BASICS
DESCRIPTION
- Bronchogenic tumor arising from the apex of the lung in the superior sulcus; invades adjacent tissue
- Primarily an extrathoracic tumor and may involve the lymphatic system, intercostal nerves, brachial plexus, stellate ganglion, the sympathetic chain, ribs, and vertebrae
EPIDEMIOLOGY
Incidence
- Much less common than other lung cancers, comprising 3 " 5% of all nonsmall cell lung cancers (NSCLCs)
- Otherwise has similar demographics to other lung cancers
ETIOLOGY AND PATHOPHYSIOLOGY
- Majority of tumors are NSCLCs, with adenocarcinomas being the most common (50%), followed by squamous cell and large cell carcinomas.
- Small cell carcinomas account for only 5% of superior sulcus tumors.
RISK FACTORS
- Tobacco use
- Secondhand tobacco exposure
- Asbestos exposure
- Exposure to industrial elements, such as gold and nickel
GENERAL PREVENTION
Avoidance of risk factors
COMMONLY ASSOCIATED CONDITIONS
Paraneoplastic syndrome (infrequent)
DIAGNOSIS
HISTORY
- Patients present with shoulder or chest wall pain, which may radiate to the neck, scapula, axilla, or arm in the C8, T1, and T2 dermatomes.
- Patients may also present with Horner syndrome and weakness/atrophy of the intrinsic muscles of the hands; along with the pain noted earlier, these symptoms are collectively known as Pancoast syndrome.
- Less commonly, patients may present with paraplegia, superior vena cava syndrome, or involvement of the phrenic or laryngeal nerves.
- Cough, dyspnea, and hemoptysis are uncommon because of the peripheral location of the tumor.
PHYSICAL EXAM
- Weakness and atrophy of the intrinsic muscles of the hands
- Absent triceps reflex
- Supraclavicular lymphadenopathy
- Weight loss
- May be associated with Horner syndrome (ipsilateral ptosis, miosis, enophthalmos [recession of eye into socket], and anhidrosis)
DIFFERENTIAL DIAGNOSIS
Broadly grouped into three processes (1)
- Neoplasms
- Primary bronchogenic carcinoma
- Other primary thoracic neoplasms: adenoid cystic carcinoma, hemangiopericytoma, and mesothelioma
- Metastatic neoplasm: carcinoma of the larynx, thyroid, cervix, and urinary bladder
- Hematologic neoplasm: plasmacytoma, lymphomatoid granulomatosis, lymphoma, and multiple myeloma
- Infectious processes
- Bacterial: staphylococcal, Pseudomonas, actinomycosis
- Fungal: aspergillosis, allescheriosis, cryptococcosis
- Tuberculosis
- Parasitic: hydatid cyst
- Other causes
- Cervical rib syndrome
- Pulmonary amyloidoma
- Subclavian vessel aneurysm
DIAGNOSTIC TESTS & INTERPRETATION
CBC, complete metabolic panel, and prothrombin time/partial thromboplastin time can be abnormal in patients with paraneoplastic syndrome.
- Chest x-ray findings can be minimal; they may reveal a small apical cap or pleural thickening, bone destruction of a rib or vertebral body, or mediastinal enlargement.
- CT scan of the chest, brain, and abdomen should be performed to characterize the lesion and evaluate for distant metastases.
Initial Tests (lab, imaging)
- MRI of the chest may be helpful to evaluate tumor extent and surgical resectability.
- PET scan is an alternative method for staging purposes.
- Bone scan may be performed to evaluate for bone metastasis.
- Angiogram or venogram may be helpful if vascular involvement is suspected.
Diagnostic Procedures/Other
- Percutaneous tissue biopsy via US or CT guidance to establish pathology.
- Video-assisted thoracoscopy or thoracotomy may be used if less invasive techniques are nondiagnostic.
- Mediastinoscopy may be used to evaluate for mediastinal involvement or lymphadenopathy.
- Endobronchial US may also be used to evaluate for mediastinal metastasis.
- Pulmonary function tests should be considered to evaluate lung function.
- Bronchoscopy has limited role because of peripheral location.
- Tumor markers and oncogenes may have some prognostic value, although their role remains undefined.
TREATMENT
- Depends on the pathology and staging of the tumor.
- Patients with localized NSCLC (T3 " 4, N0 " 1) should receive trimodal treatment with concurrent chemotherapy and radiation therapy followed by surgical resection (2)[A].
