Müllerian Mucinous Cystadenomas of the Ovary: A Report of 25 Cases of an Unheralded Benign Ovarian Neoplasm Often Associated With Endometriosis and a Brief Consideration of Neoplasms Arising From the Latter.

A subset of ovarian mucinous tumors demonstrates müllerian-type epithelium, with such lesions variably designated "endocervical-like" and seromucinous since their popularization based on a report of borderline examples in 1989. While müllerian mucinous borderline tumors and carcinomas have been highlighted in the literature, there has been minimal attention given to benign müllerian mucinous tumors, particularly müllerian mucinous cystadenomas. Given the paucity of literature describing the features of müllerian mucinous cystadenomas/cystadenofibromas, diagnostic difficulties may arise when papillary features are present and in cases that show a subtle transition from endometriosis. We thus reviewed 25 cases of müllerian mucinous cystadenoma/cystadenofibroma to highlight the notable characteristics of this entity, including gross, cytologic, and architectural features that aid in the distinction from müllerian mucinous borderline tumors as well as, rarely, metastatic tumors. The patients ranged in age from 26 to 85 yr old. Bilateral ovarian involvement was frequent (40%). The ovaries ranged from 2.3 to 26 cm in greatest dimension. Most were multicystic (18 cases) and contained tenacious mucoid material (14 cases). All cases demonstrated predominantly columnar mucinous epithelium with abundant pale-pink cytoplasm. A minor component of ciliated and endometrioid epithelium was seen in 15 and 2 cases, respectively. Broad papillary formations were frequently encountered (9 cases) as was epithelial papillary tufting comprising <10% of the tumor (6 cases). Endometriosis was present in 9 cases, with a transition from endometriosis to mucinous epithelium noted in 8 cases. This series highlights the morphologic features of a relatively uncommon, benign, endometriosis-associated ovarian tumor that may be confused with a müllerian mucinous borderline tumor or bland metastatic mucinous tumors. It also provides an argument for the terminology "müllerian mucinous cystadenoma" or "cystadenofibroma" rather than "seromucinous cystadenoma" due to the frequent association with endometriosis as well as the dominant mucinous epithelium.

Massive Ovarian Growth in a Woman With Severe Insulin-Resistant Polycystic Ovary Syndrome Receiving GnRH Analogue.

CONTEXT: Ovarian hyperandrogenism from polycystic ovary syndrome (PCOS) and hyperinsulinemia from insulin resistance are modulators of ovarian follicle development. We report on a woman with PCOS and hyperandrogenism and severe insulin resistance from metabolic syndrome who received long-term GnRH analogue therapy preceding bilateral salpingo-oophorectomy for massive ovarian enlargement. Ovarian histological examination showed proliferating granulosa cells within antral follicles coexistent with serous cystadenofibromas, demonstrating a unique link between hyperinsulinemia and granulosa cell mitogenesis. CASE DESCRIPTION: A 30-year-old woman with PCOS with hyperandrogenism, severe insulin resistance from metabolic syndrome, and nonalcoholic steatohepatitis experienced abdominal pain from bilaterally enlarged ovaries. She had previously experienced a pulmonary embolism while taking oral contraceptives and hepatotoxicity from metformin and spironolactone therapies. Long-term GnRH analogue therapy to induce pituitary desensitization to GnRH successfully decreased gonadotropin-dependent steroidogenesis without improving insulin resistance. Despite GnRH analogue therapy, progressive ovarian enlargement in the presence of hyperinsulinemia from worsening metabolic function eventually required bilateral salpingo-oophorectomy for removal of massively enlarged ovaries. Histological examination showed both ovaries contained proliferating granulosa cells within antral follicles coexistent with serous cystadenofibromas. CONCLUSIONS: In women with PCOS and hyperinsulinemia from severe insulin resistance due to metabolic syndrome, granulosa cell proliferation within antral follicles can occur despite long-term GnRH analogue therapy, implicating hyperinsulinemia as a granulosa cell mitogen in the absence of gonadotropin-dependent ovarian function.

