Case Study
Endobronchial leiomyoma in an immunocompetent four-year-old female child
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(3) 356–358 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313478628 aan.sagepub.com
Pallav Gupta1, Riti Aggarwal1, Prashant Rijal2 and Krishan Chugh2
Abstract Pulmonary leiomyoma are uncommonly encountered benign mesenchymal neoplasms in children, usually found in immunosuppressed individuals in association with human immunodeficiency virus or Ebstein-Barr virus infection. We describe an interesting case of a 4-year-old immunocompetent girl who presented with pleural effusion and lung collapse secondary to endobronchial leiomyoma. She underwent a left thoracotomy and a left pneumonectomy for excision of the bronchial mass.
Keywords Bronchial Neoplasms, Child, Leiomyoma
Introduction Primary leiomyoma of the lung is extremely rare. It can be located within the lung parenchyma or present as an endobronchial or endotracheal mass. Endobronchial location accounts for approximately 33% of all pulmonary leiomyoma.1,2 These tumors usually present in adults and only one third of patients are under the age of 20 years.2 There is no gender predilection for endobronchial leiomyoma.2 In children, it is usually seen in immunosuppressed individuals following organ transplantation or in association with EbsteinBarr virus (EBV) or human immunodeficiency virus (HIV) infections.3 A limited number of cases has been described in the pediatric age group, with the majority being in immunosuppressed individuals. We describe an extremely unusual case of endobronchial leiomyoma in an immunocompetent 4-year-old female child presenting with fever and chest pain, without any evidence of EBV or HIV infection or any history of immunosuppressive medication.
complaints at a private clinic. Initially, her symptoms were relieved but recurred 2 days later when she again developed high-grade fever associated with chest and abdominal pain. Chest radiography showed left lung collapse with left-sided pleural effusion. Computed tomography confirmed the left lung collapse with leftsided pleural effusion, and also revealed enlarged paratracheal lymph nodes. The patient was referred to our hospital for further management. At presentation, she was afebrile. There was no pallor, clubbing, cyanosis, or peripheral lymphadenopathy. Her pulse was 82 beatsmin 1 and regular. Examination of the chest revealed decreased air entry on the left side. Cardiovascular and abdominal examinations were within normal limits. The total leukocyte count was increased (13,200/mL), the erythrocyte sedimentation rate was 15 mm in the 1st hour, and serum lactate dehydrogenase was raised (307 IUL 1). Serology for HIV was negative. Contrast-enhanced computed tomography showed a soft tissue mass in the left main
1
Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India Department of Pediatrics, Sir Ganga Ram Hospital, New Delhi, India
Case report
2
A 4-year-old girl presented to pediatric outpatient department of a tertiary care hospital with complaints of dry cough and high-grade fever for 15 days. Earlier, she had received symptomatic treatment for these
Corresponding author: Pallav Gupta, MD, PDCC, Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India-110060. Email: [email protected]
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
Gupta et al.
357
Figure 2. Microphotograph showing mildly anisomorphic oval to spindle-shaped cells arranged in fascicles. Hematoxylin and eosin stain, original magnification 100. Figure 1. Flexible bronchoscopy showing complete occlusion of the left main bronchus by a soft tissue mass, with a patent right main bronchus.
bronchus, with total lung collapse and mediastinal shift. Flexible bronchoscopy revealed a smooth-surfaced mass obliterating the lumen of the left main bronchus, with mild narrowing of the bronchus at the anterior segment of the right upper lobe (Figure 1). The patient underwent a left thoracotomy and a left pneumonectomy for excision of the bronchial mass. Her postoperative course was uneventful. Grossly, the left pneumonectomy specimen measured 10 8 3 cm. The pleural surface was focally congested. The cut surface was unremarkable. Intrabronchial growth comprised 4 nodular soft tissue pieces, together measuring 1.5 1 0.3 cm. Histopathological examination of the endobronchial mass showed a benign spindle-cell neoplasm. The tumor cells were arranged in fascicles and had elongated nuclei with blunt ends and scanty cytoplasm; necrosis and mitotic activity were not evident (Figure 2). On immunohistochemistry, the tumor cells were immunoreactive for vimentin and smooth muscle actin (Figure 3); they did not express EBV antigen. Based on histopathology and immunohistochemistry, a diagnosis of endobronchial leiomyoma was offered. Sections from the lung parenchyma were largely unremarkable.
Discussion Bronchial adenoma is the most frequently occurring primary endobronchial neoplasm in childhood. Primary bronchial leiomyoma is an uncommon benign neoplasm in children and is usually seen in middle-aged females. Its incidence in children is not well established because of the rarity of this lesion.
Figure 3. Microphotograph showing the tumor cells to be positive for smooth muscle actin. Original magnification 200.
