Asian Cardiovasc Thorac Ann 2000;8:249-252
© 2000 Asia Publishing EXchange Pte Ltd
Primary Neoplasm of the Chest Wall: Surgical Management
Sowrangshu Kumar Chowdhury, MCh,
Subbarao Kasturi Satya Venkata Kumara, MCh,
Nachiappan Muthuraman, MCh,
Karoon Agrawal, MCh,1
Department of Cardiothoracic Surgery
1 Department of Plastic Surgery
Jawaharlal Institute of Postgraduate Medical Education & Research
Pondicherry, India
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For reprint information contact: Subbarao Kastari Satya Venkata Kumara, MCh Tel: 91 413 37 2210 Fax: 91 413 37 2375 email: jipmer{at}jipmer.ren.nic.in Department of Cardiothoracic Surgery, Jawaharlal Institute of Postgraduate Medical Education & Research, Pondicherry 605006, India.
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Abstract
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From January 1986 to December 1997, 17 patients (12 males and 5 females) aged 13 to 70 years were treated for primary neoplasms of the chest wall. There were 4 cases of fibrous dysplasia, 3 each of chondrosarcoma and Askin's tumor, 2 of plasmacytoma, and 1 each of fibrosarcoma, Ewing's sarcoma, synovial sarcoma, osteosarcoma, and enchondroma. All patients, except the case of Ewing's sarcoma, underwent wide excision or debulking for unresectable tumor, and reconstruction of the chest wall. Preoperative neoadjuvant chemotherapy was given to 1 patient with osteosarcoma, radiotherapy and chemotherapy were given to 2 others. In 8 patients, the skeletal defect was reconstructed with prosthetic material. Soft tissue reconstructive procedures with various myocutaneous flaps were performed in 6 patients. None of the patients required mechanical ventilation postoperatively. There were 2 early deaths. During follow-up of 3 months to 10 years, all patients with benign tumors were free of recurrence, 2 with Askin's tumors and 1 with osteosarcoma died. Prefabricated acrylic ribs are recommended for skeletal support during chest wall reconstruction.
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Introduction
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Primary neoplasms of the chest wall are uncommon. They encompass both bone and soft tissue diseases, excluding tumors arising in the spinal cord, meninges, vertebral bodies, skin, breast, lymph nodes, pleura, or mediastinal structures.1,2 Our experience in treating these neoplasms is reviewed.
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Patients and Methods
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Seventeen patients with primary neoplasms of the chest wall were diagnosed and treated at our institution between January 1986 and December 1997. Patients with metastatic spread to the chest wall or involvement by direct invasion from an intrathoracic neoplasm were excluded from this study. There were 12 males and 5 females, their ages ranged from 13 to 70 years. Computed tomography scanning of the thorax was performed in 7 patients to determine the extent of chest wall involvement and possible mediastinal or pulmonary dissemination. Fine-needle aspiration cytology (FNAC) was carried out in all cases. Incisional biopsy was undertaken when FNAC was unhelpful. For malignant lesions, ultrasonography of the abdomen and liver function tests were used to rule out intraabdominal metastasis. Pulmonary function tests were performed when there was pulmonary metastasis or when lung resection was contemplated. All resected specimens were sent for histopathology to confirm the diagnosis.
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Results
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Twelve patients had primary malignant tumors and 5 had benign tumors. The distribution of various tumors is shown in Table 1
. All benign tumors were located in the ribs. Among the malignant tumors, 2 were located in the ribs, 1 in the clavicle and associated chest wall tissue, and 3 in the sternum and costal cartilages. In the other cases, the apparent tissue of origin was soft tissue of the chest wall, as determined at operation or by pathological review of the surgical specimen, or both. The most common presentation of these tumors was a palpable mass. The various modes of presentation of primary neoplasms of the chest wall are shown in Table 2. Surgical treatment comprised wide excision in 8 patients, wide excision and lobectomy in 1, excision biopsy in 5, tumor debulking in 3, and lobectomy for solitary pulmonary metastasis in 1 patient. Preoperative hemostatic radiotherapy was given to 1 patient to control bleeding from the biopsy site and fungal infection. Postoperative radiotherapy and chemo-therapy were given to 2 patients. Preoperative neoadjuvant chemotherapy was given to 1 patient. The patient with Ewing's sarcoma received radiotherapy and chemotherapy. Skeletal defects were reconstructed with prosthetic material in 8 patients: acrylic ribs were used in 3 cases, Marlex mesh in 2, and stainless steel wires in 3. Soft tissue reconstructive procedures were carried out with myocutaneous flaps in 6 patients. A pectoralis major myocutaneous flap was used in 4 cases, a latissimus dorsi myocutaneous island flap and transverse rectus abdominis myocutaneous island flap were used in one case each.
