Version May 2024
INTRODUCTION — Superior sulcus tumors were first described in 1838, but they were an obscure entity until the reports of Henry Pancoast in the first third of the 20th century [1]. Pancoast mistakenly believed that these neoplasms arose from embryonal rests of the fifth branchial cleft; other investigators subsequently recognized the pulmonary origin of the vast majority of these malignancies.
Since Pancoast original reports, the terms "Pancoast tumors," "Pancoast-Tobias tumors," "superior sulcus tumors," or "superior pulmonary sulcus tumors" have been applied to neoplasms located at the apical pleuropulmonary groove, adjacent to the subclavian vessels (figure 1) [2-4]. The actual pulmonary sulcus comprises the thoracic costovertebral gutter on either side of the vertebral column and is limited by the arch of the first rib superiorly and the diaphragmatic insertion inferiorly. Tumors located at the upper part of the pulmonary sulcus near the thoracic inlet may correctly be regarded as superior sulcus tumors, although the inferior margins of the superior sulcus are not well defined.
The diagnosis and management of tumors arising within the superior sulcus are reviewed here. For patients with resectable disease, concurrent chemoradiotherapy is the recommended initial step in patient management (whereas in other cases of early-stage lung cancer, patients often proceed initially with surgery). Chemoradiotherapy is followed by surgical resection if there is no evidence of distant metastases or local progression. General issues regarding lung cancer and management of stage II and stage III non-small cell lung cancer are reviewed separately. (See "Clinical manifestations of lung cancer" and "Management of stage I and stage II non-small cell lung cancer" and "Management of stage III non-small cell lung cancer".)
CLINICAL MANIFESTATIONS — Lesions in the superior sulcus may result in shoulder and arm pain (in the distribution of the C8, T1, and T2 dermatomes), Horner syndrome (ipsilateral ptosis, miosis, and anhidrosis; caused by injury to the sympathetic nerve chain), and weakness and atrophy of the muscles of the hand, a constellation of symptoms referred to as Pancoast syndrome or Pancoast-Tobias syndrome (figure 2) [4]. The majority of patients with superior sulcus tumors present with one or more of these complaints. Due to the peripheral location of the tumor, pulmonary symptoms such as cough, hemoptysis, and dyspnea are uncommon until late in the disease.
Shoulder pain — The most common initial symptom of superior sulcus tumors is shoulder pain, present in 44 to 96 percent of patients [5-11]. Pain is produced by invasion of the brachial plexus and/or extension of the tumor into the parietal pleura, endothoracic fascia, first and second ribs, or vertebral bodies. Pain can progress and radiate up to the head and neck, or down to the medial aspect of the scapula, axilla, anterior chest, or ipsilateral arm in the distribution of the ulnar nerve [10].
Patients frequently receive treatment for presumed cervical osteoarthritis or shoulder bursitis, resulting in a delay in diagnosis of five to ten months in some series [8,12-14]. Pain ultimately becomes persistent and of such severity that the patient may need to support the involved arm with the uninvolved hand in order to obtain relief [2,6,11]. Adequate pain control measures are important when caring for patients with superior sulcus tumors. (See "Overview of cancer pain syndromes", section on 'Plexopathies' and "Brachial plexus syndromes", section on 'Neoplastic and radiation-induced brachial plexopathy'.)
Horner syndrome — Horner syndrome consists of ipsilateral ptosis with narrowing of the palpebral fissure, miosis, enophthalmos, and anhidrosis. It is caused by involvement of the paravertebral sympathetic chain and the inferior cervical (stellate) ganglion (figure 3 and picture 1). Its prevalence in patients with superior sulcus tumors has ranged from 14 to 83 percent in different series [5,7,8,10,12,14-16].
Ipsilateral flushing and increased sweating of the face may occur before the development of the full Horner syndrome, presumably due to irritation of the sympathetic chain by the tumor prior to frank invasion [4]. Ipsilateral complex regional pain syndrome (reflex sympathetic dystrophy) and facial pain similar to paroxysmal hemicrania rarely may occur [17,18]. Contralateral facial sweating and flushing, especially with exercise, due to an excessive response by the intact sympathetic pathway have also been reported; this phenomenon is known as the Harlequin sign (picture 2) [19]. (See "Horner syndrome" and "Complex regional pain syndrome in adults: Pathogenesis, clinical manifestations, and diagnosis".)