The tumor arises from the smooth muscle layer of the bronchus.4–6 These tumors usually presents as solitary lesions in children but can rarely present as multifocal lesions.7,8 Those located within lung parenchyma are usually asymptomatic, whereas endobronchial leiomyomas present with nonspecific signs and symptoms such as fever, cough, recurrent chest infection, hemoptysis, and wheeze. The diagnosis of this benign neoplasm is often delayed because of its slow-growth pattern.9 These tumors are commonly associated with immunosuppression including a congenital immune deficiency; however, cases have also been described in immunocompetent individuals.8 There is no clear hypothesis on the development of endobronchial leiomyoma in immunocompetent patients, but an inappropriate immune response has been observed in EBV-infected patients developing leiomyoma. The tumor cells in leiomyoma have been shown to express the EBV antigen, EBNA-2.8
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
358
Asian Cardiovascular & Thoracic Annals 22(3)
In our case, there was no serologic evidence of HIV infection and the patient was not taking any immunosuppressive medications. Computed tomography of the thorax is considered the best imaging modality to localize an endobronchial mass, followed by flexible bronchoscopy along with diagnostic biopsy.10 Peripherally localized tumors are usually treated by wedge or segmental resections, but endobronchial leiomyoma require anatomical resections which may be in the form of lobectomy, sleeve lobectomy or pneumonectomy. Recently, a conservative approach using an endobronchial procedure, such as neodymium-doped yttrium aluminium garnet laser resection and argon plasma coagulation, has been described. These procedures have shown good short-term results with no mortality; however, sparse literature is available about long-term disease-free survival and follow-up of these patients.10–13 Recently, video-assisted thoracic surgical lobectomy for a benign endobronchial leiomyoma has also been described.9 In our case, pneumonectomy with excision of the mass was performed because the lesion was endobronchial in location. Endobronchial leiomyoma in immunocompetent children is extremely uncommon and should be considered in the differential diagnosis of segmental or total lung collapse because it is a potentially treatable condition with good results and no mortality. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Fell CD, Tremblay A, Michaud GC, Urbanski SJ. Electrocauterization of an endobronchial leiomyoma. J Bronchol 2005;12:181–3. Available at: http://journals. lww.com/bronchology/Abstract/2005/07000/ Electrocauterization_of_an_Endobronchial_Leiomyoma. 17.aspx. Accessed January 22, 2013.
2. White SH, Ibrahim NB, Forrester-Wood CP and Jeyasingham K. Leiomyomas of the lower respiratory tract. Thorax 1985; 40: 306–311. 3. Hatano M, Takada H, Nomura A, et al. Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency. Pediatr Pulmonol 2006; 41: 371–373. 4. Karnak I, Akc¸o¨ren Z and Senocak ME. Endobronchial leiomyoma in children. Eur J Pediatr Surg 2000; 10: 136–139. 5. Ozc¸elik U, Kotilog˘lu E, Go¨c¸men A, Senocak ME and Kiper N. Endobronchial leiomyoma: a case report. Thorax 1995; 50: 101–102. 6. Curtis JM, Lacey D, Smyth R and Carty H. Endobronchial tumours in childhood. Eur J Radiol 1998; 29: 11–20. 7. Allan JS. Rare solitary benign tumors of lung. Semin Thorac Cardiovasc Surg 2003; 15: 315–322. 8. User IR, Dogru D, Talim B, Orhan D and Karnak I. Endobronchial, pulmonary and liver leiomyomata in a child with primary Immune deficiency. Eur J Pediatr Surg 2010; 20: 423–425. 9. Bartosik W, Crowther S, Narski M and Fabre A. Video-assisted lobectomy for endobronchial leiomyoma. Interact Cardiovasc Thorac Surg 2011; 12: 313–315. 10. Okada S, Yamauchi H, Ishimori S, Satoh S, Sugawara H and Tanaba Y. Endoscopic surgery with flexible bronchoscopy and argon plasma coagulation for endobronchial tumors. J Thorac Cardiovasc Surg 2001; 121: 180–182. 11. Shah H, Garbe L, Nussbaum E, Dumon J, Chiodera P and Cavaliere S. Benign tumors of the tracheobronchial tree: endoscopic characteristic and role for laser resection. Chest 1995; 107: 1744–1751. 12. Ayabe H, Tsuji H, Tagawa Y, Tomita M, Tsuda N and Chen JI. Endobronchial leiomyoma: a case report treated by bronchoplasty and a review of the literature. Surg Today 1995; 25: 1057–1060. 13. Yamada H, Katoh O, Yamaguchi T, Natsuaki M and Itoch T. Intrabronchial leiomyoma treated by localized resection via bronchotomy and bronchoplasty. Chest 1987; 91: 283–285.
