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[Surgical management of sacral neurogenic tumors].

OBJECTIVE: To observe the efficacy and complications of one-stage tumor resection to treat primary sacral neurogenic tumors and to discuss s … and cerebrospinal fluid leakage in 2 cases. Pathology confirmed 17 cases of schwannoma, 7 cases of neurofibroma and 2 cases of malignant schwannoma. Local recurrence was observed in two cases of benign neurogenic tumors. One patient with a malignant nerve sheath tumor had lung metastasis, who died 20 months after the operation. In 17 cases of upper sacral neu …

Extended length of stay in open versus minimally invasive surgery with robotic-assisted sub-analysis for spinal nerve sheath tumor resection: a nationwide analysis.

Spinal nerve sheath tumors are slow-growing neoplasms that arise from Schwann cell lineage and encompass schwannomas, neurofibromas, hybrid nerve sheath tumors, and malignant peripheral nerve sheath tumors. These lesions most commonly present as intradural extramedullary (IDEM) tumors, although extradural and dumbbell-shaped variants are also observed. Due to their typically benign behavior, gross total resection (GTR) remains the standard of care. However, there is a paucity of literature comparing the impact of open versus minimally invasive surgery (MIS) on postoperative extended length of …

Cutaneous Leiomyoma Mimicking a Keloid.

Dear Editor, Cutaneous leiomyomas (CL) are rare, benign smooth muscle tumors of the skin (1). There are 3 subtypes with different origins an … with diameters varying from several mm to 2 cm (2). Differential diagnosis includes dermatofibroma, neurofibroma, smooth muscle hamartoma, neuroma, adnexal tumors, and painful papulonodular lesions such as glomus tumor (1,2). Our case clinically resembled keloid with red-brown, stiff nodules with epidermal thinning. In the literature, a patient with cutaneous …

NF2-Related Schwannomatosis (NF2): Molecular Insights and Therapeutic Avenues.

NF2-related schwannomatosis (NF2) is a genetic syndrome characterized by the growth of benign tumors in the nervous system, particularly bilateral vestibular schwannomas, meningiomas, and ependymomas. This review consolidates the current knowledge on NF2 syndrome, emphasizing the molecular pathology associated with the mutations in the gene of the same name, the NF2 gene, and the subsequent dysfunction of its product, the Merlin protein. Merlin, a tumor suppressor, integrates multiple signaling pathways that regulate cell contact, proliferation, and motility, thereby influencing tumor growth.

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… On 10 April 2020, Selumetinib was approved by the FDA for the treatment of pediatric patients 2 years of age and older with neurofibromatosis type 1 who have symptomatic, inoperable plexiform neurofibromas. … In the phase 2 trial, most children with neurofibromatosis type 1 and inoperable plexiform neurofibromas had durable tumor shrinkage and clinical benefit from selumetinib [ 90 ] ( NCT01362803 ). …

Activated Ras as a Therapeutic Target: Constraints on Directly Targeting Ras Isoforms and Wild-Type versus Mutated Proteins.

The ability to selectively and directly target activated Ras would provide immense utility for treatment of the numerous cancers that are driven by oncogenic Ras mutations. Patients with disorders driven by overactivated wild-type Ras proteins, such as type 1 neurofibromatosis, might also benefit from progress made in that context. Activated Ras is an extremely challenging direct drug target due to the inherent difficulties in disrupting the protein:protein interactions that underlie its activation and function. Major investments have been made to target Ras through indirect routes. Inhibition…

Targeted therapy in pediatric central nervous system tumors: a review from the National Pediatric Cancer Foundation.

Central nervous system tumors represent the leading cause of cancer-related mortality in children. Conventional therapies of surgery, radiation, and cytotoxic chemotherapy have insufficient efficacy for some pediatric CNS tumors and are associated with significant morbidity, prompting an ongoing need for novel treatment approaches. Identification of molecular alterations driving tumorigenesis has led to a rising interest in developing targeted therapies for these tumors. The present narrative review focuses on recent progress in targeted therapies for pediatric CNS tumors. We outline the key i

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… A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway: TRAM-01 . … A phase 2 study of trametinib for patients with pediatric glioma or plexiform neurofibroma with refractory tumor and activation of the MAPK/ERK pathway . J Clin Oncol . ( 2022 ) 40 : 2042 . doi: 10.1200/JCO.2022.40.16_suppl.2042 PMC6935133 31881853 73 Regad T . …

Rare Diseases of the Nose, the Paranasal Sinuses, and the Anterior Skull Base.

