Middle-aged Male with Endobronchial Lesion
A 57-year-old male was referred to the pulmonary clinic for a cough that persisted for 2 months and hemoptysis for 1 week. These symptoms were associated with dyspnea and significant weight loss.
HISTORY
There was no history of fever or similar complaints in the past. His past medical history was significant for type 2 diabetes mellitus (DM). He had a 40-pack year history of smoking with no significant history of drug or alcohol use. He was taking insulin and metformin for DM.
PHYSICAL EXAMINATION
On examination, he was awake, alert, and oriented. He was afebrile with normal vitals except O2 saturation of 90% on room air. General physical examination was normal with no lymphadenopathy or clubbing. Respiratory examination revealed decreased breath sounds on left with dull percussion, along with mediatinum shift to the left side on CXR. Rest of the systemic examination was non-contributory.
Figure 1. Chest x-ray of a left-sided complete lung collapse.
Figure 2a and 2b. CT chest with contrast of a left main bronchus mass lesion.
Routine laboratory results were within normal limits, except for a blood glucose level of 256 mg and glycosylated hemoglobin of 10.6%. Chest x-ray (CXR) revealed complete atelectasis of left lung (Figure 1). Computed tomography (CT) scan of chest with contrast was performed, which showed complete atelectasis of left hemithorax with endobronchial obstruction of left main bronchus (Figure 2a and 2b). CT scan of head, abdomen, and pelvis were non-contributory. Tumors (benign and malignant), foreign body, and hematological malignancy (lymphoma) were kept as differential diagnosis of endobronchial lesion.
Flexible bronchoscopy showed complete occlusion of the left main bronchus by large endobronchial mass (Figure 3). Multiple biopsies were taken and electrocautery was done to resect the mass. Bronchio-alveolar lavage (BAL) was performed along with the biopsy and the resected lesion was sent for evaluation.
Figure 3. Bronchoscopic view of the left main bronchus endobronchial hamartoma.
BAL was negative for cytology and microbiology. On gross examination, mass lesion was 1 x 0.5 x 0.3 cm aggregate of tan white hemorrhagic tissue. Histopathology sections showed presence of large lobulated islands of cartilage without atypia or mitotic activity (Figure 4 and 5), which is consistent with endobronchial hamartoma (EH).
Figure 4. Increased proliferation of benign chondrocytes (H&E, 100X).
Figure 5. Islands of benign cartilage surrounded by fibrosis indicative of an endobronchial hamartoma (H&E, 40X).
(Answer and discussion on next page)
ANSWER: Endobronchial hamartoma
Pulmonary hamartoma is a very rare benign tumor that originates from the primitive mesenchymal tissue which differentiates into mature mesenchymal components like bone, cartilage, fat, and muscle tissues. Hamartoma accounts for 0.025% to 0.32% of all pulmonary malignancy cases.1 The mean age of patients at first presentation of EH is in the 60s and 70s. The condition is more common in males than females.2
Parenchymal hamartomas are generally asymptomatic. EH may be asymptomatic in the beginning, but symptoms generally presents secondary to occlusion of airway. They localize mostly in large diameter bronchus. Most frequent clinical features are hemoptysis and obstructive pneumonia, followed by cough and dyspnea.3 There are multiple differential diagnosis of endobronchial lesions, with multiple benign and malignant etiologies (Table 1).
EH: Diagnosis and Treatment
Radiology findings depend on the size and location of the tumor. Most frequently, the CXR findings are alveolar infiltrate and atelectasis. In a few cases, the CXR is completely normal. CT scans findings of a mass having a high fat density or calcification with no contrast uptake is highly indicative of hamartomas.4 In this case, the CT scan detected a calcified endobronchial mass, which is consistent with the classical CT scan findings of hamartomas. On bronchoscopy, EH may be well circumscribed, poypoid, or pedunculated mass with smooth and yellow surface—without submucosal infiltration.5
The mass in our case was smooth, white, and pedunculated. A definitive diagnosis of hamartomas can only be made through a histology of the resected lesion, which predominantly detects cartilage tissue with a combination of bone, fat, and muscle.6
Endoscopic resection is the gold standard for the treatment of endobronchial hamartomas. Resection can be done, using either flexible or rigid bronchoscope.5 Laser treatment is considered most effective, especially in bulky tumors. Electrocautery through flexible bronchoscope is also a very effective, simple, and quick method of tumor resection. Electrosurgical snaring through flexible bronchoscopy has been used to resect EH in several cases.7 Thoracotomy is indicated in case of irreversible parenchymal damage like fibrosis, cicatrization collapse, and bronchiectesis secondary to EH.8 Complications of endoscopic resection include bleeding, perforation, and burning of the normal bronchial wall during laser use or cauterization. The prognosis after an endoscopic resection of EH is very good.3
Clinical presentation of our case is atypical for endobronchial hamartoma. Our patient had a history of prolonged smoking and presented with massive hemoptysis and left-sided lung collapse, which is suggestive of malignant endobronchial lung tumor. Hamartomas are generally asymptomatic and slow growing but our patient was symptomatic due to bronchial obstruction. Complete resection of hamartoma by electrocautery resolved the symptoms in our patient and he was discharged home. ■
References
1.Gjevre JA, Myers JL, Prakash UBS. Pulmonary hamartomas. Mayo Clin Proc. 1996;71:14-20.
2.Salminen US. Pulmonary hamartoma: a clinical study of 77 cases in a 21-year period and review of literature. Eur J Cardiothorac Surg. 1990;4:15-18.
3.Cosío BG, Villena V, Echave-Sustaeta J, et al. Endobronchial hamartoma. Chest. 2002;122(1):202-205.
4.Ferretti GR, Bithigoffer C, Righini CA, et al. Imaging of tumors of the trachea and central bronchi. Radiol Clin North Am. 2009;47(2):227-241.
5.Kim SA, Um SW, Song JU, et al. Bronchoscopic features and bronchoscopic intervention for endobronchial hamartoma. Respirology. 2010;15(1):150-154.
6.Tomashefski JF Jr. Benign endobronchial mesenchymal tumors: their relationship to parenchymal pulmonary hamartomas. Am J Surg Pathol. 1982;6(6):531-540.
7.Mondello B, Lentini S, Buda C, et al. Giant endobronchial hamartoma resected by fiberoptic bronchoscopy electrosurgical snaring. J Cardiothorac Surg. 2011;14(6):97.
8.Tomos P, Karaiskos T, Lahanas E, et al. Transverse bronchoplasty of the membranous wall after resection of an endobronchial hamartoma. Ann Thorac Surg. 2007;83(2):703-704.
Kim Eun Ji, MD, is a resident in the department of medicine at North Shore University Hospital at Manahasset.
Sameer Verma, MD, is a research associate in endocrinology and metabolism PC. He is currently investigating the role of fatigue in patients with various metabolic problems.
Anup Singh, MD is fellow in the department of pulmonary critical care medicine at North Shore University Hospital at Manahasset.
Michael Esposito, MD is an attending in the department of pathology at North Shore University Hospital at Manahasset.
Arunabh Talwar, MD is an attending in pulmonary critical care medicine at North Shore University Hospital at Manahasset. His interest is in the management of advanced lung disease, particularly pulmonary hypertension and interstitial lung disease.