Spleen
Vascular tumors
Littoral cell angioma

Editorial Board Member: Genevieve M. Crane, M.D., Ph.D.
Editor-in-Chief: Debra Zynger, M.D.
Patricia Tsang, M.D., M.B.A.

Topic Completed: 17 January 2019

Revised: 22 August 2019

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

PubMed Search: Littoral cell angioma[TI] spleen

Patricia Tsang, M.D., M.B.A.
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Cite this page: Tsang P. Littoral cell angioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenlittoralcellangioma.html. Accessed August 26th, 2019.
Definition / general
  • Rare benign splenic vascular tumor
  • Originates from littoral cells lining splenic red pulp sinuses
Essential features
  • Strong association with diverse immunologic and neoplastic disorders
  • Dual endothelial and histiocytic differentiation of tumor cells, similar to normal splenic littoral cells
Terminology
  • Littoral cell angioma of the spleen
ICD coding
Epidemiology
Sites
  • Unique to the spleen
Pathophysiology
  • Immunodysregulation postulated to explain clinical association with various malignancies or immune / autoimmune disorders (Histopathology 2016;69:762)
Etiology
  • Unknown etiology
Clinical features
Diagnostic criteria
  • Splenomegaly with nodules comprising benign tortuous vascular channels
  • Dual endothelial and histiocytic immunophenotype lacking CD34

Laboratory
  • Thrombocytopenia, anemia in subset of patients
Radiology description
  • Enlarged spleen
  • Ultrasound varies from heterogeneous echotexture without specific nodules to hyperechogenic, hypoechogenic or isoechogenic lesions
  • CT shows solitary or multiple hypoattenuating nodules (BMJ Case Rep 2015 Nov 24;2015)
  • MRI shows hypodense lesions on T1 and T2 weighted scans due to hemosiderin (Int J Clin Exp Pathol 2015;8:8516)
Radiology images

Contributed by Patricia Tsang, M.D., M.B.A.

CT with contrast

 

Case of the Week #179

CT scan

Prognostic factors
  • Typically benign with excellent prognosis postsplenectomy
  • Increased risk of malignant transformation with massive splenomegaly (> 1500 grams or > 20 cm long) (Minerva Chir 2014;69:229)
Case reports
Treatment
  • Splenectomy
Gross description
  • Solitary or multiple distinct splenic nodules
  • Minute to large nodules of variable consistency
  • Various colors (yellow / brown / red / black) depending on degree of necrosis, cyst formation, thrombi and fibrosis (Int J Clin Exp Pathol 2015;8:8516)
  • Spongy / cystic (J Cytol 2017;34:121)
Gross images

Case of the Week #179

Cystic cut surface



Images hosted on other servers:

Multiple nodules

Microscopic (histologic) description
  • Proliferation of anastomosing, tortuous, blood filled vascular channels (Int J Clin Exp Pathol 2015;8:8516)
  • Irregular channel lumina, often with papillary projections and cystic spaces
  • Lined by tall endothelial cells with variable hemophagocytosis (BMJ Case Rep 2015 Nov 24;2015)
  • Sloughing of endothelial cells into vascular spaces
  • No sclerosis or cytologic atypia
Microscopic (histologic) images

Contributed by Patricia Tsang, M.D., M.B.A.

Tortuous vascular channels

Anastomosing vascular channels

Vascular channels appear papillary

CD31

CD34

CD68

Cytology description
  • 3 dimensional, bland appearing, epithelioid foamy cells with low nuclear:cytoplasmic ratio (J Cytol 2017;34:121)
  • May contain intracytoplasmic hemosiderin pigment
Positive stains
Negative stains
Molecular / cytogenetics description
  • No specific molecular findings
Differential diagnosis
  • Angiosarcoma:
    • Atypical sarcomatous cells with hyperchromatic nuclei and CD34 positivity
    • Frequent tumor necrosis
  • Hamartoma:
    • Disorganized blood vessels with entrapped adipocytes
    • CD8+
  • Splenic littoral cell hemangioendothelioma:
    • Typically mild to moderate cytologic atypia
    • May have solid areas
    • Potentially malignant or metastasizing
  • Kaposi sarcoma:
    • Atypical spindle cells with HHV8 and CD34 positivity
    • HIV related
  • Hemangioma:
    • Usually asymptomatic, incidental finding
    • Single layer of bland endothelial cells with CD34 positivity
  • Hemangiopericytoma:
    • Oval to spindle cells surrounding staghorn blood vessels
    • CD34+, lysozyme-
    • Relatively high malignant potential
  • Lymphangioma:
    • Cystic, malformed lymphatic channels, often subcapsular
    • Attenuated endothelial lining
Board review question #1
A 55 year old man presents with abdominal pain and multiple splenic nodules. The microscopic H&E image is shown below. The proliferative fraction is 20%. Which of the following immunostains is expected to be negative?




  1. CD31
  2. CD34
  3. CD68
  4. Factor VIII
Board review answer #1
B. CD34 is negative, even though other vascular endothelial cell associated markers are positive in littoral cell angioma.

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Board review question #2
Which of the following statements is true about littoral cell angioma?

  1. Hypersplenism related anemia or thrombocytopenia is a common finding
  2. It is pathogenetically linked to human herpesvirus 8 (HHV8)
  3. It originates from the white pulp vascular lining cells of the spleen
  4. Malignant transformation is common among middle aged and elderly patients
Board review answer #2
A. Hypersplenism related anemia or thrombocytopenia is a common finding. Hypersplenism in patients with littoral cell angioma can cause entrapment of RBCs and platelets in the spleen, leading to anemia and thrombocytopenia.

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