ABSTRACT
Introducción: La leiomiomatosis peritoneal diseminada (LPD), se trata de una enfermedad benigna, que se define por la presencia de múltiples nódulos diseminados en el peritoneo de diferentes tamaños compuestos por haces de células de músculo liso. Se postulan varias teorías sobre su origen relacionadas con el estímulo hormonal, la susceptibilidad genética y la iatrogenia tras cirugías como las miomectomías por vía laparoscópica. Hallazgos clínicos: Las pacientes suelen presentar molestias abdominales de diversa índole, incluso puede cursar de forma asintomática siendo un hallazgo casual en pruebas de imagen. Diagnósticos principales: En el diagnóstico diferencial se suelen incluir la carcinomatosis, la endometriosis, la endosalpingiosis, los tumores del tracto gastrointestinal o el leiomiosarcoma. Intervenciones terapéuticas: No hay suficiente evidencia acerca de cuál es el mejor abordaje, algunos optan por manejo expectante o tratamientos médicos y otros abogan por un manejo quirúrgico más radical. Dentro de los tratamientos médicos, uno de los más usados son los agonistas de la GnRH, también se han utilizado con buenos resultados inhibidores de la aromatasa y los moduladores selectivos de los receptores de progesterona como el acetato de ulipristal. Resultados: En este caso presentamos una paciente con LPD con 15 años de seguimiento en nuestro hospital, sin evidencia de malignización. Conclusión: Conociendo la naturaleza generalmente benigna de esta enfermedad, es necesario optar por el abordaje menos invasivo posible. Se desconoce la evolución a largo plazo de esta enfermedad, pues la mayoría de casos publicados no tienen suficiente tiempo de seguimiento.
Introduction: Disseminated peritoneal leiomyomatosis (DPL) is a benign pathology, defined by the presence of multiple disseminated nodules in the peritoneum of different sizes composed of bundles of smooth muscle cells. Several theories are postulated about its origin related to hormonal stimulus, genetic susceptibility and iatrogenesis after surgeries such as laparocopic myomectomies. Clinical findings: Patients usually present with abdominal discomfort of various kinds, and it may even be asymptomatic, being an incidental finding on imaging tests. Main diagnoses: The differential diagnosis usually includes carcinomatosis, endometriosis, endosalpingiosis, tumours of the gastrointestinal tract or leiomyosarcoma. Therapeutic interventions: There is insufficient evidence about the best approach, with some advocating expectant management or medical treatment and others advocating more radical surgical management. Among medical treatments, one of the most widely used are GnRH agonists, aromatase inhibitors and selective progesterone receptor modulators such as ulipristal acetate have also been used with good results. Results: In this case we present a patient with LPD with 15 years of follow-up in our hospital, with no evidence of malignancy. Conclusion: Knowing the generally benign nature of this disease, it is necessary to opt for the least invasive approach possible. The long-term evolution of this disease is unknown, as most published cases do not have sufficient follow-up time.(AU)
Subject(s)
Humans , Female , Diagnosis, Differential , Leiomyomatosis/diagnosis , Leiomyomatosis/drug therapy , Neoplasms , Gynecology , Genital Diseases, FemaleABSTRACT
BACKGROUND: In a recent report it was shown that molecular subgroups of early (pT1) breast tumors belonging exclusively to the most common histological variant, ductal-infiltrating carcinoma, showed significantly different clinical and biological features. OBJECTIVE: To test in a series of patients with ductal-infiltrating carcinoma encompassing all stages of the disease if the above-mentioned biological differences already detected at the earliest stage are ultimately reflected in survival differences. PATIENTS AND METHODS: All patients with ductal infiltrating carcinoma operated upon at Hospital de Móstoles, Madrid, Spain, between 1997 and 2002 were included into the study, to allow for at least five years of follow-up for survivors. Of 242 studied patients, according to the International Federation of Gynecology and Obstetrics (FIGO) classification, 37.6% were in stage I, 47.2% in stage II, 11.0% in stage III, and 4.2% in stage IV. According to the simplified molecular classification of Carey et al., 116 tumors (47.9%) expressed estrogen receptors, and did not express c-erb-B2 (Luminal A), 67 (27.7%) coexpressed hormone receptors (either estrogen receptors, progesterone receptors or both) and c-erb-B2 (Luminal B), 33 (13.3%) expressed c-erb-B2 in the absence of hormone receptors (HER-2), and 26 (10.7%) neither expressed hormone receptors, nor c-erb-B2 (triple-negative, basal). RESULTS: In a univariate model, both disease-free survival and overall survival of the patients were significantly associated with stage (p=0.0003; p<0.0001), histological grade (p<0.0001; p<0.0001), lymphatic vascular space invasion (p=0005; p=0.0044), menopausal status (p=0.04; p=0.034) and molecular subgrouping (p=0.037: p=0.01). In a multivariate model, only stage (p=0.013), grade (p<0.0001), and menopausal status (p=0.007) retained their prognostic power for predicting disease-free survival, and just stage (p<0.0001) and grade (p<0.0001) for predicting overall survival. The molecular classification of the tumors almost reached statistical significance for predicting overall survival (p=0.06).