Due to their low incidence and thus resulting limited diagnostic criteria as well as therapeutic options, rare diseases of the nose, the paranasal sinuses, and the anterior skull base are a significant challenge. The value as of which a disease has to be considered as rare amounts to a maximum of 5 patients per 10 000 people. Within these diseases, however, there are extreme differences. Some rare or orphan diseases like for example the inverted papilloma belong to regularly diagnosed and treated diseases of larger departments of oto-rhino-laryngology whereas other rare diseases and malformati

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… Due to the narrow circumstances and the small access, however, it is only possible to a limited extent in cases of schwannomas of the paranasal sinuses. 2.4.1.1.5 Neurofibromas Neurofibromas are benign peripheral nerve sheath tumors of schwann cells, perineural-like cells and intraneural fibroblasts … Neurofibromas have a shiny, fusiform, and sometimes polypoid surface and are solid on palpation 148 149 . Also neurofibromas are unencapsulated tumors that are closely associated with neural branches. …

Skin manifestations associated with systemic diseases - Part II.

The skin, by reflecting internal processes, externalizes what happens inside the body in many diseases. Thus, the skin, as an organ, extrapolates its functions of protection, barrier and signals the existence of systemic diseases, expanding the importance of the dermatologist beyond the skin surface. Thus, the dermatologist investigates diagnostic hypotheses for conditions related to all systems and refers patients to the appropriate specialty. Combined with examination by a trained eye, the skin, due to its easy access, is still the ideal place for performing biopsies, which often clarify the

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… NAME stands for: nevi, atrial myxoma, myxoid neurofibroma, and ephelides. … + plexiform neuromas | Malignant degeneration of neurofibroma + astrocytoma + glioblastoma + meningioma + bilateral pheochromocytomas Pruritus It is defined as an uncomfortable feeling that triggers the urge to scratch. 40 It can be triggered directly on the skin by mechanical or thermal stimuli or …

Многоцелевые лекарственные препараты: новые 2025 и послерыночного контроля за безопасностью наркотиков, 2022-2024.

Полифармакология предназначена для развития соединений, действующих по крайней мере на two целевые показатели (многоцелевые лиганды, МТДЛ). В 2025, Европейское агентство по медицине (ЕМА), 38 наркотиков, и 11 из них были MTDL. Большинство из них - антитела-наркотики, биконкретные антитела или ингибиторы киназы, все из которых указаны для лечения опухолей, включая датопотамаб дерукстекан (гормон-позитивный рецептор, HER2-negative Рак молочной железы), тизотумаб ведотин (сокращенная шейная карцинома), линвосельтамб (четвёртая линия лечения множественной миеломы) и эрдафитиниб (сокращенная уротел

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… Mirdametinib (Ezmekly ® ) L01EE05 kinase inhibitor (-) MEK1 (-) MEK2 symptomatic, inoperable plexiform neurofibromas in patients with neurofibromatosis type 1, aged ≥ 2 years [ 39 , 40 ] 2 + nq 8. … Neurofibromatosis type 1, caused by mutations in the NF1 gene, is an autosomal-dominant condition and results in the formation of plexiform neurofibromas. …

Assessment of New Molecular Entities Approved for Cancer Treatment in 2020.

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… govitecan-hziy | Metastatic triple-negative breast cancer | Accelerated | Tumor shrinkage in 108 patients | 33 | 7.7 mo median Selpercatinib | RET fusion lung and thyroid cancers | Accelerated | Tumor shrinkage in 314 patients (3 trials) | 72 | 76%-87% response rate lasting >6 mo Selumetinib | plexiform neurofibromas …

Risk Factors Analysis of Phantom Limb Pain in Amputees with Malignant Tumors.

PurposePostamputation neuropathic pain is a common disease in patients with malignant tumor amputation, seriously affecting amputees' quality of life and mental health. The objective of this study was to identify independent risk factors for phantom limb pain in patients with tumor amputation and to construct a risk prediction model.MethodsPatients who underwent amputation due to malignant tumors from 2013 to 2023 were retrospectively analyzed and divided into phantom limb pain group and non-phantom limb pain group. To determine which preoperative factors would affect the occurrence of phantom

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… Oseteosarcoma(OS) | 77(35.8) Ewing sarcoma(ES) | 7(3.3) Chondrosarcoma(CS) | 13(6.0) Soft tissue sarcoma(STS) | 78(36.3) Malignant melanoma(MAL) | 2(0.9) Squmous cell carcinoma(SCC) | 8(3.7) Malignant fibrous histiocytoma(MFH) | 11(5.1) Adamantinoma(AT) | 1(0.5) PENT | 1(0.5) OMGCT | 8(3.7) Malignant neurofibroma … 1.4) | – | >0.05 Squmous cell carcinoma | 1(1.0) | 5(7.0) | 0.149 (0.016–1.349) | 0.09 Malignant fibrous histiocytoma | 6(6.2) | 1(1.4) | 4.457 (0.505–39.310) | 0.178 adamantinoma | 0 | 1(1.4) | – | >0.05 PENT | 1(1.0) | 0 | – | >0.05 OMGCT | 5(5.2) | 2(2.8) | 1.857 (0.334–10.335) | 0.048 Malignant neurofibroma …

A year in pharmacology: new drugs approved by the US food and drug administration in 2025.

