Malignant peripheral nerve sheath tumour-A case report

Abstract

Introduction: Malignant peripheral nerve sheath tumors (MPNST) are rare, aggressive sarcomatous tumors that arise from peripheral nerve sheaths and show schwann cell differentiation (Thangadurai, 2017) [1]. They commonly arise among patients with neurofibromatosis I and following radiation therapy (Farid et al., 2014). High resolution PET scans confirm the diagnosis and the definitive treatment is surgical excision. Post operative radiotherapy plays an important role in disease free survival rates. Chemotherapy is offered to people who have a systemic disease.

Presentation of case: This case report is on a 47 year old lady, a known case of Von Recklinghausen, who complained of a vague mass in the pelvis with recent history of pain to her right thigh. Pelvic examination revealed a vague mass on the right side of her pelvis. She was planned for excision of the mass. Intraoperative findings were consistent with the clinical and radiological findings. She is currently on regular follow-up and disease free.

Discussion: MPNSTs are rare tumours that arise in patients with neurofibromatosis I. Mutations at the genetic level is responsible for the development of MPNST. Early presentation, radiological confirmation, aggressive margin free surgery followed by chemoradiation helps in near complete cure of the disease.

Conclusion: MPNST are difficult to manage because of their aggressive nature and the limitations in early diagnosis and management. In patients with Von Recklinghausens disease, malignancy must be suspected when a patient presents with the complaints as mentioned in this case report.

Keywords: Case; MPNST; Malignant; Nerve; Peripheral; Pre-sacral; Report; Sheath; Tumour.

Figs. 1 and 2

Shows an intro-op photo of the pre-sacral MPNST.

Figs. 3 and 4

Shows the dimensions of the resected specimen 9 × 7.5 cm).

Fig. 5

Shows a neoplasm composed of delicate spindle shaped cells arranged in bundles and fascicles (H&E ×40).

Fig. 6

Shows the tumor cells (arrow) show elongated wavy nuclei and eosinophilic cytoplasm. The cells are arranged haphazardly in a fibromyxoid stroma (circle) (H&E ×100).

Fig. 7

shows tumor cells with plump elongated nuclei and moderate eosinophilic cytoplasm (arrow) (H&E ×400).

Fig. 8

Shows focal areas showing rhabdoid differentiation - Strap cells (Arrow). Focal foamy macrophages are also seen (Circle) (H&E ×400).

Fig. 9

Shows S100 immunostaining showing strong nuclear and cytoplasmic positivity in 95% of the tumor cells (S100 IHC ×400).

Fig. 10

Shows Ki67 immunostaining showing Strong nuclear positivity in 10–15% of the tumor cells (arrows) (Ki67 IHC ×100).