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Year : 2012  |  Volume : 55  |  Issue : 1  |  Page : 109-110
Littoral cell angioma of spleen in a patient with obstructive jaundice

Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

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Date of Web Publication11-Apr-2012

How to cite this article:
Jain M, Badwal S, Rastogi A, Gupta P. Littoral cell angioma of spleen in a patient with obstructive jaundice. Indian J Pathol Microbiol 2012;55:109-10

How to cite this URL:
Jain M, Badwal S, Rastogi A, Gupta P. Littoral cell angioma of spleen in a patient with obstructive jaundice. Indian J Pathol Microbiol [serial online] 2012 [cited 2020 Nov 24];55:109-10. Available from: https://www.ijpmonline.org/text.asp?2012/55/1/109/94876

The vascular tumors constitute second most common primary neoplasms in spleen; commonest being lymphoid malignancies. Littoral cell angioma (LCA) is a recently described entity occurring exclusively in spleen. It is a rare primary vascular tumor and is more commonly benign in nature. [1] It has unique morphological and immunohistochemical features expressing vascular and histiocytic antigens representing neoplastic proliferation of littoral cells lining the splenic sinuses. Though association with visceral malignancies has been reported, majority of the cases are fortuitously discovered on abdominal explorations carried out for an unrelated cause. [2] We report a case of LCA of spleen in a patient of obstructive jaundice due to benign biliary stricture with brief review of literature.

A 45-year-male presented with progressively increasing jaundice and pruritis of 3 month duration, with high-colored urine and acholic stools. There was no evidence of fever, pain in abdomen, weight loss, or gastrointestinal bleeding. On examination, the general condition of the patient was within normal limits with unremarkable vital parameters; the patient, however, had icterus and splenomegaly of 3 cm below left costal margin. Hematological parameters revealed mild anemia, (Hb-10 gm %), and platelet count were toward lower limits of normal (1,11,000 per cmm). Biochemical parameters revealed total serum bilirubin of 16 mg% with conjugated bilirubin of 7.2 mg% and serum and alkaline phosphatase of 684 IU/L. The abdominal ultrasound and computerized tomography (CT) showed bilateral intrahepatic biliary radical dilatation, dilatation of common bile duct (CBD), and multiple gall stones with splenomegaly of 17.8 cm. The endoscopic retrograde cholangiography (ERC) revealed dilatation and stricture in the lower CBD but no mass or stone was identified. Endoscopic retrograde stenting was performed using 10 fr 10 cm stent. Fine needle aspiration of the gall bladder was noncontributory and bile cytology was suspicious for dysplastic cells.

The patient was explored with clinical suspicion of gall bladder carcinoma. Peroperatively, the spleen was enlarged and showed multiple bluish brown nodules. There were multiple gall stones which were also impacted in the neck; the gall bladder neck and hepatoduodenal ligament were thickened. Splenectomy along with biopsy from gallbladder neck was performed.

The spleen weighed 680 g and measured 15 × 12 × 5 cm. The capsule was thickened, outer as well as the cut surface of the spleen was studded with multiple bluish brown nodules, measuring 0.2-2 cm in size [Figure 1]. Histologically, the splenic parenchyma showed well-defined nodules with compression of the adjacent splenic tissue. The nodules were composed of anastomosing vascular spaces of variable sizes lined by plump cuboidal cells with oval isomorphic nuclei. The cells showed hemophagocytosis, intraluminal hemosiderin pigment; many sloughed cells in the vascular lumina were also seen. At places prominent papillary projections with plump lining cells protruding into the vascular spaces were also present [Figure 2] and [Figure 3]. Immunohistochemically, there was positivity for CD68 and CD31 in the lining tumor cells. The diagnosis of LCA of the spleen was offered. The patient had uneventful postoperative recovery. Follow-up during past 3 months did not reveal any recurrence or metastasis.
Figure 1: Cut surface of spleen studded with multiple well-defined tumor nodules

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Figure 2: Part of tumor nodule with anastomosing vascular spaces (lower side) and adjacent splenic parenchyma (H and E, ×200)

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Figure 3: Papillary projections lined with plump cuboidal cells protruding into the vascular spaces (H and E, ×200)

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   Discussion Top

Majority of the published cases of LCA were associated with visceral malignancies such as colorectal carcinomas, ovarian carcinomas, renal cell carcinomas, pancreatic tumors, and nonsmall cell carcinomas. [2],[3] Of these, the most common malignancy was that of a colorectal adenocarcinoma. [1] In majority of the cases, splenectomy was performed for splenomegaly of unknown origin with associated features of hypersplenism.

LCA originates from the cells lining the splenic sinusoids called littoral cells. The lesion invariably leads to splenomegaly with the presence of variable sized spongy and cystic nodules on gross appearance. The lesion may also occur as solitary nodule but is multifocal in about 85% of the cases. [2] The basic histopathological features are the presence of anastomosing vascular channels, reminiscent of splenic sinuses lined by sinus endothelial cells, which exhibit immunohistological features of macrophage/histiocytic and vascular endothelial cells. The vascular channels have variable appearance with papillary and solid intraluminal proliferations and cystic areas. The tumor cells display marked haemophagocytosis resulting in the clinical signs of anemia and thrombocytopenia. [4],[5]

Splenic LCA has a distinct immunohistochemical profile, being positive for CD31+ (vascular marker), CD68+ (histiocytic marker), and CD21+ (dendritic cell), whereas the hemangiomas, hamartomas, and angiosarcomas arising in spleen are CD21 negative. [5] LCA is a benign neoplasm but has a malignant counterpart in littoral cell angiosarcoma, which exhibits additional features of nuclear atypia, mitotic figures, and necrosis, which were absent in our case. [4]

LCA has characteristic gross and microscopic picture, however, other entities as hemangioma, hamartoma, hemangiopericytoma, angiosarcoma, lymphoma, or lymphangioma may be considered in differential diagnoses and need exclusion. The etiology and natural history of LCA are unclear due to rarity of cases; however, all reported cases had a benign course. [1],[5]

LCA is a rare benign vascular tumor and is a well-known clinical curiosity. We report a case of splenic LCA associated with benign etiology highlighting the fact that most cases are fortuitously diagnosed while investigating the patient for unrelated etiologies and relationship with malignancies seems to be incidental.

   References Top

1.Tan YM, Chuah KL, Wong WK. Littoral cell angioma of spleen. Ann Acad Med Singapore 2004;33:524-6.  Back to cited text no. 1
2.Chatelain D, Bonte H, Guillevin L, Balladur P, Flejou JF. Small solitary littoral cell angioma with splenic marginal zone lymphoma and villous lymphocytic leukaemia in a patient with hepatitis C. (Correspondence). Histopathology 2002;41:473-5.  Back to cited text no. 2
3.Bisceglia M, Sickel JZ, Giangaspero F, Gomes V, Amini M, Michal M. Littoral cell angioma of the spleen : A0 dditional report of four cases with emphasis on the association with the visceral organ cancers. Tumori 1998;84:595-9.  Back to cited text no. 3
4.Ziske C, Meybehm T, Sauerbruch T. Littoral cell angioma as a rare case of splenomegaly- A case report. Ann Hematol 2001;80:45-58.  Back to cited text no. 4
5.Arber DA, Strickler JG, Chen YY, Weiss LM. Splenic vascular tumours : A0 histologic, immunophenotypic and virologic study. Am J Surg Pathol 1997;21:827-35.  Back to cited text no. 5

Correspondence Address:
Manoj Jain
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow - 226 014, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.94876

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  [Figure 1], [Figure 2], [Figure 3]


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