A superior sulcus tumor, also commonly referred to as a Pancoast tumor, is a rare form of lung cancer that arises in the upper portion (apex) of either the right or the left lung and interferes with the surrounding structures — the ribs, the lining of the chest cavity, the spine, nerves and blood vessels.
These tumors were previously thought to be unresectable, but advances in radiation therapy and surgical techniques have greatly improved outcomes for patients. However, treatment of these rare tumors is complex and requires a multi-disciplinary approach from various physicians specializing in thoracic surgery, medical oncology and radiation medicine. At Roswell Park, we are proud to offer the excellence in multidisciplinary thoracic surgical care necessary for treating these tumors.
Signs and Symptoms
Pain is the most common presenting symptom occurring in 44-96 percent of patients. A classic syndrome called the Pancoast-Tobias syndrome causes severe pain in the neck, arm, wrist, armpit, shoulder and chest wall. Chest pain and shoulder pain are common and caused by the tumor invading the highly innervated lining of the chest wall overlying the ribs or spine.
Numbness, tingling in the fingers, or weakness of the hand and arm can occur on the affected side of the body. Numbness of the hand or inner arm indicates compression of the brachial plexus by the tumor and not necessarily nerve invasion. Weakness or atrophy of the muscles of the hand is caused by tumor invasion of the ulnar nerve.
A droopy eyelid and pupil constriction is a condition called Horner’s Syndrome and involves the sympathetic nervous system. This occurs in up to 40 percent of patients with Pancoast tumors.
Excessive sweating or flushing of the face on the affected side can result as well.
The overwhelming majority of superior sulcus tumors are non-small cell lung cancers, but the presence of a tumor in this location does not guarantee a diagnosis of lung cancer. Lymphoma, tuberculosis, and tumors originating from tissues of the chest wall can present with identical appearance. Superior sulcus tumors are rare and account for only 3-5 percent of all lung cancers.
The diagnosis process for Pancoast tumor has multiple steps. If initial imaging — chest x-ray or CT scan — shows suspicion of a Pancoast tumor, a PET/CT scan or an MRI is performed. An MRI is the most sensitive test for assessing the neurological and vascular structures surrounding the tumor. Once imaging is obtained, a percutaneous biopsy is performed by an interventional radiologist. This involves numbing the skin with a local anesthetic and using CT scan imaging as guidance, directing the biopsy needle into the tumor to obtain a tissue sample. This is successful in over 90 percent of cases and is the diagnostic procedure of choice in most cases. In some situations, a flexible bronchoscopy is used to biopsy both the tumor and surrounding lymph nodes.
Treatment of these rare tumors is complex and requires a multi-disciplinary approach from various physicians specializing in thoracic surgery, medical oncology, and radiation medicine. At Roswell Park, we are proud to offer the excellence in multidisciplinary thoracic surgical care necessary for treating these tumors.
Treatment consists of chemotherapy and radiation performed simultaneously prior to planned surgical resection. After completing chemotherapy and radiation, repeat staging with PET/CET is performed before proceeding with planned surgery.
Chemotherapy and Radiation
For patients with no evidence of metastatic disease, induction chemotherapy and radiation are used to treat the tumor prior to any planned surgical intervention. Typically chemotherapy is given for two or three cycles, and external beam radiation is given in daily treatments. In two large reported studies, approximately 75 percent of patients who initiated treatment with chemotherapy and radiation were able to undergo surgical resection.
Surgery for Pancoast tumors involves resecting the upper lobe of the lung as well as any portion of the chest wall, or other structures that are involved with the tumor. Roswell Park is a high-volume thoracic surgery center with expertise in the complex surgical management of these tumors. In many cases, surgeons from multiple specialties may be involved in the procedure. In selected situations, a minimally invasive approach may be feasible for your surgical resection.