What is the management of a seromucinous borderline tumor in a 25-year-old female with ovarian endometriosis (Endometriosis, a condition where Endometrium (mucous membrane) grows outside the Uterus)?

From the Guidelines

A seromucinous borderline tumor in the background of ovarian endometriosis in a 25-year-old female should be managed with fertility-sparing surgery as the initial approach, prioritizing preservation of fertility potential. This tumor is a type of borderline ovarian tumor that shows both serous and mucinous epithelial components and often arises from endometriosis. The management approach should involve unilateral salpingo-oophorectomy (removal of the affected ovary and fallopian tube) or ovarian cystectomy if possible, along with comprehensive surgical staging including peritoneal washings, omental sampling, and peritoneal biopsies, as recommended by the ESMO-ESGO consensus conference 1.

Key Considerations

• The procedure should be performed by a gynecologic oncologist to ensure proper staging and preservation of fertility potential.
• Following surgery, close surveillance is essential with transvaginal ultrasound and CA-125 testing every 3-6 months for the first 2-3 years, then annually thereafter.
• Hormonal suppression therapy with combined oral contraceptives or progestins may be considered to manage the underlying endometriosis and potentially reduce recurrence risk, as suggested by the NCCN guidelines 1.
• The prognosis for seromucinous borderline tumors is generally excellent with a low recurrence rate (5-10%) and high overall survival (>95% at 5 years), as reported in the literature 1.

Surgical Approach

• Fertility-sparing surgery is acceptable for stage IA tumors, and the use of bilateral cystectomies compared with a unilateral adnexectomy and a contralateral cystectomy increases the fertility rate without increasing the recurrence rate, as demonstrated in a recent phase III trial 1.
• In cases of stage II or III disease, the use of fertility-sparing surgery is unconventional, with high risk of recurrences reported, but it could be considered in selected cases with complete removal of peritoneal implants, as suggested by the ESMO-ESGO consensus conference 1.

Adjuvant Therapy

• The role of adjuvant chemotherapy in advanced-stage serous borderline ovarian tumors is highly debated, and recent retrospective data suggest that it may be considered in patients with invasive implants treated with surgery, but the evidence is limited 1.
• The NCCN guidelines recommend observation as one of the possible approaches for patients with borderline epithelial tumors, and postoperative chemotherapy may be considered for patients with invasive implants, but the benefit of chemotherapy is controversial 1.

From the Research

Definition and Characteristics of Seromucinous Borderline Tumor

• Seromucinous borderline tumor (BSMT) is a rare form of borderline malignancy, with a low frequency of occurrence, and many aspects of its behavior remain unclear 2.
• BSMT is a histological type of ovarian neoplasm, associated with endometriosis, and its preoperative diagnosis is often difficult due to various clinical findings 2.
• The tumor is characterized by the presence of serous and endocervical-type mucinous epithelium, as well as endometrioid, indifferent, and squamous type epithelium 3.
• Immunohistochemical studies have shown that BSMT cells are frequently positive for estrogen receptor (ER) and progesterone receptor (PR), and lack expression of CK20 and CDX2, consistent with a "müllerian" immunophenotype 3.

Management of Seromucinous Borderline Tumor in a 25-Year-Old Female

• The management of BSMT in a 25-year-old female with ovarian endometriosis would likely involve surgical intervention, such as salpingo-oophorectomy, to remove the tumor and affected ovary 2, 4.
• Preoperative diagnosis using magnetic resonance (MR) relaxometry may be useful in discriminating between benign ovarian endometrioma and endometriosis-associated ovarian cancer, and in identifying BSMT 4.
• Immunohistochemical studies can aid in the diagnosis and characterization of BSMT, and may help distinguish it from other types of ovarian tumors 3, 4.
• The presence of endometriosis and an elevated serum CA19-9 level may also aid in the diagnosis of BSMT 2.

Association with Endometriosis

• BSMT is frequently associated with endometriosis, with over 90% of cases having coexisting endometriosis histologically 2.
• The tumor's association with endometriosis suggests that it may share a common molecular pathway, involving ARID1A mutations, with other endometriosis-associated tumors, such as clear cell tumors 5, 6.
• The recognition of this association is important for the diagnosis and management of BSMT, and may have implications for the treatment and prognosis of patients with this tumor 2, 5, 6.