Malignant peripheral nerve sheath tumor (MPNST) is an aggressive soft tissue sarcoma with limited therapeutic options and a poor prognosis. Although neurofibromatosis type 1 (NF1) and radiation exposure have been identified as risk factors for MPNST, the genetic and molecular mechanisms underlying MPNST pathogenesis have only lately been roughly elucidated. Plexiform neurofibroma (PN) and atypical neurofibromatous neoplasm of unknown biological potential (ANNUBP) are novel concepts of MPNST precancerous lesions, which revealed sequential mutations in MPNST development. This review summarized the current understanding of MPNST and the latest consensus from its diagnosis to treatment, with highlights on molecular biomarkers and targeted therapies. Additionally, we discussed the current challenges and prospects for MPNST management.
Cancer represents a major cause of death worldwide and is characterized by the uncontrolled proliferation of abnormal cells that escape immune regulation. It is now understood that cancer-associated fibroblasts (CAFs), which express specific fibroblast activation protein (FAP), are critical participants in tumor development and metastasis. Researchers have developed various FAP-targeted probes for imaging of different tumors from antibodies to boronic acid-based inhibitor molecules and determined that quinoline-based FAP inhibitors (FAPIs) are the most appropriate candidate as the radiopharmaceutical for FAPI PET/CT imaging. When applied clinically, FAPI PET/CT yielded satisfactory results. Over the past few years, the utility and effectiveness of tumor detection and staging of FAPI PET/CT have been compared with FDG PET/CT in various aspects, including standardized uptake values (SUVs), rate of absorbance and clearance. This review summarizes the development and clinical application of FAPI PET/CT, emphasizing the diagnosis and management of various tumor types and the future prospects of FAPI imaging.
Peripheral nerve diseases are significantly correlated with severe fractures or trauma and surgeries, leading to poor life quality and impairment of physical and mental health. Human dental pulp stem cells (DPSCs) are neural crest stem cells with a strong multi-directional differentiation potential and proliferation capacity that provide a novel cell source for nerve regeneration. DPSCs are easily extracted from dental pulp tissue of human permanent or deciduous teeth. DPSCs can express neurotrophic and immunomodulatory factors and, subsequently, induce blood vessel formation and nerve regeneration. Therefore, DPSCs yield valuable therapeutic potential in the management of peripheral neuropathies. With the purpose of summarizing the advances in DPSCs and their potential applications in peripheral neuropathies, this article reviews the biological characteristics of DPSCs in association with the mechanisms of peripheral nerve regeneration.
IntroductionSchwannomas are the most common neoplastic lesions of the peripheral nerves when growing on the extremities, they usually have adverse effects on patients due to the exposed and functional nature of the region.MethodsIn the present single-center retrospective study, we included all patients with pathologically confirmed schwannoma located in extremities between 2011 and 2021 totaling 183 patients. Data on gender, age, duration history, clinical presentation, occurrence region, nerve affiliation, imaging data, modus operation, mass volume, immunohistochemistry, postoperative neurological function, and recurrence were collected.ResultsAs in previous studies, patients were predominantly middle-aged with a mean age of 49.5, without gender preference and a male-to-female ratio of 1.2:1. Most patients are first seen for this disease, and only five of them are recurrent. The majority presented with an isolated (91.26%), asymptomatic (37.7%) mass, with tenderness (34.97%) being the second frequent complaint. 60% of lesions occurred in the upper extremity, more commonly on the left side (55.26%) than the right. The average duration of onset was 47.50 months. MRI is more sensitive for neurogenic tumors than ultrasound, as it owns 78.93% correct. In immunohistochemistry, the top three markers for positive labeling schwannoma are S-100 (98.95%), Ki67 (98.68%) and β-Catenin. 98.36% of patients underwent complete resection of the lesion, of which 14.44% required partial sacrifice of the nerve fibers. Thanks to the application of intraoperative peripheral nerve microscopic operation, only 6 patients showed symptoms of postoperative nerve injury, and 3 of them received second surgery. Intraoperative microscopic manipulation, preservation of the main nerve, and the need for reconstruction of the affected nerve fibers are some of the points worth noting.DiscussionIn summary, the possibility of schwannoma should not be overlooked in the identification of masses that occur in the upper extremities of the middle-aged population. Preoperative ultrasound and MR are useful for determining the nature of the mass, and S100, Ki67, and β-Catenin are sensitive to it. Surgical resection can achieve satisfying functional results and a low risk of nerve injury.
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