What is the recommended treatment for a patient diagnosed with a teratoma?

Treatment of Teratoma

Surgery is the primary treatment for teratoma, with complete surgical excision being curative for mature teratomas and the essential first step for immature teratomas, followed by risk-stratified adjuvant chemotherapy based on stage and grade.

Surgical Management by Anatomic Location and Patient Population

Ovarian Teratomas in Reproductive-Age Women

• Unilateral salpingo-oophorectomy with preservation of the contralateral ovary and uterus is the standard fertility-sparing approach, even in advanced disease due to high chemosensitivity 1
• Comprehensive surgical staging must include infracolic omentectomy, biopsy of diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings 1
• Lymph node dissection should only be performed if nodal abnormality is evident—routine lymphadenectomy is not required 1
• Endometrial curettage must be performed to exclude concomitant uterine malignancy 2

Ovarian Teratomas in Postmenopausal Women

• Bilateral salpingo-oophorectomy is standard given increased malignant transformation risk with age 3
• Complete surgical staging is mandatory if malignant transformation is suspected, including omentectomy, peritoneal biopsies, and lymph node assessment 3

Testicular Teratomas

• Radical inguinal orchiectomy is the standard approach 4
• For stage I disease with completely removed pure teratoma, no adjuvant chemotherapy is indicated 4
• Nerve-sparing retroperitoneal lymph node dissection (RPLND) should be considered when contraindications exist to standard surveillance or chemotherapy, particularly with somatic transformation in the primary tumor 4
• For marker-negative stage IIA disease with single progressing lymph node suggestive of teratoma, primary nerve-sparing RPLND provides both diagnostic and therapeutic benefit 4

Neonatal and Pediatric Teratomas

• Complete surgical excision is the treatment of choice, with most neonatal teratomas being benign and occurring in the sacrococcygeal area 5
• In children and adolescents with early-stage germ cell tumors, comprehensive staging may be omitted 1

Adjuvant Chemotherapy Decision Algorithm for Immature Teratoma

No Adjuvant Chemotherapy Required

• Stage IA grade 1 immature teratoma after adequate surgical staging requires no adjuvant chemotherapy 1
• Mature teratoma without malignant transformation requires no adjuvant therapy 3

Adjuvant Chemotherapy Recommended (with Surveillance as Alternative)

• Stage IA grade 2-3 and stage IB-IC immature teratoma warrant adjuvant chemotherapy, though active surveillance is acceptable 1

Adjuvant Chemotherapy Mandatory

• Postoperative chemotherapy is mandatory for stage II-IV immature teratoma 1

Chemotherapy Regimen Specifications

BEP Protocol

• BEP (bleomycin, etoposide, cisplatin) 5-day regimen is the most widely used and recommended chemotherapy 1
• Three cycles of BEP for completely resected disease 1
• Four cycles for macroscopic residual disease, with bleomycin omitted after the third cycle to reduce lung toxicity risk 1
• For testicular non-seminoma with high-risk stage I disease (vascular invasion present), one cycle of BEP reduces relapse rate to <5% 4

Growing Teratoma Syndrome Management

Recognition and Intervention

• Growing teratoma syndrome is defined by enlarging metastatic masses consisting entirely of mature teratoma during chemotherapy with decreasing or normalized tumor markers 6
• The median growth rate is 0.7 cm per month in diameter or 12.9 cc per month in volume 6
• Any resectable residual disease should be removed, particularly in patients with normal serum markers, to prevent growing teratoma syndrome 1
• Complete resection should be considered early as there is risk of malignant transformation of residual teratoma 7

Surgical Approach

• RPLND provides excellent local control with low progression risk for retroperitoneal growing teratoma syndrome 6
• These potentially curative RPLNDs should only be performed by high-volume surgeons in expert centers 4

Critical Pitfalls to Avoid

• Expert pathology review must always be obtained to confirm diagnosis and exclude foci of yolk sac tumor or other malignant elements 1
• Incomplete surgical staging when malignancy is found leads to understaging and inadequate treatment 3
• Failure to perform complete resection of growing teratoma risks malignant transformation, which has been reported as carcinoma or sarcoma years after initial treatment 7
• In testicular teratoma, if vital germ cell tumor is found in RPLND specimen, adjuvant chemotherapy with one cycle of BEP should be considered post-RPLND 4

Surveillance Protocols

For Benign Mature Teratoma

• Clinical follow-up every 3-6 months for the first 2 years, then annual follow-up thereafter 3
• Routine surveillance with physical examination is sufficient 3

For Immature Teratoma After Treatment

• Regular clinical review with physical examination, radiological imaging including abdomen-pelvic ultrasound, and monitoring of tumor markers (AFP, β-hCG if initially elevated) over 10 years with gradually increasing intervals 1
• For patients choosing surveillance over adjuvant chemotherapy, close monitoring every 2-4 months for the first 2 years is essential to detect early recurrence 1

For Malignant Transformation

• More intensive surveillance with physical examination, imaging studies, and tumor markers every 3-4 months for the first 2 years 3

Tumor Marker Monitoring

• Alpha-fetoprotein (AFP) is the tumor marker of choice, particularly useful for assessing residual or recurrent disease 5
• β-hCG and LDH should also be evaluated when indicated 3