RESUMEN
ABSTRACT: 18 F-FDG PET/CT was performed in a 1-year-old girl who had a heterogeneous mass in the right abdominal cavity revealed by abdominal ultrasound. A heterogeneous mass with internal necrosis, cystic changes, and hemorrhage in the right kidney, accompanied by a slight increase of FDG uptake, was observed in FDG PET/CT. Malignant renal tumor was considered, and Wilms tumor was preferentially suspected. However, the mass was demonstrated as clear cell sarcoma of the kidney by histopathological examination.
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Neoplasias Renales , Sarcoma de Células Claras , Femenino , Humanos , Lactante , Tomografía Computarizada por Tomografía de Emisión de Positrones , Fluorodesoxiglucosa F18 , Sarcoma de Células Claras/diagnóstico por imagen , Riñón/patología , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patologíaRESUMEN
BACKGROUND: To investigate the role of CT radiomics in distinguishing Wilms tumor (WT) from clear cell sarcoma of the kidney (CCSK) in pediatric patients. METHODS: We retrospectively enrolled 83 cases of WT and 33 cases of CCSK. These cases were randomly stratified into a training set (n = 81) and a test set (n = 35). Several imaging features from the nephrographic phase were analyzed, including the maximum tumor diameter, the ratio of the maximum CT value of the tumor solid portion to the mean CT value of the contralateral renal vein (CTmax/CT renal vein), and the presence of dilated peritumoral cysts. Radiomics features from corticomedullary phase were extracted, selected, and subsequently integrated into a logistic regression model. We evaluated the model's performance using the area under the curve (AUC), 95% confidence interval (CI), and accuracy. RESULTS: In the training set, there were statistically significant differences in the maximum tumor diameter (P = 0.021) and the presence of dilated peritumoral cysts (P = 0.005) between WT and CCSK, whereas in the test set, no statistically significant differences were observed (P > 0.05). The radiomics model, constructed using four radiomics features, demonstrated strong performance in the training set with an AUC of 0.889 (95% CI: 0.811-0.967) and an accuracy of 0.864. Upon evaluation using fivefold cross-validation in the training set, the AUC remained high at 0.863 (95% CI: 0.774-0.952), with an accuracy of 0.852. In the test set, the radiomics model achieved an AUC of 0.792 (95% CI: 0.616-0.968) and an accuracy of 0.857. CONCLUSION: CT radiomics proves to be diagnostically valuable for distinguishing between WT and CCSK in pediatric cases.
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Quistes , Neoplasias Renales , Sarcoma de Células Claras , Tumor de Wilms , Humanos , Niño , Radiómica , Estudios Retrospectivos , Sarcoma de Células Claras/diagnóstico por imagen , Neoplasias Renales/diagnóstico por imagen , Riñón , Tomografía Computarizada por Rayos XRESUMEN
Clear cell sarcoma of the kidney (CCSK) is a rare primary renal tumor in children. It is known for its propensity to metastasize to bones and lungs at initial diagnosis. Distant metastatic relapses occur in about 15-16% of the patients, with the brain being the most frequent site of relapse. Imaging features of brain metastases from CCSK have only been reported in a few cases and most reports lack a detailed description of the imaging findings. We present brain magnetic resonance imaging (MRI) findings in an infant with relapsed CCSK who developed multiple parenchymal metastases with concentric signal alterations and no tumor-associated edema.
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Neoplasias Encefálicas , Neoplasias Renales , Sarcoma de Células Claras , Niño , Lactante , Humanos , Sarcoma de Células Claras/diagnóstico por imagen , Sarcoma de Células Claras/patología , Riñón/patología , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patología , Neoplasias Encefálicas/patología , Imagen por Resonancia MagnéticaRESUMEN
ABSTRACT: Clear cell sarcoma, a highly aggressive cell sarcoma with melanotic differentiation, typically occurs in the tendon and aponeuroses of the lower extremities and rarely develops in the intra-articular region. Herein, we present an extremely rare case of clear cell sarcoma originating from the intra-articular knee and suspected as benign tumors due to the benign-looking findings on MRI and PET/CT. The image results in our case were completely consistent with a tenosynovial giant cell tumor, resulting in the misdiagnosis. For differential diagnosis, especially malignant melanoma, histopathology, including IHC and FISH, was indispensable.
