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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 38  |  Issue : 6  |  Page : 283-286

Metastatic renal cell carcinoma of pancreas without a detectable primary tumor mimicking pancreatic cancer: A case report and review of the literature


1 Department of Internal Medicine, Division of Hematology and Oncology, Tri-Service General Hospital, National Defense Medical Center, Taipei 114, Taiwan
2 Department of Pathology, Tri-Service General Hospital, National Defense Medical Center, Taipei 114, Taiwan
3 Department of Radiology, Tri-Service General Hospital, National Defense Medical Center, Taipei 114, Taiwan

Date of Submission28-Jan-2018
Date of Decision07-Jun-2018
Date of Acceptance21-Jun-2018
Date of Web Publication22-Oct-2018

Correspondence Address:
Dr. Ping-Ying Chang
Department of Internal Medicine, Division of Hematology and Oncology, Tri-Service General Hospital, National Defense Medical Center, Number 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei
Taiwan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jmedsci.jmedsci_11_18

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  Abstract 

Distant metastases are common in patients with renal cell carcinoma (RCC). Metastatic neoplasms affecting the pancreas account for <2% of all malignancies in the pancreas and RCC is the most common malignancy among them. Metastatic RCC with the nonidentifiable primary renal tumor is extremely rare. In the present study, we reported the case of a 62-year-old male with metastatic RCC to the pancreas without a primary renal tumor mimicking pancreatic cancer. Accurate pathologic diagnosis with immunohistochemistry staining and proper multidisciplinary treatment with targeted therapy may improve the clinical outcomes of these patients.

Keywords: Renal cell carcinoma, carcinoma unknown primary, pancreatic tumor


How to cite this article:
Liu WN, Peng YJ, Chang WC, Chang PY. Metastatic renal cell carcinoma of pancreas without a detectable primary tumor mimicking pancreatic cancer: A case report and review of the literature. J Med Sci 2018;38:283-6

How to cite this URL:
Liu WN, Peng YJ, Chang WC, Chang PY. Metastatic renal cell carcinoma of pancreas without a detectable primary tumor mimicking pancreatic cancer: A case report and review of the literature. J Med Sci [serial online] 2018 [cited 2018 Dec 19];38:283-6. Available from: http://www.jmedscindmc.com/text.asp?2018/38/6/283/237425


  Introduction Top


Renal cell carcinoma (RCC) is the most common kidney cancer.[1] RCC predominantly occurs in men (male-to-female ratio of 1.5:1.0), and the majority of cases are diagnosed in patients aged in the range of 60–70 years.[1] Appropriate histological classification of RCC is important for prognostic and therapeutic strategies. The clear-cell subtype is the most common among all types of RCCs.[2]

RCC is the most common malignancy to metastasize to the pancreas.[3] Moreover, cases of metastatic RCC that present a nonidentifiable kidney mass are rarely reported.[4],[5],[6],[7] Herein, we report the case of a 62-year-old male with metastatic RCC of the pancreas who lacked the presence of a primary renal tumor that mimics primary pancreatic cancer.


  Case Report Top


A 62-year-old male with a medical history of hypertension and type 2 diabetes mellitus presented with abdominal fullness, poor appetite, intermittent tarry stool passage, and body weight loss of 5 kg within 1 month. Upon admission, the patient was afebrile and displayed normal vital signs. Physical examination revealed normal conjunctiva and mild tenderness of the upper abdomen. Laboratory studies revealed a white blood cell count of 10,730/μl (normal, 4500–11,000/μl), hemoglobin of 11.6 g/dl (normal, 12–16 g/dl), platelet count of 375 × 10[3]/μl (normal, 150–400 × 10[3]/μl), aspartate transaminase of 159 U/L (normal <40 U/L), alanine aminotransferase of 293 U/L (normal <40 U/L), total bilirubin of 0.4 mg/dL (normal <1.2 mg/dL), carcinoembryonic antigen of 1.48 ng/ml (normal, 0–5.0 ng/ml), and carbohydrate antigen of 19-9 (CA199) 55.89 ng/ml (normal: 0–37.0 ng/ml). Panendoscopy (PES) exposed the presence of one gastric tumor in the mid-body region, and pathological examination of the limited specimen showed poorly differentiated carcinoma. Contrast-enhanced computed tomography (CT) of the abdomen revealed a well-defined heterogeneous enhancing mass in the pancreatic tail, several contrast-enhanced nodules in the liver, and peritoneal carcinomatosis [Figure 1]a and [Figure 1]b, and no kidney lesions were noted [Figure 1]c. The patient refused a pancreatic tail tumor biopsy. Upon diagnosis of pancreatic tail cancer with multiple metastases to the liver and stomach, the patient received palliative concurrent chemoradiotherapy (CCRT) with weekly gemcitabine treatment.
Figure 1: Abdominal computed tomography showing (a) one pancreatic tail tumor (red arrow) (b) hepatic tumors (red arrows) with contrast enhancement, and (c) normal kidneys

