BACKGROUND: Inflammatory myofibroblastic tumor is a histopathologically distinctive neoplasm of children and young adults. According to World Health Organization (WHO) classification, inflammatory myofibroblastic tumor is an intermediate-grade tumor, with potential for recurrence and rare metastasis. There are no definite histopathologic, molecular, or cytogenetic features to predict malignant transformation, recurrence, or metastasis. METHODS: A 5-year retrospective study of histopathologically diagnosed inflammatory myofibroblastic tumors of various anatomic sites was conducted to correlate anaplastic lymphoma kinase-1 (ALK-1) expression with histological atypia, multicentric origin of tumor, recurrence, and metastasis. Clinical details of all the cases were noted from the clinical work station. Immunohistochemical stains for ALK-1 and other antibodies were performed. Statistical analysis was done using Fisher exact test. RESULTS: A total of 18 cases of inflammatory myofibroblastic tumors were found during the study period, of which 14 were classical. The female-male ratio was 1:1 and the mean age was 23.8 years. Histologically atypical (four cases) and multifocal tumors (three cases, multicentric in origin) were noted. Recurrence was noted in 30% of ALK-1 positive and 37.5% of ALK-1 negative cases, whereas metastasis to the lung, liver, and pelvic bone was noted in the ALK-1 positive group only. CONCLUSIONS: Overall, ALK-1 protein was expressed in 55.6% of inflammatory myofibroblastic tumors. There was no statistically significant correlation between ALK-1 expression, tumor type, recurrence and metastasis. However, ALK-1 immunohistochemistry is a useful diagnostic aid in the appropriate clinical and histomorphologic context.
Antibodies ; Child ; Classification ; Coloring Agents ; Cytogenetics ; Humans ; Immunohistochemistry ; Liver ; Lung ; Lymphoma ; Myofibroblasts* ; Neoplasm Metastasis ; Pelvic Bones ; Recurrence ; Retrospective Studies ; Tertiary Care Centers* ; Tertiary Healthcare* ; World Health Organization ; Young Adult

Objectives: Vascular malformation (VM) of the tongue can cause true macroglossia in children. Reduction glossectomy provides primary relief when sclerotherapy has failed or is not possible. In this study, we evaluated the surgical role in functional outcome of reduction glossectomy performed for VM of the tongue. Patients and Methods: We evaluated the functional and surgical outcomes of seven children who were treated at a tertiary care centre in Southern India between 2013 and 2018. Results: Six children underwent median glossectomy, while one child underwent lateral glossectomy. Functional assessment was performed at least 2 years after the date of surgery. At the time of assessment, speech was comprehensible for three children and was occasionally unintelligible in four children. Taste and swallowing were normal in all seven children. Six children exhibited a minimal residual lesion after surgery, of which only one was symptomatic. Residual lesions were managed with sclerotherapy (n=3), observation (n=2), or repeat surgery (n=1). Conclusion Reduction glossectomy in children with macroglossia secondary to VMs has acceptable outcomes in terms of cosmesis and speech, with no gastronomic restriction.

Objectives: Vascular malformation (VM) of the tongue can cause true macroglossia in children. Reduction glossectomy provides primary relief when sclerotherapy has failed or is not possible. In this study, we evaluated the surgical role in functional outcome of reduction glossectomy performed for VM of the tongue. Patients and Methods: We evaluated the functional and surgical outcomes of seven children who were treated at a tertiary care centre in Southern India between 2013 and 2018. Results: Six children underwent median glossectomy, while one child underwent lateral glossectomy. Functional assessment was performed at least 2 years after the date of surgery. At the time of assessment, speech was comprehensible for three children and was occasionally unintelligible in four children. Taste and swallowing were normal in all seven children. Six children exhibited a minimal residual lesion after surgery, of which only one was symptomatic. Residual lesions were managed with sclerotherapy (n=3), observation (n=2), or repeat surgery (n=1). Conclusion Reduction glossectomy in children with macroglossia secondary to VMs has acceptable outcomes in terms of cosmesis and speech, with no gastronomic restriction.