Correspondence

Littoral-Cell Angioma as a Cause of Splenomegaly

N Engl J Med 2001; 345:772-773September 6, 2001

Article

To the Editor:

Littoral-cell angioma is a rare primary tumor of the spleen.1 Considered a benign condition, littoral-cell angioma arises from the normal littoral cells lining the sinus channels of the splenic red pulp.

A 59-year-old woman with a six-month history of fatigue, low-grade fever, weight loss, sweats, and diffuse abdominal pain was admitted for an elective splenectomy. Physical examination revealed a palpable spleen. All laboratory tests were normal. An enhanced computed tomographic scan of the abdomen disclosed an enlarged spleen with multiple scattered low-attenuation nodular masses (Figure 1Figure 1Enhanced Computed Tomographic Scan Showing an Enlarged Spleen with Multiple Low-Attenuation Nodular Masses.). The 430-g resected spleen (16 by 13 by 7 cm) had a nodular surface. Cut sections revealed a spongy appearance and multiple nodular lesions measuring from 0.2 to 1.0 cm in diameter. Microscopically, the lesions consisted of anastomosing vascular channels, often with papillary projections and cyst-like spaces (Figure 2Figure 2Spleen Specimen Showing Anastomosing Vascular Channels, with Papillary Projections and Cyst-like Spaces (Hematoxylin and Eosin, ×250).). They were lined with endothelial cells showing hemophagocytosis. These cells were positive for both vascular (CD31) and histiomonocytic (CD68) markers. No atypical cells or cells in mitosis were seen. The results of karyotyping of spleen-cell suspensions were normal. Bone marrow and liver biopsies were normal. Twenty-four months after surgery, the patient was asymptomatic.

The combination of morphologic and immunohistochemical analyses showing a hybrid endothelial–histiocytic phenotype established the diagnosis of littoral-cell angioma.1 Primary tumors of the spleen other than lymphoid and hematologic tumors are quite rare, and littoral-cell angioma should be considered in the differential diagnosis of multinodular splenomegaly.2-4 There may be similar findings with lymphomas, metastatic disease, some disseminated infections, and sarcoidosis.

Although littoral-cell angioma itself is apparently benign and splenectomy is curative, one third of the previously reported cases5 were associated with cancers of visceral organs or malignant lymphomas. For this reason, we recommend close clinical follow-up of patients who have received the diagnosis of littoral-cell angioma.

Cristina Mihaela Dascalescu, M.D.
Dominique Wendum, M.D.
Norbert Claude Gorin, M.D.
Saint-Antoine University Hospital, 75571 Paris, France
ldascalescu@iutang.univ-poitiers.fr

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Citing Articles (6)

Citing Articles

1. 1

   Julia B. Pilz, Toralf Sperschneider, Thomas Lutz, Bruno Loosli, Christoph A. Maurer. (2011) Littoral cell angioma in main and accessory intrapancreatic spleen presenting as splenic rupture. The American Journal of Surgery 201:2, e15-e17
   CrossRef

2. 2

   Jason Bierenbaum, Daisy V. Alapat, Carlos Godinez, Adrian E. Park, Xianfeng Frank Zhao, Maria R. Baer. (2010) Littoral cell angioma: A correctable cause of progressive pancytopenia in a patient with myelodysplastic syndrome. Leukemia Research 34:4, e117-e119
   CrossRef

3. 3

   Moo-Kon Song, Young-Mi Seol, Young-Eun Park, Sung-Kyu An, Young-Jin Choi, Ho-Jin Shin, Joo-Seop Chung, Goon-Jae Cho. (2007) A Case of Bicytopenia Combined with Littoral Cell Angioma. The Korean Journal of Hematology 42:2, 157
   CrossRef

4. 4

   N. SUVAJDŽIĆ, V. ČEMERIKIĆ-MARTINOVI&#, ?. ŠARANOVIĆ, M. PETROVIĆ, M. POPOVIĆ, V. ARTIKO, M. ĆUPIĆ, I. ELEZOVIĆ. (2006) Littoral-cell angioma as a rare cause of splenomegaly. Clinical and Laboratory Haematology 28:5, 317-320
   CrossRef

5. 5

   Rhonda L. Harmon, Carlos A. Cerruto, Adam Scheckner. (2006) Littoral Cell Angioma: A Case Report and Review. Current Surgery 63:5, 345-350
   CrossRef

6. 6

   Vineeth Mohan, Ralph C. Jones, Almond J. Drake, Patrick L. Daly, K.M. Mohamed Shakir. (2005) Littoral Cell Angioma Presenting as Metastatic Thyroid Carcinoma to the Spleen. Thyroid 15:2, 170-175
   CrossRef