Ovarian tumours, cysts and abscesses
Key points
Ovarian cysts
Follicular Cysts
Form before ovulation
Result from hormonal stimulation causing an immature follicle to expand and mature due to the accumulation of serous fluid in the follicular antrum
If ovulation does not occur, the follicle transforms into a follicular cyst
Typically unilateral and unilocular
The cyst wall is lined by luteinised or granulosa cells
Corpus Luteum Cysts
Develop post-ovulation from the ruptured follicle
Fill with simple fluid or blood, causing them to enlarge
Can be bilateral, larger, and more complex than follicular cysts
Lined by luteinised granulosa and theca cells
Secrete oestrogen and progesterone
Simple cyst definition: Unilocular (no septations) no solid component
Simple cysts up to 1cm in prepubertal girs, and up to 3cm in post pubertal girls are normal
BritSPAG guidelines
For girls that have achieved menarche:
Simple cysts:
<5cm: Discharge
5-7cm: Yearly USS
>7cm: MRI or surgery
Any size complex cyst: tumour markers + further imaging
Hypothyroidism - TSH overlap may induce large cystic ovaries
Functional cysts in adolescents - can treat with oral contraceptive pill
Neonatal ovarian cysts
Related to maternal hormones
Cysts persistently larger than 5cm require drainage
Cysts <4cm can be followed up
Most resolve
Urgent laparoscopy if signs of torsion
1/25 risk of torsion during observation period
No definitive evidence for benefit of surgery
Ovarian torsion
USS - swirl of vessels, beaking, swelling, follicles pushed peripherally
58% of torsions associated with mass
Fallopian tube can tort in isolation
Factors predisposing: Long fallopian tube, mesosalpinx, mesoovarium
Most likely on the right as caecum more mobile
Ovarian tumours
Types of ovarian tumour:
Germ cell - more common in children
Epithelial - more common in adults
Sex-cord stromal
Ovarian tumours rare in younger patients, but more likely to be malignant with decreasing age
Benign vs Malignant - mean diameter 8cm vs 17cm
10% hormonally active
Precocious pseudopuberty (production from tumour) - Secretion of HCG from dysgerminomas, yolk sac tumours and choriocarcinomas
Most common with sex cord–stromal tumors, such as juvenile granulosa cell tumours or some Sertoli-Leydig cell tumors - raised oestrogen
Virilisation - Sertoli-Leydig cell tumours, dysgerminomas that contain syncytial trophoblastic giant cells, yolk sac tumors and polycystic ovaries
Important ovarian tumour markers
CA 125 - for epithelial + dysgerminoma + endometrioma + Sertoli-Leydig
aFP - for Yolk sac + mixed + Sertoli-Leydig
BHCG - for dysgerminoma, yolk sac, choricarcinoma
LDH - for dysgerminoma
Inhibin A + B - for granulosa cell tumours only
Endometrioma + epithelial - Only CA 125
Overlap with mixed tumours
Mature Teratoma
Clinical Scenario: Mass or incidental calcification on imaging
Imaging Features: Predominantly cystic, with solid areas in 70%, can see hair, Rokitansky nodule - this intraluminal projection may represent a dermal plug, a tooth but is concernign for malignant transformation
Markers: None
Intra-operative Features: Lobulated, smooth tumour within the ovarian capsule; up to 20% bilateral, metachronous, or synchronous. May include peritoneal implants (gliomatosis peritonei) which are non-malignant and do not worsen prognosis
Notes: Dermoid cyst = Ovarian mature teratoma
90% of ovarian tumours; common ovarian neoplasm but only 10% have immature or malignant components
Risk of developing endodermal sinus tumour (in children) or squamous cell carcinoma (in adults)
Follow up is y
early USS until 1st pregnancy
2014 Helsinki paper - 23% Metachronous contralateral tumour CCLG - 5% in 18 months
Immature/Malignant Teratoma
Clinical Scenario: Mass or incidental finding
Imaging Features: Solid and cystic
