Subject(s)
Broad Ligament/pathology , Fibrosarcoma , Genital Neoplasms, Female , Hysterectomy/methods , Ovariectomy/methods , Adult , Antineoplastic Protocols , Chemoradiotherapy, Adjuvant/methods , Combined Modality Therapy/methods , Female , Fibrosarcoma/pathology , Fibrosarcoma/physiopathology , Fibrosarcoma/therapy , Genital Neoplasms, Female/pathology , Genital Neoplasms, Female/physiopathology , Genital Neoplasms, Female/therapy , Humans , Laparotomy/methods , Neoplasm Invasiveness , Neoplasm Metastasis , Neoplasm Staging , Pelvic Neoplasms/pathology , Pelvic Neoplasms/physiopathology , Pelvic Neoplasms/therapy , Treatment OutcomeABSTRACT
We report a case of a 54-year-old Caucasian woman with an earlier diagnosis of Peutz-Jeghers Syndrome (PJS) and sex cord tumor with annular tubules (SCTAT). The sex cord stromal tumors showed aggressive malignant behavior with repeated recurrence and metastasis. This is an unusual behavior of SCTAT in patients with PJS, with only 2 such cases reported earlier. Genetic analysis revealed that the patient has a new (unreported earlier) missense mutation of the LKB1 gene. A review of the literature reporting the clinicopathologic features and biologic behavior of SCTAT in patients with and without PJS is presented. We discuss the presentation and management of this case and highlight the importance of considering the possibility of aggressive behavior of these tumors in the management of patients with PJS.
Subject(s)
Ovarian Neoplasms/pathology , Peutz-Jeghers Syndrome/complications , Sex Cord-Gonadal Stromal Tumors/pathology , AMP-Activated Protein Kinase Kinases , Female , Humans , Middle Aged , Mutation, Missense , Ovarian Neoplasms/complications , Ovarian Neoplasms/genetics , Peutz-Jeghers Syndrome/genetics , Protein Serine-Threonine Kinases/genetics , Sex Cord-Gonadal Stromal Tumors/complications , Sex Cord-Gonadal Stromal Tumors/geneticsABSTRACT
Menstruation is widely viewed as serving no purpose other than to reinitiate the endometrial cycle in the absence of pregnancy. Yet, it is striking that cyclic endometrial decidualization followed by menstrual shedding is confined to the few species, including human beings, where placenta formation entails deep trophoblast invasion of maternal tissues and its vasculature. Both menstruation and pregnancy are inflammatory conditions that cause a degree of physiological ischemia-reperfusion tissue injury, albeit much more so in pregnancy. Thus, the emergence of cyclic menstruation may not have been an evolutionary coincidence but serves to protect uterine tissues from the profound hyperinflammation and oxidative stress associated with deep placentation, a process known as preconditioning. The concept of menstrual preconditioning provides a novel paradigm for understanding how reproductive disorders impact on pregnancy outcome. For example, endometriosis could be viewed as a disorder of exaggerated menstrual preconditioning that confers protection against placentation-related disorders, such as preeclampsia.
