presentation for review during the zaria thoracic club meeting on 17 th march 2011

RECONSTRUCTION OF THE CHESTWALL AFTER EXCISION OF A GIANT MALIGNANT

PERIPHERAL NERVE SHEATH TUMOUR

Presentation for review during the zaria thoracic club meetingOn

17th march 2011AHMADU BELLO UNIVERSITY TEACHING HOSPITAL,ZARIA,NIGERIA

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.

AUTHORS

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 1 and 3–13% of them will finally develop into Malignant Peripheral Nerve Sheath Tumour, usually after latent periods of 10–20 years. Clinically, these tumours 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 hardly ever reach such large size as in our case.

ABSTRACT

Malignant Peripheral Nerve Sheath Tumour, chestwall, Excision Reconstruction

KEY WORDS

Primary CW tumours are uncommon and constitute 0.2-2% of all tumors1.

Liptay MJ, Fry WA. Malignant bone tumors of the chest wall. Semin Thorac Cardiovasc Surg 1999;11:278-84.

Metastatic tumours and tumours of local extension are more common.

INTRODUCTION

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

Introduction continues...

3–13% of them will finally develop MPNST, usually after 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 crest cells, which normally give rise to the autonomic nervous systems


Clinically, these tumours 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.


Review of the literature revealed that such tumours hardly ever 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.


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.

CASE SUMMARY

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

Case summary continues...

DIAGNOSIS An initial impression of chondrosarcoma of

the chestwall was entertained.

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.


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


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.


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.


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.

Case summary ends

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 Surg Gynecol Assoc 1898; 11:223-363.

DISCUSSION

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 myocutaneous latissimus dorsal flap without parietal rigid repair. Indian J Plast Surg 2005; 38:43-7.

Discussion continues...

Early attempts at reconstruction included the use of fascia lata and rib grafts6.


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.


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


A combination of prosthetic materials and rotational flaps, with improvement in mechanical ventilation provide good cosmetic and functional results and short hospital stays.

Latissimus dorsi myocutaneous flap is the most used methods for extensive soft tissue defects in chest wall reconstruction,

But it is best suited for lateral defects5.


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 CardioVasc Thorac Surg 2005;4:250-255.

Chestwall reconstruction in our environment is a challenge because of late presentation as in our patient.


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.


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).


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).


To achieve cure as much possible, about 5cm (King et al had recommended 4cm for aggressive tumours)8 margin 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 Thorac Surg 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.


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.

CT-Scanogram of mass

Chestwall appearance of lesion

At the beginning of excision

Suturing P. Majors together

Preparing the sandwich

Excised mass

Sandwiched mesh in position

Mobilised P. Major Muscles

Muscle Flap cover completed

Skin closure at end of surgery

Hand E stain x 400

Mason Trichrome

Patient sitting postop for BP check

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

conclusion

1. Liptay MJ, Fry WA. Malignant bone tumors of the chest wall. Semin Thorac Cardiovasc Surg 1999;11:278-84.

2. 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

3. Barnosky B, Shulman L.D, Talwar A. Massive Intrathoracic Malignant Peripheral Nerve Sheath Tumor: With Tracheobronchial Obstruction. Chest, 2005: 128(4):412(S).

4. Parham FW. Thoracic resection for tumor growing from the bony wall of the chest. Trans Surg Gynecol Assoc 1898; 11:223-363.

5. Guerrissi J.O, Brunini J.L. Large lateral thoracic defect by chondrosarcoma resection chest wall reconstruction using myocutaneous latissimus dorsal flap without parietal rigid repair. Indian J Plast Surg 2005; 38:43-7.

6. Bisgard JD, Swenson SA Jr. Tumors of sternum. Arch Surg 1948; 56:570-7. 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 CardioVasc Thorac Surg 2005;4:250-255.

8. King RM, Pairolero PC, Trastek VF, Piehler JM, Payne WS, Bernatz PE. Primary chest wall tumors: factors affecting survival. Ann Thorac Surg 1986;41:597–601.

REFRENCES

THANK YOU

presentation for review during the zaria thoracic club meeting on 17 th march 2011

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