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Well-Differentiated Angiosarcoma of Spleen: a Teaching Case Mimicking

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Well-Differentiated Angiosarcoma of Spleen: a Teaching Case Mimicking Xu et al. World Journal of Surgical Oncology (2015) 13:300 DOI 10.1186/s12957-015-0716-1 WORLD JOURNAL OF SURGICAL ONCOLOGY CASE REPORT Open Access Well-differentiated angiosarcoma of spleen: a teaching case mimicking hemagioma and cytogenetic analysis with array comparative genomic hybridization Lichen Xu1†, Yimin Zhang1†, Hong Zhao1, Qingxiao Chen2, Weihang Ma1,3* and Lanjuan Li1 Abstract Primary splenic angiosarcoma is extremely rare but aggressive malignant vascular neoplasm. Here, we report a case of vascular tumor in spleen that was initially misinterpreted as hemangioma. Two years after splenectomy, the patient admitted again with aggravated abdomen pain and severe anemia. The magnetic resonance imaging (MRI) scan showed widely metastases. The ensuing biopsy for lesion both in liver and in bone marrow showed the similar pathological findings as that in spleen, which supported the final diagnosis of well-differentiated splenic angiosarcoma with extensive metastases. The patient was dead in 3 months after discharge without chemotherapy. The copy number changes for spleen lesion detected by array comparative genome hybridization showed copy number gain at 11q23.2, 11q24.3, 12q24.33, 13q34, copy number loss at 1q24.2-q31.3, 1q41-q42.2, 1 q42.3-q43, 2q36.3-q37.3, 2q37.7, 3q13.33-q26.2, 3q28 - q29, 9p11.2, 13q11, 15q11, homozygous copy loss at 8p11.22, 22q11.23. Less than 200 cases of splenic angiosarcoma have been published in literature of English. To the best of our knowledge, this is the first time analyzed cytogenetic alteration in a well-differentiated primary splenic angiosarcoma. Keywords: Angiosarcoma, Well-differentiation, Splenectomy, Array comparative genomic hybridization, Copy number change Background cytogenetic changes to this subgroup of angiosarcoma Primary splenic angiosarcoma (PSA) is a rare malignant has been published. neoplasm of endothelial cell, originating from vascular In this article, we describe a 38-year-old female or lymphatic. PSA was first reported by Langhans in patient of malignant vascular tumor that was initially 1879. To date, no more than 200 cases of splenic angio- interpreted as benign hemangioma in spleen with sub- sarcoma have been reported in literature [1]. The tle cytological atypia and died 2 years later for multiple morphology features for this tumor are often uncharac- organ metastases. At the same time, we provided fur- teristic, ranging from well-formed anastomosing vessels ther array comparative genome hybridization (aCGH) to high-grade epithelioid or spindled cells without clear analysis to make a preliminary exploration on the earlier vasoformation [2]. Moreover, multiple appearances can identification of subgroup of angiosarcoma. To date, be seen in the same case, making great challenge for on cytogenetic analysis specific for primary splenic diagnosis. However, no comprehensive research of angiosarcoma has been reported. * Correspondence: [email protected] Case presentation †Equal contributors 1State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, A 38-year-old female patient was admitted to the hospital Collaborative Innovation Center for Diagnosis and Treatment of Infectious after suffering upper abdomen pain and fatigue for about Diseases, The First Affiliated Hospital, College of Medicine, Zhejiang 1 week in 2011. She has no history of smoking and alcohol University, 79 Qingchun Road, Hangzhou, Zhejiang 310003, China 3School of Medicine, Zhejiang University, Hangzhou, Zhejiang, China or drug abuse. The patient denied immunodeficiency, his- Full list of author information is available at the end of the article tory of malignancy and toxin exposure, and family © 2015 Xu et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Xu et al. World Journal of Surgical Oncology (2015) 13:300 Page 2 of 6 history of genetic disorders. The abdomen computed easy bleeding. The initial lab tests were as follows: low tomography (CT) (Fig. 1) has been performed for fur- hemoglobin (45 g/L, normal range 113–151), an increase ther examination. The result showed splenomegaly with of D-dimer, 78,700 ug/L (normal range 0–700ug/L), and a multifocal low-density lesions. Other organs including mild increase of aspartate aminotransferase (AST), 48 U/L liver, pancreas, renal, and adrenal were normal. Consid- (normal range 8–40U/L). Further examination of upper ering possibility of splenic neoplasm, and for