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Clear cell sarcoma is an uncommon malignant neoplasm that, on rare occasion, invades osseous structures. The authors present a case of clear cell sarcoma that appears to have an osseous origin and that was discovered on a routine radiograph of the foot.
A clear cell sarcoma, arising primarily in the ileum of a 35-year-old man, is reported. Histologically, the neoplasm infiltrated the full thickness of the intestinal wall. It consisted of strands and sheets of round to spindle-shaped cells with clear to eosinophilic cytoplasm, vesicular nuclei and prominent nucleoli. Vascular invasion was present at diagnosis. Tumour cells expressed S-100 protein, melan-A and tyrosinase. They were negative for HMB45, CD117, cytokeratins, epithelial membrane antigen, smooth muscle actin, desmin, CD31, CD34, chromogranin and synaptophysin. Reverse transcription-polymerase chain reaction analysis performed on paraffin-embedded tissue showed EWS-ATF1 fusion transcripts representative of the t(12;22) (q13;q12) clear cell sarcoma reciprocal translocation. The patient, who developed liver metastases 2 months after diagnosis, died of disease at 15 months. This case demonstrates that the gastrointestinal tract is a potential site for primary clear cell sarcoma of soft tissues, and, furthermore, that cytogenetics and/or molecular techniques play a central role in the diagnosis.
Squamous cell carcinoma (SCC) of the tongue in persons younger than 30 years of age is an extremely rare neoplasm. We report an unusual case of SCC of the tongue as a second malignancy in a young adult with a history of a stage III clear cell sarcoma of the kidney (CCSK) treated 19 years earlier. Review of the literature reveals a spectrum of cytogenetic abnormalities in SCC; however, aberrations involving the long arm of chromosome 10 have been reported in both SCC and CCSK. Long-term follow-up, with particular attention to the head and neck, may be warranted in children with CCSK.
A patient with a clear cell sarcoma of tendons and aponeuroses arising in the left sacral area is reported. The tumor grossly contained two black foci that had pigment with the staining characteristics of melanin on light microscopy. Electron microscopy of these areas showed the pigment to be within melanosomes. This is the second report of a neoplasm diagnosed as clear cell sarcoma in which melanin was demonstrated; the possibility that the tumor represents a soft tissue variant of malignant melanoma is discussed. It is suggested that clear cell sarcoma is a heterogeneous entity, and that a number of different soft tissue neoplasms may present with a clear cell pattern, making diagnosis and classification difficult.
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.
We report the first case of metastatic clear cell sarcoma with dramatic response to DAV treatment (DTIC + ACNU + VCR). Clear cell sarcoma of tendons and aponeuroses, or malignant melanoma of soft parts, is a rare tumour that occurs predominantly in the extremities of young adults. It tends to recur locally or metastasize and the prognosis is poor. Although the importance of surgery has been established, the role of adjuvant chemotherapy has yet to be determined. DAV should be considered as a first-line palliative treatment in disseminated disease as well as adjuvant therapy after surgery of primary clear cell sarcoma.
BACKGROUND: Clear cell sarcoma of the kidney (CCSK) in adults is extremely rare. Optimal treatment of adult patients with CCSK remains unclear. CASE PRESENTATION: A 22-year-old man presented with a 2-month history of left flank pain. A color duplex sonography revealed a hypervascular, heterogeneous renal mass. Abdominal and pelvic computerized tomography showed a heterogeneous mass originating from the lower pole of the left kidney and infiltrating to the psoas muscle. Further evaluation including bone scan did not demonstrate any evidence of metastases. A left radical nephrectomy with hilar lymphadenectomy through an intraperitoneal approach with an anterior subcostal incision was performed. The histopathological diagnosis of the mass was a clear cell sarcoma of the kidney. No lymph node metastases were found. Concomitant chemo-radiotherapy was performed. Therapy-related serious side effects were not observed. There was no evidence of local recurrence or metastases during the following twenty-four months after therapy. CONCLUSION: We believe that the combination therapy is efficacious for preventing the local recurrence and distant metastases. Accurate diagnosis is very important and therapy must also include doxorubicin regardless of the disease stage in adult patients with CCSK.
A patient initially presented in 1988 with a solitary axillary mass, diagnosed as a high-grade neuroendocrine spindle-cell neoplasm; there was no history of a primary cutaneous malignancy. After subsequent development of a pulmonary nodule in 2001 (14-years post initial diagnosis), the case was reviewed and the possibility of metastatic melanoma was raised. The histopathologic and immunohistochemical profile of this melanocytic neoplasm was diagnostic of clear cell sarcoma (CCS) of tendons and aponeuroses, although the differential diagnosis included malignant melanoma, follicular dendritic and interdigitating cell tumors, malignant peripheral nerve sheath tumor, and a category of so-called PEComas. It is the role of pathologists, particularly dermatopathologists, to distinguish CCS from malignant melanoma, and to alert the clinician, because proper diagnosis ultimately influences treatment. We discuss the immunophenotype, differential diagnosis, and molecular signatures of these neoplasms, and review the pertinent literature on these entities.
We report a case of clear-cell sarcoma with a t(12;22)(q13;q13) and multiple copies of chromosome 8 in addition to other abnormalities. An identical or similar translocation has previously been reported in this type of tumor, suggesting that the t(12;22) is a primary cytogenetic change in the pathogenesis of a subset of clear-cell sarcomas. In addition, the presence of extra copies of chromosome 8, commonly noted in our case and others, suggests that it represents a nonrandom secondary change in these tumors.
