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Background: Intracranial schwannomas are benign lesions, expected to exhibit characteristic ultrastructural features and immunophenotypic profile, typically being S-100, SOX10 and collagen-IV positive, while other markers, including CD34, are not usually expressed and are employed in the diagnostic elimination process. Case presentation: A 51-year-old female presented with a rapidly deteriorating neurological status. MRI showed a heterogeneous enhancing neoplasm of the anterior fossa. Preoperative differential diagnosis predilected an esthesioneuroblastoma. A bifrontal craniotomy with total excision of the lesion was performed. Dural infiltration was present. There was no association with the olfactory system. Microscopic examination revealed a uniform population of spindled to slightly epithelioid cellular proliferation with no significant atypia, low mitotic activity, and foci of necrosis. Upon immunohistochemical investigation the tumor was consistent with cellular schwannoma. Diffuse positivity for CD34 was an unusual finding. Discussion: Cellular schwannoma should be considered in the presence of an anterior fossa tumefaction. Olfactory nerve is not directly associated with these lesions. Various theories have been proposed, with the meningeal trigeminal branches' origin being the most probable. The characteristic ultrastructural Antoni formations may be scarce or even absent in cellular schwannomas creating potential diagnostic difficulties. Unusual diffuse CD34 positivity should not be employed for excluding the diagnosis. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to investigate the combined use of ultrasonography and clinical features for the differentiation of malignant peripheral nerve sheath tumors (MPNST) from benign peripheral nerve sheath tumors (BPNST) and to compare the efficacy of ultrasonography with that of magnetic resonance imaging (MRI). Methods: This retrospective study included 28 MPNSTs and a control group of 57 BPNSTs. All patients underwent an ultrasound scan using the Logiq E9 (GE Health Care, Milwaukee, WI) or EPIQ7 equipment (Philips Medical System, Bothell, WA). A 3.0‐T MRI machine (Ingenia; Philips Healthcare, Best, the Netherlands) was used for scanning, and conventional MRI was performed on different regions based on the patient's clinical situation. The following variables were evaluated: palpable mass, pain, nerve symptoms, maximum diameter, location, shape, boundary, encapsulation, echogenicity, echo homogeneity, presence of a cystic component, calcification, target sign, posterior echo, and intertumoral vascularity of the tumors. The diagnostic efficacy of ultrasonography and clinical factors was compared with that of MRI. Independent factors for predicting MPNST versus BPNST were also assessed. Results: The parameters of location, shape, boundary, encapsulation, and vascularity were significantly different between MPNSTs and BPNSTs. Multiple logistic regression analysis showed that shape, boundary, and vascularity were independent predictors of MPNSTs. The sensitivity, specificity, and Youden index of the three clinical and ultrasound factors (shape, boundary, and vascularity) were 0.89, 0.81, and 0.69, respectively, whereas those of MRI were 0.71, 0.89, and 0.61, respectively. No significant differences in the area under the curve (AUC) of the three combined clinical and ultrasound factors and those of MRI were found (P >.05). Conclusions: MRI was useful in the differential diagnosis between MPNSTs and BPNSTs. However, the combination of clinical and ultrasound diagnoses can achieve the same effect as MRI, including shape, boundary, and vasculature. [ABSTRACT FROM AUTHOR]

Aim and background: To demonstrate the clinical findings and management of a retroperitoneal Schwannoma, which presented as a gynecological mass. Nerve sheath tumors are benign tumors affecting the cranial and peripheral nerves. Schwannoma refers to tumors arising from the Schwann cells of neurons. Case description: A 56-year-old female presented to us with vague lower abdominal pain since 3 months. It was initially diagnosed as a inclusion cyst. Intraoperatively diagnosed with retroperitoneal benign looking mass, which turned out to be a schwannoma. We present a case of leftsided retroperitoneal schwannoma which masqueraded as a gynecological mass and operated by laparoscopy. Conclusion: Schwannomas are benign, encapsulated, slow-growing nerve sheath tumors. Rarely seen in the pelvis and retroperitoneum. Laparoscopy aids in better visualization with more accurate surgical removal of these masses and can be considered the treatment of choice for these tumors. Clinical significance: Often asymptomatic and may go unnoticed, but when presenting with symptoms or when discovered incidentally on scans for other reasons, they are mistaken for gynecological or urological masses and are more often presented to gynecologist/urologist. [ABSTRACT FROM AUTHOR]

Objective: Spinal intradural tumors are commonly removed using laminectomy or laminotomy; however, these approaches can lead to postoperative pain, discomfort, kyphosis, and instability. Unilateral hemilaminectomy (UHL) is a minimally invasive alternative that may minimize these issues by using a smaller incision and less disruption to spinal structures. Materials and Methods: From 2010 to 2021, 79 patients with schwannoma underwent UHL surgery. Surgery was generally limited to three levels, and the tumor was removed en bloc or piecemeal after ultrasonic debulking. Paramedial opening of the dura was performed, and neurophysiological monitoring was performed. Baseline medical data on demographic and clinical variables were analyzed, including patients' sex, age, tumor location and volume, overall operative time, length of postoperative immobilization, duration of hospitalization, preoperative (1-month/3-month postoperative Denis Pain Scale value), comorbidities, and modified McCormick Scale. Results: Of these patients, 35 were female and 44 were male, with ages ranging from 19 to 72 years (mean 48.9±14.6). Pain and radiculopathy were the first symptoms in 56 (70.8%) and 36 (45.5%) patients, respectively. No spinal instability was observed postoperatively, and the mean estimated blood loss was 285 mL, ranging from 245-420 mL. The maximal sagittal diameter of the tumor was measured on contrastenhanced T1-weighted images, with a mean of 26.0±12.4 mm (range, 8.2-36.2 mm). Conclusion: Unilateral microsurgery for spinal intradural tumor removal has resulted in good neurologic and oncologic outcomes and reduced postoperative pain and discomfort. In addition, this approach has preserved spinal stability, eliminating the need for bracing, and enabling earlier rehabilitation. [ABSTRACT FROM AUTHOR]

Background: Diabetes mellitus (DM) in children and adolescents is typically caused by type 1 DM, followed by type 2 DM and maturity‐onset diabetes of the young (MODY). We report an unusual Asian Indian family in which three members presented with DM at ages 15, 20, and 30, but not fitting the typical clinical picture of type 1 DM, type 2 DM, or MODY. The primary objective was to elucidate the molecular genetic basis of DM in this family. Methods: The proband, a 22‐year‐old man, had short stature, gray hair, osteoporosis, and markedly reduced subcutaneous fat on the body, especially on the extremities along with acanthosis nigricans, and developed myxoid malignant peripheral nerve sheath tumor. Detailed family history revealed multiple loops of consanguinity. The proband underwent whole‐genome sequencing, and seven relatives underwent whole‐exome sequencing. Results: The proband and three additional family members were found to have the homozygous c.561A>G nucleotide variant of WRN RecQ‐like helicase (WRN) gene consistent with the diagnosis of Werner's syndrome. The c.561A>G variant induces a new splicing site on exon 6 resulting in a truncated WRN protein, p.Lys187Trpfs*13. Conclusion: Our report brings to attention the onset of DM during childhood or early adulthood in patients with Werner's syndrome who typically develop type 2 DM around the age of 30–40 years. Presence of consanguinity among parents, dysmorphic features, and malignancy should prompt consideration of diagnosis of Werner's syndrome. [ABSTRACT FROM AUTHOR]

