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Pancoast Syndrome

Pancoasts Syndrome

Pancoast syndrome is a term used to describe unilateral shoulder and arm pain, weakness, and atrophy of hand muscles and Horner's syndrome as a result of a mass in the lung apex compressing blood vessels and nerve trunks. Bronchogenic carcinoma is the most common underlying etiology. A detailed clinical and imaging workup, as well as biopsy, is necessary to make a definite diagnosis.


Presentation

Pancoast syndrome is an important clinical presentation of several disorders with different etiologies that develop in the apex of the lungs [1] [2] [3] [4]. Bronchogenic carcinoma, however, is by far the most common cause of this clinical syndrome characterized by one or more of the following symptoms [5]:

  • Shoulder pain - Proliferation of a mass from the lung apex eventually compresses the brachial plexus, parietal pleura, and the surrounding blood vessels, leading to severe ipsilateral and progressive pain in the shoulder [2] [3]. The pain can spread to the neck, anterior chest wall, and axilla, or progress down to the arm and hand, simultaneously producing weakness and atrophy of the muscles innervated by nerves stemming from C8-T1 roots of the brachial plexus (the ulnar nerve being predominantly affected) [2] [3] [4]. In some cases, blood vessel occlusion may lead to unilateral edema that further contributes to muscle atrophy [2] [6].
  • Horner syndrome - Enophthalmos, anhidrosis, ipsilateral miosis and ptosis comprise this neurological syndrome, which is encountered in up to 50% of patients with superior sulcus tumors, and the pathogenesis stems from a mechanical damage of the sympathetic plexus by a growing mass [2].

Although bronchogenic carcinomas are responsible for the development of Pancoast syndrome in the majority of cases (hence the terms Pancoast tumors or superior sulcus tumors), other conditions are neoplastic diseases (non-Hodgkin lymphomas, multiple myelomas, metastatic cancers of the liver and uterus), fungal infections (hydatid cysts as a result of Echinococcosis, histoplasmosis, aspergillosis, and mucormycosis) and pulmonary tuberculosis, whereas bacterial infections and other benign pathologies rarely induce Pancoast syndrome [1] [3] [6].

