Wilms tumour with neural differentiation: A rare histological presentation
Keywords:
Wilm's Tumor, Neural Differentiation, WT1, Synaptophysin
Abstract
Wilm's tumour is the most common malignant renal tumour of childhood presenting most commonly between the age groups of 1 to 6 years. It exhibits histogenetic heterogeneity. A 4 year old female child presented with complaints of right abdominal pain and mass per abdomen for the past six months .The patient underwent right sided nephrectomy and the specimen was sent for histopathological examination .Cut surface of the tumour was variegated with extensive areas of necrosis and haemorrhage On microscopy the tumour cells were predominantly blastematous , arranged as diffuse sheets of tumor cells with focal nesting pattern in some areas. Tumour cells showed diffuse anaplasia.These tumour cells showed positivity for WT1 ,vimentin, synaptophysin and p53. No evidence of any epithelial or stromal differentiation was noted. Findings suggested a diagnosis of monophasic variant( blastemal) Wilm's tumor with neural differentiation.References
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11. Masson P. The role of neural crests in the embryonal adenosarcoma of the kidney. Am I Cancer. 1938: 33: 1-32.
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13. Magee F, Mah RG, Taylor GP, Dimmick JE. Neural differentiation in Wilms’ tumor. Hum Pathol. 1987; 18: 33-37.
14. Orazi A, Lombardi L, Trumper L, Cattoretti G, Ballerini E. Nephroblastoma with neuroendocrine differentiation.Tumori .1989;75(2):171-6.
15. Seo J, Suh YL, Choi HY. Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation. Pathol Int. 2009; 59 (1): 44-48.
16. Kösem M, Ýbiloðlu Y, Bakan V, Köseoðlu B. Ectomesenchymoma: case report and review of the literature. Turk J Pediatr. 2004;46: 82-87.
17. Labanaris AP, Zugor V, Smiszek R, Nützel R, Kühn R. Anaplastic sarcoma of the kidney. The Scientific World Journal. 2009; 9:97-101.
18. Beckwith JB, Zuppan CE, Browning NG, et al. Histological analysis of aggressiveness and responsiveness in Wilms’ tumor. Med Pediatr Oncol 1996;27:422-8.
2. Beckwith JB: Wilm’s tumour and renal tumors of childhood: a selective review from the National Wilms’ Tumor Study Pathology Center. Hum Pathol. 14: 481-492, 1983.
3. Osterheld et al: Role of DNA Ploidy and Immunohistochemistry in Wilms’ Tumours Adverse prognosis. Anticancer Research .2008.28: 751-756.
4. Perlman E, Grosfeld JL, Togashi K, Boccon-Gibod L. Pathology and Genetics of Tumors of the Urinary System and Male GenitalOrgans. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, eds.World Health Organization Classification of Tumours. Lyon,France: IARC Press; 2004:48-52.
5. Folpe AL, Patterson K, Gown AM. Antibodies to desmin identify the blastemal component of nephroblastoma. Mod Pathol.1997;10:895-900.
6. Meis-Kindblom JM, Stenman G, Kindblom LG. Differential diagnosis of small round cell tumors. Semin. Diagn. Pathol. 1996;13:213–241.
7. Gregorio A, Corrias MV, Castriconi R, Dondero A. Small round blue cell tumours: diagnostic and prognostic usefulness of the expression of B7-H3 surface molecule. Histopathology. 2008 Jul; 53(1): 73–80.
8. Harbitz F: Sind einige der sogennanten 'adeno-sarcome' in dennieren in Wierlich Neuroblastome? Acta Pathol Microbiol Immunol Scand. 1932: 9: 199.
9. Amato R.J., Ellerhorst J.A., Ayala A.G. Intraabdominal desmoplastic small round cell tumor: report and discussion of five cases. Cancer. 1996;78(4):845–851.
10. Gerald WL., Rosai J. Desmoplastic small round cell tumor with multi-phenotypic differentiation. Zentralbl Pathol. 1993;139:141–151.
11. Masson P. The role of neural crests in the embryonal adenosarcoma of the kidney. Am I Cancer. 1938: 33: 1-32.
12. Argani P, Beckwith BJ. Renal Neoplasms of Childhood.In: Mills SE, editors. Sternberg's Diagnostic Surgical Pathology. Lippincott Williams & Wilkins;2010.1804-1805.
13. Magee F, Mah RG, Taylor GP, Dimmick JE. Neural differentiation in Wilms’ tumor. Hum Pathol. 1987; 18: 33-37.
14. Orazi A, Lombardi L, Trumper L, Cattoretti G, Ballerini E. Nephroblastoma with neuroendocrine differentiation.Tumori .1989;75(2):171-6.
15. Seo J, Suh YL, Choi HY. Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation. Pathol Int. 2009; 59 (1): 44-48.
16. Kösem M, Ýbiloðlu Y, Bakan V, Köseoðlu B. Ectomesenchymoma: case report and review of the literature. Turk J Pediatr. 2004;46: 82-87.
17. Labanaris AP, Zugor V, Smiszek R, Nützel R, Kühn R. Anaplastic sarcoma of the kidney. The Scientific World Journal. 2009; 9:97-101.
18. Beckwith JB, Zuppan CE, Browning NG, et al. Histological analysis of aggressiveness and responsiveness in Wilms’ tumor. Med Pediatr Oncol 1996;27:422-8.
Published
2016-07-29
Issue
Section
Case Report
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