Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review

Iatrou, Ioannis; Theologie-Lygidakis, Nadia; Tzermpos, Fotis; Vessala, Anna-Maria

doi:10.1016/j.jcms.2017.03.005

Cited by 26 publications

(27 citation statements)

References 43 publications

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“…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.).…”

Section: Discussionmentioning

confidence: 99%

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

Ji²,

Souza³

et al. 2018

Med Oral

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BackgroundTo describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature.Material and MethodsCases diagnosed as RMS affecting the head and neck region were retrospectively retrieved from the files of two Brazilian institutions from January 2006 to January 2017. Data on clinical features (sex, age and affected site), microscopic subtype, immunohistochemical results, treatment employed and follow-up status were obtained from the patient’s medical charts.ResultsDuring the period considered, 10 cases of RMS were identified. Females predominated (4M:6F), the mean age at diagnosis was 16.5 years-old and the orbit was the most affected site (4 cases). Microscopically, most cases were classified as embryonal RMS (6 cases) and the Desmin/Myogenin/Myo-D1 immunohistochemical positivity was useful to confirm the diagnosis. Chemotherapy and radiotherapy were applied to 9 and 8 patients respectively, whereas 2 patients were treated by surgery. Recurrences occurred in 3 patients and distant metastasis in 2 cases. Nine patients were alive in their last follow-up, 3 of them with disease, whereas 1 patient died due to the disease.ConclusionsHead and neck RMS is an aggressive malignant neoplasm which demands especial concern to achieve early diagnosis and successful treatment. Key words:Rhabdomyosarcoma, soft tissue tumors, head and neck, oral cavity, chemotherapy.

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Section: Discussionmentioning

confidence: 99%

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

Ji²,

Souza³

et al. 2018

Med Oral

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“…Primary intraosseous rhabdomyosarcomas are rare, with only few case reports being described, more often involving the head and neck bones, and less commonly the long bones or pelvis. (1)(2)(3)(4)(5)(6)(7)(8) Recently, Watson et al (9) reported a group of 3 rhabdomyosarcomas in young adult females (age range 16-38 yrs) involving the pelvis, chest wall and sphenoid bones that showed a distinctive epithelioid to spindle cell morphology and had EWSR1-TFCP2 or FUS-TFCP2 gene fusions. Dashti et al (10) subsequently reported an additional case of a skeletal rhabdomyosarcoma of the mandible of a 72 year-old woman showing a spindle cell morphology and a FUS-TFCP2 gene fusion.…”

Section: Introductionmentioning

confidence: 99%

Expanding the Spectrum of Intraosseous Rhabdomyosarcoma

Agaram

Zhang

Sung

et al. 2019

American Journal of Surgical Pathology

106

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Primary intraosseous rhabdomyosarcomas are extremely rare. Recently two studies reported 4 cases of primary intraosseous rhabdomyosarcoma with EWSR1/FUS-TFCP2 gene fusions, associated with somewhat conflicting histologic features, ranging from spindle to epithelioid. In this study we sought to further investigate the pathologic and molecular abnormalities of a larger group of intraosseous rhabdomyosarcomas by a combined approach using targeted RNA sequencing analysis and fluorescence in-situ hybridization (FISH). We identified 7 cases, 3 males and 4 females, all in young adults, age range 20-39 years (median 27 years). Three cases involved the pelvis, 2 involved the femur and 1 each involved the maxilla and the skull. Molecular studies identified recurrent gene fusions in all 7 cases tested, including: a novel MEIS1-NCOA2 fusion in 2 cases, EWSR1-TFCP2 in 3 cases and FUS-TFCP2 gene fusions in 1 case. One case showed a FUS gene rearrangement, without a TFCP2 gene abnormality by FISH. The MEIS1-NCOA2 positive cases were characterized by a more primitive and fascicular spindle cell appearance, while the EWSR1/FUS rearranged tumors had a hybrid spindle and epithelioid phenotype, with more abundant eosinophilic cytoplasm and mild nuclear pleomorphism. Immunohistochemically, all tumors were positive for desmin and myogenin (focal). In addition, 4 tumors with TFCP2 associated gene fusions also co-expressed ALK and Cytokeratin. In conclusion, our results suggest a high incidence of gene fusions in primary rhabdomyosarcomas of bone, with two molecular subsets emerging, defined by either MEIS1-NCOA2 or EWSR1/FUS-TFCP2 fusions, showing distinct morphology and immunophenotype. Additional studies with larger numbers of cases and

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“…According to its pathological features, RMS is categorized into embryonal RMS (ERMS), alveolar RMS (ARMS), pleomorphic RMS (PRMS), and fusiform/sclerosing RMS according to the Intergroup Rhabdomyosarcoma Study group . Among these, ERMS is the most common type in children, accounting for 65% to 80% of all children with RMS . ERMS may arise in any part of a child's body and is closely correlated with prognosis .…”

Section: Discussionmentioning

confidence: 99%

“…2 Among these, ERMS is the most common type in children, accounting for 65% to 80% of all children with RMS. 3 ERMS may arise in any part of a child's body and is closely correlated with prognosis. 4 Based on clinical analysis of patients with RMS in Beijing over the past 10 years, previous studies have found that the genitourinary system is the most common onset site for RMS, followed by the head and neck, and the retroperitoneal space and limbs; no cases have been located in the mediastinum.…”

Section: Discussionmentioning

confidence: 99%

Retrospective analysis of rhabdomyosarcoma (RMS) in children in a single center

Bian

Jing

et al. 2018

Thoracic Cancer

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BackgroundThis study was conducted to explore the influence of clinical features of rhabdomyosarcoma (RMS) and a refined therapeutic protocol on the therapeutic efficacy and prognosis in children in the past five years.MethodsForty children diagnosed with RMS were retrospectively studied, using a version of the therapeutic protocol refined by Shanghai Children's Medical Center (version 2009.9.1). The patients’ demographic characteristics, clinical manifestations, pathological features, therapeutic efficacy, and prognosis were analyzed.ResultsOf the 40 children, 17 abandoned treatment. Of the remaining 23 cases, two children were rated as low risk, 12 as medium risk, and nine as high risk, and all received treatment. Patients in the low and medium‐risk groups had better prognosis than those in the high‐risk group, and treated patients had higher survival rates and longer survival than untreated patients.ConclusionChildren with RMS should be treated positively. Combined treatment shows better therapeutic efficacy and prognosis. The refined therapeutic protocol seems more effective than the standard treatment, with a significant impact on long‐term RMS prognosis.

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Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review

Cited by 26 publications

References 43 publications

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

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

Expanding the Spectrum of Intraosseous Rhabdomyosarcoma

Retrospective analysis of rhabdomyosarcoma (RMS) in children in a single center

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