Superior vena cava syndrome secondary to an angiotropic large cell lymphoma.

ABSTRACT

BACKGROUND: Angiotropic large cell lymphoma (ALCL) is characterized by the intravascular proliferation of malignant lymphoid cells in small and medium-sized blood vessels. In the current study, the authors report an unusual case in which the initial presentation of the ALCL was that of superior vena cava (SVC) syndrome. METHODS: The case is presented, followed by a general review of the literature regarding ALCL. RESULTS: Surgical intervention was required for diagnosis in this case. Successful treatment with chemotherapy followed by involved field radiation ensued with a maintained disease remission at 48 months of follow-up. CONCLUSIONS: Although usually presenting in small blood vessels, ALCL can present initially with large blood vessel involvement and should be considered in the differential diagnosis of this condition, even in the absence of extravascular lymph node involvement. Aggressive treatment with antineoplastic therapy is warranted and may result in long term recurrence free survival.