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

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

Journal

Autopsy & case reports
ISSN: 2236-1960
Titre abrégé: Autops Case Rep
Pays: Brazil
ID NLM: 101640070

Informations de publication

Date de publication:
Historique:
received: 04 04 2019
accepted: 25 05 2019
entrez: 24 8 2019
pubmed: 24 8 2019
medline: 24 8 2019
Statut: epublish

Résumé

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.

Identifiants

pubmed: 31440480
doi: 10.4322/acr.2019.097
pii: autopsy-09-03e2019097-g
pmc: PMC6655857
doi:

Types de publication

Case Reports

Langues

eng

Pagination

e2019097

Déclaration de conflit d'intérêts

Conflict of interest: None

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Auteurs

Vigneshwaran Balasubiramaniyan (V)

All India Institute of Medical Sciences, Department of Surgical Oncology. Bhubaneswar, Orissa, India.

Dillip Muduly (D)

All India Institute of Medical Sciences, Department of Surgical Oncology. Bhubaneswar, Orissa, India.

Swagatika Samal (S)

All India Institute of Medical Sciences, Department of Pathology. Bhubaneswar, Orissa, India.

Madhabananda Kar (M)

All India Institute of Medical Sciences, Department of Surgical Oncology. Bhubaneswar, Orissa, India.

Mahesh Sultania (M)

All India Institute of Medical Sciences, Department of Surgical Oncology. Bhubaneswar, Orissa, India.

Susama Patra (S)

All India Institute of Medical Sciences, Department of Pathology. Bhubaneswar, Orissa, India.

Prasanta Kumar Das (PK)

All India Institute of Medical Sciences, Department of Anesthesiology. Bhubaneswar, Orissa, India.

Classifications MeSH