Primary mesenchymal chondrosarcoma of the kidney with synchronous implant and infiltrating urothelial carcinoma of the ureter
© Xu et al.; licensee BioMed Central Ltd. 2012
Received: 19 June 2012
Accepted: 17 September 2012
Published: 21 September 2012
Primary mesenchymal chondrosarcoma of the kidney is rare, and it shows distinct undifferentiated tumor cells and well differentiated cartilagenous components. Also assident infiltrating urothelial carcinoma of the ureter is an extremely rare cancer. We report a case of primary mesenchymal chondrosarcoma occurring in the left kidney with an ipsilateral and distinct distal ureteric implant, and a coexisting infiltrating urothelial carcinoma of the ureter in a 64-year-old man. Histopathological examination and immunohistochemical studuies showed the classic features of mesenchymal chondrosarcoma in kidney, as well as a few infiltrating urothelial in ureter. Multitarget fluorescence in situ hybridization (FISH) suggested that the development of the urothelial carcinoma in the ureter may be triggered or induced by the chondrosarcoma component. The patient died 2 month after left nephro-ureterectomy. This is the first reported case of a primary mesenchymal chondrosarcoma of the kidney with coexisting infiltrating urothelial carcinoma of the ureter.
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KeywordsMesenchymal chondrosarcoma Urothelial carcinoma Kidney Ureter
Mesenchymal chondrosarcoma was first reported by Lichtenstein and Bernstein in 1959. This malignant tumor mostly arises in the skeleton, and one third of the cases arise in the soft tissue and other organs. Extraskeletal mesenchymal chondrosarcoma is rare, and uaually arises in the head and neck region, followed by the lower extremity, the trunk and the retroperitoneum. Primary mesenchymal chondrosarcoma of the kidney is extremely rare, and the first case was published 25 years ago by Malhotra. To date, there are only seven cases of primary renal chondrosarcoma reported in the English literature[2–7]. We report another case of mesenchymal chondrosarcoma of the kidney with synchronous implant and a coexistent infiltrating urothelial carcinoma of the ureter firstly.
A 64-year-old man presented with gross hematuria and vague pain in the left loin. His medical history was unremarkable. Physical examination revealed percussion pain over the left kidney region. Urinalysis showed positive for protein and red blood cells. Abdominal B ultrasonography revealed left hydronephrosis and a hypoechoic mass in the inferior segment of the left ureter. Magnetic resonance imaging (MRI) confirmed left hydronephrosis, a low signal mass in the upper pole of the kidney, and another mass with high T1, low T2 signal in the inferior segment of the left ureter. Imaging examination showed no abnormality elsewhere. A clinical diagnosis of malignancy of the left kidney and the ureter was made, for which a left nephro-ureterectomy was performed.
The ratio of gene copy numbers to chromosome 3,7and 17 centromere by FISH probes in urothelial carcinoma and mesenchymal chondrosarcoma
Gene copy numbers of the p16 (9p21) gene by FISH probe
The patient died of the widespread metastases two months after surgery, autopsy was not performed.
On review of the literature, only seven cases of renal mesenchymal chondrosarcoma have been reported. Here we first describe an interesting case of renal mesenchymal chondrosarcoma with a synchronous ureter implant, which was intimately mixed or in collision with an urothelial carcinoma. The biphasic growth pattern of highly cellular undifferentiated tumor cells, with islands of differentiated cartilage is rather characteristic of mesenchymal chondrosarcoma. Such a distinctive morphology, with the absence of osteoid formation set mesenchymal chondrosarcoma apart from small cell osteosarcoma, Ewing’s sarcoma, dedifferentiated chondrosarcoma, and hemangiopericytoma.
It is important to note that the absence of epithelial component and of an underlying urothelial carcinoma from an extensively sampled tumor, is an indication of pure mesenchymal chondrosarcoma arising from the kidney. The lack of immunoreactivity for epithelial markers, suck as cytokeratin AE1/E3, EMA and E-cadherin lends further support to it. Moreover, the tumor located in the renal parenchyma and distant inferior ureter, not in the calyx and pelvis, the relatively normal collecting system and urothelium that separates the renal tumor and the distal ureteric mesenchymal chondrosarcoma is keeping with the label of tumor implant.