Schwannomas synonymously known as neurilemmomas or neurinomas are uncommon benign neoplasms of neural sheath origin. They account for 5% of all benign soft-tissue tumors. 6%-8% of tumors occur intracranially and 25%-45% occur extracranially in the head-and-neck region, of which cutaneous schwannomas located in the dermis or subcutaneous tissue in the head and neck are an unusual entity. Of the seven subtypes described by Erlandson, ancient schwannoma is a very rare distinctive histopathological variant resulting from long-term degenerative changes and accounts for 0.8% of all soft-tissue tumors. This seminal article is a systematic review of schwannomas in the ear in its entirety from 1977 to 2023 along with a case report of a profusely bleeding cutaneous ancient schwannoma occurring in the mastoid region in a swelling of a short duration of 6 months in a 75 year old female. To the best of our knowledge, this case has two firsts to its credit. It is the first reported case of an ancient schwannoma in the mastoid region and also the first reported case presenting with bleeding from the schwannoma. Due to its rarity, ancient schwannoma must be included in the differential diagnosis for any bleeding mass in the ear to avoid an erroneous diagnosis of malignancy. [ABSTRACT FROM AUTHOR]

Introduction: Granular cell tumors (GCTs) are uncommon, low-grade Schwann cell tumors found in the skin, soft tissue, and mucosal surfaces of the oral, gastrointestinal, and respiratory tracts. One in 1,000 breast cancer cases is GCT. Just 1–2% of GCTs are malignant GCTs. Case Presentation: This case report presents the clinical details and outcomes of a 34-year-old woman with a main concern of a palpable mass and pain in her right breast. In the clinical examination, we found a 1.5 × 2-cm palpable mass in her right breast with no axillary lymph node detection. The primary diagnosis was a benign GCT mimicking carcinoma of the breast. Upon evaluation, the mass was confirmed to be a benign GCT through pathology. The patient underwent breast-conserving surgery and sentinel lymph node dissection at the Cancer Research Center of Shahid Beheshti University of Medical Sciences on November 30, 2022. The surgical margins were found to be free of tumors, and there was no involvement of skin or axillary lymph nodes. The patient had a positive postoperative outcome, with no complications observed. Conclusion: The case highlights the importance of accurate diagnosis and appropriate surgical planning to avoid invasive procedures and unnecessary radical surgeries in cases of benign GCT mimicking carcinoma of the breast. [ABSTRACT FROM AUTHOR]

Schwannomas are rare slow-growing benign tumors arising from Schwann cells lining the nerve sheaths. Head and neck schwannomas account for about one-third of all cases, and only 4% of them arise from the sinonasal tract. Its diagnosis is based on histology and immunohistochemistry. Complete surgical excision is the most recommended treatment option, and endoscopic surgery has been widely performed in recent years. In this study, we presented a case of a 55-year-old female with schwannoma arising from the lateral wall of the nasal cavity, causing epistaxis and rhinorrhea. The patient underwent endoscopic excision with prompt resolution of symptoms. The reported cases of nasal cavity schwannoma were reviewed and summarized for educational purposes. [ABSTRACT FROM AUTHOR]

Greater superficial petrosal nerve (GSPN) schwannomas are an exceedingly rare nerve sheath tumor. The current literature search was conducted using Medline and Embase database by key search terms. Only 31 cases have been reported in the literature so far. Facial palsy, hearing loss, and xerophthalmia accounted for 48.4% (15), 41.9% (13), and 29% (9) of all cases, respectively. The middle cranial fossa approach was used in all previous reports. A retrospective review of 2 GSPN schwannomas patients treated by endoscopic endonasal approach (EEA) in our center was collected. Clinical records, including clinical features, pre- and postoperative images, surgery, and follow-up information, were reviewed. In all cases, clinical features including facial numbness and headache were found, with tinnitus in case 1, hearing loss, xerophthalmia in case 2. Imaging studies showed a solid mass that originated in the anterior of the petrous bone. Two patients were treated by EEA. Furthermore, no recurrence was found during the follow-up period (15-29 months) in both of the 2 cases after the operation. Complete resection of GSPN schwannomas can be achieved via the pure EEA. Endoscopic endonasal approach for radical removal of tumors is safe and feasible. [ABSTRACT FROM AUTHOR]

This document is a test answer from the journal Skeletal Radiology. It discusses the case of an incidental hip lesion found on PET-CT imaging. The MRI images show a lesion at the proximal right femur, which was isointense to muscle on protein density (PD) and hyperintense to muscle on PD fat-saturated (FS) images. The lesion had a maximum standardized uptake value (SUVmax) of 7.8 on PET-CT. A tissue core biopsy was performed, and the histological examination revealed a perineurioma. Perineuriomas are rare benign peripheral nerve sheath tumors. They are generally hypodense on CT and have nonspecific MRI signal characteristics. Perineuriomas are non-encapsulated, well-demarcated, spindle cell tumors that express antigens matching those of the normal perineurium. They usually demonstrate low FDG uptake on PET-CT. [Extracted from the article]

Schwannoma is a benign, slow-growing peripheral nerve sheath tumor that originates from Schwann cells. Orbital schwannoma is relatively rare, accounting for only 1% of all orbital neoplasms. Intraconal orbital schwannoma (within the muscle cone) is rarer still. We present a case of 68-year-old male with painless, slow-growing mass with protrusion of the right eye. The mass was excised after lateral orbitotomy and tissue diagnosis was made on histopathological examination. [ABSTRACT FROM AUTHOR]

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The article presents a case study of a 66-year-old male with an unusual eccrine poroma on the thigh, diagnosed through histopathological examination. Topics discussed include the diagnostic challenges due to its varied presentations, the differential diagnosis of similar lesions, and the treatment options for managing symptomatic or atypical cases.

A woman in her 60s with anemia was diagnosed with a small intestinal intussusception on computed tomography. She underwent a double‐balloon endoscopy, which revealed submucosal tumor in the ileum. Suspected to be the cause of anemia and intussusception, surgical intervention was carried out, revealing it to be a schwannoma. Schwannomas of the small intestine are very rare, and because exophytic growths are common, intussusception due to luminal side development is even rarer. [ABSTRACT FROM AUTHOR]

Background Although rare in small vestibular schwannomas, preoperative facial nerve paresis is often present in giant schwannomas. Preserving facial nerve function in these cases remains a herculean task. This study evaluates the facial functions after complete tumor removal and whether preoperative facial nerve involvement affects postoperative functional status.

