Objective:To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD).Methods:The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed.Results:There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years. The tumor occurred in the left thigh, right maxillofacial region, and right popliteal space. The presenting symptom was a painless mass which was accidentally discovered. The maximum diameter of tumors ranged from 3 to 5 cm. Microscopically, the tumors were poorly defined and composed of relatively monomorphic spindle cells, arranged in diffuse, fascicular growth patterns, with moderate pale eosinophilic cytoplasm. Mitoses were abundant. A few round rhabdomyoblastic tumor cells with abundant eosinophilic cytoplasm were found. There was no evidence of hemorrhage or necrosis. The tumor cells expressed vimentin, SMA, MSA, desmin, MyoD1 and myogenin; and the Ki-67 proliferation index was 10%-60%. RT-PCR showed EGFR-KDD in all three cases. Gene fusion was detected in three cases based on next generation sequencing, but only one case had EGFR-KDD. Follow-up data for 12 to 36 months showed two patients died of the disease and one patient was alive without recurrences and metastasis.Conclusions:IRFS is a rare soft tissue tumor that resembles infantile fibrosarcoma but has immunohistochemical evidence of rhabdomyoblastic differentiation. It more commonly occurs in infants and tends to appear in limbs and torso with poor prognosis. Aggressive multimodality treatment is recommended for these patients. EGFR-KDD may be a genetic driver to IRFS. Clinical response to EGFR targeted therapy might be promising in the future.

Objective:To investigate the clinicopathological and genetic features of infantile rhabdomyofibrosarcoma (IRFS) with EGFR kinase domain duplication (EGFR-KDD).Methods:The clinical, morphological and immunohistochemical features of three IRFS with EGFR-KDD diagnosed from January 2022 to January 2024 at Department of Pathology, Foshan Traditional Chinese Medicine Hospital, Foshan, China were retrospectively analyzed using PCR or next generation sequencing technique; and related literature was reviewed.Results:There were 1 male and 2 females, aged at presentation ranging from 1 to 4 years. The tumor occurred in the left thigh, right maxillofacial region, and right popliteal space. The presenting symptom was a painless mass which was accidentally discovered. The maximum diameter of tumors ranged from 3 to 5 cm. Microscopically, the tumors were poorly defined and composed of relatively monomorphic spindle cells, arranged in diffuse, fascicular growth patterns, with moderate pale eosinophilic cytoplasm. Mitoses were abundant. A few round rhabdomyoblastic tumor cells with abundant eosinophilic cytoplasm were found. There was no evidence of hemorrhage or necrosis. The tumor cells expressed vimentin, SMA, MSA, desmin, MyoD1 and myogenin; and the Ki-67 proliferation index was 10%-60%. RT-PCR showed EGFR-KDD in all three cases. Gene fusion was detected in three cases based on next generation sequencing, but only one case had EGFR-KDD. Follow-up data for 12 to 36 months showed two patients died of the disease and one patient was alive without recurrences and metastasis.Conclusions:IRFS is a rare soft tissue tumor that resembles infantile fibrosarcoma but has immunohistochemical evidence of rhabdomyoblastic differentiation. It more commonly occurs in infants and tends to appear in limbs and torso with poor prognosis. Aggressive multimodality treatment is recommended for these patients. EGFR-KDD may be a genetic driver to IRFS. Clinical response to EGFR targeted therapy might be promising in the future.

Objective To identify the characteristics and research status of symptom clusters in patients with lung cancer patients during chemotherapy.Methods This scoping review was conducted under the Joanna Briggs Institute guidelines.A literature search was performed in the PubMed,Embase,CINAHL,Web of Science,Scopus,CNKI,Wanfang,and CBM databases to identify relevant studies about symptom clusters in patients with lung cancer from inception until December 31,2023.The included studies were summarized and analyzed.Results 19 papers were included and 22 symptom clusters were extracted,mainly including psychological,gastrointestinal,lung cancer-specific,fatigue-related,and respiratory symptom clusters.There are 6 symptom cluster assessment tools,of which 4 were M.D Anderson Symptom Inventory in different version;the symptom clusters were dynamically changing during chemotherapy in patients with lung cancer,with variations in core symptom clusters at different stages.Conclusion Multiple symptom clusters exist during chemotherapy in lung cancer patients,and the naming of symptom clusters and attribution of symptoms need to be further standardized.The assessment tools for symptom clusters in patients undergoing chemotherapy for lung cancer are mainly based on the Anderson Symptom Assessment Scale and other language versions.Symptom clusters in patients with lung cancer during chemotherapy show dynamic changes,and there are differences in core symptom groups at different stages.

Both risks of type 2 diabetes mellitus (T2DM) in patients with bipolar disorder (BD) and of BD in patients with T2DM are both significantly higher than the general population, indicating the two disorders may share the common pathogenesis. Patients with BD and co-morbid T2DM behave poorly on managing their own behavior on health, thus increased risks of outcomes on related complications, disability and mortality. The article reviewed the epidemiologic features, health hazards, relations and possible mechanisms between BD and T2DM. Strategies and measures on intervention of BD and T2DM were also involved in the text to improve the awareness and research ability of the researchers.

Purpose To investigate the clinicopathologic features of desmoplastic fibroma of bone (DF).Methods The clinical and pathologic profiles of 7 cases were retrospectively analyzed.Results Desmoplastic fibroma of bone was a rare neoplasm of bone,which typically occurred in young children and adolescents.The tumor showed infiltrative and locally aggressive nature,the most common sites were long tubular bones and mandible.Microscopically,the tumor was composed of sparse fibroblasts in a rich background of collagen fibers.Immunohistochemically,the tumor cells were positive for vimentin and SMA.Conclusion Desmoplastic fibroma is a rare,locally aggressive fibroblasticlesion of bone.It can make an accurate diagnosis by integrating the clinical location with histopathological features.Trauma,hormone and horomosome aberration may be related with its pathogenesis.

Purpose To investigate the clinicopathologic features of calcifying aponeurotic fibroma (CAF). Methods The clinical and pathologic profiles of 6 cases of CAF were retrospectively analyzed. Results CAF appeared a firm, painless and slowly growing mass with a predilection for the distal portions of the extremities in young children and adolescents. Microscopically, the tumor consis-ted mainly of benign appearing spindle cells, chondroid cells and calcific debris. Immunohistochemically, the tumor cells were positive for vimentin, HHF-35, S-100, Calponin and CD68. Conclusion CAF is a rare, locally aggressive fibroblastic lesion. It can make an accurate diagnosis by integrating the clinical location with histopathological features. Repeated minor trauma to the extremities and some embryologic cell nests maybe its pathogenesis.