Neurologic complications involving the upper extremity — Extension of tumor to the C8 and T1 nerve roots results in upper extremity neurologic findings in approximately 8 to 22 percent of cases [4,10]. Involvement of these nerve roots may result in weakness and atrophy of the intrinsic muscles of the hand, or pain and paresthesia of the fourth and fifth digits and the medial aspect of the arm and forearm. Abnormal sensation and pain in the T2 territory (the axilla and medial aspect of the upper arm) may also be an early finding, and the triceps reflex may be lost [20].
Superior sulcus tumors invade the intervertebral foramina early in the course of disease in approximately 5 percent of patients and may cause spinal cord compression and paraplegia [4]. Approximately 25 percent of patients ultimately develop spinal cord compression. (See "Clinical features and diagnosis of neoplastic epidural spinal cord compression".)
Other findings — Supraclavicular lymph node enlargement and prominent weight loss are each observed in approximately 25 to 35 percent of cases [4,10]. Superior sulcus tumors may produce a phrenic or recurrent laryngeal neuropathy or superior vena cava syndrome in 5 to 10 percent of patients [4,10]. (See "Malignancy-related superior vena cava syndrome".)
DIAGNOSIS AND PATHOLOGY — A histologic diagnosis is mandatory prior to definitive treatment because of the wide variety of malignancies and benign pathologic processes that can produce Pancoast syndrome.
Diagnosis — Core needle biopsy is generally preferred to provide tissue for histology and molecular markers. The location of superior sulcus tumors allows the majority to be diagnosed by percutaneous needle biopsy. This technique can be performed via a posterior or cervical approach with the use of biplane fluoroscopy, ultrasonography, or computed tomography to localize the lesion. Diagnostic yields of more than 90 percent are reported [15,21,22]. Video-assisted thoracoscopy (VATS) or thoracotomy can be performed if less invasive techniques are nondiagnostic.
By contrast, the peripheral location of superior sulcus tumors decreases the diagnostic yield of bronchoscopy and is therefore less useful for diagnostic purposes for superior sulcus tumors. Fiberoptic bronchoscopy with cytology and biopsies provides a diagnosis in 30 to 40 percent of cases, but unexpected endobronchial tumors may occasionally be detected, significantly altering therapeutic decisions [8,14,23].
Pathology — The overwhelming majority of superior sulcus tumors are non-small cell lung cancers (NSCLCs), and in the past were mainly squamous cell carcinomas [2,8,15,24-27], although in subsequent series adenocarcinomas predominate [28-30]. Although NSCLC predominates as the cause of superior sulcus tumors, less than 5 percent of all NSCLCs arise in this region [5,24]. Small cell carcinomas account for up to 5 percent of cases in most series [5,9,28,31-33].
Broad molecular testing for epidermal growth factor receptor (EGFR) mutations, anaplastic lymphoma kinase (ALK) rearrangement, c-ROS oncogene 1 (ROS1) rearrangement, programmed cell death receptor 1 (PD-1) expression, and other targetable mutations in nonsquamous NSCLC should be performed for patients who have stage IV disease, are not candidates for definitive local therapies, or for those who recur after definitive local treatment. In recurrent tumors, this testing is ideally carried out on tissue obtained at the time of recurrence [34]. (See "Personalized, genotype-directed therapy for advanced non-small cell lung cancer".)
Differential diagnosis — A variety of rarer lesions other than lung cancer can arise in the superior sulcus and cause Pancoast syndrome. The differential diagnosis of superior sulcus mass lesions includes adenoid cystic carcinoma, hemangiopericytoma, mesothelioma, lymphoma, plasmacytoma, and metastatic malignancies from the cervix, larynx, liver, bladder, and thyroid gland [35-43]. Lymphomatoid granulomatosis, vascular aneurysms, amyloid nodules, cervical rib syndrome, and various infections (eg, tuberculosis, fungi, hydatid cysts, sequelae of bacterial pneumonia) can also result in Pancoast syndrome [44-58].
