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Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature

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Abstract

Background

Angiosarcoma is a rare high-grade neoplasm that frequently involves the skin and subcutaneous tissue. Rarely, angiosarcoma can occur in the gastrointestinal tract where it frequently exhibits multicentric epithelioid morphology.

Design

We report a case of multicentric epithelioid angiosarcoma (EAS) of the small intestine in a 73-year-old male patient who presented with weakness and melena, and was found to have bleeding lesions in the small intestine on upper gastrointestinal endoscopy. In addition to this case, we extensively reviewed the clinical and pathological features of previously reported cases of angiosarcoma of the small intestine in the English literature since 1970.

Results

Our patient presented with rare and aggressive EAS of the small intestine. Despite surgical resection of the lesions, the patient continued to worsen and developed rapidly progressive metastatic disease. He died within 4 months of the diagnosis.

Conclusions

Angiosarcoma, especially of the deep tissues and the gastrointestinal tract, is very aggressive and rapidly metastatic. The survival rate in these patients is extremely poor, and most patients die within 6 months to 1 year of the diagnosis. Treatment usually involves surgical resection of the bleeding lesions and frequent blood transfusions for symptom alleviation.

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References

  1. Awrich AE, Irish CE, Vetto RM, Fletcher WS (1980) A twenty-five year experience with primary malignant tumors of the small intestine. Surg Gynecol Obstet 151(1):9–14

    PubMed  CAS  Google Scholar 

  2. Barclay TH, Schapira DV (1983) Malignant tumors of the small intestine. Cancer 51(5):878–881

    Article  PubMed  CAS  Google Scholar 

  3. Darling RC, Welch CE (1959) Tumors of the small intestine. N Engl J Med 260(9):397–408

    Article  PubMed  CAS  Google Scholar 

  4. Ebert PA, Zuidema GD (1965) Primary tumors of the small intestine. Arch Surg 91:452–455

    PubMed  CAS  Google Scholar 

  5. Goel IP, Didolkar MS, Elias EG (1976) Primary malignant tumors of the small intestine. Surg Gynecol Obstet 143(5):717–719

    PubMed  CAS  Google Scholar 

  6. Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH (1961) Life history of the carcinoid tumor of the small intestine. Cancer 14:901–912

    Article  PubMed  CAS  Google Scholar 

  7. Waterhouse G, Skudlarick JL, Adkins RB (1981) A clinical review of small bowel neoplasms. South Med J 74(10):1201–1203

    PubMed  CAS  Google Scholar 

  8. Wilson JM, Melvin DB, Gray GF, Thorbjarnarson B (1974) Primary malignancies of the small bowel: a report of 96 cases and review of the literature. Ann Surg 180(2):175–179

    Article  PubMed  CAS  Google Scholar 

  9. Chami TN, Ratner LE, Henneberry J, Smith DP, Hill G, Katz PO (1994) Angiosarcoma of the small intestine: a case report and literature review. Am J Gastroenterol 89(5):797–800

    PubMed  CAS  Google Scholar 

  10. Maddox JC, Evans HL (1981) Angiosarcoma of skin and soft tissue: a study of forty-four cases. Cancer 48(8):1907–1921

    Article  PubMed  CAS  Google Scholar 

  11. Fletcher CD, Beham A, Bekir S, Clarke AM, Marley NJ (1991) Epithelioid angiosarcoma of deep soft tissue: a distinctive tumor readily mistaken for an epithelial neoplasm. Am J Surg Pathol 15(10):915–924

    Article  PubMed  CAS  Google Scholar 

  12. Meis-Kindblom JM, Kindblom LG (1998) Angiosarcoma of soft tissue: a study of 80 cases. Am J Surg Pathol 22(6):683–697

    Article  PubMed  CAS  Google Scholar 

  13. Aitola P, Poutiainen A, Nordback I (1999) Small-bowel angiosarcoma after pelvic irradiation: a report of two cases. Int J Colorectal Dis 14(6):308–310