Purpose: This study sought to investigate the role of radiotherapy (RT) in addition to surgery for oncological outcomes in patients with malignant peripheral nerve sheath tumors (MPNST). Methods: In this single-center, retrospective cohort study, histopathologically confirmed MPNST were analyzed. Local control (LC), overall survival (OS), and distant metastasis-free survival (DMFS) were assessed using the Kaplan–Meier estimator. Multivariable Cox regression analysis was performed to identify factors associated with LC, OS, and DMFS. Results: We included 57 patients with a median follow-up of 20.0 months. Most MPNSTs were located deeply (87.5%), were larger than 5 cm (55.8%), and had high-grade histology (78.7%). Seventeen patients received surgery only, and 25 patients received surgery and pre- or postoperative RT. Median LC, OS, and DMFS after surgery only were 8.7, 25.5, and 22.0 months; after surgery with RT, the median LC was not reached, while the median OS and DMFS were 111.5 and 69.9 months. Multivariable Cox regression of LC revealed a negative influence of patients presenting with local disease recurrence compared to patients presenting with an initial primary diagnosis of localized MPNST (hazard ratio: 8.86, p = 0.003). Conclusions: The addition of RT to wide surgical excision appears to have a beneficial effect on LC. Local disease recurrence at presentation is an adverse prognostic factor for developing subsequent local recurrences. Future clinical and translational studies are warranted to identify molecular targets and find effective perioperative combination therapies with RT to improve patient outcomes. [ABSTRACT FROM AUTHOR]

The objective of this study is to preliminarily investigate the surgical safety, efficacy, techniques, and clinical value of fully neuroendoscopic surgery for the resection of cerebellopontine angle (CPA) tumors via a retrosigmoid approach. The clinical data of 47 cerebellopontine angle area (CPA) tumors that were treated by full neuroendoscopic surgery from June 2014 to June 2023 were retrospectively analyzed. The efficacy and advantages of the surgical techniques were evaluated based on indicators such as duration of the surgery, neuroendoscopic techniques, intraoperative integrity of nerves and blood vessels, extent of tumor resection, outcomes or postoperative symptoms, and incidence of complications. The 47 cases of cerebellopontine angle tumors include 34 cases of epidermoid cysts, 7 cases of vestibular schwannomas, and 6 cases of meningiomas. All patients underwent fully neuroendoscopic surgery. Twenty tumors were removed using the one-surgeon two-hands technique, and 27 tumors were removed using the two-surgeons four-hands technique. The anatomical integrity of the affected cranial nerves was preserved in all 47 cases. None of the patients suffered a postoperative hemorrhage, cerebrospinal fluid leak, and aseptic or septic meningitis, or died. The rate of total tumor resection was 72.3% (34/47), and the symptom improvement rate was 89.4% (42/47). All patients were followed up for 2 to 12 months, and none died nor showed any signs of tumor recurrence. By analyzing 47 fully neuroendoscopic resections of CPA tumors using the posterior sigmoid sinus approach in our center, we believe that such method allows complete, safe, and effective resection of CPA tumors and is thereby worthy of clinical promotion. [ABSTRACT FROM AUTHOR]

Vestibular schwannomas in pregnancy have rarely been reported, and there is a lack of in-depth discussion on the experience of management of massive acoustic neuromas in pregnancy. Herein, we present a pregnant woman with a giant vestibular schwannoma and obstructive hydrocephalus who presented at 30 weeks of gestation. She was initially misdiagnosed as having a pregnancy-related reaction of headache, dizziness, and vomiting that had occurred 2 months earlier. After observation at home, her symptoms progressed at 30 weeks of gestation, and imaging findings revealed a brain tumor in the CPA region with secondary cerebella tonsil herniation and obstructive hydrocephalus, and she was transferred to our center for treatment. Consequently, we relieved her hydrocephalus with a ventriculoperitoneal shunt (V-P shunt) and used corticosteroids to simulate fetal maturation. After 10 days, her mental condition deteriorated, and her right limb muscle strength gradually decreased until grade 0 (MMT Grading). Finally, under a joint consultation with the Department of Neurosurgery, Obstetrics, and Anesthesiology, she underwent a cesarean section under general anesthesia and first-stage tumor removal at 31 weeks of gestation. Upon discharge, the previously observed neurological deficits, which were reversible and had manifested during her gestational period, had been successfully resolved, and the fetus had been conserved. The neuroimaging confirmed the complete tumor removal, while the neuropathologic examination revealed a vestibular schwannoma. Therefore, we recommend early diagnosis and treatment for these patients, especially people with headaches, vomiting, and sudden hearing loss during pregnancy. Herein, we concluded that our cases provide a valuable experience in the latest acceptable time frame for the operation to prevent irreversible neurological impairment and premature delivery in late pregnancy. [ABSTRACT FROM AUTHOR]

Peripheral nerve sheath tumors (PNST) comprise schwannomas and neurofibromas. The finding of increased adipose tissue around benign PNSTs has been described as the "split fat sign" on magnetic resonance imaging exams, which is suggestive of an intramuscular or intermuscular location of the tumor. However, few studies have described this sign as a salient ultrasound feature of PNSTs. The main purpose of this study was to retrospectively evaluate the presence of increased fatty tissue deposition around benign PNSTs diagnosed by high-resolution ultrasound. In addition, we aimed to corroborate the presence of vascularization around the affected area. A retrospective analysis of ten cases of PNSTs and two cases of post-traumatic neuromas diagnosed by high-resolution ultrasound was performed with a Logiq® P8 ultrasound with a 2–11 MHz multifrequency linear probe L3-12-D (central frequency: 10 MHz). Localized types of neurofibromas and schwannomas in any location were seen as predominantly hypoechoic tumors with an oval or fusiform shape. Exiting and entering nerves (tail sign) were observed in six cases, showing localized lesions both in intermuscular and subcutaneous locations. The presence of increased hyperechoic tissue (the split fat sign) was noted in cases of solitary intermuscular and intramuscular peripheral nerve sheath tumors, mainly the schwannomas. Though small tumors did not demonstrate the tail sign, the increase in adipose tissue and vascularity on US was well demonstrated. In conclusion, the nerve in continuity forms the basis of the ultrasonographic diagnosis of PNSTs. However, high-resolution US can convincingly demonstrate the increased presence of fat in the upper and lower poles as well as circumferentially in intermuscular or intramuscular benign PNSTs. [ABSTRACT FROM AUTHOR]

Purpose: Cochlear implantation in patients with vestibular schwannomas is of increasing importance and interest. Two remaining challenges are the assessment of conduction of the cochlear nerve and the possibility of postoperative surveillance with magnetic resonance imaging. The aim of the current study was to assess follow-up imaging and determine the visibility of the internal auditory canal after vestibular schwannoma resection and cochlear implantation as well as in patients with persistent vestibular schwannomas and cochlear implants in place. Visibility of the internal auditory canal, cerebellopontine angle, and labyrinth were evaluated and graded. Methods: For this retrospective study, 15 MR examinations of 13 patients after translabyrinthine vestibular schwannoma resection and ipsilateral cochlear implantation were included. All patients had been implanted with an MED-EL cochlear implant. Magnetic resonance imaging was carried out on a 1.5T device. All patients were prepped according to the manufacturer's recommendations. Results: All 15 examinations were carried out without any adverse event during imaging, such as pain, magnet dislocation, or malfunction. The internal auditory canal and the cerebellopontine angle were sufficiently visible in all cases to allow for vestibular schwannoma follow-up. Conclusion: Magnetic resonance imaging surveillance of the internal auditory canal following vestibular schwannoma resection and cochlear implantation is feasible and safe with modern implants with a 1.5T magnetic resonance imaging device using metal artifact reduction sequences. Necessary follow-up imaging should not be a contraindication for cochlear implantation in patients with vestibular schwannomas. [ABSTRACT FROM AUTHOR]

