Document Detail

Primary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature.
Jump to Full Text
MedLine Citation:
PMID:  24067058     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Angiosarcoma is a rare disease with a poor prognosis; significantly, patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare. This article describes the case of a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which had increased in severity 2 weeks prior to presentation. After a complicated diagnostic and therapeutic process, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was made by pathological and immunohistochemical examinations. We reviewed previous cases of angiosarcoma described in the English literature to determine their risk factors, diagnosis and treatment, and we found that angiosarcoma is extremely rare, especially in the small intestine. To the best of our knowledge, this may be the youngest case of primary angiosarcoma of the small intestine with metastasis to the liver reported in the English literature.
Authors:
Qingqiang Ni; Dong Shang; Honghao Peng; Manish Roy; Guogang Liang; Wei Bi; Xue Gao
Related Documents :
10858328 - The problems of using the transmission/disequilibrium test to infer tight linkage.
15935798 - Exposure to benzene and non-hodgkin lymphoma, an epidemiologic overview and an ongoing ...
20142658 - The use of recombinant activated fvii in postpartum hemorrhage.
16886988 - Risk of aplastic anemia in patients using antiepileptic drugs.
19208668 - Tobacco use among sexual minorities in the usa, 1987 to may 2007: a systematic review.
7722838 - Recurring infantile digital fibromatosis: report of two cases.
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-9-25
Journal Detail:
Title:  World journal of surgical oncology     Volume:  11     ISSN:  1477-7819     ISO Abbreviation:  World J Surg Oncol     Publication Date:  2013 Sep 
Date Detail:
Created Date:  2013-9-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101170544     Medline TA:  World J Surg Oncol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  242     Citation Subset:  -    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): World J Surg Oncol
Journal ID (iso-abbrev): World J Surg Oncol
ISSN: 1477-7819
Publisher: BioMed Central
Article Information
Download PDF
Copyright © 2013 Ni et al.; licensee BioMed Central Ltd.
open-access:
Received Day: 24 Month: 4 Year: 2013
Accepted Day: 3 Month: 9 Year: 2013
collection publication date: Year: 2013
Electronic publication date: Day: 25 Month: 9 Year: 2013
Volume: 11First Page: 242 Last Page: 242
PubMed Id: 24067058
ID: 3851255
Publisher Id: 1477-7819-11-242
DOI: 10.1186/1477-7819-11-242

Primary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature
Qingqiang Ni1 Email: niqingqiang2011@163.com
Dong Shang1 Email: tougao1971@163.com
Honghao Peng12 Email: 277567252@qq.com
Manish Roy13 Email: zonektm@gmail.com
Guogang Liang1 Email: anniu@163.com
Wei Bi1 Email: biwei1767@sina.com
Xue Gao4 Email: xueer128@163.com
1Department of General Surgery, First Affiliated Hospital, Dalian Medical University, Dalian, Liaoning 116011, P.R. China
2Department of General Surgery, Linyi Economic And Technological Development Zone People's Hospital, Linyi, Shandong 276000, P.R. China
3Department of Surgery, Kathmandu Medical College, 184 Baburam Acharya Sadak, Sinamangal, P.O.Box: 21266, Kathmandu, Nepal
4Department of Pathology, First Affiliated Hospital, Dalian Medical University, Dalian, Liaoning 116011, P.R. China

Background

Angiosarcomas, which account for only 1 to 2% of all soft tissue sarcomas, are rare malignant tumors of endothelial origin [1,2]. These tumors are usually found in the scalp and facial skin of elderly individuals, with a male preponderance [3]. Primary angiosarcoma of the small intestine is extremely rare; moreover, due to the difficulty of prompt and accurate diagnosis, its prognosis is very poor [1,4]. To the best of our knowledge, few cases of primary angiosarcoma of the small intestine have been reported in the English literature [5], especially primary angiosarcoma of the small intestine with metastasis to the liver in a young patient.

Here, we report a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which worsened in the last 2 weeks prior to presentation.


Case presentation

A 33-year-old gentleman admitted to our hospital on 5 August 2011 presented with a 4-month history of pain in the left lower quadrant of the abdomen, which had worsened in the last 2 weeks before presentation.

