Roubaud M Jean-Christophe, Prevot Julien, Leclere Jean-Christophe, Mistretta Charlotte, Mornet Emmanuel, Marianowski Rémi
CHRU de Brest, Department of Otolaryngology, Head & Neck Surgery, Brest, France.
CHRU de Brest, Department of Otolaryngology, Head & Neck Surgery, Brest, France.
Int J Surg Case Rep. 2020;75:322-326. doi: 10.1016/j.ijscr.2020.09.076. Epub 2020 Sep 12.
Rhabdomyosarcoma (RMS) arises from mesenchymal cells committed to skeletal muscle. It is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. In this population, there are two main histological forms, which are the embryonal or the alveolar RMS. The retro auricular location is extremely rare. We present 2 cases of children with left retro-auricular RMS occurring at a very early stage of post natal development.
Two children were included in the RMS 2005 Protocol. The first child, aged 5-days, was managed by surgical resection in two steps after a previous incomplete resection which was followed by 8 chemotherapy cycle. The second, aged 3-days, was managed by surgical resection of the tumor bed, completed by 8 chemotherapy cycle. On regular follow up for over 6 years after the diagnosis, both patients are free of recurrence.
RMS is a fast-growing malignant and aggressive tumor originating from skeletal muscle. It occurs in the first decade of life and is associated with genetic conditions. With an unusual clinical presentation and anatomical location, both benign and malignant tumors can be suspected. Diagnosis can be performed with CT-scan or MRI and confirmed by biopsy. The treatment is based on chemotherapy followed by radiotherapy or surgical resection. In our two patients, the treatment program achieved complete disease control, with good prognosis especially because of anatomical location as well as early diagnosis.
横纹肌肉瘤（RMS）起源于定向分化为骨骼肌的间充质细胞。它是儿童中最常见的软组织肉瘤，占所有儿科恶性肿瘤的5%。在这一群体中，有两种主要的组织学形式，即胚胎型或肺泡型RMS。耳后部位极为罕见。我们报告2例出生后发育早期发生的左耳后RMS患儿。
两名儿童纳入了RMS 2005方案。第一名5天大的儿童，在先前不完全切除后分两步进行手术切除，随后进行8个化疗周期。第二名3天大的儿童，对肿瘤床进行手术切除，随后完成8个化疗周期。诊断后定期随访6年以上，两名患者均无复发。
RMS是一种起源于骨骼肌的快速生长的恶性侵袭性肿瘤。它发生在生命的第一个十年，与遗传条件有关。由于临床表现和解剖位置不寻常，可能怀疑是良性和恶性肿瘤。可通过CT扫描或MRI进行诊断，并通过活检确诊。治疗基于化疗，随后进行放疗或手术切除。在我们的两名患者中，治疗方案实现了疾病的完全控制，预后良好，特别是由于解剖位置以及早期诊断。
