Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of breast myofibroblastoma. Methods: The clinicopathological data and prognostic information of 15 patients with breast myofibroblastoma diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from 2014 to 2022 were collected. Their clinical characteristics, histological subtypes, immunophenotypes and molecular characteristics were analyzed. Results: There were 12 female and 3 male patients, ranging in age from 18 to 78 years, with a median and average age of 52 years. There were 6 cases in the left breast and 9 cases in the right breast, including 12 cases in outer upper quadrant, 2 cases in inner upper quadrant and 1 case in outer lower quadrant. Most of the cases showed a well-defined nodule grossly, including pushing growth under the microscope in 13 cases, being completely separated from the surrounding breast tissue in 1 case, and infiltrating growth in 1 case. Among them, 12 cases were classic subtype and composed of occasional spindle cells with varying intervals of collagen fiber bundles; eight cases had a small amount of fat; one case had focal cartilage differentiation; one case was epithelioid subtype, in which epithelioid tumor cells were scattered in single filing or small clusters; one case was schwannoma-like subtype, and the tumor cells were arranged in a significant palisade shape, resembling schwannoma, and one case was invasive leiomyoma-like subtype, in which the tumor cells had eosinophilic cytoplasm and were arranged in bundles, and infiltrating into the surrounding mammary lobules like leiomyoma. Immunohistochemical studies showed that the tumor cells expressed desmin (14/15) and CD34 (14/15), as well as ER (15/15) and PR (15/15). Three cases with histologic subtypes of epithelioid subtype, schwannoma-like subtype and infiltrating leiomyoma-like subtype showed RB1 negative immunohistochemistry. Then FISH was performed to detect RB1/13q14 gene deletion, and identified RB1 gene deletion in all three cases. Fifteen cases were followed up for 2-100 months, and no recurrence was noted. Conclusions: Myofibroblastoma is a rare benign mesenchymal tumor of the breast. In addition to the classic type, there are many histological variants, among which the epithelioid subtype is easily confused with invasive lobular carcinoma. The schwannoma-like subtype is similar to schwannoma, while the invasive subtype is easily misdiagnosed as fibromatosis-like or spindle cell metaplastic carcinoma. Therefore, it is important to recognize the various histological subtypes and clinicopathological features of the tumor for making correct pathological diagnosis and rational clinical treatment.
Female ; Humans ; Male ; Antigens, CD34 ; Biomarkers, Tumor/analysis* ; Leiomyoma/pathology* ; Neoplasms, Muscle Tissue/pathology* ; Neurilemmoma ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged

Humans ; Histones/genetics* ; DNA Methylation ; Neurilemmoma/pathology* ; Central Nervous System Neoplasms/genetics*

OBJECTIVE: To analyze clinical features, surgical treatment and outcomes of neurilemmoma and carotid body tumors in bifurcation of carotid artery. METHODS: The clinical data of 17 patients with neurilemmomas and 76 patients with carotid body tumors at the bifurcation of carotid artery, who were surgically treated in Zhongshan Hospital of Fudan University from March 2012 to November 2016, were retrospectively analyzed. The clinicopathological characteristics, surgical procedures and outcomes were compared between two groups. RESULTS: No difference of preoperative clinical demographic data was found between two groups. Operation time of the neurilemmoma group was significantly shorter than that of the carotid body tumor group[(93.9±30.8) min vs. (159.3±52.9) min, <0.01]. The neurilemmoma group had lower volume of intra-operative blood loss[(110±96) mL vs. (356±239) mL, <0.01] and lower rate of external carotid artery resection (11.8% vs. 68.4%, <0.01) than the carotid body tumor group. In the neurilemmoma group, 17 tumors were completely resected and no malignant disease was found. In the carotid body tumor group, 76 patients underwent complete surgical resection for the tumor, of which 5 (6.6%) were malignant. Tumor size of the neurilemmoma group was larger than that of the carotid body tumor group[(4.5±1.4) cm vs. (3.1±1.0) cm, <0.01]. There was no significant difference in the incidence of peri-operative complications and length of hospital stay between two groups (>0.05). CONCLUSIONS The clinical manifestations of neurilemmoma and carotid body tumors at carotid artery bifurcation are similar. The carotid body tumor group has a longer operating time, larger intra-operative blood loss, higher external carotid resection rate and relative higher incidence of malignancy. More cautions should be given when carotid body tumors at carotid artery bifurcation are treated.
Carotid Arteries ; surgery ; Carotid Body Tumor ; pathology ; surgery ; Humans ; Neurilemmoma ; pathology ; surgery ; Retrospective Studies ; Treatment Outcome

