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Prasad AS, Chua SS, Ramani NS, Shiralkar KG, Shanbhogue KP, Surabhi VR. Stroma-derived neoplasms and pseudoneoplastic lesions of the spleen: a select review of pathologic and CT/MRI findings. Abdom Radiol (NY) 2025; 50:480-495. [PMID: 38937338 DOI: 10.1007/s00261-024-04461-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/07/2024] [Revised: 06/09/2024] [Accepted: 06/15/2024] [Indexed: 06/29/2024]
Abstract
A wide spectrum of benign and malignant primary mesenchymal tumors and tumor-like lesions of the spleen has been recently included under the umbrella term 'stroma-derived' neoplasms and tumor-like lesions. These include dendritic cell neoplasms such as follicular dendritic cell sarcoma, EBV-positive inflammatory follicular dendritic cell sarcoma, and fibroblastic reticular cell tumor; smooth muscle and myofibroblastic lesions such as inflammatory pseudotumor, EBV-associated smooth muscle tumor and undifferentiated pleomorphic sarcoma as well as a diverse spectrum of vascular and vascular-stromal tumors and tumor-like lesions. While some tumor and tumor-like lesions are unique to the spleen, others may also occur in diverse extra-splenic viscera. These tumors and tumor-like lesions demonstrate characteristic histopathology, immunocytochemistry and biological behavior. While cross-sectional imaging studies allow detection, staging and limited characterization of these splenic lesions, histopathological confirmation permits optimal management and surveillance strategies.
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Affiliation(s)
- Aditya S Prasad
- Departments of Radiology, University of Texas MD Anderson Cancer Center, Houston, USA
| | | | - Nisha S Ramani
- Department of Pathology, Michael E DeBakey VA Medical Center, Houston, USA
| | | | | | - Venkateswar R Surabhi
- Departments of Radiology, University of Texas MD Anderson Cancer Center, Houston, USA.
- Department of Abdominal Imaging, University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Unit 1473, Houston, TX, 77030, USA.
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Krähling H, Seidensticker M, Heindel WL, Gerwing M. Diagnostic approach to splenic lesions. ROFO-FORTSCHR RONTG 2024; 196:573-581. [PMID: 37967822 DOI: 10.1055/a-2193-2292] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2023]
Abstract
BACKGROUND Splenic lesions are rare and mostly incidental findings on cross-sectional imaging. Most lesions are of benign nature and can be correctly identified based on imaging characteristics. Further, invasive evaluation is only necessary in cases of splenic lesions with uncertain or potentially malignant etiology. METHOD While in most cases a correct diagnosis can be made from computed tomography (CT), (additional) magnetic resonance imaging (MRI) can aid in the identification of lesions. As these lesions are rare, only a few of the differential diagnoses are regularly diagnosed in the clinical routine. RESULT AND CONCLUSION This review presents the differential diagnoses of splenic lesions, including imaging characteristics and a flowchart to determine the right diagnosis. In conjunction with laboratory results and clinical symptoms, histological workup is necessary only in a few cases, especially in incidental findings. In these cases, image-guided biopsies should be preferred over splenectomy, if possible. KEY POINTS · Splenic lesions are rare and are usually incidental findings on abdominal imaging. · CT imaging and MRI imaging are the diagnostic tools of choice for the further workup of splenic lesions. · Based on their image morphological characteristics, a large number of splenic lesions can be assigned to one entity and do not need histological analysis. CITATION FORMAT · Krähling H, Seidensticker M, Heindel WL et al. Diagnostic approach to splenic lesions. Fortschr Röntgenstr 2024; 196: 573 - 581.
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Affiliation(s)
| | - Max Seidensticker
- Department of Radiology, Ludwig-Maximilians-Universität München, Germany
| | | | - Mirjam Gerwing
- Clinic of Radiology, University of Münster, Münster, Germany
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3
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Jiang P, Li X, Chen J, Li D, Han Y. Spontaneous splenic rupture as the initial symptom of splenic angiosarcoma: case report and literature review. Front Oncol 2024; 14:1366554. [PMID: 38756665 PMCID: PMC11096474 DOI: 10.3389/fonc.2024.1366554] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/09/2024] [Accepted: 04/03/2024] [Indexed: 05/18/2024] Open
Abstract
Spontaneous splenic rupture is an extremely rare occurrence, often attributed to tumorous pathologies. Among these, primary splenic angiosarcoma stands as a malignancy arising from the endothelial cells within the spleen. While sporadic cases have been reported globally, there remains a lack of comprehensive consensus on standardized approaches for diagnosis and treatment. We report a case of an 83-year-old male who underwent emergency enhanced CT due to sudden shock, revealing significant intra-abdominal fluid accumulation. Emergency surgery revealed splenic rupture necessitating splenectomy. Histopathological examination confirmed the diagnosis of splenic angiosarcoma. Despite successful surgery, the patient succumbed to severe complications two weeks postoperatively.
