Points to Remember in Ovarian Eponyms Pearls: Dr. Fritz Malignant Brenner Tumor

Dear Editor, Malignant Brenner tumor (MBT) of the ovary is a rare and uncommon ovarian neoplasm that consists of both epithelial and stromal cells.[1] While benign Brenner tumors are quite common, accounting for 1%–2% of all ovarian tumors, their malignant counterpart is extremely rare, accounting for 10 cm) grayish white fleshy, polypoid left ovarian mass, (b) Hematoxylin and eosin (H and E) stained section shows that a tumor is composed of irregularly nests and sheets of cytologically atypical transitional/urothelial-type cells, resembles invasive urothelial carcinoma (H and E; ×40), (c) Higher magnification shows hyperchromatic pleomorphic nuclei, variably prominent nucleoli, dense eosinophilic cytoplasm, and prominent mitotic activity (H and E, ×400), (d) Tumor cells diffusely immunopositive for pan cytokeratin (×100), (e) Tumor cells displaying focal estrogen receptor positivity (×100), (f) Tumor cells focally positive for vimentin (×200), (g) Tumor cells immunonegative for Wilms’ tumor gene 1 (WT-1; ×100)The clinical presentation of MBT is often nonspecific, with symptoms such as abdominal distension, pelvic pain, or abdominal bulk. Consequently, the diagnosis is frequently established at an advanced stage when surgical intervention is necessary.[2,3] On imaging studies, MBTs are often indistinguishable from surface epithelial ovarian tumors, complicating preoperative assessment.[4] Histopathological testing reveals characteristic transitional epithelium and a thick, stringy stroma, exhibiting features of mild atypia, mitotic activity, and stromal invasion.[3,4] For malignant Brenner tumors, surgical resection remains the primary treatment; chemotherapy may be considered if the tumor has progressed or recurred. However, due to the rarity of MBT, little is known about the optimal treatment approach and long-term prognosis. Prognoses for patients with malignant Brenner tumors vary based on histological grade, treatment response, and disease stage at diagnosis[5,6] [Table 1].Table 1: Difference between benign, borderline, and malignant Brenner tumor[ 6 ]As per the largest systematic review done by Koufopoulos et al.,[1] complete surgical resection (hysterectomy + BSO + omentectomy + lymph node dissection) is advised in stages I to III, as surgical management continues to be the mainstay of MBT treatment. Stage IIIb/IV patients were primarily considered for neoadjuvant chemotherapy (paclitaxel-carboplatin/paclitaxel-cisplatin/melphalan) along with radiotherapy. Adjuvant chemotherapy should be recommended if there is the proof of lymph node involvement, metastasis, or peritoneal spread.[1,3] The management of MBT lacks a standardized approach, although due to the small number of cases. Like other ovarian carcinomas, platinum-based chemotherapy is typically used in cases with advanced disease or recurrence. There is not enough evidence to support radiation therapy as a standard component of treatment for MBT, and its role in the treatment is debatable.[3-4] When the tumor is limited to the ovary, the prognosis is usually better, and the outcome is more favorable. The prognosis is typically worse for patients with recurrent tumors, poor differentiation, or advanced disease. The variation in clinical results, as with many uncommon ovarian cancers, emphasizes the significance of customized treatment regimens and careful monitoring.[2,5] The prognosis for MBT patients depends on several variables, such as the disease’s stage at diagnosis, the extent of malignancy, and the patient’s reaction to treatment. Stage I MBT usually has a good prognosis with complete surgical resection, but advanced-stage tumors with peritoneal spread or high-grade features have a worse prognosis. As per the current available literature, the disease-specific 5-year survival rate for patients with stage I tumors is 94.5%, whereas the disease-specific 5-year survival rate for patients with extraovarian dissemination (stages II, III, and IV) is 51.3%.[2,4] However, due to the rarity of MBT and the lack of extensive research, it is difficult to determine the definitive prognostic factors or survival statistics.[1,4] Given the paucity of clinical experience with MBT, more investigation is required to clarify the molecular and genetic foundations of this uncommon tumor and to improve treatment strategies for afflicted individuals. An overview of the clinical, pathological, and therapeutic aspects of malignant Brenner tumors is given in this introduction, with a focus on the need for improved diagnostic tools and greater awareness in the management of this rare ovarian cancer. Ethics statement This study was conducted in accordance with the ethical principles outlined in the Declaration of Helsinki and its amendments. The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed. Author contributions Conceptualization, AK, RT, and RHP; Conceptualization, RHP; Validation, AK and RT; Formal analysis, writing original draft preparation, AK, RT and RHP; Writing-review and editing, AK, RT and RHP; Supervision, RHP. All authors have read and agreed to the final version of the manuscript. Data availability statement Data sharing is not applicable to this article as no datasets were generated or analyzed during the current study. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.