Journal of Clinical Oncology, Vol 23, No 19 (July 1), 2005: pp. 4460-4462
© 2005 American Society of Clinical Oncology.
DOI: 10.1200/JCO.2005.05.066
Patients With Unusual Bladder Malignancies and a Rare Cause of Splenomegaly
CASE 3. Littoral Cell Angioma of the Spleen in a Patient With Previous Lymphoma
Justin D. Floyd,
Paul A. Kaplan,
Edward R. Sauter,
Michael C. Perry,
Donald C. Doll
Ellis Fischel Cancer Center, University of Missouri—Columbia, Columbia, MO
A 42-year-old white man presented with a 3- to 4-day history of fever, malaise, and cough. The patient had a history of diffuse large B-cell lymphoma, nodular sclerosing Hodgkin’s disease, and diffuse large T-cell lymphoma treated previously with multiple chemotherapeutic regimens, radiation therapy, and stem-cell transplantation. During the previous 2 to 3 years, the patient had been hospitalized for six episodes of fever of unknown origin (FUO), which resolved after broad-spectrum antibiotic therapy. Physical examination was significant only for diminished breath sounds in both lung fields. Laboratory data revealed a WBC count of 8,500/mm3 with normal differential, hemoglobin 12.4 g/dL, platelets 85,000/mm3, and normal electrolytes and urinalysis. A coagulase-negative Staphylococcus found in one of two blood cultures was considered a false-positive secondary-to-skin contamination. Chest radiography and computed tomography (CT) scan of the chest, abdomen, and pelvis demonstrated bilateral pleural and pericardial effusions that had been present in previous studies. In addition, multiple splenic hypodensities were evident and similar to those identified by CT in July 1998 (Fig 1). Antimicrobial therapy was initiated, and surgical oncology was consulted for a potential diagnostic splenectomy. The patient defervesced, was discharged on oral antibiotics after 3 days of parenteral antibiotic therapy, and was scheduled for elective splenectomy. After appropriate preoperative immunization for S pneumoniae, H influenza, and N meningitidis, splenectomy was performed. The patient was discharged with follow-up evaluation in the clinic. He has not had recurrent fever, and his platelet count has normalized. Gross examination of the 920 g, 18 x 11 x 8-cm spleen showed a coarsely nodular red external surface. A small 0.6-cm accessory spleen was present within the hilus. The cut surface was extensively replaced by sharply circumscribed, nonencapsulated nodules ranging in size from 0.1 to 2.2 cm. All of the nodules were of similar character, having bulging hemorrhagic spongy tan surfaces. Microscopic examination showed that all nodules were vascular tumors composed of sinusoidal spaces with irregular compressed lumina. The sinuses within the tumors were lined by predominantly tall plump endothelial cells with regular indented nuclei lacking stratification, atypia, or mitotic activity. In multiple areas, numerous exfoliated cells were present within the lumina, many demonstrating hemophagocytosis (Fig 2B, arrows). The adjacent parenchyma showed patchy areas of hemosiderin deposition and extramedullary hematopoiesis. No morphologic or flow cytometric evidence of a lymphoproliferative disorder was present. The here-described gross and microscopic features were consistent with littoral cell angioma (LCA), present both in the spleen (Fig 2B) and an accessory spleen (Fig 2A).
LCA is considered a benign neoplasm arising from the littoral cells lining the sinuses of the red pulp in normal splenic tissue. Littoral cells possess morphologic and immunophenotypic features that reflect a dual endothelial and histiocytic potential of splenic sinus-lining cells.1 To date, including the 17 cases initially described in 1991, only 29 cases of LCA have been reported.3 The present case is the only report of LCA involving an accessory spleen. LCA presents in all age groups and has no sex predilection. LCAs are usually diagnosed in patients undergoing splenectomy for splenomegaly of unknown origin or during radiographic evaluation of other abdominal processes.2 Radiographically, LCA is characterized by multiple cystic, blood-filled hypodensities ranging in size from 0.2 to 9.0 cm in diameter.2 Nine of the 29 documented cases have been associated with malignancy: non-Hodgkin’s lymphoma, two patients; renal cell carcinoma, two patients; colorectal carcinoma, two patients; non–small-cell lung carcinoma, one patient; gastric leiomyosarcoma, one patient; and pancreatic carcinoma, one patient.2 Similar to our patient, two cases presented with fever of unknown origin that abated after splenectomy.3 In general, LCA has a benign clinical course; however, one reported patient had disseminated disease that responded to radiation and cytotoxic chemotherapy.3 Furthermore, cases have been noted describing variants of LCA possessing histologic features of malignancy.1 Our case lends further support to the apparent benign course of LCA. LCA should be included in the differential diagnosis of patients with FUO, a history of malignancy, and multiple splenic hypodensities. In such situations, splenectomy may be both diagnostic and therapeutic.
Authors' Disclosures of Potential Conflicts of Interest
The authors indicated no potential conflicts of interest.
REFERENCES
1. Levy AD, Abbott RM, Abbondanzo SL: Littoral cell angioma of the spleen: CT features with clinicopathologic comparison. Radiology 230:485-490, 2004[Abstract/Free Full Text]
2. Kutok JL, Christopher DM: Splenic vascular tumors. Semin Diagn Pathol 20:128-139, 2003[Medline]
3. Falk S, Stutte HJ, Frizzera G: Littoral cell angioma: A novel splenic vascular lesion demonstrating histiocytic differentiation. Am J Surg Pathol 15:1023-1033, 1991[Medline]
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