Four months ago, the patient began experiencing abdominal pain in the lower left quadrant without any apparent cause. The symptoms became increasingly aggravated during the last 2 weeks before presentation. The presenting symptoms included paroxysmal colic in the left lower quadrant of the abdomen, fever, nausea and vomiting. The patient also experienced fatigue and an 8 kg weight loss over the last 4 months. In a nearby local hospital, an abdominal plain film was obtained, which showed intestinal tympanites.

For further treatment, the patient came to our outpatient department and was admitted to the Department of Gastroenterology. A physical examination revealed a male patient with tenderness in the left lower abdominal region. There was no hepatomegaly or splenomegaly. Laboratory tests were performed, and the results were normal, including the levels of carcinoembryonic antigen and carbohydrate antigen 19–9. Computed tomography (CT) scans of the whole abdomen showed a significantly thickened intestinal wall located at the end of the jejunum and the proximal ileum, excessive ascites and a few enlarged lymph nodes in the abdomen (Figure 1). Contrast-enhanced CT scans showed different degrees of enhancement in the hepatic arterial phase (Figure 2).

An urgent general surgical consultation was required because of sudden severe aggravation of the abdominal pain. Abdominocentesis was performed, which showed a dark red fluid. The patient presented with peritonitis. Therefore, an emergency laparoscopic surgery was performed, which was later converted to a laparotomy following partial enterectomy. The operative findings included intra-abdominal bleeding of 1500 ml and an oedematous small intestine. Additionally, the greater omentum was found adherent to the small intestinal mesentery in the left upper abdomen with inflammation and blood oozing from the small intestine. During surgical exploration, dozens of irregular nodules of various sizes were found scattered on the surface of the liver. In addition, a mass of approximately 5.0 × 6.0 cm in size was found in the small intestine approximately 70 cm distal to the ligament of Treitz and was accompanied by an endoleak. During the operation, the tumor and the local mesentery of the small intestine were resected. Eight lymph nodes were examined, and two were found to be metastatic. On microscopic examination, vascular invasion of tumor tissue could be observed. The blood vessels of the tumor were abundant with tumor cells around them, and the tumor cells were arranged in a slit-shaped pattern (Figure 3). The immunohistochemistry results showed that the tumor cells were positive for CD31 and vimentin (Figure 4) and negative for CD34, actin, S-100, CD117 and CK56. Moreover, the Ki-67 proliferation index was less than 10% positive. On the basis of these findings, adenocarcinoma, intestinal tuberculosis, neuroendocrine tumor, malignant melanoma, Crohn’s disease, gastrointestinal stromal tumor (GIST) and lymphoma were excluded. Hence, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was confirmed.

After surgical resection, the patient was managed with adjuvant chemotherapy and palliative care. Approximately 400 to 500 ml hemorrhagic fluid was drained during the postoperative period through the abdominal drains. Moreover, the volume of hemorrhagic fluid increased to 600 ml on the 6th postoperative day. Therefore, a cytologic examination was performed, which showed malignant cells, confirming the diagnosis of malignant ascites. The patient received frequent blood transfusions due to a progressive drop in hemoglobin. In the early morning hours of the 27th postoperative day, the patient experienced a sudden disturbance of consciousness along with a progressive decrease in blood pressure. Due to the above-mentioned circumstances, further treatment was refused by the patient’s relatives. The patient died on the morning of the 27th postoperative day.


Discussion

Angiosarcoma, a malignant neoplasm derived from the endothelial cells of blood vessels or lymphatic vessels, is characterized by aggressively proliferating and widely distributed tumor cells [6].

In 1879, Langhans and colleagues reported the first angiosarcoma in the spleen [7]. Thereafter, only a few cases of primary angiosarcoma involving the small intestine have been reported. Gentry and colleagues reviewed 106 vascular tumors of the gastrointestinal tract at the Mayo Clinic between 1925 and 1944, among which 16 angiosarcomas were found; furthermore, only three involved the small intestine [8]. Karpeh and colleagues reviewed 69 adult patients admitted to the Memorial Sloan-Kettering Cancer Center between 1982 and 1990. In this study, 35 angiosarcomas were found, and only 10 involved the viscus or retroperitoneum [9]. Naka and colleagues reviewed 99 Japanese patients with angiosarcoma diagnosed between 1974 and 1990, and no small intestinal angiosarcomas were found [3]. Allison and colleagues reviewed the cases of angiosarcoma that were diagnosed from 1990 to 2002 at the University of Washington and the Cleveland Clinic Foundation, finding only eight cases of angiosarcoma involving the gastrointestinal tract [10] (Table 1).