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Affiliation(s)
- Peiwu Jiang
- Department of Vascular Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Xiaowen Li
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Jie Chen
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Du Li
- Department of Pathology, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
| | - Yehong Han
- Department of General Surgery, Hangzhou TCM Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China
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4
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Kim N, Auerbach A, Manning MA. Algorithmic Approach to the Splenic Lesion Based on Radiologic-Pathologic Correlation. Radiographics 2022; 42:683-701. [PMID: 35302864 DOI: 10.1148/rg.210071] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Splenic lesions are commonly discovered incidentally at imaging, without clinical signs or symptoms that may aid in diagnosis. As such, the differential diagnosis and subsequent management are based primarily on imaging characteristics. Much has been written about the myriad pathologic conditions that can occur in the spleen; however, there is little guidance on the approach to an incidental splenic mass. Applying an approach frequently used in imaging to the splenic mass-based on the number and consistency of lesions and refined by supplementary imaging features-allows formulation of a useful differential diagnosis. Solitary cystic masses include true cysts, pseudocysts, and parasitic cysts. When multiple cystic lesions are present, the differential diagnosis expands to include infectious lesions (abscess or microabscesses) and lymphangioma (a benign cystic neoplasm). Hemangioma is the most common solitary solid mass, although other vascular lesions (hamartoma, sclerosing angiomatoid nodular transformation) and nonvascular lesions (inflammatory pseudotumor, lymphoma) manifest as solitary and solid. When multiple solid masses are present, diffuse inflammatory disease (sarcoidosis), littoral cell angioma, and lymphoma should be considered. Malignancies, such as angiosarcoma or metastasis, can manifest as solitary or multiple and solid or cystic masses but are typically associated with symptoms or widespread primary malignancy. Careful assessment of the multimodality imaging characteristics of splenic lesions based on this approach aids the radiologist faced with the incidental splenic lesion. Online supplemental material is available for this article. Work of the U.S. Government published under an exclusive license with the RSNA.
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Affiliation(s)
- Nancy Kim
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Aaron Auerbach
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
| | - Maria A Manning
- From the Department of Radiology, MedStar Georgetown University Hospital, 3800 Reservoir Rd NW, CCC Building Ground Floor, CG 201, Washington, DC 20007 (N.K., M.A.M.); the Joint Pathology Center, Silver Spring, Md (A.A.); and the American Institute for Radiologic Pathology, Silver Spring, Md (M.A.M.)
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5
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Bhaludin BN, Thway K, Adejolu M, Renn A, Kelly-Morland C, Fisher C, Jones RL, Messiou C, Moskovic E. Imaging features of primary sites and metastatic patterns of angiosarcoma. Insights Imaging 2021; 12:189. [PMID: 34921641 PMCID: PMC8684573 DOI: 10.1186/s13244-021-01129-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Accepted: 11/14/2021] [Indexed: 12/14/2022] Open
Abstract
Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.
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6
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Ozkale Yavuz O, Ozcan HN, Oguz B, Ayaz E, Ekinci S, Ciftci TT, Haliloglu M. Imaging findings of benign and malignant pediatric splenic lesions. Abdom Radiol (NY) 2021; 46:3245-3252. [PMID: 33661318 DOI: 10.1007/s00261-021-03004-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2021] [Revised: 02/09/2021] [Accepted: 02/13/2021] [Indexed: 02/06/2023]
Abstract
Splenic lesions in children have a wide histological spectrum. The majority of pediatric splenic lesions are benign and detected incidentally, and the most common benign lesions are cysts, followed by hemangiomas and lymphatic malformations. Most of the splenic malignancies in children are secondary to leukemia or lymphoma. The purpose of this article is to describe the ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) features of benign and malignant splenic lesions in the pediatric age group.
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Affiliation(s)
- Ozlem Ozkale Yavuz
- Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey.
- Division of Pediatric Radiology, Department of Radiology, Ankara Bilkent City Hospital, Ankara, Turkey.
