Long term survival in a case of concurrent retroperitoneal liposarcoma and renal cell carcinoma: a case report
© Hoshi et al.; licensee BioMed Central Ltd. 2014
Received: 5 February 2014
Accepted: 31 July 2014
Published: 16 August 2014
Liposarcoma is one of the most common soft tissue sarcomas found in adults. It has a predilection for retroperitoneal space. Renal cell carcinoma is the most common tumor of the kidney.
Concurrent retroperitoneal liposarcoma and renal cell carcinoma were found in a 34-year-old Japanese man. The renal tumor was first detected by ultrasonography, it was confirmed by computed tomography, which also identified a presumptive retroperitoneal liposarcoma, and the tumors were further assessed with magnetic resonance imaging. The patient was treated by surgical resection of retroperitoneal liposarcoma and left nephrectomy and has been disease-free for 10 years.
The concomitant occurrence of a renal tumor and a primary primary liposarcoma is rare. The major factors promoting a good prognosis in this case were the favorable histology and the small size of the tumors.
KeywordsRetroperitoneal liposarcoma Renal cell carcinoma
Liposarcoma is one of the most common soft tissue sarcomas found in adults, and it usually occurs in the retroperitoneum and the extremities . Its incidence rate increases in middle-aged and older adults with a peak in 70–80 year olds. Renal cell carcinoma (RCC) is the most common tumor of the kidney. Its incidence is highest in those aged 50–70 years. The co-occurence of RCC and hematological malignancies as well as solid tumors has been reported.
Young patient age can indicate a hereditary RCC syndrome. However, our patient had no family history and clinical signs of tuberous sclerosis, von Hippel-Lindau disease, and succinate dehydrogenase-associated familial cancer.
Summary of the five reported cases of concurrent liposarcoma associated with renal cell carcinoma (RCC)
RCC histological subtype
1.1 × 1.1 × 0.4 cm
13.8 × 15.2 cm
Cardiac (found by autopsy)
5.4 × 5.2 cm
7.6 × 5.0 cm
NED 24 months
17 months no recurrence
0.55 cm (found incidentally)
Retroperitoneum (invasion of the proximal ureter)
40 cm, 20 cm and 14 cm
Recurrent liposarcoma, no data on follow up
In addition to the previously described cases, we found two published case reports regarding liposarcomatous differentiation in chromophobe RCCs [7, 8]. In contrast, the renal tumor in our case was a typical clear cell RCC, and the two tumors represented distinct entities.
Over the last 15 years, 1123 patients with retroperitoneal soft tissue sarcoma have been reported in 25 series; these tumors had a mean diameter of 15.7 cm . Retroperitoneal soft tissue sarcomas represent 0.10 to 0.15% of all malignancies and 45% of all retroperitoneal tumors. Because of the localization, symptoms are nonspecific (e.g., abdominal discomfort and palpable mass) and caused by tumor growth, which is typically very large when detected. The only curative treatment modality is complete surgical resection; chemotherapy and radiation therapy show no survival benefit. It has been reported that 51.4% of these tumors can be completely excised and that 50.2% of these excisions include adjacent organs . The prognosis without complete excision is poor with reported 5- and 10-year survival rates of 16.7% and 8.0% respectively .
Local recurrence represents the major type of progression for retroperitoneal liposarcomas. Yamamoto et al. described 45 patients with well-differentiated liposarcoma who underwent surgical treatment. Among 41 patients who underwent initial surgery, only one recurrence occurred, which was localized in the retroperitoneal space. For 4 patients who underwent a reoperation, the mean time between the initial surgery and the recurrence was 16.5 years. None of the 45 patients developed distant metastasis. In our case, during the 10 years of follow-up to date, no recurrence or metastasis has been detected. However, continued follow-up is necessary because late recurrences are common with liposarcoma.
Previously reported liposarcomas have demonstrated heterogeneous signal intensity on MRI with great variation depending on the components and histological patterns of a particular tumor. Retroperitoneal liposarcomas have been classified into several clinico-pathological subtypes . Myxoid liposarcoma, consisting of a myxoid matrix and a small amount of mature fat, shows low signal intensity on T1 weighted image and high signal intensity on T2 weighted image . Well-differentiated liposarcoma presented high signal intensity on T1 weighted images, intermediate signal intensity on T2 weighted images, drop-out signal intensity on fat-suppressed MR images . Round-cell liposarcoma and pleomorphic liposarcoma exhibit the signal intensity of a soft-tissue tumor without a characteristic fat signal . Liposarcomas can present with intratumoral hemorrhage and may invade adjacent organs. In the present case, the tumor showed high signal intensity on the T2-weighted images, which is typical for myxoid liposarcoma and is inconsistent with well-differentiated liposarcoma which was diagnosed pathologically. Five-year and ten-year disease specific survival is the highest for well-differentiated liposarcoma (100% and 87%) followed by myxoid liposarcoma (88% and 76%), and is the lowest for pleomorphic liposarcoma (56% and 39%) (http://sarcomahelp.org/liposarcoma.html).
We have presented a case of concomitant RCC and retroperitoneal liposarcoma in a young male. Although no data exists regarding an association between these two malignant tumors, a genetic predisposition for cancer is likely present given patient’s young age. Both tumors were completely surgically excised and no relapse has been seen during ten-year follow-up to date. The major factors promoting a good prognosis in this case were favorable histology and small size of the tumors at initial diagnosis.
Written informed consent was obtained from 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.
The authors would like to express their gratitude to Edanz (Edanz Group Japan) for editing the manuscript by a native-English speaker with scientific expertise.
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