Department of Orthopedics, The First Affiliated Hospital, Zhejiang University, Hangzhou, P. R. China.
Department of Basic Medicine, School of Basic Medicine and Clinical Pharmacy, China Pharmaceutical University, Nanjing, P. R. China.
Yale J Biol Med. 2021 Dec 29;94(4):613-622. eCollection 2021 Dec.
: Desmoplastic small round cell tumor (DSRCT) is an aggressive malignant tumor commonly found in young men; most occurs in the abdominal cavity. Here we conducted an in-depth analysis of a pregnant patient in our hospital and explored all the case information in the literature on small round cell carcinoma of women. : A 27-year-old pregnant woman underwent tumor resection in our hospital at 29 weeks gestational age for a large progressive shoulder lump. The postoperative pathology showed that the mass was a DSRCT. Genetic testing found no fusion gene. At 36 weeks gestation, a painful mass was found in the breast and proved to be a metastatic focus of the desmoplastic small round cell tumor. Twenty days after a successful cesarean section at 40 weeks gestation, she received the VAC-IE chemotherapy regimen, successfully completed the first course, but when awaiting the next chemotherapy, unfortunately, the patient died during follow-up due to tumor recurrence and metastasis. : The treatment of DSRCT in pregnant women requires a multidisciplinary consultation, and the treatment and examination during pregnancy are subject to many constraints, which may have a negative impact on the patient's prognosis. Also, a review of the literature found that there is still no standard treatment protocol for DSRCT, and its prognosis in female patients is independent of age and tissue origin.
促结缔组织增生性小圆细胞肿瘤(DSRCT)是一种常见于年轻男性的侵袭性恶性肿瘤,大多数发生在腹腔内。在此,我们对我院收治的 1 例妊娠患者进行了深入分析,并对女性小圆细胞癌的所有文献病例资料进行了探讨。
一位 27 岁的孕妇在我院于 29 孕周时因肩部进行性增大肿块行肿瘤切除术。术后病理提示为 DSRCT。基因检测未发现融合基因。36 孕周时,发现乳房有一疼痛性肿块,证实为促结缔组织增生性小圆细胞肿瘤转移灶。40 孕周剖宫产术后 20 天行 VAC-IE 化疗方案,顺利完成第 1 疗程,但在等待下一次化疗时,不幸的是,患者在随访中因肿瘤复发和转移而死亡。
妊娠合并 DSRCT 的治疗需要多学科会诊,妊娠期的治疗和检查受到诸多限制,这可能对患者的预后产生负面影响。此外,文献复习发现,DSRCT 目前仍没有标准的治疗方案,且女性患者的预后与年龄和组织来源无关。