HLC 1809 No. of Pages 3
CLINICAL SPOTLIGHT
Heart, Lung and Circulation (2015) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.02.015
Giant Cervico-mediastinal Well-differentiated Liposarcoma Ottavio Rena, MD, PhD a, Fabio Davoli, MD, PhD a*, Francesco Pia, [3_TD$IF]MD, Prof. b, Alberto Roncon, MD a, Esther Papalia, MD a, Caterina Casadio, MD[4_TD$IF], Prof. a a
Thoracic Surgery Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Novara, Italy ORL Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Novara, Italy
b
Received 9 July 2014; received in revised form 26 December 2014; accepted 22 February 2015; online published-ahead-of-print xxx
Liposarcomas are rare entities and those located in the neck and mediastinum are exceptional. We report the case of a 58 year-old Caucasian male who presented with dysphonia, mild dysphagia to solids, dyspnoea at exertion and cough due to a giant mass of the neck originating from the deep cervical space at C6 vertebral level spreading into the posterior mediastinum. The lesion, that was associated with a right vocal cord palsy, caused severe lateral displacement of the trachea and oesophagus. The case has been managed by complete surgical resection through a combined cervical and thoracic approach associated with a tracheostomy due to the risk of bilateral recurrent nerve dysfunction at the end of the intervention. The tumour was diagnosed as a encapsulated low-grade liposarcoma. Six months after surgery the tracheostomy was closed. At 24 months clinical and radiological follow-up the patient is doing well and disease free. Keywords
Neck Mediastinum Soft tissue tumour Liposarcoma Surgery
Introduction [1_TD$IF]Liposarcoma is the one of the most common soft tissue sarcomas in the adult with a reported incidence of 0.41 cases/100,000 person-year in the US [1]. The cervical or mediastinal liposarcomas are rare entities constituting the 2.7% of all liposarcomas and less than 1% of mediastinal tumours [1,2]. We report the case of a giant liposarcoma of the neck spreading into the thoracic inlet into the mediastinum with related symptoms.
Case Report A 58 year-old Caucasian male was referred to our hospital because of the recent onset of dysphonia, mild dysphagia to solids, dyspnoea on exertion and cough. Physical examination demonstrated a left-sided fullness in the neck and in the supraclavicular fossa. Right-sided shift of the trachea was
noted. Fiberoptic examination by the otorhinolaryngologist revealed a right vocal cord palsy. Chest X-ray was carried out demonstrating enlargement of the mediastinal shadow at the thoracic inlet. Barium swallow confirmed a latero-cervical and mediastinal mass pushing the oesophagus laterally with reduction of the oesophageal lumen at the upper third of the mediastinum with cranial enlargement. No endoluminal masses were demonstrated. Total body CT scanning revealed a large, lipoid welldefined mass extending from the deep cervical space in the neck at C6 vertebral level down through the posterior mediastinum 3 cm below the carina (Figure 1). The oesophagus was displaced laterally with cranial distension due to the compression by the tumour without signs of infiltration. The lesion had a cranio-caudal extension of 18 cm with a maximum transverse diameter of 9.4 cm. Combined cervical and thoracic surgical approach was planned. The cervical portion of the mass was mobilised through a left cervicotomy after careful dissection and
*Corresponding author at: Thoracic Surgery Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Corso Mazzini 18, 28100- Novara - Italy. Tel.: +39 3491597046; fax: +39 03213733578, Email: [email protected] © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
HLC 1809 No. of Pages 3
2
O. Rena et al.
Figure 1 CT scanning of the neck and chest demonstrating the lipoid mass displacing the trachea, the oesophagus and great vessels without direct invasion (a: sagittal view – b: coronal view).
