Superior vena cava syndrome associated with lymphoma
A. Yellin, M. Mandel, G. Rechavi, Y. Neuman, B. Ramot and Y. Lieberman
Department of Thoracic Surgery, Chaim Sheba Medical Center, Tel Hashomer, Israel.
OBJECTIVE--To evaluate the workup and treatment of children with lymphoma
and superior vena cava syndrome. DESIGN--A retrospective survey.
SETTING--State hospital serving as a secondary and tertiary referral center
for pediatric oncology and pediatric cardiac surgery. PARTICIPANTS--Eleven
children aged 11 months to 12 years diagnosed as having lymphoma or T-cell
acute lymphoblastic leukemia who presented with superior vena cava syndrome
during an 11-year period. INTERVENTIONS--Lymph node biopsy (two patients),
thoracenthesis (five patients), bone marrow aspiration (two patients), and
thoracenthesis in addition to bone marrow aspiration (two patients). All
aspirates were evaluated with immunohistochemical studies. Chemotherapy was
the only management intervention. RESULTS--T-cell lymphoma or leukemia
accounted for nine cases and Hodgkin's disease for two cases. Respiratory
symptoms occurred in 10 patients, including tracheal compression in six
patients (compression was life-threatening in one patient). Diagnosis of
superior vena cava syndrome was achieved in eight patients using
surface-marker analysis of aspirates. The syndrome disappeared within 2 to
10 days. Seven of nine children whose conditions were diagnosed more than 1
year before this writing were alive and free of disease after mean
follow-up of 37 months. CONCLUSIONS--(1) A specific diagnosis can be
achieved in most children with superior vena cava syndrome and lymphoma;
(2) Thoracic computed tomographic scans are essential, identifying minute
pleural effusions that can aid diagnosis; (3) Anesthetic hazard is related
only to severe tracheal compression; (4) Chemotherapy achieves excellent
symptomatic relief; and (5) Long-term survival, without disease, is
achievable.
