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肾上腺皮质癌的局部区域复发:一例报告

Loco-regional recurrence of adrenocortical carcinoma: A case report.

作者信息

Sah Bikash Chandra, Luitel Prajjwol, Shrestha Sundar, Shrestha Mandesh, Subedi Nirajan, Pokhrel Sharad

机构信息

Department of Surgical Gastroenterology, National Academy of Medical Sciences, Bir Hospital, Nepal.

Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, Nepal.

出版信息

Int J Surg Case Rep. 2024 Sep;122:110095. doi: 10.1016/j.ijscr.2024.110095. Epub 2024 Jul 30.

Abstract

INTRODUCTION

Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with a high recurrence rate. Approximately half of the patients are asymptomatic, while others experience symptoms due to the tumor's size or hormone secretion. Ro resection if possible is the best option for treatment of primary as well as locoregional recurrent ACC.

CASE PRESENTATION

A 20-year-old female who previously underwent open left adrenalectomy for Stage III ACC presented with complaints of heaviness and vague discomfort in the left upper abdomen. Current hormonal assays were normal. Imaging revealed a lesion in the spleen suggestive of recurrence. She underwent elective surgery involving en bloc resection of the spleen, diaphragm, and associated structures. Postoperative recovery was uneventful, histopathology confirmed recurrence and subsequent PET-CT showed no recurrence. She is currently on mitotane and remains symptom-free with no signs of recurrence after initial surgery.

CLINICAL DISCUSSION

Complete resection (Ro) if possible, for recurrent and metastatic disease has been linked to long-term survival and offers significant palliative benefits, particularly in cases involving symptomatic steroid production.

CONCLUSION

ACC has a high frequency of local recurrence therefore management of recurrence should be considered from the initial diagnosis. Ro resection of recurrence is the best potential treatment. Follow-up protocols and improving integration between surgical, oncological, and supportive care departments are crucial for overcoming healthcare challenges in Nepal.

摘要

引言

肾上腺皮质癌(ACC)是一种罕见且侵袭性强的内分泌恶性肿瘤,复发率高。约半数患者无症状,而其他患者则因肿瘤大小或激素分泌出现症状。若有可能,根治性切除是原发性及局部复发性ACC的最佳治疗选择。

病例介绍

一名20岁女性,曾因III期ACC接受左侧肾上腺开放切除术,现主诉左上腹沉重及隐痛不适。目前激素检测正常。影像学检查显示脾脏有一病变,提示复发。她接受了择期手术,包括整块切除脾脏、膈肌及相关结构。术后恢复顺利,组织病理学证实复发,随后的PET-CT显示无复发。她目前正在服用米托坦,初始手术后无症状,无复发迹象。

临床讨论

对于复发和转移性疾病,若有可能进行根治性切除(Ro)与长期生存相关,并具有显著的姑息治疗益处,特别是在涉及有症状的类固醇分泌的病例中。

结论

ACC局部复发频率高,因此应从初始诊断时就考虑复发的管理。复发的Ro切除是最佳的潜在治疗方法。随访方案以及改善外科、肿瘤学和支持护理部门之间的协作对于克服尼泊尔的医疗挑战至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9945/11375248/82d4e13183d7/gr1.jpg

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