Arm Pain
  • Reference work entry DOI: 10.1007/978-3-540-85516-3_525 Definition The classic Pancoast syndrome presents symptoms and signs that include shoulder and arm pain along the distribution of the eighth cervical nerve trunk and first and second thoracic nerve[link.springer.com]
  • Shoulder and arm pain (from the brachial plexus invasion C8-T1) with wasting of the intrinsic hand muscles and paraesthesiae in the medial side of the arm.[patient.info]
  • Pancoast syndrome is a term used to describe unilateral shoulder and arm pain, weakness, and atrophy of hand muscles and Horner's syndrome as a result of a mass in the lung apex compressing blood vessels and nerve trunks.[symptoma.com]
  • Pancoast's syndrome is a constellation of characteristic symptoms and signs that includes shoulder and arm pain along the distribution of the eighth cervical nerve trunk and first and second thoracic nerve trunks, Horner's syndrome, and weakness and atrophy[nejm.org]
  • Pancoast's syndrome refers to superior sulcus tumors along with the triad of (1) shoulder and arm pain, (2) wasting of the hand muscles, and (3) ipsilateral Horner's syndrome (i.e., ptosis, miosis, and anhidrosis owing to invasion of the stellate ganglion[mhmedical.com]
Enophthalmos
  • •Horner’s Syndrome: miosis, ptosis, enophthalmos and anhidrosis. •Lower brachial plexus injury (C8-T1): Klumpke’s palsy. •Paresthesia of the upper extremity due to compression of subclavian artery & vein.[abcradiology.blogspot.com]
  • Horner syndrome - Enophthalmos, anhidrosis, ipsilateral miosis and ptosis comprise this neurological syndrome, which is encountered in up to 50% of patients with superior sulcus tumors, and the pathogenesis stems from a mechanical damage of the sympathetic[symptoma.com]
  • Clinical This is accompanied by (1) Severe pain in the shoulder region radiating toward the axilla and scapula along the ulnar aspect of the muscles of the hand, (2) Atrophy of hand and arm muscles, (3) Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos[pallipedia.org]
  • Presentation This syndrome results from the invasion of a number of structures and tissues around the thoracic inlet and may be characterised by: Ipsilateral invasion of the cervical sympathetic plexus, leading to Horner's syndrome (miosis, enophthalmos[patient.info]
  • Horner’s syndrome Horner’s syndrome, comprised of • ipsilateral partial ptosis, • meiosis, • enophthalmos, • anhydrosis, and • loss of ciliospinal reflex, results from invasion of the stellate ganglion and paravertebral cervical sympathetic chain. 42.[slideshare.net]
Shoulder Pain
  • A 61-year-old patient had the clinical findings of Pancoast syndrome with shoulder pain, weakness and atrophy of band muscles, and an opacified area in the apical lung field.[ncbi.nlm.nih.gov]
  • Shoulder pain: due to involvement of upper ribs and intercostal nerves.[abcradiology.blogspot.com]
  • Clinical presentation The syndrome consists of: shoulder pain C8-T2 radicular pain Horner syndrome The classical syndrome is uncommon, with Horner syndrome present in only 25%.[radiopaedia.org]
  • Pancoast'syndrome (known in French as Pancoast-Tobias's syndrome) associates shoulder pain, lower brachial plexopathy (C8 and T1 nerve roots) irradiating down to the auricular finger, an Horner's syndrome (Claude-Bernard-Horner's syndrome) with myosis[oncoprof.net]
  • Cause(s) : Non-small cell lung carcinoma Sometimes small cell carcinoma Symptoms : Severe shoulder pain, radiating towards axilla and scapula along the ulnar aspect of the muscles of the hand Atrophy of hand and arm muscles Compression of the blood vessels[medindia.net]
Neck Pain
  • […] ulnar aspect of the muscles of the hand, (2) atrophy of hand and arm muscles, (3) Horner syndrome (ptosis, miosis, hemianhidrosis, enophthalmos), and (4) compression of the blood vessels with edema." [6] Common presentations include: Pancoast syndrome Neck[physio-pedia.com]
Upper Back Pain
  • National Institutes of Health, National Cancer Institute, Lung Cancer Pancoast Tumor Pictures A 53-year-old man with a 50-pack-per-year history of smoking began experiencing upper back pain for several weeks.[emedicinehealth.com]
Edema of the Upper Extremity
  • […] of the upper extremity if the subclavian vein has to be ligated; Persistent post-thoracotomy intercostal neuralgia; Myatrophies and muscular weakness of the upper extremity if the ulnar nerve has to be sacrificed; Damage of the dura resulting in cerebrospinal[jtd.amegroups.com]

Workup

As Pancoast syndrome is the likely manifestation of a malignant disease, its early recognition is vital for ensuring optimal patient outcomes. Unfortunately, tumors are already at an advanced stage when Pancoast syndrome appears (T3 or T4 stage is observed in the majority of cases) [4]. Nevertheless, the immediate workup should start with a patient history that will note the timeline of complaints and their progression, whereas a properly conducted physical examination could identify unilateral pain of the shoulder and weakness of the ipsilateral arm, as well as constitutive features of Horner's syndrome. There should be a clinical suspicion regardless of the severity of symptoms, and imaging studies should be performed promptly, as they are the cornerstone in determining the etiology. Plain chest X-rays often reveal a homogeneous shadow or opacity in the lung apex and asymmetry of the apical caps of > 5 mm, but computed tomography (CT) is the gold standard [2] [3] [4] [5]. CT is able to confirm the state of the surrounding tissues and determine whether the mass has invaded the ribs, vertebral bodies, the mediastinum, the brachial plexus, but also if blood vessel occlusion has occurred [2] [4] [5]. Furthermore, magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) can be employed to obtain an even better view of the tissues [2] [4]. Even though bronchoscopy and subsequent biopsy of the lesion yields a definite diagnosis, the hardly accessible location of the lung apex does not allow for appropriate sampling [4] [5]. For this reason, transthoracic image-guided needle aspiration biopsy has established itself as the main technique for obtaining a viable sample for histopathological examination [1] [5].

