Background: Littoral cell angioma (LCA) is a rare vascular tumour of the spleen with unique histologic and immunophenotypic features. LCA is generally diagnosed incidentally and has a benign clinical course. However, it has rarely been reported to behave as a malign entity.
Case presentation: A 49-year-old woman with cerebral palsy admitted to the hospital suffering from abdominal pain and weight loss. Laboratory results revealed iron deficiency anaemia and mild thrombocytopenia. Computed tomography scan revealed splenomegaly with multiple hypoattenuating lesions. A diagnostic and therapeutic open splenectomy and sigmoid colectomy were performed to exclude possible lymphoproliferative disorders or malignant lesions. The histologic and immunohistochemical studies confirmed the diagnosis of LCA and sigmoid volvulus. The post-operative period was uneventful with a significant increase in haemoglobin and platelet levels. Unfortunately, the patient died due to sepsis 36 days after surgery.
Conclusion: This is the first case of LCA and sigmoid volvulus. Although this association is likely to be entirely coincidental, clinicians should be aware of this rare clinicopathologic entity whilst evaluating the differential diagnosis of splenomegaly with solitary or multiple nodules.
Key words: Littoral cell angioma, spleen, vascular tumour, cerebral palsy, sigmoid volvulus, case report
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