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Heart, Lung and Circulation (2015) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.02.016
CLINICAL SPOTLIGHT
Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically Alexander C. Ziegler a, Gideon Karplus, MD b, Francis Serour, MD b, Michael Peer, MD c* a
Ohio State University, Columbus, Ohio, USA Department of Pediatric Surgery, Edith Wolfson Medical Center, Holon, affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel c Department of General Thoracic Surgery, Assaf Harofeh Medical Center, Zerifin, affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel b
Received 16 July 2014; received in revised form 14 January 2015; accepted 22 February 2015; online published-ahead-of-print xxx
A nine year-old boy presented with symptoms of dyspnoea. The chest radiograph and computed tomography scan revealed a large mediastinal tumour. A decision to operate on the patient was made. A huge 2.45 kg mediastinal lipoblastoma was successfully removed from the boy’s chest. At three years no evidence of recurrence was found. Keywords
Chest wall Mediastinal tumour Phrenic nerve Thoracotomy Tumour (mediastinal)
Introduction Lipoblastomas are rare benign tumours originating from embryonic white fat cells [1–5]. They constitute less than 1% of childhood neoplasms [3]. Approximately two-thirds are found in the extremities [1,2]. They grow variably, and in 90% of cases present in children younger than three years of age. Treatment of lipoblastomas involves complete surgical removal and close follow-up to monitor for recurrence [1–3,6]. Mediastinal lypoblastomas are extremely rare tumours and the largest reported case found weighed 996 gram in a six year-old boy [7]. We present the case of nine yearold boy with a large (2.45 kg) mediastinal lipoblastoma successfully removed by our paediatric and thoracic surgical teams.
Case Report A nine year-old boy presented to a regional hospital in Gaza (Palestinian Authority) exhibiting dyspnoea, and a respiratory
rate of up to 30 breaths a minute. The mother informed the staff that he had been complaining of shortness of breath for one year prior to the hospital visit. The physical examination revealed significant protrusion of the left chest with an absence of breath sounds, normal blood pressure, and tachycardia with a heart rate of up to 130 beats a minute. Chest radiography revealed total opacification of the left pleural cavity with a mediastinal shift to the right side. Pulmonary effusion was suspected and a chest tube was inserted into the left pleural cavity without penetrating the tumour capsule, but less than 100 cc of pleural effusion was drained without improvement on repeat chest X-ray (Fig. 1). At this point, the child was transferred to the Edith Wolfson Medical Center, Israel. At the Emergency Department, the patient underwent a computed tomography scan that revealed a huge intra-thoracic mass in the left chest, total left lung atelectasis and shifting of all mediastinal organs to the right side (Figure 1). Because of this, a decision was made not to perform a pulmonary function test and the child was transferred to the operating room.
*Corresponding author at: Department of General Thoracic Surgery, Assaf Harofeh Medical Center, Zerifin 70300, Israel. Tel.: +972 8 977-9822; fax: +972 8 977-8149, Emails: [email protected], [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: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
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Figure 1 Chest radiograph and computed tomography scan of the child show a huge left chest tumour.
Figure 2 Photographs of the huge left chest mass during removal from the thorax.
Please cite this article in press as: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
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Large Mediastinal Lipoblastoma Excision
Figure 3 Chest radiograph of the chest on discharge demonstrates sutures of the resected ribs using wires.
The surgery was performed under general anaesthesia with a single lumen endotracheal tube. Extended anteriorposteroior thoracotomy was performed, ribs #5 - #7 were transected and the chest was opened by two retractors (Finochietto) in two directions. A single huge thoracic tumour shifting all intrathoracic organs was visible through the thoracotomy incision and the left phrenic nerve was stretched across the mass. The nerve was protected and the mediastinal pleura over the mass was cut in front of the phrenic nerve, after which the mass was simply delivered from the thoracic cavity (Figure 2). The tumour was disclosed as an intra-pericardial mass surrounded by a continuation of the pericardium and the mediastinal pleura, and the remaining connection of the tumour arising from the mediastinal pleura was severed. The tumour weighed 2.45 kg (Figure 2). A 24 French chest tube was placed in the chest, the ribs connected by metallic wires, and the chest closed. The patient was transported postoperatively to the Pediatric Surgical Department, the repeat chest films revealed total expansion of the left lung (Figure 3), on fourth postoperative day the chest tube was removed and on fifth postoperative day the boy was discharged home to Gaza. The follow-up today is complete, and three years after the surgery the child is in a good general health and disease free, according to clinical examinations and repeat chest X-rays
Comment A lipoblastoma is a rapidly growing, but benign tumour, which can cause severe medical problems by compressing
major organs [7]. Although some have recommended always using a thoracoscopic or staged removal approach [8], we decided according to our scans (Figure 1) that a minimally invasive method was contraindicated in this situation, generally due to the massive nature of the tumour. Therefore, an extended combined incision was performed. When evaluating the choice between open and thoracoscopic methods, these authors recommended a multitude of factors that should be taken into consideration, including the mass of the tumour, the location, and the type of tumour. Since lipoblastomas have a high recurrence rate, almost 25% even when the mass is completely removed [3], the literature regarding lipoblastomas usually recommends long-term follow up. Additionally, it can be noted that recommendations about length of follow-up vary between physicians. Recurrences have been reported and the ideal follow-up is unknown and will probably remain so, since the tumours are very rare [9]. In our case, the tumour was applying force on the heart, causing a cardiac shift, and shift of other mediastinal organs, and the left lung was completely non-functioning prior to surgical removal of the tumour. Once the tumour was removed, all mediastinal structures relocated to their physiologic places and the chest films returned to normal.