BACKGROUND: Schwannomas are benign peripheral nerve sheath tumors which grow slowly and are considered common peripheral nerve tumors. The presentation varies between painful masses, sensory complaints or rarely motor deficits. They are commonly capsulated and can be diagnosed clinically and using imaging studies as ultrasonography and magnetic resonance imaging. Surgery is the mainstay for treatment. OBJECTIVE: To evaluate the surgical technique of resection of motor nerve schwannomas and the clinical outcome. PATIENTS AND METHODS: In our study, we had 10 cases of painful schwannomas of the limbs presented to our instistute in Alexandria Main University Hospital and affiliated hospitals. RESULTS: All our patients had resolution of their symptoms following surgery with no permanent deficits and no postoperative recurrences. CONCLUSION: Surgical resection is a safe and effective tool for managing patients harbouring peripheral nerves schwannomas with good postoperative outcomes. [ABSTRACT FROM AUTHOR]

An upper lip swelling, asymptomatic, and benign-looking lesion may be attributed to numerous aetiologies. About 25% to 45% of Schwannomas/Neurilemmomas occur in the head and neck region and are seldom seen in oral sites, accounting form only 1% of cases. Herein, the authors presented a case report of a 36-year-old female patient with a clinical appearance of upper labial mucosa swelling is mentioned, which mimicked a mucocele. It was a single, smooth, oval, fluctuant, and non tender swelling. Complete surgical excision was performed following proper protocol, and histological diagnosis revealed a well-outlined lesion enclosed within a fibrous capsule. The connective tissue mass of the tumour consisted predominantly of neural tissue arranged in Antoni A structures, characterised by a palisaded nuclear arrangement and the presence of distinctive eosinophilic Verocay bodies. Another area with a myxoid appearance, representing Antoni B structure, was also present, suggesting a Schwannoma/Neurilemmoma. During follow-up, complete healing with no recurrence of swelling was observed. Schwannomas/Neurilemmomas in the head and neck region are less common, particularly in the oral cavity. According to various studies, the occurrence of neurilemmomas in the intraoral aspect accounts for only 19.24% of cases, specifically involving the mucosa of the lip. Practitioners should consider this rare entity of Schwannoma/Neurilemmoma when evaluating an upper lip swelling routinely. [ABSTRACT FROM AUTHOR]

Squash cytology serves as a rapid intraoperative tool for the diagnosis of spinal lesions. However, the presence of fibrous elements and bony spicules can sometimes pose challenges to interpretation. Although another intraoperative diagnostic tool, frozen section, is available, the soft, friable nature and high-water content of tissue can hinder the preparation of good-quality frozen sections. This highlights the need to further explore the utility of squash cytology for the intraoperative diagnosis of spinal lesions. Our study emphasizes the importance of squash cytology in the diagnosis of spinal lesions. A retrospective analytical study was conducted at the Department of Neuropathology at a tertiary hospital in India. A total of 64 samples were collected for intraoperative squash cytology, followed by biopsies for histopathological correlation. Statistical analysis was performed, and various parameters like sensitivity, specificity, and diagnostic accuracy of squash cytology were calculated, with histopathology serving as the gold standard. Out of the 64 patients suspected of having spinal lesions, 39 cases (60.93%) were neoplastic. The mean age of presentation was 37.5 years, with no sex predilection. The most common spinal tumor was schwannoma (10 cases, 15.62%), followed by meningioma (6 cases, 9.37%). Concordance between squash cytology and histopathology was found in 60 cases (93.75%), with histopathology serving as the reference standard. The sensitivity, specificity, and diagnostic accuracy were 94.73%, 96.15%, and 95.31%, respectively. Squash cytology is a rapid, inexpensive, and reasonably accurate intraoperative diagnostic test for spinal lesions. When combined with clinical and radiological correlation, it demonstrates high diagnostic accuracy. [ABSTRACT FROM AUTHOR]

Malignant peripheral nerve sheath tumor (MPNST) is a rare malignancy of nervous tissue. The oral incidence of this lesion is extremely low. MPNST can occur sporadically or in association with neurofibromatosis. We report a rare intraosseous MPNST in the mandible of a 33-year-old female patient with neurofibromatosis type 1. [ABSTRACT FROM AUTHOR]

Background Although rare in small vestibular schwannomas, preoperative facial nerve paresis is often present in giant schwannomas. Preserving facial nerve function in these cases remains a herculean task. This study evaluates the facial functions after complete tumor removal and whether preoperative facial nerve involvement affects postoperative functional status. Methods This retrospective study from January 2014 to August 2021 excluded nongiant tumors (< 4 cm), neurofibromatosis type 2 cases, incomplete removals, redo surgeries, deaths, and cases done without nerve monitoring. These were grouped into preoperative facial palsy present (PFP) and no preoperative facial palsy (NFP). Facial nerve functions were assessed on first postoperative day, at the time of discharge, and at last follow-up and dichotomized into two groups: nondisfiguring (House–Brackmann [HB] grades I–III) and disfiguring (HB grades IV–VI). The cohort outcomes of patients with nondisfiguring PFP (HB grades I–III) were also analyzed. Results There were 88 cases (PFP, n = 57; NFP, n = 31). Facial nerve was preserved anatomically in 62 (70.45%) patients (PFP, n = 38; NFP, n = 24) without any statistical difference (p = 0.29). Statistically significant disfiguring facial outcomes (HB IV, V, VI) were seen in patients with preoperative facial palsy (p = 0.01); however, a comparison of facial functions in patients with only nondisfiguring PFP with those in NFP group did not show the statistical difference (p = 0.12). Conclusion Facial nerve palsy present before surgery does not seem to be a deterrent to intraoperative preservation of facial nerve during complete removal of giant vestibular schwannomas. Patients with nondisfiguring facial palsies have postoperative facial functions comparable to those without facial palsy. [ABSTRACT FROM AUTHOR]

