- Email: [email protected]
Lymphangioleiomyomatosis in a Male
Ann Thorac Surg 2015;100:1105–7
of degenerate heart valves remains the standard procedure. In contrast to a transcatheter approach, a conventional operation to achieve hemodynamic stability by patch enlargement may have also been a feasible option for this patient. This could have been performed on a beating heart. Interestingly, this case begs the question of whether a Ross procedure would be indicated in a 76-year-old patient with isolated aortic stenosis. It is our view that a root replacement with a biological conduit would be an ample treatment option, limiting an emergent operation to only one valve prosthesis.
References
Lymphangioleiomyomatosis in a Male Kazuhiro Wakida, MD, Yui Watanabe, MD, PhD, Toshio Kumasaka, MD, PhD, Kuniaki Seyama, MD, PhD, Keiko Mitani, MT, CT (IAC), Tsubasa Hiraki, MD, PhD, Go Kamimura, MD, Toshiyuki Nagata, MD, Yoshihiro Nakamura, MD, PhD, and Masami Sato, MD, PhD Department of General Thoracic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima; Department of Pathology, Japanese Red Cross Medical Centre, Tokyo; Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, Tokyo; Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, Tokyo; and Department of Human Pathology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
We report a 17-year-old male with a histopathologic diagnosis of lymphangioleiomyomatosis after surgery for a pneumothorax. In general, lymphangioleiomyomatosis has been considered a female-specific disease. However, there are a few lymphangioleiomyomatosis cases reported in males, and our patient is the youngest case reported. Spontaneous pneumothorax occurs most commonly in males in their late teens and early twenties. Histopathologic diagnosis cannot always be performed in young males with pneumothorax. However, simple diagnosis should be avoided, and lymphangioleiomyomatosis should be considered as an underlying disease. This remarkable case Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
1105
provides new and valuable clinical insights into young male pneumothorax. (Ann Thorac Surg 2015;100:1105–7) Ó 2015 by The Society of Thoracic Surgeons
L
ymphangioleiomyomatosis (LAM) is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, or other organs [1]. As a result, multiple cystic lesions are formed in the lungs [1]. LAM affects women of reproductive age almost exclusively; therefore, cases in males are extremely rare. A 17-year-old male was aware of right chest pain, and a right pneumothorax was diagnosed. He was admitted to our hospital and underwent surgery for the pneumothorax. The operative findings revealed multiple pleural cysts of the lung (Fig 1A), and air leakage was seen from the pleural cyst of the right upper lobe. Therefore, a thoracoscopic partial resection of the right upper lobe was performed via three miniports to preserve respiratory muscles. The patient was diagnosed as LAM by the histopathologic and immunochemical examinations of the resected specimen (Figs 1B, 1C, and 1D). The patient had undergone a right nephrectomy for a renal tumor at the age of 13 years. A tuberous sclerosis was diagnosed at that time. Chest CT showed bilateral diffuse cysts (Fig 2). The postoperative pulmonary function test showed a restrictive ventilatory defect: vital capacity, 3,260 mL (62.7%); forced expiratory volume in 1 second (FEV1), 2,630 mL (66.4%); FEV1 / Forced vital capacity ratio (FEV1%), 77.2%; diffusing capacity (DLCO), 27.6 mL/ min/mm Hg/L (97.4%). His postoperative course was uneventful. He returned home with no recurrence of pneumothorax for 13 months.
Comment Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder with a birth incidence of 1:5800 [1]. TSC can affect nearly every organ system, with LAM being one of the pulmonary manifestations of TSC [1, 2]. TSC1 and TSC2 have been identified as the genes responsible for LAM [1–3]. Our patient received a diagnosis of tuberous sclerosis without genetic testing, and he had no family history; however, genetic testing may be required in the future if he wishes to have children. A previous study indicated that 13% of male TSC patients and 43% of female TSC patients who underwent CT had pulmonary cysts suspected as LAM [4]. This finding suggests that LAM can occur not only in females, but also in males with TSC. Accepted for publication Nov 24, 2014. Address correspondence to Dr Watanabe, Department of General Thoracic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8570, Japan; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.11.069
FEATURE ARTICLES
1. Ruel M, Chan V, B edard P, et al. Very long-term survival implications of heart valve replacement with tissue versus mechanical prostheses in adults <60 years of age. Circulation 2007;116(11 Suppl):I294–300. 2. McElhinney DB, Hellenbrand WE, Zahn EM, et al. “Shortand medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial.” Circulation 2010;122:507–16. 3. Cullen MW, Cabalka AK, Alli OO, et al. Transvenous, antegrade Melody valve-in-valve implantation for bioprosthetic mitral and tricuspid valve dysfunction: a case series in children and adults. JACC Cardiovasc Interv 2013;6:598–605. 4. Riede FT, D€ ahnert I. Implantation of a Melody valve in tricuspid position. Catheter Cardiovasc Interv 2012;80: 474–6.
