Osteosarcoma of the sternum

Case reports

90

6. Kurtz RJ, Heimann TM, Beck AR, Holt J. Mesenteric and retroperitoneal cysts. Ann Surg 1986; 203: 109-12. 7. Takiff H, Calabria R, Yin L, Stabile BE. Mesenteric cysts and intra-abdominal cystic lymphangiomas. Arch Surg 1985; 120: 1266-9. 8. Ackerman's Surgical Pathology (7th edn). Juan Rusai, 1989: 1125-6.

9. Novell R, Standsby G, Dick R. Executive distress. Br J Radiol 1991; 64: 67-8. 10. Davidson AM, Lowe JW, Da Costa P. Adenocarcinoma arising in a mucinous cystadenoma of the lung. Thorax 1992; 47: 129-30.

Acceptedfor publication 8 November 1995

Osteosarcoma of the sternum Yvonne L. Douglas*, Koos J. Meuzelaart, Berend van der Lei:l:, Betty Pras§ and Harald J. Hoekstra* Departments of*Surgical Oncology, tCardiothoracic Surgery, ~Plastic, Reconstructive and Hand Surgery, and §Radiation Oncology, Groningen University Hospital, Groningen, The Netherlands

Primary malignant sternal tumours are very rare. The most common malignant sternal tumour is a chondrosarcoma. Until now, controversies in the management of malignant sternal tumours were mainly caused by limited clinical experience. However, treatment of malignant sternal tumours should not differ from that of chest wall malignancies. In this paper a 74-year-old man with a kyphnscoliosis and an nstensarcoma of the sternum is described who received combined treatment modalities, consisting of surgical resection and reconstruction with a myocutaneons flap, followed by external beam radiotherapy. This treatment strategy is discussed.

Key words: sternum;

osteosarcoma; kyphosis; pulmonary function.

Case history

A 74-year-old healthy man with an enormous 'kyphoscoliosis' presented with a tender, rapidly growing, painless mass of I 0 x 12 cm overlying the sternum, reaching from the manubrium to the fifth rib. Radiogram, ultrasound, and computed tomography (CT) of the sternum demonstrated a calcified mass with central necrosis, fixed to the ventral layer of the sternum, and with little involvement of the sternum. Pre-operatively, lung function was studied using spirometry, and distant metastases were ruled out by means of CT scans of the lungs and a bone scan. An incisional biopsy of the tumour revealed an unspecified sarcoma. The tumour was resected en bloc with the middle third of the sternum and a part of the left third and fourth rib. The tumour appeared to invade the anterior mediastinum. The defect of the thoracic cage (10 x 18cm) was reinforced with a Marlex mesh. A right myocutaneous latissimus dorsi flap harvested from the right site was used to cover the Marlex reinforced defect. The donor defect was closed with a split skin graft. The post-operative course was uncomplicated. The patient was ventilated on CPAP for 20 h and could be extubated without problems. Histopathological examination revealed marginal resection of the tumour. Therefore, the patient was additionally irradiated with 70Gy external beam radiotherapy consisting of a combination of megavoltage photontherapy and electrons (35 fractions of 2 Gy). Now, after 2 years' follow-up, the patient is in good condition with no evidence of local recurrence or metastatic disease. The pulmonary function is equal to what it was preoperatively (Table I).

Discussion

Most sternal tumours are secondary tumours caused by metastases from malignant tumours of the breast, lung, kidney or thyroid) '5 Correspondence to: H. J. Hoekstra, Division of Surgical Ontology, Groningen University Hospital, P.O. Box 30.001, 9700 RB Groningen, The Netherlands.

Table 1. Spirometry (standards). Pre-operative; 2 weeks postoperative and I year post-operative

Pre-operative 2 weeks postoperative IVC (4.3) FEVI (3.1) FEVI/IVC (74%) FIV1 (4.2) FlY 1/IVC (100%) FEVI/FIYl (91%)

1 year postoperative

4.2 3.7 88% 3.4 80%

4.2 3.2 76% 3.6 85°,4,

3.6 3.2 88% 3.3 91%

88%

90%

97%

Primary sternal tumours are rare. Since benign sternal tumours are extremely rare, all sternal tumours must be considered malignant until proven otherwise.5 The most common primary malignant sternal tumour is the chondrosarcoma, whereas osteosarcoma is less frequent. ~-30steosarcoma of the sternum is radiologically characterized by osteolytic or osteosclerotic masses with calcifications) The prognosis depends on the development of distant metastases which occur in 68% of the patients, mostly in the lung. Therefore, primary osteosarcoma of the sternum should be considered as a systemic disease at presentationY Resection followed by systemic chemotherapy is recommended, giving an overall 5-year survival of 15%.7 Over the last decade, adjuvant polychemotherapy has already improved the disease-free survival in patients with osteosarcoma of the extremities?'90steosarcoma of the sternum is typically diagnosed in elderly patients and therefore adjuvant chemotherapy is generally contraindicated due to agerelated cardiac and renal insufficiency. So far, only 12 patients with primary osteosarcoma of the sternum have been described in the literature) '7'~°Undoubtedly, some more patients have been reported as unspecified as part of a large study group of chest wall neoplasms in which individual data are not mentioned. The majority of patients

