A rare tumor – adrenal angiosarcoma (case report)

Background.Primary adrenal angiosarcoma is an exceptionally rare tumor with an incidence of <1% of soft tissue sarcomas. Less than 50 cases being described in literature; most of them were nonfunctional presenting with symptoms of abdominal lump, weight loss, fatigue or weakness. The treatment remains multi-modal with involvement of surgeon as well as medical oncologist. Objective. The aim of this case report is to describe an entity which is rare in terms of incidence. It was functional as well as metastatic and managed with multimodality therapy. Methods. The case is a 47-year-old man diagnosed with metastatic adrenal angiosarcoma. The patient was managed with chemotherapy followed by surgery. Results. The final histopathological examination of the specimen and immunohistochemistry revealed angiosarcoma. At present the patient is under follow-up. Conclusion. Even though surgery forms the main stay of management, the role of multi-modality therapy forms the cornerstone for adrenal angiosarcomas

forms the cornerstone for adrenal angiosarcomas.

 angiosarcoma

Primary adrPrimary adrenal angiosarcoma is an exceptionally rare tumor with an incidence of <1% of soft tissue sarcomas. Less than 50 cases being described in literature; most of them were nonfunctional presenting with symptoms of abdominal lump, weight loss, fatigue or weakness. The treatment remains multi-modal with involvement of surgeon as well as medical oncologist.enal angiosarcoma is an exceptionally rare tumor with an incidence of <1% of soft tissue sarcomas. Less than 50 cases being described in literature; most of them were nonfunctional presenting with symptoms of abdominal lump, weight loss, fatigue or weakness. The treatment remains multi-modal with involvement of surgeon as well as medical oncologist

adrenal angiosarcoma; suprarenal; adrenalectomy; vasoformative tumor

https://doi.org/10.11603/ijmmr.2413-6077.2022.2.13221

[1] Weiss SW, Goldblum JR, Folpe AL. Enzinger and Weiss’s Soft Tissue Tumors. Elsevier Health Sciences; 2007. 4348 p.

[2] Li XM, Yang H, Reng J, Zhou P, Cheng ZZ, Li Z, et al. A case report of primary adrenal angiosarcoma as  depicted  on  magnetic  resonance  imaging.  Medicine (Baltimore). 2017 Nov 10;96(45):e8551.https://doi.org/10.1097/MD.0000000000008551.

[3] Grajales-Cruz  A,  Baco-Viera  F,  Rivé-Mora  E,  Ramírez-Tanchez C, Tasso D, Arroyo-Portela N, et al. Primary Adrenal Angiosarcoma: A Rare and Potentially  Misdiagnosed  Tumor.  Cancer  Control.  2017 Apr;24(2):198­201.https://doi.org/10.1177/107327481702400213.

[4] Stavridis S, Mickovski A, Filipovski V, Sasho B, Sasho D, Ljupcho L. Epithelioid Angiosarcoma of the Adrenal Gland. Report of a Case and Review of the Literature. Maced J Med Sci. 2010 Dec 15;3.

[5] Antao N, Ogawa M, Ahmed Z, Piao J, PoddarN.Adrenal Angiosarcoma: A Diagnostic Dilemma. Cureus. 11(8):e5370.

[6] Kareti  LR,  Katlein  S,  Siew  S,  Blauvelt  A.  Angiosarcoma of the adrenal gland. Arch Pathol Lab Med. 1988 Nov;112(11):1163­5.

[7] Fuletra JG, Ristau BT, Milestone B, Cooper HS, Browne  A,  Movva  S,  et  al.  Angiosarcoma  of  the  Adrenal Gland Treated Using a Multimodal Approach. Urol Case Rep. 2016 Dec 1;10:38­41.https://doi.org/10.1016/j.eucr.2016.11.003.

[8] Livaditou  A,  Alexiou  G,  Floros  D,  Filippidis  T,  Dosios  T,  Bays  D.  Epithelioid  Angiosarcoma  of  the  Adrenal  Gland  Associated  with  Chronic  Arsenical Intoxication? Pathol ­Res Pract. 1991 Mar 1;187(2): 284-9. https://doi.org/10.1016/S0344­0338(11)80785­5.

[9] Kumar  A,  Popli  V,  roy  N,  Jafri  H,  Ponnam  A.  Giant adrenal endothelial Cyst: A case report. Med Sci Int Med J. 2022 Aug 21;11.https://doi.org/10.5455/medscience.2022.04.102.

[10] Sung  JY,  Ahn  S,  Kim  SJ,  Park  YS,  Choi  YL.  Angiosarcoma arising within a long-standing cystic lesion of the adrenal gland: a case report. J Clin Oncol Off J Am Soc Clin Oncol. 2013 Mar 20;31(9):e132­6.https://doi.org/10.1200/JCO.2012.44.0800.

[11] Rodriguez-Pinilla SM, Benito-Berlinches AB, Ballestin C, et al. Angiosarcoma of adrenal gland: report of a case and review of the literature. Rev Esp Patol. 2002;35(2):227­32.

[12] Criscuolo M, Valerio J, Gianicolo ME, et al. A vinyl chloride-exposed worker with an adrenal gland angiosarcoma: a case report. Ind Health. 2014; 52(1):66­70. https://doi.org/10.2486/indhealth.2013­0044.

[13] Hendry S, Forrest C. Epithelioid angiosarcoma arising in an adrenal cortical adenoma: a case report and  review  of  the  literature.  Int  J  Surg  Pathol.  2014;22(8):744­8. https://doi.org/10.1177/1066896914532541.

[14] National  Institutes  of  Health.  NIH  state-of-the-science statement on management of the clini-cally inapparent adrenal mass (“incidentaloma”). NIH Consens State Sci Statements. 2002;19(2):1­25.