| | - Hatice Nursun Ozcan
- Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey
| | - Berna Oguz
- Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey
| | - Ercan Ayaz
- Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey
- Department of Radiology, Diyarbakır Children Hospital, Diyarbakır, Turkey
| | - Saniye Ekinci
- Department of Pediatric Surgery, Hacettepe University School of Medicine, Ankara, Turkey
| | - Turkmen Turan Ciftci
- Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey
| | - Mithat Haliloglu
- Division of Pediatric Radiology, Department of Radiology, Hacettepe University School of Medicine, Sıhhiye, 06230, Ankara, Turkey
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Barat M, Hoeffel C, Aissaoui M, Dohan A, Oudjit A, Dautry R, Paisant A, Malgras B, Cottereau AS, Soyer P. Focal splenic lesions: Imaging spectrum of diseases on CT, MRI and PET/CT. Diagn Interv Imaging 2021; 102:501-513. [PMID: 33965354 DOI: 10.1016/j.diii.2021.03.006] [Citation(s) in RCA: 13] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Revised: 03/29/2021] [Accepted: 03/31/2021] [Indexed: 12/12/2022]
Abstract
The spleen can be affected by a variety of diseases. Some of them are readily identified as variations of normal or benign diseases on imaging. However, for a substantial number of focal splenic abnormalities, the diagnosis can be difficult so that histopathologic analysis may be required for a definite diagnosis. In this review, the typical splenic abnormalities that can be diagnosed with imaging with a high degree of confidence are illustrated. The complementary role of computed tomography (CT), magnetic resonance imaging and positron emission tomography/CT that helps make a diagnostic approach is discussed. Finally, current applications and future trends of radiomics and artificial intelligence for the diagnosis of splenic diseases are addressed.
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Affiliation(s)
- Maxime Barat
- Department of Radiology, Hôpital Cochin, AP-HP, 75014 Paris, France; Université de Paris, 75006 Paris, France.
| | - Christine Hoeffel
- Department of Radiology, Reims University Hospital, 51092 Reims, France; CRESTIC, University of Reims Champagne-Ardenne, 51100 Reims, France
| | | | - Anthony Dohan
- Department of Radiology, Hôpital Cochin, AP-HP, 75014 Paris, France; Université de Paris, 75006 Paris, France
| | - Amar Oudjit
- Department of Radiology, Hôpital Cochin, AP-HP, 75014 Paris, France
| | - Raphael Dautry
- Department of Radiology, Hôpital Cochin, AP-HP, 75014 Paris, France
| | - Anita Paisant
- Department of Radiology, University Hospital of Angers, 49100 Angers, France; Faculté de Médecine, Université d'Angers, 49045 Angers, France
| | - Brice Malgras
- Department of Digestive and Endocrine Surgery, Bégin Army Training hospital, 94160 Saint-Mandé, France; École du Val-de-Grâce, 75005 Paris, France
| | - Anne-Ségolène Cottereau
- Université de Paris, 75006 Paris, France; Department of Nuclear Medicine, Hôpital Cochin, AP-HP, 75014 Paris, France
| | - Philippe Soyer
- Department of Radiology, Hôpital Cochin, AP-HP, 75014 Paris, France; Université de Paris, 75006 Paris, France
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8
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Chatterjee S. Benign Lesions of the Spleen—Are They Really So Benign? A Report of Two Cases. Indian J Surg 2020. [DOI: 10.1007/s12262-020-02125-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022] Open
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9
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Cao L, Hong J, Wang Y, Yu J, Ma R, Li J, Wu J, Zheng S. A primary splenic angiosarcoma hepatic metastasis after splenectomy and its genomic alteration profile. Medicine (Baltimore) 2019; 98:e16245. [PMID: 31305404 PMCID: PMC6641855 DOI: 10.1097/md.0000000000016245] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2019] [Revised: 05/03/2019] [Accepted: 06/07/2019] [Indexed: 02/05/2023] Open
Abstract
RATIONALE Primary splenic angiosarcoma (PSA) is a rare mesenchymal malignancy of the splenic vascular origin often with a dismal prognosis. Genomic profile may provide evidence for the solution of therapy. PATIENT CONCERNS We reported a case of a 51-year-old woman with splenectomy 4 years ago and the postoperative histopathology diagnosis revealed "splenic hemangioma" with spontaneous rupture. Two years after the operation, the patient's rechecked abdominal computed tomography (CT) showed multiple hepatic occupations. DIAGNOSES Pathological test suggested PSA hepatic metastasis. INTERVENTIONS The patient was treated with trans-catheter arterial chemoembolization (TACE) and a pathological diagnosis of PSA was highly suspected in the hepatic biopsy. Four somatic alterations, phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha (PIK3CA), Fos proto-oncogene, AP-1 transcription factor subunit (FOS), MCL1 apoptosis regulator (MCL1), and phosphoinositide-3-kinase regulatory subunit 1 (PIK3R1) were detected in the tumor tissue using a Next generation sequencing (NGS) technology. The results prompted that the patient may get clinical benefit from using some agents for targeted therapy, Everolimus, Temsirolimus, or Copanlisib. OUTCOMES The patient refused targeted therapy. As a result, the patient passed away within 51 months after splenectomy. LESSONS PSA is an aggressive disease that often presented with a high propensity for metastasis and rupture hemorrhage. Some of these mutations were first discovered in PSA and these findings added new contents to the genomic mutation profile of PSA.
