A rare case of primary clear cell sarcoma of the leg in an elderly (original) (raw)
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Clear Cell Sarcoma of Soft Tissue: A Rare Malignancy Entity
Scholars Journal of Medical Case Reports
Clear cell sarcoma (CCS) of soft tissue or soft tissue melanoma is a rare malignant tumor in young adults, preferentially affecting the lower limbs. We report a new observation of a 17-year-old patient, without a significant history, consulted for a nodular lesion of the left ankle, painless, appeared 06 months before, evolving in a context of general impairment. The clinical examination found a purplish nodule, about 2/2 cm, hard, fixed, associated with multiple nodular lesions along the homolateral lower limb, with the presence of homolateral inguinal lymphadenopathy. There was no associated motor or vascular disorder. The nodule ultrasound showed a mass of soft tissue, hypoechogenic, heterogeneous. The computed tomography of the ankle revealed a lesional process of the medial face infiltrating the tendon and ligament structures and encompassing the posterior tibial artery. Histological examination showed round cell malignant tumor proliferation. The immuno-histochemical supplement showed an expression of the S100 protein and monoclonal antibody HMB-45 by tumor cells. The biopsy of an inguinal node affirmed its metastatic nature. The anatomo-clinical confrontation allowed to retain the diagnosis of clear cell sarcoma. The extension assessment made of a chest-abdominal-pelvic computed tomography showed metastatic lymph nodes intra and retroperitoneal. The patient was given chemotherapy (ifosfamide-adriamycin). Sarcoma with clear soft tissue cells or melanoma of the soft parts is a rare malignant tumor. It occurs most often in young adults at the lower limbs. It is a firm subcutaneous mass, slowly progressive, well limited, usually deep attached to tendon and aponeurotic structures. The positive diagnosis is based on histology coupled with immunohistochemistry. It can be helped by molecular biology in case of doubt diagnosis with melanoma. Its poor prognosis requires early and adequate management.
Clear Cell Sarcoma: A Case Report and Review of Literature
International Journal of Hematology-Oncology and Stem Cell Research, 2018
Clear cell sarcoma (CCS), a deep-rooted tumor with a predilection for lower extremities, has a proclivity to involve the tendons and aponeuroses. This sarcoma is seen predominantly around the foot and ankle region. Diagnosis is mainly finalized using histological and immunohistochemical assessment. The main treatment strategy is surgery followed by chemotherapy. An erratic challenge is posed by histological similarity and immunohistochemical overlap to the diagnosis and distinguishing of clear cell sarcoma from primary or metastatic malignant melanoma (MM) which is more common. Here, we described a CCS case located in the left leg of a 37-year-old male patient.
Clear Cell Sarcoma or Malignant Melanoma of Soft Parts
Cancer Genetics and Cytogenetics, 1998
Malignant melanoma of soft parts, also termed clear cell sarcoma (CCS), is a rare malignancy of neural crest origin which is different from cutaneous malignant melanoma. Although a translocation involving chromosomes 12 and 22 is characteristic of clear cell sarcoma and not malignant melanoma, there are a paucity of methods to differentiate the two. Therefore, a study of microsatellite instability (MIN) was undertaken to determine if mechanisms of DNA mismatch repair can differentiate these malignancies. MIN has been described in a variety of malignancies including 25% of malignant melanomas. Paraffin-embedded neoplastic and non-neoplastic cells were obtained from 11 individuals (five males; six females; age range from seven to 60 years) with CCS. Isolated DNA was PCR amplified at 17 separate microsatellite loci using radioactive-labeled primers. Tumor tissue was compared to normal tissue for each analysis. No MIN was detected. Loss of heterozygosity was detected in only one patient at a single locus (IFNA). The lack of MIN in clear cell sarcoma further defines the distinction between this tumor and malignant melanoma. Clinically, local recurrence and metastasis were indicators of poor outcome. The size of the tumor was not a significant prognostic indicator. Local recurrence, satellitosis, or nodal metastasis was not proven to be uniformly fatal. Utilization of chemotherapy and/or radiation demonstrated no obvious survival advantage. The histologic parameters of mitotic rate and the presence of necrosis were not prognostic. Limb-preserving surgical procedures were as effective as amputation for local disease control. The actuarial survival rate was calculated to be 48% at five years.