further abdominal magnetic resonance imaging (MRI) was imple- clarification, splenectomy was carried out. Histopath- mented. The result (Fig. 3) showed multiple nodes in the ology (Fig. 2) evaluation showed well-formed anasto- liver and accessory spleen with rich blood supply and mosing vessel channels and proliferated endothelial cell signals of intratumoral hemorrhage. With these find- with mild atypia but inconspicuously malignant. Immu- ings in MRI, fine-needle biopsy was obtained of the nohistochemistry (Fig. 2) revealed positive staining of hepatic lesions. Due to further clarification of anemia, CD31and CD34 but negative staining of factor VIII, bone marrow trephine biopsy as well as smear for cyto- CD68, p53. The pathology findings supported the diagno- logical examination were performed. The pathology sis of hemangioma. Although no necrosis or hemorrhage evaluation (Fig. 2) on hepatic demonstrated a small was found in macroscopic and histological examin- amount of proliferated endothelial cells. The result of ation, in consideration of splenomegaly and anasto- pathology findings of bone marrow biopsy (Fig. 2) mosing capillary-sized vessels, the well-differentiated showed anastomosing vascular channels, proliferated angiosarcoma cannot be excluded. After splenectomy, endothelial cells with few mitotic figures and mild nu- the patient was quickly relieved from the pain and dis- clear atypia. The similarity of pathological findings in charged without any postoperative complications. Then, the liver, spleen, and bone marrow and clinical image the patient got lost to follow-up. presentation supported that the final diagnosis was Two years after splenectomy in December 2013, the well-differentiated angiosarcoma of the spleen with patient presented with worsening upper abdomen pain, bone and liver metastasis. The patient was initially fatigue, and frequent dizziness for 3 months. On phys- treated with analgesia and blood transfusion. Because ical examination, she showed severe anemic appear- few data can be acquired to guide chemotherapy and ance. There were no clinically palpable node and no poor therapeutic effect, she gave up further chemother- papilledema. There was no evidence of bruising and apy after discussing with her family members. She was Fig. 1 Abdomen CT scan in 2011 (a, b) demonstrated splenomegaly and multiple irregular low-density lesions. Liver, renal, adrenal, and pancreas are normal. The MRI in 2013 presented multiple cystic lesions in different sizes diffused in liver. The lesions displayed as inhomogeneous mixed signal, with high signal intensity on T2-weighted image (T2WI, c) and diffusion-weighted image (DWI, d). Some lesions that indicated eccentric or central low signal intensity were suspected intertumoral hemorrhage. Nodule lesion with abnormal sign as that in the liver was also found in remnant accessory spleen (arrow) Xu et al. World Journal of Surgical Oncology (2015) 13:300 Page 3 of 6 Fig. 2 Tumor in spleen shows well-formed anastomosing vessel channels (a, HE 100×), proliferated endothelial cells with mild atypia (b,HE400×), immunohistochemistry revealed positive staining of CD31 (c,100×)andCD34(d, 100×). Tumor cells from bone biopsy showed anastomosing vessel channels with proliferated endothelial cells without obvious malignant as that in spleen (e, HE 100×; f, HE 400×). Tumor cells from liver biopsy showed a handful of endothelial cells without obvious malignant (g,HE100×) discharged in a few days after slight relief from pain mutations for known cancer genes. The arrays were and fatigue. Follow-up showed she passed away 3 months scanned by GeneChip® Scanner 3000 (Cat#00-00212, after discharge. Affymetrix, Santa Clara, CA, USA) and Command DNA was extracted from ten 20-μm thick ribbons of Console Software 3.1 (Affymetrix, Santa Clara, CA, USA) paraffin-embedded tumor of spleen from FFPE tissue with default settings. Raw data that passed quality control blocks using DNeasy Blood &Tissue Kit (Cat#69506, were further analyzed by Affymetrix® OconScan Analysis QIAGEN, GmBH, Germany), following the manufac- Suite (Affymetrix, Santa Clara, CA, USA). turer’s instructions. A total of 80 ng sample of DNA was Statistical analysis of the CGH data was performed sent for processing via OconScanTM FFPE Express 2.0 by FASST2 Segmentation method. In order to adjust service as manufacturer’s instructions. The OncoScan thesensitivityofthesegmentationalgorithm,wede- assay contains more than 300,000 copy number and single termined the significant threshold at 1.0E-8, specified nucleotide polymorphism (SNP) oligonucleotide
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