Complications occurred in 7 patients, which included irreversible circulatory failure in 1, undetermined per-sistent tachycardia in 1 (requiring propranolol to control the heart rate), paraplegia in 2, muscle flap necrosis in 1, and prosthetic graft infection in 1. There were 2 early deaths (within 30 days of surgery) and 2 late deaths. The survivors were followed up for 3 months to 10 years. All patients who had benign tumors are alive and without recurrence. Three patients, 1 with Askin's tumor, 1 with osteosarcoma, and another with Askin's tumor died during the follow-up at 3 weeks, 5 weeks, and 4 months, re-spectively. The patient with synovial sarcoma returned within 6 months with extensive recurrence and involve-ment of the muscle planes and neurovascular bundle of the left upper limb; palliative debulking of the tumor was performed.
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Discussion
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Primary tumors of the chest wall are uncommon and require preoperative diagnosis in order to plan the best management. Malignancy in primary chest wall tumors is approximately 50%.1 In this study, it was 70.59% (12/17). The most common mode of presentation of a primary chest wall tumor is as a palpable tender mass and in these patients, 53.3% (8/15) of the tumors were painful, in contrast to the findings of King and colleagues3 where 90% presented as a palpable tender mass. However, these features are unreliable.4 Most reports stress that a primary neoplasm should be diagnosed by excision rather than incision or needle biopsy.3,5 We found FNAC to be reasonably useful in providing information for planning proper management in our patients. In 82.35% of cases, FNAC was able to provide sufficient information on the nature of the tumor, which was subsequently confirmed by histopathologic diagnosis of the resected specimen. Gupta and Naran6 in their recent study on FNAC in 146 cases of cutaneous and subcutaneous metastatic deposits from epithelial malignancies, found no false-negative or false-positive results. Gattuso and colleagues7 demon-strated that the majority of cutaneous and subcutaneous masses of the chest wall subjected to FNAC are neoplastic and it is an effective technique for primary assessment of these lesions as only 3% of the aspirates were unsatis-factory for cytological evaluation. Moreover, in some tumors such as Ewing's sarcoma, there is no need to perform extensive resection.8,9
Fibrous dysplasia was the most common benign tumor in this series, comprising 24% of all tumors and 80% of benign tumors, although it represented only 7.5% to 15.7% in previous reports.1,5 It sometimes grows to an enormous size, requiring extensive resection and reconstruction. Wide excision ensures a complete cure, and malignant transformation is rare.2 Enchondromas are the most common benign tumor, originating in the manubrium sterni, and growing as a painless mass.2 Pain is due to fracture or rapid growth during puberty. It is extremely difficult to differentiate benign chondroma from chon-drosarcoma by microscopy, hence wide excision is re-commended. In the only case in our series, the tumor originated in the rib and the patient is doing well 8 years after wide excision.
The most common malignant tumor in the chest wall is chondrosarcoma.1,2,10 Sabanathan and colleagues5 and Burt and colleagues10 reported an incidence of 24.5% and 23%, respectively. The affected age group is 20 to 40 years. Often, there is a history of chest wall trauma. As neither chemotherapy nor radiotherapy are effective, wide excision remain the treatment of choice.5 The differential figure for 10-year survival with chondrosarcoma is 67%.11 Of the 3 patients in our series, 2 were available for follow-up; 1 is alive after 8 years and the other at 2 years after surgery, and both are free from recurrence.