To gain a deeper understanding of primary angiosarcoma of the small intestine, we searched the PubMed database. The following search terms were used: (“hemangiosarcoma”[MeSH Terms] OR “hemangiosarcoma”[All Fields] OR “angiosarcoma”[All Fields]) AND (“intestine, small”[MeSH Terms] OR “small intestine”[All Fields] OR “small bowel”[All Fields]) AND (English[Language]). Only 54 eligible articles were retrieved. We excluded articles that described secondary cases and articles describing animal angiosarcoma involving the small intestine. Finally, only 27 articles remained [1,5,11-35] (Table 2).

Only one younger case, a 25-year-old woman, was reported as having primary angiosarcoma of the small intestine with normal liver, spleen, rectum, urinary bladder, kidneys, uterus and adnexae [5]. To our knowledge, there are few reports of primary angiosarcoma of the small intestine with metastasis to the liver among such young patients in the English literature.

Intestinal angiosarcoma often presents with gastrointestinal bleeding, abdominal pain, intestinal obstruction, abdominal distention, weight loss, shortness of breath, anemia and weakness [5,10].

Young and colleagues reviewed angiosarcoma with a focus on clinical trials and outlined its risk factors [2]. According to these authors, the risk factors for angiosarcoma were varied and are listed in Figure 5.

Magnetic resonance imaging (MRI), CT, positron emission tomography (PET), X-rays, ultrasound, endoscopy, immunohistochemical testing and a pathological examination can contribute to the diagnosis of angiosarcoma [2,30,31]. MRI, CT, abdominal X-rays and ultrasound are used to delineate the extent of the lesions in the preoperative period of abdominal angiosarcoma [2,31]. CT and PET may be helpful for detecting metastases in the preoperative period [2]. The lesions and the sources of bleeding can be detected by endoscopy. Meanwhile, endoscopic biopsy can be performed; significantly, wireless capsule endoscopy is a new endoscopic method that can help to improve the diagnosis of deep small intestinal pathology [30]. Von Willebrand factor, CD34, CD31, Ulex europaeus agglutinin 1, vascular endothelial growth factor, melanocytic markers (such as S100), human melanoma black-45, melanoma antigen and cytokeratins all are useful for diagnosis and differential diagnosis [2]. Moreover, poorly differentiated adenocarcinoma, intestinal tuberculosis, neuroendocrine tumor, malignant melanoma, Crohn’s disease, GIST, lymphoma and mesothelioma should be excluded, although with difficulty, in differential diagnosis [10,33]. Pathological and immunohistochemical examinations can contribute to the definitive diagnosis of angiosarcoma [21].

Due to the rarity of randomized trials and prospective studies, the management guidelines for other soft tissue sarcomas tend to be utilized when dealing with angiosarcoma [2]. Complete surgical excision tends to be impossible due to the aggressive proliferation and wide metastasis of angiosarcoma [1]. In the current setting, surgical excision associated with adjuvant radiotherapy and/or chemotherapy may be useful; however, the efficacy of these treatments for angiosarcoma remains unclear. Therefore, further studies are desperately needed to determine the optimal treatment for angiosarcoma [1].

Generally speaking, the prognosis of angiosarcoma is very poor. Moreover, several investigators have noted that the site of angiosarcoma tends to affect prognosis [2], and the prognosis of angiosarcoma of the small intestine is significantly worse than that of angiosarcoma at any other site [17]. Indeed, most patients die within a few months of diagnosis, and some die within the postoperative period [17]. Patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare [14].


Conclusion

Primary angiosarcoma of the small intestine is an extremely rare and aggressive soft-tissue malignant tumor. The findings of this case report are of extreme significance. To our knowledge, this may be the first report of primary angiosarcoma of the small intestine with metastasis to the liver in such a young patient. In the future, we should focus on similar cases to ensure early diagnosis and proper treatment.


Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Abbreviations

CT: Computed tomography; GIST: Gastrointestinal stromal tumor; MRI: Magnetic resonance imaging; PET: Positron emission tomography.


