All issues > Volume 44(8); 2001

Case Report

J Korean Pediatr Soc. 2001;44(8):959-964. Published online August 15, 2001.

Malignant Ectomesenchymoma in a Two-Month-Old Boy

Ji Young JH Hwang¹, Ji Hyoun JH Seo¹, Jeum-Su JS Kim¹, Young-Suk YS Kim², Sue-Jin SJ Lee¹, Chan-Hoo CH Park¹, Yoo Kyung YK Kim², Sun Hoo SH Park², Jung Hee JH Lee², In Oak IO Ahn³, Ki Hyun KH Chung⁴, Byung-Kiu BK 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

Abstract

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.

Keywords :Malignant ectomesenchymoma, Ectomesenchyme, Prostatic region, Immunohistochemistry, Ganglioneuroma, Rhabdomyosarcoma