RCOG World Congress 2023

Background demographics: 30 year old female, BMI 34, G5P2+2 (2 x previous SVD). Booking BMI 34. Routine 1st and 2nd trimester USS as well as additional 33/40 scan for fetal growth did not identify any adnexal pathology.

15th October 2021 - 6:13 Operative vaginal delivery, 9:30 Sinus tachycardia and abdominal pain, 15:15 Tachycardia and abdominal distension, Hb 91, AXR dilated bowel loops

16th October 2021 - 13:30 Hb 68, 3 units RBCs transfused, TA USS intra-abdominal free fluid, CT 17cm haematoma in left pelvis, 23 45 CT angiogram free intra-peritoneal blood and haematoma, no active bleeding

17th October 2021 - 00:15 MDT review, Hb stable, conservative management, 12:00 Febrile, worsening pain, Midline laparotomy and left ovarian cystectomy, intra-operative findings 20cm left pelvic haematoma, haemorrhagic cyst, 3.3 litres haemoperitoneum

Histology confirmed Poorly differentiated Sertoli-Leydig tumour.

•CA125 23 , beta hCG <1, AFP 6, CEA 1, Inhibin B 55

•Underwent laparoscopic left salpingo-oophorectomy with omental biopsies and adjuvant chemotherapy (Carboplatin and Taxol)

•Repeat histology demonstrated no evidence of residual malignancy.

Literature review

Sex cord stromal tumours account for <0.2% of all ovarian neoplasms1. They are characterised by the presence of testicular structures (Sertoli and Leydig cells) that can produce androgens. Not all tumours are functionally active. Tumour grading determines degree of androgenic manifestation.

Clinical presentation - Median age 30, androgenic features; facial hair, clitoromegaly,amenorrhoea, oestrogenic features; menorrhagia, IMB, PMB, non-hormonal features; abdominal pain, distension, torsion

Diagnosis - USS +/- CT ?MRI (complex pelvic mass +/- ascites)

Tumour markers - Testosterone / 17-hydroxycorticosteroid, Oestrogen (from Sertoli cells or peripheral conversion by aromatase cytochrome P450, associated with hyper-oestrogenic pathology e.g, breast cancer, endometrial hyperplasia, endometrial cancer), AFP (foci of intestinal like mucinous epithelium with well-formed glands with occasional goblet cells)

Grading -

•Well differentiated

•Intermediately differentiated

•Poorly differentiated tumours

•Heterogenous elements (cell types foreign to developing gonad e.g., GI epithelium, cartilage, skeletal muscle)

•Retiform pattern

FIGO staging -

I Confined to ovaries / fallopian tubes

II Involving 1 or both ovaries / fallopian tubes with pelvic extension

or primary peritoneal cancer

III Cytologically or histologically confirmed spread to the

peritoneum outside the pelvis or retroperitoneal lymph nodes

IV Distant metastases

Management and prognosis - Surgical – Salpingo-oophorectomy / Total abdominal hysterectomy + bilateral salpingo-oophorectomy, Adjuvant CT - Cisplatin, etoposide, bleomycin, For advanced disease, intermediate and poor tumour differentiation, retiform pattern and presence heterologous elements. FIGO staging most useful prognostic factor. 59% poorly differentiated tumours malignant vs 11% of intermediately differentiated.

Discussion

•The low prevalence of this condition justifies the low clinical suspicion of sex cord stromal tumour in above case.

•An insidious onset of generalised symptoms (e.g pressure) may lead to missed or delayed diagnosis, especially during pregnancy.

•Initial postnatal CT imaging did not identify an ovarian mass in this patient. Would ultrasound have identified an ovarian mass, despite it not being seen antenatally?

•CT angiogram reported no active bleeding (despite ongoing clinical concern and reducing Hb). How sensitive is CT angiogram in the immediate postnatal period?

•Although disease free survival has promising outcomes, little is known about fertility sparing treatment outcomes and future pregnancy success.

RCOG World Congress 2023

Postpartum tachycardia and abdominal distension: A case of poorly differentiated Sertoli-Leydig tumour

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