Clear cell sarcoma in an elderly female -A rare case report with literature review
IP Innovative Publication Pvt. Ltd, 2017
The Clear Cell Sarcoma of Tendons and Aponeurosis is an extremely rare melanin producing soft tissue tumor. It accounts for nearly 1% of all soft tissue tumors. Clear cell sarcoma affects commonly young adults between age group of 20-40 years and most common sites are foot and ankle. It has histological, immunohistochemical, and ultrastructural similarities with the more common primary (or metastatic) malignant melanoma causing major diagnostic confusion. We present a case of a 55-year lady with clear cell sarcoma of right foot which is extremely rare for this age group.
Oncology in Clinical Practice, 2019
Clear cell sarcoma (CCS), also referred as to melanoma of soft tissues, is a rare malignant tumour of soft tissues. This tumour harbors the characteristic features of soft tissue sarcoma (STS) and is a slowly growing, painless tumour, which then acquires an aggressive course. CCS is characterized by a translocation t(12; 22)(q13; q12), which in addition to the diagnostic implications may be important for targeted treatment in the future. CCS occurs mainly on the limbs, most often shin (in feet and ankle area) in the tendons and aponeurosis, often at a young age. CCS is characterized by high potential to develop metastases in regional lymph nodes (about 30% of cases). In the diagnostic process one should consider performing a sentinel node biopsy with possible subsequent radical lymphadenectomy in the case of metastases detection. Treatment of localized disease is limited to radical local excision with complementary radiotherapy. Due to the resistance to classical chemotherapy and the presence of characteristic molecular abnormalities, trials of molecular targeted therapies in this group of cancers are ongoing. In clinical trials, MET inhibitors, tyrosine kinase inhibitors (TKI)-sunitinib and pazopanib were evaluated. CCS was also one of the subtypes of tumours evaluated in the CREATE clinical trial with crizotinib.
Primary Clear Cell Sarcoma of the Dermis Mimicking Malignant Melanoma
Balkan Medical Journal, 2018
Background: Clear cell sarcoma is a rare malignant soft tissue neoplasm that typically involves tendons and aponeurosis. Clear cell sarcoma in the dermis is an extremely rare occurrence, and it is difficult to differentiate between this neoplasm and dermal malignant melanoma because they have similar morphologic and immunohistochemical features. Although rare, clear cell sarcoma of the skin typically occurs in the extremities. To our knowledge, there are no reported cases of primary clear cell sarcoma of the skin occurring in the neck. Here, we report an unusual case of clear cell sarcoma arising in the skin of the neck. Case Report: A 43-year-old female presented with a right neck lesion. Histologic sections of the lesion showed a nodular proliferation of spindle cells with pale cytoplasm with epithelioid features involving the entire dermis with no epidermal component. The tumour cells were positive for melanocytic markers, including S100 and Human Melanoma Black 45, which led to an initial diagnosis of malignant melanoma. Fluorescence in situ hybridization showed a rearrangement of the EWSR1 gene on chromosome 22q12, which led to a diagnosis of primary clear cell sarcoma in the skin. Conclusion: Because the treatments for clear cell sarcoma and conventional melanoma are different, fluorescence in situ hybridization for EWSR1 should be performed in any dermal lesions with melanocytic features that do not have an in situ component.
CLEAR CELL SARCOMA OF GLUTEAL REGION MALIGNANT MELANOMA OF SOFT PARTS
National Journal of Medical Research, 2013
Clear cell sarcoma (CCS) is described as variant of sarcoma characterized by prominent clear cells showing features similar to malignant melanoma of soft parts. This neoplasm was first described by Dr. Franz m. Enzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Clear cell sarcoma (CCS) is a rare malignant tumor with a propensity for slow progressive invasion. It is a tumor derived from Melanoblast like cell. They occur most commonly in the extremities, with a predilection for young females. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) and conventional malignant melanoma may demonstrate significant morphologic overlap at the light microscopic and ultra structural level. The tumor is very rare and can pose clinical challenges in early diagnosis. This case report demonstrates an unusual site of occurrence for clear cell sarcoma.