide the best chance for cure in both benign and malignant lesions. Primary tumors of the bony chest wall are uncommon, although a wide variety of both benign and malignant tumors arise within the chest wall. These various tumors are often considered as a group because of the similarities in diagnostic and therapeutic problems they pose. Primary neoplasms of the bony chest have been reported to comprise 7 to 8%of all intrinsic bone tumors [ l , 21. Neoplasms of the ribs are far more common than those of the sternum [l-41. Primary rib tumors are as frequently benign as they are malignant [2, 41. Benign tumors of the sternum are uncommon [l, 31. The results of treatment by surgical means are dependent on the nature of the tumors and the adequacy of the removal [l-81. The prognosis for patients with benign tumors is invariably excellent after excision, whereas the 10-year survival for patients with primary malignant chest wall tumors is only 13.3% [9]. A familiarity with the natural history of these tumors is fundamental for early recognition and prompt treatment. Bearing this in mind, we reviewed our experience with chest wall tumors.

Material and Methods The present study is a retrospective analysis of all patients with primary tumors of the ribs or sternum who From the Departments of Thoracic Surgery and Histopathology, City Hospital, Nottingham, England. Accepted for publication Mar 26, 1984. Address reprint requests to Mr. Morgan, Department of Thoracic Surgery, City Hospital, Hucknall Rd, Nottingham NG5 IPD, England.

have been diagnosed and treated at Nottingham City Hospital since 1957. Patients with metastatic spread to the chest wall or involvement by direct invasion from intrathoracic neoplasms were excluded. In each instance, we reviewed the clinical presentation, roentgenographic appearance, histopathological findings, treatment, and subsequent progress. The series consists of 53 chest wall tumors, 49 of which were primary tumors of the ribs and the remaining 4, the sternum. Follow-up was complete for all of the patients. The mean follow-up was 8.3 years (range, 2 months to 26 years). There were 29 male and 24 female patients. All had incisional biopsy, complete excisions, or both. The Table summarizes the types of benign and malignant chest wall tumors encountered in our series.

Symptoms and Signs Pain and the presence of a mass were the two most common complaints of patients with chest wall tumors, and many patients experienced both. Twelve patients (22.6%)were asymptomatic, the tumor being discovered during routine radiographic examination (see Table). Pain, muscle weakness, and atrophy in an upper extremity secondary to brachial plexus compression by a tumor of the first or second rib were present in 5 patients, only 1 of whom had a benign tumor. Pleural effusion was present in 4 patients; 2 of them had Ewing's tumor and the other 2, chondrosarcoma. The average duration of symptoms was 12 months (range, 2 to 26 months) for benign and 20 months (range, two weeks to 13 years) for malignant chest wall tumors.

Results Benign Tumors Twenty-six of the 53 chest wall tumors were benign. Two were primary sternal tumors, and the rest were rib tumors. Sex incidence was equal. The age range of the patients was 4 to 66 years, with an average of 32 years. All of these patients were treated by operative excision. All survived, and there were no recurrences. CHONDROMA. There were 11 cases of chondroma (enchondroma) of the ribs and sternum. They accounted for 42.3%of the benign tumors and 20.8% of all chest wall tumors. Nine of these tumors occurred in the ribs and 2 in the sternum (see Table). Radiologically the typical chondroma appeared as a well-defined oval or slightly lobulated area of rarefaction (Fig 1). This was situated either centrally, with or without expansion of the cortex of the bone, or eccentrically. Calcification was either diffuse, giving a dense radiopaque appearance, or focal, resulting in stippling. The great majority (54%) occurred anteriorly near the cos-