separation from the neurovascular bundle of the neck and the cervical oesophagus with respect to the identified left recurrent laryngeal nerve. After complete mobilisation of the mass from the visceral structures of the neck down through the thoracic inlet, the patient was turned on the left lateral decubitus and a right lateral muscle-sparing mini-thoracotomy at the fourth intercostal space was carried out. The thoracic portion of the mass was mobilised after the incision of the mediastinal pleura with the ligation and section of the azygos vein arch and the careful identification of the oesophagus which was completely respected. The tumour was removed through the thoracic incision after its complete mobilisation. Tracheostomy was done at the end of the intervention because of the pre-existing right cord palsy and the risk of a nerve stupor on the left side during surgery. Cervical and thoracic drains were placed. The postoperative recovery was uneventful and the patient was discharged after seven days. Definitive pathological diagnosis was low-grade completely encapsulated liposarcoma (Figure 2). Six months after surgery a fiberoptic laryngoscopy was carried out confirming the right cord palsy without other dysfunctions; the tracheostomy was closed.
Discussion Primary liposarcomas of the mediastinum are rare entities originating from the primitive mesenchymal cells. Even if some cases have been occasionally detected, the great
proportion are symptomatic due to the invasion of local structures or because of large dimensions [2–4]. Every mediastinal compartment may be the origin of liposarcoma due to the ubiquitous distribution of soft tissue [2]. Some cases have been reported arising from the neck and subsequently spreading into the thoracic inlet, into the anterior or posterior mediastinum, along the fascial planes according to the primary site of origin. Dyspnoea on exertion,
Figure 2 The well-differentiated liposarcoma showing sheets of multivacuolated lipoblasts with atypical nuclei and interspersed arborising thin-walled vessels (haematoxylin/eosin 200X HPF section).
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
HLC 1809 No. of Pages 3
3
Giant cervico-mediastinal liposarcoma
chest pain and dysphonia are the most common referred symptoms [2–4]. At CT scanning, the appearance of mediastinal liposarcomas varies from a predominantly fat-containing mass to a solid one [5]. When the invasion of cervicomediastinal vessels at the thoracic inlet is suspected, MRI is better than CT in ruling out this condition [5]. As in all sites, the surgical removal is the treatment of choice for cervico-mediastinal liposarcomas. The best treatment is the complete removal of the tumour, [2] however partial excision or debulking may relieve the effect of compression of advanced or highly infiltrating tumours. Liposarcomas are well-known to have low sensitivity to radiotherapy and chemotherapy; furthermore post-radiation fibrosis of the mediastinum has been frequently described. Long-term survival has been described to be dependent on several factors: in particular small dimensions, encapsulated tumours and complete excision are factors positively
influencing prognosis [2]. Local recurrence is primarily related to the incomplete removal at the time of primary surgery.
References [1] Toro JR, Travis LB, Wu HJ, Zhu K, Fletcher CMD, Devesa SS. Incidence patterns of soft tissue sarcomas, regardless of primary site, in the Surveillance, Epidemiology and End Results program, 1978-2001: An analysis of 26,758 cases. Int J Cancer 2006;119:2922–30. [2] Burt M, Ihde JK, Hajdu SI, Smith JW, Bains MS, Downey R, et al. Primary sarcomas of the mediastinum: results of therapy. J Thorac Cardiovasc Surg 1998;115:671–80. [3] Kara M, Ozkan M, Dizbay SS, Kavukcu ST. Successful removal of a giant recurrent mediastinal liposarcoma involving both hemithoraces. Eur J Cardiothorac Surg 2001;20:647–9. [4] Punpale A, Pramesh CS, Jambhekar N, Mistry RC. Giant Mediastinal liposarcoma: a case report. Ann Thorac Cardiovasc Surg 2006;12:425–7. [5] Munk PL, Lee MJ, Janzen DL. Lipoma and liposarcoma: evaluation using CT and MR imaging. AJR Am J Roentgenol 1997;169:589–94.
