Solitary fibrous tumor (SFT) of the pancreas is rare, with 15 adult cases reported in the English literature. We described a 14-month-old boy who presented with obstructive jaundice. Dominantly elevated serum CA19-9 was detected. Imaging studies revealed a well-circumscribed, solid mass in the pancreatic head. A pancreaticoduodenectomy (child procedure) was performed using Shen’s anastomosis technique. After resection of the tumor, liver function and serum tumor markers normalized and clinical signs receded. The boy was disease free after a follow-up of 12 months. Histological examination showed the tumor consisted of “patternless pattern” arranged spindle tumor cells and keloid-like hyalinized collagen. Immunohistochemical staining was positive for CD34 and vimentin. Mutation analysis of CTNNB1 was negative. To the best of our knowledge, our patient was the first case of pancreatic SFT in a pediatric population. SFT should be considered in differential diagnosis when confronted with a pancreatic tumor in children. Complete resection should be meticulously pursued.

Background: Clinical evaluation of the postural balance in adolescent idiopathic scoliosis has been evaluated by coronal and sagittal parameters. The relationship between coronal and sagittal parameters has not been fully studied in adolescent idiopathic scoliosis. Objective: This study aimed to evaluate the relationship between coronal and sagittal parameters in idiopathic scoliosis. Methods: One hundred and three patients of Adolescent Idiopathic Scoliosis (AIS) were recruited. Radiographs were evaluated for the following spine and pelvic measurements. Coronal parameters including thoracic Cobb angle (TC), lumbar Cobb angle (LC), global coronal balance (GCB), apical translation of the major curve (AT), and coronal pelvic tilt (CPT) were analyzed. Sagittal parameters including thoracic kyphosis (TK), lumbar lordosis (LL), sagittal vertical axis (SVA), pelvic incidence (PI), pelvic tilt (PT), and sacral slope (SS) were analyzed. A canonical correlation analysis was performed between all of the coronal parameters and sagittal parameters. Results: One hundred and three patients were recruited (male 15, female 88; aged 12.5 ± 1.5 years). Compared with the published age-matched normal population, TK, PI, LL, and SS were not statistically different in the normal group. However, PT was significantly increased in patent groups (P = 0.003). Canonical correlation analysis showed strong correlation between coronal parameters and sagittal parameters, F (20,313) = 2.44, p = 0.001. SVA was not correlated with any coronal parameter. Conclusions: Children with AIS showed an increased pelvic tilt sign. In this study, TC, LC, GCB, AT, CPT, TK, LL, SVA, PI, PT, and SS contributed to the postural balance. In AIS, coronal balance is contributed to sagittal balance.

BackgroundThe proximal chromosome 15q is prone to unequal crossover, leading to rearrangements. Although 15q11q13 duplications are common in patients with developmental delays and mental impairment, 15q aneusomies resulting in greater or equal to 4 copies of 15q11q13 are rare and no pentasomy 15q11q13 has been reported in the literature. Thus far, all reported high copy number 15q11q13 cases are from the West populations and no such study in Chinese patients have been documented. Dosage-response pattern of high copy number 15q11q13 on clinical presentations is still a subject for further study.Case PresentationIn this study, we characterized two Han Chinese patients with high copy number 15q11q13. Using chromosome banding, high resolution SNP-based cytogenomic array, Fluorescence in situ hybridization, and PCR-based microsatellite analysis, we identified two patients with tetrasomy 15q11q13 and pentasomy 15q11q13. Both 15q11q13 aneusomies resulted from a maternally inherited supernumerary marker chromosome 15, and each was composed of two different sized 15q11q13 segments covering the Prader-Willi/Angelman critical region: one being about 10 Mb with breakpoints at BP1 and BP5 regions on 15q11 and 15q13, respectively, and another about 8 Mb in size with breakpoints at BP1 and BP4 regions on 15q. Both patients presented with similar clinical features that included neurodevelopmental delays, mental impairment, speech and autistic behavior, and mild dysmorphism. The patient with pentasomy 15q11q13 was more severely affected than the patient with tetrasomy 15q11q13. Low birth weight was noted in patient with pentasomy 15q1q13.ConclusionsTo the best of our knowledge, this is the first case of pentasomy 15q11q13 and the first study of high copy number 15q11q13 in Han Chinese patients. Our findings demonstrate that patients with tetrasomy and pentasomy of chromosome 15q11q13 share similar spectrum of phenotypes reported in other high copy number 15q11q13 patients in the West, and positive correlation between 15q11q13 copy number and degree of severity of clinical phenotypes. Low birth weight observed in the pentasomy 15q11q13 patient was not reported in other patients with high copy number 15q11q13. Additional studies would be necessary to further characterize high copy number 15q11q13 aneusomies.