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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 2  |  Issue : 3  |  Page : 74-77

Bilateral sarcomatoid renal cell carcinoma: An uncommon case in young female patient


1 Graduate School of Shanxi Medical University, Taiyuan, Shanxi, China
2 Department of Urology, The First Hospital of Shanxi Medical University, Taiyuan, Shanxi, China

Date of Submission23-Feb-2017
Date of Acceptance24-Jun-2017
Date of Web Publication15-Sep-2017

Correspondence Address:
Weibing Shuang
Department of Urology, The First Hospital of Shanxi Medical University, No. 85, Jiefang South Road, Yingze District, Taiyuan 030001, Shanxi
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ts.ts_6_17

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  Abstract 

Sarcomatoid renal cell carcinoma (SRCC), an advanced and uncommon form of renal cell carcinoma (RCC), is more likely to manifest as a unilateral condition, especially in the right kidney, in older people. Here, we report a rare case of bilateral SRCC in a young female, aged 30 years - the youngest reported case of SRCC to the best of our knowledge. The patient complained of osphyalgia and intermittent fever which recurred after symptomatic treatment. Detailed examination, including histopathological analysis of biopsies, confirmed bilateral SRCC (RCC IV) with distant metastases. The patient denied receiving any form of the treatment, including surgery, radiotherapy, and chemotherapy and died approximately 6 months after the initial symptoms.

Keywords: Bilateral sarcomatoid renal cell carcinoma, natural course, young female


How to cite this article:
Gao Y, Shuang W, Tong X, Zhang Y. Bilateral sarcomatoid renal cell carcinoma: An uncommon case in young female patient. Transl Surg 2017;2:74-7

How to cite this URL:
Gao Y, Shuang W, Tong X, Zhang Y. Bilateral sarcomatoid renal cell carcinoma: An uncommon case in young female patient. Transl Surg [serial online] 2017 [cited 2017 Nov 23];2:74-7. Available from: http://www.translsurg.com/text.asp?2017/2/3/74/214808


  Introduction Top


Sarcomatoid renal cell carcinoma (SRCC), a rare variant of renal cell carcinoma (RCC), represents only 5%–8% of RCC.[1] This entity was first described in 1968 by Farrow et al.[2] SRCC is considered as a stage of the high-grade transformation of all primary RCC, instead of an independent pathological subtype, and it can occur in all histological subtypes of RCC and urothelial tumor of renal pelvis. The SRCC easily infiltrates the surrounding tissue and metastasizes to distant organs, especially lungs and bones, and thus is associated with an extremely poor prognosis with a survival period of 9–19 months.[3] Epidemiological data show that SRCC is more common in the elderly, >50 years of age, with an average age of onset of about 60 years (33–81 years old), with a male and female prevalence ratio of 1.6:1. Moreover, SRCC in unilateral kidney, especially in the right, is common in clinical cases. Here, we report a rare case of bilateral SRCC in a young patient with a natural course of 6 months.


  Case Report Top


A 30-year-old female presented with the history of osphyalgia and intermittent fever for 1 month. Although her symptoms improved after symptomatic treatment, she was admitted to Department of Infections of the First Hospital of Shanxi Medical University due to palindromia of preceding symptoms, 2 weeks later. During the physical examination, the inferior pole of the right kidney could be palpated in the region below costal arch, while the superficial lymph nodes were not enlarged. The computed tomography (CT) scan and spiral CT 3-D imaging of the abdomen showed a rounded opacity at the middle pole of the right kidney and multiple enlarged lymph node lesions at the right renal hilum and retroperitoneum which indicated a possibility of lymphoma. A mass at the upper pole of the left kidney was also observed [Figure 1] and [Figure 2]. Bone window showed multiple lesions in thoracic and lumbar vertebrae, and sacrum, indicating a possibility of bone metastasis or invasion. Hepatic atypical hemangioma was a major concern on account of nodular enhancement in the arterial phase, at the right posterior lobe of liver. The patient was then admitted to Department of Urology for the further assessments of renal space-occupying lesions in both the kidneys. Magnetic resonance imaging scan of the abdomen revealed an enlarged right kidney, 128.9 mm × 73.8 mm in size, the inferior pole of which demonstrated an irregular equilong T2 sign measuring 59.2 mm × 82.5 mm [Figure 3]. A small round-like equilong T2 sign, 20.1 mm × 15.9 mm in size, with a localized mass measuring 113.4 mm × 68.4 mm protruding across the outline was detected at the upper pole of left kidney [Figure 3]. Within the scan slices, multiple flaky long T2 signs were detected at ilia, pubes, ischia and sacrum, the right femoral head, and greater trochanter. Further, an ovoid long T2 sign, 19.5 mm × 14.5 mm in size, was observed in the right adnexal area [Figure 3]. Bone scan showed multifocal hypermetabolic lesions in the systemic skeleton, indicating a possibility of bone metastasis of malignant lesions. A positron emission tomography-CT scan was performed and detected bilateral enlarged kidneys with hypermetabolic parenchyma, which was more prominent in the right kidney. Both suprarenal glands were enlarged and hypermetabolic. Multiple hypermetabolic lesions were observed on the uterine wall. Multiple enlarged lymph nodes with hypermetabolism were detected at the left hilum of lung and retroperitoneum. Multiple hybrid bone destruction and hypermetabolism were evident across systemic skeleton. Especially, the lesion located in the range of the first and second sacrum involved the rear vertebral canal. The results indicated toward lymphoma involving multiple locations and Langerhans cell histiocytosis before examination of the bone marrow, obtained from the posterior iliac spine and renal biopsies.
Figure 1: The computed tomography scans showed a rounded opacity at the middle pole of the right kidney and multiple enlarged lymph node lesion at the right renal hilum and retroperitoneum. There was a mass at the upper pole of the left kidney

