Clear Cell Sarcoma Of Tendons Research Articles

Cutaneous balloon-cell melanoma metastases to the axillary lymph node: Exploring cytomorphologic features and differential diagnoses on fine needle aspiration biopsy.

Balloon cell melanoma (BCM) is an exceptionally uncommon histological variant, making up <1% of all malignant melanomas. Diagnosing balloon cell melanoma on cytological specimens can be challenging due to its scarcity and its similarity to other medical conditions. A comprehensive clinical assessment and histological analysis, coupled with immunohistochemical staining, play a crucial role in distinguishing balloon cell melanoma from various benign and malignant skin conditions. The differential diagnoses encompass spitz nevus, balloon cell nevus, clear cell sarcoma of tendons and aponeuroses (melanoma of soft tissues), metastatic clear cell renal cell carcinoma, sebaceous carcinoma, and benign adnexal tumours like clear cell hidradenoma. Malignant melanoma encompasses a spectrum of histopathological subtypes, each with unique clinical and cytological characteristics. Notably, balloon-cell melanoma (BCM) emerges as an exceptionally rare and diagnostically challenging variant. Marked by the presence of distinct balloon-like, clear cytoplasmic vacuoles within melanoma cells, BCM stands apart from other melanoma subtypes. Despite its rarity, the distinctive cytological features of BCM make it a compelling subject of investigation, emphasizing the crucial role of Fine Needle Aspiration (FNA) cytology in ensuring accurate diagnosis and guiding subsequent management decisions.

Clear Cell Sarcoma of Tendon and Aponeuroses with Excessive Melanin Production – An Unusual Presentation

Clear cell sarcoma of tendons and aponeuroses also called as malignant melanoma of soft parts is a rare tumor of neural crest origin with 70% of them possessing balanced translocation t(12; 22). Approximately 50% of the tumor produces melanin which often shows focal pigmentation or needs special stains to demonstrate melanin. Production of excessive melanin by this tumor is very unusual and very few case reports are available in literature. We report a case of Clear cell sarcoma with excessive melanin production in a 50-year-old female patient involving the left popliteal fossa.

Boron neutron capture therapy for clear cell sarcoma

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.

Is TLE1 Expression Limited to Synovial Sarcoma? Our Experience at Shifa International Hospital, Pakistan.

Introduction: Synovial sarcoma (SS) accounts for 10-15 percent of adult soft tissue sarcomas. Transducin-like enhancer of split 1 (TLE1), a transcriptional repressor, is essential in hematopoiesis, neuronal differentiation, and terminal epithelial differentiation. TLE1 proteins inhibit Wnt signaling and other cell fate determination signals, and so have an established role in repressing differentiation. TLE1 has recently been shown to be a highly sensitive and relatively specific marker of SS.Materials and methods: Study design is retrospective, descriptive. A total of 25 cases of SS and 28 of soft tissue lesions were retrieved from the record. TLE1 (clone 1F5) expression was evaluated and scored as negative (<5% of cells positive), 1+ (5-25% of cells positive), 2+ (26-50% of cells positive), or 3+ (>50 % of cells positive).Result: Twenty-four out of twenty-five (96%) cases of SS showed 3+ TLE1 expression. One (4%) case of poorly differentiated SS showed 2+ positivity. 3+ TLE1 positivity was seen in one (100%) case each of infantile fibrosarcoma and low-grade fibromyxoid sarcoma, while two cases (100%) of schwannoma also showed 3+ positivity. All cases of solitary fibrous tumor) (n=2), clear cell sarcoma of tendons and aponeurosis (n=2), embryonal rhabdomyosarcoma (n=1), and de-differentiated liposarcoma (n=2) showed 2+ positivity. 1+ positivity was seen in alveolar soft part sarcoma (n=2), Ewing's sarcoma (n=4), undifferentiated pleomorphic sarcoma (n=1), myxoid liposarcoma (n=1) and malignant peripheral nerve sheath tumor (n=1). TLE1 was negative in all cases of chordomas (n=2), lipomas (n=2), nodular fasciitis (n=2), malignant perivascular epithelioid cell tumor (n=1) and dermatofibrosarcoma protuberans (n=1).Conclusion: TLE1 may be a reliable immunostain for diagnosing SS, but its expression is not limited to SS. Its expression should be interpreted in the light of morphological features and a panel of antibodies.

Malignant Extragastrointestinal Neuroectodermal Tumor Located at Right Cervical Region

Malignant Gastrointestinal Neuroectodermal Tumor (GNET) is a recently described rare malignant neoplasm, resembling “clear cell sarcoma of tendons and aponeuroses” morphologically, immunohistochemically and molecular pathologically. We will describe a case of soft tissue sarcoma located at right cervical region of a 9-year-old male patient, mimicking malignant GNET, which can be considered as extragastrointestinal counterpart of this tumor. These tumors have poor prognosis. This is unique, possibly the first case of a soft tissue sarcoma resembling GNET.

