Mezri Sameh, Zitouni Chaima, Abid Ilyes, Gargouri Faten, Laabidi Besma, Akari Khemaies
Department of Otorhinolaryngology, Military Hospital of Instruction, Tunis, Tunisia.
University of Tunis El Manar, Tunis, Tunisia.
Ear Nose Throat J. 2023 Nov 24:1455613231215196. doi: 10.1177/01455613231215196.
Undifferentiated small round cell sarcoma is an extremely rare and highly aggressive tumor touching the soft tissues and bones. Here, we report the case of a 7-month-old girl who presented to our department with respiratory distress due to a hypopharyngeal mass causing airway obstruction. The patient underwent a tracheotomy and had a direct laryngoscopy. Histological examination confirmed the diagnosis of an undifferentiated small round cell sarcoma. The patient received chemotherapy and radiotherapy and underwent neck dissection. One year later, the patient was diagnosed with local recurrence as well as metastatic cervical nodes and liver and pulmonary metastases. The case is original by the tumor's location and the age of onset. To the best of our knowledge, this could be the first case in English and French literature reporting an undifferentiated small round cell sarcoma affecting the hypopharynx among infants. The purpose of this article is to report our case and discuss its clinical and anatomopathological features as well as its prognosis and treatment options.
未分化小圆形细胞肉瘤是一种极其罕见且侵袭性很强的肿瘤，可累及软组织和骨骼。在此，我们报告一例7个月大的女童病例，该女童因下咽肿物导致气道梗阻，出现呼吸窘迫症状前来我科就诊。患者接受了气管切开术并进行了直接喉镜检查。组织学检查确诊为未分化小圆形细胞肉瘤。患者接受了化疗、放疗并进行了颈部清扫术。一年后，患者被诊断为局部复发以及颈部淋巴结转移、肝转移和肺转移。该病例因其肿瘤位置和发病年龄而具有独特性。据我们所知，这可能是英法文献中首例报道婴儿期发生的累及下咽的未分化小圆形细胞肉瘤病例。本文旨在报告我们的病例，并讨论其临床和解剖病理学特征以及预后和治疗方案。
