Autopsy and Case Reports
https://www.autopsyandcasereports.org/article/doi/10.4322/acr.2019.097
Autopsy and Case Reports
Article / Clinical Case Report

Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature

Vigneshwaran Balasubiramaniyan; Dillip Muduly; Swagatika Samal; Madhabananda Kar; Mahesh Sultania; Susama Patra; Prasanta Kumar Das

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Abstract

Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of uncertain tissue origin. Although it has been classified as a benign tumor under the WHO classification, it is locally aggressive, and multiple recurrences have been reported. PHAT commonly involves the lower extremities; however, various unusual sites of origin have been reported.

We present the case of a 30-year-old female with dysmenorrhea, who presented a presacral mass on imaging. The core biopsy confirmed the diagnosis of PHAT. She underwent laparotomy and excision. Histopathology confirmed the presence of a tumor comprised of aggregates of ectatic vessels with perivascular hyalinization. An immunohistochemical study showed diffuse CD34 positivity, but S100, MDM2, and smooth muscle actin negativity. After surgical procedures, the patient is disease free as at the 12-month follow-up.

Only 120 cases have been published in the English literature to date. Our study is only the third case of PHAT arising from the pelvis to be reported. Though considered to be a rare condition, the diagnosis of PHAT should always be considered in the differential diagnosis of well-defined hypervascular soft tissue mass in the pelvis. The typical histopathological findings along with immunohistochemistry should clinch the diagnosis.

Keywords

Pelvic Neoplasms, Vascular Malformation, Soft Tissue Sarcoma, Pelvic neoplasm, Chronic pelvic pain

References

Labanaris AP, Zugor V, Witt JH, Kühn R. Pleomorphic hyalinizing angiectatic tumor of the scrotum. Urol J. 2011;8(3):248-50.21910109

Idrees MT, Kieffer T, Badve S. Pleomorphic hyalinizing angiectatic tumor of renal hilum. Ann Diagn Pathol. 2012;16(6):489-93. 10.1016/j.anndiagpath.2011.03.010.21724433

Vilanova JC. WHO Classification of Soft Tissue Tumors. In: Vanhoenacker F, Parizel P, Gielen J, editors. Imaging of soft tissue tumors. Cham: Springer; 2017. p. 187-96. 10.1007/978-3-319-46679-8_11.

Schepper D, Parizel PM, Vanhoenacker F. Imaging of Soft Tissue Tumors. Cham: Springer; 2006. 10.1007/3-540-30792-3.

Folpe AL, Weiss SW. Pleomorphic hyalinizing angiectatic tumor: Analysis of 41 cases supporting evolution from a distinctive precursor lesion. Am J Surg Pathol. 2004;28(11):1417-25. 10.1097/01.pas.0000141406.02581.fb.15489645

Raghavan V, Shivaprashanth K, Ramesh Rao K. Pleomorphic Hyalinizing Angiectatic Tumor: Immunohistochemical Study with Review of Literature. IJSS Case Reports Rev. 2015;1:46-9.

Iascone C, Sadighi A, Ruperto M, Paliotta A, Borrini F, Mingazzini P. Pleomorphic hyalinizing angiectatic tumour of the mesorectal soft tissue. A case report and review of the literature. Chir Ital. 2008;60(1):159-63.18389762

Suzuki K, Yasuda T, Hori T, et al. Pleomorphic hyalinizing angiectatic tumor arising in the thigh: A case report. Oncol Lett. 2014;7(4):1249-52. 10.3892/ol.2014.1883.24944701

Smith MEF, Fisher C, Weiss SW. Pleomorphic hyalinizing angiectatic tumor of soft parts: A low-grade neoplasm resembling neurilemoma. Am J Surg Pathol. 1996;20(1):21-9. 10.1097/00000478-199601000-00002.8540605

Tallarigo F, Squillaci S, Putrino I, Zizzi N, Bisceglia M. Pleomorphic hyalinizing angiectatic tumor of the male breast : A heretofore unreported occurrence. Pathol Res Path. 2009;205(1):69-73. 10.1016/j.prp.2008.07.010.18789606

Subhawong TK, Subhawong AP, Montgomery EA, Fayad LM. Pleomorphic hyalinizing angiectatic tumor: Imaging findings. Skeletal Radiol. 2012;41(12):1621-6. 10.1007/s00256-012-1443-0.22660836

Chu ZG, Liu MQ, Zhu ZY, Lv FJ, Ouyang Y. Pelvic retroperitoneal Pleomorphic Hyalinizing Angiectatic Tumor (PHAT) of soft tissue: A case report. BMC Med Imaging. 2016;16(1):1-5. 10.1186/s12880-016-0130-3.27044420

 

 

 


Publication date:
07/19/2019

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