5 Sabanathan et al: Primary Chest Wall Tumors

tochondral junction. The typical enchondroma was a well-defined lobular lesion located in the medullary cavity and consisting of bluish hyalin material with foci of calcification. Surrounding bone was invariably intact although sometimes attenuated. Histological studies showed cartilage of variable cellularity with occasional binucleate chondrocytes but lacking mitotic activity and nuclear pleomorphism. All of these tumors were treated by en bloc excision with a 4 to 5 cm margin of healthy tissue. OSTEOCHONDROMA. There were 3 cases of osteochondroma (ecchondroma, exostosis) in this series. They constituted 5.7% of the 53 chest wall tumors and 11.5% of the 26 benign tumors (see Table). Radiographically the tumor appeared as a lobulated projection of cortical bone with a cartilaginous cap (Fig 2). It usually was poorly visualized on the radiograph, although occasionally the presence of calcification within the cartilage enabled it to be identified. The osteochondromas consisted of a central bony protuberance with an expanded cauliflowerlike distal end covered by lobulated gray hyalin material over which lay the periosteum from adjacent bone. Microscopy revealed a benign cartilaginous cap overlying bony trabeculae formed by orderly enchondral ossification (Fig 3). The 3 patients with osteochondroma were treated by local excision. There were no recurrences. OSTEOBLASTOMA. An osteoblastoma involved the right seventh rib of a 26-year-old woman. The presenting complaint was progressively worsening pain of 2 years’ duration. Radiographic examination showed an expanded anterior end of the involved rib, within which was a small, focal radiolucent area surrounded by osteosclerotic tissue. The osteoblastoma was dark red and well circumscribed. Microscopic examination showed a vascular connective tissue stroma containing irregular osteoid and bony trabeculae (Fig 4). Abundant osteoblasts and giant cells were present, but there was no evidence of nuclear atypia or abnormal mitoses. The patient was treated by local excision. There was no recurrence. ANEURYSMAL BONE CYST. Four aneurysmal bone cysts of the rib were encountered in this series. They constituted 7.5% of all primary chest wall tumors and 15.4% of the benign tumors (see Table). The radiological appearance was generally that of an eccentric, “blowout” lytic lesion, sharply demarcated by a thin shell of periosteum (Fig 5). This radiolucent area was often oriented in line with the long axis of the bone. Mottled calcification was seen in the fibro-osseous septa lining the vascular spaces, and imparted a soap-bubble appearance. All 4 aneurysmal bone cysts were resected intact. The medullary cavity was expanded and filled with dark red spongelike material that had eroded into, and in one instance through, the adjacent cortex. On microscopy, the lesions consisted of numerous blood-filled spaces,

many of which were lined with endothelium, and were separated by variable amounts of fibrous stroma containing osteoid, multinucleate giant cells, extravasated blood, and hemosiderin. The 4 tumors were removed by wide excision. There have been no recurrences over periods ranging up to 9 years. FIBROUS DYSPLASIA. The 53 tumors comprised 4 cases of fibrous dysplasia of the rib. They constituted 7.5% of all the tumors and 15.4% of the benign tumors (see Table). Radiographically, fibrous dysplasia appeared as a central, fusiform lytic lesion that had expanded and eroded the cortical bone (Fig 6A). Throughout the mass were trabeculae of bone, but calcification was not prominent. Grossly, part of the rib was replaced by well-demarcated firm tissue that had eroded into, but not through, the overlying cortex. On microscopy, the lesion consisted of cellular fibrous tissue and poorly oriented trabeculae of incompletely mineralized woven bone lacking a rim of osteoblasts (Fig 6B). All patients were treated by wide local excision. There were no recurrences. EOSINOPHILIC GRANULOMA. Eosinophilic granulomas occurred in 3 patients, thereby representing 5.7% of ,the 53 primary chest wall tumors and 11.5% of the 26 benign tumors (see Table). Radiologically the lesion was characterized by a geographic lytic defect with well-defined margins. There was also a little widening of the rib (Fig 7). The affected ribs contained well-demarcated lesions showing focal hemorrhage. Histological studies revealed the characteristic infiltrate of various cells, predominantly histiocytes and eosinophils, with some giant cells, fibroblasts, lymphocytes, and plasma cells. All of these lesions were treated by local excision. There have been no recurrences over periods ranging up to 10 years.