Staphylococcus Aureus
  • We report the unusual occurrence of a case of Pancoast syndrome caused by a destructive sclerosing fibrosis after pulmonary Staphylococcus aureus infection.[ncbi.nlm.nih.gov]
  • Pancoast syndrome: an unusual complication of pulmonary infection by Staphylococcus aureus. Ann Thorac Surg . 1992 May. 53(5):903-4. [Medline] . Simpson FG, Morgan M, Cooke NJ. Pancoast's syndrome associated with invasive aspergillosis.[emedicine.medscape.com]
  • Pancoast’s syndrome secondary to lung infection with cutaneous fistulisation caused by Staphylococcus aureus. J Clin Pathol 2006;59:997-8. [ PubMed ] Beshay M, Roth T, Stein RM, et al. Tuberculosis presenting as Pancoast tumor.[jtd.amegroups.com]

Treatment

  • Early detection and prompt treatment of paraspinal metastasis are important to relieve symptoms and prevent permanent loss of neurological functions.[ncbi.nlm.nih.gov]
  • We describe the case of a patient with Pancoast syndrome caused by small cell lung carcinoma and discuss the aspects related to the diagnosis and treatment.[ncbi.nlm.nih.gov]
  • These patients had not responded to any other treatment, including nonsteroidal anti-inflammatory drugs, opioids, dexamethasone, tricyclic antidepressants, anticonvulsants, ketamine, and transcutaneous electric nerve stimulation.[ncbi.nlm.nih.gov]
  • Treatment Although superior sulcus tumors were considered categorically unresectable and resistant to radiotherapy (RT), multimodality therapy has been shown to be the optimal approach for the treatment of these tumors. - For tumors that invade the brachial[pallipedia.org]
  • This report emphasises the importance of establishing a firm pathologic diagnosis of the etiology of Pancoast's syndrome before instituting treatment.[unboundmedicine.com]

Prognosis

  • […] metastases are contraindications for surgery Preoperative radiation therapy followed by surgical resection is the most common form of treatment Complications Surgical complications include Atelectasis Chest pain Spinal fluid leaks Horner’s syndrome Prognosis[learningradiology.com]
  • Prognosis Until the 1990s bimodal treatment (pre-operative radiotherapy followed by surgery) resulted in a five-year survival at best of only 30%, and a recurrence rate of 70% [ 11 ] .[patient.info]
  • , Prognosis, Survival Rate Non Hodgkin’s lymphoma – Symptoms, Survival Rate, Treatment, Prognosis Appendix Cancer – Symptoms, Survival Rate, Prognosis, Treatment, Ewing’s Sarcoma – Prognosis, Survival Rate, Symptoms, Treatment[cancerwall.com]
  • PROGNOSIS • Depends on ETIOLOGY,not on SEVEARITY • SVCO recur in 10-30% pts,palliated with Intravascular self expanding STENT 34. PANCOAST SYNDROME 35.[slideshare.net]
  • Those patients staged N0 have a better prognosis than those with nodal metastases. Today, the standard of care for patients with a Pancoast tumor is radiation followed by tumor and chest wall resection when feasible.[angelfire.com]

Etiology

  • Pancoast syndrome is an important clinical presentation of several disorders with different etiologies that develop in the apex of the lungs.[symptoma.com]
  • This report emphasises the importance of establishing a firm pathologic diagnosis of the etiology of Pancoast's syndrome before instituting treatment.[unboundmedicine.com]
  • Venography • Can give precise level of obstruction • Less information on etiology of SVCS • Requires larger contrast dose 22.[slideshare.net]
  • Causes Non–small cell lung cancer is the most common etiology of Pancoast syndrome; adenocarcinoma, squamous cell carcinoma, and large cell carcinoma have all been observed.[pallipedia.org]
  • Etiology The etiology of Horner’s syndrome varies with the patient age and site of lesion. The etiology remains unknown in 35-40% of cases.[eyewiki.aao.org]