Although soft tissue tumors are a major source of chest wall tumors, we had only one case of fibrosarcoma of the chest wall involving the upper lobe of the right lung.1,3 The patient underwent wide excision with a right upper lobectomy and is alive and free of recurrence 6 years after surgery. Askin's tumor deserves special mention. This neuroepithelial tumor of the thoracopulmonary region probably occurs more commonly than is generally appreciated and carries a very poor prognosis. It can be confused with Ewing's sarcoma. There is a female preponderance of 3:1 and it occurs in the younger age group with average survival of only 8 months from the time of diagnosis, even with a multimodality therapeutic approach. The 3 patients with Askin's tumor in this series were treated by surgery and radiotherapy, with a poor outcome. Ewing's sarcoma is an uncommon neoplasm that arises from a rib in only 10% of cases.9 Sabanathan and colleagues11 reported an incidence of 3.43%. Both Askin's tumor and Ewing's sarcoma show the same translocation. Histologically, Ewing's tumor cells contain abundant glycogen and no neurosecretory granules, whereas Askin's tumor contains no glycogen and abundant neurosecretory granules.2 The single case of Ewing's sarcoma in our series responded very well to radiotherapy and chemotherapy.
Solitary plasmacytoma of the chest wall is extremely uncommon.3–5,12 Local control by either resection or radiotherapy is 62% to 100% effective.12 However, radiotherapy appears to be the treatment of choice.8,11 Seventy-five percent of solitary plasmacytomas develop multiple myeloma.12 Five-year survival is 59%.11 One of our surviving patients is doing well without recurrence 2 years after surgery, with postoperative radiotherapy and chemotherapy. Only 3% of osteosarcomas arise in the chest wall and present as a rapidly enlarging mass; 35% metastasize to the lungs by the time of presentation. The role of radiotherapy in this tumor is unclear and it carries a poor prognosis.2 Synovial sarcoma occurs in the 2nd to 4th decade of life and is most common in the lower extremities but can occur in any muscle and need not be close to a joint. The anatomical location of most truncal sarcomas precludes surgical excision with a wide margin to ensure local control. Radiotherapy is used for treatment of all high-grade truncal sarcomas.13
Wide excision of a chest wall tumor is essential for successful management and the extent of resection should not be compromised because of inability to close the chest wall defect, since even large defects can be reconstructed with little functional disturbance.11 Large defects (greater than 5 cm) give rise to flail chest and reconstruction is necessary to maintain adequate integrity of the chest wall. For soft tissue coverage, muscle flaps, myocutaneous flaps, and free flaps are used, depending on the location and size of the defect. Various materials have been used for skeletal support, including autologous bone graft, metal strut (stainless steel wire), methyl-methacrylate, polypropylene mesh, steel mesh, and poly-tetrafluoroethylene.2,4,12 Each has merits and drawbacks. The most popular current method of skeletal reconstruction is probably the Marlex sandwich that consists of two pieces of Marlex mesh cut slightly larger than the defect, with methylmethacrylate sandwiched between them, and moulded to conform to the body contours.12 However, methylmethacrylate requires preparation outside the operative field as there is a risk of toxicity if the resin is polymerized in situ. Currently, our prosthetic material of choice is acrylic ribs that are made according to the patient's bony thorax preoperatively, sterilized, and ready for use at the time of surgery. The details of acrylic ribs and their use in chest wall reconstruction have been described elsewhere.14 This provides an excellent chest wall contour and rigid fixation, minimizes delay, and avoids cumbersome procedures during surgery. The cost involved also is less, which is to be considered in developing countries. Although there is a possibility of foreign body reaction and later graft extrusion, a large series with long-term follow-up is required to confirm the suitability of acrylic ribs. However, we believe that prefabricated acrylic ribs for skeletal support during chest wall reconstruction offer an inexpensive and dependable alternative to many other expensive prosthetic materials.
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References
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Abstract
Introduction