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
Endobronchial leiomyoma in an immunocompetent four-year-old female child
Asian Cardiovascular & Thoracic Annals 2014, Vol. 22(3) 356–358 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492313478628 aan.sagepub.com
Pallav Gupta1, Riti Aggarwal1, Prashant Rijal2 and Krishan Chugh2
Abstract Pulmonary leiomyoma are uncommonly encountered benign mesenchymal neoplasms in children, usually found in immunosuppressed individuals in association with human immunodeficiency virus or Ebstein-Barr virus infection. We describe an interesting case of a 4-year-old immunocompetent girl who presented with pleural effusion and lung collapse secondary to endobronchial leiomyoma. She underwent a left thoracotomy and a left pneumonectomy for excision of the bronchial mass.
Keywords Bronchial Neoplasms, Child, Leiomyoma
Introduction Primary leiomyoma of the lung is extremely rare. It can be located within the lung parenchyma or present as an endobronchial or endotracheal mass. Endobronchial location accounts for approximately 33% of all pulmonary leiomyoma.1,2 These tumors usually present in adults and only one third of patients are under the age of 20 years.2 There is no gender predilection for endobronchial leiomyoma.2 In children, it is usually seen in immunosuppressed individuals following organ transplantation or in association with EbsteinBarr virus (EBV) or human immunodeficiency virus (HIV) infections.3 A limited number of cases has been described in the pediatric age group, with the majority being in immunosuppressed individuals. We describe an extremely unusual case of endobronchial leiomyoma in an immunocompetent 4-year-old female child presenting with fever and chest pain, without any evidence of EBV or HIV infection or any history of immunosuppressive medication.
complaints at a private clinic. Initially, her symptoms were relieved but recurred 2 days later when she again developed high-grade fever associated with chest and abdominal pain. Chest radiography showed left lung collapse with left-sided pleural effusion. Computed tomography confirmed the left lung collapse with leftsided pleural effusion, and also revealed enlarged paratracheal lymph nodes. The patient was referred to our hospital for further management. At presentation, she was afebrile. There was no pallor, clubbing, cyanosis, or peripheral lymphadenopathy. Her pulse was 82 beatsmin 1 and regular. Examination of the chest revealed decreased air entry on the left side. Cardiovascular and abdominal examinations were within normal limits. The total leukocyte count was increased (13,200/mL), the erythrocyte sedimentation rate was 15 mm in the 1st hour, and serum lactate dehydrogenase was raised (307 IUL 1). Serology for HIV was negative. Contrast-enhanced computed tomography showed a soft tissue mass in the left main
1
Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India Department of Pediatrics, Sir Ganga Ram Hospital, New Delhi, India
Case report
2
A 4-year-old girl presented to pediatric outpatient department of a tertiary care hospital with complaints of dry cough and high-grade fever for 15 days. Earlier, she had received symptomatic treatment for these
Corresponding author: Pallav Gupta, MD, PDCC, Department of Pathology, Sir Ganga Ram Hospital, New Delhi, India-110060. Email: [email protected]
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
Gupta et al.
357
Figure 2. Microphotograph showing mildly anisomorphic oval to spindle-shaped cells arranged in fascicles. Hematoxylin and eosin stain, original magnification 100. Figure 1. Flexible bronchoscopy showing complete occlusion of the left main bronchus by a soft tissue mass, with a patent right main bronchus.
bronchus, with total lung collapse and mediastinal shift. Flexible bronchoscopy revealed a smooth-surfaced mass obliterating the lumen of the left main bronchus, with mild narrowing of the bronchus at the anterior segment of the right upper lobe (Figure 1). The patient underwent a left thoracotomy and a left pneumonectomy for excision of the bronchial mass. Her postoperative course was uneventful. Grossly, the left pneumonectomy specimen measured 10 8 3 cm. The pleural surface was focally congested. The cut surface was unremarkable. Intrabronchial growth comprised 4 nodular soft tissue pieces, together measuring 1.5 1 0.3 cm. Histopathological examination of the endobronchial mass showed a benign spindle-cell neoplasm. The tumor cells were arranged in fascicles and had elongated nuclei with blunt ends and scanty cytoplasm; necrosis and mitotic activity were not evident (Figure 2). On immunohistochemistry, the tumor cells were immunoreactive for vimentin and smooth muscle actin (Figure 3); they did not express EBV antigen. Based on histopathology and immunohistochemistry, a diagnosis of endobronchial leiomyoma was offered. Sections from the lung parenchyma were largely unremarkable.
Discussion Bronchial adenoma is the most frequently occurring primary endobronchial neoplasm in childhood. Primary bronchial leiomyoma is an uncommon benign neoplasm in children and is usually seen in middle-aged females. Its incidence in children is not well established because of the rarity of this lesion.
Figure 3. Microphotograph showing the tumor cells to be positive for smooth muscle actin. Original magnification 200.