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Tumor de Células Gigantes de las Vainas Tendinosas , Sarcoma de Células Claras , Humanos , Sarcoma de Células Claras/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones , Tumor de Células Gigantes de las Vainas Tendinosas/patología , Articulación de la Rodilla/diagnóstico por imagen , Articulación de la Rodilla/patología , Extremidad Inferior/patologíaRESUMEN
BACKGROUND: Clear cell sarcoma of the kidney is an uncommon pediatric renal malignant neoplasm that is typically characterized in 2-3-year-olds by aggressive behavior and late relapses. Our literature review revealed fewer than ten previously reported cases of CCSK with inferior vena cava thrombus, with only five in the pediatric age group. CASE PRESENTATION: We report the case of a 14-year-old Syrian girl who complained of mild pain in the left lumbar region pain with hematuria. On physical examination, a mass was palpated in the left flank. Abdominal ultrasonography revealed a left renal mass (7 × 5 × 2 cm3), associated with dilatation of the left renal vein. Contrast abdominal computed tomography showed a mass measuring 7 × 5 × 3 cm3 with the presence of thrombus extending into the inferior cavity down to the right atrium that was initially diagnosed as Wilms' tumor. Radical right nephrectomy with excision of the thrombus was undertaken. Histological immunostaining revealed a diagnosis of the tumor as clear cell sarcoma with vascular tumor thrombus extending to the inferior vena cava. CONCLUSION: Clear cell sarcoma and Wilms' tumor are similar in terms of typical age of appearance, clinical features, and histopathology, but with different methods of treatment and prognosis. The differential diagnosis of such masses is thus very important. We present the case of a patient with clear cell sarcoma with unusual age, with complete removal of the thromboses in the inferior vena cava and the right atrium.
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Carcinoma de Células Renales , Neoplasias Renales , Sarcoma de Células Claras , Trombosis , Trombosis de la Vena , Tumor de Wilms , Adolescente , Carcinoma de Células Renales/complicaciones , Carcinoma de Células Renales/diagnóstico por imagen , Carcinoma de Células Renales/cirugía , Niño , Preescolar , Femenino , Humanos , Riñón/patología , Neoplasias Renales/complicaciones , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/cirugía , Nefrectomía/métodos , Dolor/cirugía , Sarcoma de Células Claras/diagnóstico , Sarcoma de Células Claras/diagnóstico por imagen , Trombosis/complicaciones , Vena Cava Inferior/diagnóstico por imagen , Vena Cava Inferior/cirugía , Trombosis de la Vena/complicaciones , Trombosis de la Vena/diagnóstico por imagen , Tumor de Wilms/patologíaRESUMEN
BACKGROUND: Compared to Wilms, non-Wilms renal tumors in children are less well understood due to their rare occurrence which limits precise definition of the typical imaging patterns. OBJECTIVE: This study aims to identify distinctive imaging findings, demographic characteristics and prognosis of pediatric non-Wilms renal tumors. MATERIALS AND METHODS: From January 2007 to December 2018, 207 patients with a diagnosis of primary kidney neoplasia were yielded from our radiology archive, 171 of whom were diagnosed with Wilms tumor, 4 with angiomyolipoma and one with nephrogenic rest. The remaining 31 patients with a diagnosis of primary kidney neoplasia were enrolled in this retrospective study. Imaging data, age, gender, prognosis and findings regarding follow-up were noted. RESULTS: Eight patients had renal cell carcinoma, seven had clear cell sarcoma, six had mesoblastic nephroma, four had rhabdoid tumor, three had desmoplastic small round cell tumor, two had cystic nephroma and one had metanephric stromal tumor. The age of diagnosis was > 8 years for renal cell carcinoma and desmoplastic small round cell tumor, < 5 years for rhabdoid tumor and < 7 months for mesoblastic nephroma. There was no gender preference for any tumor type. The prognosis for rhabdoid tumor was extremely poor in that all the patients followed up in our institute were deceased, whereas no recurrence was found in other tumors. Translocation type renal cell carcinoma had lower T2-weighted signal intensity, mesoblastic nephroma was a predominantly cystic mass, clear cell sarcoma was generally larger at presentation and extensive amorphous calcifications were seen in desmoplastic small round cell tumor. CONCLUSION: For the differential diagnosis of pediatric non-Wilms renal tumors, age is the most important factor, followed by propensity to metastasize/aggressive behavior of the mass. Knowledge of specific imaging findings of these tumors may help to narrow the differential diagnosis.
