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      Splenic Angiosarcoma Diagnosed on Bone Marrow Biopsy: Case Report and Literature Review

      case-report

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          Abstract

          Primary splenic angiosarcoma carries a poor prognosis and is among the rarest forms of malignancy. An overwhelming majority of patients with splenic angiosarcoma will develop metastases. However, osseous metastatic disease is rare. We present an 83 year old hispanic female who was diagnosed with primary splenic angiosarcoma on bone marrow biopsy performed for a hematologic workup. We highlight key historical, laboratory, imaging, and pathological features of splenic angiosarcoma. The synthesis of both imaging features and clinical history is essential for establishing early diagnosis in these patients.

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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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            Primary and secondary neoplasms of the spleen

            R.K. Kaza, S. Azar, M.M. Al-Hawary … (2010)
            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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              Angiosarcoma of the spleen: imaging characteristics in 12 patients.

              Phillip Levy, R Abbott, Luis Gorospe … (2005)
              To retrospectively review clinical, pathologic, and imaging features of angiosarcoma of the spleen in 12 patients. Institutional review board approval was obtained, and informed consent was not required. Records of 12 cases of proved angiosarcoma of the spleen were accessed from the files of the Armed Forces Institute of Pathology. Clinical, pathologic, and imaging findings were reviewed. Presenting signs and symptoms were recorded, and the pathologist confirmed the diagnosis and determined gross and microscopic morphologic findings in each spleen. Radiologists reviewed available images to determine the size of the spleen and mass, amount of splenic involvement by the tumor (if enhanced at computed tomography [CT] and magnetic resonance [MR] imaging), and amount of nonviable tumor determined as decreased echogenicity at ultrasonography (US) and lack of enhancement at CT and MR imaging. Imaging and pathologic findings were compared. Five US, 10 CT, three MR, and two angiographic images were reviewed by two experienced abdominal radiologists. There were seven men and five women (age range, 36-86 years; mean, 55 years). The most common symptom was upper abdominal pain in eight (67%) patients: Pain was acute for 24 hours prior to admission in one patient and chronic (range, 1-6 months) in seven patients. At imaging, the spleen was enlarged (>12 cm in length) in nine patients. The most common finding, seen in seven (58%) patients, was a complex mass or masses in an enlarged spleen. Four of these patients had evidence of metastases and one had intraperitoneal hemorrhage. Two patients had solitary hypervascular tumors and liver metastases. One patient had a normal-sized spleen with multiple lesions that ranged 2-3 cm in size, as well as metastases to the spine. The 11th patient had two small lesions, with small calcifications in the periphery of one lesion. The 12th patient had intraabdominal hemorrhage around the spleen and no obvious mass at CT. Tumor necrosis was confirmed at histologic evaluation in nine patients. The most common clinical finding was upper abdominal pain. Angiosarcoma of the spleen could be suggested in the majority of cases (83%) by using the imaging features of splenic mass with evidence of metastatic disease. (c) RSNA, 2005
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                Author and article information

                Contributors
                Asaph C.J. Levy
                Journal
                Journal ID (nlm-ta): Radiol Case Rep
                Journal ID (iso-abbrev): Radiol Case Rep
                Title: Radiology Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 1930-0433
                Publication date PMC-release: 04 January 2019
                Publication date Collection: March 2019
                Publication date (Electronic): 04 January 2019
                Volume: 14
                Issue: 3
                Pages: 390-395
                Affiliations
                [a ]Department of Radiology, Northwell Health System, Lenox Hill Hospital, 100 East 77th St, New York, NY 10075 USA
                [b ]Department of Pathology, Mount Sinai Hospital Health System, Mount Sinai Hospital, New York, NY 10075 USA
                [c ]Department of Pathology, Northwell Health System, Lenox Hill Hospital, 100 East 77th St, New York, NY 10075 USA
                Author notes
                [* ]Corresponding author. asaphlevy@ 123456gmail.com
                Article
                Publisher ID: S1930-0433(18)30532-6
                DOI: 10.1016/j.radcr.2018.12.008
                PMC ID: 6321865
                PubMed ID: 30627296
                SO-VID: 18362a20-f474-4446-b2e4-160d8b60c3fa
                Copyright © © 2019 Published by Elsevier Inc. on behalf of University of Washington.
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 22 November 2018
                Date revision received : 21 December 2018
                Date accepted : 22 December 2018
                Categories
                Subject: Oncology

                Keywords: primary,splenic,angiosarcoma,spleen,bone,metastasis
                Data availability:
                Keywords: primary, splenic, angiosarcoma, spleen, bone, metastasis

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