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Year : 2017  |  Volume : 60  |  Issue : 1  |  Page : 111-114
Dual tumor – Clear cell sarcoma of kidney with differentiating neuroblastoma: A rare case with review of literature

Department of Pathology, Bharati Vidyapeeth University Medical College, Pune, Maharashtra, India

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Date of Web Publication14-Feb-2017


Occurrence of two distinct synchronous primary tumors is a rare event in children as well as in adults. Here, we report an extremely rare case of an infant found to have two synchronous Tumours namely clear cell sarcoma of kidney(CCSK) and differentiating neuroblastoma of preaortic region. To our knowledge, this may be the first case of synchronous CCSK and differentiating neuroblastoma being reported.

Keywords: Clear cell sarcoma of kidney, differentiating neuroblastoma, dual tumor

How to cite this article:
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

How to cite this URL:
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 [serial online] 2017 [cited 2020 Jul 3];60:111-4. Available from: http://www.ijpmonline.org/text.asp?2017/60/1/111/200047

   Introduction Top

Dual tumor is defined as the presence of two tumors occurring at anatomically two different sites in a patient. A collision tumor is defined as the coexistence of two adjacent, but histologically distinct tumor components in close proximity. Collision tumors involving the kidney are much more common as compared to dual tumors.

Clear cell sarcoma of kidney (CCSK) was originally called “Bone-metastasizing renal tumor of childhood” by Marsden and Lawler [1] and is rarely found in infants. Differentiating neuroblastoma is a rare peripheral neuroblastic tumor that is derived from developing neuronal cells of the sympathetic nervous system and is found mostly, but not exclusively, in young children.

   Case Report Top

An 11-month- boy presented with fever and acute gastroenteritis. There was no history of hematuria and weight loss, Parents gave a history of poor weight gain for 6 months. Clinical examination showed a firm, nontender mass occupying the right hypochondrium and lumbar regions. Ultrasonography showed a 6.1 cm × 2 cm heterogeneous mass at the lower pole of the right kidney. Provisional diagnosis of Wilms tumor (WT) was made. Right nephrectomy was done, Kidney was removed en mass along with right ureter. Biopsy was taken from a preaortic mass suspecting it to be an enlarged lymph node.

Gross examination revealed a right nephrectomy specimen, measuring 9 cm × 6 cm × 4.5 cm with a 5 cm segment of ureter. Cut surface showed a well-circumscribed tumor mass measuring 4.5 cm × 3.5 cm × 3 cm at the lower pole. The surface was grayish solid without areas of necrosis or hemorrhage. Perinephric fat and Gerota fascia were grossly free from the tumor. The outer capsular surface appeared smooth. The preaortic mass showed a soft yellowish bit measuring 0.5 cm × 0.5 cm with an area of hemorrhage.

Microscopic examination of the mass from kidney showed a highly cellular tumor consisting of undifferentiated malignant spindle cells of small-to-medium size, arranged in compact sheets and fascicles. The cells had elongated vesicular nuclei with a few inconspicuous nucleoli. The cells had moderate amount of clear or faintly eosinophilic cytoplasm. Abnormal mitotic figures of 1–5/hpf were noted. The other features seen were many thin-walled branching vascular channels, hyalinized stroma, an occasional area of cystic change, and an area of extravasated red blood cells. An occasional normal renal tubule was seen surrounded by the tumor cells at the periphery of tumor. Pelvicalyceal system and ureter showed normal histology. Necrosis was not seen. Section studied from the hilar blood vessels did not show tumor emboli [Figure 1].
Figure 1: Gross and microphotograph of the right kidney with well-circumscribed tumor (4.5 cm × 3.5 cm × 3 cm) at the lower pole. Microphotograph of clear cell sarcoma of kidney showing highly cellular tumor composed of undifferentiated malignant spindle cells

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Immunohistochemical (IHC) markers showed vimentin and cyclin D1 positivity in tumor cells and negativity for cytokeratin, epithelial membrane antigen, WT-1, CD34, smooth muscle actin, desmin, and S100 protein.

