Benign Lung Tumors

 

INTRODUCTION

Solitary pulmonary nodules are found at a rate of 1-2 per 1000 chest radiographs. About 30% of these nodules prove to be malignant. Of the rest of the nodules, benign tumors of the lung make up about 2-5% of primary lung tumors. Benign lung tumors are a heterogenous group of neoplastic lesions originating from pulmonary structures. These tumors include bronchial adenomas, hamartomas, and a group of uncommon neoplasms (eg, chondromas, fibromas, lipomas, leiomyomas, hemangiomas, teratomas, pseudolymphomas, endometriosis).

Problem: Although benign lung tumors do not pose a significant health problem, complications can result from an obstructive lesion that could predispose the patient to pneumonia, atelectasis, and hemoptysis. These tumors are considered benign, and, therefore, their malignant/cancerous potential is minimal.

 

Frequency: Benign tumors make up 2-5% of all primary lung tumors. The exact incidence is not known because these tumors are often asymptomatic and are only detected during autopsy. Reported series suggest that benign lung tumors affect men more frequently than women (adenoma and hamartoma). The age range of patients affected is 17-77 years, with a mean age of 56.2 years for all types.

Etiology: The cause of benign lung tumors is not known.

 

Pathophysiology: The cause and pathogenesis of benign lung tumors are poorly understood. The nomenclature of benign lung tumors is based on histological findings. Some of these tumors have benign features, while others are hamartomas.

Neoplastic lesions are characterized by the autonomous proliferation of cells without a response to the normal control mechanisms governing their growth. An additional characteristic of benign tumors is extension without local tissue invasion or spread to other sites.

Hamartomas, on the other hand, consist of haphazardly organized mature cells and tissues. Hamartomas are composed mostly of masses of hyaline cartilage with a myxoid connective tissue, adipose cells, smooth cells, and clefts lined by respiratory epithelium.

Benign lung tumors can be classified pathologically, but a clinically useful classification would combine location (ie, endobronchial or parenchymal) and information about whether the lesions are single or multiple. Adenomas and hamartomas constitute the largest group of benign lung tumors and, thus, deserve detailed descriptions.

 

Hamartomas

Hamartomas (chondroadenomas) are the most common type of benign lung tumor. They mainly occur in adults but, on occasion, occur in children. Hamartomas are peripherally located. Grossly, they have a firm marblelike consistency. Histologically, hamartomas generally consist of epithelial tissue and other tissues such as fat and cartilage. Hamartomas can be easily enucleated, but wedge resection is also appropriate.

 

Bronchial adenomas

Bronchial adenomas make up 50% of all benign pulmonary tumors. The term bronchial adenoma, when used loosely, includes carcinoid tumors, adenocystic carcinomas, and mucoepidermoid carcinomas, which in fact are low-grade malignant tumors. The use of the term bronchial adenoma should be discouraged because it encompasses several benign and malignant tumors.

 

Mucous gland adenomas

Mucous gland adenomas are true benign bronchial adenomas. Mucous gland adenomas are also called bronchial cystadenomas, and they arise in the main or local bronchi. Histologically, they consist of columnar cell–lined cystic spaces with a papillary appearance.

 

Other rare tumor types

Tracheobronchial tumors

Multiple laryngeal papillomatosis is a viral disease of the upper airway that affects children. Multiple laryngeal papillomatosis has malignant potential and may spread to the tracheobronchial tree later.

Solitary papillomas usually are less than 1.5 cm in diameter. They usually are lobar or segmental in location and are histologically similar to viral papillomatosis.

Inflammatory papilloma is a solitary polypoid mass of granulation tissue that is associated with an underlying pulmonary inflammatory condition.

Granular cell myoblastomas are of neural cell origin. A granular cell myoblastoma contains polygonal or spindle cells with granular cytoplasm. Granular cell myoblastomas tend to be multiple in 10% of cases and are more common in men aged 30-50 years.

Other parenchymal tumors occasionally occurring in the endobronchial tree (eg, leiomyoma, lipoma) almost exclusively are found at an endobronchial location.

