The splenic Littoral cell angioma in China: a case report and review
© Hu et al; licensee BioMed Central Ltd. 2011
Received: 20 September 2011
Accepted: 15 December 2011
Published: 15 December 2011
Littoral cell angioma (LCA) is a rare splenic vascular neoplasm that arises from the cells lining the red pulp sinuses. It is deemed to be a benign and incidental lesion. The earliest literature report of littoral cell angioma has been described by Falk. The examination of samples after splenectomy reveals similar pathological change and its change rule is summarized. However, many recent reports have described it to be a malignant tumor with congenital and immunological associations. Generally speaking, the definitive diagnosis can only be made after histological and immunohistochemical profiles. In this case report, we presented the case of a 48-year-old woman with multiple splenic LCAs. Initially, the patient was characteristics of abdominal distension, weakness and fatigue. Multiple hemangiomas were observed in the spleen through abdominal ultrasonic diagnosis. Computed tomography (CT) scans revealed the splenomegaly with multiple round and hyperdense lesions. The patient subsequently underwent splenectomy. Postoperative histological and immunohistochemical studies confirmed the diagnosis of LCA. Based on the presentation of this case, clinical, radiographic and pathological results of LCA as well as recent advances in our understanding of this uncommon splenic lesion were reviewed. LCA is an uncommon splenic tumor diagnosed in patients with or without abdominal discomfort. Only a few case reports regarding this kind of tumor have been published as inconsistent results. In the present paper, we have reported a case of LCA and reviewed the literature.
LCA is a benign neoplasm of the spleen that was first described by Falk et al. in 1991 [1, 2], when they illustrated 17 cases of "a novel type of vascular tumor" . Since then, no more than 80 additional cases have been reported [4, 5]. The clinical representation of LCA ranges from being completely asymptomatic and discovered incidentally, to presenting with aconstellation of signs and symptoms such as abdominal distension, complex constitutional symptoms, splenomegaly, and hypersplenism [6, 7]. Although the computed tomography (CT) and ultrasound (US) features of this neoplasm have been well described, there is a lack of specificity in differentiating the tumor from other primary vascular splenic tumors, namely, hemangiomas and angiosarcomas [8–10]. The definitive diagnosis can only be made after histological and immunohistochemical studies . Since this is initial description, there only have scattered case reports and few case series of LCA. In this article, we would like to report a case of the rare splenic vascular neoplasm.
The differential diagnosis of splenic vascular tumors are broad and may represent benign (haemangioma, haemartoma and lymphangioma), indeterminate (LCA, haemangioendothelioma and haemangiopericytoma), or malignant neoplasm (angiosarcoma) . Since the 1930s, endothelial cells lining the vascular sinuses of the spleen were considered as unique due to the exhibition of both phagocytic and hematopoietic properties . Neoplasia of these cells results in the formation of LCA, which exhibits histological and molecular features consistent with both these epithelial and histiocytic cell types [17, 18].
The exact incidence of LCA is unknown although the incidence of splenic haemangioma varies from 0.03% to as high as 14% in a series of autopsy reports . Clinically, most patients (> 55%) are asymptomatic. The remaining patients with LCA present with splenomegaly, thrombocytopenia, anemia, or constitutional symptoms such as the fever of unknown origins . The symptoms of anemia and thrombocytopenia may be hypersplenism-associated. Patients may also present with abdominal pain, or the tumor is diagnosed as an incidental finding .
Usually, the diagnosis is made after splenectomy for some other reasons. However, approximately 45% patients will present with splenomegaly, fever and features of hypersplenism (anaemia and thrombocytopenia) .
LCA may occur at any ages without sex-based predilection. Clinically, the disease may manifest as splenomegaly, anemia, and, less frequently, thrombocytopenia. LCA may also appear as a single or multiple lesions in the spleen. Diagnosis is usually made incidentally during surgery. Massive splenomegaly because of LCA can be mistaken as a pancreatic tumor.
Recent reports describe LCA as being associated with neoplasms of the colon, kidney, pancreas, lung and ovary [23, 24]. Associations with leiomyosarcoma, melanoma and lymphoma have also been reported. In view of these findings, visceral neoplasm should be ruled out in all LCA patients.
As a result of its unique histological and immunohistochemical characteristics, the definitive diagnosis can only be made on tissue samples obtained either cytologically or post-splenectomy because radiological findings are not specific for LCA. Diagnosis can be made by fine-needle biopsy, but surgery seems crucial because of suspected malignancy. Therefore, an evaluation for concomitant malignancy and surveillance is recommended.
Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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