Ranieri Girolamo, Porcelli Mariangela, Mastrorosa Alessandro, Di Palo Alessandra, Ferrari Cristina, Zito Alfredo Francesco, Ammendola Michele, Laface Carmelo, Mastrandrea Giovanni, Berardone Simona
Unità Operativa di Oncologia Integrata, IRCCS Istituto Tumori "Giovanni Paolo II", Bari, Italy.
Unità Operativa di Oncologia Urologica, IRCCS Istituto Tumori "Giovanni Paolo II", Bari, Italy.
Front Oncol. 2025 Sep 2;15:1610783. doi: 10.3389/fonc.2025.1610783. eCollection 2025.
Müllerian adenocarcinoma is a very rare and aggressive cancer originating from the uterus and ovary, affecting almost exclusively women. Few cases of extragenital Müllerian adenocarcinomas have been reported, in various locations ranging from pelvic peritoneum to diaphragm peritoneum. Very few cases of Müllerian adenocarcinoma in men have been reported in scientific literature, usually localized in the prostate seminal vesicles and testicles, associated to a very poor prognosis.
In this paper, we describe the unique clinical case of a man affected by advanced Müllerian adenocarcinoma on the left side of the pelvis, treated with a combination of surgery, chemotherapy, and radiotherapy, with a long follow-up. Patient survival was exceptional (almost 15 years from diagnosis), and the patient experienced a good quality of life during the numerous treatments. To the best of our knowledge, this is the first case report regarding a Müllerian adenocarcinoma of the pelvis in a man treated with a multimodal therapy approach and with a very long follow-up and very long survival. In consideration of the embryological origin of ovaries from Müllerian ducts and the absence of specific guidelines for standard treatment for this tumor, the patient was treated as if he had ovarian cancer, with optimal results.
The management of this patient with modern available lines of chemotherapy classically employed in ovarian cancer plus radiotherapy combined with several bouts of cytoreductive surgery could explain this long survival.
苗勒管腺癌是一种非常罕见且侵袭性强的癌症，起源于子宫和卵巢，几乎仅影响女性。文献报道的发生于盆腔腹膜至膈肌腹膜等不同部位的生殖器外苗勒管腺癌病例很少。科学文献中报道的男性苗勒管腺癌病例极少，通常局限于前列腺、精囊和睾丸，预后很差。
在本文中，我们描述了一名患有盆腔左侧晚期苗勒管腺癌男性的独特临床病例，该患者接受了手术、化疗和放疗联合治疗，并进行了长期随访。患者存活情况异常（自诊断起近15年），且在多次治疗期间生活质量良好。据我们所知，这是首例关于采用多模式治疗方法、进行长期随访且存活期很长的男性盆腔苗勒管腺癌的病例报告。鉴于卵巢起源于苗勒管且缺乏针对该肿瘤的标准治疗的具体指南，该患者被当作卵巢癌患者进行治疗，结果理想。
采用卵巢癌经典化疗方案联合放疗并多次进行减瘤手术来治疗该患者，或许可以解释其长期存活的原因。
