Ultrasound Department, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Department of Pathology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Medicine (Baltimore). 2023 May 19;102(20):e33850. doi: 10.1097/MD.0000000000033850.
Only 20 cases of pediatric primary renal non-Hodgkin's lymphoma have been reported since 1995, rare cases and a variety of imaging manifestations have led to difficulties in its diagnosis and treatment.
Herein, we share in detail a case of primary renal lymphoma (PRL) in a child and summarize the common clinical manifestations, imaging features, and prognostic factors of pediatric PRL by retrospectively analyzing cases reported in the literature. A 2-year-old boy presented to the clinic with a large mass on the right side of his abdomen along with loss of appetite.
Imaging revealed a large right renal mass, nearly replacing the entire renal tissue, along with numerous small nodules in the left kidney. Given no local adenopathy and metastases, the diagnosis was unclear. A percutaneous renal puncture was performed, which proved the diagnosis of Burkitt's lymphoma. Since no bone marrow involvement, this child was diagnosed with pediatric PRL.
This PRL boy was treated with the NHL-BFM95 protocol and supportive care.
Unfortunately, this boy died of multiple organ failure in the fifth month of treatment.
As per literature review, the presentation of pediatric PRL is fatigue, loss of appetite, weight loss, abdominal swelling, or other nonspecific symptoms. Although in 81% of cases it often infiltrates the bilateral kidneys, urine abnormalities caused by pediatric PRL are uncommon. 76.2% of pediatric PRL were boys and 2/3 of all cases presented as diffuse renal enlargement. Those PRL presented as masses could easily be misdiagnosed as WT or other malignancies. Absent of local enlarged lymph node, no necrosis or calcification suggest atypical presentation of renal masses and a percutaneous biopsy is needed in timely establishing the accurate diagnosis for appropriate treatment. Based on our experience, percutaneous renal puncture core biopsy is a safe procedure.
自 1995 年以来，仅报道了 20 例儿童原发性肾非霍奇金淋巴瘤病例，罕见病例和多种影像学表现导致其诊断和治疗困难。
本文详细介绍了一例儿童原发性肾淋巴瘤（PRL）病例，并通过回顾性分析文献报道的病例，总结了儿童 PRL 的常见临床表现、影像学特征和预后因素。一名 2 岁男孩因右侧腹部肿块和食欲不振就诊。
右肾巨大肿块，几乎取代整个肾组织，左肾多个小结节。由于无局部淋巴结肿大和转移，诊断不明确。行肾穿刺活检，证实为伯基特淋巴瘤。由于无骨髓受累，该患儿被诊断为儿童 PRL。
该 PRL 患儿采用 NHL-BFM95 方案和支持治疗。
不幸的是，该患儿在治疗第 5 个月因多器官衰竭死亡。
根据文献复习，儿童 PRL 的表现为疲劳、食欲不振、体重减轻、腹部肿胀或其他非特异性症状。虽然 81%的病例常累及双侧肾脏，但儿童 PRL 引起的尿液异常并不常见。76.2%的儿童 PRL 为男性，2/3的病例表现为弥漫性肾增大。表现为肿块的 PRL 容易误诊为 WT 或其他恶性肿瘤。无局部肿大淋巴结、无坏死或钙化提示肾肿块的不典型表现，需要及时进行经皮肾穿刺活检以明确准确诊断，从而进行适当治疗。根据我们的经验，经皮肾穿刺活检是一种安全的操作。
