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Eight patients with localized pigmented villonodular synovitis (LPVNS) of the knee were treated with arthroscopic and open techniques, with diagnosis confirmed by histological examination. Average patient age was 29 years (range: 13-50 years). At arthroscopy, all lesions except one were in the anterior compartment of the involved knee. Treatment consisted of complete local excision with partial synovectomy. This procedure was completed arthroscopically in seven patients. No recurrence was reported at average 24-month follow-up (range: 12-33 months). Arthroscopy is a valuable tool in the diagnosis and treatment of LPVNS. [ABSTRACT FROM AUTHOR]

Abstract Introduction Gastrointestinal stromal tumors are rare tumors. They commonly metastasize within the abdominal cavity, particularly to the liver. Less commonly, metastases can be found in the bone. Case presentation We here present a case of metastasis to the scapula in a 54-year-old Caucasian male patient with an advanced gastrointestinal stromal tumor, which was subsequently successfully treated with resection and sunitinib. Conclusion The present study is, to the best of our knowledge, the second to describe scapular metastasis of a gastrointestinal stromal tumor. Our patient was treated by scapulectomy. The overwhelming majority of scapular tumors are metastases that arise from soft tissue, hepatocellular and thyroid tumors. Gastrointestinal stromal tumor metastasis occurs rarely. Scapular surgery can successfully provide local control of the disease. After the surgery, patients should continue with medical treatment.

Hirschsprung disease, a congenital disease characterized by the absence of ganglion cells, presents significant surgical challenges. Addressing a critical gap in intraoperative diagnostics, we introduce transformative artificial intelligence approach that significantly enhances the detection of ganglion cells in frozen sections. The data set comprises 366 frozen and 302 formalin-fixed-paraffin-embedded hematoxylin and eosin-stained slides obtained from 164 patients from 3 centers. The ganglion cells were annotated on the whole-slide images (WSIs) using bounding boxes. Tissue regions within WSIs were segmented and split into patches of 2000 × 2000 pixels. A deep learning pipeline utilizing ResNet-50 model for feature extraction and gradient-weighted class activation mapping algorithm to generate heatmaps for ganglion cell localization was employed. The binary classification performance of the model was evaluated on independent test cohorts. In the multireader study, 10 pathologists assessed 50 frozen WSIs, with 25 slides containing ganglion cells, and 25 slides without. In the first phase of the study, pathologists evaluated the slides as a routine practice. After a 2-week washout period, pathologists re-evaluated the same WSIs along with the 4 patches with the highest probability of containing ganglion cells. The proposed deep learning approach achieved an accuracy of 91.3%, 92.8%, and 90.1% in detecting ganglion cells within WSIs in the test data set obtained from centers. In the reader study, on average, the pathologists' diagnostic accuracy increased from 77% to 85.8% with the model's heatmap support, whereas the diagnosis time decreased from an average of 139.7 to 70.5 seconds. Notably, when applied in real-world settings with a group of pathologists, our model's integration brought about substantial improvement in diagnosis precision and reduced the time required for diagnoses by half. This notable advance in artificial intelligence-driven diagnostics not only sets a new standard for surgical decision making in Hirschsprung disease but also creates opportunities for its wider implementation in various clinical settings, highlighting its pivotal role in enhancing the efficacy and accuracy of frozen sections analyses., (Copyright © 2024 United States & Canadian Academy of Pathology. Published by Elsevier Inc. All rights reserved.)

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5-44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objectives: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a tertiary referral hospital from Turkey.Methods: The files between 1989 and 2015 of 80 patients with LCH were retrospectively analyzed., Results: During the 25 years, 80 patients were diagnosed with LCH. The median age at diagnosis was 53 months (2-180 months) and the median follow-up time of patients was 10 years and 9 months (24 months-25 years). Bone was the most frequently affected organ (n:60, 75%). Initially, 43 patients (54%) had single system (SS) disease, 20 patients (25%) had multisystem (MS) disease without risk organ involvement (MS-RO - ), and 17 patients (21%) had a multisystem disease with risk-organ involvement (MS-RO + ). The overall survival (OS) rate was 91%, and event-free survival (EFS) rate was 67% at 10 years. 10-year OS rate was lower for patients with MS-RO + (65%) when compared to those with, MS-RO - , and SS (100%, 97%, p value=<0.001). The overall survival rate was also lower in patients with lack of response to systemic chemotherapy on 12th week (p=<0.001), younger age (<2 years) at presentation (p=<0.02), skin involvement (<0.001) and lack of bone lesions at presentation (<0.001)., Discussion: In the group with MS-RO + , OS is significantly low compared to other groups. Further efforts are warranted to improve survival in MS-RO + patients., Competing Interests: Competing interests: The authors have declared that no competing interests exist.