STAGING — Superior sulcus tumors are staged in the same way as non-small cell lung cancers (NSCLCs) located elsewhere in the thorax (figure 4). The eighth edition of the tumor, node, metastasis (TNM) system is used to stage primary lung tumors (table 1 and table 2). Superior pulmonary sulcus tumors are typically T3 or T4 lesions, depending on whether the tumor extends only as far as the chest wall or invades the brachial plexus, mediastinal structures, or vertebral bodies. In the absence of metastases to scalene nodes, supraclavicular nodes, contralateral mediastinal nodes (figure 4), or distant sites, the tumors usually fall into the category of stage IIB (T3,N0), IIIA (T3,N1, or T4, N0-1), or IIIB (T3-4,N2) disease, classified according to the American Joint Committee on Cancer/Union for International Cancer Control eighth edition TNM staging system. (See "Overview of the initial evaluation, diagnosis, and staging of patients with suspected lung cancer" and "Tumor, node, metastasis (TNM) staging system for lung cancer".)
PRETREATMENT EVALUATION — Accurate staging of superior sulcus tumors is critical so that optimal therapy may be provided and unnecessary treatment-related morbidity can be avoided. As in other situations, preoperative evaluation should be performed to determine the patient's ability to tolerate resection based on cardiovascular and pulmonary risk stratification and should include a shared decision-making discussion with patient about risks and benefits. (See "Preoperative physiologic pulmonary evaluation for lung resection" and "Evaluation of cardiac risk prior to noncardiac surgery".)
Metastatic spread to the mediastinum or distant sites should be diligently sought by history, physical examination, blood tests, and imaging. Computed tomography (CT) of the chest and upper abdomen should be performed to evaluate the mediastinum, liver, and adrenal glands for possible metastatic foci. Positron emission tomography (PET) scanning is necessary for preoperative evaluation of mediastinal lymph nodes as well as distant metastases. (See "Overview of the initial evaluation, diagnosis, and staging of patients with suspected lung cancer" and "Tumor, node, metastasis (TNM) staging system for lung cancer".)
Initial imaging — Radiographic findings of superior sulcus tumors include a unilateral apical cap of more than 5 mm, asymmetry of bilateral apical caps of more than 5 mm, an apical mass, and bone destruction [8,11,59]. Lordotic chest views and upper thoracic and lower cervical spine radiographs may be helpful in the visualization of the tumor (image 1).
CT of the chest provides additional information about a superior sulcus tumor and its extension, including the presence of satellite pulmonary nodules, parenchymal disease, and hilar or mediastinal lymphadenopathy (image 2) [59-62]. CT is limited, however, in the evaluation of brachial plexus, subclavian vessel, and chest wall involvement.
Although the comparative value of magnetic resonance imaging (MRI) over CT for detection of chest wall invasion is not clearly established, many studies suggest that MRI is more accurate [61-68]. MRI is especially useful in documenting the extent of involvement of the brachial plexus and subclavian vessels, and evaluating vertebral bodies and the spinal canal for tumor extension (image 3A-C). A magnetic resonance angiogram may be useful for detecting vascular involvement of the subclavian artery [69].
Although some studies have suggested that MRI is superior to CT in the detection of mediastinal lymph node involvement, the accuracy of these two methods is generally similar [70,71]. Studies evaluating the efficacy of CT staging of mediastinal lymph node metastases for non-Pancoast NSCLC have reported sensitivity and specificity of 60 to 70 percent. fluorodeoxyglucose PET-CT is more accurate for mediastinal lymph node evaluation than CT alone. (See "Magnetic resonance imaging of the thorax" and "Overview of the initial evaluation, diagnosis, and staging of patients with suspected lung cancer" and "Tumor, node, metastasis (TNM) staging system for lung cancer".)
Mediastinal evaluation — CT and MRI cannot definitively establish the presence or absence of mediastinal invasion or nodal metastasis [72-74], and surgical staging of the mediastinum should generally be undertaken prior to attempts at curative surgery.