Epidemiology

  • He has since completed further training in emergency medicine, clinical toxicology, clinical epidemiology and health professional education.[lifeinthefastlane.com]
  • […] pseudoaneurysm in a child (caused by a hydatid cyst ) Risk factors are similar for almost all lung cancers and include the following: Prolonged asbestos exposure Exposure to industrial elements (eg, gold, nickel) Tobacco smoking Secondary smoke exposure Epidemiology[emedicine.medscape.com]
  • ICD-9 337.9 Epidemiology In a population based study of Horner’s syndrome in the pediatric age group, the incidence of Horner’s syndrome was estimated to be 1.42 per 100 000 patients younger than 19 years, with a birth prevalence of 1 in 6250 for those[eyewiki.aao.org]
  • […] disorder resulting from compression of the brachial plexus and/or subclavian vessels in the interval between the neck and axilla treatment may be nonoperative or include surgical decompression or a vascular procedure depending on the specific etiology Epidemiology[orthobullets.com]
Sex distribution
Age distribution

Pathophysiology

  • Grasp the nuances of the pathophysiology of today’s major respiratory conditions—including pulmonary hypertension, COPD, asthma, environmental lung disease, sleep disorders, infections of the immunocompromised, neonatal breathing disorders, and drug-resistant[books.google.com]
  • Pathophysiology Pancoast tumors are a subset of lung cancers that invade the apical chest wall. Because of their location in the pleural apex, they invade adjoining tissue.[emedicine.medscape.com]
  • Given the complexity of the pain pathophysiology seen in Pancoast tumors, and the positive results found in a small body of older and more recent research, surgical therapies such as cordotomy should be considered more often and earlier in the illness[oncnursingnews.com]
  • Pathophysiology Horner's syndrome results from a lesion to the sympathetic pathways that supply the head and neck, including the oculosympathetic fibers. Relevant anatomy The pupil is innervated by sympathetic and parasympathetic fibers.[eyewiki.aao.org]
  • […] population demographics females males (3:1) tend to be thin with long necks and drooping shoulders age 20-60 type neurogenic is most common (95%) vascular may be venous (4%) or arterial ( 1%) more common in athletic males compared to athletic females Pathophysiology[orthobullets.com]

Prevention

  • Early detection and prompt treatment of paraspinal metastasis are important to relieve symptoms and prevent permanent loss of neurological functions.[ncbi.nlm.nih.gov]
  • Swelling of the Supraclavicular Fossa and Posterior Upper Back Loss of Mobility of the Apex with Mueller's and Valsalva Maneuvers Loss of Mobility of Apex An infiltrative tumor at the apex will tether the soft tissue of the supraclavicular fossa and prevent[angelfire.com]
  • Preventing Pancoast Tumor The most important preventive step is to avoid using tobacco products. Quitting tobacco also reduces the risk of lung cancer .[webmd.com]
  • Primary prevention Prevention strategies depend on the etiology of Horner’s syndrome. These may include prevention of trauma (especially head trauma), control of cardiovascular risk factors and smoking cessation.[eyewiki.aao.org]
  • Preventing pancoast tumor involves smoking cessation and reduced exposure to the mentioned harmful substances.[cancerwall.com]

References

Article

  1. Sarkar A, Das A, Basuthakur S, Pandit S, Das SK, Choudhury S. Pancoast syndrome: A rare presentation of non-Hodgkin’s lymphoma. Lung India. 2013;30(3):209-211.
  2. 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.
  3. Dao I, El Mostarchid B, Onen J, Mandour C, El Asri CA, Boucetta M. Pancaost syndrome related to hydatid cyst. Pan Afr Med J. 2013;14:118.
  4. Panagopoulos N, Leivaditis V, Koletsis E, et al. Pancoast tumors: characteristics and preoperative assessment. J Thorac Dis. 2014;6(Suppl 1):S108-S115.
  5. Fontenele e Silva J, Barbosa Mde P, Viegas CL. Small cell carcinoma in Pancoast syndrome. J Bras Pneumol. 2009;35(2):190-193.
  6. Bansal M, Martin SR, Rudnicki SA, Hiatt KM, Mireles-Cabodevila E. A rapidly progressing Pancoast syndrome due to pulmonary mucormycosis: a case report. J Med Case Reports. 2011;5:388.

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Last updated: 2018-06-22 06:23