The US Food and Drug Administration approved 45 new drugs and 4 new cellular and gene therapy products in 2025, that is, a total of 49 new medical therapies. Oncology, hematology/immunotherapy, and cardiovascular/respiratory drugs remain most frequent. Among oncology drugs, there is a trend for targeting ever smaller subtypes of malignancies and/or rarer malignancies. Trends over the past 6 years include steady levels of first-in-indication (about 10%), first-in-class (about 38%), and next-in-class (about 51%) approvals. Along with a large share of approvals in orphan indications (> 50%), fast

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… Selumetinib, the first MEK inhibitor approved by the FDA in 2020, is indicated for the diagnosis of inoperable plexiform neurofibromas in children with neurofibromatosis type 1 (Saara and Rani 2025 ). … Pain Ther 14:655–674. 10.1007/s40122-024-00697-0 Oztosun G, Armstrong A, Hirbe AC (2025) Mirdametinib, an FDA-approved MEK1/2 inhibitor for adult and pediatric NF1-associated plexiform neurofibromas. …

Targeted therapies in pediatric oncology: A start.

The treatment of cancers was revolutionized by the use of combination "conventional" chemotherapy, first in children, during the 20th century. That foundation of therapy, while having achieved significant advances in survival, has reached its limits. Innovations in targeted therapies are now building upon that foundation to further improve outcomes in pediatric oncology. These targeted agents are designed to narrowly utilize specific biologic processes for greater efficacy and optimally fewer generalized toxicities. I will provide an overview of the current spectrum of targeted therapies avail

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… Downstream of the pathogenic mutation in the NF1 gene, MEK acts as a critical node in the development of plexiform neurofibromas (PNs), noncancerous growths that can have significant mass effect and morbidity. … in patient with NF1, ≥2 y/o Moertel et al. 260 Selumetinib dual-specificity kinase inhibitor MEK symptomatic inoperable plexiform neurofibroma in patients with NF1, ≥1 year/o Chen et al. 258 ; Gross et al. 259 Everolimus Cis -trans prolyl isomerase inhibitor mTORC1 complex (via FKBP1A) unresectable …

Primary and postoperative radiotherapy in acute neurological symptoms due to malignant spinal compression: retrospective analysis from a German university hospital.

Malignant spinal cord compression (MSCC) can lead to immediate neurological impairment. In order to preserve and, optimally, restore neurological functions, urgent treatment (usually, within 24 h) is necessary. Treatment options mainly consist of decompressive surgery (DS) and / or radiotherapy (RT) combined with steroids. Whereas historically, RT was the treatment of choice, DS has become standard of care, where applicable. Despite a variety of excellent studies, real world data of treatment in a large academic center is currently underrepresented. We performed a retrospective analysis of pat

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… fibrous histiocytoma | 1 (0.7) | Low Langerhans cell histiocytosis | 1 (0.7) | Intermediate Bladder Carcinoma | 1 (0.7) | Low Glioblastoma | 1 (0.7) | Low Adenocarcinoma of the tear gland | 1 (0.7) | Intermediate Esophageal Carcinoma | 1 (0.7) | Intermediate Undifferentiated Sarcoma | 1 (0.7) | Low Neurofibroma … Only two patients had an intended dose above 40 Gy: one patient suffering from spinal cord compression by a dedifferentiated adenocarcinoma of the lung, one suffering from neurofibroma; each being described 28*1.8 Gy. …

Acceptance and commitment therapy reduces psychological distress in patients with cancer: a systematic review and meta-analysis of randomized controlled trials.

ObjectiveThis study aimed to systematically review and meta-analyze the clinical efficacy of acceptance and commitment therapy (ACT) in patients with cancer and psychological distress.MethodsRandomized controlled trials (RCTs) from seven English electronic databases were systematically investigated from inception to 3 October 2023. A total of 16 RCTs from 6 countries with 711 participants were included in this study. Estimated pooled effect sizes (ESs) were calculated via inverse-variance random-effects or fixed-effects (I2 ≤ 50%) model and presented by standardized mean difference (SMD). Subg

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… Ability to understand and the willingness to sign a written informed consent document. (7) No anticipated major changes in their pain treatment regimen or enrollment in a new treatment study presumed to impact pain soon. (8) Comprehension of the English language. | Neurofibromatosis type 1/ Plexiform Neurofibromas … BMJ 372 : n71 . doi: 10.1136/bmj.n71 , PMID: 33782057 PMC8005924 Peron S. ( 2022 ). " Acceptance and commitment training for adolescents and young adults with Neurofibromatosis type 1, plexiform Neurofibromas, and chronic pain ". …

Unusual Prostate-Specific Membrane Antigen (PSMA) Splenic Uptake in a Patient With Prostate Cancer.

