Late Recurrence of Granulosa Cell Tumour Involving the Retroperitoneum and Spleen

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Late Recurrence of Granulosa Cell Tumour Involving the Retroperitoneum and Spleen Padmapriya Balakrishnan1 • Vijayan Sharmila2 • Thirunavukkarasu Arun Babu2 Received: 18 July 2020 / Revised: 23 August 2020 / Accepted: 18 September 2020 Ó Association of Gynecologic Oncologists of India 2020

Abstract Granulosa cell tumour of the ovary is a sex-cord stromal tumour that accounts for 2–5% of all ovarian neoplasms. It is a low-grade malignant tumour that is often diagnosed in early stages and has a favourable prognosis. The peculiarity of this tumour is its tendency for late recurrence, and the mean time to recurrence is 5–10 years after initial diagnosis. The risk of late recurrence necessitates long-term follow-up of treated patients. We report an unusual case of late recurrence of granulosa cell tumour involving the retroperitoneum and spleen after 20 years of initial treatment. Keywords Granulosa cell tumour  Recurrence  Retroperitoneum

Introduction Ovarian granulosa cell tumours (GCTs) are low-grade malignant tumours arising from sex cord and stromal cells of ovary and represent approximately 2–5% of all ovarian tumours [1]. These are the most common type of ovarian sex-cord stromal tumours and are of two types based on age of onset and histological feature—adult type and juvenile type [2]. Adult granulosa cell tumour (AGCT) is the most common type and accounts for 95% of GCTs. AGCTs occur predominantly in peri-menopausal and postmenopausal age group with a median age at diagnosis of 50–55 years [3]. Conversely, less than 5% of tumours are juvenile granulosa cell tumour (JGCTs) and occur mainly in pre-menarchal girls and women younger than 30 years. Patients usually present with nonspecific symptoms such as & Vijayan Sharmila [email protected] Padmapriya Balakrishnan [email protected] Thirunavukkarasu Arun Babu [email protected] 1

Indira Gandhi Medical College & Research Institute (IGMC&RI), Kathirkamam, Puducherry, India

2

Department of Obstetrics and Gynecology, All India Institute of Medical Sciences (AIIMS), Mangalagiri, Andhra Pradesh 522 503, India

abdominal discomfort, abdominal mass or distension due to enlarging tumour [4]. Patients can also present with symptoms such as precocious puberty, abnormal uterine bleeding and postmenopausal bleeding. This is because granulosa cells convert androstenedione secreted by theca cells into estradiol and excess estradiol can lead to endometrial hyperplasia and carcinoma [3]. These tumours are often unilateral and are diagnosed at FIGO stage I. Most of these patients are cured by surgery, and the longterm disease-free survival following treatment is 85–95%. However, insidious recurrence of the tumour is reported in 20–30% cases, even in early-stage disease [5]. We report a case of 43-year-old women who presented with late recurrence of GCT involving the retroperitoneum and spleen, after 20 years of primary treatment that posed diagnostic and surgical challenges.

Case Report A 43-year-old multiparous woman attended the gynaecol