Background: Advances in microscopic and endoscopic surgical techniques have outpaced traditional classification and transcranial surgical strategies, especially with reference to the treatment of trigeminal schwannomas (TSs). A modified TS classification is proposed and appropriate surgical strategies are discussed. Methods: The cases of 93 patients who underwent surgical treatment in Beijing Tiantan Hospital in the previous 6 years were analyzed retrospectively, and a literature review was conducted. Results: Classification is based on surgical direction. Tumors were classified as follows: type A, backward orientation, located in the orbit or orbit and middle cranial fossa (8 cases, 8.6%); type B, upward orientation, located in the pterygopalatine fossa, infratemporal fossa or pterygopalatine fossa, infratemporal fossa, and middle cranial fossa (23 cases, 24.7%); type C, forward and backward orientations, located in the middle cranial fossa, posterior cranial fossa or both (58 cases, 62.4%); and type D, located in multiple regions (4 cases, 4.3%). 91.40% of patients underwent gross total resection (GTR) with 29 cases receiving endoscopic resection of whom 93.10% (27/29) experienced GTR. Conclusion: The 93 cases were satisfactorily divided into four types, according to tumor location and surgical orientation, enabling safe and effective removal by appropriate surgery. [ABSTRACT FROM AUTHOR]

Shwannomas are rare benign tumours especially in tracheal. A 16‐year‐old male presented with a chronic cough, and a thoracic CT scan revealed a pedunculated tumour measuring approximately 11 × 13 mm in size, located 22 mm away from the main carina. Tissue sample was obtained via rigid bronchoscopy and cryobiopsy, and the pathological analysis confirmed the diagnosis of a benign nerve sheath tumour consistent with schwannoma. The patient subsequently underwent resection of the tumour and tracheal anastomosis. Schwannomas are uncommon pulmonary tumours that typically occur in adults, with a higher incidence among females. The presenting symptoms vary depending on the size and location of the tumour. Treatment options include therapeutic bronchoscopy or surgical resection, with the choice of approach based on tumour characteristics (pedunculated or sessile), preoperative surgical risk, and risk of recurrence. The prognosis is generally favourable, with a low risk of recurrence and excellent outcomes. [ABSTRACT FROM AUTHOR]

Anecdotally, cystic vestibular schwannomas (cVSs) are regarded to have unpredictable biologic activity with poorer clinical results, and most studies showed a less favorable prognosis following surgery. While stereotactic radiosurgery (SRS) is a well-established therapeutic option for small- to medium-sized VSs, cVSs are often larger, thus making upfront SRS more complicated. The purpose of this retrospective study was to assess the efficacy and safety of upfront SRS for large cVSs. The authors reviewed the data of 54 patients who received upfront, single-session Gamma Knife radiosurgery (GKRS) with a diagnosis of large cVS (> 4 cm3). Patients with neurofibromatosis type 2, multiple VSs, or recurrent VSs and < 24 months of clinical and neuroimaging follow-up were excluded. Hearing loss (48.1%) was the primary presenting symptom. The majority of cVSs were Koos grade IV (66.7%), and the most prevalent cyst pattern was "mixed pattern of small and big cysts" (46.3%). The median time between diagnosis and GKRS was 12 months (range, 1–147 months). At GKRS, the median cVS volume was 6.95 cm3 (range, 4.1–22 cm3). The median marginal dose was 12 Gy (range, 10–12 Gy). The mean radiological and clinical follow-up periods were 62.2 ± 34.04 months (range, 24–169 months) and 94.9 ± 45.41 months (range, 24–175 months), respectively. At 2, 6, and 12 years, the tumor control rates were 100%, 95.7%, and 85.0%, respectively. Tumor shrinkage occurred in 92.6% of patients (n = 50), tumor volume remained stable in 5.6% of patients (n = 3), and tumor growth occurred in 1.9% of patients (n = 1). At a median follow-up of 53.5 months, the pre-GKRS tumor volume significantly decreased to 2.35 cm3 (p < 0.001). While Koos grade 3 patients had a greater possibility of attaining higher volume reduction, "multiple small thick-walled cyst pattern" and smaller tumor volumes decreased the likelihood of achieving higher volume reduction. Serviceable hearing (Gardner-Robertson Scale I–II) was present in 16.7% of patients prior to GKRS and it was preserved in all of these patients following GKRS. After GKRS, 1.9% of patients (n = 1) had new-onset trigeminal neuralgia. There was no new-onset facial palsy, hemifacial spasm, or hydrocephalus. Contrary to what was believed, our findings suggest that upfront GKRS seems to be a safe and effective treatment option for large cVSs. [ABSTRACT FROM AUTHOR]

Background: Schwannomas are solitary neurogenic tumors originating from the myelin-producing cells of the neural sheath. Hypoglossal nerve schwannomas are exceedingly rare, particularly those extracranially originating and mimicking a submandibular salivary gland tumor. Methods: We report the case of a 31-year-old female who presented to our ENT department with a painless swelling in her left submandibular region that has persisted for approximately five months. Discussion: Due to the rarity of these tumors and their unique nature, a comprehensive diagnostic workup is imperative for accurate diagnosis. Surgical excision remains the gold standard treatment. Conclusions: Extracranial hypoglossal nerve schwannomas represent a rare clinical entity requiring a thorough diagnostic process for precise identification. The preferred treatment strategy for managing hypoglossal gland schwannomas involves complete tumor excision while preserving the facial nerve. [ABSTRACT FROM AUTHOR]

Neurofibroma (NF) is a benign tumor in the peripheral nervous system, but it can infiltrate around structures and cause functional impairment and disfigurement. We incidentally found that the expression of CD16a (Fc gamma receptor IIIA) was increased in NFs compared to in non-neoplastic nerves and hypothesized that CD16 could be relevant to NF progression. We evaluated the expressions of CD16a, CD16b, CD68, TREM2, Galectin-3, S-100, and SOX10 in 38 cases of neurogenic tumors (NF, n = 18; atypical neurofibromatous neoplasm of uncertain biologic potential (ANNUBP), n = 14; and malignant peripheral nerve sheath tumor (MPNST), n = 6) by immunohistochemical staining. In the tumor microenvironment (TME) of the ANNUBPs, CD16a and CD16b expression levels had increased more than in the NFs or MPNSTs. CD68 and Galectin-3 expression levels in the ANNUBPs were higher than in the MPNSTs. Dual immunohistochemical staining showed an overlapping pattern for CD16a and CD68 in TME immune cells. Increased CD16a expression was detected in the ANNUBPs compared to the NFs but decreased with malignant progression. The CD16a overexpression with CD68 positivity in the ANNUBPs potentially reflects that the TME immune modulation could be associated with NF progression to an ANNUBP. Further studies should explore the role of CD16a in immunomodulation for accelerating NF growth. [ABSTRACT FROM AUTHOR]

