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.
Highlights
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare tumor of uncertain tissue origin
Contrast-enhanced computed tomography of the abdomen and pelvis showed the presence of a well-defined mass with heterogenous contrast‐enhancement in the left presacral area abutting the left lateral wall of the rectum (Figure 1)
The stromal cells were diffusely positive for CD34, but negative for S100, smooth muscle actin, and MDM2 (Figure 4A,4B)
Summary
A 30-year-old female presented complaining of dysmenorrhea for 1 year. She had normal bladder and bowel habits. A pelvic examination revealed an extra-rectal mass on the right para-rectal space, pushing the uterus towards the left side. AAll India Institute of Medical Sciences, Department of Surgical Oncology. BAll India Institute of Medical Sciences, Department of Pathology. CAll India Institute of Medical Sciences, Department of Anesthesiology. Microscopic examination revealed the presence of a well-encapsulated tumor composed of numerous ecstatic vascular channels with fibrin deposition, myxoid change, and hyaline degeneration (Figure 3). Perl Prussian blue staining demonstrated iron deposits in the lesion (Figure 4D). With these findings, the final diagnosis of PHAT was made
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