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Extrarenal nephroblastoma.

Teerthanath S - J Lab Physicians (2011)

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Surgery, K.S. Hegde Medical Academy, Nithyanand Nagar, Derelakatte, Mangalore, Karnataka, India.

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Sir, Nephroblastoma is the commonest primary malignant tumor of kidney in children occurring between the ages of 2 and 5 years... Nephroblastoma or Wilms′ tumor is the commonest malignant tumor of children occurring between 2 and 5 years of age, arising from embryonic kidney tissue... Extrarenal nephroblastomas vary in their clinical presentation depending on their location and pressure effects on bowel, bladder, ureter, blood vessels, and nerves... Nephrogenic rests (NR) and nephroblastomatosis are considered to be the precursor lesions of nephroblastoma and most rests are destined for eventual atresia... Perilobular NR occurs in fetal overgrowth and with certain overgrowth syndromes... Intralobular NR are frequently associated with deletions or mutations of WT1... Nephroblastomatosis can be perilobular (subcapsular), pan lobular (diffuse cortical), or intralobular (in the renal parenchyma along the columns of bertin)... Nephroblastomatosis is also associated with (1) WAGAR syndrome, (2) Denys-Drash Syndrome, and (3) Beckwith Weidman Syndrome... These syndromes are associated with increased risk of developing Wilms′ tumor, suggesting related manifestations of genetic damage, affecting single gene or closely linked genes WT1 and WT2... Staging and management of extrarenal nephroblastoma will be the same as those of renal nephroblastomas... Distribution of anaplasia is having critical prognostic relevance... The present case did not show any anaplastic features... This case was treated surgically with postoperative adjuvant chemotherapy... There was no evidence of recurrence of the tumor after 4 years of treatment.

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Photomicrography shows spindle cell stroma, separating lobules of blastemal tissue containing abortive tubules and glomeruli (H and E, 400×)
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Figure 2: Photomicrography shows spindle cell stroma, separating lobules of blastemal tissue containing abortive tubules and glomeruli (H and E, 400×)

Mentions: A girl aged 6 years was brought to the hospital for pain abdomen, mass per abdomen, increased frequency of defecation and micturation of 6 months duration. There was no history of passing blood in stool and urine. A nontender freely mobile mass, with rounded border, firm to hard in consistency, was situated in right lower lumbar and iliac fossa. No other abnormalities were detected on systemic examination. Ultrasonography revealed a heterogeneous mass measuring 58 × 65 × 51 mm3 with specks of calcification, situated anterior to right iliac vessels and lateral to urinary bladder. Right kidney showed minimal hydronephrosis, and left kidney appeared normal. Urine examination showed specific gravity 1.015, pH 6. Urine sediment showed 2–3 polymorphs per high power field, calcium oxalate crystals, and no erythrocytes were seen. At operation, a single fully encapsulated spherical retroperitoneal mass was seen in right iliac fossa which was seen sitting on right ureter and right iliac vessels. The mass was displacing small bowel, cecum and appendix. Both kidneys and ovaries appeared normal. The mass was measuring 6 cm in diameter and was shelled out easily and sent for histopathological examination. Cut surface appeared soft fleshy to greyish white with areas of necrosis and small foci of cystic change [Figure 1.Microscopy revealed predominantly islands and sheets of blastemal cells with foci of abortive glomeruli and tubular epithelial differentiation separated by variable amounts of connective tissue containing spindle cells, myocytes with striations, and myxoid areas [Figure 2]. Tumor cells showed mild pleomorphism, occasional mitotic figures with extensive areas of tumor necrosis. The capsule contained islands of occasional compressed tubules. Areas of anaplasia were not seen.


Extrarenal nephroblastoma.

Teerthanath S - J Lab Physicians (2011)

Photomicrography shows spindle cell stroma, separating lobules of blastemal tissue containing abortive tubules and glomeruli (H and E, 400×)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3118062&req=5

Figure 2: Photomicrography shows spindle cell stroma, separating lobules of blastemal tissue containing abortive tubules and glomeruli (H and E, 400×)
Mentions: A girl aged 6 years was brought to the hospital for pain abdomen, mass per abdomen, increased frequency of defecation and micturation of 6 months duration. There was no history of passing blood in stool and urine. A nontender freely mobile mass, with rounded border, firm to hard in consistency, was situated in right lower lumbar and iliac fossa. No other abnormalities were detected on systemic examination. Ultrasonography revealed a heterogeneous mass measuring 58 × 65 × 51 mm3 with specks of calcification, situated anterior to right iliac vessels and lateral to urinary bladder. Right kidney showed minimal hydronephrosis, and left kidney appeared normal. Urine examination showed specific gravity 1.015, pH 6. Urine sediment showed 2–3 polymorphs per high power field, calcium oxalate crystals, and no erythrocytes were seen. At operation, a single fully encapsulated spherical retroperitoneal mass was seen in right iliac fossa which was seen sitting on right ureter and right iliac vessels. The mass was displacing small bowel, cecum and appendix. Both kidneys and ovaries appeared normal. The mass was measuring 6 cm in diameter and was shelled out easily and sent for histopathological examination. Cut surface appeared soft fleshy to greyish white with areas of necrosis and small foci of cystic change [Figure 1.Microscopy revealed predominantly islands and sheets of blastemal cells with foci of abortive glomeruli and tubular epithelial differentiation separated by variable amounts of connective tissue containing spindle cells, myocytes with striations, and myxoid areas [Figure 2]. Tumor cells showed mild pleomorphism, occasional mitotic figures with extensive areas of tumor necrosis. The capsule contained islands of occasional compressed tubules. Areas of anaplasia were not seen.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology and Surgery, K.S. Hegde Medical Academy, Nithyanand Nagar, Derelakatte, Mangalore, Karnataka, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Sir, Nephroblastoma is the commonest primary malignant tumor of kidney in children occurring between the ages of 2 and 5 years... Nephroblastoma or Wilms′ tumor is the commonest malignant tumor of children occurring between 2 and 5 years of age, arising from embryonic kidney tissue... Extrarenal nephroblastomas vary in their clinical presentation depending on their location and pressure effects on bowel, bladder, ureter, blood vessels, and nerves... Nephrogenic rests (NR) and nephroblastomatosis are considered to be the precursor lesions of nephroblastoma and most rests are destined for eventual atresia... Perilobular NR occurs in fetal overgrowth and with certain overgrowth syndromes... Intralobular NR are frequently associated with deletions or mutations of WT1... Nephroblastomatosis can be perilobular (subcapsular), pan lobular (diffuse cortical), or intralobular (in the renal parenchyma along the columns of bertin)... Nephroblastomatosis is also associated with (1) WAGAR syndrome, (2) Denys-Drash Syndrome, and (3) Beckwith Weidman Syndrome... These syndromes are associated with increased risk of developing Wilms′ tumor, suggesting related manifestations of genetic damage, affecting single gene or closely linked genes WT1 and WT2... Staging and management of extrarenal nephroblastoma will be the same as those of renal nephroblastomas... Distribution of anaplasia is having critical prognostic relevance... The present case did not show any anaplastic features... This case was treated surgically with postoperative adjuvant chemotherapy... There was no evidence of recurrence of the tumor after 4 years of treatment.

No MeSH data available.