Pleomorphic hyalinizing angiectatic tumor of the mesorectum: a rare case and review of literature
Keywords:Pelvic Neoplasms, Vascular Malformation, Soft Tissue Sarcoma, Pelvic neoplasm, Chronic pelvic pain
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.
How to Cite
Copyright and publishing license
Authors retain copyright granting the journal right of first publication with the work simultaneously licensed under the Creative Commons Attribution Non-Commercial License.
Autopsy and Case Reports accepted articles are published under the Creative Commons Attribution Non-Commercial License. Under this License, the authors agree to have the CC-BY-NC license applied to their work, which retains the author's ownership of the copyright for their article and permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original authors and source are properly cited. This facilitates freedom in re-use and also ensures that the journal's content can be mined without barriers for the needs of research.