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BRIEF COMMUNICATION
Year : 2016  |  Volume : 12  |  Issue : 2  |  Page : 1093-1097

Bronchoscopic and surgical management of rare endobronchial tumors


1 Institute of Pulmonology, Medical Research and Development; Lung Care and Sleep Centre, Fortis Hiranandani Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, SRL Laboratories, Fortis Hiranandani Hospital, Mumbai, Maharashtra, India
3 Department of Pathology, SRL Laboratories, Mumbai, Maharashtra, India
4 Department of Thoracic Oncology, Somaiya Ayurvihar, Mumbai, Maharashtra, India

Date of Web Publication25-Jul-2016

Correspondence Address:
Prashant N Chhajed
Institute of Pulmonology, Medical Research and Development, 405, Sangam, Corner of SV Road and Sai Baba Road, Opposite Surya Hospital, Santacruz West, Mumbai - 400 054, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.186692

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 > Abstract 


Pure endobronchial neoplasms are a rare entity, and they may present with diverse pathological findings. Malignant diseases are more common than benign ones, and they mostly originate from the surface epithelium. Endobronchial tumors usually present with symptoms such as cough, hemoptysis, recurrent pneumonia, wheezing, and chest pain. Flexible bronchoscopy is necessary for diagnosis and evaluation of these endobronchial tumors. Advance bronchoscopy or definitive surgery is the modality of treatment for these tumors based on the presentation. We present a case series of patients suffering from different rare endobronchial tumors and their management along with the review of literature.

Keywords: Chondroid hamartoma, leiomyoma, mucoepidermoid carcinoma, rare endobronchial tumors, schwannoma


How to cite this article:
Pandey K, Vaidya PJ, Kate AH, Chavhan VB, Jaybhaye P, Patole K, Deshpande RK, Chhajed PN. Bronchoscopic and surgical management of rare endobronchial tumors. J Can Res Ther 2016;12:1093-7

How to cite this URL:
Pandey K, Vaidya PJ, Kate AH, Chavhan VB, Jaybhaye P, Patole K, Deshpande RK, Chhajed PN. Bronchoscopic and surgical management of rare endobronchial tumors. J Can Res Ther [serial online] 2016 [cited 2019 Dec 6];12:1093-7. Available from: http://www.cancerjournal.net/text.asp?2016/12/2/1093/186692




 > Introduction Top


Pure endobronchial neoplasms are a rare entity, and they may present with diverse pathological findings.[1] Malignant diseases are more common than benign ones, and they mostly originate from the surface epithelium.[2] Endobronchial tumors usually present with symptoms such as cough, hemoptysis, recurrent pneumonia, wheezing, and chest pain. Flexible bronchoscopy is necessary for diagnosis and evaluation of these endobronchial tumors. Advanced bronchoscopic resection of these tumors using interventional bronchoscopy techniques has been an effective modality of treatment for these tumors.[3] However, the definitive treatment of these tumors depends on the location, size, and histopathological diagnosis. We present a case series of patients who had these rare endobronchial tumors that were managed with advanced bronchoscopic interventions or definitive surgical treatments.


 > Cases Top


Case 1

A 56-year-old male, diabetic and hypertensive, presented with complaints of mild intermittent hemoptysis for 2 months and retrosternal chest pain for 3 days. General physical examination was unremarkable, and the respiratory system physical examination did not reveal any obvious abnormality. Flexible bronchoscopy revealed a mass in the right intermedius bronchus almost causing 80% luminal narrowing. This mass lesion was excised bronchoscopically using electrocautery snare. Histopathology of the mass showed lobules of mature hyaline cartilage surrounded by abundance of mature adipocytes and fibrovascular stroma covered by bronchial epithelium consistent, with a diagnosis of an endobronchial chondroid hamartoma [Figure 1].
Figure 1: Mature hyaline cartilage surrounded by abundance of mature adipocytes and fibrovascular stroma covered by bronchial epithelium (H and E, ×10)

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Case 2

A 26-year-old male presented with complaints of cough, hemoptysis, and dyspnea on exertion. General physical examination was unremarkable, and respiratory system examination revealed diminished breath sounds on the left side and no foreign breath sounds. Computed tomography scan of the chest revealed mediastinal mass with endotracheal extension. Diagnostic flexible bronchoscopy showed a mass lesion in the lower end of trachea occluding the left main bronchus with distortion of the carina [Figure 2]. A rigid bronchoscopy was performed, and debulking of the endobronchial component of the tumor was performed using electrocautery snare. Histopathology of the mass revealed a spindle cell tumor with spindly cells and moderate cytoplasm [Figure 3]. On immunohistochemistry, a diagnosis of schwannoma was made as the spindle cells were positive for S-100 protein and negative for smooth muscle actin (SMA) [Figure 4] and [Figure 5]. The patient underwent surgical resection with tracheoplasty uneventfully. The patient was asymptomatic on follow-up.
Figure 2: Flexible bronchoscopic image mass lesion in the lower end of trachea occluding the left main bronchus with distortion of the carina

