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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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Cutaneous malignant peripheral nerve sheath tumors.

Crystal Thomas¹, Najwa Somani, Lafayette G Owen

¹Department of Pathology and Laboratory Medicine, Beth Israel Medical Center, Continuum Hospitals of New York, New York, NY, USA.

Journal of Cutaneous Pathology

|July 10, 2009

Summary

Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are rare sarcomas. Early biopsy of growing neurofibromas is crucial for diagnosing these skin-based tumors.

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Area of Science:

Dermatopathology
Oncology
Surgical Pathology

Background:

Cutaneous malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas.
They can arise sporadically or in patients with neurofibromatosis I.
MPNSTs are frequently associated with pre-existing neurofibromas.

Observation:

Two cases of cutaneous MPNSTs are presented.
Case 1: A 27-year-old woman with neurofibromatosis I and a growing back nodule.
Case 2: An 88-year-old man with a rapidly growing calf tumor, lacking neurofibromatosis stigmata.

Findings:

Biopsies revealed densely cellular areas with atypical, hyperchromatic spindled cells and frequent mitotic figures.
Both tumors showed transition from conventional or diffuse neurofibroma to MPNST.
Histological hallmarks include cellular atypia, increased cellularity, and mitotic activity.

Implications:

Cutaneous MPNSTs are rare but important diagnoses in dermatology and oncology.
An increase in the size of a pre-existing neurofibroma warrants prompt biopsy.
Accurate histological assessment is key for timely MPNST diagnosis and management.