Primary retroperitoneal carcinomas are very rare tumors. Their pathogenesis remains unknown but may be associated with that of ovarian carcinomas, considering the similarity in morphology and gender preference. Although metaplasia of coelomic epithelium is the most widely accepted theory, the pathogenesis of retroperitoneal carcinomas may differ by histologic subtype, like ovarian carcinomas. Mucinous carcinoma, which develops in both women and men, may originate in both primordial germ cells and Walthard cell nests that may be derived from the fallopian tube. Serous carcinomas may be associated with endosalpingiosis, the presence of fallopian tube-like epithelium outside the fallopian tube, and a remnant Müllerian tract. Endometrioid and clear cell carcinomas appear to be associated with extraovarian endometriosis. Additionally, both carcinomas in the retroperitoneal lymph nodes may be metastatic diseases from endometrial and/or renal cell cancer that regress spontaneously (carcinoma of unknown primary). Retroperitoneal carcinomas are difficult to diagnose, as they have no characteristic symptoms and signs. Surgery is the cornerstone of treatment, but the necessity of chemotherapy may depend on histological subtype. Further studies are necessary, in particular studies on endosalpingiosis, as endosalpingiosis is a poorly understood condition, although it is associated with the development of both serous and mucinous carcinomas.
原发性腹膜后癌是极为罕见的肿瘤。其发病机制尚不明确,但由于在形态学和性别偏好方面与卵巢癌相似,其发病机制可能与卵巢癌相关。尽管体腔上皮化生理论最为广泛接受,但腹膜后癌的发病机制可能因组织学亚型而异,这与卵巢癌类似。黏液性癌在女性和男性中均可发生,可能起源于原始生殖细胞和可能来源于输卵管的瓦尔塔德细胞巢。浆液性癌可能与输卵管内膜异位症(即输卵管样上皮存在于输卵管外)以及残留的苗勒管有关。子宫内膜样癌和透明细胞癌似乎与卵巢外子宫内膜异位症相关。此外,腹膜后淋巴结中的这两种癌也可能是子宫内膜癌和/或肾细胞癌自发消退后的转移性疾病(原发灶不明癌)。腹膜后癌难以诊断,因其缺乏特征性症状和体征。手术是治疗的基石,但化疗的必要性可能取决于组织学亚型。需要进一步研究,特别是对输卵管内膜异位症的研究,因为尽管输卵管内膜异位症与浆液性癌和黏液性癌的发生相关,但目前对其认识仍不足。