Non-Peutz-Jeghers syndrome-associated ovarian sex cord tumor with annular tubules treated by radiotherapy: a case report and literature review.

非 Peutz - Jeghers 综合征相关卵巢性索肿瘤伴环状小管放疗 1 例并文献复习.

• 影响指数: 1.573
• 发表时间: Mar 2021
• 来源期刊: J Int Med Res
• DOI: 10.1177/0300060521996563
• PMID: 33715493
• 文章类型: Case Reports
• 中科院分区: Q4
• 方向: 医学
• 作者列表: Li C,Aishajiang R,Teng Y,Xu T,Ding L,Dong L

关键词: Ovarian tumors; Peutz–Jeghers syndrome; metastasis; radiotherapy; sex cord tumor with annular tubules; surgery

Mesh: Estrogens; Female; Humans; Ovarian Neoplasms; Peutz-Jeghers Syndrome / complications; Prospective Studies; Sex Cord-Gonadal Stromal Tumors / radiotherapy

Abstract

There are no standard treatment options for metastatic and recurrent non-Peutz-Jeghers syndrome (PJS)-associated sex cord tumor with annular tubules (SCTAT). The effects of chemotherapy and/or radiotherapy are still not well-defined. Herein, we present a case of a metastatic and recurrent non-PJS-associated SCTAT showing high serum estradiol and progesterone concentrations after surgery and chemotherapy. Radiotherapy (50 Gy/25 fractions) triggered a sharp reduction in the sizes of the metastatic and recurrent masses, and estradiol and progesterone concentrations. Accordingly, we consider that radiotherapy might be effective and safe for metastatic and recurrent SCTAT. The roles of radiotherapy in non-PJS SCTAT should be further validated in large-scale prospective clinical trials.

摘要

对于转移性和复发性非Peutz-Jeghers综合征（PJS）相关的性索肿瘤和环形小管（SCTAT），没有标准的治疗选择。化疗和/或放疗的效果仍然不明确。在这里，我们介绍了一例转移性和复发性非PJS相关SCTAT,显示手术和化疗后血清雌二醇和孕酮浓度高.放射治疗（50Gy/25分）导致转移性和复发性肿块的大小急剧减少，雌二醇和孕酮浓度。因此，我们认为放疗对于转移性和复发性SCTAT可能是有效和安全的。放疗在非PJSSCTAT中的作用应在大规模前瞻性临床试验中进一步验证。