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Cervical Infantile Fibrosarcoma: a rare cause of paediatric parapharyngeal neck mass
Madhu Priya1, Parvendra Singh1, Manu Malhotra1
1All India Institute of Medical Sciences, Department of Otorhinolaryngology & Head-Neck Surgery, Rishikesh, Uttarakhand, India.
Insights
Infantile fibrosarcoma (IFS) is a rare pediatric neck tumor. Early diagnosis and combined surgery with chemotherapy offer a good prognosis for this uncommon childhood malignancy.
Area of Science:
- Pediatric Oncology
- Surgical Pathology
- Rare Childhood Malignancies
Background:
- Soft tissue tumors are diverse in childhood, including rare malignancies like infantile fibrosarcoma (IFS).
- Infantile fibrosarcoma (IFS) predominantly affects infants under two years old.
- Data on cervical infantile fibrosarcoma (IFS) in the Indian subcontinent is limited.
Observation:
- A case of an eight-year-old male with a rapidly growing left neck mass is presented.
- This represents a rare occurrence of infantile fibrosarcoma (IFS) in an older child.
- The cervical mass was successfully treated with combined modality therapy.
Findings:
- Infantile fibrosarcoma (IFS) in children has a favorable prognosis with a lower metastatic potential than adult fibrosarcoma.
- Surgical excision is the primary treatment, but chemotherapy plays a crucial role in managing primary tumors and metastases.
- The case highlights infantile fibrosarcoma (IFS) as a critical differential diagnosis for pediatric cervical masses.
Implications:
- Increased awareness of infantile fibrosarcoma (IFS) can lead to earlier diagnosis and more effective treatment strategies.
- Understanding this rare entity can improve outcomes and reduce morbidity and mortality in affected children.
- This case contributes valuable data on infantile fibrosarcoma (IFS) incidence in the Indian subcontinent.
Abstract:
Soft tissue tumors are not uncommon in childhood and comprise entities that range from common to very rare malignancies. Infantile fibrosarcoma (IFS) is a rare pediatric malignancy mainly seen in the first two years of life. The data about the incidence of infantile fibrosarcoma occurring in the neck in the Indian subcontinent is scarce. To the best of our knowledge, only one case of infant cervical IFS has been reported previously in the Indian subcontinent. We present another case of an eight-year-old male patient with a rapidly growing mass on the left side of the neck. He was successfully treated with a combined modality of surgery and chemotherapy with a good outcome. Among the soft tissue tumors of childhood, IFS is a rare entity. It has a good prognosis and lesser chance of distant metastasis as compared to adult fibrosarcoma. Though surgical excision is the mainstay of treatment, chemotherapy also has a significant role in the treatment of primary tumor and metastasis. We discuss the stated case to bring to the notice this uncommon cause, which can be considered as a differential diagnosis of upper cervical swellings. A better understanding of this entity would help in early diagnosis and aggressive treatment, reducing the overall morbidity and mortality.
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