Solitary parenchymal tumors

Sclerosing hemangioma is an uncommon tumor derived from the epithelial cells of pneumocytes (terminal bronchiolar cells). This tumor consists of several elements, including solid cellular areas, papillary structures, sclerotic regions, and blood-filled spaces. This tumor is most commonly found in middle-aged women. Chest radiograph shows a well-defined nodule that is less than 4 cm.

Other mesenchymal tumors include lipoma, leiomyoma, neural tumors, fibroma, benign clear-cell tumor, teratoma, plasma cell granuloma, fibrous histiocytoma, xanthoma, pulmonary hyaline granuloma, pulmonary endometrioma, and pseudolymphoma.

Multiple parenchymal tumors

Many of these benign lung tumors may occasionally have multiple origins. Among these are hamartomas, hyalinizing granulomas, leiomyomas, and sclerosing hemangiomas.

The Carney triad is a syndrome of gastric epithelioid leiomyosarcoma, pulmonary chondromas, and extra-adrenal paragangliomas. The Carney triad mainly affects women.

Pulmonary tumorlets are minute collections of neuroendocrine cells scattered throughout the lung. Pulmonary tumorlets predominantly affect older women.

Clinically significant intrapulmonary chemodectomas apparently are paragangliomas. They behave in a benign fashion. Multiple minute pulmonary tumors, on the other hand, are of no clinical significance.

Clinical: When recording a patient’s history, include questions related to the usual differential diagnoses and lung cancer. For example, smoking history, age, exposure to carcinogens, previous malignant tumor or lung disease, domicile, and travel history are important.

The diagnostic approach is basically the same for these tumors. Clinical presentations depend upon the location of the tumor (eg, trachea, other airways, parenchyma). Hemoptysis, lung mass on chest radiograph, and unresolved infiltrates may be present. Symptoms may include pseudoasthmatic wheezing; persistent coughing; shortness of breath; hemoptysis; fever, especially when associated pneumonia is present; diminished breath sounds; dullness to percussion; and rales.

If a patient is asymptomatic, the tumor may be found serendipitously and, sometimes, classified as a solitary pulmonary nodule.

INDICATIONS

The purpose of surgical intervention for benign lung tumors is to avoid missing potentially malignant lesions. Otherwise, remove benign lung tumors when they are symptomatic, which indicates the presence of complications such as pneumonia, atelectasis, and/or hemoptysis.

 

RELEVANT ANATOMY AND CONTRAINDICATIONS

Relevant Anatomy: Determination of whether a lung mass is benign or malignant based solely on its anatomical location is an incorrect practice. Anatomical location has no predictability on the malignant potential of a tumor.

Contraindications: Perform a biopsy on lung masses in order to determine the mass malignant potential. Contraindications to a biopsy procedure are bleeding diathesis and cardiopulmonary conditions, which may place the patient's life at risk as a result of the procedure.


WORKUP

Lab Studies:

  • Perform a complete preoperative evaluation on all patients who undergo tissue sampling.
  • The cell count helps to determine the general health status of the patient and also to diagnose complications such as pneumonia and anemia.
  • Determine adequacy of the platelet function and perform coagulation studies before the patient undergoes any invasive procedures.
  • Electrolytes and renal and liver function tests help to evaluate the presence of an abnormality that indicates the need for either intervention or further workup before performing an invasive procedure.
  • Tuberculin skin test
  • Sputum cytologic and microbiological studies
  • A patient with a carcinoid tumor, with or without carcinoid syndrome, may exhibit a high level of serotonin and 5-hydroxyindoleacetic acid (5-HIAA).
  • Arterial blood gas and pulmonary function tests (PFTs) are indicated in patients presenting with shortness of breath and are indicated before invasive procedures or thoracotomy. The presence of hypoxia and hypercarbia generally suggests poor tolerability for resective surgery. PFTs are useful tests when determining patients' suitability for lung resection. Patients must have satisfactory parameters as measured by forced vital capacity (FVC), forced expiratory volume in one second (FEV1), FEV1/FVC, and total lung volume.
  • Diffusing capacity of lung for carbon monoxide (DLCO) is a complimentary test that provides additional information about oxygen exchange.

