Clear cell variant of oral squamous cell carcinoma: case report and review

Abstract The clear cell variant of Oral Squamous Cell Carcinoma (OSCC) is an uncommon histological variant. Kuo first discovered it in the skin, and Frazier et al. found it in the oral cavity. We know of only nine cases of clear cell variant of OSCC reported in the literature till now. The present case is in a 60-year-old male patient with an ulcer on the left posterolateral border of the tongue. The patient had a history of chewing tobacco for 22 years. Clinical examination showed features of malignant ulcer associated with pain and discomfort. Histopathological examination revealed sheets and islands of atypical epithelial cells with clear cytoplasm, nuclear and cellular pleomorphism, and few keratin pearls in the connective tissue suggesting OSCC. Various special stains were performed to identify clear cells. Periodic Acid Schiff-Diastase (PAS-D) and Mucicarmine stains showed positive and negative reactions in clear cells, respectively. Immunohistochemical (IHC) analysis for cytokeratin (AE1/AE3) showed diffuse positivity in clear cells and other epithelial cells. Based on special stains and IHC markers, we confirmed the diagnosis as a clear cell variant of OSCC. This variant is rare and presents diagnostic challenges. It is said to be aggressive in nature. More such cases should be reported to understand its biological behavior and prognosis.

Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma / 임상소아혈액종양

BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution.METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017.RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free.CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.

Clinical Features and Treatment Outcome of Soft Tissue Clear Cell Sarcoma / 임상소아혈액종양

BACKGROUND: Soft tissue clear cell sarcoma is a rare tumor which originates from neural crest cells. Due to its rarity and lack of established treatment, the prognosis of clear cell sarcoma is poor. Here, we reviewed the clinical data and outcome of patients diagnosed with soft tissue clear cell sarcoma in our institution. METHODS: A retrospective study was conducted on pediatric patients who were treated for pathologically confirmed soft tissue clear cell sarcoma at the Seoul National University Hospital, between January 2000 and July 2017. RESULTS: Six patients (3 boys and 3 girls) were diagnosed with soft tissue clear cell sarcoma at a median age of 14 years 4 months (range 11 years 7 months - 19 years 3 months). The median size of the tumor was 5.6 cm (range, 0.6 cm to 7.9 cm). The most frequent symptom was pain (67%), and the most common primary site was the lower limb (67%). Three patients (50%) presented with metastases at diagnosis. Four patients underwent chemotherapy with various therapeutic combinations. Four patients received surgical resection. Only one patient received local radiotherapy. One patient died of primary refractory disease, three patients relapsed, while the remaining two survive event-free. CONCLUSION: Soft tissue clear cell sarcoma is a rare and highly aggressive tumor, for which there is no established treatment. All surviving patients received surgery, indicating that surgery is a key treatment modality. Further genetic studies of soft tissue clear cell sarcoma are needed to find a better treatment strategy.

A Case of Clear-Cell Sarcoma in the Stomach / 대한내과학회지

Clear-cell sarcoma (CCS) is a rare soft tissue sarcoma that usually develops in the lower extremities of young adults. CCS of the gastrointestinal tract is extremely rare. We report here the first case of CCS of the stomach in Korea. A 28-year-old female developed a large tumor of the stomach that was initially considered Ewing sarcoma, based on the results of a needle biopsy. She had paraneoplastic syndrome; fever, anemia, and hyperglycemia. Follow-up abdominal computed tomography after four-cycles of chemotherapy with vincristine-doxorubicin-cyclophosphamide or ifosfamide-etoposide showed no tumor shrinkage and revealed a fistula between the tumor and the gastric lumen. The infected tumor prompted debulking surgery, which resulted in improvement of her symptoms. The pathologic findings were consistent with CCS, and fluorescence in situ hybridization for EWS gene rearrangement was positive, confirming the diagnosis. This case highlights the importance of clinical suspicion for CCS in chemotherapy-refractory Ewing sarcoma.