CASE REPORT WAKIDA ET AL MALE LYMPHANGIOLEIOMYOMATOSIS
1106
CASE REPORT WAKIDA ET AL MALE LYMPHANGIOLEIOMYOMATOSIS
Ann Thorac Surg 2015;100:1105–7
FEATURE ARTICLES
Fig 1. (A) The operative findings revealed multiple pleural cysts on the lung (arrows). (B) The histopathologic findings showed scattered diffuse small nodules of aggregated LAM cells (hematoxylin and eosin staining, 10). Lymphatic hyperplasias were mild in these LAM nodules. (C, D) The immunohistologic findings showed positive cells for anti a-SMA and D2-40 antibodies, respectively (immunostaining, 10).
Birt-Hogg-Dub e syndrome (BHDS) can also cause pulmonary cysts, pneumothorax, or renal tumors in young men. However, there are many characteristic and histologic differences between BHDS and LAM. LAM always shows LAM cell proliferation in the cyst walls. Other investigators reported that FEV1 and DLCO in LAM patients decreased 75 9 mL and 0.69 0.07 mL/min/mm Hg annually on average, respectively [1]. In our case, careful follow-up was recommended because the patient already had restrictive ventilatory impairment despite minimally invasive thoracoscopic surgery and mild histopathologic changes. Recently, sirolimus was made available for the treatment of LAM. Sirolimus stabilized lung function, reduced serum VEGFD levels, and it was associated with a reduction in symptoms and improved quality of life in patients with LAM [5]. The therapy may improve prognosis of our patient. Usually, female LAM progresses during pregnancy under the influence of female hormones, including
estrogen. Although four male cases diagnosed with LAM histopathologically have been reported [2, 3, 6, 7], most of the patients were in their late thirties or older. Teenage male pneumothorax cases are extremely rare. The clinical courses during long-term follow-up are unknown. Lung transplantation [8] is an option for end-stage LAM in either males or females. Our patient may require lung transplantation in the future because he is young. We reported the youngest case of teenage male pneumothorax diagnosed with LAM histopathologically. LAM is erroneously considered to be a female-specific disease. Tissue diagnosis is not always possible in young male pneumothorax cases. However, our case suggests that we should avoid simple diagnosis and consider LAM as an underlying disease even in young teenage male pneumothorax cases if the tissue specimen can be obtained by surgery.
References
Fig 2. Chest computed tomography shows bilateral diffuse cysts (arrows).
1. Taveira-DaSilva AM, Steagall WK, Moss J. Lymphangioleiomyomatosis. Cancer Control 2006;13:276–85. 2. Aubry MC, Myers JL, Ryu JH, et al. Pulmonary lymphangioleiomyomatosis in a man. Am J Respir Crit Care Med 2000; 162:749–52. 3. Schiavina M, Di Scioscio V, Contini P, et al. Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. Am J Respir Crit Care Med 2007;176:96–8. 4. Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol 2011;66:625–8. 5. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011;364:1595–606.
Ann Thorac Surg 2015;100:1107–9
CASE REPORT KIM ET AL BRONCHOSCOPY FOR LARGE ENDOBRONCHIAL HAMARTOMA
6. Kim NR, Chung MP, Park CK, Lee KS, Han J. Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man. Pathol Int 2003;53:231–5. 7. Miyake M, Tateishi U, Maeda T, et al. Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex. Radiat Med 2005;23:525–7. 8. Pigula FA, Griffith BP, Zenati MA, Dauber JH, Yousem SA, Keenan RJ. Lung transplantation for respiratory failure resulting from systemic disease. Ann Thorac Surg 1997;64: 1630–4.