Malignant Tumors Twenty-five of the 49 rib tumors and 2 of the 4 sternal tumors were malignant. The patients ranged from 14 to 78 years old, with an average of 48.4 years. Sixteen patients were male, and ll were female. There was l immediate postoperative death; the patient had a terminal malignancy. The overall 5-year survival was 33.3% and the 10-year survival, 18.5%. All of the deaths were disease related. In 15 patients there was recurrence of tumor; only 6 were considered candidates for further attempts at excision. One of these 6 patients is alive after 10 years. CHONDROSARCOMA. Chondrosarcoma was found in 13 patients and involved the ribs in 12 and the sternum in 1. These tumors constituted 24.5% of all the chest wall tumors and 48.1% of the malignant tumors (see Table). Chest radiography often revealed a large, lobulated tumor that had destroyed the cortical bone and usually was calcified in a mottled manner (Fig 8A). The tumors ranged from 5 to 25 cm in diameter. The gross appear-

6 The Annals of Thoracic Surgery Vol 39 No 1 January 1985

Data on Patients with Primary Chest Wall Tumors

Type

Age Rangeb Patientsa (yr)

Chondroma

11 (615)

6-66 (36.1)

4

6

2

Compression of brachial plexus (1)

Osteochondroma Osteoblastoma Aneurysmal bone cyst Fibrous dysplasia Eosinophilic granuloma Chondrosarcoma

3 (013) 1 (011) 4 (212) 4 (311) 3 (211) 13 (914)

4-33 (17) 3 26 1 18-60 (40.7) 1 1 22-45 (34) 18-26 (21.3) 3 21-78 (50.8) 13

3 0 1 0 0 13

0 0 3 3 0 0

0 0 0 0 0

Tumor

Symptoms

No of

Pain Swelling Asymptomatic Others

Ewing's tumor

6 (2/4)

14-29 (20.3)

5

4

1

Compression of brachial plexus (4); spinal cord compression (1); pleural effusion (2) Pleural effusion (2)

Plasmacytoma

8 (513)

49-73 (65.4)

4

1

3

0

"Numbers in parentheses are the numbers of malelfemale patients bNumbers in parentheses are the average ages.

ances of the chondrosarcomas were variable. These tumors tended to show a lobulated, smooth, firm, bluish surface. Although apparently well demarcated, they caused destruction of cortex and often invaded surrounding soft tissues. Foci of myxoid material, hemorrhage, necrosis, or calcification was commonly present. Histological findings included a range of tumors, from a poorly differentiated spindle cell sarcoma showing only focal microscopic areas of chondroid differentiation to an extremely well differentiated lesion indistinguishable from a benign cartilaginous tumor (Fig 8B). This latter finding reinforces the caveat concerning the unpredictable biological behavior of histologically benign cartilaginous tumors of the ribs, long bones, and pelvis in adults. The tumor was removed by radical en bloc resection in 10 patients. Five of them died of recurrence within 2 years, and the other 5 are well and free from recurrence 3 to 16 years after treatment. Four of these 5 patients have been followed up for more than 10 years. Of the 3 patients treated by local excision, only 1 patient is still living after 10 years but has recurrence. EWING'S TUMOR. Ewing's tumor was encountered in 6 patients. This represents 11.3% of all of the primary chest wall tumors and 22.2% of the malignant tumors (see Table). The only fairly consistent radiographic finding was evidence of destruction or lysis of bone, in itself a rather nonspecific feature (Fig 9A). In some instances, there was a diffuse, expanded bone lesion with little periosteal reaction and irregular absorption of the medullary area. In other instances, there was widening of the medulla, sclerosis of the widened cortex, and multiple layers of periosteal new bone formation, thereby producing the

characteristic onion-peel appearance. Although the tumor was usually confined to one rib, it is not uncommon for more than one rib to be involved in growth of the lesion. Grossly, Ewing's tumors were firm and pale with variable, sometimes extensive, areas of hemorrhage and necrosis. Microscopy of the viable tumor showed sheets of uniform round, closely packed cells, larger than lymphocytes, with scanty cytoplasm. Tumor cells tended to aggregate around blood vessels, thereby forming perivascular rosettes (Fig 9B). The microscopic appearance alone is not pathognomonic, and the histological differential diagnosis includes lymphoma and metastatic neuroblastoma. Four patients seen prior to 1975 who were treated by local excision and radiotherapy to the tumor bed died of widespread metastases within 18 months. The 2 patients treated since 1975 had radical surgical excision, radiotherapy to the tumor bed, and systemic adjuvant chemotherapy. Both are alive and well at 8 and 3 years postoperatively. SOLITARY PLASMACYTOMA. There were 8 cases of solitary plasmacytoma. They constituted 15.1% of all the chest wall tumors and 29.6% of the malignant tumors (see Table). Radiographically, plasmacytoma appeared as a welldefined, punched-out lytic lesion in the ribs. It was not so readily recognized when it took the form of a solitary lesion in the sternum. When that happened, the cortex was usually destroyed in several places, and the tumor invaded soft tissue. Solitary plasmacytomas are both grossly and microscopically indistinguishable from multiple myeloma deposits. To the naked eye, they are soft and pale reddish

