Piti Techavichit1,2, M John Hicks3, Dolores H López-Terrada3, Norma M Quintanilla3, R Paul Guillerman4, Stephen F Sarabia3, Hadi Sayeed3, Jed G Nuchtern5, Arnold C Paulino6,7, Jodi A Muscal1, M Fatih Okcu1, Murali Chintagumpala1. 1. Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 2. Department of Pediatrics, Division of Hematology and Oncology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand. 3. Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 4. Department of Pediatric Radiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 5. Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas. 6. Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas. 7. Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
Abstract
PURPOSE AND OBJECTIVE: To determine the clinicopathologic and molecular features and outcome of children with mucoepidermoid carcinoma (MEC). METHODS: A retrospective analysis of clinical and histopathologic findings was performed in patients with MEC diagnosed at Texas Children's Cancer Center between 2000 and 2014. RESULTS: Ten female and four male patients with median age 12 years (range 7-19 years) were included in the study. Tumors involved major salivary glands, minor salivary glands of the palate, and the tracheobronchial tree. Nine of 11 patients with salivary MEC underwent more than one surgical resection at the time of initial diagnosis to achieve a gross total resection. Three patients with tracheobronchial tumors underwent pulmonary lobectomy. Three patients received postoperative radiation therapy. No patient was treated with chemotherapy. Histopathologic grades were classified as low (n = 2), intermediate (n = 9), and high (n = 3). All 12 patients with tumor tissue available for testing were positive for MECT1/MAML2 fusion transcripts. There were no deaths, metastases, or recurrences in this series, with a median follow-up of 24 months (range 5-96 months). CONCLUSIONS: Low to intermediate histopathologic grade MECs are more common than high grade MEC in children. In contrast to adults, MECT1/MAML2 fusion transcripts occur with a frequency of 100% in our pediatric MEC series. Complete excision is the treatment of choice and is associated with excellent outcome. The role of radiotherapy is unclear, but may be indicated in patients with high grade tumors with positive surgical margins.
PURPOSE AND OBJECTIVE: To determine the clinicopathologic and molecular features and outcome of children with mucoepidermoid carcinoma (MEC). METHODS: A retrospective analysis of clinical and histopathologic findings was performed in patients with MEC diagnosed at Texas Children's Cancer Center between 2000 and 2014. RESULTS: Ten female and four male patients with median age 12 years (range 7-19 years) were included in the study. Tumors involved major salivary glands, minor salivary glands of the palate, and the tracheobronchial tree. Nine of 11 patients with salivary MEC underwent more than one surgical resection at the time of initial diagnosis to achieve a gross total resection. Three patients with tracheobronchial tumors underwent pulmonary lobectomy. Three patients received postoperative radiation therapy. No patient was treated with chemotherapy. Histopathologic grades were classified as low (n = 2), intermediate (n = 9), and high (n = 3). All 12 patients with tumor tissue available for testing were positive for MECT1/MAML2 fusion transcripts. There were no deaths, metastases, or recurrences in this series, with a median follow-up of 24 months (range 5-96 months). CONCLUSIONS: Low to intermediate histopathologic grade MECs are more common than high grade MEC in children. In contrast to adults, MECT1/MAML2 fusion transcripts occur with a frequency of 100% in our pediatric MEC series. Complete excision is the treatment of choice and is associated with excellent outcome. The role of radiotherapy is unclear, but may be indicated in patients with high grade tumors with positive surgical margins.
Authors: Olga Micol Martínez; Elena Daghoum Dorado; María Dolores Amorós García; María Isabel Oviedo Ramírez; Isabel de la Fuente Muñoz; Jose Luis Fuster Soler Journal: Rare Tumors Date: 2016-10-06
Authors: André Fehr; Sarah Werenicz; Pietro Trocchi; Markus Falk; Reinhard E Friedrich; Angelika Stammler; Andreas Stang; Florian Oesterling; Laura Khil; Göran Stenman; Werner Böcker; Katharina Tiemann; Thomas Löning Journal: Virchows Arch Date: 2021-07-07 Impact factor: 4.064