    Article  PubMed  CAS  Google Scholar 

  14. Chen KT, Hoffman KD, Hendricks EJ (1979) Angiosarcoma following therapeutic irradiation. Cancer 44(6):2044–2048

    Article  PubMed  CAS  Google Scholar 

  15. Hansen SH, Holck S, Flyger H, Tange UB (1996) Radiation-associated angiosarcoma of the small bowel. A case of multiploidy and a fulminant clinical course. Case report. APMIS 104(12):891–894

    Article  PubMed  CAS  Google Scholar 

  16. Hwang TL, Sun CF, Chen MF (1993) Angiosarcoma of the small intestine after radiation therapy: report of a case. J Formos Med Assoc 92(7):658–661

    PubMed  CAS  Google Scholar 

  17. Nanus DM, Kelsen D, Clark DG (1987) Radiation-induced angiosarcoma. Cancer 60(4):777–779

    Article  PubMed  CAS  Google Scholar 

  18. Policarpio-Nicolas ML, Nicolas MM, Keh P, Laskin WB (2006) Postradiation angiosarcoma of the small intestine: a case report and review of literature. Ann Diagn Pathol 10(5):301–305

    Article  PubMed  Google Scholar 

  19. Su CC, Jin YT, Chien CH, Yu CY, Lin PW (1991) Postirradiation angiosarcoma of the terminal ileum. Zhonghua Yi Xue Za Zhi (Taipei) 48(2):147–152

    CAS  Google Scholar 

  20. Suzuki F, Saito A, Ishi K, Koyatsu J, Maruyama T, Suda K (1999) Intra-abdominal angiosarcomatosis after radiotherapy. J Gastroenterol Hepatol 14(3):289–292

    Article  PubMed  CAS  Google Scholar 

  21. Wolov RB, Sato N, Azumi N, Lack EE (1991) Intra-abdominal “angiosarcomatosis” report of two cases after pelvic irradiation. Cancer 67(9):2275–2279

    Article  PubMed  CAS  Google Scholar 

  22. Locker GY, Doroshow JH, Zwelling LA, Chabner BA (1979) The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature. Medicine (Baltimore) 58(1):48–64

    Article  CAS  Google Scholar 

  23. Pereira TC, Vi LH, Prichard JW, Sturgis CD (2001) Pathologic quiz case. Elderly man with bright red blood per rectum. Pathologic diagnosis: metastatic hepatic epithelioid angiosarcoma. Arch Pathol Lab Med 125(7):968–970

    PubMed  CAS  Google Scholar 

  24. Popper H, Thomas LB, Telles NC, Falk H, Selikoff IJ (1978) Development of hepatic angiosarcoma in man induced by vinyl chloride, thorotrast, and arsenic. Comparison with cases of unknown etiology.. Am J Pathol 92(2):349–369

    PubMed  CAS  Google Scholar 

  25. Smith VC, Eisenberg BL, McDonald EC (1985) Primary splenic angiosarcoma. Case report and literature review. Cancer 55(7):1625–1627

    Article  PubMed  CAS  Google Scholar 

  26. Al Ali J, Ko HH, Owen D, Steinbrecher UP (2006) Epithelioid angiosarcoma of the small bowel. Gastrointest Endosc 64(6):1018–1021

    Article  PubMed  Google Scholar 

  27. Allison KH, Yoder BJ, Bronner MP, Goldblum JR, Rubin BP (2004) Angiosarcoma involving the gastrointestinal tract: a series of primary and metastatic cases. Am J Surg Pathol 28(3):298–307

    Article  PubMed  Google Scholar 

  28. Ben-Izhak O, Kerner H, Brenner B, Lichtig C (1992) Angiosarcoma of the colon developing in a capsule of a foreign body. Report of a case with associated hemorrhagic diathesis. Am J Clin Pathol 97(3):416–420