Objective: This study aims to investigate the value of histogram analysis based on iodine-based material decomposition (IMD) images obtained through dualenergy computed tomography (DECT) to differentiate gastric schwannoma (GS) from gastric stromal tumor (GST) (≤ cm) preoperatively. Methods: From January 2015 to January 2023, 15 patients with GS and 30 patients with GST (≤ cm) who underwent biphasic contrast-enhanced scans using DECT were enrolled in this study. For each tumor, we reconstructed IMD images at the arterial phase (AP) and venous phase (VP). Nine histogram parameters were automatically extracted and selected using MaZda software based on the IMD of AP and VP, respectively, including mean, 1st, 10th, 50th, 90th, and 99th percentile of the iodine concentration value (Perc.01, Perc.10, Perc.50, Perc.90, and Perc.99), variance, skewness, and kurtosis. The extracted IMD histogram parameters were compared using the Mann-Whitney U-test. The optimal IMD histogram parameters were selected using receiver operating characteristic (ROC) curves. Results: Among the IMD histogram parameters of AP, the mean, Perc.50, Perc.90, Perc.99, variance, and skewness of the GS group were lower than that of the GST group (all P < 0.05). Among the IMD histogram parameters of VP, Perc.90, Perc.99, and the variance of the GS group was lower than those of the GST group (all P < 0.05). The ROC analysis showed that Perc.99 (AP) generated the best diagnostic performance with the area under the curve, sensitivity, and specificity being 0.960, 86.67%, and 93.33%, respectively, when using 71.00 as the optimal threshold. Conclusion: Histogram analysis based on IMD images obtained through DECT holds promise as a valuable tool for the preoperative distinction between GS and GST (≤ cm). [ABSTRACT FROM AUTHOR]

Schwannoma can rarely mimic an intraneural ganglion cyst clinically and radiographically. This is a rare case report of a wrist schwannoma mimicking an intraneural ganglion cyst. The surgery was successful, and the histopathological report confirmed the diagnosis of benign schwannoma. After two years of follow-up, the patient is still symptom-free. [ABSTRACT FROM AUTHOR]

To investigate clinical data and computed tomographic (CT) imaging features in differentiating gastric schwannomas (GSs) from gastric stromal tumours (GISTs) in matched patients, 31 patients with GSs were matched with 62 patients with GISTs (1:2) in sex, age, and tumour site. The clinical and imaging data were analysed. A significant (P < 0.05) difference was found in the tumour margin, enhancement pattern, growth pattern, and LD values between the 31 patients with GSs and 62 matched patients with GISTs. The GS lesions were mostly (93.5%) well defined while only 61.3% GIST lesions were well defined.The GS lesions were significantly (P = 0.036) smaller than the GIST lesions, with the LD ranging 1.5–7.4 (mean 3.67 cm) cm for the GSs and 1.0–15.30 (mean 5.09) cm for GIST lesions. The GS lesions were more significantly (P = 0.001) homogeneously enhanced (83.9% vs. 41.9%) than the GIST lesions. The GS lesions were mainly of the mixed growth pattern both within and outside the gastric wall (74.2% vs. 22.6%, P < 0.05) compared with that of GISTs. No metastasis or invasion of adjacent organs was present in any of the GS lesions, however, 1.6% of GISTs experienced metastasis and 3.2% of GISTs presented with invasion of adjacent organs. Heterogeneous enhancement and mixed growth pattern were two significant (P < 0.05) independent factors for distinguishing GS from GIST lesions. In conclusion: GS and GIST lesions may have significantly different features for differentiation in lesion margin, heterogeneous enhancement, mixed growth pattern, and longest lesion diameter, especially heterogeneous enhancement and mixed growth pattern. [ABSTRACT FROM AUTHOR]

Vestibular schwannoma is the most common benign neoplasm of the cerebellopontine angle. Its first symptoms include hearing loss, tinnitus, and vestibular symptoms, followed by cerebellar and brainstem symptoms, along with palsy of the adjacent cranial nerves. However, the clinical picture has unpredictable dynamics and currently, there are no reliable predictors of tumor behavior. Hence, it is desirable to have a fast routine method for analysis of vestibular schwannoma tissues at the molecular level. The major objective of this study was to verify whether a technique using in‐sample specific protein digestion with trypsin would have the potential to provide a proteomic characterization of these pathological tissues. The achieved results showed that the use of this approach with subsequent liquid chromatography‐tandem mass spectrometry (LC‐MS/MS) analysis of released peptides allowed a fast identification of a considerable number of proteins in two differential parts of vestibular schwannoma tissue as well as in tissues of control healthy samples. Furthermore, mathematical analysis of MS data was able to discriminate between pathological vestibular schwannoma tissues and healthy tissues. Thus, in‐sample protein digestion combined with LC‐MS/MS separation and identification of released specific peptides followed by mathematical analysis appears to have the potential for routine characterization of vestibular schwannomas at the molecular level. Data are available via ProteomeXchange with identifier PXD045261. [ABSTRACT FROM AUTHOR]

Background: Intrathoracic neurogenic tumors arise from sympathetic nerve trunks and intercostal nerves; more than 90% are benign. Schwannomas are the most common histological variety, but fatalities due to giant schwannomas are rare. Case presentation: We report a case of a 65-year-old woman who presented with chest pain and cough. Computed tomography (CT) revealed a large left chest wall mass of 130-mm in size, and the patient was referred to our department. Tumor biopsy was performed under local anesthesia, and a diagnosis of schwannoma was made. Ten years previously, a 30-mm tumor had been noted in the left third intercostal space by a previous doctor, but follow-up had been interrupted owing to depressive disorder. Although we planned to perform intercostal artery embolization followed by chest wall tumor resection, the patient did not consent to surgery due to uncontrolled depression. After four months, she developed respiratory failure caused by compression due to an enlarged tumor and died. Autopsy also revealed a benign schwannoma with no malignant findings. Conclusions: Although schwannomas are benign tumors, there are some very rare cases in which they can become huge and life-threatening. Therefore, a benign tumor should not be neglected, and if surgery is not possible at the time of diagnosis, a regular follow up is necessary, in order not to miss the right timing for surgery. [ABSTRACT FROM AUTHOR]

Aim: The incidence of spinal cord tumors (SCTs) in a population considering sex, age, histopathology, and emplacement position of tumors is different between various regions. Therefore, the present study aimed to evaluate the frequency of various SCTs in surgically treated patients in a reference spinal surgery center in Shiraz, Iran. Method: Documented information related to 109 SCT patients surgically treated was collected from the archive of Shahid Chamran Hospital (the reference center of spinal cord surgery in the south of Iran) in Shiraz from 2012 to 2022. This information includes demographics, medical history, histology, and position of SCTs within the spinal cord. Result: The population of patients included 65 men and 44 women, with a mean age of 44.7 years old. Most patients (26 cases) were in the age group of 50–59 years. Neurological function in most patients (51 cases) was equivalent to level D of the ASIA impairment scale (AIS), and the most common complaints of patients (83 cases) were related to pain. Finally, anatomic and histologic evaluations indicated that intradural and schwannoma tumors were the most common types of tumors, with 64 and 25 cases, respectively. Conclusions: In the present study, most of the findings are consistent with the previous reports. Schwannoma and ependymoma tumors were the most common types of SCTs and the most common types of intramedullary tumors, respectively. The sixth decade of life was the most common age of incidence. Neurological function in most patients with SCT was equivalent to levels of D and E of AIS. However, regarding the factor of gender, our results are similar to those of the Asian population. More comprehensive studies in the future likely reveal the probable role of environmental, genetic, and hormonal factors in the etiology of SCTs. [ABSTRACT FROM AUTHOR]