Conflicts of Interests There are no competing interests to declare.
Financial Support There was no external financial support.
References [1] Mentzel T, Calonje E, Fletcher CD. Lipoblastoma and lipoblastomatosis: a clinicopathological study of 14 cases. Histopathology 1993;23: 527–33. [2] Ko SF, Shieh CS, Shih TY, Hsiao CC, Ng SH, Lee TY, et al. Mediastinal lipoblastoma with intraspinal extension: MRI demonstration. Magn Reson Imaging 1998;16:445–8. [3] Chun YS, Kim WK, Park KW, Lee SC, Jung SE. Lipoblastoma. J Pediatr Surg 2001;36:905–7. [4] Kok KY, Telisinghe PU. Lipoblastoma: clinical features, treatment, and outcome. World J Surg 2010;34:1517–22. [5] Fallon SC, Brandt ML, Rodriquez JR, Vasudevan SA, Lopez ME, Hicks MJ, et al. Cytogenetic analysis in the diagnosis and management of lipoblastomas: results from a single institution. J Surg Res 2013;184: 341–6. [6] Hicks J, Dilley A, Patel D, Barrish J, Zhu SH, Brandt M. Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural, and cytogenetic features. Ultrastruc Pathol 2001;25: 321–33. [7] Thakur B, Shan ZC. Giant mediastinal lipoblastoma: a case report with review of the literature. Indian J Surg 2006;68:108–10. [8] Pederiva F, Zanazzo GA, Gregori M, Schleef J. Suprascapular lipoblastoma extending in to the thorax. APSP J Case Rep 2013;27:20. [9] Salem R, Zohd M, Njim L, Maazoun K, Jellali MA, Zrig A, et al. Lipoblastoma: a rare lesion in the differential diagnosis of childhood mediastinal tumors. J Pediatr Surg 2011;46:e21–3.
Please cite this article in press as: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
Heart, Lung and Circulation (2015) xx, 1–3 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.02.016
CLINICAL SPOTLIGHT
Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically Alexander C. Ziegler a, Gideon Karplus, MD b, Francis Serour, MD b, Michael Peer, MD c* a
Ohio State University, Columbus, Ohio, USA Department of Pediatric Surgery, Edith Wolfson Medical Center, Holon, affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel c Department of General Thoracic Surgery, Assaf Harofeh Medical Center, Zerifin, affiliated to the Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel b
Received 16 July 2014; received in revised form 14 January 2015; accepted 22 February 2015; online published-ahead-of-print xxx
A nine year-old boy presented with symptoms of dyspnoea. The chest radiograph and computed tomography scan revealed a large mediastinal tumour. A decision to operate on the patient was made. A huge 2.45 kg mediastinal lipoblastoma was successfully removed from the boy’s chest. At three years no evidence of recurrence was found. Keywords
Chest wall Mediastinal tumour Phrenic nerve Thoracotomy Tumour (mediastinal)
Introduction Lipoblastomas are rare benign tumours originating from embryonic white fat cells [1–5]. They constitute less than 1% of childhood neoplasms [3]. Approximately two-thirds are found in the extremities [1,2]. They grow variably, and in 90% of cases present in children younger than three years of age. Treatment of lipoblastomas involves complete surgical removal and close follow-up to monitor for recurrence [1–3,6]. Mediastinal lypoblastomas are extremely rare tumours and the largest reported case found weighed 996 gram in a six year-old boy [7]. We present the case of nine yearold boy with a large (2.45 kg) mediastinal lipoblastoma successfully removed by our paediatric and thoracic surgical teams.
Case Report A nine year-old boy presented to a regional hospital in Gaza (Palestinian Authority) exhibiting dyspnoea, and a respiratory
rate of up to 30 breaths a minute. The mother informed the staff that he had been complaining of shortness of breath for one year prior to the hospital visit. The physical examination revealed significant protrusion of the left chest with an absence of breath sounds, normal blood pressure, and tachycardia with a heart rate of up to 130 beats a minute. Chest radiography revealed total opacification of the left pleural cavity with a mediastinal shift to the right side. Pulmonary effusion was suspected and a chest tube was inserted into the left pleural cavity without penetrating the tumour capsule, but less than 100 cc of pleural effusion was drained without improvement on repeat chest X-ray (Fig. 1). At this point, the child was transferred to the Edith Wolfson Medical Center, Israel. At the Emergency Department, the patient underwent a computed tomography scan that revealed a huge intra-thoracic mass in the left chest, total left lung atelectasis and shifting of all mediastinal organs to the right side (Figure 1). Because of this, a decision was made not to perform a pulmonary function test and the child was transferred to the operating room.
