How are teratomas managed in individuals with Lynch syndrome?

Management of Teratomas in Individuals with Lynch Syndrome

Teratomas are not specifically addressed in Lynch syndrome management guidelines, but should be managed according to standard oncologic principles while considering the patient's overall Lynch syndrome surveillance and management plan.

Lynch Syndrome Background

• Lynch syndrome is an autosomal dominant inherited cancer syndrome caused by pathogenic variants in DNA mismatch repair genes (MLH1, MSH2, MSH6, and PMS2) 1
• Lynch syndrome accounts for 2-4% of all colorectal cancer cases and 2-3% of endometrial cancer cases 1
• Individuals with Lynch syndrome are at increased risk for multiple cancers, primarily colorectal and endometrial cancers, but also ovarian, gastric, small intestinal, pancreatic, urinary tract, biliary tract, and brain cancers 1

Cancer Surveillance in Lynch Syndrome

Colorectal Cancer Surveillance

• Colonoscopy every 1-2 years beginning at age 20-25 or 2-5 years younger than the youngest diagnosis in the family, whichever comes first 1
• Colonoscopic surveillance has been shown to reduce colorectal cancer incidence by 62% and mortality in Lynch syndrome 1
• Chromoendoscopy may improve detection of lesions and flat adenomas 1

Gynecologic Cancer Surveillance

• Women with Lynch syndrome have up to 60% lifetime risk of endometrial cancer and up to 24% risk of ovarian cancer 1, 2
• Annual gynecologic examination with endometrial sampling and transvaginal ultrasound starting at age 30-35 years may be considered 1
• Education about recognizing symptoms of gynecologic cancers (e.g., dysfunctional uterine bleeding) is recommended 1

Other Cancer Surveillance

• Surveillance for other Lynch-associated cancers should be based on family history 1
• May include upper endoscopy and abdominal ultrasound with urine cytology from age 30-35 at 1-2 year intervals 1

Prophylactic Surgery Options

Colorectal Surgery

• Prophylactic colectomy is not routinely recommended for unaffected Lynch syndrome carriers 1
• For those with Lynch syndrome who develop colorectal cancer, subtotal colectomy (versus segmental resection) is a reasonable option due to the high risk of metachronous colorectal cancers 1
• Patient preference and compliance with surveillance are major factors in this decision 1

Gynecologic Surgery

• Total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH/BSO) is an effective option for risk reduction in women who have completed childbearing 1, 2
• Evidence shows no endometrial or ovarian cancers developed in women who had prophylactic surgery compared to 33% endometrial cancer and 5.5% ovarian cancer incidence in those who did not have surgery 1
• May be reasonable to offer this option to women aged 35 years or older who do not want to preserve fertility 1
• Consider coordinating risk-reducing gynecological surgery with colorectal surgery when applicable 2

Chemoprevention and Lifestyle Modifications

• Aspirin chemoprevention is recommended to reduce colorectal and other cancer risks 2
• Combined oral contraceptives may reduce endometrial and ovarian cancer risk in women requiring contraception 2
• Maintain healthy body mass index, eat a healthy diet, exercise regularly, avoid smoking, and limit alcohol consumption 1, 2

Management of Teratomas in Lynch Syndrome

While teratomas are not specifically addressed in Lynch syndrome guidelines, management should follow these principles:

• Standard surgical management of teratomas should be performed according to oncologic principles 1
• Coordinate teratoma management with the patient's overall Lynch syndrome surveillance plan 2
• For ovarian teratomas in women with Lynch syndrome who have completed childbearing, consider concurrent risk-reducing hysterectomy and bilateral salpingo-oophorectomy at the time of teratoma removal 1, 2
• For women desiring future fertility, ovarian-sparing surgery for teratomas should be considered when feasible, with continued Lynch syndrome surveillance 2

Common Pitfalls and Caveats

• Failure to identify Lynch syndrome in patients with teratomas who have personal or family history suggestive of Lynch syndrome 1
• Overlooking the need for coordinated care between gynecologic and colorectal specialists 2
• Delaying risk-reducing surgery beyond recommended age increases cancer risk 2
• Failure to provide estrogen-only HRT after oophorectomy in premenopausal women can lead to significant quality of life issues 2