What is the recommended diagnostic work‑up and surgical management for large bilateral ovarian teratomas, including imaging, tumor marker assessment, fertility‑preserving options, and postoperative follow‑up?

Management of Large Bilateral Ovarian Teratomas

Diagnostic Work-Up

For large bilateral ovarian teratomas, obtain MRI imaging and measure serum AFP, β-hCG, and LDH prior to surgical intervention, which is mandatory for bilateral disease of this size. 1, 2

Imaging Strategy

• MRI is the preferred imaging modality for definitive diagnosis, reliably demonstrating fatty, calcified, hair, tooth, and cartilage components that are pathognomonic for dermoid cysts 1, 2
• Ultrasound may show characteristic features but MRI provides superior tissue characterization 2
• CT or MRI should assess for solid components, papillary projections, or vascular elements that raise suspicion for malignancy 2

Laboratory Evaluation

• Measure serum AFP, β-hCG, and LDH in all patients to screen for malignant germ cell tumors, particularly important in younger women with pelvic masses 1, 2, 3
• These markers help distinguish benign mature teratomas from immature teratomas or other malignant germ cell tumors 1

Red Flags Requiring Urgent Evaluation

• Solid components or papillary projections on imaging 2
• Elevated tumor markers (AFP, β-hCG, LDH) 2
• Rapid growth or evolving imaging characteristics 2
• Cysts ≥10 cm merit surgical intervention regardless of other characteristics 2

Surgical Management

In reproductive-age women with bilateral ovarian teratomas, perform unilateral salpingo-oophorectomy with contralateral ovarian cystectomy to preserve fertility, even in large bilateral disease. 1

Fertility-Preserving Approach

• Unilateral salpingo-oophorectomy with preservation of at least a healthy part of one ovary (contralateral cystectomy) and the uterus should be encouraged in cases of macroscopic bilateral ovarian disease, particularly dysgerminoma or immature teratoma 1
• Systematic ovarian biopsy of the contralateral ovary is not necessary when macroscopically normal 1
• Laparoscopic ovarian cystectomy is the gold-standard technique for reproductive-age women requiring surgery, with primary goal of preserving ovarian tissue 2
• Minilaparotomy may be considered as an alternative to avoid cyst rupture when laparoscopic extraction poses high spillage risk 4

Surgical Technique Considerations

• Careful examination of the abdominal cavity is required 1
• For mature cystic teratomas (dermoid cysts), minimally invasive surgery is associated with decreased perioperative complications but increased risk of cyst spillage 5
• Despite increased spillage risk with laparoscopy, chemical peritonitis is rare (1 in 394 cases with spillage) 5
• Staging procedures for suspected immature teratoma include infracolic omentectomy, biopsy of diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings 1, 3

Lymph Node Management

• Nodal dissection should be carried out only if there is evidence of nodal abnormality during surgical exploration or on initial CT scan 1
• Systematic lymphadenectomy is not required for germ cell tumors given their high chemosensitivity 1
• Potential nodal metastases can be cured by adjuvant chemotherapy if needed 1

Postmenopausal or Advanced Disease

• In postmenopausal women or patients with advanced-stage disease, abdominal hysterectomy and bilateral salpingo-oophorectomy should be performed with careful surgical staging 1

Pathology-Specific Management

Mature Cystic Teratoma (Dermoid Cyst)

• Most common type, accounting for up to 20% of all ovarian tumors 1
• Malignant transformation rate is 1-2%, with squamous cell carcinoma comprising >80% of malignancies 2, 5, 6
• Bilateral teratomas occur in approximately 10-11% of cases 5
• Torsion occurs in 5.6% overall, with higher rates in larger cysts 5, 6

Immature Teratoma

• Stage IA grade 1 immature teratoma requires no adjuvant chemotherapy after adequate surgical staging 1, 3
• Stage IA grade 2-3 and stage IB-IC immature teratoma: adjuvant chemotherapy is recommended, though active surveillance is an option 1, 3
• BEP (bleomycin, etoposide, cisplatin) 5-day regimen is the standard chemotherapy: 3 cycles for completely resected disease, 4 cycles for macroscopic residual disease (omit bleomycin after cycle 3 to reduce lung toxicity) 1, 3
• Expert pathology review should always be obtained to confirm diagnosis and exclude foci of yolk sac tumor or other malignant elements 3

Postoperative Follow-Up

Surveillance for Mature Cystic Teratoma

• Annual ultrasound surveillance is recommended for conservatively managed cysts 2
• For surgically treated benign teratomas, routine surveillance is typically not required unless concerning features were present 2

Surveillance for Immature Teratoma

• Regular clinical review with physical examination, abdomen-pelvic ultrasound, and tumor marker monitoring (AFP, β-hCG if initially elevated) over 10 years with gradually increasing intervals 1, 3
• Relapses most often occur within 12-18 months, most commonly in peritoneal cavity or retroperitoneal lymph nodes 1
• For patients choosing surveillance over adjuvant chemotherapy, close monitoring every 2-4 months for first 2 years is essential 3
• Any resectable residual disease should be removed, particularly in patients with normal serum markers, to avoid growing teratoma syndrome 1, 3

Critical Pitfalls to Avoid

• Do not perform radical surgery and full staging in reproductive-age women, as it is usually unnecessary and inappropriate 1, 2
• Do not delay surgery for symptomatic large bilateral teratomas, as torsion risk increases with cyst size 5, 7
• When diagnosing large ovarian tumor torsion, perform preoperative examinations with possibility of malignancy in mind, as malignant transformation can present as torsion 8
• Ensure expert pathology review to distinguish mature from immature teratomas and identify any malignant transformation 3
• Treatment at large cancer centers is associated with superior outcomes due to the rare nature of these tumors 1