Prognostic factors associated with clear cell sarcoma in 14 Chinese patients.

PURPOSE. To identify prognostic factors associated with clear cell sarcomas in 14 Chinese patients. METHODS. Medical records of 7 men and 7 women (mean age, 36 years) with histologically confirmed clear cell sarcoma of tendons and aponeuroses were reviewed. Patient demographics, tumour characteristics, and treatment modalities were retrieved. Prognostic factors associated with favourable 5-year survival were determined. RESULTS. The most affected sites were the thigh (n=5) and the foot (n=4); the mean time from symptom onset to diagnosis was 9.5 months. The tumour stage at diagnosis was IIA in 8 patients, IIB in 2, and III in 4. The mean tumour size was 4.5 cm in diameter. One patient was lost to follow-up. For the remaining 13 patients, the mean time to disease-related mortality was 2.5 years. Nine patients had distant metastases; the most common sites were lungs and pleura (n=7), followed by distant lymph nodes (n=4), bone (n=2), pericardium (n=2), and brain (n=1). All patients underwent surgical excision. Three women and one man (mean age, 27 years) attained 5-year disease-free survival. All had stage IIA tumours at diagnosis. Their mean tumour size was 1.75 cm in diameter, which was significantly smaller than that of all patients (4.5 cm). Tumour size of ≤ 2.5 cm in diameter (p=0.004) and stage IIA tumour at diagnosis (p=0.04) were significant prognostic factors for 5-year survival. CONCLUSION. Tumour size of ≤ 2.5 cm and early stage tumour are associated with 5-year disease-free survival. Early detection is crucial for the prognosis of clear cell sarcomas.

English

Clear cell sarcoma of tendons & aponeuroses, is a rare malignancy derived from neural crest cells. It commonly presents in young adults in extremities. It is usually a high grade tumor with poor survival rates. Grossly, the tumor is circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemistry reveals neopalstic cells which are positive for HMB-45 & react with antibody against S-100 protein. Mainstay treatment remains wide excision of tumor. The role of chemotherapy & radiotherapy are reported to be limited. We report this case in view of its rarity.

Clear cell sarcoma of tendons and aponeuroses

Clear cell sarcoma of tendons and aponeuroses

Boron neutron capture therapy (BNCT) selectively destroys human clear cell sarcoma in mouse model

Clear cell sarcoma of tendons and aponeuroses (CCS) is a rare malignant tumor with no effective treatment. This study demonstrates the efficacy of BNCT with the use of human CCS-bearing nude mice. Groups A and C were administered saline, and groups B and D were injected with p-borono-l-phenylalanine-fructose complex. Groups C and D were then irradiated with thermal neutrons. The tumors in only group D disappeared, demonstrating that BNCT is a potentially new option for the treatment of human CCS.

Tendon ve Aponörozların Berrak Hücreli Sarkomu: 4 Olgu Sunumu

Tendon ve aponörozların berrak hücreli sarkomu yumuşak dokunun nadir görülen bir tümörüdür. Bu tümörler sıklıkla adolesan ve genç erişkin erkeklerde yavaş büyüyen sert kitleler şeklinde gözlenir. En sık alt ekstremitenin kasları ile ilişkili tendon ve fasyalarda izlenir. Mikroskobik olarak kas liflerini ve bağ dokusunu yararak ilerleyen üniform neoplastik hücrelerin oluşturduğu küçük ada ve fasiküllerin düzensiz diziliminden oluşur. Malign melanomla benzer immünohistokimyasal bir profil gösterir. Histomorfolojik ve immünohistokimyasal olarak malign melanomla benzer özellikler gösterse de, moleküler ve klinik çalışmalarla bu iki lezyonun farklı olduğu kanıtlanmıştır. Prognostik olarak berrak hücreli sarkomlar malign melanoma göre daha yavaş seyirli olmaları nedeniyle, ayırıcı tanının doğru yapılması önemlidir. Bu çalışmada, nadir görülen bu tümörlerin klinik ve patolojik özellikleri, sunduğumuz bu dört olgu üzerinden tartışılmıştır.

Primary sarcoma of the anterior cruciate ligament — A case report

Clear cell sarcoma of tendons and aponeuroses (CCSTA) is a rare, aggressive soft tissue malignancy, which is found in intimate association with tendon, aponeurosis or fascia. It has not previously been reported in association with intraarticular ligaments. We report the first case of an intraarticular CCSTA, in this case of the anterior cruciate ligament and describe the diagnostic and treatment challenges of intraarticular tumours of the knee.