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Affiliation(s)
- Linping Cao
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | - Jiawei Hong
- Key Lab of Combined Multi-Organ Transplantation, Ministry of Public Health
| | - Yacong Wang
- Department of Gerontology, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou
| | - Jun Yu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | | | - Jia Li
- OrigiMed, Shanghai, China
| | - Jian Wu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery
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10
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Gaballah AH, Jensen CT, Palmquist S, Pickhardt PJ, Duran A, Broering G, Elsayes KM. Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol 2017; 90:20170039. [PMID: 28471264 DOI: 10.1259/bjr.20170039] [Citation(s) in RCA: 144] [Impact Index Per Article: 18.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Angiosarcoma is a rare, aggressive subtype of soft-tissue sarcoma with a propensity for local recurrence and metastasis associated with a generally poor prognosis, unless diagnosed early. Given the vascular endothelial cell origin of angiosarcoma, tumours may develop in essentially any organ; however, there is a predilection for the skin where half of all tumours arise, increasing in prevalence with age. The most common risk factors are chronic lymphoedema and history of radiation. We review the most important radiological findings along the spectrum of angiosarcoma from head to toe throughout the body, including uncommon and rare locations. Key imaging features of angiosarcoma across multiple organ systems will be described, as well as the impact on management and prognosis.
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Affiliation(s)
- Ayman H Gaballah
- 1 Department of Diagnostic Radiology, University of Missouri-Columbia, Columbia, MO, USA
| | - Corey T Jensen
- 2 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Sarah Palmquist
- 3 Department of Diagnostic Radiology, Baylor College of Medicine, Houston, TX, USA
| | - Perry J Pickhardt
- 4 Department of Radiology, University of Wisconsin-Madison, Madison, WI, USA
| | - Alper Duran
- 2 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
| | - Gregory Broering
- 3 Department of Diagnostic Radiology, Baylor College of Medicine, Houston, TX, USA
| | - Khaled M Elsayes
- 2 Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
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Batouli A, Fairbrother SW, Silverman JF, Muniz MDLA, Taylor KB, Welnick MA, Mancini SA, Hartman MS. Primary Splenic Angiosarcoma: Clinical and Imaging Manifestations of This Rare Aggressive Neoplasm. Curr Probl Diagn Radiol 2016; 45:284-7. [DOI: 10.1067/j.cpradiol.2015.07.004] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/23/2015] [Accepted: 07/13/2015] [Indexed: 01/27/2023]
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12
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Yang KF, Li Y, Wang DL, Yang JW, Wu SY, Xiao WD. Primary splenic angiosarcoma with liver metastasis: A case report and literature review. World J Gastroenterol 2016; 22:3506-3510. [PMID: 27022233 PMCID: PMC4806209 DOI: 10.3748/wjg.v22.i12.3506] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/28/2015] [Revised: 09/28/2015] [Accepted: 10/17/2015] [Indexed: 02/07/2023] Open
Abstract
Primary splenic angiosarcoma (PSA) is an unusual and highly malignant vascular tumour with a high rate of metastatic. Moreover, the research on prognosis of the disease is poor. The epidemiology, etiology, clinical diagnosis and treatment of the disease remain challenging, because case reports of the disease are few in number. In accordance with other malignant tumors, PSA is very aggressive, and the majority of patients in which this disease is found are at an advanced stage. Almost all patients die within 12 mo of diagnosis irrespective of treatment. We report here a woman who had complained of upper bellyache and anorexia for 10 d. Magnetic resonance imaging showed enlargement of the spleen with multiple heterogeneous masses in the lower pole of the spleen. A hand-assisted laparoscopic splenectomy was performed which allowed histopathologic diagnosis. The patient was diagnosed with PSA and liver metastasis, and succumbed to the disease 35 d after surgery. The literature was finished combined with the clinical features, diagnosis and management of PSA.
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13
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Chen F, Jin HF, Fan YH, Cai LJ, Zhang ZY, Lv B. Case report of primary splenic angiosarcoma with hepatic metastases. World J Gastroenterol 2015; 21:11199-11204. [PMID: 26494974 PMCID: PMC4607917 DOI: 10.3748/wjg.v21.i39.11199] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2015] [Revised: 06/29/2015] [Accepted: 08/31/2015] [Indexed: 02/06/2023] Open
Abstract
Primary splenic angiosarcoma (PSA) is the most unusual type of malignancy with early multifocal metastasis through hematogenous spread. PSA is generally believed to originate from splenic sinusoidal vascular endothelium with a high rate of metastasis and to have a poor prognosis. Its etiology and pathogenetic mechanisms have not yet been clearly described. Thus far, only approximately 200 cases have been reported. PSA has variable symptomatology with the potential to present with life-threatening complications. The diagnosis of PSA is challenging; and often late. PSA should be considered in the differential diagnosis of patients with splenomegaly and anemia of unknown etiology. Surgical treatment with splenectomy is considered the only curative intervention for potential long-term disease-free survival. Early diagnosis and treatment are very important. It is important that clinical doctors improve the understanding of PSA. Herein, we report one rare case of PSA with hepatic metastases, along with a review of the current literature.
