Clinical Character of Pediatric Head and Neck Rhabdomysarcomas: A 7-Year Retrospective Study

Linicopathological analysis of head and neck rhabdomyosarcoma: A series of 10 cases and literature review head and neck rhabdomyosarcoma

“…In contrast to our series where females predominated, in the literature RMS presents a slight male predilection, with a male:female ratio of 1.5:1 ( 5 , 6 , 11 , 12 , 14 ). Clinical signs and symptoms mainly depend on the affected site and may vary considerably.…”

“…Microscopically, RMS can be classified into different histologic subtypes, and as shown in our study, the most common is the embryonal subtype (EMB), accounting for 60% of all cases, characterized by undifferentiated, small, round and hypercromatic cells with variable number of strap or tadpole-shaped, eosinophilic rhabdomyoblasts ( 11 , 20 ). Alveolar subtype (ALV) represents approximately 30% of the cases, and it is characterized by small round rhabdomyoblasts arranged in nests separated by connective tissue trabeculae and focal areas of alveolar architecture with hypercromatic nuclei and eosinophilic cytoplasm ( 11 , 15 , 16 ). ALV RMS is more common in older patients than EMB, ranging between 10 and 25 years-old with no gender predilection and usually with a more unfavorable prognosis ( 8 , 11 , 16 , 26 ).…”

“…RMS is a mesenchymal malignant neoplasm with skeletal muscle differentiation that represents the most common soft tissue sarcoma in the pediatric population. Approximately 40% of the cases develop in the head and neck region and the appropriate treatment demands a multi-modality approach ( 5 , 6 , 11 - 13 ). In this study we described a series of 10 cases of head and neck RMS and reviewed all published clinical series dealing with RMS of the head and neck with at least 3 cases reported to better understand the clinicopathological features of this aggressive malignancy (Table Table 2 , Table 2 continue, Table 2 continue-1, Table 2 continue-2, Table 2 continue-3.).…”

“…Alveolar subtype (ALV) represents approximately 30% of the cases, and it is characterized by small round rhabdomyoblasts arranged in nests separated by connective tissue trabeculae and focal areas of alveolar architecture with hypercromatic nuclei and eosinophilic cytoplasm ( 11 , 15 , 16 ). ALV RMS is more common in older patients than EMB, ranging between 10 and 25 years-old with no gender predilection and usually with a more unfavorable prognosis ( 8 , 11 , 16 , 26 ). Moreover, approximately 75% of ALV carry a chromosomal translocation that results in the fusion of two transcript factor-encoding genes, the PAX3 gene (or less commonly PAX7 gene) and the FOXO1 gene, resulting in the expression of the chimeric PAX3/7-FOXO1 protein ( 7 ).…”

Utilization of Free Tissue Transfer for Pediatric Oromandibular Reconstruction

“…However, sarcoma studies have shown the importance of obtaining negative surgical margins as evidenced by local failure and poor survival if positive margins remain. [2][3][4][5][6][7] Five-year survival dropped from 80% to 55% in pediatric sarcoma patients who had a positive surgical margin. 2,3 In addition, surgical resection is usually recommended after the patient has been treated with radiation therapy, chemotherapy, or both, resulting in compromise of the recipient bed and limiting the use of adjacent tissue transfer or bone grafts.…”