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Video-assisted thoracic surgery resection for pediatric mediastinal neurogenic tumors
Journal of Pediatric Surgery (2012) 47, 1349–1353
www.elsevier.com/locate/jpedsurg
Video-assisted thoracic surgery resection for pediatric mediastinal neurogenic tumors Jose Carlos Fraga a,b,⁎, Steven Rothenberg c , Edward Kiely b , Agostino Pierro b a
Pediatric Thoracic Surgery Unit/Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre RS 90035-903, Brazil b Surgical Unit of the Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom c Pediatric Surgery Service, The Rocky Mountain Hospital For Children, Denver, CO 80218, USA Received 25 July 2011; revised 30 January 2012; accepted 31 January 2012
Key words: Mediastinal neurogenic tumors; Video-Assisted Thoracoscopic Surgery (VATS); Children
Abstract Background/Purpose: Video-assisted thoracoscopic surgery (VATS) resection of mediastinal neurogenic tumors is still controversial in children. The aim of this study was to review the cases of VATS resection of such tumors in children from 3 institutions located in different countries. Methods: This retrospective study included 17 children treated between July 1995 and February 2011. Medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, tumor recurrence, and mortality. Results: Thirteen (76.5%) males and 4 (23.5%) females were studied. Median age was 16 months (range, 10.6-60 months), and median weight was 11.9 kg (range, 9.3-27.4 kg). Ten children had neuroblastoma (58.8%), 4 had ganglioneuroma (23.5%), and 3 had ganglioneuroblastoma (17.7%). The median duration of the operation was 90 minutes (range, 45-180 minutes), with complete thoracoscopic resection in all cases. Two children (11.8%) developed Horner syndrome postoperatively. No deaths were reported, and no recurrence was noted during a median follow-up period of 16 months (range, 8.9-28.6 months). Conclusions: Video-assisted thoracoscopic surgery resection of mediastinal neurogenic tumors in children produced good results, with no recurrence and minimal postoperative complications. The major advantages of this approach are the avoidance of thoracotomy complications and the enhanced surgical accuracy provided by improved visualization. © 2012 Elsevier Inc. All rights reserved.
Intrathoracic neurogenic tumors originate in the posterior mediastinum and comprise about a third of all mediastinal tumors in children [1]. In this age group, 60% of posterior ⁎ Corresponding author. Pediatric Thoracic Surgery Unit/Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre RS 90035-903, Brazil. Tel.: +55 51 3359 8232. E-mail address: [email protected] (J.C. Fraga). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.01.067
mediastinal tumors are malignant, and neuroblastoma is the most common histologic type [2]. Video-assisted thoracic surgery (VATS) has been increasingly used for diagnosis and treatment of various thoracic diseases in children, especially after smaller endoscopic instruments have become available and endoscopic skills have improved [3,4]. In addition, the results obtained with VATS are similar to those obtained with thoracotomy, with
1350 the advantages of better visualization of mediastinal structures, less postoperative pain, reduced hospital stay, and excellent cosmetic results [5]. In addition, this minimally invasive approach can prevent the complications of classic thoracotomy, such as scoliosis, shoulder elevation, winged scapula (scapula alata), or chest wall asymmetry [6]. Many investigators have enthusiastically reported their experience with VATS for pediatric mediastinum neurogenic tumor resection [5,7-10]. Despite these benefits, the use of VATS to treat intrathoracic malignancies in childhood is still controversial [11]. The aim of this study was to review the results obtained with VATS in children with mediastinal neurogenic tumors in 3 tertiary-care hospitals located in different countries.