Although a negative PET scan appears highly accurate in predicting an uninvolved mediastinum, surgical staging of the mediastinum prior to definitive treatment is generally considered the standard of care. PET scanning may also be the most sensitive test for identifying the presence of distant metastases in liver, adrenal gland and other lymph nodes. PET scans are routinely performed as a component of the staging evaluation.
Endobronchial ultrasound (EBUS) is a useful tool for establishing mediastinal metastasis, especially with enlarged or PET positive lymph nodes in the mediastinum. Establishing N2 or N3 metastases by EBUS precludes the need for mediastinoscopy. (See "Endobronchial ultrasound: Indications, contraindications, and complications".)
Brain imaging — MRI or CT scans of the brain provide complementary staging information. We recommend routine preoperative brain imaging with MRI (CT if the patient cannot undergo MRI) for superior sulcus tumors because of the high propensity for brain metastasis from bronchogenic carcinoma in this location [75]. (See "Overview of the initial evaluation, diagnosis, and staging of patients with suspected lung cancer" and "Tumor, node, metastasis (TNM) staging system for lung cancer".)
TREATMENT — For patients with resectable disease, concurrent chemoradiotherapy is the recommended initial step in patient management. This is followed by surgical resection if there is no evidence of distant metastases or local progression and postoperative chemotherapy. Adjuvant atezolizumab is now used for patients with stage II to IIIA non-small cell lung cancer (NSCLC) with programmed cell death ligand 1 (PD-L1) expression of 1 percent or greater [76], and adjuvant osimertinib is offered for patients with resected epidermal growth factor receptor (EGFR)-positive NSCLC [77].
Induction chemoradiotherapy plus surgery — In patients with locally advanced, stage III NSCLC, randomized trials have demonstrated that induction chemotherapy followed by definitive radiation therapy (RT) improves survival compared to RT alone. Further studies showed that concurrent chemotherapy plus RT provides an additional survival advantage over sequential treatment. The success of these approaches in the treatment of non-Pancoast, locally advanced, stage III NSCLC has led to their use in patients with superior sulcus tumors and defined the standard of care for these tumors. (See "Management of stage III non-small cell lung cancer".)
Although there are no randomized trials comparing induction chemoradiotherapy with preoperative RT or surgery alone specifically for patients with superior sulcus tumors, several studies have focused on this approach in this setting. The potential value of this is illustrated by the following results:
●A best-evidence analysis of the literature concluded that induction chemoradiotherapy followed by surgery resulted in superior survival compared with radiotherapy followed by surgery or surgery alone for patients with superior sulcus tumors [78].
●The largest prospective study was conducted in North America as a cooperative intergroup study in 111 patients, and evaluated concurrent chemotherapy (two cycles of cisplatin and etoposide) and thoracic RT (45 Gy in 25 fractions) followed by resection three to five weeks later, and two additional cycles of postoperative chemotherapy [79]. Eligible patients had pathologically proven T3-4, N0-1 NSCLC presenting in the superior sulcus, and all underwent staging mediastinoscopy. Patients with positive N2 lymph node metastases were excluded from the study.
Mature results from this study found that 80 percent of patients underwent thoracotomy and 76 percent had a complete (R0) resection of tumor [80]. The overall five-year survival rate for the entire series was 44 percent. Patients with a complete pathologic response at resection had a significantly better prognosis than those with either microscopic or gross residual disease.
●Similar results were seen in a Japanese study that utilized two cycles of chemotherapy with mitomycin, vindesine, and cisplatin concurrent with split-course RT (45 Gy in 25 daily fractions). This was followed by surgery two to four weeks after completion of induction therapy [81]. There were three treatment-related deaths. Of the 76 enrolled patients, 57 underwent surgery (76 percent), of whom 51 (68 percent) had a complete (R0) resection. There were 12 complete pathologic responses. The disease-free and overall survival rates at five years were 45 and 56 percent, respectively. Systemic recurrence was the main reason for death in both trials, reported in approximately 80 percent of patients who recurred.