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14
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Nonneoplastic, benign, and malignant splenic diseases: cross-sectional imaging findings and rare disease entities. AJR Am J Roentgenol 2014; 203:315-22. [PMID: 25055265 DOI: 10.2214/ajr.13.11777] [Citation(s) in RCA: 46] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/31/2022]
Abstract
OBJECTIVE Splenic lesions are commonly encountered and are often incidental in nature. Benign splenic vascular neoplasms include hemangioma, hamartoma, lymphangioma, extra-medullary hematopoiesis (EMH), and sclerosing angiomatoid nodular transformation (SANT). Uncommonly encountered entities of the spleen include focal EMH, focal myeloma, angiomyolipoma, and SANT. Primary splenic angiosarcoma is the most common malignant nonhematolymphoid malignancy of the spleen. Lymphoma, myeloma, and metastases are the other malignant entities involving the spleen. The clinical presentation, key imaging findings, and associations of benign, neoplastic, and malignant diseases that can involve the spleen will be discussed. CONCLUSION Radiologists can use multimodality imaging to diagnose entities involving the spleen by recognizing key imaging features and considering patient characteristics. However, biopsy may be warranted for definitive diagnosis when imaging findings are nonspecific.
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15
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Primary angiosarcoma of the spleen: an oncological enigma. Case Rep Oncol Med 2014; 2014:193036. [PMID: 25105042 PMCID: PMC4101939 DOI: 10.1155/2014/193036] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2014] [Revised: 06/08/2014] [Accepted: 06/15/2014] [Indexed: 02/07/2023] Open
Abstract
Introduction. Primary splenic angiosarcoma is an extremely unusual neoplasm originating from sinusoidal vascular endothelium. Surgical extirpation is the mainstay of treatment of this highly malignant disease. Case Presentation. An 82-year-old woman was admitted with left pleural effusion and a palpable left upper quadrant abdominal mass, secondary to splenomegaly by two large splenic tumors. Classic open splenectomy was performed and angiosarcoma of the spleen was the final histopathological diagnosis, which was primary since no other disease site was revealed. Discussion. The incidence of the disease is 0.14-0.23 cases per million, with slight male predominance. Etiology is not established and clinical presentation may confuse even experienced physicians. Imaging modalities cannot differentiate the lesion from other vascular splenic neoplasms and the correct diagnosis is mainly set after histopathological examination of the resected spleen. As with other sarcomas, surgery is the only curative approach, while chemo- and radiotherapy have poor results. Prognosis remains dismal.
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16
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Duan YF, Jiang Y, Wu CX, Zhu F. Spontaneous rupture of primary splenic angiosarcoma: a case report and literature review. World J Surg Oncol 2013; 11:53. [PMID: 23497454 PMCID: PMC3599178 DOI: 10.1186/1477-7819-11-53] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/05/2012] [Accepted: 02/16/2013] [Indexed: 02/08/2023] Open
Abstract
BACKGROUND Primary angiosarcoma of the spleen is a rare mesenchymal malignant tumor of vascular origin often with a poor prognosis, due to its high metastatic potential. This disease often presents with atraumatic rupture and lethal hemorrhage. CASE PRESENTATION We report a case of a 65-year-old man who presented with abdominal pain, anemia, thrombocytopenia, and palpable abdominal mass with unstable blood pressure. Laparotomy revealed a huge actively bleeding spleen, thus splenectomy was performed. Some liver metastasis foci were also found during the procedure. Histopathology diagnosis of the removed spleen was primary splenic angiosarcoma. The patient was discharged on the 10th day post operation with no complication. CONCLUSIONS Splenic angiosarcoma should be considered one of the differential diagnoses in patients with spleen parenchymal lesions. Definitive diagnosis requires laparotomy followed by splenectomy. In the majority of the patients with spleen angiosarcoma, metastatic diseases have already occurred at the time of laparotomy, so splenectomy is an approach more for diagnostic purpose rather than curative purpose.
