신장의 원발성 원시성 신경외배엽 종양 -2예 보고-
Primary Primitive Neuroectodermal Tumor of the Kidney -Report of Two Cases-
구현영, 전선영, 최진, 노재윤, 안한종, 조경자,
소속 상세정보
구현영 ( Koo Hyun-Lyoung )
울산대학교 의과대학 서울아산병원 병리과
전선영 ( Jun Sun-Young )
울산대학교 의과대학 서울아산병원 병리과
최진 ( Choi Gene )
울산대학교 의과대학 서울아산병원 병리과
노재윤 ( Ro Jae Y. )
울산대학교 의과대학 서울아산병원 병리과
안한종 ( Ahn Han-Jong )
울산대학교 의과대학 서울아산병원 비뇨기과학교실
조경자 ( Cho Kyung-Ja )
울산대학교 의과대학 서울아산병원 병리과
KMID : 0357920030370020145
Abstract
Primitive neuroectodermal tumor (PNET) is a small round cell neoplasm that mainly develops in the central nervous system and soft tissue of children. Primary occurrence in the kidney is rare and the identification of immunopositivity for CD99 and t(11;22)(q24;q12) is essential in differential diagnoses. We report two cases of PNET developed in a 21-year-old woman and a 44-year-old man. Resected tumors were composed of sheets of round or ovoid cells with hyperchromatic nuclei and minimal eosinophilic cytoplasm. Rosette formations, more prominent in the first case, were observed. The tumor cells of both cases were diffusely positive for CD99, vimentin, and neuron specific enolase, while they were negative for cytokeratin, desmin, and chromogranin. Synaptophysin was focally expressed only in the first case. The EWS-FLI1 chimeric gene was identified by a reverse transcriptase-polymerase chain reaction in the first case. The first patient is alive with a recurrent tumor two years after the diagnosis, and has received combination chemotherapy. The second patient is alive with no evidence of recurrence or metastasis nineteen months after the diagnosis.
키워드
Kidney;Neoplasm;Peripheral Neuroectodermal Tumor;CD99;RT;PCR
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