Surgery for Peripheral Nerve Sheath Tumours of the Buttocks, Legs and Feet in 90 Patients With Neurofibromatosis Type 1

Friedrich, Reinhard E; Tuzcu, Caglayan T

doi:10.21873/invivo.12329

Cited by 7 publications

(10 citation statements)

References 67 publications

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“…The clustering corresponds to data in the literature in which the surgical treatment results of brachial plexus PNF have been analyzed [ 15 , 21 , 22 ]. The description of cutaneous PNF distribution of the neck corresponds to results for the PNF distribution pattern developing in the extremities [ 23 , 24 ]. In the regions of the trunk and extremities, segmental distribution of tumors across dermatomes and units was obvious [ 23 – 25 ].…”

Section: Discussionmentioning

confidence: 99%

“…The histological distinction is based on the relationship between the axon and the tumorous Schwann cells. In histological definition, the diagnosis “plexiform” has no relationship to tumor size [ 24 ]. In contrast, in the clinical-diagnostic field, the term “plexiform” is predominantly used to describe both large tumors with infiltrative growth patterns (lumpy masses) and nodular PNSTs arising, for example, in the plexus (nodular PNF).…”

Section: Discussionmentioning

confidence: 99%

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Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients

Friedrich

Lohmann

2023

Oral Maxillofac Surg

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Purpose Plexiform neurofibromas (PNF) are rare tumors arising from peripheral nerve sheath cells. PNF are a hallmark in patients with neurofibromatosis type 1 (NF1), a tumor predisposition syndrome. PNF often grow invasively and destructively, what may complicate surgical treatment. Data on frequency, location, and surgical procedures of patients with NF1-associated FPNF are scarce. This study provides treatment data of NF1 patients. Methods Localization and treatment data of 69 NF1 patients with neck PNF were analyzed. Frequency of lesions was recorded in coded colors on schematic neck drawings. Results The tumors showed no side preference, were located in the entire area under investigation, and did not respect anatomical units/dermatomes. However, the sternocleidomastoid region was particularly frequently affected. The mean number of surgical measures per patient was 1.33. Complications were extensive swelling, hematoma, and bleeding. Histological assessment usually confirmed the clinical assessment of neoplasm. However, histologic differentiation of PNST reveals differences in between tumors that have been unified in clinical assessment as PNF. Conclusion The color-coded, schematic overview of the frequency distribution of surgical neck interventions in NF1 patients with PNF proved a useful tool to gain assessment of preferred treatment needs. The imaging procedure may be suitable for controlling the external aspect of natural tumor development (growth, effects of aging) in the same way as the documentation of the post-surgical course. Treatment plans for patients with these tumors should consider that repeated interventions may be necessary to achieve a longer-term stable result.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients

Friedrich

Lohmann

2023

Oral Maxillofac Surg

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“…Несмотря на эффективность применения ингибиторов MEK, данные препараты приводили к уменьшению размеров опухолей, но не к полному их исчезновению. Поэтому хирургическое удаление нейрофибром, особенно ПН, остается единственным способом избавить пациентов от косметических дефектов [22]. Однако для бессимптомных неоперабельных ПН рекомендуется избегать планового хирургического лечения в связи с наличием высоких рисков интраоперационного кровотечения, инвалидизирующих последствий операции и частых рецидивов.…”

Section: консервативная терапия нейрофиброматоза I типаunclassified

“…Анализ оперативного лечения ПН у 90 больных НФ1 показал трансформацию опухолей в MPNST у 2 (2,2 %) из них после удаления. Основной тактикой при наличии MPNST была ампутация пораженной конечности [22]. Исследование 121 ребенка с НФ1 за 20-летний период после удаления ПН показало отсутствие прогрессии в послеоперационном периоде лишь у 54 % пациентов.…”

Section: хирургическое лечение нейрофиброматоза I типаunclassified

“…Частым осложнением при этом является потеря функции пораженного нерва [11]. Эффективность ингибиторов MEK в отношении уменьшения размеров ПН свидетельствует о возможности комбинации применения данных препаратов с хирургическими способами лечения MPNST [22], поскольку для данных опухолей также показана перспективность применения ингибиторов MEK. На линии клеток MPNST (NF1-/-) была показана эффективность селуметиниба в комплексной терапии с LDN-193189 (ингибитор рецептора BMP2 I типа), в то время как изолированное применение LDN-193189 не давало должного антипролиферативного эффекта [10].…”

Section: хирургическое лечение нейрофиброматоза I типаunclassified

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Questions on surgical treatment of neurofibromatosis type 1

Мустафин

2022

Ross. ž. det. gematol. onkol.