OBJECTIVE: Because the anatomical structure of the brachial plexus is very complex, surgical treatment of tumors in this region is challenging. Therefore, a lot of clinical and surgical experience is required for successful treatment; however, many neurosurgeons have difficulty accumulating this experience owing to the rarity of brachial plexus tumors. The purpose of this report is to share our surgical experience with brachial plexus tumor with other neurosurgeons.METHODS: The records of 18 consecutive patients with brachial plexus tumors who underwent surgical treatment between January 2010 and December 2017 in a single institution were retrospectively reviewed. The surgical approach was determined according to the tumor location and size, and intraoperative neurophysiological monitoring (IONM) was used in most of cases to prevent iatrogenic nerve injury during surgery. In addition, to evaluate the differences in tumor characteristics according to pathologic diagnosis, the tumors were divided twice into two groups, based on two separate classifications, and statistical analysis was performed.RESULTS: The 18 brachial plexus tumors comprised 15 (83.3%) benign peripheral nerve sheath tumors including schwannoma and neurofibroma, one (5.6%) malignant peripheral nerve sheath tumor, one (5.6%) benign tumor of non-neural sheath origin (neurogenic cyst), and one (5.6%) metastatic tumor (papillary carcinoma). The authors analyzed relationship between tumor size/location and tumor characteristic parameters such as age, size, right-left, and pathology. There were no statistically significant differences except a tendency of bigger tumor size in young age.CONCLUSION: For a successful surgical outcome, an appropriate surgical approach is essential, and the appropriate surgical approach is determined by the location and size of the tumor. Furthermore, applying IONM may prevent postoperative complications and it is favorable option for brachial plexus tumors surgery.
Brachial Plexus Neuropathies ; Brachial Plexus ; Classification ; Diagnosis ; Humans ; Intraoperative Neurophysiological Monitoring ; Monitoring, Intraoperative ; Nerve Sheath Neoplasms ; Neurilemmoma ; Neurofibroma ; Neurosurgeons ; Pathology ; Peripheral Nerves ; Postoperative Complications ; Retrospective Studies

PURPOSE: Malignant peripheral nerve sheath tumors (MPNSTs) are a rare subtype of sarcoma that occur spontaneously or in association with neurofibromatosis type 1 (NF-1). This study aimed to clinically differentiate these types of MPNSTs. MATERIALS AND METHODS: The study reviewed 95 patients diagnosed with and treated for MPNST at Yonsei University Health System, Seoul, Korea over a 27-year period. The clinical characteristics, prognostic factors, and treatment outcomes of sporadic MPNST (sMPNST) and NF-1 associated MPNST (NF-MPNST) cases were compared. RESULTS: Patients with NF-MPNST had a significantly lower median age (32 years vs. 45 years for sMPNST, p=0.012), significantly larger median tumor size (8.2 cm vs. 5.0 cm for sMPNST, p < 0.001), and significantly larger numbers of imaging studies and surgeries (p=0.004 and p < 0.001, respectively). The 10-year overall survival (OS) rate of the patients with MPNST was 52±6%. Among the patients with localized MPNST, patients with NF-MPNST had a significantly lower 10-year OS rate (45±11% vs. 60±8% for sMPNST, p=0.046). Univariate analysis revealed the resection margin, pathology grade, and metastasis to be significant factors affecting the OS (p=0.001, p=0.020, and p < 0.001, respectively). Multivariate analysis of the patients with localized MPNST identified R2 resection and G1 as significant prognostic factors for OS. CONCLUSION: NF-MPNST has different clinical features from sMPNST and requires more careful management. Further study will be needed to develop specific management plans for NF-MPNST.
Humans ; Korea ; Multivariate Analysis ; Neoplasm Metastasis ; Neurilemmoma* ; Neurofibromatoses* ; Neurofibromatosis 1* ; Pathology ; Sarcoma ; Seoul

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the most common cause of euvolemic hypo-osmotic hyponatremia. There are several etiologies of SIADH including neuroendocrine tumor, pulmonary disease, infection, trauma, and medications. Here, we report a case of SIADH associated with a schwannoma involving the mediastinum in a 75-year-old woman who presented with nausea, vomiting, and general weakness. Laboratory testing showed hypo-osmolar hyponatremia, with a serum sodium level of 102mmol/L, serum osmolality of 221mOsm/kg, urine osmolality of 382mOsm/kg, urine sodium of 55 mmol/L, and plasma antidiuretic hormone (ADH) of 4.40 pg/mL. Chest computed tomography identified a 1.5-cm-sized solid enhancing nodule in the right lower paratracheal area. A biopsy specimen was obtained by video-assisted thoracoscopic surgery, which was diagnosed on pathology as a schwannoma. The hyponatremia was completely resolved after schwannoma resection and plasma ADH level decreased from 4.40 pg/mL to 0.86 pg/mL. This case highlights the importance of suspecting and identifying the underlying cause of SIADH when faced with refractory or recurrent hyponatremia, and that on possibility is mediastinal schwannoma
Aged ; Biopsy ; Female ; Humans ; Hyponatremia ; Inappropriate ADH Syndrome ; Lung Diseases ; Mediastinum ; Nausea ; Neurilemmoma* ; Neuroendocrine Tumors ; Osmolar Concentration ; Pathology ; Plasma ; Sodium ; Thoracic Surgery, Video-Assisted ; Thorax ; Vomiting

