Case Presentation
A Mediastinal Mass Presenting With Unilateral Periscapular and Arm Pain Zack McCormick, MD, Karina J. Bouffard, MD, MPH, Daniel Neudorf, MS, Ellen Casey, MD We describe a case of a patient with mediastinal lymphoma who presented with arm and scapular pain, which is an atypical referral pattern for pain originating from the mediastinum. We use this case as a platform to discuss mediastinal pain referral patterns and the importance of maintaining a broad differential diagnosis of arm and scapular pain, especially when symptoms atypical of pure neuromusculoskeletal structural disease are present. PM R 2013;-:1-4
INTRODUCTION Mediastinal disease typically refers to pain in the anterior chest or mid back [1,2]. Less commonly, it can refer pain to the upper extremity or shoulder girdle [3-8]. We describe a case of a patient with mediastinal lymphoma who presented for care at a musculoskeletal clinic because of arm and scapular pain. We discuss mediastinal pain referral patterns and the importance of maintaining a differential diagnosis of arm and scapular pain that extends beyond structural abnormalities of the spine.
CASE PRESENTATION A 29-year-old, right-handed woman presented with 2 days of pain in the right side of the neck with radiation to the right arm and all 5 digits. She reported that 6 weeks earlier she began experiencing right neck, pectoralis, and axillary pain the day after performing a typical aerobic and resistance training workout but that this pain spontaneously resolved over the course of days. With regard to her current symptoms, she described pain with an intensity of 7 out of 10 on a numeric rating scale. This pain was burning, sharp, and stabbing in character and worse when she used the computer or lifted objects at her side with her right arm. Her pain was somewhat relieved with the use of acetaminophen and ice. Her vital signs were within normal limits. She had tenderness to palpation of the C5-T1 spinous processes. Cervical range of motion was full and nonpainful. She had full strength, sensation, and muscle stretch reflexes in the bilateral upper extremities. A Spurling test did not reproduce her typical symptoms. She was treated with a course of diclofenac and extension-based mechanical diagnosis and treatment (McKenzie) therapy, and her pain resolved after 1-2 weeks. Six months later, the patient presented with acute right infrascapular pain and paresthesias radiating along the right medial arm to the elbow 3 days after lifting a heavy weight. She described pain as 6 out of 10 on a numeric rating scale; the pain was burning and sharp in character and worsened when sitting or lying flat or during deep inhalation. During the previous 3 months, the patient noted multiple upper respiratory infections, fatigue, fevers, chills, night sweats, and a 10-lb weight loss. Her primary care physician had performed laboratory testing, with negative findings for any infection or identifiable illness. Her vital signs were within normal limits. She had tenderness to palpation of the C6-T1 spinous processes and right upper trapezius musculature. Cervical range of motion was full and nonpainful. She had full strength, sensation, and muscle stretch reflexes in the bilateral upper extremities. The Spurling test (on the right side) reproduced her typical symptoms, PM&R 1934-1482/13/$36.00 Printed in U.S.A.
Z.M. Department of Physical Medicine and Rehabilitation, Rehabilitation Institute of Chicago, 345 East Superior St, Chicago, IL 60611. Address correspondence to: Z.M.; e-mail:
[email protected] Disclosure: nothing to disclose K.J.B. Physical Medicine and Rehabilitation/ Pain Medicine Rehabilitation Institute of Chicago, Chicago, IL (ad interim) Disclosure: nothing to disclose D.N. Chicago College of Osteopathic Medicine, Midwestern University, Chicago, IL Disclosure: nothing to disclose E.C. Department of Physical Medicine and Rehabilitation, Rehabilitation Institute of Chicago, Chicago, IL Disclosure: nothing to disclose Submitted for publication May 5, 2013; accepted November 14, 2013.
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and cervical traction improved them. Magnetic resonance imaging (MRI) of the cervical spine was obtained and showed no disk pathology, stenosis, or spinal lesions. However, the scout image partially captured a superior mediastinal mass (Figure 1). Chest computed tomography (CT) and body positron emission tomography/CT scans better elucidated this mass (Figure 2) and showed a conglomeration of right peritracheal lymph nodes measuring 4.1 3.6 cm, a group in the anterior mediastinum measuring 4.3 2.9 cm, a right hilar lymph node measuring 2.2 1.7 cm, and a cardiophrenic lymph node measuring 2.8 1.4 cm. A lymph node biopsy revealed lymphoma with intermediate features between Hodgkin and diffuse large B-cell lymphoma. The patient was treated with 6 cycles of R-EPOCH chemotherapy (ie, rituximab, etoposide, prednisone, vincristine, and hydroxydaunorubicin). Her pain symptoms progressively decreased as the size of these lymph nodes decreased (as visualized on serial CT scans). She currently remains in complete remission and without pain.
