Duodenal Soft Tissue Sarcoma with GLI1 Gene Rearrangement: A Case Report and Literature Review.

BACKGROUND Soft tissue tumors have various subtypes, among which sarcomas exhibit high malignant potential and poor prognosis. Malignant epithelioid tumor with GLI1 alterations was originally found in myopericytoma with t(7;12) translocation. However, recent studies indicated that it is a distinct tumor type characterized by multiple nodular distributions of oval or round epithelioid cells with a rich capillary network and a lack of specific immunophenotype. There are only a few cases reported worldwide and the optimal treatment is still being explored. CASE REPORT We report the case of a 31-year-old patient who presented with severe anemia and a large soft tissue mass in the duodenum. The patient underwent surgical resection with a negative margin, and none of the 15 lymph nodes tested positive for the tumor. Postoperative pathology and FISH testing further confirmed the presence of GLI1 disruption and S-100 and SMA negativity. Genetic testing revealed the ACTB-GLI1 fusion. No specific medication was offered after the surgery. No tumor recurrence was found during the 23-month follow-up period. The patient's quality of life is currently satisfactory. CONCLUSIONS Soft tissue sarcomas characterized by GLI1 gene rearrangement have a relatively less aggressive and metastatic nature, with the solid mass spreading minimally even as it grows. Patients can benefit from surgical resection, resulting in a relatively long period of tumor-free survival.

Comparison of the accuracy of two⁃dimensional and three⁃dimensional photography in the facial measuremenof patients with unilateral cleft lip t / 口腔疾病防治

Objective@#To compare the measurement accuracy of two⁃dimensional (2D) photography and three⁃di⁃mensional (3D) photography in the analysis of the asymmetric appearance of children with cleft lip and palate and the influence of the severity of asymmetric deformity on the accuracy of facial measurement to guide clinical measurement work@* Methods@#Children with unilateral cleft lip were enrolled in this prospective study. Seven parameters⁃⁃the devia⁃tion of the pronasale, subnasale, and labrale superius, as well as the cleft/noncleft ratio of the width of nostrils, length and height of lateral lips and height of columella⁃were measured with Vernier calipers as the gold standard. Traditional 2D photography and 3D stereophotogrammetry photos were taken and measured. The extent of cleft malformation is indi⁃cated by the ratio of the cleft side to the noncleft side. The error size is represented by the ratio difference between two⁃dimensional photography or stereophotogrammetry with the ratio of the gold standard@* Results@#Thirteen patients were eventually recruited. The measurement results of the ratio of lateral lip height by 2D photography tended to be larger (P＝0.019), and the measurement results of the ratio of columella height tended to be smaller (P＝0.008). The measure⁃ment results of the deviation of the subnasale by stereophotogrammetry tended to be smaller (P＝0.003). The pronasale deviation (P＝0.022) with two⁃dimensional photography, the deviation of the labrale superius (P＝0.025) and the ratio of lateral lip length (P＝0.036) with stereophotogrammetry had a significant negative correlation with the extent of cleft malformation@* Conclusion@#Both two⁃dimensional photography and stereophotogrammetry have errors and biases that underexaggerate or overexaggerate the extent of cleft malformation, and some errors may increase with the decrease in the extent of cleft malformation. When applying two⁃dimensional and three⁃dimensional photography to analyze cleft lip and palate deformities, these biases should be considered to evaluate the face more objectively