Unusual case of anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis and autoimmune polyglandular syndrome (APS).

Anti-N-methyl-D-aspartic acid-receptor (NMDA-R) encephalitis is a novel disease discovered within the past 10 years. It is an autoimmune disease (AD) that has been associated with other ADs, such as Graves' disease. However, association with autoimmune polyglandular syndromes (APS) has not been previously described. A 58-year-old woman presented with altered mental status and an 8-month history of weight loss, apathy and somnolence. Laboratory evaluation confirmed Graves' disease with thyrotoxicosis and type 1 diabetes mellitus. Despite treatment, she continued to have a fluctuating mental status. Further diagnostic evaluation included an abdominal MRI that showed a cystic lobular left adnexal mass. Serum anti-NMDA-R antibodies were positive, raising concern for NMDA-R encephalitis. Bilateral salpingo-oophorectomy was performed, with pathology consistent with cystadenofibroma. She had a favourable recovery with marked clinical improvement. Anti-NMDA-R antibodies were negative 2 months following surgery. The concomitant occurrence of APS and anti-NMDA-R encephalitis suggests a shared mechanism of autoimmune pathophysiology.

Large functional benign endometroid cystadenofibroma of the ovary leading to endometrial cystic glandular hyperplasia and postmenopausal bleeding.

Benign endometroid cystadenofibroma of the ovary is a rare tumour with few reported cases. The association of endometroid cystadenofibroma with endometrial cystic glandular hyperplasia has not been previously reported to authors' knowledge. We are reporting a 75-year-old postmenopausal woman who presented with a large abdominopelvic mass corresponding to 30 weeks size gravid uterus and postmenopausal bleeding. She has a well-oestrogenised vagina. Ultrasound pelvis revealed a large cystic mass extending from pelvis to epigastrium with no solid component and few incomplete septations and no internal echoes. MRI findings showed mainly cystic component with few moderately enhancing, fine, incomplete septa. Endometrial aspiration reported histopathology of cystic glandular hyperplasia without atypia. Serum oestrogen level reported to be high (210 pg/mL). Hysterectomy with bilateral salpingo-oophorectomy was performed. Histopathology of the specimen revealed benign endometroid cystadenofibroma. She remained asymptomatic and disease free during her 6-month follow-up.

Anti-N-methyl-d-aspartate encephalitis with ovarian cystadenofibroma.

We report the case of an adolescent girl with anti-N-methyl-D-aspartate-receptor (NMDAR) encephalitis who presented with focal seizures and hemichorea, followed by agitation, speech disturbance, mutism, and autonomic dysfunction. The institution of immunotherapy and removal of an ovarian cystadenofibroma led to full resolution of her symptoms with disappearance of serum NMDAR antibodies. This is the first report linking ovarian cystadenofibroma to anti-NMDAR encephalitis.

Ovarian benign clear cell adenofibroma in a patient with premature ovarian failure after 8-year hormone replacement therapy.

Ovary benign clear cell adenofibroma (CCAF) is extremely rare. Here, we reported a 38-year-old woman with CCAF who was given sequential cyclic hormone replacement therapy for eight years due to premature ovarian failure (POF). At operation, we found adenofibromas in both ovaries and well encapsulated. The cut surface was cystic and solid in appearance, and multilocular cysts embedded in hard stroma. Determination of sex hormone levels in the fluid of cysts of the ovary showed normal level. Clear cell cystadenofibroma proliferation was found in ovarian stroma while atrophy in ovarian cortex. Histological examination revealed that the glands were lined by 1 or 2 layers of hobnail cells with scant to moderate pale or clear cytoplasm. The nuclei were small uniform, and flat to round. Neither primary follicles nor secondary follicles were observed in ovary cortex. Immunohistochemical results showed that EMA and p53 were positive while Calretinin was negative. In this article, we reviewed literatures and analyzed both clinical and pathological features of ovary benign clear cell adenofibromas.