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Figure 2: The spiral computed tomography 3-D imaging showed a rounded opacity at the middle pole of the right kidney and a mass at the upper pole of the left kidney

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Figure 3: Magnetic resonance imaging scans revealed an enlarged right kidney, 128.9 mm × 73.8 mm in size, on whose inferior pole, an irregular equilong T2 sign measuring 59.2 mm × 82.5 mm was revealed. A small round-like equilong T2 sign, 20.1 mm × 15.9 mm in size, with a localized mass protruding the outline of the kidney was revealed at the upper pole of left kidney measuring 113.4 mm × 68.4 mm

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Routine tests of tumor and bone marrow did not show any significant anomalies. No significant abnormal immunophenotypic cells were revealed except for a high ratio of granulocyte in CD45/SSC gating. Ultrasound-guided core needle biopsy of both renal masses was performed. The pathological diagnosis was bilateral SRCC (RCC IV) [Figure 4]. Immunohistochemical analysis showed that the tumor cells were strongly positive to vimentin and cytokeratin (CK), positive to epithelial membrane antigen and embryonal carcinoma [Figure 5], and negative to CK7, CD10, CD117, HMB45, and Melan A.
Figure 4: Ultrasound-guided puncture of both renal masses was performed. The pathological diagnosis was sarcomatoid renal cell carcinoma (renal cell carcinoma IV, Hematoxylin and Eosin stain. Right kidney [a and b]; left kidney [c and d])

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Figure 5: Immunohistochemically, vimentin (a) and cytokeratin (b) were strongly positive, epithelial membrane antigen (c) and embryonal carcinoma (d) were positive

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The patient opposed to undergo surgery, radiotherapy, and chemotherapy for the disease, and autonomously ingested sorafenib, a vascular endothelial growth factor (VEGF)-targeted agent, at a dose of 800 mg/day. However, the patient stopped the drug 2 days later due to the associated side effects; nausea and vomiting. Then, the patient chose to take everolimus, a mammalian target of rapamycin (mTOR) inhibitors, at a dose of 10 mg/day. At the reappearance of the side effects of nausea and vomiting, she discontinued this drug too. Subsequently, the symptoms of osphyalgia aggravated, followed by right lower extremity restraint. Moreover, her ability to perform activities of daily living gradually decreased. Later, the patient died due to multiple organ dysfunction syndrome, approximately 6 months after the initial symptoms of osphyalgia and fever.


  Discussion Top


The present case is one of the few reported bilateral SRCC, a rare event of the uncommon tumor of renal parenchyma. In this report, the initial symptoms of the patient were osphyalgia and intermittent fever. Osphyalgia is one of the most common symptoms in RCC, while fever may fall within the ambit of paraneoplastic syndromes. Paraneoplastic syndromes associated with RCC include hypercalcemia, hypertension, polycythemia, nonmetastatic hepatocellular enzyme elevation (Stauffer syndrome), fever, weight loss, cachexia, Cushing's syndrome, abnormalities in glucose metabolism, neuromyopathies, amyloidosis, anemia, dermatomyositis, galactorrhea, and glomerulopathies.[4],[5],[6] These can be the initial manifestations of the occurrence or recurrence of the condition.[5]

In the present case, given that the patient already had many metastatic sites, it is possible that the tumor in one of the kidneys is the metastatic site of the other. However, since genetic analysis was not performed, we could not determine which side was the primary and which side was metastatic. The definite diagnosis was confirmed as bilateral SRCC (RCC IV) according to pathological findings and immunohistochemistry. Considering the clinical findings, this is termed as the terminal bilateral SRCC case, the natural course of which is 6 months because of the lack of the available treatments: surgery, radiotherapy, chemotherapy, and targeted therapy for the tumor.

SRCC is typically an advanced stage of renal cancer and is associated with a poor prognosis.[7],[8],[9],[10] The current data show that most patients will die in the first 3 months after being diagnosed with SRCC.[11] The effect of tumor excision on survival in terminal SRCC, with distant metastases, remains unclear in the literature review. Shuch et al.[12] report that the metastatic RCC patients, with sarcomatoid components, undergoing cytoreductive nephrectomy have a poor prognosis despite aggressive surgery and postoperative therapy. Other treatments, such as radiotherapy, chemotherapy, and immunotherapy, reportedly have a poor curative effect on the condition.[13] Adjuvant chemotherapy and targeted agents, investigated for their survival effect in recent studies, had a low response rates in metastatic SRCC.[14],[15],[16],[17],[18] In addition, data related to adjuvant radiation therapy in metastatic or locally recurrent SRCC are very limited.[19],[20] Lekili et al.[21] reported an excellent response of metastatic SRCC case to sorafenib administration. In another report, 15 patients who had predominately sarcomatoid RCC were treated with sorafenib, postgemcitabine plus doxorubicin administration. No positive response was seen with gemcitabine and doxorubicin, while one partial response (3 months) and four stable diseases (range, 3–9 months) were documented when patients received sorafenib.[22] Another study concluded that patients with metastatic RCC involving sarcomatoid differentiation can demonstrate objective response and tumor shrinkage to VEGF-targeted therapy.[23] Voss et al.[24] indicated in their study that a subset of patients with nonclear-cell and sarcomatoid variant clear-cell RCC subtypes benefit from mTOR inhibitors, but most have poor outcome. A standardized therapy regime for SRCC is not yet available due to the rarity of SRCC and the scarcity of information regarding its biology.[11] Further studies/reports are essential in this regards.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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