1. Methods A nonrandomized, retrospective review of all children with neurogenic tumors in the posterior mediastinum cavity operated by VATS at the Pediatric Surgery Service of The Rocky Mountain Hospital for Children (RMHC) in Denver, Colorado, the Great Ormond Street Hospital (GOSH) in London, United Kingdom, and at the Hospital de Clínicas de Porto Alegre (HCPA), Brazil, was performed. At each hospital, all pediatric (age 0-14 years) VATS procedures for posterior mediastinum tumors in the period from July 1995 to February 2011 were identified, and all cases were reviewed. The study was approved by the research ethics committees of each hospital. During the period of this study, the decision to use VATS or open surgery was made by the surgeon based on his/her own experience and confidence at GOSH and HCPA; at RMHC, all the patients were treated with VATS. Cases with abdominal neurogenic tumor or intraabdominal extension and those in whom surgical removal of tumor was not possible (submitted only to biopsy) were not included. The 3 institutions use similar criteria for chest tube use and removal and for hospital discharge in these cases [12,13]. The medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, postoperative follow-up time, tumor recurrence, and mortality. Tumor size was determined during surgery, but because these tumors are irregularly shaped, only the largest diameter was considered in this study. Tumors were staged using the International Neuroblastoma Staging System (INSS) [14]. Surgical resection by VATS was performed with the child placed in lateral decubitus or modified prone position, using general anesthesia and selective bronchial contralateral intubation in most cases. In older children, selective ventilation of the contralateral hemithorax was performed using a double lumen endotracheal tube, whereas younger children were submitted to mainstem bronchus intubation with
J.C. Fraga et al. uncuffed tube or bronchial blocker. It was not possible to do selective bronchial intubation in small children. The ipsilateral lung was collapsed by insertion of low-pressure carbon dioxide (3-5 mm Hg). Three or more trocars were arranged to form a triangle. Trocars ranged from 3 to 10 mm, depending on the size of the patient. The first trocar was placed in the midaxillary line, between the fifth and the seventh intercostal spaces, according to the location of the tumor. Depending on the preference of the surgeon, 0° or 30° optical lenses were used; to perform dissection, 2 other trocars were then inserted between the anterior and posterior axillary lines. The pleural tissue covering the mass was incised around the overall circumference of the lesion, and the tumor was released by dissection with scissors. The intercostal and vertebral vessels involved were occluded with clips, electrocautery, or other energy devices. Once fully released, the tumor was placed in a plastic bag and removed through an enlarged trocar site. In 1 child with a big ganglioneuroma tumor (18 cm), the tumor was morcellated in the plastic bag before removal. In some children, after the procedure was performed, a single chest tube was placed into the thoracic cavity. None of the children in this series required conversion to open surgical procedures. The Kolmogorov-Smirnov test was used to assess normality of data distribution. Because the distribution of quantitative data was not normal, quantitative results are described by median and interquartile range. Categorical results are described by frequency and percentage. Data were stored in Microsoft Excel 2008 spreadsheets (Microsoft, Redmond, WA) and analyzed using SPSS, version 14.0 (SPSS, Chicago, IL). The confidence interval was considered to be at 95%.
2. Results Seventeen children with mediastinal neurogenic tumors were operated by VATS: 11 at RMHC, 5 at GOSH, and 1 at HCPA (Table 1). Thirteen patients (76.5%) were male, and 4 (23.5%) were female. The tumor was located on the left hemithorax in 11 children (64.7%) and on the right side in 6 (35.3%). Tumor type was defined after surgical resection as neuroblastoma in 10 cases (58.8%), ganglioneuroma in 4 (23.5%), and ganglioneuroblastoma in 3 (17.7%). The median (interquartile range) age and weight of patients undergoing surgery was 16 months (range, 10.6-60 months) and 11.9 kg (range, 9.3-27.4 kg), respectively. Seven children (41.2%) had no clinical manifestations of the tumor, and diagnosis was made by chest radiography for assessment of upper respiratory tract infections. Table 2 shows the clinical manifestations in the remaining 10 children (48.8%). Coughing was the most common symptom. As for INSS staging, 4 tumors were classified as stage I (3 neuroblastomas and 1 ganglioneuroblastoma), 5 as stage II (3 neuroblastomas and 2 ganglioneuroblastomas), 3 as stage III (all neuroblastomas), and 1 as stage IV-S (neuroblastoma).
VATS resection for pediatric mediastinal neurogenic tumors Table 1
1351
Children with VATS resection of mediastinal tumor
Patient/ hospital
Tumor pathology
Age at surgery (mo)
Weight at surgery (kg)
No. of trocars
Tumor size (cm)
Surgery time (min)
Follow-up time (mo)
1/RMHC 2/RMHC 3/RMHC 4/RMHC 5/RMHC 6/RMHC 7/RMHC 8/RMHC 9/RMHC 10/RMHC 11/RMHC 12-GOSH 13/GOSH 14/GOSH 15/GOSH 16/GOSH 17/HCPA
NB NB GN GN GN NB GNB NB NB NB NB NB GN NB GNB NB GNB
11.00 16.00 108.00 36.00 144.00 12.00 60.00 10.00 0.25 9.00 48.00 8.70 91.17 28.37 139.57 10.57 16.00
8 10 30 19 42 9.3 23 10 3.2 8 32 8 25 27.4 55.1 10.7 11.9
3 3 3 3 4 3 3 3 3 3 3 3 4 3 3 3 4
4 5 6 8 18 5 6 3 3 3 8 2 6.4 2 5 2 2.4
30 40 30 40 240 65 120 90 45 80 90 140 180 200 180 125 180
48.00 48.00 12.00 6.00 16.00 6.00 24.00 6.00 6.00 48.00 48.00 28.63 28.20 23.47 9.53 8.97 2.00
NB indicates neuroblastoma; GN, ganglioneuroma; GNB, ganglioneuroblastoma.
