The Cystic Procreant-Uterine Adenosarcoma

Authors: Bajaj A*

DOI: 10.23880/whsj-16000183

Volume 7, Issue 2

Abstract

Uterine Adenosarcoma is an infrequent, malignant, biphasic, lobulated neoplasm with polyp-like lesions comprised of benign glandular epithelial component admixed with sarcoma component or heterologous elements as skeletal muscle, cartilage, foci of rhabdomyosarcoma or ovarian sex cord-like differentiation. Uterine Adenosarcoma commonly exhibits JAZF1-BCORL1 genetic rearrangement, EP300-BCORL1 or internal BCORL1 genomic rearrangement, inactivating BCORL1 chromosomal mutation and homozygous BCORL1 genomic deletion. A dense proliferation of uniform, spindle shaped cells configuring fascicles, a distinct herringbone pattern or focal areas of leaf-like architecture with intra-glandular polyploidy stromal projections is observed with superimposed benign endometrial glandular epithelium. Subjacent cellular stroma is composed of miniature spherical cells pervaded with moderate, pale cytoplasm and spherical to elliptical, vesicular nuclei with prominent nucleoli. Benign epithelial component is immune reactive to cytokeratin whereas neoplastic mesenchyme component is immune reactive to CD10, WT1, oestrogen receptor (ER), progesterone receptor (PR), androgen receptor (AR), smooth muscle actin (SMA), vimentin or desmin. Uterine Adenosarcoma requires segregation from conditions such as uterine adenofibroma, endometrial stromal sarcoma or uterine Carcinosarcoma. Next generation sequencing (NGS) exhibits amplification of E3 ubiquitin protein ligase (MDM2) and cyclin dependent kinase 4 (CDK4) along with alterations within phosphatidylinositol 4,5-bisphosphate 3 kinase (PIK3CA/AKT/PTEN) pathway. Uterine Adenosarcoma can be appropriately treated with surgical procedures as hysterectomy with bilateral salpingo-oophorectomy. Tumours of advanced grade, vascular invasion, occurrence of heterologous elements, differentiation into rhabdomyosarcomas, appearance of rhabdoid cells and tumour overgrowth within sarcoma component are accompanied by inferior prognostic outcomes.

Keywords: Epithelium; Stroma; Malignant

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