Hysteroscopic treatment of uterine tumor resembling ovarian sex cord-like tumor (UTROSCT). (2nd November 2015)

• Record Type: Journal Article
• Authors: Watrowski, Rafal; Jäger, Christoph; Möckel, Jochen; Kurz, Philipp; Schmidt, Dietmar; Freudenberg, Nikolaus
• Is Part Of: Gynecological endocrinology. Volume 31:Number 11(2015:Nov.)
• Page Start: 856
• Page End: 859
• Publication Date: 2015-11-02
• Subjects: Hysteroscopy -- low malignant potential -- uterus preserving -- uterine tumors -- UTROSCT; Endocrine gynecology -- Periodicals; Generative organs, Female -- Diseases -- Periodicals; 618.1
• DOI: 10.3109/09513590.2015.1080682
• Languages: English
• ISSNs: 0951-3590

Abstract

Abstract: The decision to preserve the uterus in a young nulliparous woman with an extremely rare tumor is challenging. Uterine tumor resembling ovarian sex cord-like tumor (UTROSCT) belongs to the rarest uterine pathologies. A 22-year-old nulligravida with uterine bleeding underwent a hysteroscopic resection of an intrauterine mass presumed as grade-1 submucous myoma. According to the presence of sex cord-like differentiation and positivity for calretinin, CD99, estrogen receptor, vimentin, WT1 and Melan-A, the tumor was diagnosed as UTROSCT. After 28 months, without any adjuvant therapy, the patient is still free of recurrence. This is the youngest patient with UTROSCT reported so far, with the longest follow-up among all five cases treated via hysteroscopy. Although UTROSCT has been traditionally treated with hysterectomy (with or without bilateral salpingo-oophorectomy), no established treatment protocol for UTROSCT exists. UTROSCT shows a low-malignant potential, but metastasizing and recurrent cases occur. In light of the probably less aggressive tumor biology and with respect to the patient's autonomy, a conservative, uterus preserving treatment appears to be justified in selected cases in which close follow-up can be guaranteed. Further case reports are needed to prove the safety of organ-preserving strategy in UTROSCT. Chinese abstract: 决定保留一位患有极其罕见肿瘤的年轻未育女性的子宫是具有挑战性的。似卵巢性索样肿瘤子宫肿瘤(UTROSCT)属于最罕见的子宫疾病。一位22岁的子宫出血未育女性行宫腔镜下宫内肿物（被推测为1型粘膜下肌瘤）切除术。根据性索样分化的出现以及钙结合蛋白、CD99、雌激素受体、波形蛋白、WT1和Melan-A均阳性，这个肿瘤被诊断为似卵巢性索样肿瘤子宫肿瘤(UTROSCT)。28个月后，尽管无任何辅助治疗，患者仍无复发。这是目前报道的五例经宫腔镜治疗中随访时间最长、最年轻的似卵巢性索样肿瘤子宫肿瘤患者。虽然似卵巢性索样肿瘤子宫肿瘤历来行子宫切除术（伴或不伴双侧输卵管卵巢切除术），但并没有确定的治疗方案存在。似卵巢性索样肿瘤子宫肿瘤呈低度恶性潜能，但有转移和复发病例发生。根据较小可能侵袭性的肿物生物学和尊重患者的自主权，在可以保证密切随访的病例中采取保留子宫的保守治疗似乎是合理的。尚需进一步的病例报告证明在似卵巢性索样肿瘤子宫肿瘤中保留器官的安全性。