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Category: Volume 13 Issue 02 February 2025
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Title: Epithelioid Angiosarcoma of Perineum with Lung and Bone Metastasis; A Case Report and Review of Literature

Authors: Poonam Gautam, S.N. Prasad

 DOI: https://dx.doi.org/10.18535/jmscr/v13i02.02

Abstract

Epithelioid angiosarcoma (EA) is an extremely rare subtype of angiosarcoma, which is characterized by large cells with an epithelioid morphology. Epithelioid angiosarcoma is a rare malignancy of lymphatic or vascular endothelium[1]. That can arises from any part of body like vulva, peritoneum, ovary, uterus, spine, or bones, cutaneous etc. A 47 yrs old man presenting with complain of right inguinal swelling since one year, swelling was progressively increases in size. CEMRI(6/10/2018) Whole abdomen showed Evidence of multiloculated solid focal lesion of size 3.5x3.2 cm at perineum with right predominance with 2.0x2.0 nodular discrete lesion involving upper most right thigh. Initially biopsy was done on 23/1/2018, biopsy was suggestive of round cell soft tissue tumour probably Alveolar rhabdomyosarcoma showed vimentin, CD34, pan-CK, fli-1 positive. IHC confirmed diagnosis of epithelioid angiosarcoma of perineal regions. Contrast enhance computed tomography thorax was suggestive of lung and bony metastasis. This case highlights the difficulty of diagnosing EA, which requires careful pathological examination and immunophenotype labelling. At present, CD31 and CD34 are the most sensitive marker for detecting EA

Keywords- Epithelioid angiosarcoma,immunohistochemistry, perineum, endothelium.

References

  1. Young R.J., Brown N.J., Reed M.W., Hughes D., Woll P.J. Angiosarcoma. Lancet Oncol. 2010;11(10):983–991.
  2. Pawlik TM, Paulino AF, McGinn CJ, Baker LH, Cohen DS, Morris JS, et al. Cutaneous angiosarcoma of the scalp: A multidisciplinary approach. Cancer 2003;98:1716-26.
  3. Barnett CR, Bakr FS, Grossman ME. Cutaneous angiosarcoma with skin metastases and persistent bloody pleural effusions. Cutis 2012;89:129-32
  4. Huang J., Mackillop W.J. Increased risk of soft tissue sarcoma after radiotherapy in women with breast carcinoma. Cancer. 2001;92(1):172–180
  5. Stewart F.W., Treves N. Classics in oncology: lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. CA Cancer J. Clin. 1948;31(5):284–299
  6. Hart and Mandavilli, 2011Arch Pathol Lab Med. 2011 Feb;135(2):268-72. doi: 10.1043/1543-2165-135.2.268.
  7. Hart J, Mandavilli S. Epithelioid angiosarcoma: a brief diagnostic review and differential diagnosis. Arch Pathol Lab Med 2011;135:268–72.
  8. Seo CJ, Lek SM, Tan GHC, et al. Radiation-associated peritoneal angiosarcoma. BMJ Case Rep20172017.
  9. Hart and Mandavilli, 2011Arch Pathol Lab Med. 2011 Feb;135(2):268-72. doi: 10.1043/1543-2165-135.2.268.
  10. Bhardwaj M, Gautam RK, Sharma PK, Kar HK, Magoon N, Sharma A. Angiosarcoma of scalp: A case report. Indian J Pathol Microbiol 2005;48:497-9
  11. Trinh NQ, Rashed I, Hutchens KA, Go A, Melian E, Tung R. Unusual clinical presentation of cutaneous angiosarcoma masquerading as eczema: A case report and review of the literature. Case Rep Dermatol Med 2013;2013:906426

Corresponding Author

Poonam Gautam

Junior Resident J.K. Cancer Institute Kanpur, Uttar Pradesh, India