Primary cutaneous pecoma: a case report

Introduction Perivascular epithelioid cell tumour (PEComa) of skin is a rare tumour. In contrast to more common presentations of PEComa, primary cutaneous PEComa appears not to be associated with tuberous sclerosis and its related tumours. Here, we present a case of primary cutaneous PEComa and discuss the differential diagnosis. Case report A 61-year-old female presented with a solitary non-tender nodule on her right knee slowly growing over 3 months. Excisional biopsy showed a pale orange-coloured firm nodule 12 × 7 mm. Microscopic examination revealed a dermal infiltrate composed of plump epithelioid cells with vacuolated to pale granular eosinophilic cytoplasm, round to oval nuclei and prominent nucleoli. Scattered amongst tumour cells, there were small aggregates of chronic inflammatory cells and occasional multinucleate giant cells as well as a delicate network of capillary-sized vessels. Mitoses, necrosis and pleomorphism were not present. Special stains showed no alcohol or acid-fast bacilli. PAS stain revealed glycogen within the cytoplasm of the epithelioid cells. Immuno-histochemistry showed strong positivity for HMB45, CD10 and CD68, and negative staining for S100, Melan A and CD34 in the tumour cells. Discussion To date, there are approximately 23 cases of primary cutaneous PEComa in English literature have been reported. 1,2,3 None of the cases had tuberous sclerosis. 2 Primary cutaneous PEComas have shown benign biological behavior, although reported cases of deep subcutaneous or non-cutaneous involvement may exhibit malignant behaviour. 2 There are a number of benign and malignant lesions in the differential diagnosis of a cutaneous PEComa; for instance, dermatofibroma with clear cell change, xanthomatous lesions, granular cell tumours, balloon cell naevus and melanoma, clear cell sarcoma of tendons and aponeuroses, and metastatic renal cell carcinoma. PEComas show a unique immunohistochemical profile with positivity for melanocytic (the most sensitive: HMB45, 100%) and smooth muscle markers (the most sensitive: desmin, 50%). 1,2 Of note, primary cutaneous PEComa is negative for S100 in contrast to PEComas in other sites which show up to 33% positivity for this marker. 1 CD68 has been shown to react with tumour cells in some of the published cases of skin PEComa; 2 whereas, CD10 status of this tumour, to our knowledge, has not been clarified in the English literature yet.

51 Clear Cell Sarcoma of Tendons and Aponeuroses: A Review With Discussion of Molecular Techniques in Diagnosis Surgical Pathology No. SP 08-5 (SP-357)

51 Clear Cell Sarcoma of Tendons and Aponeuroses: A Review With Discussion of Molecular Techniques in Diagnosis Surgical Pathology No. SP 08-5 (SP-357)

PET Monitoring of Clear Cell Sarcoma of Tendons and Aponeuroses

Abstract: Clear cell sarcoma of tendons and aponeuroses is a rare soft tissue sarcoma easily mistaken for metastatic malignant melanoma because of their histochemical and ultrastructural similarities. Like melanoma, the former neoplasm exhibits an aggressive behavior with a high propensity for local recurrences and lymphatic and hematogeneous dissemination. The authors present 2 cases of such an uncommon but highly malignant lesion with great impact of posttherapeutic evaluation by F-18 FDG PET/CT.

Clear cell sarcoma of tendons and aponeuroses: a review.

Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

Clear Cell Sarcoma of Tendons and Aponeuroses: A Review

Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

Clear cell sarcoma of tendons and aponeuroses

Clear cell sarcoma (CCS) of tendons and aponeuroses (malignant melanoma of soft parts) is a rare melanocytic soft tissue sarcoma. The objective of this study was to determine the clinical features, prognostic factors, and optimal treatment policy for patients with this rare disease. Seventy-five consecutive patients with histologically confirmed CCS who received treatment between 1980 and 2004 were analyzed retrospectively. There were 41 men and 34 women, and the median age was 36 years. Sixty-five tumors were located in the extremities, and 10 tumors were located in the trunk. The median tumor size was 4 cm. Seventy-one patients underwent surgical excision, and 56 patients received chemotherapy. Sixteen patients developed local recurrences, and 52 patients developed metastasis. The overall patient survival rates was 47% at 5 years and 36% at 10 years. Univariate analysis showed that sex (P = .018), tumor size (P = .001), tumor depth (P = .002), TNM classification (P = .001), and surgical margin (P = .042) were significant prognostic factors. Among the 52 patients who presented with localized disease, sex (P = .023), tumor size (P = .002), tumor depth (P = .011), TNM classification (P = .004), and chemotherapy (P = .032) were identified as significant prognostic factors. Multivariate analysis showed that tumor size remained an independent prognostic factor in both groups. The current results supported the contention that early diagnosis and initial wide excision are essential for a favorable outcome of CCS. The role of chemotherapy for CCS should be investigated further.

