Table of Contents
Definition / general | Epidemiology | Clinical features | Diagnosis | Radiology description | Radiology images | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Positive stains | Differential diagnosisCite this page: Shankar V. Hemangioendothelioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenhemangioendothelioma.html. Accessed May 29th, 2023.
Definition / general
- Very rare, low grade malignant primary vascular tumor of spleen, with intermediate biologic behavior between hemangioma and angiosarcoma
Epidemiology
- Often young, no gender predilection
Clinical features
- Vague pain, palpable mass and fatigue
- Functional hyposplenism, spontaneous or traumatic rupture can occur
- Rarely mild anemia, indirect hyperbilirubinemia
Diagnosis
- Based on radiological examination and histopathological confirmation
Radiology description
- Ultrasound: hypoechoic mass distinct from surrounding spleen
- CT: low attenuation mass
- MRI: heterogenous solid lesion
Case reports
- 3 year old boy with epithelioid and spindle cell hemangioendothelioma (Am J Surg Pathol 1992;16:785)
- 9 year old girl with functional hyposplenism due to a primary epithelioid hemangioendothelioma (Arch Pathol Lab Med 1995;119:755)
- 9 year old boy with hemangioendothelioma of liver and spleen and trauma induced consumptive coagulopathy (J Pediatr Surg 2002;37:E29)
- 38 year old woman with kaposiform hemangioendothelioma (Pathol Oncol Res 2011;17:969)
- Patient with chronic anemia (Arch Pathol Lab Med 1992;116:1079)
- Malignant hemangioendothelioma (Arch Pathol Lab Med 1981;105:300)
Treatment
- Splenectomy
Gross description
- Well separated lesions, solitary or multiple of variable size
Microscopic (histologic) description
- More cellular than hemangioma, less atypical than angiosarcoma
- Epithelioid or spindled
- Ill defined vascular spaces lined by cells with mild or moderate atypia
- Low mitotic index
Differential diagnosis