Lipoblastic nerve sheath tumors of soft tissue are characterized as schwannoma tumors that exhibit adipose tissue and lipoblast-like cells with signet-ring morphology. They have been documented to arise in various anatomic locations, including the thigh, groin, shoulder, and retroperitoneum. However, to our knowledge, this tumor has not been previously reported as a lymph node primary. We present herein the first case of a benign primary lipoblastic nerve sheath tumor arising in an inguinal lymph node in a 69-year-old man. Microscopic examination revealed a multinodular tumor comprising fascicles of spindle cells, as well as adipocytic and lipoblast-like signet-ring cell component in the context of schwannoma. Despite the presence of some bizarre cells with nuclear atypia, no obvious mitotic activity or necrosis was observed. Immunohistochemical analysis showed strong and diffuse expression of S-100, SOX10, CD56, and NSE in the spindle cells as well as in the signet-ring lipoblast-like cells and the mature adipocytes. Sequencing analysis of the neoplasm identified six non-synonymous single nucleotide variant genes, specifically NF1, BRAF, ECE1, AMPD3, CRYAB, and NPHS1, as well as four nonsense mutation genes including MRE11A, CEP290, OTOA, and ALOXE3. The patient remained alive and well with no evidence of recurrence over a period of ten-year follow-up. [ABSTRACT FROM AUTHOR]

Primary masses rarely originate from the heart and great vessels, and a malignant peripheral nerve sheath tumor (MPNST) is extremely rare. A 76-year-old male with pleural effusion underwent contrast-enhanced computed tomography, which revealed a hypoattenuating mass involving the right pulmonary vein and left atrium. Ultrasonography showed that the mass originated from the right pulmonary vein. Surgical resection confirmed an MPNST that originated from the pulmonary vein. We report the first Korean case of a primary MPNST originating from the pulmonary vein. We have also described the radiologic findings suggestive of a pulmonary vein mass. [ABSTRACT FROM AUTHOR]

Purpose: To highlight the changes in the management of vestibular schwannoma (VS) since 2004 with a focus on small- to middle-size VS. Methods: Retrospective analysis of the decisions made in skull base tumor board between 2004 and 2021. Results: 1819 decisions were analyzed (average age 59.25, 54% females). Overall, 850 (47%) cases were allocated to a Wait and Scan (WS) approach, 416 (23%) received radiotherapy and 553 (30%) were treated surgically (MS). All stages considered WS increased from 39% before 2010 to 50% after 2010. Similarly, Stereotactic Radio Therapy (SRT) increased from 5 to 18%. MS decreased from 46 to 25%. It was more commonly proposed to younger patients and larger tumors, p < 0.001. For Koos stages 1, 2, and 3 there was a statistically significant increase in SRT, and a decrease in MS, p < 0.001. WS also increased for stages 1 and 2. However, such a trend was not observed for stage 3. MS remained the primary treatment modality for stage 4 tumors throughout the study period, p = 0.057. The significance of advanced age as a factor favoring SRT decreased over time. The opposite is true for serviceable hearing. There was also a decrease in the percentage of the justification "young age" in the MS category. Conclusion: The is a continuing trend towards non-surgical treatment. Small- to medium-sized VS witnessed an increase in both WS and SRT. There is only an increase in SRT for moderately large VS. Physicians are less and less considering young age as a factor favoring MS over SRT. There is a tendency towards favoring SRT when hearing is serviceable. [ABSTRACT FROM AUTHOR]

Intraosseous schwannoma is a rare diagnosis, particularly so in the skull. Accordingly, little data exists to unify common features of this disease. Here, we present the fourth known case of a primary intraosseous schwannoma of the frontal bone: a 46-year-old male with severe, progressive headache and an osteolytic frontal bone lesion. Gross total resection of the lesion was performed with excellent clinical outcome. Histological analysis confirmed the diagnosis. The limited existing literature on this topic was reviewed to identify emerging trends surrounding presenting symptoms and treatment. Early literature suggests symptoms are often nonspecific, except for lesions of the petrous apex. No cases of recurrence have been demonstrated after gross total resection, though incomplete resection has been associated with recurrence. This diagnosis appears to be becoming a more often-considered differential for osteolytic, expansile skull lesions. [ABSTRACT FROM AUTHOR]

Schwannomas are benign slow-growing tumors arising from the embryonic neural crest cells of the nerve sheaths of peripheral and cranial nerves, and they are a rare type of soft tissue mass that is usually always solitary. Generally, it grows in the head, neck, and flexor portions of the limbs, where many nerves are located. Schwannomas of the lung are extremely rare. Fewer than ten cases of schwannomas in this organ region have been reported in the existing literature. In this case report, a 40-year-old male non-smoker was hospitalized with occasional chest pain. His chest computed tomographic scan revealed a 3.8 cm space occupying lesion in the upper lobe of the right lung. This lesion has clear boundaries and uneven internal density. And it was concluded as a benign lesion possibility, it is preferred to be considered as a haematoma. This space occupying lesion was eventually confirmed as a schwannoma by needle biopsy tissues. Due to the presence in rare locations, such as the lung, the clinical presentation of this space occupying lesion is non-specific, making diagnosis difficult. The data presented in this case report can help clinicians to obtain information on the identification of this disease, which highlighted lung schwannoma as a differential diagnosis for patients with intermittent pain. It can also alert clinicians and radiologists to observe every detail of the radiology imaging findings. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Palisaded encapsulated neuroma (PEN) is generally seen in the head and neck area as an asymptomatic nodule with the same color as the surrounding skin and rarely occurs in the oral cavity. The exact etiology of PEN is not known, but there is evidence supporting the role of trauma as its etiological factor. Palisaded encapsulated neuroma (PEN) is one of the benign nerve sheath tumors of Schwann cell origin, which is commonly found in the skin of the head and neck area, and rarely occurs in the oral cavity. Its exact etiology is unknown, but there is evidence that supports the role of trauma as an etiological factor. Here we present a case of PEN in the hard palate of a 30‐year‐old patient and review the differential diagnoses of these nerve sheath tumors of the oral cavity. [ABSTRACT FROM AUTHOR]

The diagnosis of large vestibular schwannomas (VS) with retained useful hearing has become increasingly common. Preservation of facial nerve (FN) function has improved using intraoperative EMG monitoring, hearing preservation remains challenging, with the recent use of cochlear nerve action potential (CNAP) monitoring. This prospective longitudinal series of VS with useful hearing operated on using a retrosigmoid approach included 37 patients with a mean largest extrameatal VS. diameter of 25 ± 8.7 mm (81% of Koos stage 4). CNAP was detected in 51% of patients, while auditory brainstem responses (ABR) were present in 22%. Patients were divided into two groups based on the initial intraoperative CNAP status, whether it was present or absent. FN function was preserved (grade I–II) in 95% of cases at 6 months. Serviceable hearing (class A + B) was preserved in 16% of the cases, while 27% retained hearing with intelligibility (class A–C). Hearing with intelligibility (class A–C) was preserved in 42% of cases when CNAP could be monitored in the early stages of VS resection versus 11% when it was initially absent. Changes in both the approach to the cochlear nerve and VS resection are mandatory in preserving CNAP and improve the rate of hearing preservation. [ABSTRACT FROM AUTHOR]

