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播散性腹膜平滑肌瘤病:一种具有诊断和治疗挑战的良性疾病。

Disseminated peritoneal leiomyomatosis: A benign condition with diagnostic and therapeutic challenges.

作者信息

Akanbi Olusola O, Omoregie Paul, Akunaeziri Uche A, Wakeel Muritala O, Iyabor Nelson O, Akanni Oluwasegun A

机构信息

Surgery Department, Federal Medical Centre Keffi, Keffi, Nigeria *Email:

Obstetrics and Gynaecology Department, Federal Medical Centre Keffi, Keffi, Nigeria.

出版信息

Qatar Med J. 2024 Sep 24;2024(3):40. doi: 10.5339/qmj.2024.40. eCollection 2024.

DOI:10.5339/qmj.2024.40
PMID:39372685
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11450273/
Abstract

BACKGROUND

Disseminated peritoneal leiomyomatosis (DPL) is a variant of parasitic leiomyomas that is characterized by multiple peritoneal and subperitoneal nodules of proliferating smooth muscle cells that histologically resemble uterine leiomyoma. We report a case of recurrent DPL to highlight its diagnostic and therapeutic challenges at the Federal Medical Centre, Keffi, Nigeria.

CASE REPORT

The patient is a 25-year-old woman with a previous history of myomectomy 3 years before presentation to the hematology unit on account of abdominal lymphoma. Based on the working diagnosis, she was referred to the general surgery unit for an open biopsy and cytoreductive surgery. She was explored, and intraoperative findings were in keeping with multiple well-circumscribed intra-abdominal masses of varying sizes. The multiple and widespread locations of the masses precluded the complete removal of the masses. Four months post-surgery, she presented with similar lesions and had a repeat laparotomy. At the surgery, she had a total abdominal hysterectomy with bilateral salpingophorectomy and excision of the abdominal masses. She was then placed on letrozole, which prevented further tumor growth and abated her symptoms.

DISCUSSION

DPL is often rarely diagnosed preoperatively and thus poses a diagnostic challenge, with many cases asymptomatic and therapeutic challenges due to its tendency to recur. Its management currently lacks consensus and is often determined by many factors, such as the age of the patients, the number of nodules, and the desire to have more children, among others. Surgical excision combined with hormonal therapy is recommended for patients who wish to conceive. For postmenopausal women and those who no longer desire conception, total abdominal hysterectomy with bilateral salpingo-oophorectomy should be considered to prevent recurrence.

CONCLUSION

DPL is a rare form of multiple extrauterine leiomyomas. We report a case of DPL in a woman that was managed with surgical intervention and hormonal manipulation therapy following the failure of the initial surgical excision alone. We thus suggest a combination of surgical intervention and postoperative hormonal manipulation in its management, as such a multi-modality of therapy was employed in the index case without evidence of recurrence after a year post-surgery.

摘要

背景

播散性腹膜平滑肌瘤病(DPL)是寄生性平滑肌瘤的一种变体,其特征是多个腹膜和腹膜下结节,由增生的平滑肌细胞构成,组织学上类似于子宫平滑肌瘤。我们报告一例复发性DPL病例,以突出其在尼日利亚凯菲联邦医疗中心的诊断和治疗挑战。

病例报告

该患者为一名25岁女性,3年前因腹部淋巴瘤到血液科就诊前有子宫肌瘤切除术史。基于初步诊断,她被转诊至普通外科进行开放活检和肿瘤细胞减灭术。对她进行了探查,术中发现符合多个大小不一、边界清晰的腹腔内肿块。肿块数量众多且分布广泛,无法完全切除。术后4个月,她出现了类似病变,再次接受剖腹手术。手术中,她接受了全腹子宫切除术、双侧输卵管卵巢切除术及腹部肿块切除术。然后她开始服用来曲唑,该药阻止了肿瘤进一步生长并缓解了她的症状。

讨论

DPL术前常难以诊断,因此构成诊断挑战,许多病例无症状,且由于其复发倾向也带来治疗挑战。其治疗目前缺乏共识,常由多种因素决定,如患者年龄、结节数量以及生育意愿等。对于希望怀孕的患者,建议手术切除并联合激素治疗。对于绝经后女性和不再希望怀孕的女性,应考虑行全腹子宫切除术及双侧输卵管卵巢切除术以预防复发。

结论

DPL是一种罕见的多发性子宫外平滑肌瘤形式。我们报告了一例女性DPL病例,在单纯初始手术切除失败后,采用手术干预和激素治疗相结合的方法进行管理。因此,我们建议在其治疗中采用手术干预和术后激素治疗相结合的方法,因为在该病例中采用了这种多模式治疗,术后一年无复发迹象。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/55855d8fc3e6/qmj-2024-03-040-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/55c2899dbf01/qmj-2024-03-040-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/ad3a5843e5e9/qmj-2024-03-040-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/08f70c6ab0f4/qmj-2024-03-040-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/55855d8fc3e6/qmj-2024-03-040-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/55c2899dbf01/qmj-2024-03-040-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/ad3a5843e5e9/qmj-2024-03-040-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/08f70c6ab0f4/qmj-2024-03-040-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a894/11450273/55855d8fc3e6/qmj-2024-03-040-g004.jpg

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