| Malignant Ectomesenchymoma in a Two-Month-Old Boy |
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Ji Young Hwang1, Ji Hyoun Seo1, Jeum-Su Kim1, Young-Suk Kim2, Sue-Jin Lee1, Chan-Hoo Park1, Yoo Kyung Kim2, Sun Hoo Park2, Jung Hee Lee2, In Oak Ahn3, Ki Hyun Chung4, Byung-Kiu Park5 |
1Departments of Pediatrics, Gyeongsang National University College of Medicine, Chinju, Korea
2Departments of Pathology, Gyeongsang National University College of Medicine, Chinju, Korea
3Departments of Pediatrics, Gyeongsang National University College of Medicine, Chinju, Korea
4Departments of Urology, Gyeongsang National University College of Medicine, Chinju, Korea
5Departments of Pediatrics, Gyeongsang National University College of Medicine, Chinju, Korea |
| 2개월 남아에서 발병한 Malignant Ectomesenchymoma |
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황지영1, 서지현1, 김점수1, 김용숙2, 이수진1, 박찬후1, 김유경2, 박선후2, 이정희2, 안인옥3, 정기현4, 박병규5 |
1경상대학교 의과대학 소아과학교실
2경상대학교 의과대학 해부병리학교실
3경상대학교 의과대학 방사선과학교실
4경상대학교암연구소, 비뇨기과학교실
5경상대학교암연구소 |
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| Abstract |
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Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The tumor appeared to arise from the prostatic region and enlarged to compress the bladder leading to bilateral hydronephrosis. Immunohistochemical studies for the resected tumor confirmed the presence of mixed ganglioneuroma and rhabdomyosarcoma establishing the diagnosis of malignant ectomesenchymoma. However, in the initial biopsy specimen of tumor, poorly differentiated round to oval cells positive for both desmin and vimentin staining were uniformly noted and the incorrect diagnosis of rhabdomyosarcoma was made. Intensive multi-agents chemotherapy, surgery and radiotherapy had failed in preventing the development of local recurrences. Subsequently, invasion of pubic bone and lung metastases ensued. This report enlightens the need for immunohistochemistry to seek possible neuroectodermal components in a tumor specimen suggestive of rhabdomyosarcoma. |
| Key Words:
Malignant ectomesenchymoma, Ectomesenchyme, Prostatic region, Immunohistochemistry, Ganglioneuroma, Rhabdomyosarcoma |
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