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Case Reports and Images in Pathology

 
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Case Report
 
Simultaneous occurrence of renal cell carcinoma and angiomyolipoma in the same kidney
Tuba Dilay Kokenek-Unal1, Ipek Coban2, AyseSelcen Oguz-Erdogan3, Hatice Karaman4, Murat Alper5
1MD, Department of Pathology, Kayseri Research and Training Hospital, Kayseri, Turkey.
2MD, Asst. Prof., Department of Pathology Gayrettepe Florence Nightingale Hospital, Istanbul, Turkey.
3MD, Department of Pathology, Rize State Hospital, Rize, Turkey.
4MD, Assoc. Prof., Chairman, Department of Pathology, Kayseri Research and Training Hospital, Kayseri, Turkey.
5MD, Prof., Chairman, Department of Pathology, DiskapiYildirimBeyazit Research and Training Hospital, Ankara, Turkey.

Article ID: 100001Z11TU2015
doi:10.5348/Z11-2015-1-CR-1

Address correspondence to:
Tuba Dilay Kokenek-Unal
Yakut Mah. Akmescid Cad. 3850
Sok. 3/30 Kocasinan, Kayseri
Turkey
Phone: +90 505 934 3329; +90 352 320 73 13

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How to cite this article:
Kokenek-Unal TD, Coban I, Oguz-Erdogan AS, Karaman H, Alper M. Simultaneous occurrence of renal cell carcinoma and angiomyolipoma in the same kidney. J Case Rep Images Pathol 2015;1:1–6.


Abstract
Introduction: Renal cell carcinoma and angiomyolipoma are well-known tumors of kidney. However, coexistence of these tumors in the same patient is a rare condition. Synchronous occurrence of renal cell carcinoma and angiomyolipoma is more commonly seen in patients with tuberous sclerosis.
Case Report: A 76-years-old female was admitted to urology clinic with a complaint of flank pain. She had pain and tenderness on her right flank on physical examination. Radiological imaging revealed a solid mass in right kidney. She underwent right radical nephrectomy.
Conclusion: We report a case of clear cell renal cell carcinoma and angiomyolipoma occurring in the same kidney without stigmata of tuberous sclerosis and with the review of related literature.

Keywords: Angiomyolipoma, Renal cell carcinoma, Simultaneous neoplasms


Introduction

Renal cell carcinoma (RCC) is a common neoplasm in general population. Clear cell (conventional) type, the most common, accounts for approximately 60% of all renal tumors. It affects primarily adults and shows male predominance. Angiomyolipoma is an uncommon benign mesenchymal tumor of kidney. Sporadic form is nearly 1% of surgically removed renal tumors but it may be seen more commonly in association with tuberous sclerosis (TS). It preferably affects female adults in sporadic form, but there is no sex predilection in TS patients [1]. However, coexistence of these tumors was reported in limited number in literature. We represent such a unique case in this report.


Case Report

A 76-year-old female was admitted to urology clinic with a new onset of right flank pain. On physical examination, she had tenderness and pain on her right flank area. Laboratory results showed minimal inflammation and blood urea nitrogen level was elevated. Abdominal CT revealed a solid mass in lower pole of the right kidney with cystic necrotic areas showing irregular inferior margin with an exophytic extension. Besides, there were many cysts located in the parapelvic region on radiological examination. The patient underwent to right radical nephrectomy with suspect of renal tumor.

Surgical specimen weighted 485 g and was 20x9x6.5 cm in size with perirenal adipose tissue. On cross section, two well-defined lesions were seen in the lower pole of the kidney (Figure 1). Main lesion had solid cystic appearance and dirty yellow color and it was 5 cm in diameter. It had a small protrusion in the inferior part but it did not exceed to the renal capsule. The other lesion, misconceived as an extension on imaging, was 3 cm in diameter with solid and yellowish appearance and separated from main lesion with a definite margin. Even though radiological imaging did not disclose any difference between these two lesions, each of them has distinct histological features on microscopical examination. Main lesion consisted of tumor cells in solid sheets, in which occasional dilated acinar structures formed a cystic pattern. The tumor cells had round, uniform nuclei with finely granular open chromatin and inconspicuous nucleoli and mainly clear cytoplasm surrounded by definite margins. Besides, regular small thin walled vessels were observed throughout the lesion (Figure 2A). It was diagnosed with conventional type RCC (Fuhrmann grade 2). This lesion was stained positively with CD10 and Vimentin by immunohistochemistry. The other lesion was separated by a thin fibrous capsule from the main lesion (Figure 2B) It was composed of mature adipocytes, smooth muscle cells and poorly organized thick walled blood vessels (Figure 2C). It was diagnosed with angiomyolipoma. Immunohistochemically, angiomyolipoma was stained by HMB-45, while there was no expression of HMB45 in RCC. Surgical margins including ureter and renal vein were free of tumor. The patient had neither lymph node nor distant organ metastasis.