The surgical management of vestibular schwannomas should be based on their presentation, neuro-imaging findings, surgeons' expertise, and logistics. Multi-stage surgery can be beneficial for large-sized lesions with acute presentations. Herein, we highlighted the indications for two cases managed initially through the retrosigmoid and, subsequently, translabyrinthine approaches. The first case presented with acute balance and gait issues and a long history of hearing loss and blurred vision. Neuroimaging findings revealed a cerebellopontine angle lesion, resembling a vestibular schwannoma, with significant brainstem compression and hydrocephalus. Due to the rapidly deteriorating clinical status and large-sized tumor, we first proceeded with urgent decompression via a retrosigmoid approach, followed by gross total resection via a translabyrinthine approach two weeks later. The second case presented with gradually worsening dizziness and hemifacial numbness accompanied by acute onset severe headaches and hearing loss. Neuroimaging findings showed a large cerebellopontine angle lesion suggestive of a vestibular schwannoma with acute intratumoral hemorrhage. Given the acute clinical deterioration and large size of the tumor, we performed urgent decompression with a retrosigmoid approach followed by gross total resection through a translabyrinthine approach a week later. Post-surgery, both patients showed excellent recovery. When managing acutely presented large-sized vestibular schwannomas, immediate surgical decompression is vital to avoid permanent neurological deficits. [ABSTRACT FROM AUTHOR]

Objective: To describe the clinical and sonographic characteristics of benign, retroperitoneal, pelvic peripheral‐nerve‐sheath tumors (PNSTs). Methods: This was a retrospective study of patients with a benign, retroperitoneal, pelvic PNST who had undergone preoperative ultrasound examination at a single gynecologic oncology center between 1 January 2018 and 31 August 2022. All ultrasound images, videoclips and final histological specimens of benign PNSTs were reviewed side‐by‐side in order to: describe the ultrasound appearance of the tumors, using the terminology of the International Ovarian Tumor Analysis (IOTA), Morphological Uterus Sonographic Assessment (MUSA) and Vulvar International Tumor Analysis (VITA) groups, following a predefined ultrasound assessment form; describe their origin in relation to nerves and pelvic anatomy; and assess the association between their ultrasound features and histotopography. A review of the literature reporting benign, retroperitoneal, pelvic PNSTs with preoperative ultrasound examination was performed. Results: Five women (mean age, 53 years) with a benign, retroperitoneal, pelvic PNST were identified, four with a schwannoma and one with a neurofibroma, of which all were sporadic and solitary. All patients had good‐quality ultrasound images and videoclips and final biopsy of surgically excised tumors, except one patient managed conservatively who had only a core needle biopsy. In all cases, the findings were incidental. The five PNSTs ranged in maximum diameter from 31 to 50 mm. All five PNSTs were solid, moderately vascular tumors, with non‐uniform echogenicity, well‐circumscribed by hyperechogenic epineurium and with no acoustic shadowing. Most of the masses were round (n = 4 (80%)), and contained small, irregular, anechoic, cystic areas (n = 3 (60%)) and hyperechogenic foci (n = 5 (100%)). In the woman with a schwannoma in whom surgery was not performed, follow‐up over a 3‐year period showed minimal growth (1.5 mm/year) of the mass. We also summarize the findings of 47 cases of benign retroperitoneal schwannoma and neurofibroma identified in a literature search. Conclusions: On ultrasound examination, no imaging characteristics differentiate reliably between benign schwannomas and neurofibromas. Moreover, benign PNSTs show some similar features to malignant retroperitoneal tumors. They are solid lesions with intralesional blood vessels and show degenerative changes such as cystic areas and hyperechogenic foci. Therefore, ultrasound‐guided biopsy may play a pivotal role in their diagnosis. If confirmed to be benign PNSTs, these tumors can be managed conservatively, with ultrasound surveillance. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Peripheral nerve sheath tumors include a range of well-defined pathologic entities from benign tumors such as schwannoma and neurofibromas to high-grade malignant neoplasms such as malignant peripheral nerve sheath tumors (MPNST). Hybrid neurofibromas-schwannomas have recently been described in the literature as nerve sheath tumors with schwannoma-like nodules within a neurofibroma-like tumor. We present a case of a 72-year-old female who initially presented with a non-painful soft tissue mass to the right ankle. Magnetic resonance imaging (MRI) was obtained which revealed a peripheral nerve sheath tumor located adjacent to the posterior tibial nerve. Due to her concerns of malignancy, the patient underwent surgical excision of the peripheral nerve tumor. The pathology report revealed a large peripheral nerve sheath tumor, consistent with hybrid neurofibroma/schwannoma which was positive for biological markers SOX-10, S100 and CD34, consistent with the diagnosis of a schwannoma-neurofibroma hybrid. [ABSTRACT FROM AUTHOR]

Background: Malignant peripheral nerve sheath tumor (MPNST) is an exceedingly rare and aggressive tumor, with limited literature on its management. Herein, we present our series of surgically managed craniospinal MPNSTs, analyze their outcomes, and review the literature. Methods: We retrospectively reviewed surgically managed primary craniospinal MPNSTs treated at our institution between January 2005 and May 2023. Patient demographics, tumor features, and treatment outcomes were assessed. Neurological function was quantified using the Frankel grade and Karnofsky performance scores. Descriptive statistics, rank-sum tests, and Kaplan–Meier survival analyses were performed. Results: Eight patients satisfied the inclusion criteria (4 male, 4 female). The median age at presentation was 38 years (range 15–67). Most tumors were localized to the spine (75%), and 3 patients had neurofibromatosis type 1. The most common presenting symptoms were paresthesia (50%) and visual changes (13%). The median tumor size was 3 cm, and most tumors were oval-shaped (50%) with well-defined borders (75%). Six tumors were high grade (75%), and gross total resection was achieved in 5 patients, with subtotal resection in the remaining 3 patients. Postoperative radiotherapy and chemotherapy were performed in 6 (75%) and 4 (50%) cases, respectively. Local recurrence occurred in 5 (63%) cases, and distant metastases occurred in 2 (25%). The median overall survival was 26.7 months. Five (63%) patients died due to recurrence. Conclusions: Primary craniospinal MPNSTs are rare and have an aggressive clinical course. Early diagnosis and treatment are essential for managing these tumors. In this single-center study with a small cohort, maximal resection, low-grade pathology, young age (< 30), and adjuvant radiotherapy were associated with improved survival. [ABSTRACT FROM AUTHOR]

This article presents a rare case of a benign tumor called schwannoma in a child's nasal cavity. The tumor was discovered through a painless swelling in the nose that had been growing slowly. The diagnosis was confirmed through examination of tissue samples, and the article emphasizes the importance of considering schwannoma as a possible cause of nasal masses, even in children. Surgical removal of the tumor is the standard treatment, with endoscopic surgery being the preferred method. The article also mentions a potential link between schwannomas and nerve compression or physical trauma, but more research is needed to confirm this. [Extracted from the article]