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Undifferentiated Small Round Cell Sarcoma of the Hypopharynx in a 7-Month-Old Patient
Sameh Mezri1,2, Chaima Zitouni1,2, Ilyes Abid1,2
1Department of Otorhinolaryngology, Military Hospital of Instruction, Tunis, Tunisia.
Insights
This case report details an extremely rare undifferentiated small round cell sarcoma in a 7-month-old infant
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Rare Cancers
Background:
- Undifferentiated small round cell sarcoma (URSC) is a rare and aggressive malignancy.
- Pediatric soft tissue and bone sarcomas are uncommon, with URSC being exceptionally rare in infants.
Purpose of the Study:
- To report a unique case of URSC in the hypopharynx of an infant.
- To discuss the clinical presentation, diagnosis, and treatment of this rare pediatric tumor.
Main Methods:
- Case report of a 7-month-old female infant.
- Diagnostic procedures included direct laryngoscopy and histological examination.
- Treatment involved chemotherapy, radiotherapy, and neck dissection.
Main Results:
- Histological confirmation of undifferentiated small round cell sarcoma.
- The patient experienced local recurrence and distant metastases (cervical nodes, liver, lungs) within one year.
- This represents a potentially novel case in English and French literature due to tumor location and infant age.
Conclusions:
- URSC in the hypopharynx is exceptionally rare in infants.
- Early diagnosis and aggressive multimodal treatment are crucial.
- Further research is needed to understand prognosis and optimize therapeutic strategies for this rare pediatric malignancy.
Abstract:
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.
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