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.

Can pulmonary sclerosing haemangioma be accurately diagnosed by intra-operative frozen section?

AIMS: Pulmonary sclerosing haemangioma is a rare benign tumour with a characteristic variegated histological pattern. In this retrospective study we aimed to identify features that can aid in making a correct diagnosis and avoiding potential pitfalls at the time of intra-operative frozen section. METHODS AND RESULTS: Twenty cases of pulmonary sclerosing haemangioma with intra-operative frozen section were reviewed. The four major histological patterns (solid, sclerotic, papillary and haemorrhagic) were found in various combinations in the frozen sections. In 17 cases, three or more patterns were present. There could be focal areas mimicking epithelioid haemangioendothelioma or carcinoid tumour. Intra-operative imprint/scrape cytology served as a helpful adjunct in confirming the cytological blandness, although occasional atypical cells could be present. An intra-operative frozen section diagnosis of 'sclerosing haemangioma' or 'benign tumour' was given in 14 cases; the diagnosis was deferred in six cases. Retrospective analysis of the deferred cases showed that a definitive intra-operative diagnosis could have been made in three, because three or more major histological patterns were present. One case showed a pure papillary pattern at frozen section, mimicking the appearance of papillary adenocarcinoma (primary or secondary), bronchioloalveolar carcinoma, epithelioid mesothelioma or papillary adenoma; two tumours from a patient with multicentric disease showed widespread significant cytological atypia in the tumours raising a serious consideration of malignancy. CONCLUSION: A diagnosis of pulmonary sclerosing haemangioma can be made at intra-operative frozen sections in most cases based on the tumour circumscription and variegated histological patterns. When only a single histological pattern is identified or when there is significant cytological atypia, distinction from other tumours can be problematic, and the diagnosis is best deferred.

Nodular fasciitis: MRI appearance and literature review.

OBJECTIVE: To describe the MRI features of nodular fasciitis and to review the clinical, MRI and histologic aspects of the tumor. DESIGN AND PATIENTS: Three patients with biopsy-proven nodular fasciitis were selected for a retrospective study. A literature review was also carried out. RESULTS AND CONCLUSIONS: All the lesions appeared slightly hyperintense to skeletal muscle on T1-weighted images, and hyperintense on T2-weighted images with fat saturation [either frequency saturation or Short TI Inversion Recovery (STIR) sequences]. Two enhanced homogeneously after intravenous gadolinium, whereas the third showed heterogeneous enhancement with a nonenhancing area. Despite the difference in enhancing patterns, the histologic appearances of these lesions were similar. Our study shows that the MRI appearance of nodular fasciitis may not be related to the location of lesion. It is thought that the age of nodular fasciitis may reflect its gross morphology, and it is possible that the MRI and histologic appearances could correlate with the age of the lesion, but it would require a larger series to evaluate this concept.