Markers: +/- αFP
Intra-operative Features: Often indistinguishable intraoperatively from mature lesions
Notes: Contains immature and malignant elements (usually yolk sac tumour or neural elements)
Yolk Sac Tumour
Clinical Scenario: Distension and pain
Imaging Features: Solid +/- cystic
Markers: αFP, LDH
Intra-operative Features: Unilateral
Notes: Second most common malignancy
Choriocarcinoma
Clinical Scenario: Precocious puberty
Imaging Features: Solid
Markers: βhCG
Intra-operative Features: Cytotrophoblast and syncytiotrophoblast elements, very vascular, invades surrounding structures
Notes: Generally observed as part of a mixed tumour
Gonadoblastoma
Clinical Scenario: Primary amenorrhea or virilisation
Intra-operative Features: Streak or dysgenic gonad, intra-abdominal "testicle" in females
Notes: Principally found in phenotypic females with XY chromosomes
Dysgerminoma
Clinical Scenario: Distension and pain
Imaging Features: Solid
Markers: LDH
Intra-operative Features: Thick, white, bulbous tumour, potentially rupturing upon presentation; bilateral in 15-30% of cases
Notes: Most frequent ovarian malignancy; paraneoplastic syndrome of hypercalcaemia resolves after resection
Embryonal Cell Tumour
Clinical Scenario: Precocious puberty
Imaging Features: Solid
Markers: βhCG
Intra-operative Features: Unilateral
Notes: Rare as an isolated tumour in girls; typically part of a mixed tumour
Mixed Germ Cell Tumour
Clinical Scenario: Dependent on components
Imaging Features: Dependent on components
Markers: None or any; occasionally CA-125
Intra-operative Features: Dependent on components
Notes: Typically consists of dysgerminoma or yolk sac tumour with other components; treatment depends on the most malignant subtype
Sex Cord Stromal Tumours (Granulosa - Theca cell most common)
Clinical Scenario: Precocious puberty (Sertoli - Leydig tumours masculinise)
Markers: Inhibin A + B, αFP in some
Notes: Low stage treated with excision only; standard germ cell chemotherapy for advanced stages
Epithelial tumours
15% of ovarian masses in children
Serous tumours - bilateral in 20% - few are malignant
Mucinous tumours - unilateral - 10% malignant
CA-125 (possibly not needed - Cochrane review) + USS follow up
CCLG staging
1 - Complete removal, no capsule rupture
2 - Capsule rupture/piecemeal, tumour >10cm removed laparoscopic, no peritoneal involvement
3 - Gross residual or positive nodes or peritoneal involvement
4 - Metastases
Tubo-ovarian abscess
Secondary to pelvic inflammatory disease
E.coli, bacteroides, strep - not usually chlamydia/gonnorhoea
Usually resolves with IV Cef+ Met
If not, IR guided aspiration/drain
Consider safeguarding implications
McCune Albright syndrome
GNAS gene mutation, Non-hereditary, Rare
Triad of:
• Fibrous dysplasia of bone
• café-au-lait skin spots
• precocious puberty
Also:
Hyperthyroidism
Renal phosphate wasting
Cushing syndrome in neonatal period
Can have multiple functioning ovarian cysts
Investigations
LH/FSH - will be low if oestrogen being produced
Thyroid function
XR: typical expansile lesions with endosteal scalloping and thinning of the cortex with the matrix of the intramedullary tissue demonstrating a "ground glass" appearance
Management
Aromatase inhibitors
Treatment of manifestations
Standard scenario
Ovarian cyst/mass
Concerns:
1. Malignancy
2. Hormone secretion
3. Mass effect/torsion
Ensure resuscitated, analgesia
History:
Mass, pain
Virilisation, precocious puberty
Family history of tumour syndromes
Examination:
Mass
Secondary sexual characteristics
Investigations:
CA 125 - for epithelial + dysgerminoma + endometrioma + Sertoli-Leydig
aFP - for Yolk sac + mixed + Sertoli-Leydig
BHCG - for dysgerminoma, yolk sac, choricarcinoma
LDH - for dysgerminoma