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Carcinoma de Células Renales , Tumor Desmoplásico de Células Pequeñas Redondas , Neoplasias Renales , Nefroma Mesoblástico , Tumor Rabdoide , Sarcoma de Células Claras , Tumor de Wilms , Niño , Humanos , Carcinoma de Células Renales/patología , Sarcoma de Células Claras/diagnóstico por imagen , Estudios Retrospectivos , Tumor de Wilms/diagnóstico por imagen , Tumor de Wilms/patología , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/patologíaRESUMEN
ABSTRACT: A 26-year-old man presented with right facial pain for more than 20 days. Admission diagnosis was considered as jaw osteomyelitis. CT of the chest before surgery showed multiple bone destructions, which were considered as bone metastases. Then the 18F-FDG PET/CT was recommended for finding the primary tumor and evaluation of disease extent. 18F-FDG PET/CT revealed multiple abnormal foci at the skeleton and the left arm. Pathological examination and molecular analysis revealed the diagnosis of clear cell sarcoma. We present a case of clear cell sarcoma with multiple bone metastases, which is an extremely rare tumor.
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Neoplasias Óseas , Sarcoma de Células Claras , Adulto , Neoplasias Óseas/secundario , Fluorodesoxiglucosa F18 , Humanos , Masculino , Tomografía Computarizada por Tomografía de Emisión de Positrones , Tomografía de Emisión de Positrones , Radiofármacos , Sarcoma de Células Claras/diagnóstico por imagenRESUMEN
OBJECTIVE: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK. MATERIALS AND METHODS: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3-10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed. RESULTS: The age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770-104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337-25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively. CONCLUSION: Perinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.
Asunto(s)
Neoplasias Renales , Sarcoma de Células Claras , Tumor de Wilms , Diferenciación Celular , Niño , Preescolar , Femenino , Humanos , Lactante , Riñón/diagnóstico por imagen , Neoplasias Renales/diagnóstico por imagen , Masculino , Sarcoma de Células Claras/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Tumor de Wilms/diagnóstico por imagenRESUMEN
BACKGROUND: It is very rare for clear cell sarcomas (CCS) to arise in the bone. During diagnosis, it is important to distinguish primary CCS of bone from bone metastasis of melanoma because this difference fundamentally changes the therapeutic options. Recently, characteristic fusion genes of CCS have been detected using reverse transcription polymerase chain reaction (RT-PCR) or direct sequencing which allowed to distinguish CCS from melanoma. However, there was no study applying these analyses with positive results. In this case, we describe the use of fusion gene analysis to diagnose a primary CCS of the bone. CASE PRESENTATION: A 36-year-old male presented with a four-months history of left knee pain. Magnetic resonance imaging showed a lesion in the left femoral medial epicondyle. Histological examination of the biopsy specimen revealed proliferating oval or rounded cells. These cells had clear cytoplasm arranged in fascicles or compact nests with frequent deposits of brown pigment. Furthermore, immunohistochemistry analysis revealed that tumor cells were positive for S-100 protein, HMB-45, Melan-A, and SOX10. It stained negative for CD34 and BRAF v600e. Conclusively, detection of the EWSR1/ATF1 fusion gene using RT-PCR and direct sequencing confirmed that the lesion was a primary CCS of the bone. Wide-margin resection and reconstruction with a tumor endoprosthesis were performed. CONCLUSIONS: Herein, we diagnosed a rare case of primary CCS of the bone by detecting EWSR1/ATF1 fusion gene using RT-PCR and direct sequencing. Since fluorescence-in situ hybridization (FISH) and RT-PCR could show false positive by mainly due to technical problems, it is better to perform direct sequencing to confidently diagnose the tumor as a primary CCS especially at very rare site such as bone.