Large Endobronchial Hamartoma Successfully Resected by Snare Through Flexible Bronchoscopy Jin Hyoung Kim, MD, Gyeong Min Jeong, MD, Kyung Hye Park, MD, Se Eun Han, MD, Jong Min Lee, MD, Chui Yong Park, MD, Seung Won Ra, MD, Kwang Won Seo, MD, Yangjin Jegal, MD, and Taehoon Lee, MD
Benign pulmonary tumors are rare, composing less than 1% of pulmonary tumors; of these, hamartomas are the most common. Endobronchial hamartomas are rarer still, composing 1.4% of all pulmonary hamartomas. As therapeutic bronchoscopy has improved, it has replaced conventional operations for resection of endobronchial benign tumors, although there are limitations to the procedure in the case of large tumors causing complete obstruction. The present report describes a case of a large endobronchial hamartoma inducing total lung atelectasis, which was successfully resected using an electrosurgical snare through flexible bronchoscopy. This could be a useful option in select endobronchial tumors. (Ann Thorac Surg 2015;100:1107–9) Ó 2015 by The Society of Thoracic Surgeons
B
enign lung tumors compose as few as 1% of all pulmonary neoplasms; the majority of primary lung tumors are malignant [1]. Among benign lung tumors, hamartoma is the most prevalent [2]. Hamartomas are usually located in the lung parenchyma, and endobronchial hamartomas compose only 1.4% of hamartomas [3]. Surgical resection is the traditional treatment for benign lung tumors, including hamartoma [1]. Endobronchial benign tumors, however, can be successfully treated using bronchoscopy, usually rigid bronchoscopy [2]. In the present report, we describe a case of a large endobronchial hamartoma that induced complete lung Accepted for publication Nov 17, 2014. Address correspondence to Dr Lee, Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, 877 Bangeojinsunhwan-doro, Dong-gu, Ulsan, 682-714, Korea; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
atelectasis. The patient was successfully treated by flexible bronchoscopy under conscious sedation using an electrosurgical snare. A 50-year-old male patient was examined emergently after a 3-week history of progressive dyspnea. He also complained of a mild cough and phlegm. He was a 30-pack/year current smoker and was occupied as a Buddhist monk. He was diagnosed with hypertension 10 years previously and with diabetes 5 years previously. On initial examination, he appeared acutely ill but was mentally alert. His initial vital signs were blood pressure, 124/90 mm Hg; heart rate, 80 beats per minute; respiration rate, 20 breaths per minute; and temperature, 36.5 C. Auscultation revealed decreased breathing sounds in the left lung. Chest radiography showed total consolidation of the left lung (Fig 1A). All laboratory test results were in the normal range except the C-reactive protein level, which was increased to 14 mg/dL. Chest computed tomography was performed and showed a tightly packed elongated endobronchial mass of soft tissue attenuation in the left main bronchus and total atelectasis of the left lung. Mediastinal lymphadenopathy and left pleural effusion were also noted (Fig 1B). Flexible bronchoscopy was then performed. A lobulated polypoid movable mass was identified approximately 2 cm from the carina, and the lumen of the left main bronchus was completely obstructed by the mass (Fig 1C). Histopathologic evaluation of a biopsied specimen suggested hamartoma. Endoscopic resection using an electrosurgical snare (symmetrical polypectomy snare, 20 40 mm; MTW, Wesel, Germany] through flexible bronchoscopy (BF260 video bronchoscope [outer diameter, 6 mm, channel diameter, 2 mm; Olympus, Southend-on-Sea, UK) was performed to obtain definitive diagnosis and treatment. With the patient under conscious sedation, the mass was resected in stages using the electrosurgical snarecutting method. Intraoperative hemorrhage was minimal and easily controlled by argon plasma coagulation (APC). Three sequential endoscopic treatments were performed at 2- to 3-day intervals over 10 days. The root of the mass was located at the spur of the lingular bronchus. The mass was completely excised until the lingular bronchial passage was completely open (Fig 2A). To prevent recurrence of the neoplasm, the mass root was cauterized with APC. Final pathologic evaluation of the entire mass was identical to the initial diagnosis and confirmed the mass as a hamartoma. No additional treatment was required, and the patient was discharged and followed at the outpatient clinic. Over several weeks, the atelectasis in the left gradually improved. A follow-up bronchoscopy performed 3 months postoperatively showed no residual tumor. A chest computed tomographic scan showed that the left lung atelectasis was completely resolved except at the lingula (Figs 2B, 2C). At 1 year postoperatively, the patient was doing well without any complications noted. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.11.063
FEATURE ARTICLES
Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Republic of Korea
1107
of degenerate heart valves remains the standard procedure. In contrast to a transcatheter approach, a conventional operation to achieve hemodynamic stability by patch enlargement may have also been a feasible option for this patient. This could have been performed on a beating heart. Interestingly, this case begs the question of whether a Ross procedure would be indicated in a 76-year-old patient with isolated aortic stenosis. It is our view that a root replacement with a biological conduit would be an ample treatment option, limiting an emergent operation to only one valve prosthesis.
