137 Views

826 - Enhancing Function in a Neurofibromatosis Type 1 Patient with Treatment Refractory Malignant Peripheral Nerve Sheath Tumor: The Benefit of Intensive Cancer Rehabilitation

Friday, February 28, 2025

5:00 PM - 6:30 PM MST

Presenting Author(s)

SS

Semiat O. Shafau, BS

Medical Student
Michigan State University College of Osteopathic Medicine
sterling heights, Michigan, United States

Co-Author(s)

ER

Emily Rothermel, MD

Physician
Carolinas Rehabilitation
Charlotte, North Carolina, United States
TP

Terrence M. Pugh, MD

Physician
Carolinas Rehabilitation
Charlotte, North Carolina, United States

Case Diagnosis: Neurofibromatosis Type 1 (NF1) complicated by Malignant Peripheral Nerve Sheath Tumor (MPNST).

Case Description: A 55-year-old woman with a medical history of NF1, complicated by a three-year history of posterior scalp MPNST, was treated with local excision. She developed pulmonary metastasis and underwent chemotherapy. Later, she was found to have progressive right lower extremity weakness secondary to right frontal lobe metastasis and underwent tumor resection. She was admitted to inpatient rehabilitation with impairments including lower extremity ataxia, proprioception deficits, right foot drop, vertigo, insomnia, and indigestion. Treatments included AFO and heel wedge use for ambulation and balance, scopolamine for vertigo, and tapering of dexamethasone, with noted improvement of insomnia and indigestion. Initially, she required set up assistance with feeding, grooming, upper-body dressing, and maximum assistance with lower-body dressing and toileting. She could ambulate 7 feet with a walker. Upon discharge, she was independent in self-care, performed transfers, and walked 150 feet with supervision.

Discussions: NF1 is a neurocutaneous disorder characterized by multiple clinical manifestations. An uncommon complication is MPNST, a leading cause of mortality in these patients. Functional impairments may present secondary to malignancy. Surgical and chemotherapeutic intervention are first line treatments for this malignancy, but individualized therapy may be necessary to improve quality of life and minimize disability. Despite disease progression, this patient showed significant improvements from requiring assistance with daily activities to achieving independence in self-care and improvements with ambulation.

Conclusions: This case illustrates the complex management of NF1-associated MPNST with metastases. Due to the aggressive nature of MPNSTs, patients often experience disease progression and worsening neuromusculoskeletal symptoms. It is important to screen cancer patients for impairments and treat them with a multimodal approach. Patients may benefit from inpatient rehabilitation for functional recovery. This case underscores the importance of tailored treatment plans and rehabilitation strategies for managing NF1-related malignancies.