Clear Cell Sarcoma of Tendons and Aponeuroses: A Historical Perspective and Tribute to the Man Behind the Entity

Clear cell sarcoma of tendons and aponeuroses is a unique sarcoma initially described by Dr Franz M. Enzinger. The tumor has a proclivity to involve the tendons and aponeuroses of distal extremities of relatively young individuals and is characterized by multiple local recurrences with late metastases and a high rate of tumor deaths. Since its seminal description in 1965, there have been many studies verifying the uniqueness of this entity and probing its differentiation. Ultrastructural and immunohistochemical studies have shown melanocytic differentiation, whereas molecular genetic studies have shown cytogenetic rearrangements resulting in a EWSR1-ATF1 fusion gene that is characteristic but not entirely unique for clear cell sarcoma (similar fusion genes are also seen in angiomatoid fibrous histiocytoma). Detection of this fusion gene and the absence of BRAF gene mutations clearly distinguish clear cell sarcoma from cutaneous melanoma. Adverse prognostic factors identified to date include larger tumor size and any microscopic tumor necrosis. Surgery is the mainstay of treatment for this high grade sarcoma, with chemotherapy having little effect. Although the melanocytic differentiation of clear cell sarcoma is indisputable, its precise lineage remains unclear. Thus, clear cell sarcoma maintains the status of a unique yet enigmatic clinicopathologic entity of ever increasing complexity 40 years after its original description by an extraordinarily gifted man.

Clear cell sarcoma of tendons and aponeuroses: An analysis of 75 cases

9572 Background: Clear cell sarcoma (CCS) of tendons and aponeuroses is a rare malignant tumor that occurs most commonly in the extremities of young adults. Because of the rarity of the disease (1% of all soft tissue sarcomas), most reports about CCS have dealt with a few cases during a long time period. We conducted a multi-institutional study of CCS to clarify the clinical findings and prognostic factors of CCS treated during the era of modern multidisciplinary treatment. Methods: The records of 75 consecutive patients with histologically confirmed CCS treated at the JMOG affiliated institutions between 1980 and 2004 (follow-up; average 44 months) were reviewed. There were 41 men and 34 women with an average age of 36 years (range, 10–71 years). 65 tumors were in the extremities (foot 22, hand 13, thigh 9, others 21) and 10 were in the trunk. The tumor size ranged from 1 to 11 cm (mean, 4 cm). 52 patients presented with localized disease (M0) and 23 with metastatic disease (M1). Results: Surgical excision of the primary tumor was performed in 71 patients (limb-sparing surgery; 56, amputation; 15). Microscopic surgical margin was negative in 60 and positive in 11. 56 patients received chemotherapy (30 with measurable disease) and 17 had radiotherapy. Local recurrence occurred in 16 patients and 48 patients developed metastasis. The 5-year overall survival rate was 47% (M0; 55%, M1; 20%). On univariate analysis, sex (p=0.02), size (p=0.001), depth (p=0.002), TNM stage (p=0.001), IRS group (p=0.001) and surgical margin (p=0.04) were prognostic factors. On multivariate analysis, size (p=0.02) remained to be a significant prognostic factor. Objective response to chemotherapy was observed in 8 (27%) patients. In the group of M0 patients, those who received adjuvant chemotherapy had better prognosis (5-year survival, 65%) than those without chemotherapy (5-year survival, 23%)(p=0.03). Conclusions: The results support the contention that early diagnosis and initial excision with negative surgical margin are essential for favorable outcome of CCS. The role of chemotherapy for CCS should be further investigated. No significant financial relationships to disclose.

Sentinel node biopsy for clear cell sarcoma

Clear cell sarcoma (CCS), also known as clear cell sarcoma of tendons and aponeuroses or malignant melanoma of soft tissue, is a rare malignant tumor and is histogenitically related to melanoma. The aim of this study was to describe our experience with the sentinel node (SN) procedure for CCS patients and to discuss the potential value of this technique for CCS patients. Five patients with a subcutaneous CCS, who underwent an SN procedure, are described. Two patients had positive SNs, with additional tumor positive nodes in both lymph node dissection specimens. Only the patients with tumor positive SNs developed recurrent disease during an average follow-up of 33 months. None of the negative SN patients developed recurrent disease and all were alive after an average follow-up of 39 months. SN status seems to predict additional nodal involvement and recurrent disease as well as survival. The SN procedure might be a useful and accurate staging procedure in CCS patients, comparable to the situation in melanoma.