There were no cases of tumor extension into the medullary canal. Preoperative chemotherapy was performed in 2 children diagnosed with neuroblastoma. The median tumor diameter measured during the procedure was 5 cm (range, 2-18 cm). The median operative time was 90 minutes (range, 45-180 minutes). According to the postsurgery pathology report, tumor resection was complete in all children. Postoperative thoracic drainage was used in 11 children (64.7%), with a median of removal of 2 days (1-2 days). In 6 children (35.3%), no thoracic drain was used. All tumors were removed using VATS, without conversion to any open surgical technique. The median duration of postoperative hospital stay was 3 days (range, 2-3 days). Regarding complications, Horner syndrome developed in 2 children (11.8%), one operated on for ganglioneuroblastoma, and the other, for ganglioneuroma. Both seemingly
Table 2 Clinical presentation of children with mediastinal neurogenic tumor operated by VATS (n = 17) Clinical manifestations (n/%)
RMHC (n) GOSH (n) HCPA (n)
Asymptomatic a (7/41.2) Cough (6/35.3) Horner syndrome (2/11.8) Sibilance (1/5.9) Dancing eye syndrome (1/5.9) Dyspnea (1/5.9) Thoracic pain (1/5.9) Abdominal pain (1/5.9) Growth deficit (1/5.9)
6 3 1 0 1
0 3 1 1 0
1 0 0 0 0
1 1 0 1
0 0 1 0
0 0 0 0
a
Diagnosis by chest x-ray for upper airway infection.
improved during the follow-up period; however, they presented with symptoms during the last postoperative assessment. In 2 other children with Horner syndrome at the moment of diagnosis, symptoms did not disappear postoperatively. Postoperative chemotherapy was performed in 6 children diagnosed with neuroblastoma; one of them required postoperative radiotherapy. No deaths or recurrence of intrathoracic neurogenic tumors was observed in the children undergoing VATS during a median follow-up period of 16 months (range, 8.928.6 months).
3. Comment Both thoracotomy and thoracoscopy may be used to treat thoracic neurogenic tumors. In adults, VATS is often the preferred method of surgical resection [9], especially because most of neurogenic tumors in adults is benign (only 5% are malignant). In that situation, the increased risk of postoperative thoracic pain caused by the incisions required by thoracotomy, usually with division of the latissimus dorsi or the serratus anterior muscles and rib spreading, is not justified. In children, however, 60% of posterior mediastinal tumors are malignant [7], and therefore, the use of VATS resection is still controversial. An inadequate surgical margin is feared, as well as the recurrence of the lesion within the thorax or at the thoracostomy tube insertion site [15]. However, the development of smaller and more accurate surgical instruments and the improvement of surgical techniques have translated into better results for complex thoracoscopic procedures in infants and children [7]. Even in the absence of prospective or randomized studies with
1352 children, the literature so far indicates that video-assisted surgery is highly effective for the removal of mediastinal neurogenic tumors in this age group [7,8,10,16,17]. Videoassisted thoracoscopic surgery is associated with low morbidity and shorter operative time, surgery time, and hospital stay when compared with the conventional surgical techniques [7]. As observed in our case series, VATS allowed complete resection and no recurrence at trocar sites, without any of the problems associated with open chest incision. Our study is the first to review cases from 3 institutions located in different countries and continents, and our findings corroborate those of previous studies. It should also be noted that, in the present case series, the outcome of VATS was good even in children with advanced stage malignant tumors, whereas the largest VATS-treated pediatric case series reported in the literature so far included only INSS stage 1 tumors [7]. Despite the absence of cases with extension of the tumor into the spinal cord, our results indicate that complete resection of malignant neurogenic tumors is possible even in patients with more advanced stage disease [5,8]. Video-assisted thoracoscopic surgery for mediastinal neurogenic tumor resection is preferentially performed by contralateral mainstem intubation. However, in a few cases with smaller tumors, it is possible to only use CO2 insufflation. The surgery starts with 3 trocars arranged as a triangle, 2 of them placed under direct vision. One or 2 more trocars may be used as required. The video camera may be repositioned in different trocars, providing views from various angles. Moreover, zoom magnification greatly improves the visualization, hence making the surgery easier and safer. A meticulous hemostatic procedure is essential for safe and complete surgical resection of intrathoracic neurogenic tumors; that may be accomplished using clips, electrocautery, or other energy device. When using electrocautery, heat dispersion must be monitored because it may cause postoperative surgical complications. Although VATS is the preferred technique for removing neurogenic tumors in adults, some authors do not recommend this method when the lesion is malignant or greater than 5 cm in length [18]. In our pediatric group, however, tumors measuring 6, 8, and even 18 cm were completely removed using VATS. Like others [10], we also believe that the size of intrathoracic neurogenic tumors is not a contraindication for VATS removal. Large tumors can be resected using 4 or more trocars. In such cases, we place the tumor in a bag before morcellation to prevent contamination. It should be noted that tearing the bag during morcellation may cause tumor dissemination into the thoracic cavity, and therefore, it should be strictly avoided. To ensure an adequate surgical margin, biopsies can be obtained from the resection site. Postoperative thoracic drainage was not used in all children, therefore showing that drainage is not routinely required after VATS resection of mediastinal lesions. The lack of thoracic drainage did not represent risk in the selected cases [5,13] and, in fact, may have prevented pain and decreased the risk of contamination.
