Hem/Onc Tumor Board Presentation Advanced Topics in Cancer Biology Katy Van Hook June 3, 2009

MA 32 y/o female 12/08 she is seen for epigastric pain, night sweats, and nausea 28 weeks pregnant Symptoms persist and she receives an abdominal ultra sound Enlarged spleen (14 x 12.7 x 5.7 cm) Innumerable variable-sized relatively well circumscribed hyperechoic lesions

MA 32 y/o female CT Multiple lesions in the spleen Flow cytometry of peripheral blood lymphocytes was normal suggesting that it is not lymphoma Just watch

MA 32 y/o female Delivers baby (#9!) on 3/5/09 3/28/09 her condition improves but is still having night sweats History of malaria, + PPD, parasites, etc… Normal labs except for mild thrombocytopenia (low platelet count) She is immunized for an encapsulated organism Infection? Marginal zone lymphoma? Metastatic disease? Surgery vs. Biopsy Encapsulated organism is a general term for a group of polysaccharide coated bacteria including: pneumococcus, meningococcus (meningitis) and salmonella typhi (thyphoid fever) Thrombocytopenia can be caused by a lot of different things: vitamin imbalance, leukemia, liver failure, sepsis, dengue fever, and many more. Echinococcus granulosus Marginal zone lymphoma is typically associated with heliobacter pylori Surgery—larger procedure that will leave her with the risk of sepsis and other types of infection Biopsy—run the risk of not getting a good sample (decide on CT guided) but she would still have her spleen

Pathology No evidence of lymphoma CD3 (T cell) CD20 (B cell) are normal Weakened D2-40 Dilated vascular spaces lined by flattened endothelial cells Lots of red blood cells Vascular lesion Hemangioma or lymphangioma Looks benign Splenic lesions 57% lymphoma 7% benign of malignant vascular neoplasm D2-40: monoclonal antibody for lymphatic endothelial cells. Antibody against podoplanin, a mucin-type transmembrane protein Internet Journal of Pathology vol 8 no. 1

Hemangioma Hemangioma Splenic hemangiomas Benign tumor of endothelial cells that line the blood vessels Most common childhood tumor affecting 10% of Caucasians Most common sites are face and neck Splenic hemangiomas The most common primary splenic neoplasm Incidence between 0.03%-14% at autoposy Malginant transformation is extremely rare <100 patients reported in medical literature

Lymphangioma Childhood Lymphangioma Benign hyperplasia of lymphatic vessels 6% of benign tumors in children Most common sites are face and neck Thought to be a developmental malformation Adult Lymphangioma VERY rare in adults Lymphatic vessel with proliferative behavior Initial lymphatics that never fully form Initiated by injury or infection? Dysregulated GF? Splenic lymphangiomas/hemangiomas are often discovered incidentally

Mechanisms are relatively unknown Lymphangioma Elevated expression of VEGR2/3, and VEGF-C Hemangioma Elevated expression of VEGF and fibroblast growth factor (FGF) BMC Cancer (2007) 7:105

Treatment options No treatment-monitor patient Splenectomy Risk of rupture Compression of other organs Splenectomy Embolization Polyvinyl alcohol calibrated spheres Vascular growth inhibitors VEGF inhibition has been suggested studies are ongoing

References Fagen K, et al. (2008) JVIR 20:559-560 Wiegand S., et al. (2008) Virchows Arch 453:1 Norgall S., et al. (2007) BMC Cancer 7:105 Willcox TM. et al. (2000) J Gastrointest Surg 4:611-613 Disler DG and Chew FS. (1991) AJR 157:44 Internet Journal of Pathology. Vol. 8 no. 1