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病例报告:原发性卵巢伯基特淋巴瘤:儿科人群中的一个令人困惑的病例

Case report: Primary ovarian Burkitt's lymphoma: A puzzling scenario in pediatric population.

作者信息

Persano Giorgio, Crocoli Alessandro, Martucci Cristina, Vinti Luciana, Cassanelli Giulia, Stracuzzi Alessandra, Cardoni Antonello, Inserra Alessandro

机构信息

Surgical Oncology Unit, Department of Surgery, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy.

General Surgery Unit, Department of Surgery, Bambino Gesù Children's Hospital - IRCCS, Rome, Italy.

出版信息

Front Pediatr. 2023 Jan 11;10:1072567. doi: 10.3389/fped.2022.1072567. eCollection 2022.

Abstract

Burkitt's lymphoma (BL) is defined as a highly invasive B-cell lymphoma, usually characterized by an excellent prognosis, more than 90% of children and adolescents being cured with highly dose-intensive multiagent chemotherapy. Primary ovarian localization without involvement of other organs is a rare manifestation of BL, especially in pediatric population. Symptoms at diagnosis are similar to other ovarian lesions and differential diagnosis may be challenging for clinicians. A 12-year-old girl was referred to our institution for abdominal pain and palpable mass observed by the pediatrician. Diagnostic work-up demonstrated a large mass arising from the right ovary, causing compression on abdominal aorta, inferior vena cava, ureters and bowel, with a second smaller lesion on the left ovary. At surgery, a 15 cm-large, ruptured mass arising from the right ovary was found, associated with a second lesion originating from the left ovary (8 cm) and multiple nodules of the greater omentum. Right salpingo-oophorectomy was performed, incisional biopsies were taken from the left ovary and omental nodules and peritoneal fluid samples were collected for cytology. Pathology revealed a Burkitt lymphoma and the patient underwent chemotherapy according to AIEOP LNH-97 Protocol, group R3 with . Preoperative diagnosis of primary ovarian lymphoma is extremely difficult. Surgical exploration is often necessary in patients presenting with acute abdominal or pelvic pain; when the suspicion of primary ovarian lymphoma arises intraoperatively, every effort should be made to minimize invasive procedure in order to enhance post-operative recovery.

摘要

伯基特淋巴瘤(BL)被定义为一种高度侵袭性的B细胞淋巴瘤,通常预后良好,超过90%的儿童和青少年通过高剂量密集多药化疗得以治愈。原发性卵巢定位且无其他器官受累是BL的一种罕见表现,尤其是在儿科人群中。诊断时的症状与其他卵巢病变相似,临床医生进行鉴别诊断可能具有挑战性。一名12岁女孩因腹痛被转诊至我院,儿科医生发现可触及肿块。诊断检查显示右卵巢有一个大肿块,压迫腹主动脉、下腔静脉、输尿管和肠道,左卵巢有一个较小的病变。手术中,发现一个来自右卵巢的15厘米大的破裂肿块,伴有一个来自左卵巢的第二个病变(8厘米)和大网膜上的多个结节。进行了右侧输卵管卵巢切除术,从左卵巢和网膜结节取了切口活检,并收集了腹腔液样本进行细胞学检查。病理显示为伯基特淋巴瘤,患者根据AIEOP LNH - 97方案,R3组接受化疗。原发性卵巢淋巴瘤的术前诊断极其困难。对于出现急性腹痛或盆腔疼痛的患者,手术探查往往是必要的;当术中怀疑原发性卵巢淋巴瘤时,应尽一切努力尽量减少侵入性操作,以促进术后恢复。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/20c4/9875037/5217ccebe808/fped-10-1072567-g001.jpg

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