J.C. Fraga et al. Similarly to other studies, the most common complication after VATS removal of intrathoracic neurogenic tumor in our study was Horner syndrome, with an incidence that is consistent with previous reports (3% to 29%) [5,7,8,10,19]. The occurrence of Horner syndrome in the postoperative period is commonly linked to the location of intrathoracic neurogenic tumors, especially the upper thorax, near the sympathetic chain, rather than to the type of surgical resection [5]. In our patients, Horner syndrome did not disappear, but it has not progressed in the follow-up period. In our study, surgical resection of the neurogenic tumor was considered complete based on the postoperative pathology report. In addition, no tumor recurrence was noted after a median follow-up of 16 months (range, 8.9-28.6 months). In addition to the surgical resection with tumor-free margins, the less aggressive biological behavior of these tumors located in the thorax has also contributed to a better prognosis regarding regression and aggressiveness [5,14,17]. There is no absolute contraindication for VATS resection in children with thoracic neurogenic tumor. Relative contraindications can be anatomical or physiologic. Adverse anatomic considerations include difficult access to the thoracic cavity because of a very large tumor, with potential for dissemination or recurrence of malignancy. The major physiologic obstacles to safe VATS in pediatric thoracic neurogenic tumors are abnormalities of cardiac output, difficulty to tolerate single lung ventilation, and/or thoracic CO2 insufflation and coagulopathy. Another important point is that inadequate equipment and/or insufficient training and experience of surgical staff can be considered as a contraindication to this advanced thoracoscopic surgery. In summary, this study demonstrated that the removal of intrathoracic neurogenic tumors by VATS in children is feasible and safe. Video-assisted thoracoscopic surgery can be used to remove both benign and malignant neurogenic tumors, even in advanced stages. The size of the neurogenic tumor was not a contraindication for thoracoscopic resection, which enabled complete removal of the tumor, without conversion to open surgery in any case. There was no tumor recurrence, and only minor complications were observed in the postoperative period. Our experience suggests that this minimally invasive approach is comparable with the open surgical technique, with the advantage of sparing the patient the complications inherent to any thoracotomy incision. However, VATS removal of mediastinal neurogenic tumor is an advanced thoracoscopic surgery and should be performed only by surgeons with sufficient training.
Acknowledgments We are thankful to the Research Incentive Fund (FIPE) of HCPA, which has financially aided this study, and to Drs Bahattin Aydogdu and Roberto Aufieri for their help in
VATS resection for pediatric mediastinal neurogenic tumors reviewing the medical records of children from Great Ormond Street Hospital for Children, London, United Kingdom.
References [1] Azarow KS, Pearl RH, Zurcher R, et al. Primary mediastinal masses. A comparison of adult and pediatric populations. J Thorac Cardiovasc Surg 1993;106:67-72. [2] King DR, Groner JI, Teich S. Mediastinal cysts and tumors. In: Ziegler MZ, Azizkhan RG, Weber TR, editors. Operative pediatric surgery. New York (NY): McGraw-Hill; 2003. p. 407-19. [3] Cani I, Anton-Pacheco JL, Garcia A, et al. Video-assisted thoracoscopic lobectomy in infants. Eur J Cardiothorac Surg 2006;29: 997-1000. [4] Rothenberg SS. Thoracoscopy in infants and children: the state of the art. J Pediatr Surg 2005;40:303-6. [5] Malek MM, Mollen KP, Kane TD, et al. Thoracic neuroblastoma: a retrospective review of our institutional experience with comparison of the thoracoscopic and open approaches to resection. J Pediatr Surg 2010;45:1622-6. [6] Kucukarslan N, Kirilmaz A, Arslan Y, et al. Muscle sparing thoracotomy in pediatric age: a comparative study with standard posterolateral thoracotomy. Pediatr Surg Int 2006;22:779-83. [7] Lacreuse I, Valla JS, Lagausie P, et al. Thoracoscopic resection of neurogenic tumors in children. J Pediatr Surg 2007;42:1725-8. [8] Petty JK, Bensard DD, Partrick DA, et al. Resection of neurogenic tumors in children: is thoracoscopy superior to thoracotomy? J Am Coll Surg 2006;203:699-707.