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Figure 3: Spindle cell tumor with spindle cells and moderate cytoplasm (H and E, ×10)

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Figure 4: Immunohistochemistry in schwannoma showing spindle cells positive for S-100 protein

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Figure 5: Immunohistochemistry in schwannoma spindle cells showing negativity for smooth muscle actin uptake

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Case 3

A 63-year-old male presented with cough, dyspnea on exertion, streaky hemoptysis for 3 months. The patient also complained of choking sensations and recurrent respiratory infections. General physical examination was unremarkable, and the patient was hemodynamically stable. Diagnostic flexible bronchoscopy showed a polypoid growth on the posterior wall of the trachea proximal to the carina. The mass lesion was sessile with a loose thin stalk attached to the posterior wall of trachea and blocked the right main bronchus or left main bronchus with change in its position [Figure 6]. A therapeutic bronchoscopic resection of the mass was performed using electrocautery snare. Histopathological examination showed a benign circumscribed tumor composed of interlacing short and long fascicles of spindle cells, suggesting a diagnosis of leiomyoma [Figure 7]. Immunohistochemistry examination of the section showed that the spindle cells were positive for SMA and negative for S-100 protein, confirming the diagnosis of a benign leiomyoma [Figure 8] and [Figure 9]. Recheck bronchoscopy done at 3 months, 6 months, and 1 year did not show any recurrence of the tumor.
Figure 6: Flexible bronchoscopic image of sessile mass with a loose thin stalk attached to the posterior wall of trachea

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Figure 7: Benign circumscribed tumor composed of interlacing short and long fascicles of spindle cells suggesting leiomyoma (H and E, ×10)

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Figure 8: Immunohistochemistry showing spindle cells being positive for the smooth muscle actin

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Figure 9: Immunohistochemistry showing spindle cells being negative for the S-100 protein

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Case 4

A 19-year-old male patient presented with cough and hemoptysis for 3 weeks. General physical examination was unremarkable, and respiratory system examination showed diminished breath sounds on right infra-axillary and infrascapular areas. Flexible bronchoscopy showed a mass lesion in the right bronchus intermedius with areas of vascularity [Figure 10]. Multiple biopsies were taken with the help of hot biopsy forceps. The histopathology showed submucosal low grade mucoepidermoid carcinoma (MEC) of the bronchus [Figure 11]. The patient underwent right thoracotomy with right middle and lower lobectomy with bronchovascular sleeve resection and complete mediastinal lymph node excision. Lymph nodes did not reveal any metastasis. The patient was asymptomatic at 1 year follow-up.
Figure 10: Flexible bronchoscopy showing a mass lesion in right bronchus intermedius with areas of vascularity

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Figure 11: Submucosal low-grade mucoepidermoid carcinoma of the bronchus composed of nests of polygonal intermediate type cells with central round nuclei and pink cytoplasm along with mucinous cells having intracellular mucin (H and E, ×10)

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 > Discussion Top


Lung hamartomas are a form of benign lung tumors, with an incidence between 0.025% and 0.32%.[4] In most cases, the tumor is located intrapulmonary and endobronchial location is a rare occurrence with an incidence of 1.4% in the largest review series.[5] Endobronchial hamartoma is often symptomatic in comparison to pulmonary hamartoma which is asymptomatic, and the most common complaints of patients are hemoptysis and obstructive pneumonia.[6] Endobronchial hamartomas are benign tumors with low risk of malignancy and recurrence. Histopathological features of endobronchial hamartoma typically show well-circumscribed unencapsulated lesions comprising lobules of mature cartilage and surrounded by mature adipocytes and fibrovascular stroma.[7] Bronchoscopic treatment with flexible or rigid bronchoscopy using endobronchial ablative therapies is safe and an effective therapeutic choice in a symptomatic patient.[8] The current case study also shows that therapeutic endobronchial therapy with electrocautery snare is an effective modality in the treatment of endobronchial hamartomas.