Competing interests

The authors declare that they have no competing interests.


Authors’ contributions

QN searched the database, selected the articles, and wrote the manuscript. DS supervised the methodology, the selection of the articles, and the writing of the manuscript and is the corresponding author of the paper. HP assisted in drafting the manuscript. MR supervised the writing of the manuscript. WB, GL, XG and DS performed the surgeries. All the authors have read and approved the final manuscript.


Acknowledgements

We thank Prof. Dong Shang for assisting in the preparation of this manuscript.


References
Al Ali J,Ko HH,Owen D,Steinbrecher UP,Epithelioid angiosarcoma of the small bowelGastrointest EndoscYear: 2006111018102110.1016/j.gie.2006.04.02017140922
Young RJ,Brown NJ,Reed MW,Hughes D,Woll PJ,AngiosarcomaLancet OncolYear: 20101198399110.1016/S1470-2045(10)70023-120537949
Naka N,Ohsawa M,Tomita Y,Kanno H,Uchida A,Aozasa K,Angiosarcoma in Japan. A review of 99 casesCancerYear: 19951198999610.1002/1097-0142(19950215)75:4<989::AID-CNCR2820750414>3.0.CO;2-K7842420
Yoshida K,Ito F,Nakazawa H,Maeda Y,Tomoe H,Aiba M,A case of primary renal angiosarcomaRare TumorsYear: 200911e2821139907
Mohammed A,Aliyu HO,Liman AA,Abdullahi K,Abubakar N,Angiosarcoma of the small intestineAnn Afr MedYear: 20111124624810.4103/1596-3519.8470221912012
Antonescu CR,Targeted therapies in gastrointestinal stromal tumorsSemin Diagn PatholYear: 20081129530310.1053/j.semdp.2008.08.00419013895
Delacruz V,Jorda M,Gomez-Fernandez C,Benedetto P,Ganjei P,Fine-needle aspiration diagnosis of angiosarcoma of the spleen: a case report and review of the literatureArch Pathol Lab MedYear: 2005111054105616048401
Gentry RW,Dockerty MB,Glagett OT,Vascular malformations and vascular tumors of the gastrointestinal tractSurg Gynecol ObstetYear: 19491128132318113323
Karpeh MS Jr,Caldwell C,Gaynor JJ,Hajdu SI,Brennan MF,Vascular soft-tissue sarcomas. An analysis of tumor-related mortalityArch SurgYear: 1991111474148110.1001/archsurg.1991.014103600440081842176
Allison KH,Yoder BJ,Bronner MP,Goldblum JR,Rubin BP,Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic casesAm J Surg PatholYear: 20041129830710.1097/00000478-200403000-0000215104292
Maeyashiki C,Nagata N,Uemura N,Angiosarcoma involving solid organs and the gastrointestinal tract with life-threatening bleedingCase Rep GastroenterolYear: 20121177277710.1159/00034639823341800
Siderits R,Poblete F,Saraiya B,Rimmer C,Hazra A,Aye L,Angiosarcoma of small bowel presenting with obstruction: novel observations on a rare diagnostic entity with unique clinical presentationCase Rep Gastrointest MedYear: 20121148013523198186
Taxy JB,Battifora H,Angiosarcoma of the gastrointestinal tract. A report of three casesCancerYear: 19881121021610.1002/1097-0142(19880701)62:1<210::AID-CNCR2820620132>3.0.CO;2-83383117
Chami TN,Ratner LE,Henneberry J,Smith DP,Hill G,Katz PO,Angiosarcoma of the small intestine: a case report and literature reviewAm J GastroenterolYear: 1994117978008172159
Ordonez NG,del Junco GW,Ayala AG,Ahmed N,Angiosarcoma of the small intestine: an immunoperoxidase studyAm J GastroenterolYear: 1983112182216404159
Hwang TL,Sun CF,Chen MF,Angiosarcoma of the small intestine after radiation therapy: report of a caseJ Formos Med AssocYear: 1993116586617904504
Fraiman G,Ganti AK,Potti A,Mehdi S,Angiosarcoma of the small intestine: a possible role for thalidomide?Med OncolYear: 20031139740210.1385/MO:20:4:39714716038
