Tongue Spindle Cell Rhabdomyosarcoma: A Rare Case Report and Literature Review
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CASE REPORT
Tongue Spindle Cell Rhabdomyosarcoma: A Rare Case Report and Literature Review Ursula M. Jariod–Ferrer1 • Miguel Angel Trigo-Cebrian2 • Bianca Pantilie1 • Marı´a V. Simon Sanz1 • Leire Esparza Lasaga1 • Marina A. Gavin–Clavero1 • Elena Delso-Gil1 • Javier Martinez-Trufero3
Received: 6 April 2020 / Accepted: 7 September 2020 Ó The Association of Oral and Maxillofacial Surgeons of India 2020
Abstract Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm with its origin in the skeletal muscle and is extremely rare in adults. By the World Health Organization (WHO), a new variant of RMS has been classified, i.e. the spindle cell (Sc) and sclerosing (S) RMS. While the ScRMS shows intersecting fascicles of nonpleomorphic spindle cells, the S-RMS is characterized by a marked hyalinization in a pseudovascular growth pattern associated with round-to-spindled tumour cells. According to the analysed data, the Sc/S-RMS variant has a worse outcome than other variants. The new classification of the Sc/SRMS variant is valuable to the clinical practice. There are not many oral Sc/S-RMS cases reported. The aim of this paper is to demonstrate that an early diagnosis, an adequate treatment and a multidisciplinary approach have a positive effect on the prognosis of the patient. In this study, we analyse a new case of Sc-RMS variant in a young adult with an early diagnosis and a favourable outcome as a result of an appropriated multidisciplinary treatment: early surgery, radiotherapy and chemotherapy treatment. Keywords Rhabdomyosarcoma Oral cancer Spindle cell rhabdomyosarcoma Sarcoma & Ursula M. Jariod–Ferrer [email protected] 1
Oral and Maxillofacial Department, Miguel Servet University Hospital, Paseo Isabel La Cato´lica, n8 1-3, 50009 Zaragoza, Spain
2
Pathological Anathomy Department, Miguel Servet University Hospital, Paseo Isabel La Cato´lica n8 1-3, Zaragoza, Spain
3
Medical Oncology Department, Miguel Servet University Hospital, Paseo Isabel La Cato´lica n8 1-3, 50009 Zaragoza, Spain
Introduction Head and neck sarcomas are extremely rare tumours, so therapeutic algorithms are based on retrospective studies of the treatment of sarcomas from other locations. Its diagnosis might become really tough because of the clinical variability in its behaviour. In fact, the sarcomas count make up 2% of all the malignant head and neck tumours and 4–10% of all the adult sarcomas only [1]. Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm of skeletal muscle origin. It is the most common soft tissue tumour during childhood and responsible for approximately one-half of all soft tissue sarcomas in this age group [2]. However, RMS in adults is extremely rare [3]. Aetiology and risk factors are still unknown. Most cases of RMS seem to be sporadic, but the disease is associated with some familial syndromes such as neurofibromatosis, the Li-Fraumeni, Beckwith-Wiedemann, and Costello syndromes [4]. There are four categories of RMS: alveolar, embryonal, pleomorphic and spindle cell/sclerosing [5]. Th
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