Case reports with a sternal tumour complain of a mass, giving cosmetic or mechanical discomfort. Pain is a bad prognostic sign, often representing peri-ostal damage. 7 Important diagnostic means are conventional X-rays of the sternum, computed tomography (CT), Magnetic Resonance Imaging (MRI) and biopsy. In bony turnouts, usually an incisional or core bone biopsy is performed. The preoperative evaluation includes blood and urine analysis and spirometry. When the histological diagnosis is determined, multidisciplinary therapeutic planning, dependent on turnout type, grade and prognosis, is necessary. 6'" Anticipating an eventual operation, respiratory losses must be calculated.: When indicated, wide resection with a 2-5 cm margin for skin and a 4-5 cm margin for bony structures is acceptable) '7 Choice of reconstruction is based on location, size and thickness of the defect and condition and prognosis of the patient): Although primary closure is often preferred, and in particular after total sternectomy, skeletal and soft tissue reconstruction are necessary) 3 Nowadays, nearly all defects can be closed surgically. However, success is only optimal when there is minimal deterioration of pulmonary function. Meadows et al.~° reported minor changes of pulmonary mechanics in six patients after resection of sternum and manubrium. Also, there is one reported case of hemithoracectomy, pneumonectomy and forequarter amputation with tolerable pulmonary changes) 4 However, in case of (kypho)scoliosis, lung volumes are often reduced mainly due to a decreased compliance of the respiratory system and impaired development of the lung and thoracic cage) ~ In our opinion, it is noteworthy that this patient maintained a strictly normal pulmonary function, despite his severe thoracic kyphosis and the extensive surgical resection and radiation treatment.

Conclusion

Malignant primary sternal turnouts are rare. For each patient, multidisciplinary individual evaluation for the best treatment modalities is warranted. Therapeutic success is dependent on tumour type, grade, completeness of resection, possibilities for reconstruction and deterioration of pulmonary function. The patient reported here had an osteosarcoma with a severe kyphoscoliosis. Two years after resection, reconstruction and postoperative radiation, his pulmonary function has hardly changed and there are no signs of local recurrence or distant metastases.

91 References

I. Martini N, Huvos AG, Smith J, Beattie EJ. Primary malignant tumors of the sternum. Surg Gynaecol Obstet 1974; 138:391-5. 2. Gabbay S, Bennett RD, Amato J, Cherny EJ. Controversies in management of sternal tumors. Ann Thorac &lrg 1989; 48: 428-3 I. 3. Peabody CN. Chondrosarcoma of sternum. J Thorac Cardiovasc Surg 1971; 61: 636-40. 4. Cavanaugh DG, Cabellon S, Peake JB. A logical approach to chest wall neoplasms. Ann Thorac Surg 1986; 41: 436-7. 5. Boker SM, Cullen GM, Swank M, Just JF. Case report 593. Skeletal Radiol 1990; 19: 77-8. 6. Anderson BO, Burt ME. Chest wall neoplasms and their management. Ann Thorac Surg 1994; 58:1174-8 I. 7. Burt M. Primary malignant tumors of the chest wall: the Memorial Sloan-Kettering Cancer Center experience. Chest Surg Clin N Am 1994; 4: 137-54. 8. Rosen G, Caparros B, Huvos AG, et al. Preoperative chemotherapy for osteogenic sarcoma: selections of postoperative adjuvant chemotherapy based on the response of the primary tumor to preoperative chemotherapy. Cancer 1982; 49: 1221-30. 9. Winkler K, Beron G, Delling G, et al. Neoadjuvant chemotherapy of osteosarcoma: results of a randomized cooperative trial (COSS-82) with salvage chemotherapy based on histological tumor response. J Clin Oncol 1988; 6: 329-37. 10. Meadows JA, Staats BA, Pairolero PC, Rodarte JR, Arnold PhG. Effect of resection of the sternum and manubrium in conjunction with muscle transposition on pulmonary function. Mayo Clin Proc 1985; 60: 604-9. 11. Benfield JR. Primary chest wall tumors. Ann Thorac Surg 1985; 39: 1. 12. Pairolero PC, Arnold PG. Thoracic wall defects: surgical management of 205 consecutive patients. Mayo Clin Proc 1986; 61: 557-63. 13. Paris F, Blasco E, Tarazona V, Pastor G, Zarza AG, Padilla J. Total sternectomy for malignant disease. J Thorac Cardiovasc Surg 1980; 80: 459-62. 14. Kuhn JA, Wagman LD, Lorant JA, Grannis FW, Dunst M, Dougherty WR, Jacobs DI. Radical forequarter amputation with hemithoracectomy and free extended forearm flap: technical and physiologic considerations. Ann Surg Onco11994; 1: 353-9. 15. Kafer ER. Respiratory function in paralytic scoliosis. Am Rev Respir Dis 1974; 110: 450-7. Accepted for publication 9 October 1995

CORRESPONDENCE

Sh; I read with interest the paper by Querci della Rovere et al (Eur J Surg Oncol 1996; 22: 478-82) which helps with the difficult problem of effective excision of mammographic lesions through a cosmetically appropriate incision. The technique they outline is sound but they imply that the wire must be followed by the surgeon to locate the lesion. There is, however, an easier technique which we have used here in all cases over the last 5 years. This involves simply leaving the introducer needle in place over the C o o k wire. During the operation angulation of the hub of the needle enables the tip of the needle to be palpated within the breast tissue, enabling a cosmetically satisfactory placement of the incision. This manoeuvre can also be used to control

subsequent progress of the exploration even in the large breast and the approach need not be along the track of the wire. Manipulation has not, in our experience, led to displacement of the hook. Leaving the needle in situ has not increased discomfort for the patient between placement of the wire and the visit to the operating theatre. The hub of the needle does, however, need to be carefully protected with dressings and the technique is probably not appropriate if a patient is to be moved from one hospital to another between localization and surgery, or where there is a gap of more than a few hours between localization and surgery. A. E. Young MA, MChir, F R C S St Thomas' Hospital, Lambeth Palace Road, London S E I 7EH, U K