●A single institution study from France enrolled 107 patients with Pancoast tumors and treated with induction chemoradiotherapy (etoposide and cisplatin). Patients with bulky N2 and N3 disease were excluded. Of these, 72 had a thoracotomy and all but one had macroscopic complete resections. There were 21 pneumonectomies performed, and four patients died of surgical complications. A pathological complete response or microscopic residual disease was observed in 40 percent of all surgical participants. After induction therapy, 61 percent of pathological N2 positive participants (28 of 46) were downstaged to pN0/N1. Overall survival for the entire cohort was 55 and 40 percent at two and three years, respectively, with a median survival time of 26.7 months [82].
Although a randomized trial comparing induction chemoradiotherapy with preoperative RT or surgery alone has not been conducted, a retrospective review of 35 patients from a single institution found that concurrent chemoradiotherapy with a cisplatin-based regimen followed by surgery resulted in improved survival rates (93 versus 49 percent at two years and 84 versus 49 percent at four years, compared to induction RT followed by surgery) [83]. There was also a higher rate of complete or near complete pathologic response with concurrent chemoradiotherapy (13 of 15 [87 percent] versus 7 of 20 [35 percent]).
Similarly, another center utilizing a trimodality approach for Pancoast tumors observed a pathologic complete response of 40 percent in 36 patients [84]. The overall median survival was 2.6 years in all patients and eight years for those with a pathologic complete response.
These results are better than those obtained with either preoperative RT or surgery alone. As a result, induction chemoradiotherapy followed by surgery has been adopted by most centers as the standard treatment for Pancoast tumors without distant or mediastinal metastases.
Choice of regimen — Although the optimal regimen has not been established, a reasonable choice is that used in the North America Intergroup study [79]:
●Concurrent thoracic RT (45 Gy over five weeks in daily 1.8 Gy fractions) and chemotherapy, consisting of two cycles of cisplatin (50 mg/m2 on days 1, 8, 29, and 36) and etoposide (50 mg/m2 on days 1 to 5 and 29 to 33). Some clinicians have advocated increasing the thoracic RT dose to 60 to 66 Gy.
●Thoracotomy for tumor resection three to five weeks after completion of chemoradiotherapy, if there is no evidence of distant metastases or local progression.
●Two additional postoperative courses of chemotherapy with a platinum-based doublet. (See "Initial management of advanced non-small cell lung cancer lacking a driver mutation", section on 'Preference for platinum-based regimens'.)
Based upon the favorable results of the phase II study (SWOG 9504) with consolidation docetaxel after definitive chemoradiotherapy in patients with stage III NSCLC, the lung intergroup completed a trial (SWOG-S0220) in patients with Pancoast tumor, but found that consolidation chemotherapy with single-agent docetaxel following surgery was not feasible [85]. Of note, the phase III confirmatory trial demonstrated that consolidation docetaxel after definitive chemoradiotherapy did not lead to improved survival and resulted in increased toxicities [86]. (See "Management of stage III non-small cell lung cancer", section on 'Preferred approach: Chemoradiotherapy, followed by durvalumab'.)
Incorporation of immunotherapy into neoadjuvant regimens for locally or locoregionally advanced NSCLC is discussed elsewhere, although this is not a standard approach at this time . (See "Systemic therapy in resectable non-small cell lung cancer", section on 'Patients receiving neoadjuvant treatment'.)
Surgery — Surgery is generally undertaken three to five weeks after the completion of chemoradiotherapy. This is typically carried out through en bloc resection of the tumor and the chest wall, and, depending upon the extent of local invasion, may require resection of the paravertebral sympathetic chain, stellate ganglion, lower trunks of the brachial plexus, subclavian artery, or portions of the thoracic vertebrae. One of two surgical approaches may be utilized:
●The posterior approach, also termed the Paulson operation, is performed via a long incision along the medial edge of the scapula starting between the second or third thoracic vertebra and the scapula, extending underneath its inferior tip, and ending at the anterior axillary line [2,87,88].
