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Plexopathies 3601_e18_p413-422 2/19/02 8:58 AM Page 413 18 Plexopathies KURT A. JAECKLE Tumors may invade the cervical, brachial, and lum- dysfunction due to epidural compression by tumor, bosacral plexus by direct extension from primary neoplastic meningitis, and other disorders of pe- tumors in regional organs or by secondary invasion ripheral nerves. In clinical practice, physicians following metastases to regional lymph nodes. Oc- usually encounter patients with brachial or lum- casionally, tumor tracks along the epineurium sur- bosacral plexopathy. Cervical plexopathy is also rounding the trunks of the nerve plexuses. In the discussed as its clinical presentation, tumor his- previously treated cancer patient, the major differ- tologies, and therapeutic implications differ some- ential diagnostic consideration is radiation-induced what, although there is considerable overlap with plexopathy. Less frequently, the plexopathy results brachial plexopathy. Finally, one must be able to as a complication of regional (usually intra-arter- distinguish between those patients with plex- ial) chemotherapy or is due to fibrosis following opathies due to radiation or other causes, which is surgical resection of primary tumor within the re- discussed separately. gion. Tumor plexopathy is a symptomatic complication in approximately 1 of 100 patients with cancer. In CERVICAL PLEXOPATHY two retrospective reviews from cancer hospitals, based on 12,000 patient visits per year, the preva- Anatomy lence of brachial plexopathy resulting from tumor in- vasion was 0.43%, and the prevalence of lumbosacral The cervical plexus is formed from the ventral rami plexopathy was 0.71% (Kori et al., 1981; Jaeckle et of the four upper cervical nerve roots (C1–C4). Af- al., 1985). The actual prevalence may be higher, as ferent sensory fibers from the skin and soft tissues of not all patients with plexopathy accompanying termi- the neck travel via the greater and lesser occipital nal malignancy are reported. The best estimates have nerves (posterior occiput and postauricular area); been obtained from breast cancer patients treated the great auricular and transverse cervical nerves with conventional radiotherapy in whom the preva- (preauricular area, mandibular angle, anterosupe- lence of brachial plexopathy at 5 years was 1.8% to rior neck, and submandibular area); and the medial 4.9%; however, this study likely included some pa- and lateral supraclavicular nerves (inferior antero- tients with radiation-induced plexopathy (discussed lateral neck) through the cervical plexus. The cervi- below) (Pierce et al., 1992; Powell et al., 1990; Shel- cal plexus supplies motor innervation to the di- don et al., 1987). aphragm and, deep cervical and hyoid muscles and Familiarity with plexus neuroanatomy is neces- via the spinal accessory nerve to the sternocleido- sary to correctly interpret the clinical findings and mastoid and trapezius muscles (Truex and Carpen- to distinguish between plexopathies and neurologic ter, 1969). 413 3601_e18_p413-422 2/19/02 8:58 AM Page 414 414 SYMPTOMS SECONDARY TO CANCER AND ITS TREATMENT Pathophysiology terior cervical chain or supraclavicular nodes are present. Local sensory loss may be demonstrable, but The cervical plexus is usually invaded by tumor from is often incomplete due to overlap of root zones. Of- neighboring soft tissue or bony structures (Fig. ten, one must distinguish postsurgical sensory loss 18–1). The neoplasm may invade the plexus directly from that due to tumor recurrence. The timing and or indirectly by metastatic spread to regional lymph location of sensory loss are useful points of distinc- nodes, ribs, or vertebral bodies. The most commonly tion. Most commonly, surgical dissection transects associated tumors of the cervical plexus include squa- superficial branches of the greater auricular nerve mous cell carcinoma of the head and neck, lym- over the preauricular area and mandibular angle, or phoma, and adenocarcinomas of the lung and breast. the transverse cervical branches, producing numb- ness in the skin of the upper anterior neck and sub- mandibular area. Clinical Presentation Tumor may involve the phrenic nerve producing a Patients with cervical plexopathy usually present with paralyzed hemidiaphragm, which can be confirmed pain located in the neck, shoulder, or throat, which by chest radiograph or fluoroscopy. Patients may have is often exacerbated by neck movement, swallowing, dyspnea and paradoxical diaphragmatic excursions. and coughing. The pain is often deep, boring, and Motor dysfunction of branches to the deep cervical constant, but may have intermittent sharp compo- and hyoid muscles are usually asymptomatic. In- nents or caulsalgic features. On examination, the cer- volvement of the spinal accessory XIth cranial nerve vical musculature may be tender to palpation. One may produce shoulder weakness and instability. Ster- should suspect tumor plexopathy from tumor inva- nocleidomastoid weakness may produce head devia- sion if large palpable tumors or firm anterior or pos- tion or tilt. Head and neck tumors and lymphomas Figure 18–1. Cervical plexopathy. Magnetic resonance imaging scan of metastatic adenocarcinoma from submandibular primary tumor to lower left cervical plexus (arrows), producing C4–C5 pain, tingling, hoarseness, and Horner’s syndrome. 