Presentation for review during the zaria thoracic club meeting On 17 th march 2011

1. RECONSTRUCTION OF THE CHESTWALL AFTER EXCISION OF A GIANT MALIGNANT PERIPHERAL NERVE SHEATH TUMOUR Presentation for review during the zaria thoracic club meeting On 17th march 2011 AHMADU BELLO UNIVERSITY TEACHING HOSPITAL,ZARIA,NIGERIA

2. AUTHORS • S.A. EDAIGBINI (FWACS)*, I.Z.DELIA (FWACS)*M.B.AMINU (FWACS)*. • A. IBRAHIM (FWACS) **, • M.O.A SAMAILA (FMCPath) ***,K.ABDULLAHI(MBBS)***, A.A.LIMAN (MBBS) *** • *Cardiothoracic Surgery Unit, Department of Surgery, Ahmadu Bello • University, Zaria. • **Division of plastic surgery, Dept of Surgery, Ahmadu Bello University, • Zaria. • ***Department of Pathology, Ahmadu Bello University Zaria.

3. ABSTRACT • Primary chest wall tumours are uncommon and constitute 0.2-2% of all tumours. Metastatic tumours and tumours of local extension are more common. Malignant Peripheral Nerve Sheath Tumour of the chestwall is even rarer and its incidence on the chestwall not stated in the literatures but the incidence in the general population is 0.0001% while the risk is approximately 4600 times higher in patients with neurofibromatosis 1and 3–13% of them will finally develop into Malignant Peripheral Nerve Sheath Tumour, usuallyafter latent periods of 10–20 years. Clinically, thesetumours are aggressive, locally invasive, and highly metastatic. Excision of giant chestwall tumour leaves a defect that is made good by reconstruction using either musculocutaneous flaps with or without a mesh. We report the case of a 24 year old boy who presented at the surgical outpatient clinic with seven months history of persistent left sided chest pain minimally relieved by analgesics, five months of cough and worsening dyspnoea and three months history of anterior chest swelling in the region of the left side of the manubrium. Following evaluation and investigations, the tumour was excised and the residual defect closed with methylmetacrylate sandwiched between two prolene meshes and overlaid with both Pectolralis Major muscles. The histology of the excised mass was reported as Malignant Peripheral Nerve Sheath Tumour. He made an uneventful postoperative recovery but died barely 3 months later from widespread pulmonary metastases. A review of the literature reveals that such tumours hardlyever reach such large size as in our case.

4. KEY WORDS • Malignant Peripheral Nerve Sheath Tumour, • chestwall, • Excision Reconstruction

5. INTRODUCTION • Primary CW tumours are uncommon and constitute 0.2-2% of all tumors1. • Liptay MJ, Fry WA. Malignant bone tumors of the chest wall. SeminThoracCardiovascSurg 1999;11:278-84.  • Metastatic tumours and tumours of local extension are more common.

6. Introduction continues... • Actual incidence MPNST of the CW is not stated in the literature • However incidence in the general population is 0.0001%. • The risk is approximately 4600 times higher in patients with neurofibromatosis type I

7. Introduction continues... • 3–13% of them will finally develop MPNST, usuallyafter latent periods of 10–20 years2. • Ducatman BS, Scheithauer BW, Piepgraas DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 1986;57:2006–21 • Previously known as malignant schwannoma, neurogenic sarcoma, and neurofibrosarcoma Arises from embryonic neural crestcells, which normally give rise to the autonomic nervous systems

8. Introduction continues... • Clinically, thesetumours are aggressive, locally invasive, and highly metastatic3. Barnosky B, Shulman L.D, Talwar A. Massive Intrathoracic Malignant Peripheral Nerve Sheath Tumor: With Tracheobronchial Obstruction. Chest, 2005: 128(4):412(S). • Excision of giant CW leaves a defect that is made good by reconstruction using musculocutaneous flaps with or without a mesh.

