Extraovarian sex cord tumor with annular tubules discovered arising from a leiomyoma

Abstract

BackgroundSex cord tumors with annular tubules (SCTAT) are a rare (2%) subtype of ovarian sex cord-stromal tumor. SCTATs are usually cured at time of diagnosis by surgical resection with an oophorectomy. SCTATs have a 100%(disease related) five-year survival. One third of SCTAT tumors are associated with Peutz-Jeghers syndrome. Literature review discovered only two published cases of extra-ovarian SCTAT. Due to the rarity there is no standard treatment for extraovarian SCTATs.CaseA 39-year-old para-1 female with a symptomatic fibroid uterus, heavy menstrual bleeding, and a history of a uterine myomectomy, underwent an elective total abdominal hysterectomy. Intraoperative findings showed a 7.5 cm retroperitoneal mass adhered between the uterus and the right pelvic sidewall that on frozen section was found to be a degenerating leiomyoma. Final pathology demonstrated a 2 mm focus of incidental SCTAT adjacent to the serosal surface of the leiomyoma. The SCTAT was not associated with ectopic ovarian tissue or endometriosis. The patient's ovaries were normal on direct intraoperative examination, preoperative ultrasound and MRI. Six month postoperative surveillance ultrasound also demonstrated normal premenopausal ovaries.ConclusionThis is the first extraovarian SCTAT in the published literature arising from a leiomyoma. Our patient had no family history and displayed no syndromic features for Peutz-Jeghers Syndrome. Ultimately, she declined genetic testing. The lack of evidence of ovarian involvement on both imaging and on intraoperative examination made localization to either ovary impossible. The patient is currently being managed with surveillance since the morbidity associated with bilateral oophorectomy in the 4th decade of life exceeds the theoretical risk of SCTAT.

A recurrence of advanced malignant sex cord tumor with annular tubules: case report

“…Qian et al ( 5 ) demonstrated a 100% survival rate in 5 year, while their median progression-free survival time was 97.8 months by a retrospective study, which showed the overall good prognosis. The annual surveillance for 8–10 years with or without ultrasound and tumor makers serology (estradiol and/or progestin) depending on clinical suspicion was recommended ( 4 , 22 ). In our case, regular supervision has persisted in 2 years.…”

“…SCTAT is reported that about one third of patients experience Peutz-Jeghers syndrome (PJS) with mucocutaneous pigmentation in special locations and gastrointestinal tract polyposis, whose form is usually benign and has excellent prognosis through surgery. However, SCTAT without PJS may have a 15–20% malignancy rate and malignant behavior with metastases might be examined in approximately 20% of patients ( 4 ), which is a most common phenomenon in women of reproductive age. The tumors both may express the hyperestrogenic manifestations, regardless of the clinical form.…”

Peutz‑Jeghers syndrome: Skin manifestations and endocrine anomalies (Review)

“…SCTAT, representing 2% of all sex cord-stromal tumors, are considered very rare neoplasias (54). An increased progesterone production has been revealed in some cases (55).…”

Role of the Serine/Threonine Kinase 11 (STK11) or Liver Kinase B1 (LKB1) Gene in Peutz-Jeghers Syndrome

“…This risk may approach a 20-fold increase over the general population if all organs are considered, although the increased risk for any particular site is necessarily more modest. About three quarters of PJS cases occur in families, with the remainder resulting from new mutations or low penetrance variants 1,2 .…”