Selk A,Wehrli B,Taylor BM,Chylous ascites secondary to small-bowel angiosarcomaCan J SurgYear: 20041138338415540697
Berry GJ,Anderson CJ,Pitts WC,Neitzel GF,Weiss LM,Cytology of angiosarcoma in effusionsActa CytolYear: 1991115385421927194
Watanabe K,Hoshi N,Suzuki T,Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivityVirchows Arch A Pathol Anat HistopatholYear: 19931130931410.1007/BF016068968236827
Khalil MF,Thomas A,Aassad A,Rubin M,Taub RN,Epithelioid angiosarcoma of the small intestine after occupational exposure to radiation and polyvinyl chloride: a case report and review of literatureSarcomaYear: 20051116116410.1080/1357714050038906918521426
Suzuki F,Saito A,Ishi K,Koyatsu J,Maruyama T,Suda K,Intra-abdominal angiosarcomatosis after radiotherapyJ Gastroenterol HepatolYear: 19991128929210197502
Cilursu AM,Massive hemorrhage due to angiosarcomatosis diagnosed by intraoperative small bowel endoscopyEndoscYear: 199111245
Delvaux V,Sciot R,Neuville B,Moerman P,Peeters M,Filez L,Van Beckevoort D,Ectors N,Geboes K,Multifocal epithelioid angiosarcoma of the small intestineVirchows ArchYear: 200011909410.1007/s00428000018310963385
Policarpio-Nicolas ML,Nicolas MM,Keh P,Laskin WB,Postradiation angiosarcoma of the small intestine: a case report and review of literatureAnn Diagn PatholYear: 20061130130510.1016/j.anndiagpath.2005.09.00616979525
Hansen SH,Holck S,Flyger H,Tange UB,Radiation-associated angiosarcoma of the small bowel. A case of multiploidy and a fulminant clinical course. Case reportAPMISYear: 19961189189410.1111/j.1699-0463.1996.tb04954.x9048867
Aitola P,Poutiainen A,Nordback I,Small-bowel angiosarcoma after pelvic irradiation: a report of two casesInt J Colorectal DisYear: 19991130831010.1007/s00384005023510663901
Knop FK,Hansen MB,Meisner S,Small-bowel hemangiosarcoma and capsule endoscopyEndoscYear: 200311637
Ogawa S,Minowa O,Ozaki Y,Kuwatsuru R,Sumi Y,Maehara T,Small bowel intussusception caused by intestinal angiosarcomatosis: usefulness of MR enteroclysis with infusion of water through a nasojejunal catheterEur RadiolYear: 20021153453611870466
de Mascarenhas-Saraiva MN,da Silva Araujo Lopes LM,Small-bowel tumors diagnosed by wireless capsule endoscopy: report of five casesEndoscYear: 200311865868
Turan M,Karadayi K,Duman M,Ozer H,Arici S,Yildirir C,Kocak O,Sen M,Small bowel tumors in emergency surgeryUlus Travma Acil Cerrahi DergYear: 20101132733320849049
Liu DS,Smith H,Lee MM,Djeric M,Small intestinal angiosarcoma masquerading as an appendiceal abscessAnn R Coll Surg EnglYear: 201311e22e2410.1308/003588413X1351160995537323317721
Kelemen K,Yu QQ,Howard L,Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literatureArch Pathol Lab MedYear: 200411959814692839
Zacarias Fohrding L,Macher A,Braunstein S,Knoefel WT,Topp SA,Small intestine bleeding due to multifocal angiosarcomaWorld J GastroenterolYear: 2012116494650010.3748/wjg.v18.i44.649423197897
Grewal JS,Daniel AR,Carson EJ,Catanzaro AT,Shehab TM,Tworek JA,Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literatureInt J Colorectal DisYear: 20081174575610.1007/s00384-007-0420-x18080128

Figures

[Figure ID: F1]
Figure 1 

Computed tomography scans of the whole abdomen showed a significantly thickened intestinal wall (yellow arrows) located at the end of the jejunum and the proximal ileum, excessive ascites (white arrows), a few enlarged lymph nodes in the abdomen (red arrow) and the location of the primary angiosarcoma of the small intestine (black arrow).