Primary meningioma of jugular foramen is extremely rare, while paraganglioma or nerve sheath tumor are relatively common in jugular foramen. We reported a case of primary meningioma of jugular foramen. A 79-year-old female who had left tinnitus and hearing loss for three month came to the department of otorhinolaryngology. Temporal bone computed tomography scan showed sclerotic change and slightly irregular margins of left jugular foramen with relatively preservation of bony architecture. Temporal bone magnetic resonance image showed well defined homogeneous enhancing mass in left jugular foramen with extension to carotid space on gadolinium enhanced T1 weighted image. Prominent dural tail was also noted. On T2 weighted image, this mass showed intermediated signal intensity with no vascular signal voids. Meningioma was confirmed by pathology. In this article, we describe a case of primary en plaque meningioma of jugular foramen and review image findings of differential diagnosis.
Aged ; Diagnosis, Differential* ; Female ; Gadolinium ; Glomus Jugulare Tumor ; Hearing Loss ; Humans ; Meningioma* ; Neurilemmoma ; Otolaryngology ; Paraganglioma ; Pathology ; Tail ; Temporal Bone ; Tinnitus

OBJECTIVE: A fibrin sealant is commonly applied after closure of an incidental or intended durotomy to reduce the complications associated with the leakage of cerebrospinal fluid. Routine usage might not be essential after closure of an intended durotomy, which has clear cut-margins. We investigated the efficacy of fibrin sealants for primary intradural spinal cord tumor surgery. METHODS: A retrospective review was performed for 231 consecutive surgically treated patients with primary intradural spinal cord tumors without extradural extension. Fibrin sealants were not used for 47 patients (group I: age, 51.57±16.75 years) and were applied to 184 patients (group II: age, 48.8±14.7 years). The surgical procedures were identical except for the use of a fibrin sealant after closure of the durotomy. The primary outcome was the occurrence of complications (wound problems, hematoma collection, infection, and neurological deterioration). The covariates were age, sex, body mass index, operation time, pre-/postoperative ambulation, number of laminectomies, and type of tumor. RESULTS: Schwannoma was the most common pathology (n=134), followed by meningioma (n=35) and ependymoma (n=31). Complications occurred in 13 patients (3 in group I and 10 in group II, p=0.73). The postoperative ambulation status (p<0.01; odds ratio, 28.8; 95% confidence interval, 6.9-120.0) and operation time (p=0.04; cutoff, 229 minutes; sensitivity, 62%; specificity, 72%) were significant factors, whereas the use of a fibrin glue was not (p=0.47). CONCLUSION: The use of a fibrin sealant might not be essential to reduce complications after surgery for primary spinal intradural tumor.
Body Mass Index ; Cerebrospinal Fluid ; Ependymoma ; Fibrin Tissue Adhesive* ; Fibrin* ; Hematoma ; Humans ; Laminectomy ; Meningioma ; Neurilemmoma ; Odds Ratio ; Pathology ; Retrospective Studies ; Sensitivity and Specificity ; Spinal Cord Neoplasms ; Spine ; Surgical Wound Infection ; Walking

Neurogenic tumors located in the posterior mediastinum, generally require surgery which be confronted with greater risk,therefore, to design the best surgical approach and surgical methods is essential. A 67-year-old female patient had pharyngeal foreign body sensation and dysphagia. Thyroid ultrasound showed the right thyroid had a little nodule, and the left thyroid had a hypoechoic lumps. Neck enhanced CT showd mediastinal mass, esophageal tumor origin or stromal tumor? We used the jugular approach to resect the tumor which eventually diagnosed as schwannoma. The meditational benign tumor with an envelope easy to peel can employ the jugular approach to resect it completely.
Aged ; Female ; Humans ; Jugular Veins ; Mediastinal Neoplasms ; surgery ; Mediastinum ; pathology ; Neck ; surgery ; Neurilemmoma ; surgery

Schwannomas are the most common benign nerve sheath tumors originating in Schwann cells. With special conditions like neurofibromatosis type 2 or entity called schwannomatosis, patients develop multiple schwannomas. But in clinical setting, distinguishing schwannomatosis from neurofibromatosis type 2 is challengeable. We describe 58-year-old male who presented with severe neuropathic pain, from schwannomatosis featuring multiple schwannomas of spine and trunk, and underwent surgical treatment. We demonstrate his radiologic and clinical findings, and discuss about important clinical features of this condition. To confirm schwannomatosis, we performed brain magnetic resonance imaging, and took his familial history. Staged surgery was done for pathological confirmation and relief of the pain. Schwannomatosis and neurofibromatosis type 2 are similar but different disease. There are diagnostic hallmarks of these conditions, including familial history, pathology, and brain imaging. Because of different prognosis, the two diseases must be distinguished, so diagnostic tests that are mentioned above should be performed in caution.
Brain ; Diagnostic Tests, Routine ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Nerve Sheath Neoplasms ; Neuralgia ; Neurilemmoma* ; Neurofibromatosis 2 ; Neuroimaging ; Pathology ; Prognosis ; Schwann Cells ; Spine*