Section from the preaortic mass showed an encapsulated cellular tumor. The cells were arranged in sheets separated by fibrovascular septae of varying thickness, forming zellballen pattern. The individual tumor cells had round-to-oval nuclei with stippled chromatin and moderate amount of cytoplasm with indistinct cytoplasmic borders. The background showed large amount of fibrillar neutrophil. A few Homer Wrights rosettes were seen. Interspersed with these cells were large numbers of immature ganglion cells comprising more than 5% of the tumor cell population. These cells were multinucleated. The nuclei were eccentrically placed; cytoplasm was moderate in amount with distinct cytoplasmic borders. The stroma showed foci of lymphocytic infiltration and dystrophic calcification. Mitosis and necrosis were absent. Lymphoid architecture could not be identified in the section. The histological picture of the two tumors and absence of lymphoid tissue in the preaortic mass confirmed that the preaortic mass was not a metastasis of the renal tumor [Figure 2].
Figure 2: Gross and microphotograph of differentiating neuroblastoma from preaortic mass

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In view of the above morphological and IHC findings, diagnoses of CCSK of the right kidney and differentiating neuroblastoma of preaortic mass were confirmed.

The patient was operated again 15 days later for complete removal of the preaortic mass. Gross examination showed a well-circumscribed, gray-tan tumor mass of 4 cm × 2 cm. Cut section showed an area of hemorrhage. Microscopic examination showed similar picture like the differentiating neuroblastoma of the earlier biopsy.

Postoperatively, the child received chemotherapy with ifosfamide and doxorubicin and did not show relapse after 3 cycles.

   Discussion With a Review of Literature Top

Multiple primary malignancies in a single patient were first described in 1879 by Billroth and later defined by Warren and Gates [2] espite their increasing rates, multiple primary malignancies remain rare. Three criteria must be fulfilled so as to characterize multiple primary tumors (1) each tumor must be distinct from the other, (2) the tumor must present definite features of malignancy, and (3) the possibility that the one is a metastasis of another must be ruled out.[3] The neoplasm may be limited to a single organ or, as in our case, may involve multiple and anatomically separate organs.

The North American Association of Central Cancer Registries classifies multiple primary tumors into two categories: [1] Synchronous, in which the cancers occur at the same time. The Surveillance Epidemiology and End Results Program definition describes occurrence within 2 months. [2] Metachronous, in which the cancers follow in sequence, i.e., they are detected more than2 months apart.

The study of multiple primary malignancies provides useful information for clinical purposes, etiology, and may help in better management of cancer. They categorize multiple primaries into three major groups depending on their etiologic factors. The first group includes treatment-related neoplasm, the second includes syndrome cases, and the third includes neoplasms that may share common etiologic factors, such as genetic predisposition or same environmental factors.[4]

Takalkar et al. reported a case of an elderly woman with triple primary metachronous malignancies. She had adenocarcinoma of small intestine. After 21 months, she developed infiltrating duct carcinoma of breast. Again after a latent period of 10 months, she had papillary adenocarcinoma of ovary.[4]

Sakellakis et al. reported two cases of multiple tumors where the first patient was a 66-year-old male with synchronous colorectal cancer, renal cell carcinoma (RCC), and nonsmall cell lung cancer. The second patient was a 64-year-old female with breast cancer, transitional cell carcinoma of the ureter, and endometrial cancer.[5]

Acharya et al. reported a case of dual tumor in a 63-year-old female smoker who was elevated for lump in the right breast, fine needle aspiration cytology of which showed infiltrating ductal carcinoma. Investigations also revealed the presence of left upper lobe lung mass lesion, the biopsy of which suggested small cell carcinoma.[6]

Di Martino et al. reported the survival in second primary malignancies of patients with head and neck cancers. Their 8 years study included 39 patients presenting with synchronous and metachronous cancers. Fourteen cases were classified as synchronous tumors and 25 as metachronous tumors. Patients with metachronous tumors showed longer median overall survival than those with synchronous tumors (92.9 months vs. 15.7 months). In the subgroup of 11 patients with second primary lung carcinoma diagnosed more than 5 years after head and neck squamous cell carcinoma, prognosis was better.[7]

There are reports in literature where the associations of RCC with malignancies of breast, colon, rectum, stomach, prostate, bladder, endometrium, ovary, nasopharynx, lung, etc., have been reported.