[Figure ID: F2]
Figure 2 

Enhanced computed tomography scan. (A) A significantly thickened intestinal wall (yellow arrows) located in the end of the jejunum and the proximal ileum, excessive ascites (white arrows) and a few enlarged lymph nodes in the abdomen (red arrow). (B) Different degrees of enhancement were noted in the hepatic arterial phase (blue arrows).



[Figure ID: F3]
Figure 3 

Microscopic examination. (A) Tumour tissue can be observed invading the vascular cavity (hematoxylin and eosin stain; ×100). (B) Tumor blood vessels were abundant, with the tumor cells surrounding them (hematoxylin and eosin stain; ×400). (C) The tumor cells were arranged in a slit-shaped pattern (hematoxylin and eosin stain; ×400).



[Figure ID: F4]
Figure 4 

Immunohistochemistry results. The tumor cells were positive for (A) CD31 (hematoxylin and eosin stain; ×400) and (B) vimentin (hematoxylin and eosin stain; ×200).



[Figure ID: F5]
Figure 5 

Risk factors for angiosarcoma.



Tables
[TableWrap ID: T1] Table 1 

Previous data regarding angiosarcoma and small intestinal angiosarcoma in the study population


Reference Study population Number of patients with angiosarcoma Number of patients with small intestinal angiosarcoma
Gentry et al. [8] 1949
106 patients with vascular tumors of the gastrointestinal tract between 1925 and 1944.
16
3
Karpeh et al. [9] 1991
69 adult patients admitted to the Memorial Sloan-Kettering Cancer Center between 1982 and 1990.
35
≤10
Naka et al. [3] 1995
99 Japanese patients with angiosarcoma between 1974 and 1990.
99
0
Allison et al. [10] 2004 19 previously reported cases of angiosarcoma involving the gastrointestinal tract between 1990 and 2002. 19 8

[TableWrap ID: T2] Table 2 

Cases of primary angiosarcoma involving the small intestine reported in the English literature


