Spleen
Vascular tumors
Hamartoma


Topic Completed: 1 October 2013

Revised: 18 March 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Hamartoma[TI] spleen

Vijay Shankar, M.D.
Page views in 2018: 2,138
Page views in 2019 to date: 1,300
Cite this page: Shankar V. Hamartoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenhamartoma.html. Accessed July 22nd, 2019.
Definition / general
  • Rare benign tumor, usually congenital, composed of aberrant mixture of normal splenic tissue
  • Nodular lesion of variable size, derived from splenic sinus lining cells
Terminology
  • Also called splenoma, splenoadenoma, nodular hyperplasia of the spleen
Epidemiology
  • All age groups, equal sex preponderance
Clinical features
  • Usually an incidental finding
  • Can present with pain, palpable mass or spontaneous rupture
  • May be associated with tuberous sclerosis, Wiskott-Aldrich-like syndrome, hypersplenism (thrombocytopenia, anemia, pancytopenia)
  • Rarely associated with malignant hematologic conditions
Radiology images

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Hypodense mass of the spleen

Homogenous and hypoechoic mass

Isointense mass (arrows)

Case reports
Treatment
  • Splenectomy (complete or partial; laparoscopic or hand assisted laparoscopic)
Gross description
  • Circumscribed nodular lesion, dark red, compressing splenic parenchyma, ranging from a few mm to several cm
  • May be single or multiple
Gross images

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Tumor-like mass

Nodule of increased stiffness

Resected spleen

Varying sizes of vascular spaces

Microscopic (histologic) description
  • Either follicular or pulpous and fibrous
  • Well circumscribed lesion with disorganized blood vessels of varying sizes intermingled with splenic red pulp element
  • Also entrapped adipocytes, focal extramedullary hematopoiesis
Microscopic (histologic) images

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Red pulp splenic sinusoidal structure

Splenic red pulp element

Entrapped clusters of adipocytes

Nonencapsulated nodule

Positive for reticulin

Positive stains
Electron microscopy images

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Tall lining cells

Molecular / cytogenetics description
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