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      Primary and secondary neoplasms of the spleen

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      Author(s): , , ,
      Publication date (Electronic):
      Journal: Cancer Imaging
      Publisher: e-Med
      Keywords: Spleen, neoplasms, CT, MRI, FDG-PET

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          Abstract

          With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving the spleen are lymphoma and angiosarcoma. Primary benign neoplasms involving the spleen include hemangioma, lymphangioma, littoral cell angioma and splenic cyst and solid lesions such as hamartoma and inflammatory pseudotumor.

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          From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation.

          Robert Abbott, Angela Levy, Nadine S I Aguilera … (2004)
          Primary vascular neoplasms of the spleen constitute the majority of nonhematolymphoid splenic tumors. The benign primary vascular tumors include hemangioma, hamartoma, and lymphangioma, whereas those of variable or uncertain biologic behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma. The primary malignant vascular neoplasm of the spleen is angiosarcoma. Peliosis is a rare lesion of unknown cause that is usually found incidentally in asymptomatic patients but may be associated with hematologic or metastatic disease. Although these vascular neoplasms of the spleen are uncommon, their importance lies in that they must be differentiated from the more common neoplastic disorders of the spleen, such as lymphoma and metastasis. The most common echogenic solid or complex cystic mass in an asymptomatic patient is splenic hemangioma. However, the imaging appearance of splenic hemangiomas may be complex, and differentiation of these lesions from malignant disease may not be possible. The diagnosis of splenic hamartoma may be suggested when findings of increased blood flow on color Doppler images are seen in association with a homogeneous solid echogenic mass. A large subcapsular solitary cystic abnormality discovered incidentally in a child in association with internal septations and tiny mural nodules favors the diagnosis of lymphangioma. Any invasion of the surrounding splenic parenchyma by a splenic lesion should indicate a more aggressive or malignant process. Evaluation of a focal splenic abnormality identified on sonograms should be followed up with computed tomography or magnetic resonance imaging with and without contrast material enhancement. Splenectomy may be required for definitive evaluation of a splenic mass with atypical features.
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            Metastatic tumors to the spleen: a 25-year clinicopathologic study.

            Hugo Lam, V Tang (2000)
            The clinicopathologic features of splenic metastases have seldom been investigated. The aim of this study was to evaluate the clinical and pathological impact of splenic metastases. We reviewed the clinical/autopsy records and pathologic features of 92 Chinese patients (58 men, 34 women) with secondary nonlymphoid splenic tumors recorded during a 25-year period. The incidence of splenic secondary tumors at autopsy was 0. 6% and at splenectomy, 1.1%. The lesions were often seen in elderly patients (mean age, 60 years). Seven (8%) of the splenic lesions were symptomatic. The symptomatic splenic lesions, as compared with asymptomatic lesions, were bigger and were found more often in women and younger patients. Two patients experienced spontaneous splenic rupture because of metastatic carcinoma. Eighty-seven (95%) of the secondary splenic tumors were carcinomas. Lung was the most common primary tumor site (21%), followed by the stomach (16%), pancreas (12%), liver (9%), and colon (9%). Rarely reported sources of primary tumor, such as esophageal carcinomas, nasopharyngeal carcinoma, and choriocarcinoma, were also found. Splenic metastases could be identified macroscopically in 74 (80%) of our patients. Grossly, splenic metastases involved the splenic capsule (n = 8) or were solitary (n = 31), multiple (n = 30), or diffuse (n = 8) lesions in the splenic parenchyma. Isolated splenic metastases were noted in 5.3% of the metastases found at autopsy. Many of the metastatic lesions in the spleen were identified shortly after primary tumors were detected (mean latent period, 6.7 months). The time from diagnosis of the primary tumor to metastasis to the spleen was more than 2 years in 14 patients. Splenic metastases are uncommon. A variety of clinical and pathologic patterns were noted in our series.
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              Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases.

              Kim Krishnan, J Meis, Christine S. Falk (1993)
              Forty primary splenic angiosarcomas occurring in 21 men and 19 women, 19-84 years old (median 59 years) are reported. Patients presented with splenomegaly (35 of 38, 92%), abdominal pain (33 of 40, 83%), and systemic symptoms such as fatigue (2 of 40, 5%), fever (4 of 40, 10%), and/or weight loss (16 of 40, 40%). Five (13%) experienced splenic rupture associated with hemoperitoneum. Abnormal laboratory findings included cytopenia (31 of 34, 91%), leukocytosis (8 of 21, 38%), and thrombocytosis (1/39, 3%). Most spleens weighed 500-1,000 g (mean, 1,180 g). The cut splenic surfaces showed multiple hemorrhagic nodules that were frequently associated with infarction, although some had a diffuse pattern of involvement. Microscopically, there were a variety of histologic patterns displayed by the vasoformative component. A honeycomb or sponge-like pattern was common in some, whereas others simulated a cavernous hemangioma or normal splenic sinuses (pseudosinusoidal pattern). Papillary endothelial tufts and solid proliferations of spindled to round to epithelioid cells were also seen. Factor VIII-related antigen was detected in 19 of 23 cases, BMA-120 in 18 of 23, UEA-1 receptor in 18 of 23, and vimentin in 23 of 23 as well as CD68 antigen in 1 of 23 cases. S-100 protein and cytokeratin were not found in any of the 23 cases studied. Metastases in 22 of 32 patients (69%) were to the liver (13 patients), bone or bone marrow (7 patients), lymph nodes (1 patient), and brain (1 patient). Three patients had concomitant malignancies and one had a prior history of a mixed B-cell lymphoma 5 years previously that had been treated with chemotherapy. Follow-up in 38 patients revealed that 30 (79%) are dead at a median interval of 6 months (range 0-48 months) and 8 are alive 5-21 months after diagnosis. These findings indicate that splenic angiosarcoma is an aggressive neoplasm with a high metastatic rate and an abysmal prognosis. Recognition of the wide range of histologic patterns is of diagnostic value but no apparent prognostic significance.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cancer Imaging
                Journal ID (publisher-id): CI
                Journal ID (publisher-id): Cancer Imaging
                Title: Cancer Imaging
                Publisher: e-Med
                ISSN (Print): 1740-5025
                ISSN (Electronic): 1470-7330
                Publication date Collection: 2010
                Publication date (Electronic): 13 August 2010
                Volume: 10
                Issue: 1
                Pages: 173-182
                Affiliations
                Department of Radiology, Box 30, University of Michigan Hospitals, Ann Arbor, MI, USA
                Author notes
                Corresponding address: Ravi Kaza, MD, Department of Radiology, Box 30, Room B2, University of Michigan Hospitals, 1500, East Medical Center Drive, Ann Arbor, MI 48109-0030, USA. Email: ravikaza@ 123456med.umich.edu
                Article
                Publisher ID: ci100026
                DOI: 10.1102/1470-7330.2010.0026
                PMC ID: 2943678
                PubMed ID: 20713317
                SO-VID: a8877eb2-dc94-4f11-ad5a-2f596e2b4902
                Copyright © © 2010 International Cancer Imaging Society
                History
                Date accepted : 13 July 2010
                Categories
                Subject: Review

                Keywords: spleen,neoplasms,ct,mri,fdg-pet
                Data availability:
                Keywords: spleen, neoplasms, ct, mri, fdg-pet

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