Indian Journal of Pathology and Microbiology
Home About us Instructions Submission Subscribe Advertise Contact e-Alerts Login 
Users Online: 58
Print this page  Email this page Bookmark this page Small font sizeDefault font sizeIncrease font size


 
  Table of Contents    
LETTER TO EDITOR  
Year : 2011  |  Volume : 54  |  Issue : 1  |  Page : 223-225
Splenic hamartoma: A rare vascular space occupying lesion of the Spleen


1 Department of Surgical Gastroenterology, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai, Tamilnadu, India
2 Department of Pathology, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra University, Chennai, Tamilnadu, India

Click here for correspondence address and email

Date of Web Publication7-Mar-2011
 

How to cite this article:
Sankar S, Thanka J, Jagdishchandrabose S, Rajendran S. Splenic hamartoma: A rare vascular space occupying lesion of the Spleen. Indian J Pathol Microbiol 2011;54:223-5

How to cite this URL:
Sankar S, Thanka J, Jagdishchandrabose S, Rajendran S. Splenic hamartoma: A rare vascular space occupying lesion of the Spleen. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Jul 25];54:223-5. Available from: http://www.ijpmonline.org/text.asp?2011/54/1/223/77416


Sir,

Vascular space occupying lesions of the spleen are rare clinical entities and clubbed under the category of nonlymphoid tumors of the spleen. Herein we report a case of splenic hamartoma with emphasis on the radiological and histological features. Hamartomas of the spleen are usually diagnosed incidentally on imaging. Although various radiological findings are described, the final diagnosis is based on histology and immune-histochemistry. Herein we report a massive and symptomatic splenic hamartoma in a young lady and reviewed the relevant literature

A 19-year-old female patient presented with complaints of left upper quadrant pain of 6 months duration. The pain was constant, dull aching in nature without any radiation. There were no other significant symptoms. There were no constitutional symptoms. There were no significant past history of any systemic illness. The only positive physical finding was a tender splenomegaly. Investigations like hemogram, liver function tests, renal profile, and coagulation profile were essentially normal. Ultrasound showed a well-defined hyperechoic mass arising from the spleen. Contrast-enhanced computerized tomogram (CT) scan [Figure 1] showed a mass measuring 10 cm in diameter arising from the spleen that was very vascular as evidenced by the heterogenous enhancement pattern. Patient underwent splenectomy and the cut section of the specimen is shown in [Figure 2]. Splenectomy specimen showed a well-circumscribed spongy tumor measuring 10 cm in diameter. Cut section shows varying sizes of vascular spaces with intervening fat in the background of splenic parenchyma. Histological picture was consisting of blood vessels of varying sizes intermingled with splenic red pulp element showing disorganization and entrapped clusters of adipocytes [Figure 3] and [Figure 4]. The vascular channels were positive for CD 68, CD 34, SMA, and reticulin stain [Figure 5], which were consistent with the diagnosis of hamartoma.
Figure 1: A mass measuring 10 cm in diameter arising from the spleen that was very vascular as evidenced by the heterogenous enhancement pattern

Click here to view
Figure 2: Cut section of the splenic tumor shows varying sizes of vascular spaces with intervening fat in the background of splenic parenchyma

Click here to view
Figure 3: Low-power view showing blood vessels of varying sizes intermingled with splenic red pulp element showing disorganization and entrapped clusters of adipocytes (Hematoxylin and eosin, ×100)

Click here to view
Figure 4: Blood vessels of varying sizes intermingled with splenic red pulp element showing disorganization and entrapped clusters of adipocytes (Hematoxylin and eosin, ×400)

Click here to view
Figure 5: (a) Positive for reticulin (IHC, ×100) (b) Positive for SMA (IHC, ×100) (c) Positive for CD 68 (IHC, ×100) (d) Positive for CD 34 (IHC, ×100)

Click here to view


Vascular neoplasms are nonhematolymphoid primary neoplasm of the spleen, arising from the vascular components of the red pulp of the spleen. Hemangioma is the most common of these lesions and the rare tumors include hamartoma, lymphangioma, and Littoral cell angioma. Angiosarcoma and hemangiopericytoma are the malignant vascular neoplasms of the spleen.

