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Littoral Cell Splenic Angioma: Facts

 
  • It is a primary vascular tumor arising from the cells lining the splenic red pulp venous sinuses, hence the name littoral cells. 
  • LCA is a rare splenic vascular tumor discovered either as an incidental finding or patients may present with abdominal pain. 
  • The gross appearance of the spleen can be slight to moderately enlarged size and weight, with a cross-section showing widened splenic trabeculae and more commonly multifocal than a solitary nodule.

 

Littoral Cell Splenic Angioma: Facts

The LCA can present as an incidental finding in an asymptomatic patient or as abdominal pain. The physical exam can reveal the presence of splenomegaly in some cases. Other less common clinical features include the presence of anemia, thrombocytopenia, hepatitis, cirrhosis, and portal hypertension.

 

Littoral Cell Splenic Angioma: Facts

The radiological imaging studies like magnetic resonance imaging (MRI) and computed tomography scan (CT scan) have not proven to be adequate in diagnosing littoral cell angioma, given that it is difficult to differentiate from other splenic neoplasms like angiosarcomas, lymphomas, metastatic tumors on imaging. Owing to the hemosiderin deposits in the tumor cells, hypodense lesions are visible on T1 and T2 weighted MRI images. Sonography evaluation is not helpful because of the variable findings of heterogeneous echotexture and no definite lesions.

 

Littoral Cell Splenic Angioma: Facts

The differential diagnosis includes other vascular neoplasms of the spleen, including splenic hemangioma, lymphangioma, hamartoma, angiosarcoma. The LCA can be differentiated from these lesions based on the histopathological and immunophenotyping findings, as detailed above. Imaging studies (MRI, CT scan, ultrasound) have not demonstrated usefulness in differentiating between these.

 

Littoral Cell Angioma

Littoral Cell Angioma

 

MDCT of the Spleen

 

MDCT of the Spleen

 

“Inflammatory pseudotumor of the spleen is a rare benign process with nonspecific CT features. The mass was generally hypodense with delayed enhancement on CT.”
Computed tomographic and clinicopathological features of inflammatory pseudotumor of the spleen. 
Lu T, Yang C
J Comput Assist Tomogr. 2015;39(3):409‐413.

 

Inflammatory Pseudotumor of the Spleen

Inflammatory Pseudotumor of the Spleen

 

MDCT of the Spleen

 

MDCT of the Spleen

 

MDCT of the Spleen

 

 ”The definitive diagnosis is established with the histological findings, characterized by the presence of inflammatory cells with areas of necrosis and fibrosis. There are multiple differentials diagnoses: metastasis, lymphoma, splenic infarction, hemangiomas, vascular malformations, lymphangioma, plasmacytoma, reactive lymphoid hyperplasia, abscess and infectious granulomatous processes; therefore suspicion of malignant neoplasm must be considered, being indicated splenectomy to confirm the diagnosis.”
Inflammatory pseudotumor of spleen: a case report. 
Ugalde P et al.
Int J Surg Case Rep. 2015;7C:145‐148.

 

Sclerosing Angiomatoid Nodular Transformation (SANT) of the Spleen

  • Recently recognized
  • Rare, 30 reported cases
  • Nonneoplastic vascular lesion
  • Microscopically, all the reported cases have multiple angiomatoid nodules in a fibrosclerotic stroma
  • DDX: splenic hamartoma, inflammatory myofibroblastic tumor, littoral cell angioma, and hemangioendothelioma
  • FM
  • Age 22-74 years

 

Sclerosing Angiomatoid Nodular Transformation (SANT) of the Spleen

  • Most incidental findings in asymptomatic patients
  • Some present with abdominal pain or discomfort
  • Splenomegaly rare
  • Benign course with splenectomy curative

 

MDCT of the Spleen

 

“ While there are several imaging features of SANT which have been described in the literature, it is not routinely possible to make a prospective diagnosis based on the imaging features alone. Moreover, it may not be possible to exclude malignancy based on the imaging features, and needle biopsy or splenectomy may be required in certain cases.”
Sclerosing Angiomatoid Nodular Transformation of the Spleen (SANT) – Multimodality Imaging Appearance of Five Cases with Radiology-Pathology Correlation
Raman SP, Hruban RH, Singhi A, Horton KM, Fishman EK
Abdom Imaging 2013 Aug;38(4):827-34

