Background: Alveolar soft-part sarcoma (ASPS) is an uncommon malignancy that may present in a manner similar to benign vascular tumors. Methods: A 6-year-old boy with autism spectrum disorder was referred to the Johns Hopkins All Children's Hospital vascular anomalies clinic for the evaluation of a tongue mass. Results: Prior to the presentation, at 5 years of age, neck computed tomography (CT) was performed. This showed a well circumscribed, enhancing mass at the anterior aspect of the tongue. The radiologic impression was that this lesion was most likely a hemangioma. Two years later, the patient was evaluated in the vascular anomalies clinic. At that examination a 2-3 cm swelling was noted on the dorsal aspect of the tongue. The mass was fleshy and firm with discrete borders. Handheld Doppler examination indicated a high-flow lesion. The patient underwent an excisional biopsy. The lesion was identified as an alveolar soft-part sarcoma based on pathologic characteristics. Conclusion: Familiarity with common vascular tumors and malformations allows providers to diagnose the majority of these lesions on a combination of clinical history and physical examination. Atypical and combined lesions do benefit from imaging to help characterize and aid in the differential diagnosis. Biopsy enables definitive diagnosis but is necessary in the minority of cases. When in doubt, referral to a specialized, multidisciplinary vascular anomalies clinic will ensure that these patients receive management for this challenging collection of conditions.
Objective: We aimed to assess significant ethnic variabilities in infants’ nasolabial anthropometry to motivate variations in surgical correction of a synchronous bilateral cleft lip/nasal anomaly, specifically whether a long columella is a European feature, therefore accepting a short columella and/or delayed columellar lengthening suitable for reconstruction in ethnic patients. Methods: Thirty-three infants without craniofacial pathology (10 African American [AA], 7 Hispanic [H], and 16 of European descent [C]), ages 3 to 8 months, presenting to the Johns Hopkins All Children’s general pediatric clinic were recruited. Four separate 3D photographs (2 submental and frontal views each) were taken using the Vectra H1 handheld camera (Canfield Imaging). Eighteen linear facial distances were measured using Mirror 3D analysis (Canfield Imaging Systems). Difference between ethnicities was measured using analysis of variance with the Bonferroni/Dunn post hoc comparisons. Pearson correlation was employed for interrater reliability. All statistical analyses were carried out using SPSS version 21.0 (IBM Corp), with statistical significance set at P < .05. Results: Nasal projection (sn-prn) and columella length (sn-c) did not differ significantly between groups ( P = .9). Significant differences were seen between ethnic groups in nasal width (sbal-sbal [C-AA; P = .02]; ac-ac [C-AA; P = .00; H-AA; P = .04]; al-al [C-AA; P = .00; H-AA; P = .001]) and labial length (sn-ls [C-AA; P = .041]; sn-sto [C-AA; P = .005]; Cphs-Cphi L [C-AA; P = .013]; Cphs-Cphi R [C-AA; P = .015]). Interrater reliability was good to excellent and significantly correlated for all measures. Conclusions: African American infants exhibited wider noses and longer lips. No difference was noted in nasal projection or columella length, indicating that these structures should be corrected during the primary cleft lip and nasal repair for all patients and should not be deferred to secondary correction.
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