Pembrolizumab Achieves a Complete Response in an NF-1 Mutated, PD-L1 Positive Malignant Peripheral Nerve Sheath Tumor: A Case Report and Review of the Benchmarks

Kristian Larson, Atlantis Russ, Hina Arif-Tiwari, Daruka Mahadevan, Alex Elliott, Achyut Bhattacharyya, Hani Babiker

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Malignant peripheral nerve sheath tumors (MPNSTs) represent a rare subtype of neural crest cell-derived soft tissue sarcomas (STS). Standard of care therapy comprises surgical resection followed by adjuvant radiation, and most clinical studies have demonstrated finite survival benefit of radiation and chemotherapy. In metastatic disease, palliative chemotherapy provides very limited efficacy. We report a 60-year-old male patient with a primary para vertebral tumor at T7-T8 with lung metastases who recurred after surgical resection and later progressed on epirubicin plus ifosfamide. He was an international patient and referred to the phase 1 clinic. Molecular profiling and immunohistochemistry of the tumor revealed a PD-L1 expression of 70% (2+) and pathogenic genetic alterations by next-generation sequencing in ARID1A, CDKN2A, KMT2A, NF1, and TP53. Immune checkpoint therapy (ICT) with pembrolizumab was commenced, and interval computed tomography revealed a complete remission by cycle 6. Randomized clinical trials illustrate that ICTs such as anti-PD-1 and anti-CTLA4 monoclonal antibodies in STS cohorts display low or modest response rates by variable PD-L1 expression. This and 3 other case reports of disparate PD-L1 expression demonstrate complete responses in PD-L1 positive MPNSTs treated with ICT. These case reports necessitate further study of ICT in neural crest cell subtype of STS.

Original languageEnglish (US)
Pages (from-to)222-226
Number of pages5
JournalJournal of Immunotherapy
Volume45
Issue number4
DOIs
StatePublished - May 1 2022
Externally publishedYes

Keywords

  • immunotherapy
  • malignant peripheral nerve sheath tumor
  • pembrolizumab
  • soft tissue sarcoma

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Pharmacology
  • Cancer Research

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