Authors Sex/age (years) Primary or secondary Site Immunohistochemical staining History of prior radiation or other predisposing factor Presentation Treatment Follow-up
Maeyashiki et al. [11]
M/72
Indeterminate
Small bowel
Positive for CD31, CD34 and factor VIII
None
Anemia, melaena
Resection and daily blood transfusions
Died on hospital day 103
Siderits et al. [12]
M/79
Primary
Small bowel
Strongly positive for CD31
None
Obstruction
Resection
Unknown
Taxy and Battifora [13]
M/64
Primary
Small bowel
Positive for Factor VIII, collagen type IV and vimentin
Not available
Gastrointestinal bleeding
Resection
Died 1 year after the initial diagnosis
Taxy and Battifora [13]
F/57
Primary
Small bowel
Positive for Factor VIII, collagen type IV
Not available
Not available
Resection
Died shortly after surgery
Chami et al. [14]
M/59
Primary
Small bowel
Weakly positive for factor VIII-related antigen, Ulex europaeus I antigen and cytokeratin
None
Gastrointestinal bleeding, bowel obstruction, anorexiaand weight loss
Resection and transfusions
Died on the 11th day after surgery
Ordonez et al. [15]
M/80
Primary
Small bowel
Positive immunoreaction for FVIII-RAG
None
Anemia, undue tiredness and weakness
Resection
Died on the 20th postoperative day
Hwang et al. [16]
F/60
Primary
Small bowel
Positive for Ulex europaeus agglutinin 1
History of radiotherapy
Diffuse abdominal pain
Resection
Died 2 months after discharge
Mohammed et al. [5]
F/25
Primary
Small bowel
Not available
None
Intermittent abdominal pain, weight loss, abdominal distension, hematemesis and malaena
Resection
Died on the 11th day after surgery
Fraiman et al. [17]
M/85
Primary
Small bowel
Strong positivity for vimentin and CD31; focal positivity for factor VIII and CD34
None
Weight loss, anemia, weakness and abdominal pain
Resection and thalidomide
Not available
Selk et al. [18]
M/57
Primary
Small bowel
Not available
History of radiation therapy
Progressive abdominal distention and shortness of breath
Resection
Died 4 months after surgery
Berry et al. [19]
M/51
Primary
Small bowel
Positive for Ulex europaeus and vimentin
History of 3-year irradiation
Peritonitis
Resection, adriamycin and dacarbazine
Died 5 months after initial presentation
Watanabe et al. [20]
M/64
Primary
Duodenum and upper jejunum
Positive for vimentin and anti-endothelin-1
Not available
Persistent gastrointestinal bleeding
Not available
Died of pulmonary metastasis 1 year after the operation
Al Ali et al. [1]
M/87
Indeterminate
Small bowel
Positive for CD31
None
Lethargy, weakness and anemia
Resection
Died 6 weeks after the initial diagnosis
Khalil et al. [21]
M/68
Primary
Small bowel
Strongly positive for CD31, CD34 and vimentin
30 year history of heavy occupational exposure to radiation and polyvinyl chloride
Gastrointestinal bleeding and melaena
Resection
Died 6 months after initial presentation
Suzuki et al. [22]
F/61
Primary
Ileum
Positive for factor VIII-related antigen and Ulex europaeus agglutinin 1
20 year history of radiotherapy
Abdominal pain
Resection and intra-abdominal cisplatin
Died 1 year after initial presentation
Cilursu [23]
F/74
Indeterminate
Small bowel
Not available
Not available
Melaena
Resection
Not available
Delvaux et al. [24]
M/67
Primary
Small bowel
Positive for CD 31, CD 34, factor VIII-related antigen and keratin
Not available
Weight loss, intermittent severe abdominal pain and melaena
Resection
Died 3 months after diagnosis
Policarpio-Nicolas et al. [25]
F/51
Primary
Small bowel
Positive for CD 31, CD 34 and factor VIII-related antigen
History of irradiation
Abdominal pain
Resection
Died 10 months after laparotomy
Hansen et al. [26]
F/76
Primary
Small bowel
Positive for factor VIII and vimentin
History of irradiation
Watery diarrhea, vomiting, weight loss and abdominal pain
Resection
Died 5 months after operation
Aitola et al. [27]
F/50
Primary
Small bowel
Positive for CD 31, CD 34 and factor VIII-related antigen
≥10 year history of radiotherapy
Intestinal obstruction
Resection followed by combination chemotherapy with doxorubicin
1 year and 9 months after diagnosis, she was alive
Aitola et al. [27]
F/78
Primary
Jejunum
Positive for factor VIII-related antigen, CD31, CD34 and Ulex europaeus
≥10 year history of radiotherapy
Intestinal obstruction
Resection
Died of sepsis 2 years after diagnosis
Knop et al. [28]
M/72
Primary
Small bowel
Not available
Not available
Gastrointestinal bleeding and anemia
Resection
Not available
Ogawa et al. [29]
M/36
Primary
Small bowel
Positive for factor VIII-related antigen
Not available
Abdominal pain and nausea
Surgical treatment
Not available
de Mascarenhas-Saraiva et al. [30]
M/82
Primary
Ileum
Not available
Not available
Melaena and increasing shortness of breath
Surgical treatment and transfusions
Not available
Turan et al. [31]
Not available
Indeterminate
Jejunum
Not available
Not available
Acute abdominal signs
Resection and chemotherapy
Not available
Liu et al. [32]
F/39
Primary
Terminal ileum
Positive for CD31 and CD34
None
Increasing right iliac fossa pain, abdominal bloating and vomiting
Resection and chemotherapy
Not available
Kelemen et al. [33]
M/76
Primary
Small bowel
Positive for CD31
None
Abdominal pain and fatigue
Resection
Died of cardiac arrest on the 9th day after surgery
Fohrding et al. [34]
M/84
Primary
Small bowel
Positive for CD31, cytokeratin and vimentin; slightly weaker for CD34; Focally positive for factor VIII
Not available
Gastrointestinal bleeding
Resection, adjuvant chemotherapy with paclitaxel and transfusion
Not available
Grewal et al. [35]
M/73
Primary
Small bowel
Positive for CD31
None
Gastrointestinal bleeding, weakness and melaena
Resection
Died within 4 months of the diagnosis
M, male; F, female


Article Categories:
  • Case Report

Keywords: Small intestine, Primary angiosarcoma, Hepatic metastasis.

Previous Document:  NSAID is inversely associated with asymptomatic gastric ulcer: Local health examination data from th...
Next Document:  Induced pluripotent stem cell-derived neural stem cells: new hope for stroke?