A Rare Case of Splenic Littoral Cell Angioma in a Child

Abstract

Littoral cell angioma (LCA) is a rare, benign primary vascular neoplasm of the spleen. The tumor originates from the littoral cells lining the sinuses of the red pulp of the spleen. Preoperative distinction of this tumor from other benign or malign splenic lesions is difficult. Radiologically most cases present as multiple nodules. Definitive diagnosis can only be made histopathologically and immunohistochemically following splenectomy. This clinical situation can coexist with various malignancies and autoimmune disorders. Even though, it is mostly benign, since it has the potential to become malignant after splenectomy, long-term follow-up is required. We present an LCA case, which appeared as a solitary mass in the spleen of an 11-year-old girl with abdominal pain admitted to our hospital.

Littoral cell splenic angioma, a rare diagnosis of abdominal pain in a child: case report and literature review

“…In the case reported by Bedir et al (2014), [8] in an 11-year-old girl with upper left quadrant abdominal pain and an ultrasound finding of a solid lesion, measuring 3.7x3.2cm, characterizing isoechoic and echogenic patterns and a magnetic resonance suspicious of a hemangioma, there was no response to clinical management. Only after splenectomy and immunohistochemical tests were positive for CD31 and CD68, but negative for CD34, littoral cell angioma was diagnosed.…”

“…On early phase contrastenhanced computed tomography, it may appear as multiple or solitary hypodense lesions and show changes in density during delayed image testing. [7,8] In contrast to the literature, computed tomography has led to a correct diagnostic hypothesis. [6] Most littoral cell angiomas have multiple nodules.…”

“…[1] In this test, CD34 and CD8 markers are usually negative, while CD31, CD68, and CD21 can be positive. [4,[7][8][9] The patient in question was positive for CD31 and CD68 markers, indicating a littoral cell angioma.…”

Clinical Landscape of Littoral Cell Angioma in the Spleen Based on a Comprehensive Analysis

“…The age of LCA onset showed a normal distribution with the oldest and youngest being 86 y (11) and 26 d (19), respectively. Although most of the patients were aged 40-60 years, the incidence of LCA in children was not uncommon (5.7%) (1,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30). With regard to the natural history of LCA, 11 patients had an untreated LCA.…”

“…The average age of 267 patients was 48.4 ± 16 years, with the oldest and youngest being 86 years old and 26 days old, respectively (11,19). As shown in Figure 2, LCA was more prevalent in the 40-60-year age group; however, it was detected in 15 minors (1,(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29)(30).…”