    PubMed  CAS  Google Scholar 

  29. Berry GJ, Anderson CJ, Pitts WC, Neitzel GF, Weiss LM (1991) Cytology of angiosarcoma in effusions. Acta Cytol 35(5):538–542

    PubMed  CAS  Google Scholar 

  30. Butron Vila T, Garcia Villar O, Alonso Garcia S et al (2005) Angiosarcoma in the small intestine. Apropos of a particular case. Hepatogastroenterology 52(64):1139–1142

    PubMed  CAS  Google Scholar 

  31. Cilursu AM (1991) Massive hemorrhage due to angiosarcomatosis diagnosed by intraoperative small bowel endoscopy. Endoscopy 23(4):245

    PubMed  CAS  Google Scholar 

  32. Delvaux V, Sciot R, Neuville B et al (2000) Multifocal epithelioid angiosarcoma of the small intestine. Virchows Arch 437(1):90–94

    Article  PubMed  CAS  Google Scholar 

  33. Fraiman G, Ganti AK, Potti A, Mehdi S (2003) Angiosarcoma of the small intestine: a possible role for thalidomide. Med Oncol 20(4):397–402

    Article  PubMed  Google Scholar 

  34. Kelemen K, Yu QQ, Howard L (2004) Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literature. Arch Pathol Lab Med 128(1):95–98

    PubMed  Google Scholar 

  35. Lo YM, Gillett MB, Vina M, Collin J, Fleming KA (1989) Hemangiosarcoma of the rectum after chronic anorectal ulceration. J Clin Gastroenterol 11(1):77–81

    Article  PubMed  CAS  Google Scholar 

  36. Ordonez NG, del Junco GW, Ayala AG, Ahmed N (1983) Angiosarcoma of the small intestine: an immunoperoxidase study. Am J Gastroenterol 78(4):218–221

    PubMed  CAS  Google Scholar 

  37. Taxy JB, Battifora H (1988) Angiosarcoma of the gastrointestinal tract. A report of three cases. Cancer 62(1):210–216

    Article  PubMed  CAS  Google Scholar 

  38. Usuda H, Naito M (1997) Multicentric angiosarcoma of the gastrointestinal tract. Pathol Int 47(8):553–556

    PubMed  CAS  Google Scholar 

  39. Watanabe K, Hoshi N, Suzuki T, Suzuki T (1993) Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity. Virchows Arch A Pathol Anat Histopathol 423(4):309–314

    Article  PubMed  CAS  Google Scholar 

  40. Vrind HM, Becker AE (1970) Multiple malignant (haem) angioendotheliomas of the stomach and small intestine. Arch Chir Neerl 22(1):15–23

    PubMed  CAS  Google Scholar 

  41. Fineberg S, Rosen PP (1994) Cutaneous angiosarcoma and atypical vascular lesions of the skin and breast after radiation therapy for breast carcinoma. Am J Clin Pathol 102(6):757–763

    PubMed  CAS  Google Scholar 

  42. Goette DK, Detlefs RL (1985) Postirradiation angiosarcoma. J Am Acad Dermatol 12(5 Pt 2):922–926

    Article  PubMed  CAS  Google Scholar 

  43. Falk H, Telles NC, Ishak KG, Thomas LB, Popper H (1979) Epidemiology of thorotrast-induced hepatic angiosarcoma in the United States. Environ Res 18(1):65–73

    Article  PubMed  CAS  Google Scholar 

  44. Telles NC, Thomas LB, Popper H, Ishak KG, Falk H (1979) Evolution of thorotrast-induced hepatic angiosarcomas. Environ Res 18(1):74–87

    Article  PubMed  CAS  Google Scholar 

  45. Byers RJ, McMahon RF, Freemont AJ, Parrott NR, Newstead CS (1994) Angiosarcoma at the site of a ligated arteriovenous fistula in a renal transplant recipient. Nephrol Dial Transplant 9(1):112

