Prognostic Factors for Survival in Patients with Clear Cell Sarcoma: An Analysis of the Surveillance, Epidemiology, and End Results (SEER) Database.

BACKGROUND Clear cell sarcoma (CCS) of soft tissue, or malignant melanoma of soft parts, is a rare disease. We aimed to identify prognostic factors linked to patient survival in CCS by analyzing demographic and clinical features using the Surveillance, Epidemiology, and End Results (SEER) database. This study aimed to identify prognostic factors associated with CCS that would be of clinical value. MATERIAL AND METHODS We collected data from patients diagnosed with CCS between 1973 and 2009 from the SEER database. The Kaplan-Meier method and Cox regression analysis were performed to identify prognostic factors for patient survival. RESULTS A total of 175 patients with CCS were identified from the SEER database. The 5-year survival rate was 62.9%, and the 10-year survival rate was 51.3%. Patients with CCS with local stage, and with tumor size ≤3 cm were more likely to have good survival rates. CONCLUSIONS The findings from this study showed that the identifiable prognostic factors in patients with CCS were stage and tumor size. Local stage and tumor size ≤3 cm were favorable prognostic factors for patient survival in CCS.

Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

Sarcoma de células claras del pie. Presentación de un caso / Clear cell sarcoma of the foot. Case presentation

RESUMEN El sarcoma de células claras fue descrito por primera vez por Franz M. Enzinger en 1965. Está íntimamente asociado a tendones y aponeurosis, excepcionalmente compromete la epidermis. Afecta fundamentalmente a pacientes jóvenes y se caracteriza por múltiples recurrencias locales y metástasis tardías. Se presenta un paciente de 22 años de edad, masculino que fue sometido a tratamiento quirúrgico radical (amputación transmetatarseana del 1er y 2do rayo). Los estudios anatomopatológicos confirmaron el diagnóstico de un sarcoma de células claras. El paciente se encuentra libre de la enfermedad después de 6 años de operado e incorporado a su vida social (AU).

ABSTRACT The clear cell sarcoma was firstly described by Franz M. Enzinger in 1965. It is intimately associated to tendons and aponeurosis, exceptionally compromising the epidermis. It mainly affects young patients and is characterized by multiple local recurrences and late metastases. We present a male patient, aged 22 years, who underwent a radical surgical treatment (transmetatarsal amputation of the 1st and 2nd rays). The anatomic-pathological studies confirmed the diagnosis of clear cell sarcoma. 6 years after surgery, the patients is free of the disease and reincorporated to his social life (AU).

The epidemiology and survivorship of clear cell sarcoma: a National Cancer Database (NCDB) review.

PURPOSE: Clear cell sarcoma (CCS) is a rare soft tissue tumor thought to originate from tendons and aponeuroses. This is the largest and most comprehensive study of CCS to the best of our knowledge. In addition, this is the first study to determine the estimated 10-year overall survival, specific treatment modalities including neoadjuvant and adjuvant combinations, and sites of distant metastasis in CCS utilizing a national database. METHODS: The National Cancer Database (NCDB) was used to study 489 patients diagnosed with CCS from 2004 to 2014. Kaplan-Meier methods were used to estimate 5- and 10-year overall survival, and log-rank tests were used to compare survival amongst stage. RESULTS: Median age at diagnosis was 39 years. Males and females were equally affected. Race distribution was 78% Caucasian and 15% Black. Most common primary site was lower limb or hip (53%). Percentage of patients with distant organ metastases was 15%, with lung being the most common site (4%). Median overall survival was 57.2 months. Overall estimated 5- and 10-year survival was approximately 50 and 38%, respectively. Approximate 5-year survival for Stages I-IV was 75, 65, 35, and 15%, respectively. Stages at disease presentation were 34% at Stage I, 13% at Stage II, 16% at Stage III, and 38% at Stage IV. Surgery was the most common form of treatment (83%); 34% received radiation and 20% received chemotherapy. CONCLUSION: The 5-year overall survival for CCS in Stages III and IV was much worse than Stages I and II. Overall estimated 5- and 10-year survival was approximately 50 and 38%, respectively. Men and women were equally affected and had a median age at diagnosis of 39 years. The most common tumor location was lower limb and hip and the most common site of metastases was the lung.

