Fibrous Dysplasia of Ribs and Spine: Multidisciplinary Solution – a Case Report

Czech version

Authors: P. Habal ¹; K. Kaltofen ²; R. Kostyšyn ²; M. Štětina ¹; V. Málek ²
Authors‘ workplace: LF UK a FaN Hradec Králové Kardiochirurgická klinika ¹; LF UK a FaN Hradec Králové Neurochirurgická klinika ²
Published in: Cesk Slov Neurol N 2010; 73/106(6): 734-737
Category: Case Report

Overview

By relating the case history of a 56-year-old patient with multicentric fibrous dysplasia of the 5th and 6th ribs, the authors outline treatment procedures employed by both a thoracic surgeon and a neurosurgeon. A tumour was compressing the right lung and causing breathlessness by creating pleural effusions. One part of the tumour penetrated into the canal of the vertebral column and compressed the spinal cord. The patient had been examined in various departments; a surgical solution was suggested after spastic paraparesis of the lower limbs developed. The acute phase of spinal cord compression was relieved by laminectomy. In the second phase, the tumour was extirpated from the thoracic wall, with resection of the affected parts of the ribs and thoracic vertebrae. In the third phase, the spine was stabilized with plastic surgery to the thoracic wall. The tumour, measuring 60 × 110 × 80 mm, weighed 1,200 g. The large resection area gave rise to bleeding that was difficult to stop; local application of haemostat Traumacel TAF reduced the blood loss significantly. This result indicates that we may recommend surgical treatment in the case of enormous, benign tumours, with cooperation from exponents of other surgical specialities.

Key words:
thoracic wall tumours – benign fibrous dysplasia – lower paraparesis

Sources

1. Liechtenstein L. Bone tumors. 5th ed. The St. Louis: Mosby 1977: 270.

2. Harris WH, Dudley HR, Barry R. The natural history of fibrous dysplasia. An orthopaedic, pathological, and roentgenographic study. J Bone Point Surg 1962; 44A: 207–233.

3. Andrisano A, Soncini G, Calderoni PP, Stilli S. Critical review of infantile fibrous dysplasia: surgical treatment. J Ped Orthop 1991; 11(4): 478–481.

4. Ala-Kulju K, Ketonen P, Järvinen A, Salo J, Luosto R. Primary tumor sof the ribs. Scand J Thorac Cardiovasc Surg 1988; 22(2): 97–100.

5. Tresserra F, Rami R, Salas A, Domingo A, Forcada P, Gonzalez-Pont G et al. Polyostotic coexisting fibrous dysplasia and aneurysmal bone cyst of the chest wall. Thorac Cardiovasc Surg 1993; 41(5): 321–324.

6. Simpson AH, Creasy TS, Williamson DM, Wilson Dj, Spivey JS. Cystic degeneration of fibrous dysplasia masquerading as sarcoma. J Bone Joint Surg (Br) 1989; 71(3): 434–436.

7. Ruggieri P, Sim FH, Bond JR, Unni KK. Malignancies in fibrous dysplasia. Cancer 1994; 73(5): 1411–1424.

8. Karanjia ND, Sayer RE. Thoracic outlet syndrome due to monostotic fibrous dysplasia of the first rib. J R Coll Surg Edinb 1990; 35(2): 111.

9. Waller DA, Newman RJ. Primary bone tumours of the thoracic skeleton: an audit of the Leads regional bone tumours registry. Thorax 1990; 45: 850–855.

10. Turková L, Brtková J, Žižka J. Aneuryzmatická kostní cysta páteře v MR obrazu – diferenciální diagnostika – soubor kazuistik. Ces Radiol 2007; 61(3): 335–338.

11. Cavanaugh DG, Cabellon S jr, Peake JB. A logical approach to chest wall neoplazma. Ann Thorac Surg 1986; 41(4): 436–437.