Mature renal teratoma and a synchronous malignant neuroepithelial tumour of the ipsilateral adrenal gland.
نویسندگان
چکیده
The main primary childhood renal neo-plasms are nephroblastoma, mesoblastic ne-phroma, clear cell sarcoma, and rhabdoid tumour. Other primary renal neoplasms include primitive neuroectodermal tumour (PNET), renal cell carcinoma, and angiomy-olipoma. Nephroblastoma is the most common renal tumour in children. It is a complex embryonal tumour of metanephric blastemal derivation, which often contains diverse epithelial and stromal tissues. Diagnostic problems are often encountered when tumours contain a variety of heterologous elements. The term teratoid nephroblastoma has been used to describe a variant of nephroblastoma with a predominance of het-erologous tissues. 1 It is this variant that can be confused with a teratoma. Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retro-peritoneal teratomas secondarily invading the kidney. 2 The diVerentiation between these two neoplasms in the kidney is often problematic. Neurogenic tissues in the kidney can be found in primary tumours or as part of meta-static tumours. The primary tumours are nephroblastoma, which may contain ganglion cells, neuroblast, and neuroglial tissue, 2 and PNET. 3 4 Adrenal neuroblastomas can directly invade the adjacent kidney. 5 We describe the pathology of a right renal mass in a 3 year old child and discuss the diVerential diagnosis. A 3 year old girl presented with abdominal pain and diarrhoea. On examination she was found to have signs of pulmonary tuberculosis and was started on antituberculous treatment. Subsequently, a large, firm, tender , right flank mass clearly separate from the liver was detected and she was referred to the Regional Paediatric Surgical Unit for further investigation and management. On admission, the child was apyrexial, emaciated, and weighed 13 kg. She had bilateral coarse crackles and a wheeze. The abdomen was distended and a non-tender 3 cm hepatomegaly was palpated. Furthermore, a 10 × 12 cm non-tender, firm, non-pulsatile right flank mass was detected. Results of routine laboratory tests were as follows: haemoglobin, 90 g/litre (normal, 112–143); white blood cell count, 8.2 × 10 9 / litre (normal, 5.5–15.5); and platelet count, 224 × 10 9 /litre. Urinary catecholamine values were as follows: noradrenaline, 0.279 µM/ mM creatinine (CRT) (normal, 0–0.08); adrenaline, 0.023 µM/mM CRT (normal, 0–0.035); dopamine, 0.67 µM/mM CRT (normal, 0–1.13); vanillylmandelic acid 9 µM/mM CRT (normal, 0–15); and ho-movanillic acid, 11 µM/mM CRT (normal, 0–15). Renal and liver function tests were normal. Computed tomography (CT) scan of the abdomen revealed a large tumour involving the right side …
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ورودعنوان ژورنال:
- Journal of clinical pathology
دوره 54 3 شماره
صفحات -
تاریخ انتشار 2001