Inhibin A + B - for granulosa cell tumours only
Endometrioma + epithelial - Only CA 125
USS + MRI
Looking for cystic, solid features
MDT
CCLG staging for extracranial GCT
Others: FIGO (involvement of tubes, other ovary, uterus, abdomen, extent beyond pelvis)
Management:
UK consensus survey 2022
If torted (or found incidentally) detort, do not fix, close and follow process above
MDT decision based on probability of malignancy (biomarkers, symptoms, imaging (solid components), age)
If large cyst and unlikely malignant:
Ovarian sparing surgery whilst still maintaining oncological principles
Pfannenstiel incision
Tegaderm and glue technique - aspirate cyst without spillage before delivering
If possible malignancy: ESGO-SIOPE guidelines
Midline staging laparotomy and oophorectomy
(Peritoneal washing, omental biopsy, palpation of liver + peritoneum, examination of aortic nodes)
If too stuck to surrounding structures - biopsy and close, give chemo and second look
Should not do pelvic exenteration as first line
Malignant - stages 2-4 - give chemotherapy
Follow up
CCLG nationwide study - benign tumours
8% will have recurrent or metachronous disease within 3 years
Ovarian sparing vs oophorectomy no effect on recurrence
Unilateral oophorectomy in childhood has been shown to advance menopause by seven years (Thomas-Teinturier 2012)
ESGO-SIOPE guidelines - for Malignant GCT:
Exam, USS, bloods - every 2 months for 2 years
Then reducing frequency up to 10 years
CXR/CT and Abdo MRI/CT every 6 months for 2 years
References
J.Ritchie, F.O’Mahony, A.Garden. Guideline for the management of ovarian cysts in children and adolescents. On behalf of British Society for Paediatric & Adolescent Gynaecology 2018
CCLG Interim Guidelines for the Treatment of Extracranial Germ Cell Tumours in Children and Adolescents
June 2018
Aldrink, Jennifer, et al. "Ovarian Tumors." Pediatric Surgery NaT, American Pediatric Surgical Association, 2024. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829126/all/Ovarian_Tumors.
Burnweit, Cathy, et al. "Germ Cell Ovarian Tumors." Pediatric Surgery NaT, American Pediatric Surgical Association, 2024. Pediatric Surgery Library, www.pedsurglibrary.com/apsa/view/Pediatric-Surgery-NaT/829129/all/Germ_Cell_Ovarian_Tumors.
Gaillard F, Sharma R, Silverstone L, et al. Mature cystic ovarian teratoma. Reference article, Radiopaedia.org (Accessed on 25 Jul 2024) https://doi.org/10.53347/rID-1808
Weerakkody Y, Ranchod A, Sharma R, et al. Rokitansky nodule. Reference article, Radiopaedia.org (Accessed on 25 Jul 2024) https://doi.org/10.53347/rID-8611
Braungart S, Williams C, Arul SG, et al. Standardizing the surgical management of benign ovarian tumors in children and adolescents: A best practice Delphi consensus statement. Pediatr Blood Cancer. 2022; 69:e29589 https://doi.org/10.1002/pbc.29589
Sessa C, Schneider DT, Planchamp F, Baust K, Braicu EI, Concin N, Godzinski J, McCluggage WG, Orbach D, Pautier P, Peccatori FA, Morice P, Calaminus G. ESGO-SIOPE guidelines for the management of adolescents and young adults with non-epithelial ovarian cancers. Lancet Oncol. 2020 Jul;21(7):e360-e368. doi: 10.1016/S1470-2045(20)30091-7. PMID: 32615119.
Braungart S; CCLG Surgeons Collaborators; Craigie RJ, Farrelly P, Losty PD. Ovarian tumors in children: how common are lesion recurrence and metachronous disease? A UK CCLG Surgeons Cancer Group nationwide study. J Pediatr Surg. 2020 Oct;55(10):2026-2029. doi: 10.1016/j.jpedsurg.2019.10.059. Epub 2019 Nov 26. PMID: 31837839.
Cécile Thomas-Teinturier et al. Age at menopause and its influencing factors in a cohort of survivors of childhood cancer: earlier but rarely premature, Human Reproduction, Volume 28, Issue 2, February 2013, Pages 488–495, https://doi.org/10.1093/humrep/des391