7 Sabanathan et al: Primary Chest Wall Tumors

Tumor Location Duration of Symptoms

Survival

Ant. End

Post. End

Distribution Sternum in Ribs

Treatment

Recurrence

5 Yr

10 Yr

6mo-2yr

6

3

2

Even

Excision

None

CR

CR

6-26mo 2 Y'

2-3 mo 6wk-13 yr

3 1 2 3 0 4

0 0 2 1 3 8

0 0 0 0 0 1

Ribs 3, 8, 11 Rib 7 Ribs 1, 6, 6, 9 Ribs 4, 5, 5, 9 Ribs 4, 5, 8 Upper 7 ribs

Excision Excision Excision Excision Excision Total excision (10); subtotal excision (3)

None None None None None 7

CR CR CR CR CR 5

CR CR CR CR CR 5

2wk-6mo

6

0

0

Upper 7 ribs

4

1

0

3 mo-4yr

4

3

1

Upper 7 ribs

4

3

0

~~~

... ...

CR

=

Excision + radiotherapy (4) + chemotherapy (2) Excision (7); biopsy + irradiation (1)

complete recovery.

Fig 2. Radiograph of left side of chest showing a typical osteochon droma of left eleventh rib.

Fig 1. Chondroma of left second rib in a 14-year-old girl. Tumor caused symptoms and signs of brachial plexus compression.

8 The Annals of Thoracic Surgery Vol 39 No 1 January 1985

Fig 3. Osteochondroma. Low-power view shows the exophytic architecture of the lesion, which consists of a core of bone (white arrow) capped by benign cartilage (black arrow). ( H B E ; x54 before 25% reduction.)

Fig 4. Osteoblastoma. Contrast the normal bone trabeculae (top left) with the irregular osteoid and bony trabeculae separated by vascular connective tissue. (HBE; X 54 before 25% reduction.)

9

Sabanathan et al: Primary Chest Wall Tumors

Fig 5 . Aneurysmal bone cyst of left ninth rib.

gray. Histologically, plasmacytomas consist of closepacked cells with plasmacytoid features such as an eccentric "clock-face" nucleus and a perinuclear halo, and copious heterophilic cytoplasm (Fig 10). Cells in welldifferentiated plasmacytomas closely resemble normal plasma cells. Nuclear pleomorphism, prominent nucleoli, and multinucleate tumor cells are noticeable in poorly differentiated lesions. Radical resection was carried out in all 7 patients who had lesions in the rib. One patient with sternal solitary plasmacytoma underwent biopsy and irradiation. Four of the 8 patients are alive and free from disease at 1%,3, 5, and 8 years. In the other 4 patients, multiple myelomas developed within 2 years, and only 1patient was alive at 5 years.

Comment Our finding that primary rib tumors are about as frequently benign as malignant agrees with the findings of Teitelbaum [2] and Hochberg and Crastnopol [4], and differs from those of Pascuzzi and co-authors [l],who found more primary malignant bone tumors than benign ones. Ochsner and associates [3] reported that benign chest wall tumors outnumber malignant ones. The

even distribution of the benign tumors throughout the entire rib cage and the confinement of all malignant tumors to the upper seven ribs in our series match the findings of Pascuzzi and associates [l]. Although our series includes only 4 primary sternal tumors, our experience with them is unusual in that 2 were benign and 2, malignant. In contrast, two previous series of primary sternal tumors showed a marked excess of malignant tumors [l, 31. Common clinical features such as pain and the presence of a palpable swelling are unreliable in distinguishing between benign and malignant tumors. Although 22 (81.5%) of the 27 malignant tumors were painful, so were 13 (50%)of the 26 benign lesions. Palpable swelling was present in 66.7% of the malignant tumors and in 38.5% of the benign lesions. The age of the patient and duration of symptoms are not useful criteria for judging likely biological behavior of a growth, since the age ranges for patients with benign and malignant tumors overlapped considerably, as did the ranges of duration of symptoms. The clinical features that suggest that a tumor is or has become malignant are recent rapid increase in size, invasion of adjacent structures, and the presence of metastasis, particularly to the lungs. However, the majority of malignant neoplasms in this series lacked these features. When visualized together, cortical destruction and soft tissue involvement are the only specific radiological features of malignancy [3, 10-121. The fact that a tumor involves only one rib does not necessarily imply that it is benign, since a malignant tumor may be monostotic early in its natural history. Many types of diagnostic biopsy have been reported, including curettage, needle aspiration biopsy, and