References
Lymphangioleiomyomatosis in a Male Kazuhiro Wakida, MD, Yui Watanabe, MD, PhD, Toshio Kumasaka, MD, PhD, Kuniaki Seyama, MD, PhD, Keiko Mitani, MT, CT (IAC), Tsubasa Hiraki, MD, PhD, Go Kamimura, MD, Toshiyuki Nagata, MD, Yoshihiro Nakamura, MD, PhD, and Masami Sato, MD, PhD Department of General Thoracic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima; Department of Pathology, Japanese Red Cross Medical Centre, Tokyo; Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, Tokyo; Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, Tokyo; and Department of Human Pathology, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima, Japan
We report a 17-year-old male with a histopathologic diagnosis of lymphangioleiomyomatosis after surgery for a pneumothorax. In general, lymphangioleiomyomatosis has been considered a female-specific disease. However, there are a few lymphangioleiomyomatosis cases reported in males, and our patient is the youngest case reported. Spontaneous pneumothorax occurs most commonly in males in their late teens and early twenties. Histopathologic diagnosis cannot always be performed in young males with pneumothorax. However, simple diagnosis should be avoided, and lymphangioleiomyomatosis should be considered as an underlying disease. This remarkable case Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
1105
provides new and valuable clinical insights into young male pneumothorax. (Ann Thorac Surg 2015;100:1105–7) Ó 2015 by The Society of Thoracic Surgeons
L
ymphangioleiomyomatosis (LAM) is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes, or other organs [1]. As a result, multiple cystic lesions are formed in the lungs [1]. LAM affects women of reproductive age almost exclusively; therefore, cases in males are extremely rare. A 17-year-old male was aware of right chest pain, and a right pneumothorax was diagnosed. He was admitted to our hospital and underwent surgery for the pneumothorax. The operative findings revealed multiple pleural cysts of the lung (Fig 1A), and air leakage was seen from the pleural cyst of the right upper lobe. Therefore, a thoracoscopic partial resection of the right upper lobe was performed via three miniports to preserve respiratory muscles. The patient was diagnosed as LAM by the histopathologic and immunochemical examinations of the resected specimen (Figs 1B, 1C, and 1D). The patient had undergone a right nephrectomy for a renal tumor at the age of 13 years. A tuberous sclerosis was diagnosed at that time. Chest CT showed bilateral diffuse cysts (Fig 2). The postoperative pulmonary function test showed a restrictive ventilatory defect: vital capacity, 3,260 mL (62.7%); forced expiratory volume in 1 second (FEV1), 2,630 mL (66.4%); FEV1 / Forced vital capacity ratio (FEV1%), 77.2%; diffusing capacity (DLCO), 27.6 mL/ min/mm Hg/L (97.4%). His postoperative course was uneventful. He returned home with no recurrence of pneumothorax for 13 months.
Comment Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder with a birth incidence of 1:5800 [1]. TSC can affect nearly every organ system, with LAM being one of the pulmonary manifestations of TSC [1, 2]. TSC1 and TSC2 have been identified as the genes responsible for LAM [1–3]. Our patient received a diagnosis of tuberous sclerosis without genetic testing, and he had no family history; however, genetic testing may be required in the future if he wishes to have children. A previous study indicated that 13% of male TSC patients and 43% of female TSC patients who underwent CT had pulmonary cysts suspected as LAM [4]. This finding suggests that LAM can occur not only in females, but also in males with TSC. Accepted for publication Nov 24, 2014. Address correspondence to Dr Watanabe, Department of General Thoracic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8-35-1 Sakuragaoka, Kagoshima 890-8570, Japan; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.11.069
FEATURE ARTICLES
1. Ruel M, Chan V, B edard P, et al. Very long-term survival implications of heart valve replacement with tissue versus mechanical prostheses in adults <60 years of age. Circulation 2007;116(11 Suppl):I294–300. 2. McElhinney DB, Hellenbrand WE, Zahn EM, et al. “Shortand medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US melody valve trial.” Circulation 2010;122:507–16. 3. Cullen MW, Cabalka AK, Alli OO, et al. Transvenous, antegrade Melody valve-in-valve implantation for bioprosthetic mitral and tricuspid valve dysfunction: a case series in children and adults. JACC Cardiovasc Interv 2013;6:598–605. 4. Riede FT, D€ ahnert I. Implantation of a Melody valve in tricuspid position. Catheter Cardiovasc Interv 2012;80: 474–6.