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Affiliation(s)
- Yun-Fei Duan
- Department of Hepatobiliary Surgery, The Third Affiliated Hospital of Soochow University, 185 Juqian Street, 213000, Changzhou, Jiangsu, China
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17
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Qi R, Yu JQ, Xu H, Zhou XP, Li XM. Primary angiosarcoma of the spleen as depicted on computed tomography. Clin Imaging 2012; 36:619-22. [PMID: 22920376 DOI: 10.1016/j.clinimag.2011.12.016] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2011] [Accepted: 12/14/2011] [Indexed: 02/05/2023]
Abstract
Primary angiosarcoma of the spleen is rare, usually widespread or has a splenic rupture by the time of presentation, and almost always has poor prognosis; early radiological diagnosis is necessary for improving the survival rate. We report two special cases of this disease. Both patients did not have pathologic metastasis or splenic rupture, but their computed tomography (CT) manifestations were distinct from each other and had uncommon characteristics. We compared the CT features with pathological findings.
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Affiliation(s)
- Rui Qi
- Department of Radiology, West China Hospital, Sichuan University, 37# Guo Xue Xiang, Chengdu, Sichuan 610041, China
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18
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Qiu LL, Yu RS, Chen Y, Zhang Q. Sarcomas of abdominal organs: computed tomography and magnetic resonance imaging findings. Semin Ultrasound CT MR 2012; 32:405-21. [PMID: 21963162 DOI: 10.1053/j.sult.2011.04.003] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Sarcomas originating from abdominal organs are extremely rare, and literature in which investigators focus on their imaging features is scarce. This article aims to review the computed tomography and magnetic resonance imaging features of these rare tumors and to help in clinical diagnosis. Various sarcomas (eg, angiosarcoma, undifferentiated embryonal sarcoma, leiomyosarcoma, carcinosarcoma, rhabdomyosarcoma) originating, respectively, from different abdominal visceral organs (liver, pancreas, spleen, kidney, adrenal gland, uterus, and prostate gland) are reviewed along with cases we encountered.
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Affiliation(s)
- Ling-Ling Qiu
- Department of Radiology, Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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19
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Mohr Z, Klippel S, Spiethoff A, Trick D, Willis S. [Laparoscopic splenectomy for sclerosing angiomatoid nodular transformation]. Chirurg 2012; 82:714-8. [PMID: 21290093 DOI: 10.1007/s00104-010-2045-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
Abstract
Sclerosing angiomatoid nodular transformation (SANT) is a benign lesion of the spleen which can be cured by splenectomy. In the literature about 45 cases have been reviewed. Although it is defined by the morphological details, data regarding surgical therapy are scarce. To the best of our knowledge, a laparoscopic approach has not been published before. We investigated in one case of SANT the feasibility of a laparoscopic approach. Histological investigations confirmed the diagnosis of a SANT which was resected in toto. This report shows that the laparoscopic splenectomy is a feasible, safe and effective method for treatment of SANT.
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Affiliation(s)
- Z Mohr
- Chirurgische Klinik A, Allgemein-, Viszeral-, Thorax- und Unfallchirurgie, Klinikum der Stadt Ludwigshafen, Bremserstrasse 79, Ludwigshafen, Germany.
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20
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Hadidy A, Alsharif A, Sheikh-Ali R, Abukhalaf M, Awidi A, Abukaraki A, Nimri C, Omari A. Odontogenic myxofibroma synchronous with primary angiosarcoma of the spleen. Br J Radiol 2010; 83:e10-3. [PMID: 20139242 DOI: 10.1259/bjr/14078580] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
Abstract
Odontogenic myxofibroma of the temporomandibular joint (TMJ) is a rare tumour; moreover, primary splenic angiosarcoma (PAS) in paediatric patients is extremely rare. We report on a 15-year-old boy who presented with right TMJ swelling and subsequently developed epigastric and right upper quadrant pain. The TMJ swelling proved to be odontogenic myxofibroma and the abdominal pain was a result of primary splenic angiosarcoma with hepatic metastasis. We report for the first time the synchronous presentation of PAS and odontogenic myxofibroma in a paediatric patient, and we describe the radiological features along with the histological diagnosis and clinical outcome. Uptake in (18)F-2-fluoro-2-deoxy-D-glucose positron emission tomography is also described for the first time for both these tumours.
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Affiliation(s)
- A Hadidy
- Department of Radiology and Nuclear Medicine, Jordan University Hospital, Amman, Jordan.
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21
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22
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Koutelidakis IM, Tsiaousis PZ, Papaziogas BT, Patsas AG, Atmatzidis SK, Atmatzidis KS. Spleen rupture due to primary angiosarcoma: a case report. J Gastrointest Cancer 2008; 38:74-7. [PMID: 19016352 DOI: 10.1007/s12029-008-9034-y] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/07/2008] [Accepted: 07/01/2008] [Indexed: 11/26/2022]
Abstract
A case of a 79-year-old female with rupture of the spleen due to primary angiosarcoma is presented. Symptoms were non-specific. Diagnosis was based on histology postoperatively. Primary angiosarcoma of the spleen is a very rare and aggressive neoplasm with a high metastatic rate and almost uniformly fatal. Due to small number of reported cases, there are no guidelines concerning adjuvant or palliative treatment or any beneficial protocols of chemotherapy or radiotherapy up to date. Splenectomy prior to rupture seems to have a positive impact on long-term survival.