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
CLINICAL SPOTLIGHT
Heart, Lung and Circulation (2015) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.02.015
Giant Cervico-mediastinal Well-differentiated Liposarcoma Ottavio Rena, MD, PhD a, Fabio Davoli, MD, PhD a*, Francesco Pia, [3_TD$IF]MD, Prof. b, Alberto Roncon, MD a, Esther Papalia, MD a, Caterina Casadio, MD[4_TD$IF], Prof. a a
Thoracic Surgery Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Novara, Italy ORL Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Novara, Italy
b
Received 9 July 2014; received in revised form 26 December 2014; accepted 22 February 2015; online published-ahead-of-print xxx
Liposarcomas are rare entities and those located in the neck and mediastinum are exceptional. We report the case of a 58 year-old Caucasian male who presented with dysphonia, mild dysphagia to solids, dyspnoea at exertion and cough due to a giant mass of the neck originating from the deep cervical space at C6 vertebral level spreading into the posterior mediastinum. The lesion, that was associated with a right vocal cord palsy, caused severe lateral displacement of the trachea and oesophagus. The case has been managed by complete surgical resection through a combined cervical and thoracic approach associated with a tracheostomy due to the risk of bilateral recurrent nerve dysfunction at the end of the intervention. The tumour was diagnosed as a encapsulated low-grade liposarcoma. Six months after surgery the tracheostomy was closed. At 24 months clinical and radiological follow-up the patient is doing well and disease free. Keywords
Neck Mediastinum Soft tissue tumour Liposarcoma Surgery
Introduction [1_TD$IF]Liposarcoma is the one of the most common soft tissue sarcomas in the adult with a reported incidence of 0.41 cases/100,000 person-year in the US [1]. The cervical or mediastinal liposarcomas are rare entities constituting the 2.7% of all liposarcomas and less than 1% of mediastinal tumours [1,2]. We report the case of a giant liposarcoma of the neck spreading into the thoracic inlet into the mediastinum with related symptoms.
Case Report A 58 year-old Caucasian male was referred to our hospital because of the recent onset of dysphonia, mild dysphagia to solids, dyspnoea on exertion and cough. Physical examination demonstrated a left-sided fullness in the neck and in the supraclavicular fossa. Right-sided shift of the trachea was
noted. Fiberoptic examination by the otorhinolaryngologist revealed a right vocal cord palsy. Chest X-ray was carried out demonstrating enlargement of the mediastinal shadow at the thoracic inlet. Barium swallow confirmed a latero-cervical and mediastinal mass pushing the oesophagus laterally with reduction of the oesophageal lumen at the upper third of the mediastinum with cranial enlargement. No endoluminal masses were demonstrated. Total body CT scanning revealed a large, lipoid welldefined mass extending from the deep cervical space in the neck at C6 vertebral level down through the posterior mediastinum 3 cm below the carina (Figure 1). The oesophagus was displaced laterally with cranial distension due to the compression by the tumour without signs of infiltration. The lesion had a cranio-caudal extension of 18 cm with a maximum transverse diameter of 9.4 cm. Combined cervical and thoracic surgical approach was planned. The cervical portion of the mass was mobilised through a left cervicotomy after careful dissection and
*Corresponding author at: Thoracic Surgery Unit, University of Eastern Piedmont, Azienda Ospedaliero-Universitaria ‘‘Maggiore della Carita`’’, Corso Mazzini 18, 28100- Novara - Italy. Tel.: +39 3491597046; fax: +39 03213733578, Email: [email protected] © 2015 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
HLC 1809 No. of Pages 3
2
O. Rena et al.
Figure 1 CT scanning of the neck and chest demonstrating the lipoid mass displacing the trachea, the oesophagus and great vessels without direct invasion (a: sagittal view – b: coronal view).
