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Abstract：
BACKGROUND: 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.
METHODS: 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.
METHODS: 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.
METHODS: This PRL boy was treated with the NHL-BFM95 protocol and supportive care.
RESULTS: Unfortunately, this boy died of multiple organ failure in the fifth month of treatment.
CONCLUSIONS: 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.
METHODS: 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.
METHODS: 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.
METHODS: This PRL boy was treated with the NHL-BFM95 protocol and supportive care.
RESULTS: Unfortunately, this boy died of multiple organ failure in the fifth month of treatment.
CONCLUSIONS: 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.
摘要：
背景：自1995年以来，仅有20例小儿原发性肾脏非霍奇金淋巴瘤报道，罕见病例和多种影像学表现导致其诊断和治疗困难。
方法：这里，我们详细分享了一例儿童原发性肾淋巴瘤（PRL），并总结了其常见的临床表现，成像特征,通过对文献报道的病例进行回顾性分析，了解小儿PRL的预后因素。一名2岁男孩出现在诊所，腹部右侧有一个大肿块，食欲不振。
方法：成像显示右肾大肿块，几乎取代了整个肾脏组织，在左肾有许多小结节。鉴于没有局部淋巴结肿大和转移，诊断不清楚。进行了经皮肾穿刺,这证明了伯基特淋巴瘤的诊断。因为没有骨髓受累,这个孩子被诊断为小儿PRL.
方法：这名PRL男孩接受NHL-BFM95方案和支持治疗。
结果：不幸的是，这个男孩在治疗的第五个月死于多器官衰竭。
结论：根据文献综述，小儿PRL的表现是疲劳，食欲不振,减肥,腹部肿胀,或其他非特异性症状。尽管在81%的病例中，它经常浸润双侧肾脏，由小儿PRL引起的尿液异常并不常见。76.2％的小儿PRL为男孩，所有病例中有2/3表现为弥漫性肾脏肿大。那些表现为肿块的PRL很容易被误诊为WT或其他恶性肿瘤。局部肿大淋巴结缺失，无坏死或钙化提示肾肿块的不典型表现,需进行经皮穿刺活检,及时准确诊断,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手术。
方法：这里，我们详细分享了一例儿童原发性肾淋巴瘤（PRL），并总结了其常见的临床表现，成像特征,通过对文献报道的病例进行回顾性分析，了解小儿PRL的预后因素。一名2岁男孩出现在诊所，腹部右侧有一个大肿块，食欲不振。
方法：成像显示右肾大肿块，几乎取代了整个肾脏组织，在左肾有许多小结节。鉴于没有局部淋巴结肿大和转移，诊断不清楚。进行了经皮肾穿刺,这证明了伯基特淋巴瘤的诊断。因为没有骨髓受累,这个孩子被诊断为小儿PRL.
方法：这名PRL男孩接受NHL-BFM95方案和支持治疗。
结果：不幸的是，这个男孩在治疗的第五个月死于多器官衰竭。
结论：根据文献综述，小儿PRL的表现是疲劳，食欲不振,减肥,腹部肿胀,或其他非特异性症状。尽管在81%的病例中，它经常浸润双侧肾脏，由小儿PRL引起的尿液异常并不常见。76.2％的小儿PRL为男孩，所有病例中有2/3表现为弥漫性肾脏肿大。那些表现为肿块的PRL很容易被误诊为WT或其他恶性肿瘤。局部肿大淋巴结缺失，无坏死或钙化提示肾肿块的不典型表现,需进行经皮穿刺活检,及时准确诊断,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手术。