The tumor arises from the smooth muscle layer of the bronchus.4–6 These tumors usually presents as solitary lesions in children but can rarely present as multifocal lesions.7,8 Those located within lung parenchyma are usually asymptomatic, whereas endobronchial leiomyomas present with nonspecific signs and symptoms such as fever, cough, recurrent chest infection, hemoptysis, and wheeze. The diagnosis of this benign neoplasm is often delayed because of its slow-growth pattern.9 These tumors are commonly associated with immunosuppression including a congenital immune deficiency; however, cases have also been described in immunocompetent individuals.8 There is no clear hypothesis on the development of endobronchial leiomyoma in immunocompetent patients, but an inappropriate immune response has been observed in EBV-infected patients developing leiomyoma. The tumor cells in leiomyoma have been shown to express the EBV antigen, EBNA-2.8
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
358
Asian Cardiovascular & Thoracic Annals 22(3)
In our case, there was no serologic evidence of HIV infection and the patient was not taking any immunosuppressive medications. Computed tomography of the thorax is considered the best imaging modality to localize an endobronchial mass, followed by flexible bronchoscopy along with diagnostic biopsy.10 Peripherally localized tumors are usually treated by wedge or segmental resections, but endobronchial leiomyoma require anatomical resections which may be in the form of lobectomy, sleeve lobectomy or pneumonectomy. Recently, a conservative approach using an endobronchial procedure, such as neodymium-doped yttrium aluminium garnet laser resection and argon plasma coagulation, has been described. These procedures have shown good short-term results with no mortality; however, sparse literature is available about long-term disease-free survival and follow-up of these patients.10–13 Recently, video-assisted thoracic surgical lobectomy for a benign endobronchial leiomyoma has also been described.9 In our case, pneumonectomy with excision of the mass was performed because the lesion was endobronchial in location. Endobronchial leiomyoma in immunocompetent children is extremely uncommon and should be considered in the differential diagnosis of segmental or total lung collapse because it is a potentially treatable condition with good results and no mortality. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
Conflicts of interest statement None declared.
References 1. Fell CD, Tremblay A, Michaud GC, Urbanski SJ. Electrocauterization of an endobronchial leiomyoma. J Bronchol 2005;12:181–3. Available at: http://journals. lww.com/bronchology/Abstract/2005/07000/ Electrocauterization_of_an_Endobronchial_Leiomyoma. 17.aspx. Accessed January 22, 2013.
2. White SH, Ibrahim NB, Forrester-Wood CP and Jeyasingham K. Leiomyomas of the lower respiratory tract. Thorax 1985; 40: 306–311. 3. Hatano M, Takada H, Nomura A, et al. Epstein-Barr virus-associated bronchial leiomyoma in a boy with cellular immunodeficiency. Pediatr Pulmonol 2006; 41: 371–373. 4. Karnak I, Akc¸o¨ren Z and Senocak ME. Endobronchial leiomyoma in children. Eur J Pediatr Surg 2000; 10: 136–139. 5. Ozc¸elik U, Kotilog˘lu E, Go¨c¸men A, Senocak ME and Kiper N. Endobronchial leiomyoma: a case report. Thorax 1995; 50: 101–102. 6. Curtis JM, Lacey D, Smyth R and Carty H. Endobronchial tumours in childhood. Eur J Radiol 1998; 29: 11–20. 7. Allan JS. Rare solitary benign tumors of lung. Semin Thorac Cardiovasc Surg 2003; 15: 315–322. 8. User IR, Dogru D, Talim B, Orhan D and Karnak I. Endobronchial, pulmonary and liver leiomyomata in a child with primary Immune deficiency. Eur J Pediatr Surg 2010; 20: 423–425. 9. Bartosik W, Crowther S, Narski M and Fabre A. Video-assisted lobectomy for endobronchial leiomyoma. Interact Cardiovasc Thorac Surg 2011; 12: 313–315. 10. Okada S, Yamauchi H, Ishimori S, Satoh S, Sugawara H and Tanaba Y. Endoscopic surgery with flexible bronchoscopy and argon plasma coagulation for endobronchial tumors. J Thorac Cardiovasc Surg 2001; 121: 180–182. 11. Shah H, Garbe L, Nussbaum E, Dumon J, Chiodera P and Cavaliere S. Benign tumors of the tracheobronchial tree: endoscopic characteristic and role for laser resection. Chest 1995; 107: 1744–1751. 12. Ayabe H, Tsuji H, Tagawa Y, Tomita M, Tsuda N and Chen JI. Endobronchial leiomyoma: a case report treated by bronchoplasty and a review of the literature. Surg Today 1995; 25: 1057–1060. 13. Yamada H, Katoh O, Yamaguchi T, Natsuaki M and Itoch T. Intrabronchial leiomyoma treated by localized resection via bronchotomy and bronchoplasty. Chest 1987; 91: 283–285.
Downloaded from aan.sagepub.com at University of Birmingham on June 9, 2015
