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肾原始神经外胚层肿瘤。两例经细针穿刺细胞学诊断的病例报告。

Primitive neuroectodermal tumor of the kidney. A report of two cases diagnosed by fine needle aspiration cytology.

作者信息

Premalata Chennagiri S, Gayathri Devi Muniyappa, Biswas Siddarth, Mukherjee Geethashree, Balu Sadashivan, Sundareshan Tambarahalli S, Prabhakaran Ponnuswamy S

机构信息

Cytopathology Unit, Department of Pathology, Kidwai Memorial Institute of Oncology, M. H. Marigowda Road, Bangalore, India 560029.

出版信息

Acta Cytol. 2003 May-Jun;47(3):475-9. doi: 10.1159/000326554.

Abstract

BACKGROUND

Primitive neurocetodermal tumors (PNETs) constitute a family of neoplasms of presumed neuroectrodermal origin most often presenting as bone or soft tissue masses. There are very few reported cases of PNET of the kidney and none diagnosed by fine needle aspiration cytology (FNAC), to the best of our knowledge, in the world literature. We present two cases of renal PNET diagnosed on cytology.

CASES

Two patients with renal masses were diagnosed as having PNET on FNAC. Cytologically the tumors showed a dispersed population of malignant small round cells with focal rosette formation and perivascular arrangement of tumor cells. Immunohistochemistry on the cell blocks in both cases showed strong membrane positivity for CD99 (MIC2). Cytogenetic studies in both cases showed the characteristic t(11;22)(q24;q12) translocation, with additional chromosomal abnormalities in case 2.

CONCLUSION

PNET of the kidney is a distinct entity and can be diagnosed on fine needle aspiration smears and confirmed with immunohistochemistry and cytogenetic studies. A diagnosis of PNET must be included in the differential diagnosis of renal masses in adolescents and young adults.

摘要

背景

原始神经外胚层肿瘤(PNETs)是一类起源于神经外胚层的肿瘤,最常表现为骨或软组织肿块。据我们所知,世界文献中报道的肾PNET病例极少,且尚无通过细针穿刺细胞学检查(FNAC)确诊的病例。我们报告两例经细胞学诊断的肾PNET病例。

病例

两名肾肿块患者经FNAC诊断为PNET。细胞学检查显示肿瘤由散在的恶性小圆形细胞组成,有局灶性菊形团形成,肿瘤细胞呈血管周围排列。两例病例的细胞块免疫组化均显示CD99(MIC2)呈强膜阳性。两例病例的细胞遗传学研究均显示特征性的t(11;22)(q24;q12)易位,病例2还有额外的染色体异常。

结论

肾PNET是一种独特的实体瘤,可通过细针穿刺涂片诊断,并通过免疫组化和细胞遗传学研究得以证实。青少年和年轻成人肾肿块的鉴别诊断中必须考虑PNET的诊断。

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