Table of Contents
Definition / general | Epidemiology | Clinical features | Diagnosis | Radiology images | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Positive stains | Negative stains | Molecular / cytogenetics descriptionCite this page: Shankar V. Hemangiopericytoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenhpc.html. Accessed February 5th, 2023.
Definition / general
- Very rare vascular neoplasm of uncertain biological behavior
Epidemiology
- Primarily affects middle aged adults
- Males and females are equally affected
Clinical features
- Usually asymptomatic, can present with splenomegaly
- Can present with hemoperitoneum due to spontaneous or traumatic rupture
Diagnosis
- CT or MRI, then histopathological confirmation
Case reports
- One month old boy (J Pediatr Surg 2013;48:e21)
- 31 year old man with splenic hemangiopericytoma and serosal cavernous hemangiomatosis of adjacent colon (World J Gastroenterol 2005;11:4111)
- 35 year old man with multiple abscesses (World J Surg Oncol 2005;3:77)
- 70 year old man with spontaneous rupture of splenic hemangiopericytoma (Int J Emerg Med 2011;4:13)
Treatment
- Partial or total splenectomy
Gross description
- Single or multiple, encapsulated, soft to rubbery
- Average size is 6.5 cm
Microscopic (histologic) description
- Well developed, branching, thick walled blood vessels surrounded by connective tissue sheath
- Monotonous cells with moderate to high cellularity
- Hemorrhagic and cystic degeneration can be seen
- Rarely manifest as multiple abscesses
Negative stains
Molecular / cytogenetics description
- Usually near diploid and breakpoints in 12q13, 12q24 and 19q13 are common, with t(12;19)(q13;q13) being a recurrent translocation (Cancer Genet Cytogenet 2002;136:62)