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| Year : 2019 | Volume
: 62
| Issue : 3 | Page : 491-492 |
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| Primary epithelioid angiosarcoma of the spleen |
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Shufei Wei1, Yongliang Han2, Ting Li1, Xiao Xu1
1 Department of Pathology, Jiujiang University Hospital, Jiujiang, Jiangxi, China
2 Department of Pathology, Jiujiang No. 1 People's Hospital, Jiujiang, Jiangxi, China
Click here for correspondence address and email
| Date of Web Publication | 26-Jul-2019 |
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How to cite this article:
Wei S, Han Y, Li T, Xu X. Primary epithelioid angiosarcoma of the spleen. Indian J Pathol Microbiol 2019;62:491-2 |
A 65-year-old man was admitted with acute abdomen, which he had been experiencing for 1 hour, and which did not have an obvious cause. Physical examination revealed severe tenderness in the left upper abdomen. The spleen was palpably enlarged at the left lower edge of the costal margin. An abdominal computed tomography scan showed splenic subcapsular hematoma and a small amount of intraperitoneal fluid [Figure 1]a. Therefore, he was assumed to have a rupture of the spleen. Laparotomy and splenectomy were performed for further treatment and diagnosis. The spleen measured 15 × 10 × 3 cm with the capsule rupture. A focal lesion was evident. It measured 3.5 × 2.5 × 2.5 cm and had a tan-gray cut surface [Figure 1]b. Inspection of the splenic lesion showed that the normal spleen cells had been replaced by atypical epithelioid cells and infiltrated the surrounding tissue. The epithelioid cells were large, oval or round, and had abundant cytoplasm, large vesicular nuclei, prominent nucleoli, and frequent karyokinesis. The tumor cells showed diffuse growth and were arranged in sheets. Occasionally, they also showed vascular cavity formation and papillary structure [Figure 2]a and [Figure 2]b. On the immunohistochemical examination, the tumor cells showed strong positivity for cluster of differentiation (CD) 31 [Figure 3]a, cytokeratin (CK) [Figure 3]b, and vimentin [Figure 3]c, as well as focal positivity for CD34. However, the tumor cells were negative for epithelial membrane antigen (EMA), and factor VIII (FVIII). The Ki-67 proliferation index was about 30% [Figure 3]d. Based on the clinical characteristics, histopathology, and immunohistochemical phenotype, the patient was diagnosed with splenic epithelioid angiosarcoma. He died a month after the laparotomy. | Figure 1: Splenic subcapsular hematoma and abdominal hemorrhage (a), a focal lesion was evident and had a tan-gray cut surface (b)
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 | Figure 2: The atypical epithelioid cells showed diffuse growth and were arranged in sheets. Occasionally, vascular cavity formation (a) and papillary structure (b) were evident
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 | Figure 3: The atypical epithelioid cells that infiltrated the surrounding tissue showed positive immunohistochemical staining for cluster of differentiation 31 (CD31) (a), cytokeratin (b), and vimentin (c). Ki-67 proliferation index was about 30% (d)
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The diagnosis of epithelioid angiosarcoma is assisted by the characteristic imaging findings, clinical features, histological features, and immunohistochemical findings of positivity for CD31, vimentin, and CK.[1],[2] Splenectomy is the main treatment,[3] and combination with chemotherapy before or after the splenectomy is necessary. Overall, however, the most essential action is to diagnose and treat the disease as soon as possible.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Collini P, Barisella M, Renne SL, Pizzi N, Mattavelli D, Stacchiotti S, et al. Epithelioid angiosarcoma of the thyroid gland without distant metastases at diagnosis: Report of six cases with a long follow-up. Virchows Arch 2016;469:223-32.  |
| 2. | Mauzo SH, Yang M, Brown RE, Nguyen HN, Yan P. Primary epithelioid angiosarcoma of bone with robust cell cycle progression and high expression of SPARC: A case report and review of the literature. Ann Clin Lab Sci 2015;45:360-5. |
| 3. | Frontario SC, Goldenberg-Sandau A, Roy D, Sandau R. Primary splenic angiosarcoma presenting as idiopathic thrombocytopenic purpura: A case report and review of the literature. Case Rep Surg 2016;2016:4173060. |
Correspondence Address:
Shufei Wei
Department of Pathology, Jiujiang University Hospital, No. 57, Xunyangdong Road, Jiujiang 332000, Jiangxi
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/IJPM.IJPM_170_18
[Figure 1], [Figure 2], [Figure 3] |
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