●The anterior transcervical approach involves a long L-shaped incision that extends from the mandibular angle along the anterior border of the sternocleidomastoid muscle down to the suprasternal notch and laterally under the medial half of the clavicle [87,89,90]. This technique is generally used for tumors located anteriorly at the thoracic inlet, particularly those invading the subclavian vessels. A variation of this approach, the anterior "hemi-clamshell" incision, permits sparing of the clavicle [91-93].
The extent of lung resection is determined by the size and location of the primary tumor and the patient's underlying pulmonary function. Lobectomy is generally considered the preferable procedure for lung cancers, with fewer local recurrences and better survival when compared with sublobar resections [26,87,94].
A combined thoracic-neurosurgical approach is necessary in treating tumors that invade the brachial plexus and/or the spine [16,95-98]. Recent advances in spinal instrumentation have allowed more complete resection of tumors involving the vertebral body [96-98]. Surgery is contraindicated by extensive invasion of the brachial plexus, the intervertebral foramina, or soft tissues at the base of the neck; by mediastinal perinodal involvement; and by venous obstruction [2,6,11,14,99]. Involvement of the vertebral bodies, subclavian artery, and spread to the ipsilateral supraclavicular lymph nodes are relative, but not absolute contraindications in the opinion of some authors [74,89,97].
Complications — In addition to the standard potential complications of lung resection, extirpation of superior sulcus tumors may result in chylothorax, ulnar nerve paralysis secondary to resection of the C8 nerve root, Horner syndrome (if not present preoperatively) due to resection of the stellate ganglion and the sympathetic chain, or a cerebrospinal fluid leak and meningitis. Resection of T1 and T2 nerve roots generally does not result in major clinical sequelae. Reported surgical mortality rates are approximately 4 to 10 percent [100,101].
Chemoradiotherapy or RT alone without surgery — Patients with locally advanced, unresectable (eg, N2, N3) disease and those who are medically inoperable but fit enough to receive chemotherapy should be offered chemoradiotherapy. In the setting of non-Pancoast stage III NSCLC, concurrent chemoradiotherapy has been shown to be superior to radiation therapy (RT) alone and has become the standard of care. Adjuvant immunotherapy with durvalumab for one year after definitive chemoradiotherapy has become the standard for stage III NSCLC. (See "Management of stage III non-small cell lung cancer", section on 'Preferred approach: Chemoradiotherapy, followed by durvalumab'.)
RT as a sole primary treatment modality is suitable for patients with metastatic tumors or poor performance status (table 1), providing palliation of pain in up to 90 percent of patients [5,9,102]. A dose of 60 to 66 Gy is generally recommended for definitive treatment of unresectable disease [11,28,103]. However a lower dose (eg, 30 Gy) is reasonable for palliation in those with distant metastases.
Five-year survival rates up to 40 percent have been reported for selected patients treated definitively with radical RT for localized superior sulcus tumors [5,9,103,104]. However, it is difficult to compare results with RT alone to those obtained with combined modality therapy, since many studies of primary RT have included patients with unfavorable prognostic factors and unresectable tumors [103]. Furthermore, many series include patients treated before the era of imaging with CT and/or magnetic resonance imaging, who were thus less adequately staged.
While resection is generally accepted as a component of treatment for locally advanced NSCLC, its value is uncertain in patients with stage IIIA (N2) disease (table 1). Interim reports of the major randomized prospective study addressing the value of resection when added to chemoradiotherapy have failed to demonstrate a statistically significant survival advantage for the addition of resection to chemoradiotherapy for locally advanced non-Pancoast NSCLC [105]. (See "Management of stage III non-small cell lung cancer", section on 'No clinical mediastinal involvement prior to surgery'.)
Common side effects of RT include fatigue, esophagitis, localized lung fibrosis, and skin irritation. Less commonly patients develop symptomatic pneumonitis. Rare complications include skin fibrosis with shoulder immobility; myelitis and brachial neuritis may result in weakness and pain in the hand, arm, or shoulder [5,106]. (See "Radiation-induced lung injury" and "Brachial plexus syndromes", section on 'Neoplastic and radiation-induced brachial plexopathy'.)