 

Splenic Malignancies

Primary tumor
  • Lymphoma
  • Angiosarcoma
Metastatic disease
  • Melanoma
  • Lung cancer
  • Breast cancer
  • Renal cancer

 

Angiosarcoma Spleen and Liver

Angiosarcoma Spleen and Liver

 

MDCT of the Spleen

 

"Primary angiosarcoma of the spleen is a rare and aggressive malignant neoplasm arising from splenic vascular endothelium and mesenchymal-derived elongated endothelial cells lining the spleen's spongy network of sinusoids. In general, angiosarcomas are rapidly proliferating, highly infiltrating anaplastic cells that tend to recur locally, spread widely, and have an increased rate of lymph node and systemic metastases.”
Primary Splenic Angiosarcoma
Kamran S. Hamid et al.
JSLS. 2010 Jul-Sep; 14(3): 431–435.

 

”On CT scans, the most common finding is an ill-defined heterogeneously enhancing splenic mass with areas of necrosis. In the event of acute rupture, hemorrhage will appear hyperattenuated on unenhanced images. There is no particular pattern of calcification associated with splenic angiosarcoma, but areas of hypervascular metastases to the liver, lungs, bones, and lymphatic system are well demonstrated on CT.”
Primary Splenic Angiosarcoma
Kamran S. Hamid et al.
JSLS. 2010 Jul-Sep; 14(3): 431–435.

 

“The most common CT finding, found in six (60%) patients, was an enlarged spleen that contained a large heterogeneous complex mass or masses that almost completely replaced the spleen. In two of these patients, there was substantial contrast enhancement, and in the other four patients, there was only minimal contrast enhancement, with areas of decreased attenuation suggesting necrosis.”
Angiosarcoma of the Spleen: Imaging Characteristics in 12 Patients
Thompson WM et al.
Radiology 2005; 235:106-115

 

“CT shows an enlarged spleen with areas of low and high attenuation, due to acute hemor- rhage or hemosiderin deposits, and calci- fications can be seen. Contrast enhancement of angiosarcoma is variable depending on the degree of tumoral necrosis and can mimic that of a hepatic hemangioma by showing avid peripheral enhancement.”
Nonneoplastic, Benign, and Malignant Splenic Diseases: Cross-Sectional Imaging Findings and Rare Disease Entities
Thipphavong S et al.
AJR 2014;203: 315-322

 

Splenic Tumors: Metastatic Disease-Differential DX

  • Melanoma
  • Ovarian cancer
  • Pancreatic cancer

 

Splenic Metastases

  • Hematogenous spread
  • Direct extension
    • Pancreas
    • Kidney
    • Colon

 

“ Splenic metastases can occur with widespread disease, and parenchymal disease is caused by hematogenous dissemination. The most common primary cancers with splenic metastases include melanoma and cancers of the breast, lung, ovary, stomach, and prostate.”
Nonneoplastic, Benign, and Malignant Splenic Diseases: Cross-Sectional Imaging Findings and Rare Disease Entities
Thipphavong S et al.
AJR 2014;203: 315-322

 

Metastatic Ovarian cancer to Spleen

Metastatic Ovarian cancer to Spleen

 

MDCT of the Spleen

 

Splenic and Adrenal Metastases from RCC

Splenic and Adrenal Metastases from RCC

 

MDCT of the Spleen

 

Metastatic Neuroendocrine Lung to Spleen

Metastatic Neuroendocrine Lung to Spleen

 

MDCT of the Spleen

 

Melanoma Metastatic to the Liver and Spleen

Melanoma Metastatic to the Liver and Spleen

 

MDCT of the Spleen

 

MDCT of the Spleen

 

MDCT of the Spleen

 

The Spleen May be Involved By Adjacent Tumors

  • Pancreatic cancer
  • Gastric cancer
  • Renal cancer
  • Retroperitoneal sarcomas

 

Pancreatic Cancer Invades the Spleen

Pancreatic Cancer Invades the Spleen

 

MDCT of the Spleen

 

 
 

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