Di Tian
Department of Pathology & Laboratory Medicine, Children’s Hospital Los Angeles and Keck School of Me, USA
Publications
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Case Report
Extensive glandular and neuroendocrine differentiation of a malignant peripheral nerve sheath tumor in a neurofibromatosis-1 patient
Author(s): Julie Huss-Bawab, Jennifer Sakioka, Nishant Tiwari, Vernon Tolo, Amit Sura, Ramzi Bawab, Brian Cooper, Shengmei Zhou, Di Tian and Nick Shillingford*
The histologic diagnosis of a malignant peripheral nerve sheath tumor (MPNST) is complex with no defining biomarkers or generally accepted diagnostic criteria, but based on whether it is identified arising from a peripheral nerve, arising from a benign nerve sheath tumor, in the setting of neurofibroma or when its constellation of features suggest a Schwann-cell differentiation [1- 3]. Further complicating the histologic diagnosis is the small subset of these tumors with divergent differentiation demonstrating various heterologous elements [4]. Divergent differentiation presenting in a glandular form in MPNST is particularly rare, though has been described, usually in context of Neurofibromatosis I (NF1) patients, as discrete localized areas occasionally associated with neuroendocrine features [3,5,6]. Being aware of the possible extent of variable histologic features is critical for .. Read More»