Case report
Steroid cell tumour not otherwise specified during pregnancy: a case report and diagnostic work-up for virilisation in a pregnant patient

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Abstract

Steroid cell tumours not otherwise specified are rare ovarian tumours, which can cause foetal and maternal virilisation. This is the first case report that describes a steroid cell tumour not otherwise specified during pregnancy. Differential diagnosis, a diagnostic work-up and treatment are discussed.

Introduction

Steroid cell tumours not otherwise specified (NOS) are sex cord-stromal tumours, which account for less than 0.1% of all ovarian tumours [1], [2]. In literature only a few cases of steroid cell tumours NOS have been described. In the following case report we describe the occurrence of a steroid cell tumour NOS during pregnancy.

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Case report

A 37-year-old Caucasian primigravida was referred at 12 weeks of gestation when a solid, homogenous tumour of the left adnex (98mm×69 mm) and a discrete amount of ascites was seen during a routine first trimester screening pelvic ultrasonography (Fig. 1). She reported having progressive hair growth on her face, arms and legs during the last 5 months. She also noticed an abdominal distension during the last few weeks. There was some deepening of her voice. She did not experience any change in

Incidence

The incidence of ovarian tumours during pregnancy ranges from 1: 112 to 1:2200, with an incidence of malignancy between 2.4 and 7.9% in various stages [4], [5]. The wide range of incidence of ovarian tumours is partly due to variation in inclusion of benign tumours. The vast majority of these tumours are dermoid cysts (48%), which are hormonally inactive [5]. Steroid cell tumours account for less than 0.1% of all ovarian tumours. Seventy-five percent of the steroid cell tumours produce

Steroid cell tumour not otherwise specified

Steroid cell tumours are classified into stromal luteoma, Leydig cell tumour and steroid cell tumour NOS, based on their embryonic origin [3]. A stromal luteoma is located within the ovarian stroma and is commonly associated with stromal hyperthecosis. In the cytoplasm of Leydig cell tumours, crystals of Reinke can be seen. When none of these histological criteria are applicable, the diagnosis of a steroid cell tumour NOS is made.

Virilisation is the most common symptom in steroid cell tumours

Treatment

Like other ovarian stromal tumours, steroid cell tumours are managed surgically. Surgery is indicated when a solid, unilateral ovarian enlargement is found, because of a 50% chance of malignancy [6], [8]. Likewise, a cystic mass (>5 cm) that is symptomatic or which does not diminish in size during gestation demands surgical intervention. Preservation of the foetus and fertility are mostly desired. Therefore, the treatment of choice is usually exploratory laparotomy with salpingo-oophorectomy.

References (17)

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Cited by (13)

  • Feto-maternal outcomes of pregnancy complicated by ovarian sex-cord stromal tumor: A systematic review of literature

    2014, European Journal of Obstetrics and Gynecology and Reproductive Biology
    Citation Excerpt :

    Recurrence of ovarian SCST during pregnancy and rapid tumor progression occurred in 8.7% and 2.2% of cases, respectively [10,19,21,37,39]. Fetal serious adverse events included three cases of intrauterine fetal demise (6.5%) [16,19,41] and one case each of stillbirth [38], post-operative fetal demise [16], neonatal death [15], and severe brain damage [42]. To identify risk factors for serious adverse events logistic regression test was performed for collected variables (Table 6).

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