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Melanoma , Sarcoma de Células Claras , Adulto , Fémur/metabolismo , Humanos , Masculino , Proteínas de Fusión Oncogénica/genética , Proteínas de Fusión Oncogénica/metabolismo , Proteína EWS de Unión a ARN/genética , Reacción en Cadena de la Polimerasa de Transcriptasa Inversa , Sarcoma de Células Claras/diagnóstico por imagen , Sarcoma de Células Claras/genéticaAsunto(s)
Neoplasias Duodenales/diagnóstico por imagen , Endoscopía del Sistema Digestivo , Sarcoma de Células Claras/diagnóstico por imagen , Neoplasias Gástricas/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Anciano , Biomarcadores de Tumor/análisis , Diagnóstico Diferencial , Resultado Fatal , Humanos , MasculinoRESUMEN
Clear cell sarcoma of tendons and aponeuroses (CCS) is a rare, malignant tumor arising in lower extremities with no effective treatment other than wide surgical resection. Here described is a case of primary CCS in the peroneal tendon of the right foot of a 54-year-old woman enrolled to undergo BNCT. The tumor mass post-BNCT disappeared totally without damage to other normal tissue, demonstrating, for the first time, the potential efficacy of BNCT in complete local control of CCS.
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Terapia por Captura de Neutrón de Boro/métodos , Enfermedades del Pie/radioterapia , Sarcoma de Células Claras/radioterapia , Tendones , Biopsia con Aguja , Femenino , Enfermedades del Pie/diagnóstico por imagen , Enfermedades del Pie/patología , Humanos , Neoplasias Pulmonares/secundario , Imagen por Resonancia Magnética , Persona de Mediana Edad , Tomografía Computarizada por Tomografía de Emisión de Positrones , Planificación de la Radioterapia Asistida por Computador , Sarcoma de Células Claras/diagnóstico por imagen , Sarcoma de Células Claras/secundario , Tendones/diagnóstico por imagen , Tendones/patología , Resultado del TratamientoAsunto(s)
Neoplasias Abdominales/secundario , Sarcoma de Células Claras/secundario , Neoplasias Abdominales/diagnóstico por imagen , Neoplasias Abdominales/patología , Resultado Fatal , Humanos , Masculino , Persona de Mediana Edad , Neoplasias de la Boca/patología , Radiografía Abdominal , Sarcoma de Células Claras/diagnóstico por imagen , Sarcoma de Células Claras/patología , Tomografía Computarizada por Rayos XRESUMEN
Clear cell sarcoma of the Achilles tendon is an extremely rare condition, with a handful of cases reported in the literature over the past 3 decades. Patients usually present late because of the slow progress of the disease and seemingly benign nature of its presentation. Clinical and radiological findings are not enough to make a diagnosis; hence, a high index of suspicion is required. A firm diagnosis is made only after histopathologic studies. We present a rare case of this tumor in a young female, the management plan, and prognostic outlook, with subsequent review of the literature.
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Tendón Calcáneo , Sarcoma de Células Claras , Tendón Calcáneo/diagnóstico por imagen , Femenino , Humanos , Sarcoma de Células Claras/diagnóstico por imagenRESUMEN
Clear cell sarcoma (CCS) is a rare tumour that manifests in tendons and aponeuroses of the distal extremities and comprises roughly 1% of all soft tissue cancers. Only two cases of a primary CCS arising in the lung have been reported in the literature, and neither were located in the lung apex. We report a case of a primary CCS located in the lung apex that originally presented as ptosis, as well as arm pain and weakness. Distant metastasis was discovered on diagnosis, which led to irreversible paraplegia and death of the patient.