There are many benefits to early detection of prostate cancer, including improved survival rates, less invasive treatment options, and better quality of life. Apart from traditional tests and imaging (including the prostate-specific antigen (PSA) test, digital rectal exam, biopsy, computed tomography (CT), and magnetic resonance imaging (MRI)), an imaging technique called prostate-specific membrane antigen positron emission tomography (PSMA PET) is more specific and sensitive, targeting PSMA expressed by prostate cancer cells on their surface. This case report describes a 62-year-old male with

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… Published case reports show PSMA uptake in inflammatory and infectious diseases such as neurocysticercosis, tuberculosis, sarcoidosis, osteomyelitis, and nodular fasciitis; benign tumors such as meningioma, schwannoma, neurofibromas, thyroid and parathyroid adenomas, angiolipoma, hemangiomas, thymomas …

Quality of life in cancer patients receiving palliative care.

BackgroundThe main focus of palliative care services is to improve the patient's quality of life (QOL), which is defined as the subjective evaluation of life as a whole or the patient's appraisal and satisfaction with their current level of functioning compared with what they perceive to be possible or ideal.AimsIn this prospective study we attempt to validate the Hindi version of a questionnaire designed by the functional assessment of chronic illness therapy (FACIT) measurement system; to measure the subjective QOL of cancer patients receiving home-based palliative care, determine ease of us

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… Table 2 Types of cancers Ca breast | 20 | Ca alveolus | 2 | Malignant melanoma | 2 Ca cervix | 12 | Ca colon | 2 | Neurofibroma | 2 Ca esophagus | 7 | Ca ovary | 2 | Non hodgkin’s lymphoma | 2 Ca buccal mucosa | 7 | Ca prostate | 2 | Ca gall bladder | 1 Ca lung | 7 | Ca thyroid | 2 | Ca para-nasal sinus …

Posttraumatic Vernet syndrome without fracture: A case report and short literature review.

RATIONALE: The aim of this case is to emphasize the need to include nerve traction in the differential diagnosis of nerve deficits associated with Vernet syndrome. This mechanism of injury has been described only once, but must not be overlooked and should be considered and included as a possible cause in diagnostic algorithms. PATIENT CONCERNS: A patient presenting with dysphagia, extreme hoarseness, and limited shoulder movement after head injury was admitted to the emergency department. DIAGNOSES: Multidisciplinary evaluation was performed, and nerve traction-induced Vernet syndrome was e

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… Prevalent primary tumors that metastasize to the jugular foramen are breast, lung, kidney, and prostate cancer, as well as melanoma. [ 12 , 13 ] Benign tumors that can lead to Vernet syndrome, include paraganglioma, lower cranial nerve schwannoma, meningioma, aneurysmal bone cyst, neurofibroma, and temporal …

Сердечно-сосудистые заболевания

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… Neurofibroma, although uncommon, frequently causes arrhythmia and variable intensity of heart sound in affected cattle and bulls. Further the cardiac arrhythmia may coexist with paresis or paralysis caused by neurofibroma masses in the spinal canal. … Although perhaps a moot point because both diseases are fatal, further medical workup of neurofibroma patients fails to provide confirmation of lymphosarcoma. To date, postmortem examination has been the only means of definitive diagnosis for cardiac neurofibroma. …

Chemistry and Pharmacology of Fluorinated Drugs Approved by the FDA (2016-2022).

Fluorine is characterized by high electronegativity and small atomic size, which provide this molecule with the unique property of augmenting the potency, selectivity, metabolic stability, and pharmacokinetics of drugs. Fluorine (F) substitution has been extensively explored in drug research as a means of improving biological activity and enhancing chemical or metabolic stability. Selective F substitution onto a therapeutic or diagnostic drug candidate can enhance several pharmacokinetic and physicochemical properties such as metabolic stability and membrane permeation. The increased binding a

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… Selumetinib is used to treat pediatric patients with neurofibromatosis type 1 who have symptomatic, inoperable plexiform neurofibromas. With an approximate population of 1/3000 people, NF-1 is regarded as rare. … | Hypoxia-Inducible Factor-2α 22 | KRAS | Kirsten Rat Sarcoma Virus 23 | KRAS | Kirsten Rat Sarcoma Viral Oncogene Homolog 24 | LAT1, LAT3 | L-type Amino Acid Transporters 25 | MAO | Monoamine Oxidase 26 | MEK | Mitogen-activated Protein Kinase 27 | MET | Mesenchymal Epithelial Transition 28 | NF | Neurofibromas …