CASE REPORT WAKIDA ET AL MALE LYMPHANGIOLEIOMYOMATOSIS
1106
CASE REPORT WAKIDA ET AL MALE LYMPHANGIOLEIOMYOMATOSIS
Ann Thorac Surg 2015;100:1105–7
FEATURE ARTICLES
Fig 1. (A) The operative findings revealed multiple pleural cysts on the lung (arrows). (B) The histopathologic findings showed scattered diffuse small nodules of aggregated LAM cells (hematoxylin and eosin staining, 10). Lymphatic hyperplasias were mild in these LAM nodules. (C, D) The immunohistologic findings showed positive cells for anti a-SMA and D2-40 antibodies, respectively (immunostaining, 10).
Birt-Hogg-Dub e syndrome (BHDS) can also cause pulmonary cysts, pneumothorax, or renal tumors in young men. However, there are many characteristic and histologic differences between BHDS and LAM. LAM always shows LAM cell proliferation in the cyst walls. Other investigators reported that FEV1 and DLCO in LAM patients decreased 75 9 mL and 0.69 0.07 mL/min/mm Hg annually on average, respectively [1]. In our case, careful follow-up was recommended because the patient already had restrictive ventilatory impairment despite minimally invasive thoracoscopic surgery and mild histopathologic changes. Recently, sirolimus was made available for the treatment of LAM. Sirolimus stabilized lung function, reduced serum VEGFD levels, and it was associated with a reduction in symptoms and improved quality of life in patients with LAM [5]. The therapy may improve prognosis of our patient. Usually, female LAM progresses during pregnancy under the influence of female hormones, including
estrogen. Although four male cases diagnosed with LAM histopathologically have been reported [2, 3, 6, 7], most of the patients were in their late thirties or older. Teenage male pneumothorax cases are extremely rare. The clinical courses during long-term follow-up are unknown. Lung transplantation [8] is an option for end-stage LAM in either males or females. Our patient may require lung transplantation in the future because he is young. We reported the youngest case of teenage male pneumothorax diagnosed with LAM histopathologically. LAM is erroneously considered to be a female-specific disease. Tissue diagnosis is not always possible in young male pneumothorax cases. However, our case suggests that we should avoid simple diagnosis and consider LAM as an underlying disease even in young teenage male pneumothorax cases if the tissue specimen can be obtained by surgery.
References
Fig 2. Chest computed tomography shows bilateral diffuse cysts (arrows).
1. Taveira-DaSilva AM, Steagall WK, Moss J. Lymphangioleiomyomatosis. Cancer Control 2006;13:276–85. 2. Aubry MC, Myers JL, Ryu JH, et al. Pulmonary lymphangioleiomyomatosis in a man. Am J Respir Crit Care Med 2000; 162:749–52. 3. Schiavina M, Di Scioscio V, Contini P, et al. Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. Am J Respir Crit Care Med 2007;176:96–8. 4. Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M. Radiological evidence of lymphangioleiomyomatosis in female and male patients with tuberous sclerosis complex. Clin Radiol 2011;66:625–8. 5. McCormack FX, Inoue Y, Moss J, et al. National Institutes of Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med 2011;364:1595–606.
Ann Thorac Surg 2015;100:1107–9
CASE REPORT KIM ET AL BRONCHOSCOPY FOR LARGE ENDOBRONCHIAL HAMARTOMA
6. Kim NR, Chung MP, Park CK, Lee KS, Han J. Pulmonary lymphangioleiomyomatosis and multiple hepatic angiomyolipomas in a man. Pathol Int 2003;53:231–5. 7. Miyake M, Tateishi U, Maeda T, et al. Pulmonary lymphangioleiomyomatosis in a male patient with tuberous sclerosis complex. Radiat Med 2005;23:525–7. 8. Pigula FA, Griffith BP, Zenati MA, Dauber JH, Yousem SA, Keenan RJ. Lung transplantation for respiratory failure resulting from systemic disease. Ann Thorac Surg 1997;64: 1630–4.