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Neoplasias Pulmonares/patología , Sarcoma de Células Claras/patología , Neoplasias de la Columna Vertebral/secundario , Adulto , Blefaroptosis , Resultado Fatal , Humanos , Neoplasias Pulmonares/diagnóstico por imagen , Neoplasias Pulmonares/radioterapia , Masculino , Cuidados Paliativos , Sarcoma de Células Claras/diagnóstico por imagen , Sarcoma de Células Claras/radioterapia , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/radioterapiaRESUMEN
Clear cell sarcoma of the kidney is an uncommon renal neopla sm of childhood. It represents about 4% of childhood malignant neoplasms and is generally more common in children under 5 years of age. In the present article, we describe the case of a 12-year-old male patient who came to our observation with left renal mass and with a clinical-laboratory picture indicative of inflammatory pathology.
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Neoplasias Renales/diagnóstico por imagen , Enfermedades Raras/diagnóstico por imagen , Sarcoma de Células Claras/diagnóstico por imagen , Biopsia , Niño , Humanos , Riñón/diagnóstico por imagen , Riñón/patología , Neoplasias Renales/patología , Imagen por Resonancia Magnética , Masculino , Pielonefritis/diagnóstico , Enfermedades Raras/patología , Sarcoma de Células Claras/patología , Tomografía Computarizada por Rayos X , UltrasonografíaRESUMEN
We assessed the feasibility of texture analysis to differentiate Wilms tumor, clear cell sarcoma and rhabdoid tumor of the kidney in children using gray-scale ultrasonography images. Children who had pre-operative renal ultrasonography images of the three tumors from January 2002 to February 2017 were retrospectively included as the test set, and children with the same criteria from March 2017 to December 2018 were included as the validation set. From histogram and second-order statistics, features were compared between the tumors, and diagnostic performances were assessed. Among a total of 32 children (24 children with Wilms tumors, five children with clear cell sarcomas and three children with rhabdoid tumors) from the test set, features from the second-order statistics showed an area under the curve greater than 0.89 for differentiating Wilms tumor from the others. These features aided in the differentiation of tumor type in the two children with Wilms tumors in the validation set. Therefore, texture analysis from gray-scale ultrasonography images can be used to differentiate Wilms tumors from clear cell sarcomas and rhabdoid tumors in children.
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Neoplasias Renales/diagnóstico por imagen , Tumor Rabdoide/diagnóstico por imagen , Sarcoma de Células Claras/diagnóstico por imagen , Ultrasonografía/métodos , Tumor de Wilms/diagnóstico por imagen , Preescolar , Diagnóstico Diferencial , Estudios de Factibilidad , Femenino , Humanos , Lactante , Riñón/diagnóstico por imagen , Masculino , Reproducibilidad de los Resultados , Estudios RetrospectivosRESUMEN
RATIONALE: Clear cell sarcoma of tendon and aponeurosis (CCSTA) or soft parts is a rare malignant melanin producing tumor entity that is derived from the neural crest cells originating from soft tissues displaying melanocytic differentiation. Diagnosis of CCSTA is difficult as it is dependent on age, size, location, necrosis, calcifications, cystic degeneration, and local to distant metastatic deposits. These tumors have very poor prognosis with a survival rate of 5-10 years because of local recurrence, early to late metastasis to lymph nodes, lungs, bones, and liver. PATIENT CONCERNS: A 30-year-old Asian male has presented with a painful mass in the posterior aspect of the right ankle. He recalled of noticing an increase in the size of the lump after a traumatic insult 3 months ago. Physical examination revealed a mass of size 9x4 cm in the posterior ankle with no cutaneous ulcerative lesions. There is no history of any longstanding illness or malignancy. DIAGNOSES: Clear cell Sarcoma of