9. Introduction continues... • Review of the literature revealed that such tumours hardlyever reach such large size as in our case. • Herein we report not only the excision of this massive tumour but also reconstruction of the anterior chestwall thereafter.

10. CASE SUMMARY • N.M. was a 24 year old man. • Referred with 7 months history of persistent left sided chest pain minimally relieved by analgesics, • 5 months Hx of cough and worsening dyspnoea • 3 months Hx of anterior chest swelling in the region of the left side of the manubrium. • There was no fever or hemoptysis.

11. Case summary continues... EXAMINATION • At initial presentation he had a performance score of 90 % (Karnofsky). • The essential finding was that of a firm manubrial lump more to the left with normal overlying skin. It was neither tender nor warm. • The regional and other peripheral lymph nodes were not enlarged. • The intensity of the breath sound was only slightly diminished on the left side

12. CASE SUMMARY continues.... DIAGNOSIS • An initial impression of chondrosarcoma of the chestwall was entertained.

13. Case summary continues... INVESTIGATIONS • Chest x-ray- large radio-opaque shadow in the ant-superior mediastinum. • Chest CT-Scan: done 2mons after first visit • 4 months to get funds for surgery by which time the tumour was exceedingly large and he was now dyspnoeic at rest. • Initial Tru-cut needle biopsy was not representative, so we offered him surgical excision.

14. Case summary continues... TREATMENT • At surgery, the tumour was found to have invaded the whole of the upper third of the sternum with a dumb-bell shape. • The outer and smaller portion (manubriosternal) measured 8cm x12cmx14cm • The larger half wholly intrathoracic (left side) measured 18cmx24cm x30cm in widest dimensions and weighed 4.5kg

15. Case summary continues... • The tumour was completely excised en-bloc along with the medial thirds of the clavicles, the upper half of the sternum and the upper 4 costal cartilages. • Total left parietal pleurectomy was achieved. • The pericardium was not invaded and mediastinal lymph nodes were not enlarged.

16. Case summary continues... • The resulting defect created was reconstructed with methylmetacrylate sandwiched between two 15cm x 15cm prolene meshes (figure 2). • The pectoralis major muscles were mobilised and used to cover the methylmetacrylate/prolene sandwich.

17. Case summary ends • He made an uneventful recovery postoperatively. • The histology revealed a MPNST with incomplete resection margins . • He was discharged two weeks postoperative and was seen at the outpatient clinic two later.

18. DISCUSSION • The treatment of large chest wall tumours was limited for many years until 1898 when Parham described the first thoracic resection of a chest neoplasm4. Parham FW. Thoracic resection for tumor growing from the bony wall of the chest. Trans SurgGynecol Assoc 1898; 11:223-363.

19. Discussion continues... • Initially the risks of surgery were related to pneumothorax and respiratory failures5. • Advances in anaesthesia and controlled airway ventilation with positive pressure allowed chest wall resections to be done safely. Guerrissi J.O, Brunini J.L. Large lateral thoracic defect by chondrosarcoma resection chest wall reconstruction using myocutaneouslatissimus dorsal flap without parietal rigid repair. Indian J PlastSurg 2005; 38:43-7.

20. Discussion continues... • Early attempts at reconstruction included the use of fascia lata and rib grafts6.

21. Discussion continues... • Advances in anaesthesia and controlled airway ventilation with positive pressure allowed chest wall resections to be done safely. • Early attempts at reconstruction included the use of fascia lata, rib grafts6 Bisgard JD, Swenson SA Jr. Tumors of sternum. Arch Surg 1948; 56:570-7.

22. Discussion continues... • As early as 1960, Graham and Usher introduced the use of prosthetic materials when they described using Marlex mesh to repair defects of the chest wall.5

23. Discussion continues... • A combination of prosthetic materials and rotational flaps, with improvement in mechanical ventilation provide good cosmetic and functional results and short hospital stays. • Latissimusdorsimyocutaneous flap is the most used methods for extensive soft tissue defects in chest wall reconstruction, • But it is best suited for lateral defects5.