- Goal of surgery is complete removal by en bloc resection of all invaded structures.
- Contraindications to surgery include metastatic disease, invasion of the brachial plexus above C7, and invasion of vertebrae.
- For patients with locally advanced, unresectable disease or metastatic disease, chemotherapy and/or radiation therapy is recommended.
MEDICATION
- Chemotherapy regimens include mitomycin, vindesine, cisplatin, or etoposide and cisplatin.
- Pain control often requires opioid pain medicine.
ADDITIONAL THERAPIES
Concurrent preoperative radiation therapy to a total dose of 45 Gy
SURGERY/OTHER PROCEDURES
Surgical approaches include the following:
- Posterior (Paulson)/posterolateral-paravertebral thoracotomy
- Anterior-cervical
ONGOING CARE
FOLLOW-UP RECOMMENDATIONS
Patient Monitoring
History, exam, chest x-ray, and CT scan at routine intervals
PROGNOSIS
- Only 30 " 40% of cases are eligible for multimodal protocols of treatment (3).
- Those who undergo trimodal treatment with complete surgical resection have a 5-year survival of 54 " 77% (4).
- Prognosis is poor for those with mediastinal nodal involvement; extension of tumor into base of neck, vertebral bodies, or great vessels; Horner syndrome; or incomplete resection.
- High risk of recurrent local and metastatic disease; the brain is the most common site of recurrent metastasis for which prophylactic cranial irradiation has been considered.
COMPLICATIONS
- Cardiopulmonary complications are common postoperatively, including myocardial ischemia, pulmonary embolus, and respiratory failure.
- Surgical resection may also result in permanent neurologic deficits.
- Side effects of chemotherapy or radiation therapy
- Recurrent disease
REFERENCES
11 Arcasoy SM, Jett JR. Superior pulmonary sulcus tumors and Pancoast 's syndrome. N Engl J Med. 1997;337(19):1370 " 1376.22 Nikolaos P, Vasilios L, Efstratios K, et al. Therapeutic modalities for Pancoast tumors. J Thorac Dis. 2014;6(Suppl 1):S180 " S193.33 Kappers I, Belderbos JS, Burgers JA, et al. Non-small cell lung carcinoma of the superior sulcus: favourable outcomes of combined modality treatment in carefully selected patients. Lung Cancer. 2008;59(3):385 " 390.44 Foroulis CN, Zarogoulidis P, Darwiche K, et al. Superior sulcus (Pancoast) tumors: current evidence on diagnosis and radical treatment. J Thorac Dis. 2013;5(Suppl 4):S342 " S358.
ADDITIONAL READING
- Kozower BD, Larner JM, Detterbeck FC, et al. Special treatment issues in non-small cell lung cancer: diagnosis and management of lung cancer, 3rd ed: American College of Chest Physicians evidenced-based clinical practice guidelines. Chest. 2013;143(5)(Suppl):e369S " e399S.
- Panagopoulos N, Leivaditis V, Koletsis E, et al. Pancoast tumors: characteristics and preoperative assessment. J Thorac Dis. 2014;6(Suppl 1):S108 " S115.
CODES
ICD10
- C34.10 Malignant neoplasm of upper lobe, unsp bronchus or lung
- C34.11 Malignant neoplasm of upper lobe, right bronchus or lung
- C34.12 Malignant neoplasm of upper lobe, left bronchus or lung
ICD9
162.3 Malignant neoplasm of upper lobe, bronchus or lung
SNOMED
Pancoast tumor
CLINICAL PEARLS
- Initial diagnosis may be delayed because the initial presentation of shoulder pain and ulnar neuropathy can be misdiagnosed as an orthopedic or neurologic disorder, and pulmonary symptoms, if any, are often a late finding.
- Shoulder and arm pain in the C8, T1, and T2 dermatomes; ipsilateral Horner syndrome; and wasting of the hand muscles are the signs and symptoms of Pancoast syndrome.
- Tissue pathology and staging are critical for treatment recommendations.
- Only 30 " 40% of cases are T3 " 4, N0 " 1, which are eligible for multimodality treatment of concurrent chemotherapy and radiation therapy followed by surgical resection.
- Prognosis is poor for those with mediastinal nodal involvement; extension of tumor into base of neck, vertebral bodies, or great vessels; Horner syndrome; or incomplete resection.