Lymph nodes & spleen, nonlymphoma
Spleen-vascular tumors
Hamartoma
Author: Vijay Shankar, M.D.
Last author update: 1 October 2013
Last staff update: 16 March 2023 (update in progress)
Copyright: 2003-2023, PathologyOutlines.com, Inc.
PubMed Search: Hamartoma[TI] spleen
Table of Contents
Definition / general | Terminology | Epidemiology | Clinical features | Radiology images | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Positive stains | Molecular / cytogenetics description | Differential diagnosisCite this page: Shankar V. Hamartoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenhamartoma.html. Accessed May 29th, 2023.
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
Images hosted on other servers:
Hypodense mass of the spleen
Homogenous and hypoechoic mass
Isointense mass (arrows)
Case reports
- 19 year old woman with left upper quadrant pain (Indian J Pathol Microbiol 2011;54:223)
- 28 year old man (Case Rep Gastrointest Med 2012;2012:435802)
- 29 year old man (Chin Med J (Engl) 2006;119:1403)
- 29 year old woman with right sided abdominal pain due to rupture (Arch Pathol Lab Med 2009;133:147)
- 37 year old man (World J Gastrointest Surg 2010;2:147)
- 53 year old woman with sudden left chest and shoulder pain (World J Gastroenterol 2006;12:2133)
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
Images hosted on other servers:
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
Positive stains
- CD8, CD68, SMA, reticulin
- Endothelial cells stain for nonspecific esterases with both α naphthyl acetate and naphthol acid phosphate as substrate; they do not contain alkaline phosphatase
- Variable CD34
Molecular / cytogenetics description
- Clonal chromosomal abnormality can be present (Chin Med J (Engl) 2006;119:1403)