1353 [9] Liu HP, Yim APC, Wan J, et al. Thoracoscopic removal of intrathoracic neurogenic tumors: a combined Chinese experience. Ann Surg 2000;232:187-90. [10] Guye E, Lardy H, Piolat C, et al. Thoracoscopy and solid tumors in children: A Multicenter Study. J Laparoendosc Adv Surg Tech 2007; 17:825-9. [11] LaQuaglia MP, Rutigliano DN. Neuroblastoma and other tumors. In: Carachi R, Grosfeld JL, Azmy AF, editors. The surgery of childhood tumors. Berlin, DE: Springer-Verlag; 2008. p. 201-25. [12] Arul GS, Parikh DH, Narayanaswamy B. Posterior mediastinal tumours. In: Parikh DH, Crabbe DC, Auldist AW, Rothenberg SS, editors. Pediatric thoracic surgery. London, UK: Springer-Verlag; 2009. p. 235-50. [13] Ponsky TA, Rothenberg SS, Tsao K. Thoracoscopy in children: is a chest drain necessary? J Laparoendosc Adv Surg Tech 2009;19(Suppl 1):S23-5. [14] Suita S, Tajiri T, Sera Y. The characteristics of mediastinal neuroblastoma. Eur J Pediatr Surg 2000;10:353-9. [15] Hayes-Jordan AA, Daw NC, Furman WL, et al. Tumor recurrence at thoracostomy tube insertion sites: a report of two pediatric cases. J Pediatr Surg 2004;39:1565-7. [16] DeCou JM, Schlatter MG, Mitchell DS, et al. Primary thoracoscopic gross total resection of neuroblastoma. J Laparoendosc Adv Surg Tech 2005;15:470-3. [17] Fraga JC, Aydogdu B, Aufieri R, et al. Surgical treatment for pediatric mediastinal neurogenic tumors. Ann Thorac Surg 2010;90:413-8. [18] Zierold D, Halow KD. Thoracoscopic resection of posterior neurogenic tumors. Am Surg 1999;65:1129-33. [19] Kang CH, Kim YT, Jeon SH, et al. Surgical treatment of malignant mediastinal tumors in children. Eur J Cardiothorac Surg 2007;31: 725-30.
www.elsevier.com/locate/jpedsurg
Video-assisted thoracic surgery resection for pediatric mediastinal neurogenic tumors Jose Carlos Fraga a,b,⁎, Steven Rothenberg c , Edward Kiely b , Agostino Pierro b a
Pediatric Thoracic Surgery Unit/Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre RS 90035-903, Brazil b Surgical Unit of the Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 3JH, United Kingdom c Pediatric Surgery Service, The Rocky Mountain Hospital For Children, Denver, CO 80218, USA Received 25 July 2011; revised 30 January 2012; accepted 31 January 2012
Key words: Mediastinal neurogenic tumors; Video-Assisted Thoracoscopic Surgery (VATS); Children
Abstract Background/Purpose: Video-assisted thoracoscopic surgery (VATS) resection of mediastinal neurogenic tumors is still controversial in children. The aim of this study was to review the cases of VATS resection of such tumors in children from 3 institutions located in different countries. Methods: This retrospective study included 17 children treated between July 1995 and February 2011. Medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, tumor recurrence, and mortality. Results: Thirteen (76.5%) males and 4 (23.5%) females were studied. Median age was 16 months (range, 10.6-60 months), and median weight was 11.9 kg (range, 9.3-27.4 kg). Ten children had neuroblastoma (58.8%), 4 had ganglioneuroma (23.5%), and 3 had ganglioneuroblastoma (17.7%). The median duration of the operation was 90 minutes (range, 45-180 minutes), with complete thoracoscopic resection in all cases. Two children (11.8%) developed Horner syndrome postoperatively. No deaths were reported, and no recurrence was noted during a median follow-up period of 16 months (range, 8.9-28.6 months). Conclusions: Video-assisted thoracoscopic surgery resection of mediastinal neurogenic tumors in children produced good results, with no recurrence and minimal postoperative complications. The major advantages of this approach are the avoidance of thoracotomy complications and the enhanced surgical accuracy provided by improved visualization. © 2012 Elsevier Inc. All rights reserved.
Intrathoracic neurogenic tumors originate in the posterior mediastinum and comprise about a third of all mediastinal tumors in children [1]. In this age group, 60% of posterior ⁎ Corresponding author. Pediatric Thoracic Surgery Unit/Pediatric Surgery Service, Hospital de Clínicas de Porto Alegre, Porto Alegre RS 90035-903, Brazil. Tel.: +55 51 3359 8232. E-mail address: [email protected] (J.C. Fraga). 0022-3468/$ – see front matter © 2012 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2012.01.067
mediastinal tumors are malignant, and neuroblastoma is the most common histologic type [2]. Video-assisted thoracic surgery (VATS) has been increasingly used for diagnosis and treatment of various thoracic diseases in children, especially after smaller endoscopic instruments have become available and endoscopic skills have improved [3,4]. In addition, the results obtained with VATS are similar to those obtained with thoracotomy, with
1350 the advantages of better visualization of mediastinal structures, less postoperative pain, reduced hospital stay, and excellent cosmetic results [5]. In addition, this minimally invasive approach can prevent the complications of classic thoracotomy, such as scoliosis, shoulder elevation, winged scapula (scapula alata), or chest wall asymmetry [6]. Many investigators have enthusiastically reported their experience with VATS for pediatric mediastinum neurogenic tumor resection [5,7-10]. Despite these benefits, the use of VATS to treat intrathoracic malignancies in childhood is still controversial [11]. The aim of this study was to review the results obtained with VATS in children with mediastinal neurogenic tumors in 3 tertiary-care hospitals located in different countries.
