Management of renal extraskeletal mesenchymal chondrosarcoma

Abstract

BackgroundPrimary mesenchymal chondrosarcoma of the kidney is an extremely rare malignant tumor. To our best knowledge, only 9 such cases have been reported so far.Case presentationIn the current paper, we present the case of a 67 year-old patient with recurrent left lumbar pain, increased fatigability and intermittent macroscopic hematuria. He underwent a surgical resection of the left kidney and left hemicolon.The pathological diagnosis was primary extraskeletal renal mesenchymal chondrosarcoma. Overall survival was 9 months, with pulmonary metastasis and local recurrence at 6 months. The management of the patient is described, from the initial differential diagnosis, after the first clinical examination to the surgical resection, with a special emphasis on the surgical procedures that were carried out.ConclusionExtraskeletal chondrosarcoma of primary origin in the kidney are extremely rare tumors with a highly malignant potential and very poor prognosis. Because the role of chemotherapy or radiation therapy has not been evaluated properly yet, we underline the importance of surgery in the management of such cases as the main and best approach to achieve clinical remission and long-term survival, provided the patient is referred to a surgical consult in time.

Robot-assisted radical nephrectomy for primary renal mesenchymal chondrosarcoma: case report and literature review

“…A peripheral distribution of MC is found in patients aged 50 years and older, while the peak incidence for MC is in the 2nd–3rd decade of life [15–17]. As shown in Table 2, seven patients are aged 10–30 [1,3,6–9,13] while four cases [2,10–12] and one of our cases are aged ≥50, which was consistent with the former conclusion. MC occurs in both genders equally [16], while extraskeletal MC has a mild female preponderance [4].…”

Renal Extra Skeletal Mesenchymal Chondrosarcoma: A Case Report

“…It represents only 2% of all chondrosarcomas. 3 MC of the kidney is a very rare entity. In literature review, only nine cases of it have been reported so far.…”

“…Although the role of systemic chemotherapy or radiotherapy has not been evaluated in RMC, it seems that complete surgical resection with clear margins and adjuvant chemotherapy increase the patient's survival rate. 3 , 5 Recommended chemotherapy regimen for MC is a combination of vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide. Increased expression of the platelet-derived growth factor receptor α (PDGFR-α) was reported in malignant mesenchymal cells, so target agents such as dasatinib, imatinib, sorafenib and sunitinib that inhabit PDGFR-α function may play a role in treatment of MC.…”

“…To the best of our knowledge, only nine cases of MC of the kidney have been described in English literature so far. 3 …”

Gab1 regulates SDF-1-induced progression via inhibition of apoptosis pathway induced by PI3K/AKT/Bcl-2/BAX pathway in human chondrosarcoma

“…In recent years, some novel approaches to CS patients remain unsatisfactory because the postoperative patients often have the recurrence of CS, even death. Thus, the most important way to treat CS patients should be the early definitive diagnosis [5]. In recent decades, EMT has been widely reported as an initiation process in CS development, while the specific mechanisms involved in EMT of CS is still unclear.…”