PROGNOSTIC FACTORS — In addition to tumor stage, several clinical and histologic features are prognostically important in patients with superior sulcus tumors. The presence of Horner syndrome, extension of the tumor into the base of the neck, vertebral bodies or the great vessels, or involvement of the mediastinal lymph nodes is associated with a worse prognosis [104,107]. Longer duration of symptoms has also been found to adversely affect the prognosis, probably because of an association with more advanced disease [14].
A good performance status (table 3) and weight loss of no more than 5 percent of total body weight are associated with better survival [10,28]. Local control of the tumor by radiation and/or surgery as well as the achievement of pain relief after treatment are encouraging and suggest a better prognosis. Most studies have not found an association between histologic subtype and survival.
A pathologic complete response after induction chemoradiotherapy has generally been correlated with better survival. A study from the Netherlands performed fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) before and after induction therapy. Changes in standardized uptake values (SUV) were predictive of an excellent pathologic response (≤10 percent viable cells) and correlated with residual tumor cells [108].
POST-THERAPY SURVEILLANCE — The evidence from these studies does not establish a clear-cut benefit for aggressive surveillance following treatment with curative intent. Guidelines from the American Society of Clinical Oncology [109] and the National Comprehensive Cancer Network [110] differ in their use of imaging studies. We suggest a history, physical examination, and computed tomography of the chest every three to six months for the first three years, then every six months for two years and annually thereafter. (See "Management of stage I and stage II non-small cell lung cancer", section on 'Post-therapy surveillance' and "Radiation-related risks of imaging".)
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Diagnosis and management of lung cancer".)
SUMMARY AND RECOMMENDATIONS — Superior sulcus tumors are characterized by a distinct constellation of presenting signs and symptoms due to their location. Most of these tumors are non-small cell lung cancers (NSCLCs). (See 'Clinical manifestations' above and 'Diagnosis and pathology' above.)
The management of Pancoast tumors is similar to that of other locally advanced NSCLCs:
●For patients with no distant metastatic disease, we recommend concurrent chemoradiotherapy as the initial step in patient management (Grade 1B). This is followed by surgical resection if there is no evidence of distant metastases or local progression (N2 or N3) (see 'Induction chemoradiotherapy plus surgery' above). Although the optimal regimen has not been established, a reasonable choice is that used in the North America Intergroup study:
•Concurrent thoracic radiotherapy (RT) (45 Gy over five weeks in daily 1.8 Gy fractions) and chemotherapy, consisting of two cycles of cisplatin (50 mg/m2 on days 1, 8, 29, and 36) and etoposide (50 mg/m2 on days 1 to 5, and 29 to 33). Some clinicians have advocated increasing the thoracic radiotherapy dose to 60 to 66 Gy.
•Tumor resection in patients without N2 metastases, three to five weeks after completion of chemoradiotherapy, if there is no evidence of distant metastases or local progression.
•Two additional postoperative courses of chemotherapy, with a platinum-based chemotherapy doublet if the patient's physical status permits.
●For patients who undergo surgical resection, adjuvant atezolizumab (for those with stage II to IIIA NSCLC and programmed cell death ligand 1 expression ≥1 percent) or osimertinib (for those with resected epidermal growth factor receptor (EGFR)-mutant disease) are offered, as discussed in detail elsewhere. (See "Systemic therapy in resectable non-small cell lung cancer", section on 'Adjuvant immunotherapy' and "Systemic therapy in resectable non-small cell lung cancer", section on 'EGFR-mutated cancers'.)
●For patients who are medically inoperable or who have locally advanced, unresectable disease, we recommend definitive chemoradiotherapy and adjuvant immunotherapy with durvalumab for one year. (Grade 1B).
●For patients who have distant metastatic disease or a poor performance status, RT can be used to treat symptoms due to the Pancoast tumor. For patients with locally advanced or metastatic disease, systemic therapy may be useful to palliate symptoms and extend survival duration. (See 'Chemoradiotherapy or RT alone without surgery' above and "Overview of the initial treatment of advanced non-small cell lung cancer".)
●Whenever possible, patients with superior sulcus tumors should be enrolled in prospective clinical trials so that the optimal therapy may be determined.
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges James R Jett, MD, who contributed to an earlier version of this topic review.
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