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Case Reports
. 2021 Sep 20;14(3):1342-1346.
doi: 10.1159/000518317. eCollection 2021 Sep-Dec.

Pseudoprogression of Malignant Peripheral Nerve Sheath Tumor in Patient with Neurofibromatosis Type 1, a Case Report

Andrés J Lessing  1 , Gregory M Cote  2 , Thomas F DeLaney  3 , Scott R Plotkin  4 , Kevin A Raskin  5 , Juan N Lessing  6
Affiliations
Case Reports

Pseudoprogression of Malignant Peripheral Nerve Sheath Tumor in Patient with Neurofibromatosis Type 1, a Case Report

Andrés J Lessing et al. Case Rep Oncol. .

Abstract

Pseudoprogression, defined as the radiographic false appearance of disease progression, is not frequently observed in patients with malignant peripheral nerve sheath tumor (MPNST). We report on a case of a patient with neurofibromatosis type 1 (NF1) MPNST pseudoprogression that presented as suspected local recurrence 9.5 years after last treatment. The patient underwent surgical resection following growth of a mass on sequential MRI imaging; surgical pathology, however, showed skeletal muscle with atrophy, fibroadipose tissue, and fat necrosis, without any evidence of tumor. As MPNST survival rates increase, physicians should consider pseudoprogression as a potential presentation after prior treatment.

Keywords: MRI; Malignant peripheral nerve sheath tumors; Nerve tumor; Neurofibromatosis; Pseudoprogression.

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Conflict of interest statement

Mr. Andrés Lessing has no conflicts of interest to declare. Dr. Thomas DeLaney has no conflicts of interest to declare. Dr. Gregory M. Cote reports Advisory Board fees from Agios, Epizyme, and PharmaMar; Support provided to his institution for the conduct of clinical trials from Agios, Epizyme, PharmaMar, Eisai, Macrogenics, Boston Biomedical, Plexxicon, Merck KGaA/EMD Serono Research and Development Institute, CBA, SpringWorks Therapeutics, Bavarian-Nordic, and Bayer. Dr. Kevin Raskin has no conflicts of interest to declare. Dr. Scott Plotkin reports co-founding NFlection Therapeutics and NF2 Therapeutics and consulting for AstraZeneca, Akouos, and SonALAsense. Dr. Juan Lessing has no conflicts of interest to declare.

Figures

Fig. 1
Fig. 1
Historical coronal MRI images of right shoulder and axilla. Coronal MRI images of right shoulder and axilla. Images labeled TI are T1 weighted, images labeled T1+C are T1 weighted with contrast (gadolinium), images labeled T2 are T2 weighted. Images A are dedicated axilla MRI images from 2007 that show pathology-confirmed tumor mass, measuring 4.0 × 3.5 × 1.5 cm. Images B are from full-body MRI in mid-2018 that show artifact in the right axilla from prior tumor resection with interval increase in size of edema and a T2 hyperintense lesion adjacent to the right latissimus dorsi, measuring approximately 2.1 × 1.3 cm. Images C are MRI of the axilla 3 months later, showing a T2 hyperintense infiltrative enhancing mass with measurable growth, now measuring 4.1 × 2.0 × 2.4 cm.
See this image and copyright information in PMC

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