Tendon and Aponeurosis (CCSTA) or CCS of Soft parts. INTERVENTIONS: Conventional radiography demonstrated merely a soft tissue mass in the posterior compartment of the right ankle and significant calcaneal bone erosion with the sparse trabecular pattern. Plain conventional tomography showed a well-defined soft tissue heterogeneous mass with a hypoattenuating osteo-destructive focal lesion in the calcaneus. Magnetic resonance imaging (MRI) - T1 weighted imaging (T1WI) revealed an iso-intense signal relative to adjacent muscle; heterogeneous high-signal intensity on fat saturated T2 weighted imaging (T2WI). On contrast examination, lesion on T1WI, showed a heterogeneous high signal intensity, central low signal intensity with peripheral and septal enhancement. The immune-histochemistry analysis was positive for HMB-45, S-100, myoD1 and Ki67 (30%). Correlating with imaging and immune-histochemistry, a confirmatory diagnosis of CCSTA was made. OUTCOMES: CCSTA is typically a slowly growing painless mass in the deep soft tissues of ear, pancreas, kidney, penis, abdomen, especially in the lower extremities- Achilles tendon and aponeurosis of the ankle or in foot of young adults. As, these tumors are highly malignant, difficult to diagnose, early recognition by imaging and surgical excision are the mainstay of management. LESSONS: Our case emphasizes the importance of recognizing radiological characteristics of CCSTA, and its differentiation from other soft tissue tumors, when presenting atypically. MRI plays a significant role in the diagnosis supported by histopathology and immune-histochemistry. So, radiologists should be familiar about this presentation that could guide other personnel for early detection of soft tissue tumors while including CCSTA into differential diagnosis for evaluation.
Asunto(s)
Tobillo , Aponeurosis , Imagen por Resonancia Magnética , Sarcoma de Células Claras/diagnóstico por imagen , Neoplasias de los Tejidos Blandos/diagnóstico por imagen , Tendones , Adulto , Humanos , Masculino , Sarcoma de Células Claras/patología , Neoplasias de los Tejidos Blandos/patologíaRESUMEN
F-FDG PET/CT and MRI were performed in a 44-year-old woman to characterize a mass of the anterior tongue. MR images showed a voluminous mass, well circumscribed and enhanced heterogeneously after gadolinium chelates injection. There was an intense uptake on PET/CT. Pathological examination and molecular analysis revealed the diagnosis of clear cell sarcoma of the tongue. We present a case of clear cell sarcoma of the tongue, which includes imaging features. It is an extremely rare tumor, with only 3 cases previously reported in the literature.
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Imagen por Resonancia Magnética , Tomografía Computarizada por Tomografía de Emisión de Positrones , Sarcoma de Células Claras/diagnóstico por imagen , Neoplasias de la Lengua/diagnóstico por imagen , Adulto , Femenino , Fluorodesoxiglucosa F18 , HumanosAsunto(s)
Sarcoma de Células Claras/diagnóstico por imagen , Neoplasias Cutáneas/diagnóstico por imagen , Niño , Enfermedad Crónica , Femenino , Úlcera del Pie/etiología , Humanos , Imagen por Resonancia Magnética , Sarcoma de Células Claras/complicaciones , Sarcoma de Células Claras/patología , Neoplasias Cutáneas/complicaciones , Neoplasias Cutáneas/patologíaRESUMEN
Clear-cell sarcoma is a rare and malignant soft tissue tumor most commonly located at the distal end of extremities and rarely at the trunk. Data on the clinical features of the tumor is limited, complicating the analysis of prognosis and establishment of treatment protocols. In our study, we present a case diagnosed with clear-cell sarcoma in his right scapula and treated with tumor resection and chemotherapy, combination of dacarbazine, pharmorubicin and cisplatin. The patient died due to multiple organ failure eight months after operation. Early diagnosis, attentive care, and extended surgical resection combined with adjuvant therapy is of essence in treatment of clear-cell sarcoma.