1. Methods A nonrandomized, retrospective review of all children with neurogenic tumors in the posterior mediastinum cavity operated by VATS at the Pediatric Surgery Service of The Rocky Mountain Hospital for Children (RMHC) in Denver, Colorado, the Great Ormond Street Hospital (GOSH) in London, United Kingdom, and at the Hospital de Clínicas de Porto Alegre (HCPA), Brazil, was performed. At each hospital, all pediatric (age 0-14 years) VATS procedures for posterior mediastinum tumors in the period from July 1995 to February 2011 were identified, and all cases were reviewed. The study was approved by the research ethics committees of each hospital. During the period of this study, the decision to use VATS or open surgery was made by the surgeon based on his/her own experience and confidence at GOSH and HCPA; at RMHC, all the patients were treated with VATS. Cases with abdominal neurogenic tumor or intraabdominal extension and those in whom surgical removal of tumor was not possible (submitted only to biopsy) were not included. The 3 institutions use similar criteria for chest tube use and removal and for hospital discharge in these cases [12,13]. The medical charts were reviewed for collection of data on age, sex, histologic type of tumor, clinical manifestations, age and weight at surgery, tumor size, duration of thoracic drainage, surgical complications, postoperative follow-up time, tumor recurrence, and mortality. Tumor size was determined during surgery, but because these tumors are irregularly shaped, only the largest diameter was considered in this study. Tumors were staged using the International Neuroblastoma Staging System (INSS) [14]. Surgical resection by VATS was performed with the child placed in lateral decubitus or modified prone position, using general anesthesia and selective bronchial contralateral intubation in most cases. In older children, selective ventilation of the contralateral hemithorax was performed using a double lumen endotracheal tube, whereas younger children were submitted to mainstem bronchus intubation with
J.C. Fraga et al. uncuffed tube or bronchial blocker. It was not possible to do selective bronchial intubation in small children. The ipsilateral lung was collapsed by insertion of low-pressure carbon dioxide (3-5 mm Hg). Three or more trocars were arranged to form a triangle. Trocars ranged from 3 to 10 mm, depending on the size of the patient. The first trocar was placed in the midaxillary line, between the fifth and the seventh intercostal spaces, according to the location of the tumor. Depending on the preference of the surgeon, 0° or 30° optical lenses were used; to perform dissection, 2 other trocars were then inserted between the anterior and posterior axillary lines. The pleural tissue covering the mass was incised around the overall circumference of the lesion, and the tumor was released by dissection with scissors. The intercostal and vertebral vessels involved were occluded with clips, electrocautery, or other energy devices. Once fully released, the tumor was placed in a plastic bag and removed through an enlarged trocar site. In 1 child with a big ganglioneuroma tumor (18 cm), the tumor was morcellated in the plastic bag before removal. In some children, after the procedure was performed, a single chest tube was placed into the thoracic cavity. None of the children in this series required conversion to open surgical procedures. The Kolmogorov-Smirnov test was used to assess normality of data distribution. Because the distribution of quantitative data was not normal, quantitative results are described by median and interquartile range. Categorical results are described by frequency and percentage. Data were stored in Microsoft Excel 2008 spreadsheets (Microsoft, Redmond, WA) and analyzed using SPSS, version 14.0 (SPSS, Chicago, IL). The confidence interval was considered to be at 95%.
2. Results Seventeen children with mediastinal neurogenic tumors were operated by VATS: 11 at RMHC, 5 at GOSH, and 1 at HCPA (Table 1). Thirteen patients (76.5%) were male, and 4 (23.5%) were female. The tumor was located on the left hemithorax in 11 children (64.7%) and on the right side in 6 (35.3%). Tumor type was defined after surgical resection as neuroblastoma in 10 cases (58.8%), ganglioneuroma in 4 (23.5%), and ganglioneuroblastoma in 3 (17.7%). The median (interquartile range) age and weight of patients undergoing surgery was 16 months (range, 10.6-60 months) and 11.9 kg (range, 9.3-27.4 kg), respectively. Seven children (41.2%) had no clinical manifestations of the tumor, and diagnosis was made by chest radiography for assessment of upper respiratory tract infections. Table 2 shows the clinical manifestations in the remaining 10 children (48.8%). Coughing was the most common symptom. As for INSS staging, 4 tumors were classified as stage I (3 neuroblastomas and 1 ganglioneuroblastoma), 5 as stage II (3 neuroblastomas and 2 ganglioneuroblastomas), 3 as stage III (all neuroblastomas), and 1 as stage IV-S (neuroblastoma).
