Malignant transformation of WHO grade I meningiomas after surgery or radiosurgery : systematic review and meta-analysis of observational studies.

Background:The incidence and clinical features of the malignant transformation of benign meningiomas are poorly understood. This study examined the risk of the malignant transformation of benign meningiomas after surgery or stereotactic radiosurgery.Methods:We systematically reviewed studies published between 1979 and 2019 using PubMed, Scopus, and other sources. We analyzed pooled data according to the PRISMA guideline to clarify the incidence rate of malignant transformation (IMT) and factors affecting malignant transformation in surgically or radiosurgically treated benign meningiomas.Results:IMT was 2.98/1000 patient-years (95% confidence interval [CI] = 1.9-4.3) in 13 studies in a single-arm meta-analysis. Although the evidence level of the included studies was low, the heterogeneity of the incidence was mostly explained by the tumor location. In meta-regression analysis, skull base tumors had a significantly lower IMT than non-skull base tumors, but no gender association was observed. IMT after radiosurgery in 9 studies was 0.50/1000 person-years (95% CI = 0.02-1.38). However, a higher proportion of skull base tumors, lower proportion of males, and lower salvage surgery rate were observed in the radiosurgery group than in the surgery group. The median time to malignant change was 5 years (interquartile range = 2.5-8.2), and the median survival after malignant transformation was 4.7 years (95% CI = 3.7-8) in individual case data.Conclusion:IMT of benign meningioma was significantly affected by the tumor location. Radiosurgery did not appear to increase IMT, but exact comparisons were difficult because of differences in study populations

Carcinoma with Triphasic Differentiation Arising from Inverted Papilloma in Sinonasal Sinus: A Rare Case with Molecular Characterization

Small cell neuroendocrine carcinoma (SNEC) is a rare subset of tumors in the sinonasal sinus. Combined tumors are exceedingly rare. Here, we describe a 65-year-old male with a mixed tumor of SNEC and sarcomatoid carcinoma arising in an inverted papilloma, containing squamous cell carcinoma in situ (SqCCis) in the sinonasal sinus. We evaluated the molecular characteristics of the two separate carcinoma components using next-generation sequencing. The patient presented with a nasal obstruction. Computed tomography showed a mass infiltrating the right ethmoid and maxillary sinuses. An excisional biopsy was performed. The tumor was found to have three morphologically distinct components. The first was SqCCis arising in an inverted papilloma, which was positive for cytokeratin and P40. The second consisted of nests of densely packed small round cells representing SNEC-positive neuroendocrine markers. The third was a solid sheet of anaplastic spindle cell proliferation, which was negative for the above markers. Oncogenic mutations such as FBXW7, TP53, and EGFR were detected in both SNEC and sarcomatoid carcinoma, and MYCL amplification was observed only in the SNEC component. This case highlights an extremely rare presentation of combined SNEC and sarcomatoid carcinoma arising from an inverted papilloma in the sinonasal sinus

Acne keloidalis nuchae in Asian: A single institutional experience.

Acne keloidalis nuchae, a type of folliculitis involving the back of the neck, is common in black men, although rare cases have been reported in patients of other ethnicities. We analyzed the clinicopathological features of acne keloidalis nuchae in 17 Asians. Patients' age at the time of presentation ranged from 20 to 69 years. Most patients experienced the disease over 2 years (range, 3 months-20 years); follow-up data were available for 11 (65%) patients (range, 2-95 months). Nine (53%) patients had comorbidities, but none had a history of other skin disease or a family history of acne keloidalis nuchae. Macroscopically, seven (41%) patients had multiple erythematous pustulopapular lesions, and 10 (59%) had a single large plaque. Histopathologically, deep scarring folliculitis containing naked hair shafts was identified. In all cases, inflammation was most severe in the upper two-thirds of the dermis, and the differences in pustulopapular and plaque lesions were more prominent in the peri-inflammation area. Of the seven patients with plaque lesions treated with steroids alone or steroids and cryotherapy, three experienced plaque reduction. Acne keloidalis nuchae occurring in Asian patients frequently present with typical clinicopathological features, and therefore in spite of very low incidence the diagnosis of this disease entity should be considered in idiopathic scarring folliculitis of the posterior neck

Osteosarcoma Arising in Fibrous Dysplasia of the Long Bone: Characteristic Images and Molecular Profiles

Fibrous dysplasia (FD) is a benign fibro-osseous lesion that frequently involves the craniofacial bones and femur. Malignant transformation of FD is a rare occurrence. We report a 38-year-old woman with osteosarcoma (OS) arising from FD of the femur. Magnetic resonance imaging revealed a well-defined lesion in the medulla of the femur, with cortical thinning and local bone destruction. Wide excision of the femur was performed. Grossly, the inner part of the mass was hard and tan-gray in color, and the outer part of the mass adjacent to the cortex showed myxoid discoloration with infiltrative borders. Microscopically, most of the tumor consisted of curvilinear woven bone and fibrous stroma with bland spindle cells, which transitioned to the outer portion of the tumor, showing cellular proliferation of pleomorphic cells with frequent mitotic activity. Next-generation sequencing revealed GNAS and TP53 mutations, and the diagnosis of OS arising from FD was strongly supported. This case highlights the characteristic images and molecular features of the malignant transformation of FD

