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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
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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
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 Internet Journal of Pathology vol 8 no. 1
Hemangioma Hemangioma • 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 Splenichemangiomas • The most common primary splenic neoplasm • Incidence between 0.03%-14% at autoposy • Malginant transformation is extremely rare
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?
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 • 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