SRF-rearranged cellular perivascular myoid tumor: a clinicopathological analysis of two cases / 中华病理学杂志

Objective: To investigate the clinicopathological features, immunophenotype, diagnosis and differential diagnosis of SRF-rearranged cellular perivascular myoid tumor. Methods: Two cases of SRF-rearranged cellular perivascular myoid tumor diagnosed in the Department of Pathology, Fudan University Shanghai Cancer Center from October 2021 to March 2022 were collected. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and the literature was reviewed. Results: Case 1, a 3-month-old boy presented with a painless tumor of the scalp, measuring about 2 cm in diameter. Case 2, a 3-year-old girl complained with a painless tumor of the knee, measuring approximately 1.5 cm in diameter. Microscopically, the tumor had a clear boundary and showed multinodular growth. The tumor was mainly composed of spindle cells arranged in long intersecting fascicles associated with thin, slit-like or branching ectatic vessels, focally forming hemangiopericytoma-like appearance. The tumor cells were abundant, but there was no obvious atypia. Mitotic figures (3-4/10 HPF) were noted. H-caldesmon and SMA were positive in both cases. Case 1 showed diffuse and strong positivity for Desmin, and focally for CKpan. Ki-67 proliferation index was 20% and 30%, respectively. FISH displayed NCOA2 gene translocation in case 1 and the RELA gene translocation in case 2. NGS detected the SRF-NCOA2 gene fusion in case 1 and the SRF-RELA gene fusion in case 2. Both patients underwent local excisions. During the follow-up of 5-14 months, case 1 had no local recurrence, while case 2 developed local recurrence 1 year post operatively. Conclusions: SRF-rearranged cellular perivascular myoid tumor is a novel variant of perivascular cell tumor, which tends to occur in children and adolescents. The tumor forms a broad morphologic spectrum ranging from a pericytic pattern to a myoid pattern, and include hybrid tumors with a mixture of pericytic and myoid patterns. Due to its diffuse hypercellularity and increased mitotic figures and smooth muscle-like immunophenotype, the tumor is easy to be misdiagnosed as myogenic sarcomas. The tumor usually pursues a benign clinical course and rare cases may locally recur.

Epidemiological features of adult chronic kidney diseases in a community-based population in Songjiang district, Shanghai / 中华流行病学杂志

Objective: To investigate the prevalence and associated factors of chronic kidney diseases (CKD) in adult residents living in a community of Songjiang district, Shanghai. Methods: A total of 9 257 residents aged 20-75 years old in Xinqiao township of Songjiang district were selected by random cluster sampling. All the participants were interviewed to complete a set of personal questionnaire and undergo physical examinations. Urine and blood tests including markers of kidney damage and related associated factors with CKD, were carried out. Results: Eligible data from 8 207 subjects were enrolled in the study. After adjustment for age and gender, the prevalence of CKD was 8.4% (95%CI: 7.8%-9.0%), with majority of the patients (76.5%) appeared in the early stage (Ⅰ and Ⅱ) of the disease. The prevalence of CKD increased with age and higher prevalence was seen in females than in males (P<0.001). Results from logistic regression analysis showed that factors as: being elderly or female, having hypertension, hyperuricemia, and hyperlipidemia were all independently associated with CKD. Conclusions: The prevalence of CKD appeared relatively high in adult residents of Xinqiao township, Songjiang district where CKD had become a public health problem. Factors as: being female or elderly, hypertension, hyperuricemia, and hyperlipidemia were found to be associated with CKD. Our findings suggested that early prevention and control on CKD to reduce the incidence of end-stage renal diseases and related complications had called for more urgent attention.