Himanshu Jain, Kriti Bhujel* and Chandra S Rai

Department of ENT, SMIMS, SMU, India.

*Corresponding Author: Kriti Bhujel, Department of ENT, SMIMS, SMU, India.

Received: February 03, 2024; Published: March 03, 2024

Abstract

Malignant Peripheral Nerve Sheath Tumour (MPNST) is atypical and destructive sarcoma of the soft tissues which originates from peripheral nerves or their adjacent tissues. It is marked by invasiveness, malignancy, and sometimes linked to neurofibromatosis type 1 (NF1). Diagnostic complexity arises from varied histology resembling other sarcomas or benign nerve tumours. Treatment options are limited, especially for advanced cases, resulting in a grim prognosis. Surgical removal, often followed by radiation or chemotherapy, remains the primary localized treatment.
The patient in concern came with complaints of a progressively increasing swelling in the right side of neck for past 5 year, which was a solitary ovoid swelling extending from nape of neck to level of the right shoulder. CECT Neck revealed large exophytic mass involving right posterior triangle neck involving skin/ subcutaneous plane. Multiple site FNAC showed haemorrhagic aspirate with few stromal fragments and benign spindle cells. No diagnostic cells were seen. Total excision was done and sent for biopsy which revealed diverse histological features in multiple sections, some were tumour cells with serpentine shapes and myxoid backgrounds; pleomorphic hyperchromatic nuclei, including multinucleated giant cells displaying mitosis, some were round and pleomorphic tumour cells arranged in palisades or whorls. Metaplastic bone formation and necrotic areas were also present.
Additionally, focal lymphocytic aggregations around blood vessels are observed. Patient was then sent for radiotherapy after evaluating for metastasis.
In conclusion, MPNST presents a formidable challenge with limited treatments and poor outcomes. Advancements in understanding its molecular basis and ongoing clinical studies offer hope for improved therapies. Collaborative diagnostic and management approaches are vital for enhancing patient care and results.

Keywords: Malignant Peripheral Nerve Sheath Tumour; Soft Tissue Sarcoma; Malignancy; Histopathologic Examination

References

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Citation

Citation: Himanshu Jain, Kriti Bhujel and Chandra S Rai. “Malignant Peripheral Nerve Sheath Tumour- Unveiling an Unknown Neck Mass".Acta Scientific Otolaryngology 6.3 (2024): 03-07.

Copyright

Copyright: © 2024 Himanshu Jain, Kriti Bhujel and Chandra S Rai. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.