open biopsy. In general, nonexcisional biopsy of a tumor of the chest wall should be discouraged because of the risk of implantation of tumor tissue along the needle track after aspiration biopsy [13] and in the soft tissues after open biopsy [14]. Most important, interpretation of the true histological appearance of the chest wall tumor, particularly a cartilaginous tumor, can be extremely difficult. Even skilled pathologists cannot reliably predict biological behavior on the basis of histological examination [3, 7, 14-19]. In 1982, Mankin and co-workers [19] reported that 18.2% of a total of 329 patients with malignant primary bone and soft tissue tumors had to be managed by a less than optimum treatment plan and that 8.5% of patients had a distinctly adverse prognosis because of major errors in diagnosis from initial biopsy studies. Since radical excision is the most effective therapeutic method in the majority of these patients, our efforts have been directed chiefly toward preparation of patients for initial radical excision; biopsy studies have been'reserved for the few questionable large lesions. Cartilaginous tumors, both benign and malignant, are the most commonly encountered primary neoplasms of the bony chest wall [3, 151. Approximately 50% of the chest wall tumors we have encountered were cartilaginous in nature. The lesions currently believed to con-

10 The Annals of Thoracic Surgery Vol 39 No 1 January 1985

stitute the cartilaginous group are variable in clinical presentation and biological behavior. Indeed the unpredictable biological behavior of these tumors led Lichtenstein and Jaffe [17] to claim that they had never seen a benign cartilaginous tumor in either the rib or the sternum. Chondromas are often mentioned as tumors likely to become malignant after years of benign growth. Dahlin [20] wrote that it is unusual for a true solitary enchondroma to develop sarcomatous changes. In this connection, Jaffe [21] reported the development of chondrosarcoma in 3 (11%)of 28 patients with hereditary multiple exostosis, and pointed out that the incidence of malignant change in this same group might be appreciably higher, since the majority of the patients were still young at the time the survey was made. The incidence of chondrosarcomatous change in a solitary osteochondroma is substantially lower (1 to 2%) [lo]. In 1981, Gitelis and associates [22] reported that 18 of the 125 chondrosarcomas in their series were secondary to preexisting benign cartilaginous lesions. Of the 18 secondary chondrosarcomas, 15 originated from multiple exostosis and 3 from solitary osteochondroma. None of the chondrosarcomas was secondary to an isolated enchondroma. Four of the 13 patients with chondrosarcomas in this series had a previous diagnosis of benign chondroma by nonexcisional biopsy. The final diagnosis A

B Fig 6 . ( A ) Radiograph showing fibrous dysplasia of left ninth rib. IB) This lesion consists of a benign spindle cell stroma and poorly oriented irregular, clawlike trabeculae of bone. ( H B E ; x 135 before 25% reduction.)

11 Sabanathan et al: Primary Chest Wall Tumors

Fig 7 . Chest radiograph showing an eosinophilic granuloma of left fifth rib.

was made after en bloc excision. The interval between the two procedures ranged from 6 months to 10 years. There is no place for radiotherapy, since chondrosarcoma is very resistant to irradiation [23]. The place of chemotherapy has not yet been defined in chondrosarcoma or indeed in any primary malignant tumors of bone other than osteosarcoma and Ewing’s tumor. Certainly no survival figures are yet available [18]. Only adequate surgical ablation gives a high chance of cure [22,23]. Reported overall 5-year survival of patients with resected chondrosarcomas ranges from 54 to 67% and 10-year survival, from 38 to 50% [18, 221. These compare with the 38.4% 10-year survival in this series. Eosinophilic granuloma is a benign destructive bone lesion of unknown etiology that frequently involves ribs and sternum [24]. Eosinophilic granuloma principally afflicts older children and young adults, and is rarely seen in patients older than 30 years [3, 251. Eosinophilic granuloma, together with Hand-Schiiller-Christian disease and Letterer-Siwe disease, forms the entity known as histiocytosis X [26]. It has been debated whether these conditions with disparate clinical features should be grouped together as a single entity [24, 271. In a 1979 review, Daneshbod and Kissane [27] identified two separate groups of patients based on clinical and pathological features, and concluded that histiocytosis X is at

