Kwatra Devanshu, Sagar Poonam, Shukla Shailja
Department of Otolaryngology and Head and Neck Surgery, Lady Hardinge Medical College, New Delhi (India)-110001.
Department of Pathology, Lady Hardinge Medical College, New Delhi (India)-110001.
Iran J Otorhinolaryngol. 2020 Mar;32(109):121-125. doi: 10.22038/ijorl.2019.41435.2351.
Presence of two primary malignancies is rare and occurs in 3-5% of the cancer patients. As per our extensive internet research, this is the only reported case of a synchronous sino-nasal embryonal rhabdomyosarcoma with squamous cell carcinoma-tongue. The case report is important because of the rare diagnosis and the challenge we faced in the diagnosis and treatment of the patient because of the paucity of literature available on management adult rhabdomyosarcoma.
We present a very rare case of an adult male with a sino-nasal mass diagnosed to be an embryonal type rhabdomyosarcoma. The patient also had a moderately differentiated squamous cell carcinoma-tongue for the past 8 months. Radiological investigations were done to see the extent of the sino-nasal mass and the extent of tongue lesion, which was seen to be involving the base of the tongue. The patient was referred for chemoradiotherapy but succumbed to the disease after 2 weeks of treatment.
Occurrence of rhabdomyosarcoma in synchronous malignancies is extremely rare as the most common first as well as second primary malignancy in a diagnosed case of head and neck cancer is squamous cell carcinoma. A multidisciplinary approach to the treatment of adult rhabdomyosarcoma has been recommended. The combined use of chemoradiotherapy and surgery has improved treatment in the recent past but RMS in adults is still a rare head and neck tumour that carries a poor prognosis despite aggressive therapy.
存在两种原发性恶性肿瘤的情况较为罕见,在癌症患者中发生率为3% - 5%。根据我们广泛的网络研究,这是唯一一例同步发生的鼻窦胚胎性横纹肌肉瘤合并舌鳞状细胞癌的报告病例。该病例报告具有重要意义,不仅因为诊断罕见,还因为由于关于成人横纹肌肉瘤治疗的文献匮乏,我们在该患者的诊断和治疗中面临挑战。
我们呈现了一例非常罕见的成年男性病例,其鼻窦肿物被诊断为胚胎型横纹肌肉瘤。该患者在过去8个月还患有中度分化的舌鳞状细胞癌。进行了放射学检查以确定鼻窦肿物的范围以及舌部病变的范围,发现舌部病变累及舌根。该患者被转诊接受放化疗,但在治疗2周后死于该疾病。
横纹肌肉瘤在同步恶性肿瘤中极为罕见,因为在已确诊的头颈癌病例中,最常见的首要和次要原发性恶性肿瘤是鳞状细胞癌。推荐采用多学科方法治疗成人横纹肌肉瘤。近期放化疗与手术联合应用改善了治疗效果,但成人横纹肌肉瘤仍然是一种罕见的头颈肿瘤,尽管积极治疗,预后仍然很差。