Endobronchial schwannomas are rare and often benign tumors that develop from nerve sheath of peripheral nervous system.[9] This type of tumor arises from peripheral nerves located within the bronchial wall, composed of nerve sheath Schwann cells, described as neurilemomas (schwannomas) or neurofibromas.[10] Primary endobronchial schwannomas are rare endobronchial tumors with estimated incidence of 0–2% of all lung tumors.[11] Histologically, schwannoma spindle cells are arranged in a highly cellular and palisading nuclei pattern (Antoni A) or a less cellular and more fibrous pattern (Antoni B). On immunohistochemistry staining, they demonstrate positivity for S-100 protein as was found in the current case, which helped in confirmation of the diagnosis. The standard treatment for schwannoma has been a surgical resection; however, recent case series have shown successful removal of endobronchial schwannomas using rigid bronchoscopy and advanced bronchoscopic techniques.[12] Surgical resection was sought in the current case after bronchoscopic debulking due to the extrabronchial extension of the tumor causing distortion of the trachea. Bronchoscopic debulking of endobronchial tumors has been reported to permit parenchyma sparing surgery and also permit better reconstruction surgeries by better defining the endobronchial component and airway extent.[13],[14]

Endobronchial leiomyomas are extremely rare benign tumors and usually present as a solitary tumor.[15] They account for 33–45% of all pulmonary leiomyomas.[16],[17] Clinical presentation depends on the location of the tumor, its size, and changes in the lung distal to the lesion. Endobronchial leiomyomas arise from the smooth muscles of the bronchial wall and grow as solitary polypoid tumors with broad bases involving the bronchi.[18] Histopathological examination of the tissue shows tumor composed of spindle cells and is positive for SMA and desmin on immunohistochemistry differentiating it from other spindle cell tumors such as fibromas and neurofibromas.[1] The treatment for endobronchial leiomyoma depends on the size and location of the tumor in the airway. Less invasive treatment options are resection of the leiomyoma using advanced bronchoscopic techniques such as laser, electrocautery, and argon plasma coagulation which are safe and an effective modality of choice in lesions with narrow base. Bronchoscopic electrocautery snare was used in the case described to effectively remove the tumor. Prognosis of endobronchial leiomyoma is excellent after complete resection. Other treatment options are resection of leiomyoma by bronchoplasty or bronchotomy utilizing sleeve resection of the involved bronchus, lobectomy, or pneumonectomy.[19] Therapeutic bronchoscopy was curative in the patient described in the current report.

MEC is a rare tumor of the lung that accounts for 0.1–0.2% of all pulmonary tumors.[20] Pulmonary MEC patients typically present with symptoms related to bronchial obstruction and atelectasis, such as cough, hemoptysis, wheezing, and postobstructive pneumonia.[21] Most pulmonary MECs arise in the proximal bronchi.[22] Histologically, MEC is characterized by a combination of mucus-secreting, squamous, and intermediate cell types, which was consistent with the findings seen in the current case. Low-grade MEC is distinguished from high-grade MEC based on the lack of cytological atypia including nuclear pleomorphism and absence of significant mitotic activity and cellular necrosis.[23] Histological grade is an important prognostic indicator, with high-grade MECs demonstrating a greater risk for metastases, tumor recurrence, and death.[24] Our case had low-grade MEC on histopathology. MEC of the lung is preferably treated surgically by lobectomy, sleeve resection, local resection, and segmental resection.[22]

Endobronchial tumors cause respiratory symptoms and may cause hemoptysis and dyspnea due to mechanical obstruction of the airways. Both flexible and rigid bronchoscopies play an important role in the diagnosis and treatment of endobronchial tumors. The diagnosis of these lesions can be clinically challenging. Bronchoscopic removal of endobronchial tumors is a safe and effective alternative to surgical resection in selected cases. A complete histopathological diagnosis is important to diagnose and guide treatment in endobronchial tumors. Surgery should be considered for patients with having lesions with increased risk of recurrence, extrabronchial extension of the lesions, or destruction of the peripheral lung due to long-term atelectasis.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
 > References Top

1.
Wilson RW, Kirejczyk W. Pathological and radiological correlation of endobronchial neoplasms: Part I, Benign tumors. Ann Diagn Pathol 1997;1:31-46.  Back to cited text no. 1
    
2.
Wilson RW, Frazier AA. Pathological-radiological correlations: Pathological and radiological correlation of endobronchial neoplasms: Part II, malignant tumors. Ann Diagn Pathol 1998;2:31-4.  Back to cited text no. 2
    
3.
Nassiri AH, Dutau H, Breen D, Colchen A, Quiot JJ, Nguyen B, et al. A multicenter retrospective study investigating the role of interventional bronchoscopic techniques in the management of endobronchial lipomas. Respiration 2008;75:79-84.  Back to cited text no. 3
    
4.
Murray J, Kielkowski D, Leiman G. The prevalence and age distribution of peripheral pulmonary hamartomas in adult males. An autopsy-based study. S Afr Med J 1991;79:247-9.  Back to cited text no. 4
    