BACKGROUND: Clear cell sarcoma (CCS) of tendons and aponeuroses is extremely rare in childhood and little information is available on its clinical management. Originally believed to be a type of melanoma of soft tissue origin, CCS is now considered a distinct clinicopathologic entity that behaves like a high-grade soft tissue sarcoma. We report on a series of 28 pediatric patients treated from 1980 to 2000 by the Soft Tissue Sarcoma Italian Cooperative Group and the German Cooperative Group. METHODS: Patients were treated with a multimodality therapeutic approach. Surgical resection was complete in 17 patients (mutilating in 3), radiotherapy was administered to 8 patients, and 20 patients received chemotherapy. RESULTS: After a median follow-up of 102 months (range, 19-238 months), the 5-year and event-free survival rates were 66.4% and 63.3%, respectively. Seventeen patients were alive in first remission, two were alive in second remission, and nine had died of disease. The response to chemotherapy in the 7 evaluable patients included one partial remission, one minor response, and five no responses. Radiotherapy contributed to achieving local control in four of six Intergroup Rhabdomyosarcoma Study (IRS) Group II patients. Statistically significant differences in outcome were evident according to IRS group, tumor size, and site. CONCLUSIONS: Our study confirms the aggressive behavior of CCS. Complete surgical resection represents the mainstay of treatment, and even the only treatment for patients with small tumors. Radiotherapy may control microscopic residual disease after surgery. Chemotherapy is ineffective and the prognosis is unfavorable for patients with unresectable and large tumors.
An example of clear cell sarcoma, from the great toe of a young woman having an otherwise classic history and appearance is described, and a malignant giant cell component is reported for the first time. Histochemical and electron microscopic studies clearly demonstrate melanogenesis. The controversy over tissue of origin of this uncommon neoplasm is discussed and evidence in support of neurectodermal derivation is presented.
Six cases are reported of an osteoclast-rich tumor of the gastrointestinal tract that should be segregated from GIST. Five of the cases were located in the small bowel and one in the stomach. The age of the patients ranged from 13 to 37 years. The tumors behaved aggressively, with metastases to regional lymph nodes, liver, and other intra-abdominal sites. Microscopically, the tumor cells were medium-sized, predominantly oval, relatively monomorphic, diffusely immunoreactive for S-100-protein, and negative for CD117, CD34, HMB-45, and Mart-1. They were admixed with scattered osteoclast-like, multinucleated giant cells which were S-100-protein negative and KP1-positive. One case studied cytogenetically had the karyotype 46XX t(12;22)(q13;q12). The cases here reported are interpreted as examples of a distinctive type of gastrointestinal neoplasm which shares some features with clear cell sarcoma of soft parts (melanoma of soft parts), including in one case the chromosomal translocation that is characteristically associated with that entity.
We report two patients with clear cell sarcomas arising from tendons in the ankle and knee. In the current cases, calcifications, fluid-fluid levels and the marked hypointense areas are not seen within the masses. In the differential diagnosis of the mass developing in the tendons and aponeuroses, when these signs are absent, the probability of clear cell sarcoma is higher.
Clear cell sarcoma of the tendons and aponeuroses (CCSTA) are rare aggressive soft tissue tumors with tendency for lymph nodes dissemination. Lymph node involvement is a correlate for prognosis. We present three patients with CCSTA in whom simultaneous sentinel lymph biopsy (SLNB) and resection was performed. Sentinel lymph node mapping may have a role in clear cell sarcoma from a prognostic standpoint. Further investigations are needed for validation.
Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. A case had a checkup for chest bachache with a 21-year-old woman, and it was diagnosed as the right chest wall tumor. We performed the en bloc resection of parts of the 7th and 8th ribs. Composix mesh was fixed to cover a deficit in the chest wall. The pathological diagnosis was clear cell sarcoma of the chest wall. The postoperative course was uneventful. She has shown no symptoms or signs of recurrence during 14 months of follow-up.
Clear cell sarcoma of the hand. Case report. Histologic, ultrastructural and immunohistochemical features of a case of clear cell sarcoma (also called malignant melanoma of soft parts) arisen in the index finger of the right hand in a 28 year-old-woman are described in this report. Typical forms of this tumor are deeply located and associated with tendons and aponeuroses, lacking cutaneous involvement. The tumor has to be differentiated from other benign and malignant lesions of the soft parts, such as a giant cell tumor of tendon sheaths and a fibrosarcoma. The demonstration of melanin and a positive immunohistochemical reaction for S-100 protein and HMB-45 (a melanin-associated antigen) can assist in the differential diagnosis. In spite of a slow and protracted clinical course, many of the patients experience multiple local recurrences and distant metastases. Prognosis is poor in a high percentage of cases.
Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumour that occurs predominantly in the extremities of young adults. Although it appears to be histogenetically related to melanoma, its clinical behaviour resembles soft tissue sarcoma. Prognosis is reported to be poor due to the great propensity of regional and distant metastases. The risk of metastases to regional lymph nodes is very high. We describe a case of clear cell sarcoma of the hand and evaluate the feasibility of the sentinel lymph node biopsy (SLNB) technique in this kind of tumour. Up to now, there are no reports that describe the use of SLNB in clear cell sarcoma. The SLNB technique was carried out with success and the sentinel lymphnode was easily identified. Histological examination of the lymphnode allowed identification of metastatic cells and thus provide for radical lymphadenectomy. We maintain that SLNB can be successfully used for this type of tumour although this result should be evaluated and confirmed by larger case studies.
Clear cell sarcoma of the kidney rarely affects adults. It has an aggressive evolution, with a high rate of recurrence and mortality. We report the case of a 58 year old man with clear cell sarcoma of the kidney and discuss the benefit and value of medical imaging for management.