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Affiliation(s)
- Ioannis M Koutelidakis
- 2nd Department of Surgery, Aristoteles University of Thessaloniki, G. Gennimatas General Hospital, Thessaloniki, Greece
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23
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Elsayes KM, Narra VR, Mukundan G, Lewis JS, Menias CO, Heiken JP. MR imaging of the spleen: spectrum of abnormalities. Radiographics 2006; 25:967-82. [PMID: 16009818 DOI: 10.1148/rg.254045154] [Citation(s) in RCA: 192] [Impact Index Per Article: 10.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/26/2022]
Abstract
The spleen has the same relationship to the circulatory system that the lymph nodes have to the lymphatic system. A wide range of diseases can affect the spleen. Pathologic conditions of the spleen can be classified into the following categories: congenital diseases (accessory spleen, polysplenia, and asplenia); trauma; inflammation (abscess, candidiasis, histoplasmosis, and sarcoidosis); vascular disorders (infarction, diseases affecting the splenic vasculature, and arteriovenous malformation); hematologic disorders (sickle cell disease and extramedullary hematopoiesis); benign tumors (cysts, hemangioma, diffuse hemangiomatosis of the spleen, and hamartoma); malignant tumors (sarcoma, lymphoma, and metastases); and other disease processes that affect the spleen diffusely (portal hypertension, Gaucher disease, and sickle cell disease) or focally (Gamna-Gandy nodules). New magnetic resonance (MR) imaging techniques have increased the role of MR imaging in detection and characterization of splenic diseases. MR imaging is an excellent tool for diagnosis and evaluation of focal lesions and pathologic conditions of the spleen.
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Affiliation(s)
- Khaled M Elsayes
- Mallinckrodt Institute of Radiology, Washington University School of Medicine, 510 S Kingshighway Blvd, St Louis, MO 63110, USA.
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24
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Verge J, Albiol J, Navas M, Martín C. [Primary angiosarcoma of the spleen with liver metastases. Case report and literature review]. Cir Esp 2006; 78:50-2. [PMID: 16420791 DOI: 10.1016/s0009-739x(05)70884-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022]
Abstract
Angiosarcomas of the spleen are infrequent tumors that often present with liver metastases. These tumors are usually multiple and have a poor prognosis. Immunohistochemical markers can aid preoperative diagnosis since radiological and ultrasonographic images are fairly nonspecific. The recommended treatment is splenectomy. The benefits of chemotherapy and radiotherapy have not been clearly demonstrated.
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Affiliation(s)
- J Verge
- Fundació Hospital-Residència Sant Camil, Sant Pere de Ribes, Barcelona, Spain.
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25
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Hsu JT, Chen HM, Lin CY, Yeh CN, Hwang TL, Jan YY, Chen MF. Primary angiosarcoma of the spleen. J Surg Oncol 2005; 92:312-316. [PMID: 16299797 DOI: 10.1002/jso.20419] [Citation(s) in RCA: 39] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
BACKGROUND AND OBJECTIVES Primary splenic angiosarcoma is a very rare and aggressive neoplasm with a high metastatic rate and dismal prognosis. Since only a few cases have been adequately reported in the medical literature, we report here a further six cases. METHODS The records of all cases of primary splenic angiosarcoma treated at Chang Gung Memorial Hospital from April 1991 to July 2004 were retrospectively reviewed. RESULTS Of the six cases identified (three men and three women; range, 7-69 years; median, 44 years), four presented with palpable abdominal masses or left upper quadrant abdominal pain. Other systemic symptoms, such as bleeding gums, fatigue, fever, body weight loss, and gastrointestinal bleeding were noted. An abnormal hematogram was found in five patients, with all of these five showing anemia, and three thrombocytopenia. Splenomegaly was present in all patients. Hemoperitoneum due to splenic rupture was noted in one patient. Three patients had distant metastasis to the liver (n = 2), bone (n = 1), bone marrow (n = 1), and small bowel (n = 1) at diagnosis. Liver (n = 3), bone (n = 1), and bone marrow (n = 1) metastases were found in four patients after initial therapy. Five of the six cases underwent a splenectomy, one underwent partial hepatectomy, and one received chemotherapy. The respective 1-, 3- and 5-year survival rates were 60%, 40%, and 40%, and the median survival time was 36 months. One patient was disease-free 162 months after splenectomy. CONCLUSIONS The clinical presentations of splenic angiosarcoma were similar to those of previous reports apart from the higher rate of splenomegaly observed in this study. In contrast to reported pediatric cases, our patient achieved long-term disease-free survival after splenectomy alone.