DISCUSSION Lymphoma can cause pain as the result of the tumor mass itself [9,10] or involvement of skeletal muscle, the spinal cord, spinal nerve roots, peripheral nerves, or vasculature [11,12]. In an international survey study of 1095 patients
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reporting pain, the origin of their symptoms was related to the tumor itself or tumor-associated involvement of other tissues 92.5% of the time. That study demonstrated that cancer pain was related to compromise of bone or joint (41.7%), viscera (28.1%), soft tissue (28.3%), or peripheral nerves (27.8%) most commonly, causing pain in 2 or more locations 25% of the time [9]. Our patient presented with a mediastinal mass, which, when infiltrating adjacent structures, has been reported to cause referred pain to the neck, arm, scapula, and upper back. A mediastinal mass can cause medial arm [13,14] or upper back [15] neuropathic-type pain resulting from concurrent compression of the T1 or T2 intercostal nerves. Pain at the base of the neck has been reported in association with mediastinal tumor infiltration of the T3 intercostal nerve [16]. Pain in the left shoulder and neck has been described in association with mediastinal mass involvement of the pericardium [17]. Scapular pain has been reported as the result of posterior mediastinal masses that infiltrated thoracic vertebrae [18]. However, in this case, imaging did not demonstrate any infiltration of a thoracic nerve, the pericardium, or a vertebrae, and thus vicerosomatic convergence better explains the cause of pain in our patient. This theory states that noxious afferent signals from viscera converge with somatic afferents at the level of the spinal cord, in lamina I and V of the dorsal
Figure 1. Sagittal views of the cervical spine on T2-weighted magnetic resonance imaging (MRI) demonstrate a lobulated superior mediastinal mass measuring 7 8 cm on scout imaging (arrow, top row, left), mild disk desiccation without loss of disk height at multiple levels, a hemangioma in the C5 vertebral body, and the absence of spinal canal stenosis (top row, right). Axial views on T2weighted MRI of the C5-C6 (lower row, left), C6-C7 (lower row, middle), and C7-T1 (lower row, right) levels reveal no evidence of disk herniation, central stenosis, or foraminal stenosis. At the C5-C6 level, a mild disk bulge and a Tarlov cyst are apparent.
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Figure 2. A coronal section on a computed tomography (CT) scan of the chest with contrast demonstrates a lobulated superior mediastinal mass measuring 7 8 cm (arrow, left). A sagittal section on a CT scan of the chest, abdomen, and pelvis further delineates this mass (arrow, right).
horn [19]. Signals are then transmitted out to somatic structures antidromically (against the normal direction of neural impulses) via somatic afferents, such that visceral pain may be interpreted by the brain as dermatomal and sclerotomal pain [20]. For example, in a feline model, simulation of cardiopulmonary sympathetic fibers resulted in antidromic impulses in the T2-T4 spinal segments with receptive fields in the forelimb and upper thorax [21]. A study in felines in which a caustic agent was applied to the esophagus provoked action potentials in T2-T7 spinal neurons [22], which have somatic fields in the chest, axilla, and upper back [23]. The viscerosomatic convergence theory likely explains somatic pain referral patterns that have been reported in patients with mediastinal masses that do not involve adjacent structures. Anterior chest pain with unilateral paresthesias of the arm [24], unilateral shoulder [4,5], and neck [4,25]; unilateral facial pain [26]; unilateral paresthesias of the chin [27], teeth [27,28], upper back [7,8], and lower back [29]; and abdominal pain [30] have been described. In this case, mediastinal visceral pain related to enlarged pathologic lymph nodes was most likely the cause of the patient’s symptoms. Her pain was exacerbated by deep inspiration, potentially indicating that compression of the mediastinum by the lungs provoked pain. She described her pain as “burning and sharp” and thus both neuropathic and somatic in character, which is common with cancer pain. Caraceni and Portenoy [9] found that patients with cancer pain describe a combination of neuropathic and somatictype pain 39% of the time. Our patient also experienced a stepwise reduction of pain with successive tumor size reduction during chemotherapy. Other less likely possibilities were considered. It is possible that the patient had coincident, unrelated lymphoma and cervical radiculitis or diskogenic pain. The
cervical MRI demonstrated a C5-C6 disk bulge, as well as a Tarlov cyst at the right C7 nerve root. Thus C7 nerve root irritation could have been possible. However, the disk bulge was mild with no apparent nerve root impingement, and symptoms related to a Tarlov cyst in the cervical region are extremely rare, with fewer than 10 cases reported in the literature [31]. These factors suggest an alternative diagnosis to cervical radiculitis. Alternatively, the patient’s pain referral pattern could be explained by a diskogenic pain, because the C3-C4, C4-C5, C5-C6, and C6-C7 all include the scapula and posterior arm in their referral patterns based on provocation diskography studies reported by Slipman et al [32]. However, although findings of the patient’s cervical MRI examination showed multilevel disk desiccation, it was mild, she had no loss of disk height, and no high-intensity zones (that may be associated with painful annular fissures) were evident. Her progressive reduction in symptoms could have been related to the natural history of improvement of radicular or diskogenic symptoms, independent of the reduction of tumor bulk with chemotherapy, which appeared to correlate with diminished pain.
CONCLUSION This case highlights the fact that clinicians should maintain a broad differential diagnosis when considering the cause of arm and scapular pain, especially when constitutional symptoms are present. In such instances, subtle elements of the history, such as pain with deep inspiration, may provide clues to a noncervical origin of symptoms. Early recognition of pain referred from the mediastinum may facilitate treatment at earlier stages in malignant disorders or before clinical deterioration in threatening nonmalignant conditions. Our case suggests that mediastinal pathologic referral
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patterns should be better elucidated through larger observational cohort studies.
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