Case report:
A 22 year old nonsmoking female presented with high grade fever,left
sided chest pain,streaky hemoptysis,loss of weight and appetite for one
month.Her menstrual cycles were regular.On chest examination trachea
shifted to left and decreased movements and decreased breath sounds over
left hemithorax.Her blood work showed increased in total leucocyte count
of 16×103/µL with neutrophilic
predominance(70%).Chest X ray suggestive of left lingular and lower
lobe collapse. CECT chest showed endobronchial lesion in the left main
bronchus.Sputum report for gram’s stain and culture sensitivity showed
no growth.Sputum AFB smear was negative and in sputum CBNAAT, MTb was
not detected. On bronchoscopy there was a smooth polypoidal lesion with
wide base, completely obstructing the left main bronchus.Cryobiopsy done
and debulking of tumor done with electrocautery snare .On HPE,
submucosal stroma showed sheets and fascicle of spindle shaped cells
with elongated nucleus with eosinophilic rich cytoplasm with no mitotic
activity,no nuclear atypia suggestive of leiomyoma.Immunohistochemistry
showed SMA (Smooth Muscle Actin) positivity.USG abdomen showed no
features of uterine leiomyoma.Follow up FDG PET showed no residual tumor
activity in lung and elsewhere in the body.Patient was under follow up
with no complication and recurrence.
Discussion :
Pulmonary smooth muscle proliferation can either be primary, which
includes hamartomas,lymphangioleiomyomatosis,leiomyoma and
leiomyosarcoma, or metastatic, including metastatic leiomyosarcoma and
benign metastasizing uterine leiomyoma.Primary pulmonary leiomyoma is a
rare tumor of mesodermal origin.It usually develops from smooth muscles
fibers of tracheobronchial tree, blood vessel or embryonic heterotropic
muscle islets5.It was first described by Forkel in
19096.It accounts for 33-45% of all pulmonary
leiomyomas7In trachea (16%), bronchi (33%) and
pulmonary parenchyma (51%)8. Kown described
fever,dyspnea and cough to be the predominant
symptom9.Usually symptom depend on degree of
obstruction in affected bronchus and the state of lung parenchyma.Our
patient had all symptoms as described by kown, along with loss of weight
and appetite and hemoptysis.Since tuberculosis being most prevalent in
India,she was suspected to have tuberculosis. Pulmonary leiomyoma,due to
its low incidence and few literature studies,was least suspected in our
case. Diagnosis of leiomyoma cannot be made solely based on radiology
since there are no pathognomonic features10. Usually
it presents as solitary mass,if it is endobronchial it can present as
atelectasis,obstructive pneumonia,hyperlucency due to distal air
trapping.Fiberoptic Bronchoscopy is used to visualize endobronchial
lesion but the extent of extraluminal involvement and airway distal to
obstruction cannot be visualized, hence virtual bronchoscopy and 3D
airway reconstruction helps to overcome this
limitation11.Histopathologic examination shows
disorganized smooth muscle with vasculature or fibrous
component12.Immunohistochemistry shows positivity to
smooth muscle actin(SMA) and desmin which helps to differentiate it from
fibroma, neurofibromas and schwannoma.There are no standard treatment
guidelines.Usually endobronchial lesion are treated with
electrocautery,laser photocoagulation13.If there is a
parenchymal lesion depending upon the size and location
segmentectomy,lobectomy and pneumonectomy is being done14.Our patient underwent electrocautery snare
resection of tumor.Post procedure followup chest Xray became
normal.Patient was followed up with FDG PET with no residual tumor
activity in lung and elsewhere in the body.
Conclusion :
Endobronchial leiomyoma is an unusual cause for endoluminal
obstruction.Due to its rarity it was least suspected and posed a
diagnostic challenge.Bronchoscopy helps both diagnostic as well as
therapeutically as the tumor has excellent prognosis following
resection.
Ethics statement :Ethical approval was not required for the case
report as per country’s guidelines.
Consent : Written informed consent was obtained from patient to
publish the report.
Reference :
1.White SH, Ibrahim NB, Forrester-Wood CP, Jeyasingham K. Leiomyomas of
the lower respiratory tract. Thorax 1985;40:306–11.)
2.(Wilson RW, Kirejczyk W (1997) Pathological and radiological
correlation of endobronchial neoplasms: Part I, Benign tumors. Ann Diagn
Pathol 1: 31-46.)
(3.Ayabe H, Tsuji H, Tagawa Y, Tomita M, Tsuda N, Chen J. Endobronchial
leiomyoma: report of a case treated by bronchoplasty and a review of the
literature. Surg Today 1995;25:1057–60).
(4.Mathur RM, Sen G, Yadav KS (1993) Endobronchial leiomyoma. Indian J
Cancer 30: 16-19
(5.Shahian DM, McEnany MT. Complete endobronchial excision of leiomyoma
of the bronchus. J Thorac Cardiovasc Surg. 1979;77:87-91.).
(6.Freireich K, Bloomberg A, Langs EW. Primary bronchogenic leiomyoma.
Dis Chest. 1951;19:354-8).
(7.N. Sharifi, S.H. Massoum, M.K. Shahri, A. Rezaei, A.A. Ashari, A.S.
Attar, et al. Endobronchial leiomyoma; report of a case
successfully treated by bronchoscopic resection).
(8.Miller DR. Benign tumors of lung and tracheobronchial tree. Ann
Thorac Surg. 1969;8:542-60).
(9.Kwon YS, Kim H, Koh WJ, Suh GY, Chung MP, Kwon OJ, Han J. Clinical
characteristics and efficacy of bronchoscopic intervention for
tracheobronchial leiomyoma. Respirology. 2008 Nov;13(6):908-12).
(10.Ayabe H, Tsuji H, Tagawa Y, Tomita M, Tsuda N, Chen J. Endobronchial
leiomyoma: report of a case treated by bronchoplasty and a review of the
literature. Surg Today. 1995;25(12):1057-60).
11.Finkelstein SE, Schrump DS, Nguyen DM, Hewitt SM, Kunst TF, Summers
RM. Comparative evaluation of super high-resolution CT scan and virtual
bronchoscopy for the detection of tracheobronchial malignancies. Chest.
2003 Nov;124(5):1834-40).
(12.Wilson RW, Kirejczyk W. Pathological and radiological correlation of
endobronchial neoplasms. Part I. Benign tumors. Ann Diagn Pathol
1997;1:31-46).
13.Zidane A, Elktaibi A, Benjelloun A, Arsalane A, Afandi O, Bouchentouf
R. Primary leiomyoma of the lung: an exceptional localization. Asian
Cardiovasc Thorac Ann. 2016;24:393-6.)
14.Salminen US, Halttunen P, Miettinen M,Mattila S. Benign mesenchymal
tumours of the lung including sclerosing haemangiomas. Ann Chir Gynaecol
1990;79:85-91.)