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

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Abstract

Background

To describe the clinicopathological characteristics of a series of head and neck rhabdomyosarcoma (RMS) and to review the literature.

Material and Methods

Cases 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.

Results

During 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.

Conclusions

Head 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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Most cited references 29

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Sarcoma classification: an update based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone.

Leona Doyle (2014)

The 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone incorporates changes in tumor classification, as well as new genetic insights into the pathogenesis of many different tumor types that have emerged over the 11 years since the publication of the prior volume. This article reviews changes in the classification of soft tissue and bone sarcomas as well as tumors of intermediate biologic potential in the 2013 World Health Organization volume, new molecular insights into these tumors, and associated surgical and clinical implications.

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Rhabdomyosarcoma of the head and neck in children.

Rodney Hicks, C Flaitz (2002)

The purpose of this study was to determine the rhabdomyosarcoma types involving the head and neck (H&N) region in children and their immunophenotype. Anatomic pathology archives at Texas Children's Hospital were searched for all rhabdomyosarcoma cases over a 20-year period. One-hundred and thirty-seven cases were identified, with 50 being H&N cases. The cases were typed according to the Intergroup Rhabdomyosarcoma Study (IRS) criteria. Immunocytochemistry for myogenic and non-myogenic markers was performed on all H&N cases. Electron micrographs from cases (n=32) where ultrastructural examination had been performed at the time of original diagnosis were reviewed. Children with H&N rhabdomyosarcomas had a mean age of 5.3 years (median 4 years). There was a male predilection (1.7M:1.0F). Primary tumor sites were: face NOS (18%), orbit/periorbital (16%), nasal cavity/nose (14%), lymph nodes (12%), paranasal sinuses (10%), parameningeal (10%), parotid gland (6%), neck (6%), infratemporal fossa/zygoma (2%), buccal mucosa (2%), palate (2%), and larynx (2%). Metastatic disease at diagnosis (33% of all cases) occurred in the bone marrow (11%), cerebrospinal fluid (6%), peritoneal fluid (6%), lung (4%), parietal pleura (2%), pleural fluid (2%) and pericardial fluid (2%). Rhabdomyosarcoma types (IRS criteria) were: embryonal (60%), alveolar (classic and solid subtypes, 28%), botryoid (4%), undifferentiated (4%), spindle cell (2%) and anaplastic (2%). Immunocytochemical findings were: polyclonal desmin (96%); myogenin (96%); muscle-specific actin (74%), smooth muscle actin (12%). Nonmyogenic markers included: vimentin (100%), CD99 (16%), p53 (16%), pancytokeratin (10%), NSE (8%), LCA (6%), CD20 (6%), EMA (2%), and NB-84 (0%, neuroblastoma). Undifferentiated sarcoma expressed only vimentin. By ultrastructural examination, 44% had readily identified z-bands and myofilaments, 37% had infrequent to rare myofilaments and z-bands, and 19% had myotubular intermediate filaments. Distribution of H&N rhabdomyosarcoma IRS types is similar to that for all primary sites, with the exception that embryonal types are modestly increased while alveolar type is mildly decreased. There are many non-myogenic immunocytochemical markers that cross-react with rhabdomyosarcoma. Differentiation between favorable and unfavorable rhabdomyosarcoma types is important for appropriate therapy, and predicting prognosis and survival.

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Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases.

R Chigurupati, A Alfatooni, R.W.T. Myall … (2002)

Rhabdomyosarcoma (RMS) is an aggressive malignant skeletal muscle neoplasm arising from embryonal mesenchyme. It accounts for over 50% of all pediatric soft tissue sarcomas. The head and neck region is the most common site for this tumor in children. Neonatal presentation of this tumor is rare. We present the management of one neonatal case and three additional cases of orofacial RMS in children under the age of 7 years. All four patients were seen in the department of oral and maxillofacial surgery at Children's Hospital and Regional Medical Center (CHRMC) in Seattle between 1992-2000. Three of the four cases were alveolar RMS and one was botryoid sub-type of embryonal RMS. Three patients were treated with a combination of surgery, chemotherapy and radiation, while the patient with botryoid RMS was treated with surgery and chemotherapy only. The patient with congenital RMS died at 2.5 years of age due to recurrent metastatic disease. The other three patients are alive without evidence of recurrent with a mean follow up was 5.5 years (range 2.5-8.5 years). We discuss the current management, diagnosis, biological behavior, histopathology, prognosis and survival of head and neck RMS in neonates and young children.

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Author and article information

Journal

Journal ID (nlm-ta): Med Oral Patol Oral Cir Bucal

Journal ID (iso-abbrev): Med Oral Patol Oral Cir Bucal

Journal ID (publisher-id): Medicina Oral S.L.

Title: Medicina Oral, Patología Oral y Cirugía Bucal

Publisher: Medicina Oral S.L.

ISSN (Print): 1698-4447

ISSN (Electronic): 1698-6946

Publication date (Print): March 2018

Publication date (Electronic): 25 February 2018

Volume: 23

Issue: 2

Pages: e188-e197

Affiliations

[1 ]Service of Oral Pathology, João de Barros Barreto University Hospital, Federal University of Pará (Belém/Brazil)

[2 ]Department of Oral Diagnosis (Pathology), Piracicaba Dental School, University of Campinas (Piracicaba/Brazil)

[3 ]Department of Oral Medicine, Sírio-Libanês Hospital, São Paulo/Brazil

[4 ]Department of Pathology, Sírio-Libanês Hospital, São Paulo/Brazil

[5 ]Department of Oral Surgery and Pathology, School of Dentistry, Universidade Federal de Minas Gerais (Belo Horizonte/Brazil)

Author notes

Department of Oral Surgery and Pathology School of Dentistry Universidade Federal de Minas Gerais Av. Antônio Carlos, 6627 31270-901, Pampulha, Belo Horizonte, MG, Brazil , E-mail: felipepfonseca@ 123456hotmail.com

Conflict of interest statement: All authors disclose any financial and personal relationships with other people or organizations that could inappropriately influence (bias) this work.

Article

Publisher ID: 22106

DOI: 10.4317/medoral.22106

PMC ID: 5911360

PubMed ID: 29476676

SO-VID: 7cf250e3-9570-4776-aa97-e6c2e5fa359d

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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

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Abstract

Background

Material and Methods

Results

Conclusions

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25th European Students' Conference

Most cited references 29

Sarcoma classification: an update based on the 2013 World Health Organization Classification of Tumors of Soft Tissue and Bone.

Rhabdomyosarcoma of the head and neck in children.

Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases.

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