Imaging Studies:

  • Chest radiograph (posteroanterior [PA] and lateral views) is required. Review all previous chest radiographs when available. Reviewing helps to determine the onset and doubling time. Chest radiograph provides information about size, morphology, the presence of calcifications, and spicula. Benign lesions tend to have calcium deposited in central, peripheral, concentric, "popcorn," or homogeneous patterns, whereas eccentric patterns of calcifications are more characteristic of malignancies. These characteristics help when determining if the tumor is benign or malignant, although not with certainty.
  • Perform a chest computed tomography (CT) scan with and without contrast. Using both contrast and noncontrast examinations can help delineate calcification, can better confirm hamartomas and arteriovenous malformations, and can enhance the appearance of malignant tumors. Using iodinated contrast also provides more details about size, the presence of satellite lesions, the status of perihilar and mediastinal lymph nodes, and, when used in conjunction with noncontrast studies, it provides a more detailed definition of calcification.
  • Use of MRI is limited; however, it is useful in defining tumor invasion of the great vessels. MRI is superior to CT scan in defining the mediastinum, chest wall invasion, and aortopulmonary window adenopathy.

Other Tests:

  • ECG is required before surgery as part of the preoperative cardiac risk factor assessment. Address the presence of major arrhythmia and ischemia before performing the planned procedure.

Diagnostic Procedures:

  • Fiberoptic bronchoscopy: Both rigid and fiberoptic bronchoscopy are useful for diagnosing benign lung tumors. Biopsy or bronchial brushing can be performed with this procedure, as well as excision of pedunculated endobronchial lesion. Sensitivity for detection of malignancy is 10-30% when nodules are small (<2 cm). Bronchoscopy is less favored because of the concern for residual tumors, and most surgeons prefer wedge resection.
  • Percutaneous biopsy/guided transthoracic needle aspiration biopsy: The tumor must be easily accessible. Percutaneous biopsy/guided transthoracic needle aspiration biopsy is performed under CT scan or fluoroscopy guidance and has a yield as high as 85% for diagnosis. A major complication is pneumothorax, with an incidence that may be as high as 20-25%.
  • Video-assisted thoracoscopy: A biopsy can be obtained from a superficial pleural-based lesion, or the lesion can be resected using this approach.
  • Open biopsy: This procedure is not routinely used. Thoracotomy is planned after the appropriate workup and plan of management is decided. The extent of resection is decided intraoperatively.
Histologic Findings: See Pathophysiology.

TREATMENT

Medical therapy: A solitary nodule in a young nonsmoking patient can be monitored with serial radiographs as long as the solitary nodule does not double in size in less than a year and it does not significantly increase in the pattern of calcification and shape consistent with a malignancy. Otherwise, medical therapy is limited to the initial management of complications and associated comorbidity.

Surgical therapy: The extent of surgery may be simple endoscopic resection, thoracotomy with bronchotomy/local excision, sleeve resection, segmental resection, or lobectomy. The extent is usually determined at surgery and is as conservative as possible. Commonly, surgical resection is recommended for bronchial adenomas because of the potential for malignancy.

Preoperative details: Determine operability and resectability and take steps to prevent or limit operative and postoperative complications.

Intraoperative details: At the time of open thoracotomy, perform complete tumor resection. If the lesion proves to be malignant, adequate resection with appropriate lymph node staging is necessary.

Postoperative details: Triage the patient to the surgical ICU or postoperative recovery floor.

Follow-up care: When observation is elected as medical management, performing chest radiograph surveillance every 3 months for the first year, every 6 months the second year, and yearly thereafter is recommended.

 

COMPLICATIONS

Possible complications include pneumonia, atelectasis, hemoptysis, and malignancy.

 

OUTCOME AND PROGNOSIS

The primary therapy for bronchial adenomas is surgical excision. The 5-year survival rate following surgical resection is 95%, decreasing to 70% if regional nodes are involved. Expected survival is dependent on the exact nature of the lesion (eg, typical or atypical carcinoid tumor, adenoid cystic carcinoma, other histology).