The MRI of the cervical and thoracic spine with and without contrast showed an epidural, possibly subdural, lesion from C7-T5 (Figure 1), with high intrinsic cord signal change at C2-C6 (Figure 2). These findings raised concern for a hematoma compressing the spinal nerve tracts. MRI also revealed a conglomerated lymph node in the left neck at level III/IV and a supraclavicular lymph node measuring 1 cm (Figure 3).
Neurosurgery was consulted, and the patient underwent immediate spinal decompression and laminectomy. The operation revealed an intradural extramedullary mass that raised suspicion of a malignant neoplasm. The results of histopathologic examination of the mass pointed to a diagnosis of anaplastic large cell lymphoma (ALCL).
ALCL is a form of peripheral T-cell lymphoma that typically has a bimodal distribution, in which the incidence peaks during adolescence and again at about 60 years of age. The classic presentation of ALCL is similar to that of other lymphomas: rapidly progressive adenopathy with the B symptoms of fevers, night sweats, and weight loss. Approximately two thirds of patients present at later stages, with extranodal involvement.
The main predictors of prognosis are the presence or absence of an anaplastic lymphoma kinase (ALK) gene mutation, the presence of which is associated with a better outcome, and the International Prognostic Index (IPI), which incorporates the patient's age, serum lactate dehydrogenase level, Eastern Cooperative Oncology Group (ECOG) performance status, Ann Arbor clinical stage, and the number of involved extranodal sites.
This patient ultimately received a diagnosis of ALK-positive ALCL. For advanced-stage pediatric non-Hodgkin lymphomas, chemotherapy is the recommended initial treatment. After this patient received chemotherapy, a follow-up MRI of the cervical and thoracic spine showed complete resolution of the extensive neoplastic dural involvement. The enlarged lymph nodes that were noted previously on imaging had also resolved. In addition, his upper-extremity strength had returned to baseline, with minimal residual tingling in his fingertips. Although the data are limited, this patient's ALK-positive status and his initial response to treatment suggest that his prognosis is good.
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