least two different diseases. The first is disseminated histiocytosis X (Letterer-Siwe disease). It involves two or more organ systems and affects children less than 3 years old. All patients have died irrespective of therapy. The second form of histiocytosis X is composed of two clinical entities, each of which is confined to a single organ system or bone. One is eosinophilic granuloma, and the other is Hand-Schiiller-Christian disease. These are benign lesions that remit spontaneously and do not require extensive therapy. Osteoblastoma is a benign tumor that rarely occurs in the ribs [7]. Regardless of their location, these tumors are characterized clinically by pain, which is occasionally intermittent but always progressive in severity. Surgical excision of the involved rib is curative. It has been reported that osteoblastomas may occasionally undergo malignant change after some years. This complication seems to have a relatively low but significant incidence [lo]. Aneurysmal bone cyst was described as a distinct clinicopathological entity by Jaffe and Lichtenstein [28] in 1942. Although there is general agreement that an aneurysmal bone cyst is not a true neoplasm, there is no unanimity of opinion regarding its etiology. Trauma and subsequent hemorrhage have been implicated; Lichtenstein [lo] wrote that the condition results from a primary local alteration in hemodynamics, and Jaffe (291 considered it a secondary feature grafted on a preexisting bone lesion and replacing it to a greater or lesser extent. Aneurysmal bone cyst is a relatively rare lesion accounting for only 1 out of 144 chest wall tumors reported by Pascuzzi and co-workers [l].Although it is not a true neoplasm, local extension can cause paralysis [30, 311, and incomplete removal can result in persistence of growth [32]. En bloc resection of these tumors is curative. Fibrous dysplasia of bone is a morbid entity with a precise clinicopathological picture and well-defined morphological characteristics. It may appear as a solitary focus or may be multiple. Fibrous dysplasia of bone appears in childhood; after the cessation of skeletal growth, new foci do not appear and old ones cease to grow and may even regress and ossify [33]. In some patients, the foci increase in size and result in major tumoral deformities; in rare instances, malignant change may occur [lo]. Fibrous dysplasia can involve any portion of the skeletal system. Schlumberger [34] reported that in almost half of the 67 patients in his series, the lesion occurred in the ribs. This lesion usually is discovered in adulthood. Pascuzzi and his group [l]and Ochsner and colleagues [3] found the average age of patients with this tumor in a rib to be 40 and 38 years old, respectively (range, 18 to 65 years). In contrast to other tumors of the ribs, fibrous dysplasia is most often seen in the posterior segment of the rib. However, 3 of the 4 patients in our series had lesions in the anterior segment. This lesion should be distinguished from other cystic bone diseases, which may be associated with disturbances in calcium metabolism. Fibrous dysplasia of a rib should be excised, since

12 The Annals of Thoracic Surgery Vol 39 No 1 January 1985

these tumors can sometimes grow to bulky proportions. The prognosis after resection is excellent. Solitary plasmacytomas are rare tumors of plasma cell origin. Despite much attention by many investigators, their nature and relation to multiple myeloma remains unclear. Wiltshaw [35], in an extensive review of plasma cell tumors, noted a marked similarity between multiple myeloma and the disseminated stage of solitary plasmacytoma of bone. Specifically, the bone lesions are similar in appearance, distribution, and multiplicity. Therefore, he advanced the hypothesis that solitary plasmacytoma of bone, although clinically unusual, represents the same pathological process as multiple myeloma. Corwin and Lindberg [36], however, thought that solitary plasmacytoma should be considered a distinct entity, since occasionally it remains solitary throughout the life of the patient. To qualify as solitary plasmacytoma, a disease-free interval of 3 years is required [36]. The overall 5-year survival for patients with solitary plasmacytoma in our series (37.5%) is lower than that reported by Corwin and Lindberg [36] (44.4%) but higher than that in earlier reports [l].The most successful treatment is the administration of systemic antiA

