Pediatric Case Report Primary Renal Mesenchymal Chondrosarcoma: A Case Report Michael B. Rothberg, Amul A. Bhalodi, Edward F. Reda, Paul Zelkovic, and Israel Franco A 16-year-old female patient presented to our emergency department with a left upper extremity radiculopathy prompting several imaging studies. Magnetic resonance imaging of the neck revealed multiple cervical vertebrae lesions and computed tomography imaging demonstrated a 15-cm calciﬁed mass originating from the left kidney and extending into the surrounding structures. Pathologic assessment of the open left radical nephrectomy specimen revealed a primary renal mesenchymal chondrosarcoma, a tumor that has only been documented in 5 prior case reports in the literature. UROLOGY 85: 676e678, 2015. 2015 Elsevier Inc.
esenchymal chondrosarcoma is an extremely rare tumor typically found in the bone and rarely arising from extraskeletal soft tissues. First described by Litchtenstein and Bernstein in 1959, this malignancy comprises cells with cartilaginous differentiation intermixed with other cells of poor differentiation.1-2 Prior case series of extraskeletal mesenchymal chondrosarcomas have cited tumor origins in the meninges, head and neck regions, extremities, and the retroperitoneum; however, to our knowledge, very few cases arising from the kidney have been described in the literature.3-4 We report the case of a 16-year-old female patient with a massive mesenchymal chondrosarcoma of renal origin that had metastasized to the cervical spine, femur, and lung to provide for an unusual clinical presentation.
CASE REPORT A 16-year-old girl presented to the emergency department with a 2-year history of vague abdominal pain and a 6month history of progressively worsening left-sided neck, left shoulder, and left upper extremity pain and numbness associated with a constant headache. Her left upper extremity pain was exacerbated by a recent fall, prompting her presentation. On further examination, the patient was unable to voluntarily move her left upper extremity, raising suspicions for a musculoskeletal injury. A subsequent magnetic resonance imaging of the cervical spine demonstrated a degenerative lesion at the C5-C7 vertebrae with mild cord compression and degenerative lesions at the C1C2 vertebrae. In search of a primary lesion or other metastases, a computed tomography scan with contrast of the thorax, abdomen, and pelvis was performed, revealing a Financial Disclosure: The authors declare that they have no relevant ﬁnancial interests. From the Section of Pediatric Urology, Department of Urology, New York Medical College, Valhalla, NY Address correspondence to: Israel Franco, M.D., Pediatric Urology Associates, 150 White Plains Rd., Tarrytown, NY 10591. E-mail: [email protected]
Submitted: August 25, 2014, accepted (with revisions): November 7, 2014
ª 2015 Elsevier Inc. All Rights Reserved
15.2 9.2 10.8 cm exophytic heterogeneous mass arising from the mid to lower pole of the left kidney and extensive adenopathy at the renal hilum and periaortic nodes, as seen in Figure 1A,B. This mass was largely calciﬁed, and its local mass effect displaced retroperitoneal structures and the major renal vasculature anteriorly. A 0.8-cm lung nodule was also identiﬁed at the tip of the lingula anteriorly abutting the diaphragm. The patient was taken to the operating suite for an open left radical nephrectomy of the suspected primary tumor. Once the left kidney and mass were exposed, a large adhesion was noted along the mesentery of the distal descending colon, requiring segmental colon resection and anastomosis. Meticulous dissection was required to ligate numerous, large, parasitic, lumbar vessels supplying the mass and to mobilize aspects of the tumor that extended into the left renal vein and the inferior vena cava. After 8 hours of dissection, the posterior aspect of the kidney and mass was mobilized for removal. There were no intraoperative complications, and the estimated blood loss was 1000 mL. The gross specimen, as seen in Figure 2A,B, weighed 1315 g and measured 19.5 15.6 10.5 cm. The surface of the tumor had a hard consistency, and the Gerota fascia surrounding the kidney was quite ﬁxed. Photomicroscopy (Fig. 3) of the tumor demonstrated 2 components: (1) sheets of small blue cells and (2) mature hyaline cartilage. Intermediate areas of chondrogenesis with immature chondroblasts with clear cytoplasm can be identiﬁed, as well as small regions of ossiﬁcation.