separation from the neurovascular bundle of the neck and the cervical oesophagus with respect to the identified left recurrent laryngeal nerve. After complete mobilisation of the mass from the visceral structures of the neck down through the thoracic inlet, the patient was turned on the left lateral decubitus and a right lateral muscle-sparing mini-thoracotomy at the fourth intercostal space was carried out. The thoracic portion of the mass was mobilised after the incision of the mediastinal pleura with the ligation and section of the azygos vein arch and the careful identification of the oesophagus which was completely respected. The tumour was removed through the thoracic incision after its complete mobilisation. Tracheostomy was done at the end of the intervention because of the pre-existing right cord palsy and the risk of a nerve stupor on the left side during surgery. Cervical and thoracic drains were placed. The postoperative recovery was uneventful and the patient was discharged after seven days. Definitive pathological diagnosis was low-grade completely encapsulated liposarcoma (Figure 2). Six months after surgery a fiberoptic laryngoscopy was carried out confirming the right cord palsy without other dysfunctions; the tracheostomy was closed.
Discussion Primary liposarcomas of the mediastinum are rare entities originating from the primitive mesenchymal cells. Even if some cases have been occasionally detected, the great
proportion are symptomatic due to the invasion of local structures or because of large dimensions [2–4]. Every mediastinal compartment may be the origin of liposarcoma due to the ubiquitous distribution of soft tissue [2]. Some cases have been reported arising from the neck and subsequently spreading into the thoracic inlet, into the anterior or posterior mediastinum, along the fascial planes according to the primary site of origin. Dyspnoea on exertion,
Figure 2 The well-differentiated liposarcoma showing sheets of multivacuolated lipoblasts with atypical nuclei and interspersed arborising thin-walled vessels (haematoxylin/eosin 200X HPF section).
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
HLC 1809 No. of Pages 3
3
Giant cervico-mediastinal liposarcoma
chest pain and dysphonia are the most common referred symptoms [2–4]. At CT scanning, the appearance of mediastinal liposarcomas varies from a predominantly fat-containing mass to a solid one [5]. When the invasion of cervicomediastinal vessels at the thoracic inlet is suspected, MRI is better than CT in ruling out this condition [5]. As in all sites, the surgical removal is the treatment of choice for cervico-mediastinal liposarcomas. The best treatment is the complete removal of the tumour, [2] however partial excision or debulking may relieve the effect of compression of advanced or highly infiltrating tumours. Liposarcomas are well-known to have low sensitivity to radiotherapy and chemotherapy; furthermore post-radiation fibrosis of the mediastinum has been frequently described. Long-term survival has been described to be dependent on several factors: in particular small dimensions, encapsulated tumours and complete excision are factors positively
influencing prognosis [2]. Local recurrence is primarily related to the incomplete removal at the time of primary surgery.
References [1] Toro JR, Travis LB, Wu HJ, Zhu K, Fletcher CMD, Devesa SS. Incidence patterns of soft tissue sarcomas, regardless of primary site, in the Surveillance, Epidemiology and End Results program, 1978-2001: An analysis of 26,758 cases. Int J Cancer 2006;119:2922–30. [2] Burt M, Ihde JK, Hajdu SI, Smith JW, Bains MS, Downey R, et al. Primary sarcomas of the mediastinum: results of therapy. J Thorac Cardiovasc Surg 1998;115:671–80. [3] Kara M, Ozkan M, Dizbay SS, Kavukcu ST. Successful removal of a giant recurrent mediastinal liposarcoma involving both hemithoraces. Eur J Cardiothorac Surg 2001;20:647–9. [4] Punpale A, Pramesh CS, Jambhekar N, Mistry RC. Giant Mediastinal liposarcoma: a case report. Ann Thorac Cardiovasc Surg 2006;12:425–7. [5] Munk PL, Lee MJ, Janzen DL. Lipoma and liposarcoma: evaluation using CT and MR imaging. AJR Am J Roentgenol 1997;169:589–94.
Please cite this article in press as: Rena O, et al. Giant Cervico-mediastinal Well-differentiated Liposarcoma. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.015