5.
Gjevre JA, Myers JL, Prakash UB. Pulmonary hamartomas. Mayo Clin Proc 1996;71:14-20.  Back to cited text no. 5
    
6.
Cosío BG, Villena V, Echave-Sustaeta J, de Miguel E, Alfaro J, Hernandez L, et al. Endobronchial hamartoma. Chest 2002;122:202-5.  Back to cited text no. 6
    
7.
Tyagi R. Pulmonary hamartoma, a rare benign tumour of the lung – Case series. Asian J Med Sci 2014;3:112-5.  Back to cited text no. 7
    
8.
Madan K, Agarwal R, Bal A, Gupta D. Bronchoscopic management of a rare benign endobronchial tumor. Rev Port Pneumol 2012;18:251-4.  Back to cited text no. 8
    
9.
Nasiri H, Zeki AA, Albertson TE. A rare diagnosis: Endobronchial schwannoma. Access Date 2014;22.  Back to cited text no. 9
    
10.
Hsu HS, Wang CY, Li WY, Huang MH. Endotracheobronchial neurofibromas. Ann Thorac Surg 2002;74:1704-6.  Back to cited text no. 10
    
11.
Shah H, Garbe L, Nussbaum E, Dumon JF, Chiodera PL, Cavaliere S. Benign tumors of the tracheobronchial tree. Endoscopic characteristics and role of laser resection. Chest 1995;107:1744-51.  Back to cited text no. 11
    
12.
Jung YY, Hong ME, Han J, Kim TS, Kim J, Shim YM, et al. Bronchial schwannomas: Clinicopathologic analysis of 7 cases. Korean J Pathol 2013;47:326-31.  Back to cited text no. 12
    
13.
Chhajed PN, Eberhardt R, Dienemann H, Azzola A, Brutsche MH, Tamm M, et al. Therapeutic bronchoscopy interventions before surgical resection of lung cancer. Ann Thorac Surg 2006;81:1839-43.  Back to cited text no. 13
    
14.
Kate AH, Shah PS, Shukla NB, Chhajed PN, Shetty SN. Neoadjuvant bronchoscopic intervention: Bridge to lung parenchyma sparing surgery. J Cancer Res Ther 2013;9:760-1.  Back to cited text no. 14
    
15.
Ko SM, Han SB, Lee SK, Woo SK, Kim MJ, Keum DY, et al. Calcified endobronchial leiomyoma. Br J Radiol 2007;80:91-3.  Back to cited text no. 15
    
16.
Sharifi N, Massoum SH, Shahri MK, Rezaei A, Ashari AA, Attar AS, et al. Endobronchial leiomyoma; report of a case successfully treated by bronchoscopic resection. J Res Med Sci 2010;15:364-70.  Back to cited text no. 16
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17.
Kim YK, Kim H, Lee KS, Han J, Yi CA, Kim J, et al. Airway leiomyoma: Imaging findings and histopathologic comparisons in 13 patients. AJR Am J Roentgenol 2007;189:393-9.  Back to cited text no. 17
    
18.
White SH, Ibrahim NB, Forrester-Wood CP, Jeyasingham K. Leiomyomas of the lower respiratory tract. Thorax 1985;40:306-11.  Back to cited text no. 18
    
19.
Cárdenas-García J, Lee-Chang A, Chung V, Shim C, Factor S, Tibb A. Bronchial leiomyoma, a case report and review of literature. Respir Med Case Rep 2014;12:59-62.  Back to cited text no. 19
    
20.
Leonardi HK, Jung-Legg Y, Legg MA, Neptune WB. Tracheobronchial mucoepidermoid carcinoma. Clinicopathological features and results of treatment. J Thorac Cardiovasc Surg 1978;76:431-8.  Back to cited text no. 20
    
21.
Heitmiller RF, Mathisen DJ, Ferry JA, Mark EJ, Grillo HC. Mucoepidermoid lung tumors. Ann Thorac Surg 1989;47:394-9.  Back to cited text no. 21
    
22.
Liu X, Adams AL. Mucoepidermoid carcinoma of the bronchus: A review. Arch Pathol Lab Med 2007;131:1400-4.  Back to cited text no. 22
    
23.
Alsidawi S, Morris JC, Wikenheiser-Brokamp KA, Starnes SL, Karim NA. Mucoepidermoid carcinoma of the lung: A case report and literature review. Case Rep Oncol Med 2013;2013:625243.  Back to cited text no. 23
    
24.
Chin CH, Huang CC, Lin MC, Chao TY, Liu SF. Prognostic factors of tracheobronchial mucoepidermoid carcinoma – 15 years experience. Respirology 2008;13:275-80.  Back to cited text no. 24
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]



 

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