COMMENT We report what is believed to be only the sixth case of mesenchymal chondrosarcoma arising from the kidney in the youngest patient to be described in the literature.5-9 Nearly all the previously reported cases of this tumor presented with nonspeciﬁc symptoms of costovertebral angle or abdominal pain and gross hematuria. Whereas the symptoms directly related to our patient’s metastatic http://dx.doi.org/10.1016/j.urology.2014.11.032 0090-4295/15
Figure 2. (A and B, top and bottom, respectively). Radical nephrectomy gross specimen (top) and bivalved gross specimen (bottom). Figure 1. (A and B, top and bottom, respectively). Computed tomography scan with contrast of the abdomen and pelvis demonstrating a heterogeneous calciﬁed mass arising from the left kidney (top) along with extensive adenopathy at the renal hilum and periaortic lymph nodes, which, along with the mass, abut the major renal vasculature, the aorta, and the inferior vena cava (bottom).
disease prompted her arrival to the emergency department, metastases typically have not been described as part of the initial presentation for primary renal mesenchymal chondrosarcoma. Moreover, the heterogeneous and calciﬁed appearance of this lesion on computed tomography imaging, along with the presence of signiﬁcant perirenal lymphadenopathy, placed metastatic renal cell carcinoma at the forefront of our differential diagnosis, and prompted our decision to pursue surgical intervention. Although an optimal chemotherapeutic regimen for primary renal mesenchymal chondrosarcoma has not yet been deﬁned because of the rarity of this disease process, UROLOGY 85 (3), 2015
Cesari et al report on surgical and chemotherapeutic outcomes for primary mesenchymal chondrosarcoma in bone and extraskeletal sites. In their series, 28 patients with mesenchymal chondrosarcoma underwent surgical resection of the primary lesion, radiation therapy, and/or chemotherapy, which typically mirrored contemporary regimens for either Ewing sarcoma or osteosarcoma based on whether the tumor was histologically “Ewing tumorlike” or “hemangiopericytoma-like” in architecture, respectively.10 They emphasize the importance of complete surgical remission of the disease, demonstrating signiﬁcantly improved 10-year overall survival (vs patients without surgical remission) and signiﬁcantly improved 10-year disease-free survival when also receiving chemotherapy (vs patients with surgical remission and no chemotherapy). A similar analysis of 35 patients by Huvos et al,4 in which a vast majority of patients received both neoadjuvant and adjuvant chemotherapy, reports a median survival of 37.9 months with 50%, 42%, 677
with a 9.0-cm lesion treated by radical nephrectomy; this patient went on to develop diffuse lytic bone metastases refractory to both radiation and combinational chemotherapy over the course of the ﬁrst 6 years after nephrectomy. Not all cases have resulted in a dismal postoperative course, however. Kaneko et al9 report the case of a 61-year-old woman with a 3.0-cm primary renal mesenchymal chondrosarcoma treated by radical nephrectomy both recurrence and metastasis free 6 years after intervention, potentially indicating a more favorable prognosis for patients diagnosed with low volume and localized disease. Certainly, further studies of this rare pathology are warranted to determine an optimal clinical course of treatment for these patients.
Figure 3. Microscopy of the primary renal mesenchymal chondrosarcoma.
and 28% overall survival at 3, 5, and 10 years, respectively. Mesenchymal chondrosarcoma is regarded as a high-grade sarcoma both in the grading systems of the French Federation of Cancer Centers Sarcoma Group and the National Cancer Institute.11 In this series, Dantonello et al identiﬁed 15 pediatric patients with mesenchymal chondrosarcoma with a median age of 16.6 years. All tumors were resected, and most patients were treated with chemotherapy. Interestingly, they did not observe the characteristic late metastatic recurrence. The authors could not effectively attribute this to their chemotherapy regimen but hypothesized that the young age of patients in their series may have been an important factor related to a more favorable prognosis. They note that this is similar to other sarcomas in which age is a well-established prognostic factor.11 Because this is only the sixth documented report of primary renal mesenchymal chondrosarcoma, the prognosis for this pathology is largely unknown. Malhotra et al6 reported the ﬁrst case of this pathology in 1984 of a 27-year-old man
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