Splenic hamartomas are rare benign vascular tumors that were first described by Rokitansky in 1861. The other nomenclatures used to describe this entity include splenoma, splenoadenoma, and nodular hyperplasia of the spleen. The incidence of splenic hamartomas in autopsy series ranges between 0.024% to 0.13 %. [1] These lesions are usually asymptomatic and detected as incidental findings on imaging. Large lesions present as splenomegaly, pain, rupture, and rarely with thrombocytopenia and anemia. Larger and symptomatic lesions are common in females. [2] Splenic hamartomas may be associated with other conditions like tuberous sclerosis, Wiskott-Aldrich like syndrome, and other neoplastic conditions. [3],[4]

Splenic hamartomas are usually congenital in origin, but some believe in neoplastic and post-traumatic mechanisms in the etiopathogenesis. It consists of anomalous mixture of normal splenic red pulp elements. Macroscopically splenic hamartomas are well circumscribed, solid lesions that are dark red in color and compress the adjacent splenic parenchyma. Histologically the hamartomas contain a mixture of disorganized vascular channels lined by endothelial cells and surrounded by fibrotic cords of red pulp with or without white pulp. Histologically it may be difficult to differentiate between hamartoma and hemangioma, and immunohistochemical characterization is needed to confirm the diagnosis.

On ultrasound, hamartomas are well-defined homogenous hyperechoic lesions that shows increased blood flow demonstrated with Doppler. Secondary changes like hemorrhage, degeneration, and calcification makes these lesions heterogenous on ultrasound.[5] On CT, hamartomas appear isodense in plain scans and may show heterogenous enhancement after contrast administration and characteristically produce a contour abnormality. [5] Hamartomas are isointense on T1-weighted MR images, heterogeneously hyperintense on T2-weighted MR images, and heterogenous enhancement with gadolinium. [6] On angiogram, hamartoma appears as hypervascular mass consisting of tumorous vessels with aneurysmal dilatation, arteriovenous shunts, and vascular lakes. Although imaging findings may be suggestive of hamartoma, the final diagnosis is achieved by histology.

 
   References Top

1.Lam KY, Yip KH, Peh WC. Splenic vascular lesions: unusual features and a review of the literature. Aust N Z J Surg 1999;69:422-5.  Back to cited text no. 1
[PUBMED]    
2.Iozzo RV, Haas JE, Chard RL. Symptomatic splenic hamartoma: A report of two cases and review of the literature. Pediatrics 1980;66:261-5.  Back to cited text no. 2
[PUBMED]    
3.Darden JW, Teeslink R, Parrish A. Hamartoma of the spleen: A manifestation of tuberous sclerosis. Am Surg 1975;41:564-6.  Back to cited text no. 3
[PUBMED]    
4.Huff DS, Lischner HW, Go HC. Unusual tumors in two boys with Wiskott-Aldrich like syndrome. Lab Invest 1979;40:305-6.  Back to cited text no. 4
    
5.Zissin R, Lishner M, Rathaus V. Case report: Unusual presentation of splenic hamartoma; computed tomography and ultrasonic findings. Clin Radiol 1992;45:410-1.  Back to cited text no. 5
[PUBMED]    
6.Ramani M, Reinhold C, Semelka R. Splenic hemangiomas and hamartomas: MR imaging characteristics of 28 lesions. Radiology 1997;202:166-72.  Back to cited text no. 6
    

Top
Correspondence Address:
Subramanian Sankar
Department of Surgical Gastroenterology, Sri Ramachandra Medical College and Research Institute, Porur, Chennai - 600 116, Tamilnadu
India
Login to access the Email id


DOI: 10.4103/0377-4929.77416

PMID: 21393931

Get Permissions



    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Email Alert *
    Add to My List *
* Registration required (free)  


    References
    Article Figures

 Article Access Statistics
    Viewed3191    
    Printed60    
    Emailed0    
    PDF Downloaded59    
    Comments [Add]    

Recommend this journal