3601_e18_p413-422 2/19/02 8:58 AM Page 415 Plexopathies 415 involving the anterior cervical lymphatic chain may Pathophysiology grow inferiorly, producing additional involvement of More than two-thirds of tumors involving the bra- the upper brachial plexus. chial plexus originate from the lung or breast and By definition, involvement of the cervical plexus generally spread to the plexus directly from the lung implies a close proximity of the tumor to the cervi- or from regional metastases to the axillary lymph cal spine. If sharp and severe pain occurs with neck nodes or lung apex (Kori et al., 1981) (Fig. 18–2). movements, or pain is elicited with percussion over Early in the disease process, the tumor often invades the spine, there may be associated involvement of the the lower plexus, particularly the inferior trunk and cervical spine or impending epidural extension. medial cord, a pattern that was part of the original description of the superior sulcus syndrome (Pan- coast, 1932). Conversely, tumors of the head and BRACHIAL PLEXOPATHY neck usually invade the superior trunk or posterior cord from above. Often, the clinical pattern of Anatomy plexus involvement is patchy, due to irregular in- volvement of the plexus by growth from the rela- The brachial plexus originates from ventral rami of tively random arrangement of lymph node metas- the lower four cervical (C5–C8) and the first tho- tases. Patchy involvement is most commonly racic roots and forms three trunks: a superior trunk identified when formal electrophysiologic investiga- (C5–C6); a medial trunk (C7); and an inferior trunk tions are performed. (C8–T1). These trunks each divide into dorsal and ventral divisions. The three dorsal divisions join together to form the posterior cord, which then Clinical Presentation splits into (1) the thoracodorsal nerve, to the latis- simus dorsi; (2) the subscapular nerve, which in- In a large series of patients with cancer and brachial nervates the subscapularis; (3) the axillary nerve, plexopathy, pain was the most common presenting which supplies the deltoid; and (4) the radial nerve, symptom (75%), followed by dysesthesias (25%) which innervates the triceps, brachioradialis, wrist, (Kori et al., 1981). The pain was typically located in and finger extensors and provides sensory supply to the shoulder and axilla and often radiated along the the posterior arm and forearm. The ventral division medial aspect of the arm and forearm into the fourth forms two cords: the lateral cord, which receives and fifth fingers. Initially, pain was often incorrectly contributions from the superior and medial trunks, attributed to other potential causes, such as arthritis and the medial cord, which is a direct continuation and fibromyalgia. Pain, reflex changes, and atrophy of the inferior trunk. The lateral cord then divides were concentrated in the lower plexus (C8–T1) dis- into two branches: one becomes the musculocuta- tribution in 75% of patients, whereas the remaining neous nerve (C5–C7, motor to biceps, brachialis, 25% of patients had global signs of involvement of the coracobrachialis and sensory innervation of the ra- entire (C5–T1) plexus. dial forearm); and the other joins a similar branch When brachial plexopathy is due to tumor, lymph- from the medial cord to form the median nerve edema is relatively infrequent (15%). It is most often (C8–T1, forearm flexors and pronators, medial seen in the setting of prior radiotherapy or axillary hand flexor, and lumbrical muscles; and sensation lymph node dissection. Involvement of the sympa- to medial palm, first three fingers, and radial one- thetic trunk or ganglia near the upper thoracic (par- half of the fourth finger). The remaining portion of ticularly T1) vertebrae may produce unilateral the medial cord forms (1) the medial brachial cu- Horner’s syndrome, which has been identified in ap- taneous nerve (T1, sensory to medial arm); (2) the proximately 23% of patients. When Horner’s syn- medial antebrachial cutaneous nerve (C8–T1, drome is present, concomitant epidural disease can sensory to medial forearm) and; (3) the ulnar be identified in 32% of patients. In general, the pres- nerve (C8–T1, ulnar forearm flexor muscles,
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