[Clear cell sarcoma of kidney in children]. / Sarcome à cellules claires du rein chez l'enfant.

Clear cell sarcoma of the kidney (CCSK) is a rare tumor that is diagnosed most often in children between 2- and 4-years-old of age. Usually, patients with CCSK are treated in international study for intrarenal tumors, preferentially Wilms tumor, according to bad histopronostic group. The purpose of this paper is to review the most important features in 2015 about epidemiology, radiology, anatomopathology and genetic of CCSK, and above all a synthesis about successive treatment strategies with their results. Second most common pediatric renal tumor in children less than 5-years-old, its prognosis has improved dramatically in recent years with the use of anthracyclines.

Renal tumors presentation: changing trends over two decades.

PURPOSE: We have analyzed the changing trends in surgical treatment of renal tumors over the last 2 decades with regard to age incidence, presentation, incidental detection, and histopathology. MATERIALS AND METHODS: Records of renal tumors were analyzed from January 1, 1988 to December 31, 2007. Data were split into 4 parts based on a 5-year time period, 1 for each cohort of patients: cohort 1 (1988-1992)-103 patients, cohort 2 (1993-1997)-161 patients, cohort 3 (1998-2002)-243 patients, and cohort 4 (2003-2007)-304 patients. A comparative study was performed with regard to age incidence, presentation, incidentallomas, histopathology, and management with statistical analysis. RESULTS: Out of 811 renal tumors, 17.63% cases were benign and 82.37% were malignant. In the first cohort, 34.95% cases were detected in the seventh decade as compared with cohort 4 in which these were detected in the sixth (34.86%) and fifth decades (21.38%). Incidentallomas increased from 11 (10.67%) in cohort 1 to 84 (27.63%) in cohort 4 (P = 0.001). The cases of surgically treated tumors increased in number from 103 to 304 in cohort 4. Among the presenting features, incidence of weight loss, flank pain, and lump decreased while other clinical syndromes were constant. Only open radical nephrectomy was performed in the first 2 consecutive timeperiods. Laparoscopic radical nephrectomy was increasingly used in cohort 4 as compared with cohort 3 (121 vs 32, respectively). Similarly, open nephron sparing surgery (NSS) was increasingly used in cohort 4 as compared with cohort 3. Among the histopathologies, clear cell carcinoma was most common (73.35 %), but Fuhrman grading showed a trend toward more cases detected with grade 1 and 2 in cohort 4; 23.73% and 61.86%, respectively, as compared with 15.85% and 45.12% in cohort 1 (P = 0.001); more T1 tumors were detected (63.42% in cohort 4 as compared with 41.46% in cohort 1). CONCLUSIONS: A majority of renal tumors presented as symptomatic tumors. Recently, tumors are being detected at an early stage and grade; in the younger patients, with an increasing trend of laparoscopic and open NSS.

Clear cell sarcoma of the kidney in children: experience in a developing country.

INTRODUCTION: Clear cell sarcoma of the kidney (CCSK) is a rare tumour comprising 4% of primary renal tumours in children. It has a unique constellation of chromosomal and molecular features and should no longer be viewed as an unfavourable histological variant of Wilms tumour. Little is known of its clinical presentation and pathological profile in children living in a developing country. AIM: To describe the clinical and pathological features of CCSK in children in our practice and to identify factors contributing to poor patient outcomes. METHOD: A retrospective review of patients with a confirmed diagnosis of CCSK who presented for treatment at a single institution between 1990 and 2008. RESULTS: 14 patients fulfilled the inception criteria. They represented 4% of 356 patients presenting with primary renal tumours during the review period. Clinical and radiological features were indistinguishable from Wilms tumour. Tumours were large (Mean mass 1.4 kg; median 0.9 kg) and metastases were common (42%). Lung and lymph node metastases were more common than skeletal disease. Co-morbidity, particularly hypertension (64%) was common. Initial diagnosis by needle biopsy was correct in only two of seven patients (29%) leading to inappropriate neoadjuvant chemotherapy. Overall survival is poor with 57% of patients alive and disease free from 1 to 7 years off treatment. CONCLUSION: In a developing country, CCSK is rare and clinically and radiologically indistinguishable from Wilms tumour. Associated hypertension is common. Pretreatment diagnosis is difficult and sampling errors using needle biopsies may be unavoidable. Treatment results are poor and, given the propensity for late recurrence in CCSK, may not be sustained.

