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[儿童肾源性恶性非霍奇金淋巴瘤带来的诊断与治疗问题。附7例报告]

[Diagnostic and therapeutic problems posed by malignant non Hodgkin lymphoma of renal origin in children. Apropos of 7 cases].

作者信息

Donadieu J, Patte C, Kalifa C, Lemerle J

机构信息

Service de Pédiatrie, Institut Gustave-Roussy, Villejuif.

出版信息

Arch Fr Pediatr. 1992 Oct;49(8):699-704.

PMID:1337694
Abstract

BACKGROUND

The kidneys of non Hodgkin lymphoma patients frequently contain lymphoma cells, but these tumors rarely arise in the renal tissue and are rarely located there.

PATIENTS

A diagnosis of non Hodgkin lymphoma of renal origin or predominantly located in kidneys was made in 7 patients aged 2-14 years old. These patients formed part of a total of 450 patients with non Hodgkin lymphomas seen from 1974 to 1987. The first manifestation in these 7 children was an abdominal mass associated with hypertension in 3 cases; 2 of whom presented with acute kidney failure. Ultrasonography showed hypoechogenous masses in one (2 cases) or both kidneys (5 cases). The diagnosis of malignant lymphoma was made directly in only one patient who also presented with mediastinal and abdominal lymph node enlargement. In the others, the first diagnosis was Wilms tumor (5 cases) and polycystic disease of the kidney (1 case); the correct diagnosis of malignant lymphoma was later made from biopsies of renal (4 patients) or extrarenal involved tissue (2 patients). Six of the 7 cases were Burkitt lymphomas, and all 7 were in stage III (3 patients) and IV (4 patients). 6 patients (3 stage III and 3 stage IV) were successfully treated by chemotherapy with a median follow-up of 9 years.

CONCLUSIONS

Differential diagnosis between non Hodgkin lymphomas of renal origin and a Wilms tumor may be difficult even though kidney failure is more frequent in lymphoma. Sonography is the best method for diagnosis showing typical hypoechogenous masses and diffuse homogeneous infiltration of the kidneys. In the absence of extrarenal (meningeal, testis, bone marrow) dissemination, definite evidence of lymphoma depends on the histological examination of tumoral tissue obtained by surgical biopsy.

摘要

背景

非霍奇金淋巴瘤患者的肾脏常含有淋巴瘤细胞,但这些肿瘤很少起源于肾组织,也很少位于肾脏。

患者

7例年龄在2至14岁的患者被诊断为肾原发性或主要位于肾脏的非霍奇金淋巴瘤。这些患者是1974年至1987年间共450例非霍奇金淋巴瘤患者的一部分。这7名儿童的首发表现为腹部肿块,其中3例伴有高血压;其中2例出现急性肾衰竭。超声检查显示1例(2例)或双侧肾脏(5例)有低回声肿块。仅1例同时伴有纵隔和腹部淋巴结肿大的患者直接诊断为恶性淋巴瘤。其他患者最初诊断为肾母细胞瘤(5例)和肾多囊病(1例);后来通过肾组织活检(4例患者)或肾外受累组织活检(2例患者)才正确诊断为恶性淋巴瘤。7例中有6例为伯基特淋巴瘤,所有7例均处于III期(3例患者)和IV期(4例患者)。6例患者(3例III期和3例IV期)通过化疗成功治疗,中位随访时间为9年。

结论

肾原发性非霍奇金淋巴瘤与肾母细胞瘤的鉴别诊断可能困难,尽管淋巴瘤患者肾衰竭更为常见。超声检查是最佳诊断方法,可显示典型的低回声肿块和肾脏弥漫性均匀浸润。在没有肾外(脑膜、睾丸、骨髓)播散的情况下,淋巴瘤的确切证据取决于手术活检获得的肿瘤组织的组织学检查。

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