In our case, the child presented with unrelated complaints and was found to have an incidental abdominal mass which turned out to be CCSK. CCSK comprises 4% of all primary childhood renal tumors. It is classified separately from WT due to several different features. The most common differential diagnosis for clear cell sarcoma is mesoblastic nephroma.

Differentiating features of CCSK, blastemal WT, and mesoblastic nephroma are shown in [Table 1].
Table 1: Differentiating features of CCSK, Blastemal WT and mesoblastic nephroma

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The prognosis of clear cell sarcoma is guarded, as it is prone to relapse and spread long after initial diagnosis. The 5-, 10-, and 20-year disease-specific survival rates have been reported as 67%, 33%, and 10%, respectively. The largest determinant in patient prognosis is size of the tumor before surgery: Tumors <5 cm in diameter are associated with much better long-term survival than larger tumors.

In our case, the second tumor found in the preaortic area was a differentiating neuroblastoma. It is an uncommon peripheral neuroblastic tumor, occurring with equal frequency in both the genders and most commonly in young children. The most common sites are adrenal medulla, extra-adrenal retroperitoneum, and posterior mediastinum. Points in favor of good prognosis of differentiating neuroblastoma in our case are age <2 years, extra-adrenal location, and absence of necrosis and rarity of mitosis.

   Conclusion Top

This is one of the rarest cases and to our knowledge probably the first case of a dual tumor with synchronous occurrence of CCSK and differentiating neuroblastoma. The child had presented with totally unrelated complaints. The renal mass was incidentally detected. The preaortic mass was thought to be the lymph node metastasis of the renal mass but turned out to be another unrelated tumor. Dual tumors can pose both diagnostic and therapeutic difficulties. In such situations, marker studies, investigations, immunohistochemistry, and radiographic aids play a very important role not only in diagnosis but also in management. These patients should be followed for a long time for other synchronous and metachronous malignancies.


We thank Dr. Anita Borges and Dr. Jay Mehta (SRL diagnostics) for their valuable contribution by providing us the IHC microphotograph of the above case.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Marsden HB, Lawler W. Bone-metastasizing renal tumour of childhood. Br J Cancer 1978;38:437-41.  Back to cited text no. 1
Warren S, Gates O. Multiple primary malignant tumors: A survey of the literature and a statistical study. Am J Cancer 1932;16:1358-414.  Back to cited text no. 2
Aydiner A, Karadeniz A, Uygun K, Tas S, Tas F, Disci R, et al. Multiple primary neoplasms at a single institution: Differences between synchronous and metachronous neoplasms. Am J Clin Oncol 2000;23:364-70.  Back to cited text no. 3
Takalkar U, Asegaonkar BN, Kodlikeri P, Asegaonkar S, Sharma B, Advani SH. An elderly woman with triple primary metachronous malignancy: A case report and review of literature. Int J Surg Case Rep 2013;4:593-6.  Back to cited text no. 4
Sakellakis M, Peroukides S, Iconomou G, Boumpoucheropoulos S, Kalofonos H. Multiple primary malignancies: A report of two cases. Chin J Cancer Res 2014;26:215-8.  Back to cited text no. 5
Acharya P, Ramakrishna A, Kanchan T, Magazine R. Dual primary malignancy: A rare organ combination. Case Rep Pulmonol 2014;2014:760631.  Back to cited text no. 6
Di Martino E, Sellhaus B, Hausmann R, Minkenberg R, Lohmann M, Esthofen MW. Survival in second primary malignancies of patients with head and neck cancer. J Laryngol Otol 2002;116:831-8.  Back to cited text no. 7

Correspondence Address:
Dr. Preeti Rajeev Doshi
Department of Pathology, Bharati Vidyapeeth University Medical College, Pune, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.200047

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  [Figure 1], [Figure 2]

  [Table 1]


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