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Neurofibromatosis type 1 (NF1) is a hereditary tumor syndrome caused by a mutation in tumor suppressor gene NF1. The disease occurs with a frequency of 1:3000 of the population. Typical manifestations of NF1 are multiple cutaneous, subcutaneous and plexiform neurofibromas, which cause serious cosmetic defects in the appearance of patients. Since an effective method for the treatment of NF1 has not yet been introduced into clinical practice, which makes it possible to completely get rid of multiple neoplasms, surgical removal or the use of photodestruction methods remains the method of choice for the treatment of cutaneous and subcutaneous neurofibromas. However, plexiform neurofibromas infiltratively grow into the surrounding tissues, so their complete excision is often impossible. Therefore, surgical excision is indicated for localized forms of plexiform neurofibromas and in emergency situations when they are located near vital structures or in the presence of a mass effect. Moreover, excision of cutaneous and subcutaneous neurofibromas may initiate formation and growth of new tumors. This is due to the degranulation of mast cells in response to tumor damage, since the pathology of the immune system plays an important role in the development of neurofibromas. In order to avoid dissemination of tumors, it is also important to adhere to the principles of antiblastic surgery. Therefore, the most promising is the complex treatment of NF1 with the combined use of a classical approach, surgical laser, ketotifen, and an inhibitor of mitogen-activated kinase. This method can be recommended for the treatment of malignant peripheral nerve sheath tumors, as well as sporadic neoplasms, the resistance of which is caused by a somatic mutation in the NF1 gene. The goal of NF1 treatment is to improve the quality of life of the patient as a whole and reduce pain using an integrated multidisciplinary approach.

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Neurofibromatosis Type 1-Associated Plexiform Neurofibromas of the Face and Adjacent Head Regions: Topography of Lesions and Surgical Treatment Data of 179 Patients

Friedrich

Modemann

2023

J. Maxillofac. Oral Surg.

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Introduction Facial plexiform neurofibromas (FPNF) are rare tumors frequently diagnosed in patients with neurofibromatosis type 1 (NF1), a tumor predisposition syndrome. FPNF often grows invasively and destructively, which may complicate surgical treatment. Data on the frequency, location, and surgical procedures of patients with NF1-associated FPNF are scarce. This study provides treatment data from a nationally networked reference center for the treatment of NF1 patients. Material and Methods The localization and treatment data of 179 NF1 patients with FPNF were analyzed. Photographically documented tumors of the study area, further determined by imaging, were manually transferred to a facial scheme and digitized. The digitized registrations of the facial extensions of the tumors of each patient were overlaid in a single image (Photoshop™), so that the file of the facial scheme contained the sum of the tumor localization. Finally, the frequency of tumor localization was indicated with a color code. The frequency of tumor extension-related coded colors was applied to outline the lesions' topography on schematic face drawings (heat map). Results The distribution of the tumors showed no side preference. The need for the treatment of patients with orbital/periorbital manifestations became evident in the graphic representations. Tumors do not respect anatomical units. However, the classification of the face according to dermatomes, especially the trigeminal nerve, offers indications of tumor spread and guides treatment planning. The mean number of surgical measures per patient was 2.21 (median: 1). Extensive swelling, hematoma, and delayed wound healing were all common postoperative complications. Conclusion The color-coded, schematic overview of the frequency distribution of cutaneous tumor spread in NF1 patients with FPNF illustrates the importance of orbital/periorbital and cheek tumor manifestations in patients' treatment needs. The imaging procedure is suitable for controlling natural tumor growth in the same way as the documentation of the post-surgical course. Repeated interventions in the region are included in surgical planning of the progressing tumor disease.

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Surgery for Peripheral Nerve Sheath Tumours of the Buttocks, Legs and Feet in 90 Patients With Neurofibromatosis Type 1

Cited by 7 publications

References 67 publications

Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients

Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients

Questions on surgical treatment of neurofibromatosis type 1

Neurofibromatosis Type 1-Associated Plexiform Neurofibromas of the Face and Adjacent Head Regions: Topography of Lesions and Surgical Treatment Data of 179 Patients

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