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Figure 1: Macroscopic appearance of surgical specimen.



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Figure 2: Microscopic appearance of collision tumor. (H&E: Hematoxylin & Eosine staining). (A) Renal cell carcinoma: malignant tumor composed of cells with oval nuclei, inconspicuous nucleoli and clear cytoplasm, (H&E stain, x200). (B) Note the thin fibrous capsule separating two neoplasms. (H&E stain, x100), and (C) Angiomyolipoma: benign tumor composed of a mixture of adipocytes, blood vessels and smooth muscle (H&E stain, x200).



Discussion

Conventional type RCC is the most common and poorly understood tumor in both clinical and pathological aspects. Hereditary factors may play an important role in development of RCC and also RCC can be associated with familial disorders. In our case, there was no known family history of renal tumor or related familial diseases and hereditary disorders. Like RCC, angiomyolipoma can be associated with TS, an autosomal dominant disorder. Although angiomyolipoma shows higher incidence in association with TS, both RCC and angiomyolipoma can be associated with TS. The synchronous presentation of renal cell carcinoma and angiomyolipoma was described in the literature in both healthy patients and patients with TS [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23]. In 2001, Jimenez et al. denoted that approximately 50 cases had been reported in the literature [2]. They also reported coexistent RCC and angiomyolipoma of 23 cases in a study of 36 cases, which is also summarized in Table 1 [2]. In addition to that, we listed 24 more cases, including our case in Table 1 [3] [4] [5] [6] [7] [8] [9] [10][11] [12] [13] [14] [15] [16] [17] [18] [19] [20] [21] [22] [23].

Although RCC affects males twice as frequently as females in sporadic cases, it is seen more commonly in female patients with TS [2]. However angiomyolipoma has no female predominance in TS patient. Our patient was a woman but had no clinical signs of TS. Jimenez et al. suggested that coexistent neoplasms were more frequently in woman. In concurrent tumors, angiomyolipoma is mostly found incidentally [2]. The data in literature indicate that there is no considerable difference in coincidence of these tumors between in patients with and without TS (14 patients with TS, 31 patients without TS: see Table 1). Besides, left kidney is likely to be involved. Even though it was not indicated separately in all reports, the most common subtype of RCC, coexists with angiomyolipoma was clear cell RCC, with 26 cases. This may be easily explained by the fact that clear cell RCC is the most common subtype. Since the management of treatment differs in different renal tumors, interpretation of radiological imaging takes a critical place in surgical approach [3]. A huge angiomyolipoma may mislead to diagnosis of a RCC, or in the same manner a blood vessel rich RCC may imitate an angiomyolipoma. In our case, preoperative imaging did not rule out the presence of different lesions and angiomyolipoma was found incidentally. Although it is a rare occasion, the coexistence of RCC or even malignant transformation should be considered in follow-up of angiomyolipoma patients, particularly with TS.


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Table 1: Documented cases of coexistence of renal cell carcinoma and angiomyolipoma in the same kidney.




Conclusion

In summary, we represent an uncommon case of concomitant renal cell carcinoma and angiomyolipoma in the same kidney. The presence of any pathogenetic relationship between these two tumors still remains unclear. We listed a series of cases and hope that our report will be helpful for other researchers in further investigation of simultaneous renal neoplasms.


Acknowledgements

We thank to YesimUndeser for her help in laboratory works.


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Author Contributions
Tuba Dilay Kokenek-Unal – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Ipek Coban – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Ayse Selcen Oguz-Erdogan – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Hatice Karaman – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Murat Alper – Conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Critical revision of the article, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2015 Tuba Dilay Kokenek-Unal et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.





About The Authors

Tuba Dilay Kokenek-Unal is a Pathologist in Kayseri Research and Training Hospital in Kayseri, Turkey.



Ipek Coban is Assistant Proffesor at Pathology Department of Florence Nightengale Hospital in Istanbul, Turkey. She has published many research papers in national and international academic journals. Her research field is mainly pancreatobiliary system.



Ayse Selcen Oguz-Erdogan is a Pathologist at Pathology Department of Rize State Hospital. She is interested in molecular pathology field.



Hatice Karaman is an Associate Professor and head of department at Pathology Department of Kayseri Research and Training Hospital. She has published many research papers in national and international journals.



Murat Alper is Head of Department at Diskapi Research and Training Hospital in Ankara,Turkey. He has published many research papers in national and internationl journals. His main research topic is dermatopathology.


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