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Year : 2017  |  Volume : 60  |  Issue : 1  |  Page : 115-116
Concurrent occurrence of neuroblastoma and clear cell sarcoma of kidney in an infant: Is it a random event?

Department of Lab Medicine, P. D. Hinduja Hospital and Medical Research Centre, Mumbai, Maharashtra, India

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Date of Web Publication14-Feb-2017

How to cite this article:
Deshpande RB. Concurrent occurrence of neuroblastoma and clear cell sarcoma of kidney in an infant: Is it a random event?. Indian J Pathol Microbiol 2017;60:115-6

How to cite this URL:
Deshpande RB. Concurrent occurrence of neuroblastoma and clear cell sarcoma of kidney in an infant: Is it a random event?. Indian J Pathol Microbiol [serial online] 2017 [cited 2018 Jan 18];60:115-6. Available from:

The report of concurrent occurrence of two childhood tumors namely, preaortic neuroblastoma and clear cell sarcoma of kidney published in this issue of the Journal [1] records this association for the first time. Is this random event that deserves nothing more than passing curiosity only, or does it have any significance?

The prevalence of “second cancers” is increasing mainly because of increasing number of cancer survivors for longer time. While many of these are attributed to a complication of therapy, there are other factors which play a role in their pathogenesis, namely common etiology, environment, and host factors including genetic susceptibility.[2]

Renal cell carcinoma occurring after neuroblastoma is now well-recognized entity.[3] While the majority of these are thought to be related to chemotherapy, there are a few cases recorded where no therapy was administered for neuroblastoma, or where both neuroblastoma and renal cell carcinoma occurred concurrently or within short interval of time.[4]

Even in cases where renal cell carcinoma occurred several years after chemotherapy for neuroblastoma, there is no explanation why kidney is the dominant organ for the development of these second primary cancers.[5] Such dominance of kidney cancer is not known after chemotherapy for other types of cancers.

It is possible that all renal carcinomas occurring in neuroblastoma survivors are not related to therapy.

In cases where two distinct primary cancers develop concurrently or within short interval of time factors other than therapy such as common etiology, environmental factors, and host factors come into consideration.

When it comes to concurrent occurrence of two or more childhood tumors, genetic susceptibility, or some developmental factors become important.

Hereditary retinoblastoma with germ-line mutation of tumor suppressor gene RB1 is prototype childhood cancer with increased susceptibility for developing second, third, or even fourth primary cancers not only in irradiated field (head and neck region) but also in organs far away from the field of irradiation. “Trilateral retinoblastoma” refers to pinealoblastoma occurring in patients with hereditary bilateral retinoblastoma. All these additional primary cancers that occur both in irradiated field and in distal areas carry germ-line mutation of RB1 gene. This germ-line mutation appears to be pivotal factor not only in the development of retinoblastomas but also in the development of subsequent primary tumors in retinoblastoma survivors.

Two concurrent childhood tumors namely preaortic neuroblastoma and clear cell sarcoma of the kidney in a 7-month-old infant reported here point to common genetic or developmental factors not as yet understood.

Unlike in retinoblastoma, neither germline mutation has been identified either in neuroblastoma or in clear cell sarcoma of kidney nor is there any consistent somatic mutation reported.

What can be possible explanation for this event of concurrent two childhood tumors?

Embryonal tumors have an inherent potential for maturation. Neuroblastoma matures into differentiating neuroblastoma and mature ganglioneuroma. Embryonal tumors may be considered as result of maturational defect of embryonic tissue.

Is there a common maturational factor that involves both adrenal medulla and kidney? That these two organs are so closely apposed may not be without significance.

Any hypothesis based on a solitary case report is premature. We need to look out for more such cases in future.

   References Top

Doshi PR, Karandikar MN, Swami RC, Singh HA, Nimbargi RC. Dual tumor – Clear cell sarcoma of kidney with differentiating neuroblastoma: A rare case with review of literature. Indian J Pathol Microbiol 2017;60.111-4.  Back to cited text no. 1
Travis LB, Bhatia S, Allan JM, Oeffinger KC, Ng A. Second cancers. In: Devita VT, Lawrence TS, Rosenberg S, editors. Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia: Wolters Kluwer; 2015. p. 2047-67.  Back to cited text no. 2
Falzarano SM, McKenney JK, Montironi R, Eble JN, Osunkoya AO, Guo J, et al. Renal cell carcinoma occurring in patients with prior neuroblastoma: A heterogenous group of neoplasms. Am J Surg Pathol 2016;40:989-97.  Back to cited text no. 3
Medeiros LJ. Renal cell carcinoma associated with neuroblastoma. In: Eble JN, Sauter G, Epstein JI, Sesterhenn IA, editors. WHO Classification of Tumours. Pathology and Genetics Tumours of the Urinary System and Male Genital Organs. Lyon, France: IARC Press; 2004. p. 39.  Back to cited text no. 4
Wilson CL, Ness KK, Neglia JP, Hammond S, Shnorhavorian M, Leisenring WL, et al. Renal carcinoma after childhood cancer: A report from the childhood cancer survivor study. J Natl Cancer Inst 2013;105:504-8.  Back to cited text no. 5

Correspondence Address:
Dr. Ramesh B Deshpande
P. D. Hinduja Hospital and Medical Research Centre, Mumbai - 400 016, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_663_16

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