Ha Minah, Jamieson Amy, Pickett Justine, McGinnis Justin M, De Greve Tom
Gynaecological Oncology, National Women's Health, Auckland City Hospital, Auckland, New Zealand.
Department of Gynaecology and Obstetrics, Division of Gynaecologic Oncology, University of British Columbia, Vancouver, Canada.
Gynecol Oncol Rep. 2024 Aug 18;55:101488. doi: 10.1016/j.gore.2024.101488. eCollection 2024 Oct.
Pseudomyxoma peritonei (PMP) is a clinical syndrome characterised by intraperitoneal accumulation of mucus due to mucinous neoplasia. It is a rare condition affecting 1-2 per million individuals per year. The majority of PMP arises from a ruptured mucinous appendiceal tumour, with infrequent occurrences from other primary gastrointestinal tumours and mucinous ovarian tumours. PMP arising from a mature ovarian teratoma is a rare entity, with limited case reports in the literature. Given the infrequent and sporadic occurrences of these tumours, little is known about the tumour behaviour and prognosis.
Herein, we report six cases of PMP arising from a mature ovarian teratoma who were treated with primary cytoreductive surgery (CRS), with one case of recurrence. Literature review identified 21 cases from 12 manuscripts. Nineteen patients were treated with CRS alone, with two patients receiving adjuvant hyperthermic intraperitoneal chemotherapy (HIPEC). Follow up data were variably reported, with no recurrence in 20 patients during their follow up of 5-54 months. One patient reported to have died of disease at 49 months.
Despite the lack of high-quality evidence and limitations of small case series, our review indicates that close surveillance after CRS could be considered as the preferred treatment over more morbid CRS and HIPEC, with HIPEC reserved for patients who recur or progress after CRS.
腹膜假黏液瘤（PMP）是一种临床综合征，其特征是由于黏液性肿瘤导致腹腔内黏液积聚。这是一种罕见疾病，每年影响百万分之一至二的人群。大多数PMP起源于破裂的黏液性阑尾肿瘤，其他原发性胃肠道肿瘤和黏液性卵巢肿瘤引起的情况较少见。起源于成熟卵巢畸胎瘤的PMP是一种罕见实体，文献中病例报告有限。鉴于这些肿瘤罕见且散在发生，对其肿瘤行为和预后知之甚少。
在此，我们报告6例起源于成熟卵巢畸胎瘤的PMP患者，他们接受了初次肿瘤细胞减灭术（CRS），其中1例复发。文献综述从12篇手稿中确定了21例病例。19例患者仅接受了CRS治疗，2例患者接受了腹腔内热灌注化疗（HIPEC）辅助治疗。随访数据报告不一，20例患者在5至54个月的随访期间无复发。1例患者在49个月时报告死于该疾病。
尽管缺乏高质量证据且小病例系列存在局限性，但我们的综述表明，CRS后密切监测可被视为比更具创伤性的CRS和HIPEC更优的首选治疗方法，HIPEC适用于CRS后复发或进展的患者。