24. Discussion continues... • For our patient, we used both pectoralis muscles because of their proximity to the midline7. Novoa N, Benito P, Jiménez MF, Juan A, Aranda JL, Varela G. Reconstruction of chest wall defects after resection of large neoplasms: ten-year experience. Interact CardioVascThoracSurg 2005;4:250-255. • Chestwall reconstruction in our environment is a challenge because of late presentation as in our patient.

25. Discussion continues... • Other challenges include availability of prosthesis (mesh and methylmetacrylate). • The methylmetacrylate restores the rigidity of the chestwall • The mesh provides a scaffold for fibrous infiltration during healing.

26. Discussion continues... • Another challenge is affordability of these prosthesis even where there is the expertise; • This featured prominently in this patient as it took him over six months to source funds for his surgery(enough time for the tumour to more than double in size and metastasize).

27. Discussion continues... • The sum of these challenges serves to limit the amount of work done by thoracic surgeons and therefore the availability of literature in this regard (particularly in our locality).

28. Discussion continues... • To achieve cure as much possible, about 5cm (King et al had recommended 4cm for aggressive tumours)8margin was given during resection of the tumour. King RM, Pairolero PC, Trastek VF, Piehler JM, Payne WS, Bernatz PE. Primary chest wall tumors: factors affecting survival. Ann ThoracSurg 1986;41:597–601. • Including total left parietal pleurectomy as the tumour was adhered to the upper lateral and anterior parietal pleura and bilateral medial third claviculectomy.

29. This unfortunately was inadequate because the tumour disseminates hematogenously like most sarcomas. • Therefore while patient had an excellent postoperative recovery he died barely three months later from widespread pulmonary metastasis.

30. CT-Scanogram of mass

31. Chestwall appearance of lesion

32. At the beginning of excision

34. Suturing P. Majors together

36. Preparing the sandwich

37. Excised mass

38. Sandwiched mesh in position

39. Mobilised P. Major Muscles

40. Muscle Flap cover completed

41. Skin closure at end of surgery

42. Hand E stain x 400

43. Mason Trichrome

44. Patient sitting postop for BP check

45. conclusion • Chestwall reconstruction is a formidable challenge in our environment due to late presentation and therefore very advance diseases. • Affordability and availability of prosthesis also serve to limit the extent of work done by even the very experienced thoracic surgeons

46. REFRENCES • Liptay MJ, Fry WA. Malignant bone tumors of the chest wall. SeminThoracCardiovascSurg 1999;11:278-84.  • Ducatman BS, Scheithauer BW, Piepgraas DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 1986;57:2006–21 • Barnosky B, Shulman L.D, Talwar A. Massive Intrathoracic Malignant Peripheral Nerve Sheath Tumor: With Tracheobronchial Obstruction. Chest, 2005: 128(4):412(S). • Parham FW. Thoracic resection for tumor growing from the bony wall of the chest. Trans SurgGynecol Assoc 1898; 11:223-363. • Guerrissi J.O, Brunini J.L. Large lateral thoracic defect by chondrosarcoma resection chest wall reconstruction using myocutaneouslatissimus dorsal flap without parietal rigid repair. Indian J PlastSurg 2005; 38:43-7. • Bisgard JD, Swenson SA Jr. Tumors of sternum. Arch Surg 1948; 56:570-7. • Novoa N, Benito P, Jiménez MF, Juan A, Aranda JL, Varela G. Reconstruction of chest wall defects after resection of large neoplasms: ten-year experience. Interact CardioVascThoracSurg 2005;4:250-255. • King RM, Pairolero PC, Trastek VF, Piehler JM, Payne WS, Bernatz PE. Primary chest wall tumors: factors affecting survival. Ann ThoracSurg 1986;41:597–601.

47. THANK YOU