Littoral cell angioma of the spleen in a patient with previous pulmonary sarcoidosis: A TNF-α related pathogenesis?

Department of Transplantation Medicine, University Hospital, Münster, Germany.
World Journal of Surgical Oncology (Impact Factor: 1.41). 09/2011; 9(1):106. DOI: 10.1186/1477-7819-9-106
Source: PubMed


Littoral cell angioma (LCA) is a rare vascular tumor of the spleen. Generally thought to be benign, additional cases of LCA with malignant features have been described. Thus, its malignant potential seems to vary and must be considered uncertain. The etiology remains unclear, but an immune dysregulation for the apparent association with malignancies of visceral organs or immune-mediated diseases has been proposed.
We report a case of LCA in a 43-year old male patient who presented with a loss of appetite and intermittent upper abdominal pain. Computed tomography showed multiple hypoattenuating splenic lesions which were hyperechogenic on abdominal ultrasound. Lymphoma was presumed and splenectomy was performed. Pathological evaluation revealed LCA.
LCA is a rare, primary vascular neoplasm of the spleen that might etiologically be associated with immune dysregulation. In addition, it shows a striking association with synchronous or prior malignancies. With about one-third of the reported cases to date being co-existent with malignancies of visceral organs or immune-mediated diseases, this advocates for close follow-ups in all patients diagnosed with LCA. To our knowledge, this report is the first one of LCA associated with previous pulmonary sarcoidosis and hypothesizes a TNF-α related pathogenesis of this splenic tumor.

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    • "to immunosuppression, patients have been treated for years [9] [10] [11]. TNF-í µí»¼ has been suggested to play a role in the pathogenesis of LCA, both in itself in the presence of immunedysregulation [12] and in immunosuppression with anti- TNF-í µí»¼ agents [10]. Since our patient has not been exposed to biologics it further strengthens the hypothesis that LCAs are associated with immune-dysregulation. "
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    ABSTRACT: Littoral cell angioma is a rare vascular tumor of the spleen. The pathogenesis is unknown but the lesion is associated with several malignancies and immunological disorders. The diagnosis requires histopathological examination. The malignant potential of this lesion is unknown, which is why splenectomy is recommend for all cases. Symptomatic cases generally suffer from hypersplenism and pyrexia. A previously healthy 20-year-old female was diagnosed with colonic Crohn's disease; as part of the work-up a magnetic resonance enterography was performed which showed multiple signal changes of the spleen. The patient reported chronic abdominal pain in the left upper quadrant, malaise, and fever. The unknown splenic lesions prompted a laparoscopic splenectomy; pathology revealed a littoral cell angioma. The abdominal pain and malaise remitted but the fever persisted one year despite adequate treatment of the patient's Crohn's disease. Littoral cell angioma is associated with immune-dysregulation including Crohn's disease with several reported cases. Signs and symptoms of hypersplenism and splenic lesions on imaging should raise suspicion of littoral cell angioma in patients with Crohn's disease. Magnetic resonance enterography to assess disease severity in Crohn's disease may provide an opportunity to study the prevalence and natural history of this rare splenic tumor.
    01/2015; 2015:474969. DOI:10.1155/2015/474969
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    ABSTRACT: We report for the first time on the contrast-enhanced ultrasound (CEUS) features of littoral cell angioma of the spleen (LCA). A patient presented with the incidental finding of splenomegaly which was investigated using modern ultrasound techniques. B-mode technique revealed heterogenous splenic parenchyma and small hypoechoic lesions up to 30 mm in size. Colour Doppler imaging revealed no specific vascularity. CEUS showed arterial hyper- and hypoenhancement with pronounced demarcation in the late phase. In an assumption of malignancy the lesions were biopsied using ultrasound guidance. Histology showed LCA. In this case report we discuss LCA in the light of new ultrasound techniques and present a review of the literature.
    Zeitschrift für Gastroenterologie 02/2013; 51(2):209-212. DOI:10.1055/s-0032-1325556 · 1.05 Impact Factor
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    ABSTRACT: Littoral cell angioma is a rare tumor of the spleen, usually being considered benign and typically discovered incidentally. There are three different modalities of presentation: tumoral splenomegaly, long-standing iron deficient anemia or thrombocytopenia due to hypersplenism. However, some of its manifestations could generate the suspicion of a lymphoma or other more serious condition. We present the case of a 46-year-old man with splenomegaly and iron deficiency anemia. The tumor affected the whole spleen, which was surgically removed. The histopathological examination, together with immunophenotyping, established the diagnosis. Six months after the procedure, the patient is in very good condition. Several differential diagnoses were discussed, as well as the prognostic factors. The case illustrates a rare cause of anemia and the importance of pathology in uncovering such unusual causes for this.
    Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie 12/2013; 54(3 Suppl):885-8. · 0.66 Impact Factor
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