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Background: Nodular plexiform neurofibromas in individuals with neurofibromatosis type 1 often cause significant symptoms and are treated with surgical excision despite the potential risk of complications. This study aimed to clarify the surgical outcomes of deep-seated nodular plexiform neurofibromas and identify the factors associated with postoperative complications.
Methods: We retrospectively reviewed patients with neurofibromatosis type 1 who underwent surgical excision for deep-seated nodular plexiform neurofibromas in our hospital from 2015 to 2021. Enucleation while preserving the nerve fascicles was attempted first, and en bloc resection, ligating the nerve origin in cases in which the parent nerve was entrapped by the tumor, making the tumor difficult to dissect, was performed.
Results: In 15 patients, 24 nodular plexiform neurofibromas received surgical excision. Sixteen tumors were enucleated, and eight were en bloc resected. The symptoms of all 10 patients with preoperative symptoms resolved after surgery. Four patients developed new neurological deficits immediately after surgery, two of whom had retained neurological symptoms at the last visit, but these symptoms were mild.
Conclusions: The present study demonstrates that surgical treatment of nodular plexiform neurofibromas, even deep-seated neurofibromas, is safe with a low risk of severe complications and improvement in preoperative symptoms.
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http://dx.doi.org/10.3390/jcm11195695 | DOI Listing |
Cancer Diagn Progn
September 2025
Institute of Neuropathology, Eppendorf University Hospital, University of Hamburg, Hamburg, Germany.
Background/aim: Neurofibromatosis type 1 (NF1) is a genetic disorder characterized by the development of multiple tumors, including plexiform neurofibromas (PNFs), which often affect the craniofacial region and cause significant functional and aesthetic impairments. This report presents long-term surgical management of a patient with hemifacial diffuse PNF, complicated by the emergence of a rapidly enlarging midfacial tumor.
Case Report: The patient was treated for hemifacial invasive diffuse plexiform neurofibroma.
Cureus
July 2025
Department of General Surgery, ESIC Medical College and Hospital, K.K. Nagar, Chennai, IND.
A 59-year-old female presented to the hospital with acute abdominal pain. She reported a six-month history of melena, early satiety, postprandial fullness, unintentional weight loss, and loss of appetite. Contrast-enhanced CT of the abdomen revealed an exophytic mass in the antropyloric region, measuring approximately 4.
View Article and Find Full Text PDFAm J Dermatopathol
April 2025
Pathology Section, Dubai Hospital, Dubai, United Arab Emirates .
Several cutaneous neurovascular stromal lesions are clinically and pathologically ill-defined entities. They are known by different nomenclatures, often unrecognized, misinterpreted, and confused with other skin lesions. Reports have documented cases of palmar and oral lesions in both children and adults.
View Article and Find Full Text PDFAnn Diagn Pathol
April 2025
Department of Pathology, Hacettepe University Faculty of Medicine, Ankara 06230, Turkey. Electronic address:
Malignant peripheral nerve sheath tumor (MPNST) comprises 5-10 % of all soft tissue sarcomas, and their diagnosis may be challenging given the absence of robust immunohistochemical and molecular signatures. SOX11 expression has previously been shown to be present in a small subset of MPNST. In the present study, we evaluated a group of MPNST for SOX11 expression by immunohistochemistry.
View Article and Find Full Text PDFChirurgie (Heidelb)
May 2025
Universitätsklinik für Plastische, Rekonstruktive und Ästhetische Chirurgie, Medizinische Universität Wien, Wien, Österreich.
Neurofibromatosis type 1 (NF1, formerly Recklinghausen's disease) is a genetic tumor predisposition syndrome in which the mutation of a tumor suppressor gene (neurofibromin) leads to the development of mostly benign neurofibromas of the skin and the central and peripheral nervous systems and malformations or tumors of other organ systems. Patients with NF1 should receive lifelong interdisciplinary care in specialized centers and important treatment decisions should be made by a regularly meeting interdisciplinary panel of experts. Plastic surgery plays an important role in the multidisciplinary management of all clinical forms of NF1-associated peripheral nerve sheath tumors, from cutaneous and subcutaneous to deep nodular and diffuse plexiform neurofibromas.
View Article and Find Full Text PDF