Low KIBRA Expression Is Associated with Poor Prognosis in Patients with Triple-Negative Breast Cancer

Background and Objectives: Kidney and brain protein (KIBRA) is a protein encoded by the WW and C2 domain containing 1 (WWC1) gene and is involved in the Hippo signaling pathway. Recent studies have revealed the prognostic value of KIBRA expression; however, its role in breast cancer remains unclear. The aim of this study was to examine KIBRA expression in relation to the clinical and pathological characteristics of patients with breast cancer and to disease outcomes. Materials and Methods: We analyzed the expression of KIBRA and its correlation with event-free survival (EFS) outcomes in resected samples from 486 patients with breast cancer. Results: KIBRA expression was significantly different among the molecular subgroups (low KIBRA expression: luminal A, 46.7% versus 50.0%, p = 0.641; luminal B, 32.7% versus 71.7%, p < 0.001; human epidermal growth factor receptor 2 (HER2)-enriched, 64.9% versus 45.5%. p = 0.001; triple-negative, 73.6% versus 43.8%, p < 0.001). Low KIBRA expression was also associated with high nuclear grade (60.4% versus 37.8%, p < 0.001), high histologic grade (58.7% versus 37.0%, p < 0.001), and estrogen receptor (ER) negativity (54.2% versus 23.6%, p < 0.001). Low KIBRA expression was significantly associated with poor EFS (p = 0.041; hazard ratio (HR) 1.658; 95% confidence interval (CI), 1.015–2.709). Low KIBRA expression was an independent indicator of poor prognosis (p = 0.001; HR = 3.952; 95% CI = 1.542–10.133) in triple-negative breast cancer (TNBC). Conclusion: Low KIBRA expression was associated with higher histological grade, ER negativity and poor EFS of breast cancer. In particular, our data highlight KIBRA expression status as a potential prognostic marker for TNBC

Alveolar Soft Part Sarcoma of the Uterus: Clinicopathological and Molecular Characteristics

Alveolar soft part sarcoma (ASPS) is a rare malignant mesenchymal tumor mainly affecting adolescents and young adults, with a predilection for the deep soft tissues of extremities. ASPS arising in the female genital tract is extremely rare and poses a significant diagnostic challenge. We herein present two rare cases of ASPS, one occurring in the uterine corpus of a 27-year-old woman, and the other in the uterine cervix of a 10-year-old girl. We described the clinical, histological, immunophenotypical, and molecular characteristics of primary uterine ASPS. We performed immunostaining for transcription factor E3 (TFE3), human melanoma black 45 (HMB45), melan-A, desmin, pan-cytokeratin (CK), paired box 8 (PAX8), CD10, hormone receptors, and S100, and targeted RNA and DNA sequencing using commercially available cancer gene panel. In case 1, a 27-year-old woman was referred to our hospital after laparoscopic uterine myomectomy at an outside hospital. Imaging studies revealed a residual tumor in the uterine corpus. In case 2, a 10-year-old girl underwent surgical excision for the cervical mass and was diagnosed as having ASPS. She was then referred to our hospital for further management. Both patients received total hysterectomy. Histologically, they displayed characteristic histological features of ASPS. Strong nuclear TFE3 immunoreactivity, periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-shaped crystalloids or granules, and the identification of ASPSCR1–TFE3 fusion confirmed the diagnosis of ASPS in both cases. Lack of immunoreactivity for HMB45, melan-A, desmin, pan-CK, PAX8, and S100 excluded the possibility of perivascular epithelioid cell tumor, clear cell sarcoma, metastatic renal cell carcinoma, granular cell tumor, and paraganglioma. Our observations can help pathologists make an accurate diagnosis of uterine ASPS and suggest that pathologists should include primary uterine ASPS in the differential diagnosis of uterine mesenchymal tumors

Clinical features of acne keloidalis nuchae (AKN) in 17 Korean patients.

<p>Follicular papules progressed to keloidal plaques with loss of hair. (a) 20-year-old man developed a lesion 3 months ago. (b) 26-year-old man developed a lesion 2 years ago. (c) 54-year-old man developed a lesion 13 years ago.</p

Patient selection diagram.

<p>Patient selection diagram.</p

Clinical features of 17 Korean men with acne keloidalis nuchae.

<p>Clinical features of 17 Korean men with acne keloidalis nuchae.</p

Dural tail sign positive tumors: Points to make a differential diagnosis

Representative patients were treated with total surgical mass resection, and each tumor was histopathologically confirmed to have a secretory meningioma, intradural metastasis of gynecologic origin, and dural metastasis of lung origin. The imaging findings of these patients were inconclusive in differentiating meningioma from metastasis; hence, advanced magnetic resonance imaging (MRI) techniques were considered. Based on these reports, we studied how to differentiate typical meningiomas from atypical and malignant meningiomas and other dura-based malignant tumors using conventional computed tomography and MRI