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His tarry stool condition improved after completion of CCRT. However, the patient presented coffee ground vomitus and tarry stool passage 4 months later. The repeated PES revealed the presence of an actively bleeding ulcerative gastric tumor. Histopathologic examination of the gastric cancer biopsy revealed clear-cell neoplasm. Microscopically, the tumor was characterized by neoplastic solid nests with nuclear hyperchromatism and abundant cytoplasm [Figure 2]a. Immunohistochemistry (IHC) showed positive cytokeratin (CK) 18, vimentin, PAX2, PAX8, and focal CD10 staining [Figure 2]b, [Figure 2]c, [Figure 2]d and negative staining for CK 7, CK 20, CD56, synaptophysin, and chromogranin A. The morphological appearances and IHC profiles were suggestive of metastatic renal cell carcinoma. The patient also underwent liver biopsy. The histopathologic and IHC examinations had similar findings.
Figure 2: (a) Tumor cell nests with nuclear hyperchromatism and clear cytoplasm, intermixed with vascular network (H and E stain; ×400). Cells were immunoreactive for (b) cytokeratin 18, (c) vimentin, (d) PAX8 (×400)

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We treated the patient with sunitinib (12.5 mg) three times per day, from March 2013, and his symptoms improved significantly. The follow-up abdominal CT scan, 3 months later, showed decreased size of hepatic and pancreatic tail lesions. Stable disease was noted during follow-up, until August 2014, when the patient developed progressive jaundice, hepatic encephalopathy, and cachexia, due to progression of liver tumors. Palliative radiotherapy was performed for pain relief, and the patient succumbed in August 2014 because of hepatic failure.


  Discussion Top


Metastatic RCC without known primary renal tumor is rare, and only a few cases have been reported in the literature.[4],[5],[6],[7] It remains unclear why primary RCC lesions are occult, but we hypothesize that this may be due to its small size and resolution limits of detection by current imaging techniques. Spontaneous regression of the primary RCC lesion or ectopic RCC development in the kidneys with distant metastases has been reported.[8]

Fine-needle aspiration and core needle biopsies are common procedures used for histological diagnosis of carcinoma with unknown patient origins; however, the amount of specimen is often limited. IHC staining is important for diagnosis and treatment, and IHC markers commonly used for the diagnosis of primary RCC are PAX2, PAX8, RCC marker, CD10, and a combination of vimentin and CK.[9] However, the correct diagnosis of metastatic RCC remains challenging because the morphology of metastatic RCC differs from that of primary RCC. Furthermore, the currently available biomarkers are also used to diagnose other neoplasms.[9] For example, CD10 and PAX8 are helpful for the diagnosis of metastatic RCC, but they are also expressed in many other cancers.

The clear-cell morphology; positive staining for CD10, vimentin, PAX2, and PAX8; and negative staining for CK7 have contributed to the successful diagnosis of clear-cell subtype RCC in previous reports as well as in our case [Table 1]. Shen et al. suggest that the IHC panel for metastatic RCC should include PAX2 or PAX8 and RCC marker or CD10, with auxiliary markers for differential diagnoses.[9] In our case, the aforementioned subdiagnoses were excluded by IHC staining, as we observed negative staining for chromogranin and synaptophysin and positive staining for CD10 and PAX8.
Table 1: Literature review of metastatic renal cell carcinoma with no primary renal lesions