As for the lower ureteric tumor, the transition between the large bulk of mesenchymal chondrosarcoma and the relatively small urothelial carcinoma was abrupt. Moreover, there was no apparent transition between the carcinoma and the ureteric mucosa or epithelium, which is free of dysplasia or neoplastic field changes. Such an observation support a collision of two separate tumors in the lower ureter. There were some reports on a collision of separate tumors in the same location of the urinary system[9, 10]. While only vascular invasion is observed with mesenchymal chondrosarcoma, and no observed with urothelial carcinoma, such a pattern of metastasis does not necessarily imply that the two malignancies are biologically distinct.
From the view of histogenesis, some groups have proposed that a cell could generate a precursor with greater potential than the cell from which it derives, or transdifferentiation, that allows cell lineage switch. Several such multipotential malignant sarcoma have been reported, for example osteosarcoma and Ewing’s sarcoma with epithelial differentiation. The primary tumor cell in mesenchymal chondrosarcoma represents a very primitive mesenchymal cell type. Daniel Rubio clearly demonstrated a novel mesenchymal-epithelial transition (MET) associated epithelial tumorigenesis. And a work showing MET in vitro using a chondrosarcoma tumor cell line has been reported. Fitzgerald MP reported Chondrocytes are mesenchymally derived cells that reportedly acquire some epithelial characteristics, and there is an epigenetic switch associated with an MET-like phenomenon that accompanies chondrosarcoma progression. These observations support the hypothesis that not all carcinomas are derived from epithelial cells. In our present case, both the urothelial carcinoma and the mesenchymal chondrosarcoma all exhibited polysomy of chromosome 3, 7 and 17, the difference was significant (p < 0.05, chi-square test, SPSS 13.0), and loss of p16 gene by fluorescence in situ hybridization (FISH). The development of urothelial carcinoma had been linked to chromosomal instability, especialy chromosome 3,7and 17 and partial or complete loss of chromosome 9 ( p16 locus) . It is thus conceivable that the development of urothelial carcinoma may be triggered or induced by chondrosarcoma, especially since both shared the same clonal origin.
The cytogenetic and molecular mechanism on renal mesenchymal chondrosarcoma is indefinite. Lee AF showed that, in contrast to Ewing sarcoma, small cell osteosarcoma and mesenchymal chondrosarcoma lack FLI-1(Friend leukemia virusintegration-1) immunoreactivity. FLI-1 is therefore useful in the differential diagnosis of small round blue cell tumors of the bone. Wang L described the novel HEY1-NCOA2() fusion appears to be the defining and diagnostic gene fusion in mesenchymal chondrosarcomas. Nevertheless, the progression mechanism of renal mesenchymal chondrosarcoma remains to be developed investigated.
The prognosis for mesenchymal chondrosarcoma arising from bone and soft tissue is dismal. Of the seven cases of primary renal mesenchymal chondrosarcoma reported in the English literature, four cases developed local recurrences or metastases at an interval of 1 month to 2.5 years after surgical intervention. In the current case , whether the ureteric tumor represented a direct intraluminal seeding and ‘implant’ ,or a second primary arising from a background with field change remains speculative, but given its highly aggressive nature, it would be tempting to postulate that this highly aggressive tumor shows a propensity for intra-calyxceal spread. The autopsy was not performed, however vascular mesenchymal chondrosarcoma metastasis detected may implicate mesenchymal chondrosarcoma, rather than carcinoma as the lethal metastasis.
Written informed consent was obtained from the next of kin of the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Magnetic resonance imaging
Sex determining region Y-box 9
Fluorescence in situ hybridization
Friend leukemia virusintegration-1
Mesenchymalto epithelial transition.
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