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Case Report
- Fetal Rhabdomyomatous Nephroblastoma: A case report.
- Nam Hoon Kim, Chan Pil Park, Eun Kyung Hong, Poong Man Jung, Moon Hyang Park
- Korean J Pathol. 1995;29(1):96-102.
- 1,957 View
- 48 Download
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Abstract
PDF - A fetal rhabdomyomatous nephroblastoma is considered to be a predominantly monophasic mesenchymal variant of Wilms' tumor, which acts less aggressively than a conventional Wilms' tumor despite its much larger size. Bilaterality of this tumor in a nine month-old girl, however, may negatively affect the overall prognosis. A radical nephrectomy for bulky masses in the left kidney and a partial nephrectomy for right kidney with five small tumor masses was performed at the same time. Two small tumor masses in the upper part of right kidney were left behind because of preserving minimal renal functional capacity. Pathological study revealed a mixed type of nephroblastoma which was composed predominantly of mesenchymal components with fetal rhabdomyomatous differentiation. After post-operative chemotherapy with vincristine, actinomycin D and adriamycin, and radiotherapy(2,130 rad), residual tumor masses became a single tumor 5 cm in diameter and well demarcated, which was resected at 15 months after first operation when the size and renal function of remained right kidney was appropriate to resect out the residual tumor. The tumor resected out at second operation was entirely composed of scattered differentiated fetal skeletal muscle cells in the fibrovascular tissue. Only a few entrapped epithelial components were seen but no blastemal cornponents were present. Follow up abdominal CT and ultrasonographic examinations revealed no evidence of tumor recurrence. The girl has developed normally without disease.
Original Article
- Fatty Hamartoma of Epicardium.
- Hyun Wook Kang, Jung Ran Kim, Jeong Wook Seo, Je G Chi
- Korean J Pathol. 1989;23(1):132-135.
- 1,882 View
- 21 Download
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Abstract
PDF
- The fatty hamartoma of pericardium is characterized by abnormal accumulations of adipose tissue forming a recognizable mass. We reported a case that could best be put into "fatty hamartoma" group. This 66 year old male presented with atrial fibrillation came in for a anterior mediastinal mass. He received an operation for adenocarcinoma of ascending colon several months ago. The chest CT revealed an inhomogeneous mass in the right anterior mediastinal area, which was not clearly demarcated from the right cardiac border. The heart was deviated to the left side due to the mass. Surgical excision was performed under the impression of cardiac teratoma. The large mass was well encapsulated, and was bright yellow and partly pink, and measured 12x8 cm. Microscopically, the mass consisted predominantly of mature fat cells and abundant fibrous tissue with scattered nests of primitive cardiac muscle cells. Reviewing the literature we found the term "fatty hamartoma" could also refer to rhabdomyolipoma or fibrolipoma. Since it contains entrapped cardiac muscle cells, abundant fibrous tissue, multiple blood vessels and fetal fat cells, it could best the categorized into "hamartoma". The term "fatty" represents it's main component. Therefore we propose the term "fatty hamartoma" that could to be used further.
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