*Corresponding author at: Department of General Thoracic Surgery, Assaf Harofeh Medical Center, Zerifin 70300, Israel. Tel.: +972 8 977-9822; fax: +972 8 977-8149, Emails: [email protected], [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: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
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A.C. Ziegler et al.
Figure 1 Chest radiograph and computed tomography scan of the child show a huge left chest tumour.
Figure 2 Photographs of the huge left chest mass during removal from the thorax.
Please cite this article in press as: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
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Large Mediastinal Lipoblastoma Excision
Figure 3 Chest radiograph of the chest on discharge demonstrates sutures of the resected ribs using wires.
The surgery was performed under general anaesthesia with a single lumen endotracheal tube. Extended anteriorposteroior thoracotomy was performed, ribs #5 - #7 were transected and the chest was opened by two retractors (Finochietto) in two directions. A single huge thoracic tumour shifting all intrathoracic organs was visible through the thoracotomy incision and the left phrenic nerve was stretched across the mass. The nerve was protected and the mediastinal pleura over the mass was cut in front of the phrenic nerve, after which the mass was simply delivered from the thoracic cavity (Figure 2). The tumour was disclosed as an intra-pericardial mass surrounded by a continuation of the pericardium and the mediastinal pleura, and the remaining connection of the tumour arising from the mediastinal pleura was severed. The tumour weighed 2.45 kg (Figure 2). A 24 French chest tube was placed in the chest, the ribs connected by metallic wires, and the chest closed. The patient was transported postoperatively to the Pediatric Surgical Department, the repeat chest films revealed total expansion of the left lung (Figure 3), on fourth postoperative day the chest tube was removed and on fifth postoperative day the boy was discharged home to Gaza. The follow-up today is complete, and three years after the surgery the child is in a good general health and disease free, according to clinical examinations and repeat chest X-rays
Comment A lipoblastoma is a rapidly growing, but benign tumour, which can cause severe medical problems by compressing
major organs [7]. Although some have recommended always using a thoracoscopic or staged removal approach [8], we decided according to our scans (Figure 1) that a minimally invasive method was contraindicated in this situation, generally due to the massive nature of the tumour. Therefore, an extended combined incision was performed. When evaluating the choice between open and thoracoscopic methods, these authors recommended a multitude of factors that should be taken into consideration, including the mass of the tumour, the location, and the type of tumour. Since lipoblastomas have a high recurrence rate, almost 25% even when the mass is completely removed [3], the literature regarding lipoblastomas usually recommends long-term follow up. Additionally, it can be noted that recommendations about length of follow-up vary between physicians. Recurrences have been reported and the ideal follow-up is unknown and will probably remain so, since the tumours are very rare [9]. In our case, the tumour was applying force on the heart, causing a cardiac shift, and shift of other mediastinal organs, and the left lung was completely non-functioning prior to surgical removal of the tumour. Once the tumour was removed, all mediastinal structures relocated to their physiologic places and the chest films returned to normal.
Conflicts of Interests There are no competing interests to declare.
Financial Support There was no external financial support.
References [1] Mentzel T, Calonje E, Fletcher CD. Lipoblastoma and lipoblastomatosis: a clinicopathological study of 14 cases. Histopathology 1993;23: 527–33. [2] Ko SF, Shieh CS, Shih TY, Hsiao CC, Ng SH, Lee TY, et al. Mediastinal lipoblastoma with intraspinal extension: MRI demonstration. Magn Reson Imaging 1998;16:445–8. [3] Chun YS, Kim WK, Park KW, Lee SC, Jung SE. Lipoblastoma. J Pediatr Surg 2001;36:905–7. [4] Kok KY, Telisinghe PU. Lipoblastoma: clinical features, treatment, and outcome. World J Surg 2010;34:1517–22. [5] Fallon SC, Brandt ML, Rodriquez JR, Vasudevan SA, Lopez ME, Hicks MJ, et al. Cytogenetic analysis in the diagnosis and management of lipoblastomas: results from a single institution. J Surg Res 2013;184: 341–6. [6] Hicks J, Dilley A, Patel D, Barrish J, Zhu SH, Brandt M. Lipoblastoma and lipoblastomatosis in infancy and childhood: histopathologic, ultrastructural, and cytogenetic features. Ultrastruc Pathol 2001;25: 321–33. [7] Thakur B, Shan ZC. Giant mediastinal lipoblastoma: a case report with review of the literature. Indian J Surg 2006;68:108–10. [8] Pederiva F, Zanazzo GA, Gregori M, Schleef J. Suprascapular lipoblastoma extending in to the thorax. APSP J Case Rep 2013;27:20. [9] Salem R, Zohd M, Njim L, Maazoun K, Jellali MA, Zrig A, et al. Lipoblastoma: a rare lesion in the differential diagnosis of childhood mediastinal tumors. J Pediatr Surg 2011;46:e21–3.
Please cite this article in press as: Ziegler AC, et al. Huge Mediastinal Lipoblastoma in a Nine Year-old Boy Successfully Removed Surgically. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.02.016