    PubMed  CAS  Google Scholar 

  46. Jennings TA, Peterson L, Axiotis CA, Friedlaender GE, Cooke RA, Rosai J (1988) Angiosarcoma associated with foreign body material. A report of three cases. Cancer 62(11):2436–2444

    Article  PubMed  CAS  Google Scholar 

  47. Muller R, Hajdu SI, Brennan MF (1987) Lymphangiosarcoma associated with chronic filarial lymphedema. Cancer 59(1):179–183

    Article  PubMed  CAS  Google Scholar 

  48. Offori TW, Platt CC, Stephens M, Hopkinson GB (1993) Angiosarcoma in congenital hereditary lymphoedema (Milroy's disease)—diagnostic beacons and a review of the literature. Clin Exp Dermatol 18(2):174–177

    Article  PubMed  CAS  Google Scholar 

  49. Schafler K, McKenzie CG, Salm R (1979) Postmastectomy lymphangiosarcoma: a reappraisal of the concept—a critical review and report of an illustrative case. Histopathology 3(2):131–152

    Article  PubMed  CAS  Google Scholar 

  50. Sordillo PP, Chapman R, Hajdu SI, Magill GB, Golbey RB (1981) Lymphangiosarcoma. Cancer 48(7):1674–1679

    Article  PubMed  CAS  Google Scholar 

  51. Reed WB, Kamath M, Weiss L (1974) Kaposi sarcoma, with emphasis on the internal manifestations. Arch Dermatol 110(1):115–118

    Article  PubMed  CAS  Google Scholar 

  52. Gray MH, Rosenberg AE, Dickersin GR, Bhan AK (1990) Cytokeratin expression in epithelioid vascular neoplasms. Hum Pathol 21(2):212–217

    Article  PubMed  CAS  Google Scholar 

  53. Miettinen M, Fetsch JF (2000) Distribution of keratins in normal endothelial cells and a spectrum of vascular tumors: implications in tumor diagnosis. Hum Pathol 31(9):1062–1067

    Article  PubMed  CAS  Google Scholar 

  54. Raina V, Sengar M, Shukla NK et al (2007) Complete response from thalidomide in angiosarcoma after treatment of breast cancer. J Clin Oncol 25(7):900–901

    Article  PubMed  CAS  Google Scholar 

  55. Nagano T, Yamada Y, Ikeda T, Kanki H, Kamo T, Nishigori C (2007) Docetaxel: a therapeutic option in the treatment of cutaneous angiosarcoma: report of 9 patients. Cancer 110:648–651

    Article  PubMed  CAS  Google Scholar 

  56. Veliz M, Chang V, Kasimis B, Choe JK (2007) Durable response of angiosarcoma of the face and scalp to docetaxel. Clin Oncol (R Coll Radiol) 19(3):210

    CAS  Google Scholar 

  57. Inaba T, Yamanaka K, Asahi K et al (2005) Complete remission in a patient with angiosarcoma by the combination of OK-432, rhIL-2, and radiotherapy. Eur J Dermatol 15(5):411–413

    PubMed  Google Scholar 

  58. Miura H, Asada Y (2006) Treatment of eyelid lesion of angiosarcoma with facial artery recombinant interleukin-2 (rIL-2) injection. J Am Acad Dermatol 54(5):907–908

    Article  PubMed  Google Scholar 

  59. Syed SP, Martin AM, Haupt HM, Arenas-Elliot CP, Brooks JJ (2007) Angiostatin receptor annexin II in vascular tumors including angiosarcoma. Hum Pathol 38(3):508–513

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Jaspreet S. Grewal.

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Grewal, J.S., Daniel, A.R.M., Carson, E.J. et al. Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature. Int J Colorectal Dis 23, 745–756 (2008). https://doi.org/10.1007/s00384-007-0420-x

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