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Antiangiogenic drugs that target the vascular endothelial growth factor (VEGF) pathway, such as sunitinib, have markedly improved the clinical outcomes of patients with metastatic RCC, and sunitinib is considered the first-line treatment option for this disease.[10] Multidisciplinary management is important in advanced RCC, and metastasectomy may be considered in medically fit patients with oligometastatic cancer and long disease-to-recurrence time.[11] Palliative radiotherapy can be used to control bone and brain metastases,[12] and the combination of anti-VEGF tyrosine kinase inhibitors (TKIs) and radiotherapy can enhance disease control and long-term survival.[8],[13] By means of multidisciplinary treatment with sunitinib, our case and previously reported cases [Table 1] have achieved patient survival similar to those reported in clinical trials.[10]

We reported a rare case of multiple metastatic RCC without a known primary renal lesion. We suggest that clear-cell carcinoma RCC should be included as a distinct tumor diagnosis, as the phenotype presents clear-cell morphology without evidence of a primary kidney tumor. IHC staining is essential for an accurate diagnosis, to guide the appropriate targeted therapy. Multidisciplinary treatment, in combination with anti-VEGF TKIs, can improve the clinical outcomes and quality of life of patients diagnosed with this metastatic disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Capitanio U, Montorsi F. Renal cancer. Lancet 2016;387:894-906.  Back to cited text no. 1
    
2.
Lopez-Beltran A, Scarpelli M, Montironi R, Kirkali Z. 2004 WHO classification of the renal tumors of the adults. Eur Urol 2006;49:798-805.  Back to cited text no. 2
    
3.
Reddy S, Wolfgang CL. The role of surgery in the management of isolated metastases to the pancreas. Lancet Oncol 2009;10:287-93.  Back to cited text no. 3
    
4.
Kumar RM, Aziz T, Jamshaid H, Gill J, Kapoor A. Metastatic renal cell carcinoma without evidence of a primary renal tumour. Curr Oncol 2014;21:e521-4.  Back to cited text no. 4
    
5.
Costantino C, Thomas GV, Ryan C, Coakley FV, Troxell ML. Metastatic renal cell carcinoma without evidence of a renal primary. Int Urol Nephrol 2016;48:73-7.  Back to cited text no. 5
    
6.
Johnson MT, Bahnson RR, Zynger DL. Metastatic clear cell renal cell carcinoma to the adrenal gland without an identifiable primary tumor. Int J Urol 2012;19:92-3.  Back to cited text no. 6
    
7.
Heary RF, Agarwal N, Barrese JC, Barry MT, Baisre A. Metastatic renal cell carcinoma, with a radiographically occult primary tumor, presenting in the operative site of a thoracic meningioma: Long-term follow-up: Case report. J Neurosurg Spine 2014;21:628-33.  Back to cited text no. 7
    
8.
Edwards MJ, Anderson JA, Angel JR, Harty JI. Spontaneous regression of primary and metastatic renal cell carcinoma. J Urol 1996;155:1385.  Back to cited text no. 8
    
9.
Shen SS, Truong LD, Scarpelli M, Lopez-Beltran A. Role of immunohistochemistry in diagnosing renal neoplasms: When is it really useful? Arch Pathol Lab Med 2012;136:410-7.  Back to cited text no. 9
    
10.
Choueiri TK, Motzer RJ. Systemic therapy for metastatic renal-cell carcinoma. N Engl J Med 2017;376:354-66.  Back to cited text no. 10
    
11.
Dabestani S, Marconi L, Bex A. Metastasis therapies for renal cancer. Curr Opin Urol 2016;26:566-72.  Back to cited text no. 11
    
12.
Shaikh T, Handorf EA, Murphy CT, Kutikov A, Uzzo RG, Hallman M, et al. Contemporary trends in the utilization of radiotherapy in patients with renal cell carcinoma. Urology 2015;86:1165-73.  Back to cited text no. 12
    
13.
Venton G, Ducournau A, Gross E, Lechevallier E, Rochwerger A, Curvale G, et al. Complete pathological response after sequential therapy with sunitinib and radiotherapy for metastatic clear cell renal carcinoma. Anticancer Res 2012;32:701-5.  Back to cited text no. 13
    


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