B

Fig 8. (A) Posteroanterior chest radiograph showing chondrosarcoma of left first costochondral junction in a 46-year-old woman. ( B ) Highpower view shows spindle cells and chondrocytes, both exhibiting marked nuclear and cellular pleomorphism. (H&E; x 338 before 25% reduction.)

13 Sabanathan et al: Primary Chest Wall Tumors

A

B Fig 9. (A) Ewing’s tumor. Expansile destructive lesion of left seventh rib had large extrapleural mass and was found on routine chest roentgenogram. ( B ) The tumor consists of close-packed, fairly regular dark cells with scanty cytoplasm showing perivascular rosette formation. ( H 6 E ; x 780 before 25% reduction.)

metabolites; irradiation provides dependable local control [36]. Ewing’s tumor is a relatively uncommon neoplasm of uncertain origin and affects mainly children, adolescents, and young adults [7, lo]. In a collective review of 448 patients with Ewing‘s tumor, only 29 (6.5%) had primary rib lesions [37]. In the past, treatment of Ewing’s tumor by irradiation alone, operative intervention alone, or a combination of the two yielded 5-year survival ranging from 10 to 15% [38, 391. Reports published

in the 1980s, however, indicate that an integrated therapeutic approach to Ewing’s tumor consisting of surgical resection, irradiation to the tumor bed, and adjuvant chemotherapy results in a high rate of local tumor control and improved survival [37, 401. Primary tumors in the rib present advantages in treatment: they can be completely excised without causing severe functional impairment. There is increasing evidence to show that patients with Ewing‘s tumor who undergo complete excision rather than biopsy have a better prognosis [37, 38, 401. With the exception of large solitary plasmacytomas, all primary neoplasms of the bony chest wall should be treated by complete en bloc excision [l-9, 11, 14,37, 381. Just as surgeons are often unable to determine the nature of the tumor by clinical and radiographic features,

14 The Annals of Thoracic Surgery Vol 39 No 1 January 1985

Fig 10. This well-differentiated plasmacytoma consists of cells with eccentric "clock-face" nuclei and perinuclear halos. ( H B E ; x 780 before 25% reduction.)

sometimes even skilled pathologists find it difficult to predict biological behavior on the basis of histological examination [3,4, 141. Hence, wide excision is necessary not only for adequate diagnosis but also to provide the best chance for a cure in both benign and malignant lesions. Wide excision of tumors of ribs should include the ribs immediately above and below the involved rib, the adjacent muscles, and the underlying pleura. As much of the diseased rib as is feasible should be excised because of the great tendency for intramedullary [41] and periosteal extension [14]. The other tissues to which the mass may be adherent must also be excised [14]. The treatment of choice for all solid tumors of the sternum is wide surgical excision. The size of the tumor is of itself no contraindication to radical removal [14, 42, 431, since large segments of the chest wall may be removed and reconstructed with little functional disturbance. Various materials have been used to reconstruct chest wall defects: Marlex mesh, ox fascia, methyl methacrylate, metal struts, and autotransplantation of ribs or even diaphragm [3, 42, 431. Repeat excision is worthwhile for local recurrence of primary malignant tumors. Its value is demonstrated by at least 1 patient in this series in whom 4 recurrent lesions were excised after en bloc excision of chondrosarcoma of the left fourth rib in 1973. The patient is still alive. The emphasis on early diagnosis of malignant tumors as a means of reducing mortality has been carried into the field of chest wall tumors. Prognosis of benign

tumors after surgical excision is uniformly excellent. For malignant neoplasms, adequacy of surgical excision has been proven to be an important determinant of the incidence of recurrence and survival.

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15 Sabanathan et al: Primary Chest Wall Tumors

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