Mesotelioma peritoneal multiquístico benigno: reporte de un caso y revisión de la literatura / Benign multicystic peritoneal mesothelioma: case report and literature review

INTRODUCCIÓN: El mesotelioma peritoneal multiquístico benigno (MPMB) es una entidad con muy baja incidencia que se presentan con mayor frecuencia en mujeres jóvenes. CASO CLÍNICO: Paciente de 17 años que consulta por amenorrea secundaria. Además, refería pérdida de peso, estreñimiento y molestias abdominales. La exploración fue normal y en las pruebas de imagen se objetivó una pelvis ocupada por una masa multiquística, de unos 20 cm de diámetro máximo, de paredes finas y contenido anecoico. Se solicitaron marcadores tumorales de los cuales sólo se encontró elevado el CA 19.9 (35,2 U/mL). Se decidió realizar una laparoscopia exploradora para establecer un diagnóstico y ante los hallazgos y al considerarse la masa fácilmente resecable, en el mismo acto quirúrgico se extirpó por completo para su estudio anatomopatológico, que confirmó un MPMB. Se desestimaron tratamientos adicionales y actualmente se encuentra en seguimiento y asintomática. CONCLUSIÓN: La patogénesis del MPMB no está clara. Algunos autores defienden que se trata de una reacción peritoneal a un daño inflamatorio, otros apoyan que se trata de una neoplasia benigna. Esta naturaleza incierta, junto con la escasa experiencia, hacen que el manejo resulte complejo. Es necesario individualizar cada caso e intentar ser conservadores especialmente en pacientes jóvenes. Si se opta por un manejo activo, lo más acertado es la citorreducción completa seguida de quimioterapia hipertérmica peritoneal. El seguimiento, en cualquier caso, debe ser exhaustivo y multidisciplinar principalmente con pruebas de imagen.

BACKGROUND: Benign Multicystic Peritoneal Mesothelioma (BMPM) is an entity with a very low incidence that occurs more frequently in young women. CASE REPORT: A 17-year-old woman attended for secondary amenorrhea. She also referred to weight loss, constipation and abdominal discomfort. The examination was normal and echography revealed a pelvis occupied by a 20 centimeters multicystic mass, with thin walls and anechoic content. A preoperative study with tumor markers was requested in which only a CA 19.9 elevation was observed (35.2 U/ml 0-27). It was decided to perform an exploratory laparoscopy to establish a diagnosis. Due to the findings and because of the fact that the mass was considered easy to remove, it was completely removed in the same surgical act. The pathological result confirmed a BMPM. Additional treatments have been dismissed and are the patient is currently being followed up without symptoms. CONCLUSION: The pathogenesis of BMPM is unclear. Some authors argue that it is a peritoneal reaction to inflammatory damage, others support that it is a benign neoplasm. This uncertain nature coupled with limited experience make management complex. It is necessary to evaluate each case and try to be conservative especially in young patients. If surgery management is chosen, complete cytoreduction followed by hyperthermic peritoneal chemotherapy is the most successful option. The follow-up in any case must be exhaustive and multidisciplinary, mainly with imaging tests.

Benign Multicystic Omental Mesothelioma in Pregnancy: A Case Report and Review of the Literature.

Benign Multicystic Peritoneal Mesothelioma (BMPM) is a very rare condition of unknown pathogenesis, that occurs mainly in women in their reproductive age (5:1 female/male ratio). The pathogenesis of BMPM is unclear and doubts regarding its neoplastic and reactive nature exist. Furthermore, the differential diagnosis with other diseases remains uncertain. In most cases the diagnosis is accidental, during a laparotomy performed for other reasons, since the diagnostic imaging does not allow a definite diagnosis preoperatively. Radical surgical resection is the “gold standard” for treatment of BMPM, despite a high rate of recurrence (about 50%). We report on a case of a 30-year-old pregnant woman with multilocular omental cystic mass, 36 x 22 cm in size, underwent elective caesarean section at term of pregnancy. The definitive histologic diagnosis was benign multicystic mesothelioma of the omentum. BMPM detected in pregnancy or during cesarean delivery is very rare; in fact only three other cases have been reported. Particularly, we present the only case, described in the literature, of a young female with benign multicystic omental mesothelioma in pregnancy that was treated solely by surgery, without recurrence after three years follow-up. In this article we also describe diagnostic evaluation, treatment, prognosis of this rare disease as the possible positive effects of adjuvants medical treatments.