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Correspondence.(Letter to the Editor)

Journal of Clinical Pathology

| March 01, 2001 | COPYRIGHT 2003 British Medical Association. This material is published under license from the publisher through the Gale Group, Farmington Hills, Michigan.  All inquiries regarding rights should be directed to the Gale Group. (Hide copyright information)Copyright

Mature renal teratoma and a synchronous malignant neuroepithelial tumour of the ipsilateral adrenal gland

The main primary childhood renal neoplasms are nephroblastoma, mesoblastic nephroma, clear cell sarcoma, and rhabdoid tumour. Other primary renal neoplasms include primitive neuroectodermal tumour (PNET), renal cell carcinoma, and angiomyolipoma. 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 heterologous 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 retroperitoneal teratomas secondarily invading the kidney. [2] The differentiation 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 metastatic 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 differential 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 x 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 x [10.sup.9]/litre (normal, 5.5-15.5); and platelet count, 224 x [10.sup.9]/litre. Urinary catecholamine values were as follows: noradrenaline, 0.279[micro]M/mM creatinine (CRT) (normal, 0-0.08); adrenaline, 0.023 [micro]M/mM CRT (normal, 0-0.035); dopamine, 0.67 [micro]M/mM CRT (normal, 0-1.13); vanillylmandelic acid 9 [micro]M/mM CRT (normal, 0-15); and homovanillic acid, 11 [micro]M/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 of the abdomen. There were also multiple hepatic lesions consistent with metastases. A fine needle aspiration biopsy of the mass was performed. After a cytological diagnosis of neuroblastoma the child was started on the appropriate chemotherapy protocol consisting of vincristine,…

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