Large Endobronchial Hamartoma Successfully Resected by Snare Through Flexible Bronchoscopy Jin Hyoung Kim, MD, Gyeong Min Jeong, MD, Kyung Hye Park, MD, Se Eun Han, MD, Jong Min Lee, MD, Chui Yong Park, MD, Seung Won Ra, MD, Kwang Won Seo, MD, Yangjin Jegal, MD, and Taehoon Lee, MD
Benign pulmonary tumors are rare, composing less than 1% of pulmonary tumors; of these, hamartomas are the most common. Endobronchial hamartomas are rarer still, composing 1.4% of all pulmonary hamartomas. As therapeutic bronchoscopy has improved, it has replaced conventional operations for resection of endobronchial benign tumors, although there are limitations to the procedure in the case of large tumors causing complete obstruction. The present report describes a case of a large endobronchial hamartoma inducing total lung atelectasis, which was successfully resected using an electrosurgical snare through flexible bronchoscopy. This could be a useful option in select endobronchial tumors. (Ann Thorac Surg 2015;100:1107–9) Ó 2015 by The Society of Thoracic Surgeons
B
enign lung tumors compose as few as 1% of all pulmonary neoplasms; the majority of primary lung tumors are malignant [1]. Among benign lung tumors, hamartoma is the most prevalent [2]. Hamartomas are usually located in the lung parenchyma, and endobronchial hamartomas compose only 1.4% of hamartomas [3]. Surgical resection is the traditional treatment for benign lung tumors, including hamartoma [1]. Endobronchial benign tumors, however, can be successfully treated using bronchoscopy, usually rigid bronchoscopy [2]. In the present report, we describe a case of a large endobronchial hamartoma that induced complete lung Accepted for publication Nov 17, 2014. Address correspondence to Dr Lee, Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, 877 Bangeojinsunhwan-doro, Dong-gu, Ulsan, 682-714, Korea; e-mail: [email protected].
Ó 2015 by The Society of Thoracic Surgeons Published by Elsevier
atelectasis. The patient was successfully treated by flexible bronchoscopy under conscious sedation using an electrosurgical snare. A 50-year-old male patient was examined emergently after a 3-week history of progressive dyspnea. He also complained of a mild cough and phlegm. He was a 30-pack/year current smoker and was occupied as a Buddhist monk. He was diagnosed with hypertension 10 years previously and with diabetes 5 years previously. On initial examination, he appeared acutely ill but was mentally alert. His initial vital signs were blood pressure, 124/90 mm Hg; heart rate, 80 beats per minute; respiration rate, 20 breaths per minute; and temperature, 36.5 C. Auscultation revealed decreased breathing sounds in the left lung. Chest radiography showed total consolidation of the left lung (Fig 1A). All laboratory test results were in the normal range except the C-reactive protein level, which was increased to 14 mg/dL. Chest computed tomography was performed and showed a tightly packed elongated endobronchial mass of soft tissue attenuation in the left main bronchus and total atelectasis of the left lung. Mediastinal lymphadenopathy and left pleural effusion were also noted (Fig 1B). Flexible bronchoscopy was then performed. A lobulated polypoid movable mass was identified approximately 2 cm from the carina, and the lumen of the left main bronchus was completely obstructed by the mass (Fig 1C). Histopathologic evaluation of a biopsied specimen suggested hamartoma. Endoscopic resection using an electrosurgical snare (symmetrical polypectomy snare, 20 40 mm; MTW, Wesel, Germany] through flexible bronchoscopy (BF260 video bronchoscope [outer diameter, 6 mm, channel diameter, 2 mm; Olympus, Southend-on-Sea, UK) was performed to obtain definitive diagnosis and treatment. With the patient under conscious sedation, the mass was resected in stages using the electrosurgical snarecutting method. Intraoperative hemorrhage was minimal and easily controlled by argon plasma coagulation (APC). Three sequential endoscopic treatments were performed at 2- to 3-day intervals over 10 days. The root of the mass was located at the spur of the lingular bronchus. The mass was completely excised until the lingular bronchial passage was completely open (Fig 2A). To prevent recurrence of the neoplasm, the mass root was cauterized with APC. Final pathologic evaluation of the entire mass was identical to the initial diagnosis and confirmed the mass as a hamartoma. No additional treatment was required, and the patient was discharged and followed at the outpatient clinic. Over several weeks, the atelectasis in the left gradually improved. A follow-up bronchoscopy performed 3 months postoperatively showed no residual tumor. A chest computed tomographic scan showed that the left lung atelectasis was completely resolved except at the lingula (Figs 2B, 2C). At 1 year postoperatively, the patient was doing well without any complications noted. 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2014.11.063
FEATURE ARTICLES
Department of Internal Medicine, Ulsan University Hospital, University of Ulsan College of Medicine, Ulsan, Republic of Korea
1107