Clear cell sarcoma (melanoma of soft parts): The Royal Marsden Hospital experience.

INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour with a propensity for local recurrence and nodal metastasis. About 300 cases have been reported, thus further clarification regarding the course and outcome of the disease is required. METHODS: Patients with a histopathologic diagnosis of CCS were identified from prospective histopathology and sarcoma databases and supplemented with a retrospective analysis of the patients' hospital records. RESULTS: Between 1990 and 2005, a total of 72 patients with a diagnosis of CCS were identified, 35 having been referred for management and 37 having been referred for histopathologic opinion. The median age was 39 years (range 5-90 years). Of the 35 patients referred to the Royal Marsden Hospital for management, 23% developed local recurrence or in-transit metastases at a median of 9 months (2-79 months) after resection of the primary, and nodal or distant metastatic disease was seen in 63% after 14 months (range 0-177 months). Five- and 10-year survival were 52% and 25%, respectively. CONCLUSIONS: CCS has a number of similarities with melanoma, particularly in its peripheral distribution and propensity for nodal disease. Wide excision with clear margins offers the best chance of cure. Local recurrence and regional metastases are common, and are almost always followed by distant metastases and death.

Clear cell sarcoma: an institutional review.

Clear cell sarcoma of the tendons and aponeuroses (CCSTA) is an aggressive, rare soft-tissue tumor representing approximately 1 per cent of all soft tissue sarcomas. Although it appears to be histogenetically related to melanoma, its clinical behavior resembles soft tissue sarcoma with a propensity for lymph node metastases. We report our experience at a tertiary referral center. Eight cases of CCSTA evaluated at the University of Illinois at Chicago's Medical Center between 1980 and 2002 were retrospectively reviewed. Patient data analyzed included age, gender, anatomic location, size of tumor, development of local, regional, and distant recurrence, and patient status at last follow-up. Local recurrence did not develop in any patient. Metastases developed in three patients with median time to presentation of metastases of 23 months (range, 8-36 months) from the time of initial diagnosis. Two of these three patients died of their disease with an average interval until death of 22 months from the time of initial diagnosis. The disease-free 2- and 5-year survivals were 68 per cent and 50 per cent, respectively. The overall 2- and 5-year survivals were 86 per cent and 68 per cent, respectively. CCSTA is an aggressive tumor of the soft tissues. These tumors present a high risk for development of distant disease and therefore warrant aggressive surgical management and treatment. Neither aggressive multiagent chemotherapy nor radiotherapy appeared to have an impact on outcome.

[Clear-cell chondrosarcoma]. / Das Klarzellchondrosarkom.

Clear-cell chondrosarcoma is a rare, low-malignant bone tumor. The lesion most commonly occurs in adults, generally in the 3rd or 4th decade. Clear-cell chondrosarcoma has a predilection for the epiphyses of long tubular bones, particularly the femoral head. Radiologically, it is a sharply defined radiolucent lesion. Histologically, it is characterized by large tumor cells with distinct boundaries and a clear cytoplasm. In addition to areas of conventional chondrosarcoma, there are partially mineralized trabecular osteoid formations. On the basis of 16 cases of clear-cell chondrosarcoma, we present the observations of the Hamburg Bone Tumor Register. The cases were examined according to age distribution, location, and radiological and pathomorphological criteria. The proximal femur was the most frequent location; rare manifestations in the rib, os ilium, and distal phalanx of the hand were also present. In 50% of the cases high-malignant osteosarcoma or conventional chondrosarcoma was considered. In addition to the epiphysis of the proximal femur, clear-cell chondrosarcoma can also involve other, rather unusual sites in the skeleton. Knowledge of the histomorphology of this tumor is therefore of crucial importance for the diagnosis.

Clear cell sarcoma: an extremely rare cause of pleural disease.

We present the case of a 36 yr old woman with a persisting complaints of left chest pain. A chest radiograph revealed multiple left pleural thickenings. Classical exploration was negative. Thoracic surgery allowed the subtotal removal of a huge pleural tumour. The histological examination revealed a clear cell sarcoma. The literature on this extremely rare tumour is reviewed.