VATS resection for pediatric mediastinal neurogenic tumors Table 1
1351
Children with VATS resection of mediastinal tumor
Patient/ hospital
Tumor pathology
Age at surgery (mo)
Weight at surgery (kg)
No. of trocars
Tumor size (cm)
Surgery time (min)
Follow-up time (mo)
1/RMHC 2/RMHC 3/RMHC 4/RMHC 5/RMHC 6/RMHC 7/RMHC 8/RMHC 9/RMHC 10/RMHC 11/RMHC 12-GOSH 13/GOSH 14/GOSH 15/GOSH 16/GOSH 17/HCPA
NB NB GN GN GN NB GNB NB NB NB NB NB GN NB GNB NB GNB
11.00 16.00 108.00 36.00 144.00 12.00 60.00 10.00 0.25 9.00 48.00 8.70 91.17 28.37 139.57 10.57 16.00
8 10 30 19 42 9.3 23 10 3.2 8 32 8 25 27.4 55.1 10.7 11.9
3 3 3 3 4 3 3 3 3 3 3 3 4 3 3 3 4
4 5 6 8 18 5 6 3 3 3 8 2 6.4 2 5 2 2.4
30 40 30 40 240 65 120 90 45 80 90 140 180 200 180 125 180
48.00 48.00 12.00 6.00 16.00 6.00 24.00 6.00 6.00 48.00 48.00 28.63 28.20 23.47 9.53 8.97 2.00
NB indicates neuroblastoma; GN, ganglioneuroma; GNB, ganglioneuroblastoma.
There were no cases of tumor extension into the medullary canal. Preoperative chemotherapy was performed in 2 children diagnosed with neuroblastoma. The median tumor diameter measured during the procedure was 5 cm (range, 2-18 cm). The median operative time was 90 minutes (range, 45-180 minutes). According to the postsurgery pathology report, tumor resection was complete in all children. Postoperative thoracic drainage was used in 11 children (64.7%), with a median of removal of 2 days (1-2 days). In 6 children (35.3%), no thoracic drain was used. All tumors were removed using VATS, without conversion to any open surgical technique. The median duration of postoperative hospital stay was 3 days (range, 2-3 days). Regarding complications, Horner syndrome developed in 2 children (11.8%), one operated on for ganglioneuroblastoma, and the other, for ganglioneuroma. Both seemingly
Table 2 Clinical presentation of children with mediastinal neurogenic tumor operated by VATS (n = 17) Clinical manifestations (n/%)
RMHC (n) GOSH (n) HCPA (n)
Asymptomatic a (7/41.2) Cough (6/35.3) Horner syndrome (2/11.8) Sibilance (1/5.9) Dancing eye syndrome (1/5.9) Dyspnea (1/5.9) Thoracic pain (1/5.9) Abdominal pain (1/5.9) Growth deficit (1/5.9)
6 3 1 0 1
0 3 1 1 0
1 0 0 0 0
1 1 0 1
0 0 1 0
0 0 0 0
a
Diagnosis by chest x-ray for upper airway infection.
improved during the follow-up period; however, they presented with symptoms during the last postoperative assessment. In 2 other children with Horner syndrome at the moment of diagnosis, symptoms did not disappear postoperatively. Postoperative chemotherapy was performed in 6 children diagnosed with neuroblastoma; one of them required postoperative radiotherapy. No deaths or recurrence of intrathoracic neurogenic tumors was observed in the children undergoing VATS during a median follow-up period of 16 months (range, 8.928.6 months).