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Affiliation(s)
- Jun-Te Hsu
- Department of General Surgery, Chang Gung Memorial Hospital, Taoyuan, Taiwan
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26
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Segatto E, Mortele KJ, Ros PR. Spleen. RADIOLOGIC-PATHOLOGIC CORRELATIONS FROM HEAD TO TOE 2005:475-507. [DOI: 10.1007/3-540-26664-x_21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Thompson WM, Levy AD, Aguilera NS, Gorospe L, Abbott RM. Angiosarcoma of the spleen: imaging characteristics in 12 patients. Radiology 2005; 235:106-15. [PMID: 15749977 DOI: 10.1148/radiol.2351040308] [Citation(s) in RCA: 71] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
PURPOSE To retrospectively review clinical, pathologic, and imaging features of angiosarcoma of the spleen in 12 patients. MATERIALS AND METHODS 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. RESULTS 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. CONCLUSION 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.
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Affiliation(s)
- William M Thompson
- Department of Radiology, Duke University Medical Center, Erwin Road, Box 3808, Durham, NC 27710, USA.
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Abstract
Splenic tumours are occasionally found during routine physical check-ups or elective abdominal image studies. Histologically, most splenic tumours are of benign vascular origin. To avoid unnecessary surgery for asymptomatic patients with benign splenic tumours and clarify the clinicopathological features of spleen tumours, this study gathered 44 cases of primary or isolated metastatic spleen tumours confirmed by pathology from surgery specimens or biopsies. The differences in clinicopathological features and image presentations between benign and malignant spleen tumour were investigated. Thirty-two cases involved benign tumours while 12 cases were malignant. Among the benign tumours, vascular originating tumours were most common (with 14 cases of cavernous haemangiomas, 13 cases of lymphangioma, three cases of lymphangiohaemangioma and one case of Littoral cell angioma). Notably, one, case of inflammatory pseudotumour because of Schistosoma parasite infection was also noted. Among the malignant tumours, there were four cases of angiosarcomas with vascular endothelium origins, as well as lymphomas and six metastatic tumours. Image studies were non-specific. Image study alone is an inadequate basis for making differential diagnoses between benign and malignant tumours. Instead, pathological studies are required for a final diagnosis. Using previous studies and this investigation, fine needle aspiration biopsy of spleen tumours with the help of ultrasonic or computed tomography appears a safe and effective method for obtaining biopsy specimens. Splenectomy is recommended only for patients with malignancies or complications such as intractable abdominal pain, coagulopathy or tumour rupture with an unstable haemodynamic state.
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Affiliation(s)
- L W Chen
- Department of Internal Medicine, Division of Gastroenterology, Chang-Gung Memorial Hospital, Keelung, Taiwan
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29
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Clifford CA, Pretorius ES, Weisse C, Sorenmo KU, Drobatz KJ, Siegelman ES, Solomon JA. Magnetic Resonance Imaging of Focal Splenic and Hepatic Lesions in the Dog. J Vet Intern Med 2004. [DOI: 10.1111/j.1939-1676.2004.tb02554.x] [Citation(s) in RCA: 60] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
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Abstract
Splenic vascular tumors are uncommon and are more typically encountered as benign incidental findings. By contrast, splenic angiosarcoma may present acutely and dramatically and typically pursues a very aggressive clinical course. Vascular tumors in the spleen may show conventional endothelial, specialized endothelial (sinusoidal/littoral cell) or lymphatic differentiation and there is morphologic overlap between some of the currently defined diagnostic categories, within which benign, intermediate, and malignant subsets are recognized. The greatest problem in trying to better define and analyze these tumors is the availability of only relatively small case numbers. This overview describes the diagnosis and differential diagnosis of splenic vascular tumors as presently understood.
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Affiliation(s)
- Jeffery L Kutok
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA
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31
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Karakas HM, Demir M, Ozyilmaz F, Cakir B. Primary angiosarcoma of the spleen: in vivo and in vitro MRI findings. Clin Imaging 2001; 25:192-6. [PMID: 11679227 DOI: 10.1016/s0899-7071(01)00286-8] [Citation(s) in RCA: 23] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Primary splenic angiosarcomas are extremely rare tumors with very poor prognosis. These tumors, highly aggressive and lethal, present with widespread metastatic disease or splenic ruptures. The possibility to lengthen the survival by splenectomy before the development of splenic rupture makes an early radiological diagnosis a necessity. Multimodality imaging findings of a patient with splenic angiosarcoma is presented. In vivo and in vitro magnetic resonance imaging (MRI) features of the spleen are compared to macro- and micropathological findings.
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Affiliation(s)
- H M Karakas
- Department of Radiodiagnostics, Trakya University Medical School, 22030 Edirne, Turkey.
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