Müllerian Adenosarcoma: A Single-Centre Experience of 59 Cases of This Rare Entity.

Iftikhar Ali Rana,Usman Hassan,Shaarif Bashir,Mehroosh Shakeel,Sajid Mushtaq,Mudassar Hussain

doi:10.7759/cureus.13360

Iftikhar Ali Rana, Usman Hassan + Show 4 more

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https://doi.org/10.7759/cureus.13360

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Abstract

Background and objectiveMüllerian adenosarcomas (MA) are rare biphasic tumors with benign epithelial and sarcomatous stromal components. There is very limited cohort study data on MA in the South Asian countries and no such study has been attempted in Pakistan. Our aim was to evaluate the clinicopathological characteristics of MA and to review the published literature on the condition. Additionally, we also analyzed the impact of various prognostic factors on the overall survival (OS) of patients with MA.Materials and methodsThis was a retrospective observational study performed at the Shaukat Khanum Memorial Hospital and Research Centre, Lahore from 2003 to 2020. A total of 59 histologically confirmed cases of MA were included in the study and critically reviewed.ResultsThe mean age of the patients was 54 ±16 years, and the most common tumor location was the uterine corpus (48, 81.4%), followed by the cervix (eight, 13.6%), ovary (two, 3.4%), and vagina (one, 1.7%). Sarcomatous overgrowth (SO) was seen in 22 (37.3%) patients, and high-grade cytology was observed in 18 (30.5%) patients. Furthermore, lymphovascular invasion (LVI) was present in six (10.2%) patients, and myometrial invasion was noted in 25 (42.4%) patients. The follow-up details of 29 patients were available, and death was recorded in 13 (44.8%) patients with a median OS of three years.ConclusionMA is a rare and diagnostically challenging entity due to its wide differential diagnosis. It is essential to take note of different morphological features such as SO, cytological features, LVI, and heterologous differentiation because of their significant prognostic impact.

Highlights

Müllerian adenosarcoma (MA) is an uncommon biphasic neoplasm characterized by benign epithelial and malignant stromal components [1]
It is believed that MA occurring outside the female genital tract usually arises from preexisting endometriosis [8]
The presence of the benign epithelial component helps in establishing the diagnosis of MA and can exclude malignant mixed Müllerian tumor (MMMT)

Summary

Introduction

Müllerian adenosarcoma (MA) is an uncommon biphasic neoplasm characterized by benign epithelial and malignant stromal components [1]. MA accounts for less than 1% of all female genital tract malignancies and 5% of uterine sarcomas [2,3]. It is most often found in the uterine corpus; cervix and extrauterine locations including the vagina, ovary, fallopian tube, retroperitoneum, and urinary bladder have been described [4,5,6,7]. Other prognostic factors include highgrade cytology, myometrial invasion, tumor necrosis, mitotic rate, initial tumor size, and heterologous elements including rhabdomyoblasts [13]. Müllerian adenosarcomas (MA) are rare biphasic tumors with benign epithelial and sarcomatous stromal components. We analyzed the impact of various prognostic factors on the overall survival (OS) of patients with MA

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