3. Comment Both thoracotomy and thoracoscopy may be used to treat thoracic neurogenic tumors. In adults, VATS is often the preferred method of surgical resection [9], especially because most of neurogenic tumors in adults is benign (only 5% are malignant). In that situation, the increased risk of postoperative thoracic pain caused by the incisions required by thoracotomy, usually with division of the latissimus dorsi or the serratus anterior muscles and rib spreading, is not justified. In children, however, 60% of posterior mediastinal tumors are malignant [7], and therefore, the use of VATS resection is still controversial. An inadequate surgical margin is feared, as well as the recurrence of the lesion within the thorax or at the thoracostomy tube insertion site [15]. However, the development of smaller and more accurate surgical instruments and the improvement of surgical techniques have translated into better results for complex thoracoscopic procedures in infants and children [7]. Even in the absence of prospective or randomized studies with
1352 children, the literature so far indicates that video-assisted surgery is highly effective for the removal of mediastinal neurogenic tumors in this age group [7,8,10,16,17]. Videoassisted thoracoscopic surgery is associated with low morbidity and shorter operative time, surgery time, and hospital stay when compared with the conventional surgical techniques [7]. As observed in our case series, VATS allowed complete resection and no recurrence at trocar sites, without any of the problems associated with open chest incision. Our study is the first to review cases from 3 institutions located in different countries and continents, and our findings corroborate those of previous studies. It should also be noted that, in the present case series, the outcome of VATS was good even in children with advanced stage malignant tumors, whereas the largest VATS-treated pediatric case series reported in the literature so far included only INSS stage 1 tumors [7]. Despite the absence of cases with extension of the tumor into the spinal cord, our results indicate that complete resection of malignant neurogenic tumors is possible even in patients with more advanced stage disease [5,8]. Video-assisted thoracoscopic surgery for mediastinal neurogenic tumor resection is preferentially performed by contralateral mainstem intubation. However, in a few cases with smaller tumors, it is possible to only use CO2 insufflation. The surgery starts with 3 trocars arranged as a triangle, 2 of them placed under direct vision. One or 2 more trocars may be used as required. The video camera may be repositioned in different trocars, providing views from various angles. Moreover, zoom magnification greatly improves the visualization, hence making the surgery easier and safer. A meticulous hemostatic procedure is essential for safe and complete surgical resection of intrathoracic neurogenic tumors; that may be accomplished using clips, electrocautery, or other energy device. When using electrocautery, heat dispersion must be monitored because it may cause postoperative surgical complications. Although VATS is the preferred technique for removing neurogenic tumors in adults, some authors do not recommend this method when the lesion is malignant or greater than 5 cm in length [18]. In our pediatric group, however, tumors measuring 6, 8, and even 18 cm were completely removed using VATS. Like others [10], we also believe that the size of intrathoracic neurogenic tumors is not a contraindication for VATS removal. Large tumors can be resected using 4 or more trocars. In such cases, we place the tumor in a bag before morcellation to prevent contamination. It should be noted that tearing the bag during morcellation may cause tumor dissemination into the thoracic cavity, and therefore, it should be strictly avoided. To ensure an adequate surgical margin, biopsies can be obtained from the resection site. Postoperative thoracic drainage was not used in all children, therefore showing that drainage is not routinely required after VATS resection of mediastinal lesions. The lack of thoracic drainage did not represent risk in the selected cases [5,13] and, in fact, may have prevented pain and decreased the risk of contamination.
J.C. Fraga et al. Similarly to other studies, the most common complication after VATS removal of intrathoracic neurogenic tumor in our study was Horner syndrome, with an incidence that is consistent with previous reports (3% to 29%) [5,7,8,10,19]. The occurrence of Horner syndrome in the postoperative period is commonly linked to the location of intrathoracic neurogenic tumors, especially the upper thorax, near the sympathetic chain, rather than to the type of surgical resection [5]. In our patients, Horner syndrome did not disappear, but it has not progressed in the follow-up period. In our study, surgical resection of the neurogenic tumor was considered complete based on the postoperative pathology report. In addition, no tumor recurrence was noted after a median follow-up of 16 months (range, 8.9-28.6 months). In addition to the surgical resection with tumor-free margins, the less aggressive biological behavior of these tumors located in the thorax has also contributed to a better prognosis regarding regression and aggressiveness [5,14,17]. There is no absolute contraindication for VATS resection in children with thoracic neurogenic tumor. Relative contraindications can be anatomical or physiologic. Adverse anatomic considerations include difficult access to the thoracic cavity because of a very large tumor, with potential for dissemination or recurrence of malignancy. The major physiologic obstacles to safe VATS in pediatric thoracic neurogenic tumors are abnormalities of cardiac output, difficulty to tolerate single lung ventilation, and/or thoracic CO2 insufflation and coagulopathy. Another important point is that inadequate equipment and/or insufficient training and experience of surgical staff can be considered as a contraindication to this advanced thoracoscopic surgery. In summary, this study demonstrated that the removal of intrathoracic neurogenic tumors by VATS in children is feasible and safe. Video-assisted thoracoscopic surgery can be used to remove both benign and malignant neurogenic tumors, even in advanced stages. The size of the neurogenic tumor was not a contraindication for thoracoscopic resection, which enabled complete removal of the tumor, without conversion to open surgery in any case. There was no tumor recurrence, and only minor complications were observed in the postoperative period. Our experience suggests that this minimally invasive approach is comparable with the open surgical technique, with the advantage of sparing the patient the complications inherent to any thoracotomy incision. However, VATS removal of mediastinal neurogenic tumor is an advanced thoracoscopic surgery and should be performed only by surgeons with sufficient training.
Acknowledgments We are thankful to the Research Incentive Fund (FIPE) of HCPA, which has financially aided this study, and to Drs Bahattin Aydogdu and Roberto Aufieri for their help in
VATS resection for pediatric mediastinal neurogenic tumors reviewing the medical records of children from Great Ormond Street Hospital for Children, London, United Kingdom.
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