Aim: To investigate the histopathologic changes in native bladder and gastrointestinal segment, the relation between histopathologic changes, type of operation and the period passed over operation in patients with bladder augmentation., Materials and Methods: Twenty consecutive patients were enrolled in this study. Histopathologic evaluation of the cystoscopic mucosal biopsies from native bladder and enteric augment was performed in all patients., Results: Active or chronic non-specific inflammation of various degrees was found in all specimens except two. Metaplastic changes were detected in 3 patients. Two patients had squamous metaplasia (one focal, one extensive) and one patient had intestinal metaplasia. All metaplastic changes were found in native bladder specimens. The type of augmentation in patients with metaplastic changes were ileocystoplasty and sigmoidocystoplasty. No signs of malignancy were detected in any patient., Conclusion: The complexity of the disorders requiring bladder augmentation does not let the surgeons to draw a clear line between different groups of complications including malignancy formation. However, due to challenging course of the augmentation procedure itself, surgeons should be well aware of the possibility of malignancy development., Competing Interests: Conflict of interest: None declared., (Copyright® by the International Brazilian Journal of Urology.)

Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four). Tumor size (n = 6), varied from 3.1 to 15 cm (average = 8.3). Histopathologically, most tumors were multilobular, characterized by epithelioid to round-shaped, malignant cells, along with spindle cells (three cases), "rhabdoid-like" cells (seven cases) and pleomorphic giant cells (single case). By immunohistochemistry, tumor cells were positive for S100 protein (11/11) (100%), EMA (3/7) (42.8%), pan CK(2/7) (28.5%), and HMB45 (1/11) (9%), while these were negative for Melan A (0/11) and INI1 (3/11), including a single tumor, displaying HMB45 positivity. BRAF V600E mutation was positive in 1/8 cases, that lacked melanocytic marker expression. All patients (n = 5) were treated by surgical resection. During follow-up (n = 8, median duration = 23 months), four patients developed tumor recurrences and four developed metastasis, mostly to lymph nodes (3). Finally, four patients were alive with disease, two were alive with no evidence of disease, and two patients died of disease. Epithelioid MPNSTs have a diverse histopathologic spectrum. Loss of INI1 is useful, including in identifying rare forms of epithelioid MPNST, displaying melanocytic differentiation. Most tumors are treated by surgical resection. Loss of INI1 and the presence of BRAF V600E mutation in some cases raises future possibility of exploring targeted therapy in those, rare epithelioid MPNSTs., (© 2017 APMIS. Published by John Wiley & Sons Ltd.)

Our aim in this study was to describe the clinical, morphological, and molecular profile of gastrointestinal stromal tumor (GIST) metastatic to bone. We analyzed the morphological, phenotypic, and molecular characteristics of seven cases, and in addition reviewed 17 cases from literature. Sequence analysis of KIT and PDGFRA genes was possible for six cases. For the GIST cases with bone metastasis, the most common primaries were small intestine (29%), stomach (25%), and rectum (21%). Sites of bone metastases were vertebrae (11), pelvis (8), femur (8), ribs (6), humerus (5), skull (3), scapula (1), and mandible (1). The size ranged from 1.5 to 13 cm (median, 3.8 cm). Bone metastases without involvement of any other organ were seen in 17% of the cases and were solitary in 14 (58%). Adjacent soft tissue involvement was present in nearly half of the patients. Bone metastasis was either manifest at the time of diagnosis (28%) or occurred after a mean period of 4.7 years (3 months-20 years). Morphologically, neoplastic cells were spindle in 67%, epithelioid in 13%, and mixed epithelioid and spindle in 20%. CD117, DOG1, and CD34 were positive in 88, 86, and 85% of the cases, respectively. KIT Exon 11 mutations were the most frequent gene alteration (78%), followed by KIT Exon 13 mutations. Of 17 of the cases with available follow-up information, 7 (41%) patients developed bone metastasis under imatinib therapy. Five patients (29%) died of disease within a mean of 17 months. Bone metastases from GIST are usually found in patients with advanced disease and typically present as lytic masses with occasional soft tissue involvement. We could not identify any KIT or PDGFRA alterations predisposing to bone metastasis.

Laser biostimulation in medicine has become widespread supporting the idea of therapeutic effects of photobiomodulation in biological tissues. The aim of this study was to investigate the biostimulation effect of laser irradiation on healing of cutaneous skin wounds, in vivo, by means of bioimpedance measurements and histological examinations. Cutaneous skin wounds on rats were subjected to 635 nm diode laser irradiations at two energy densities of 1 and 3 J/cm 2 separately. Changes in the electrical properties of the wound sites were examined with multi-frequency electrical impedance measurements performed on the 3rd, 7th, 10th, and 14th days following the wounding. Tissue samples were both morphologically and histologically examined to determine the relationship between electrical properties and structure of tissues during healing. Laser irradiations of both energy densities stimulated the wound healing process. In particular, laser irradiation of lower energy density had more evidence especially for the first days of healing process. On the 7th day of healing, 3 J/cm 2 laser-irradiated tissues had significantly smaller wound areas compared to non-irradiated wounds (p < 0.05). The electrical impedance results supported the idea of laser biostimulation on healing of cutaneous skin wounds. Thus, bioimpedance measurements may be considered as a non-invasive supplementary method for following the healing process of laser-irradiated tissues.

Background: Osteoid osteoma is an uncommon benign bone-forming tumor of the musculoskeletal system but it is the most common primary bone tumor of the carpal region. However, only seven cases of its pisiform involvement have been described., Case: We present a 19 year-old male patient with osteoid osteoma of the pisiform which initially could not be detected in any diagnostic study for long standing wrist pain. The patient was treated with excision and curettage. In 1 year of follow-up, the patient was asymptomatic without recurrence., Conclusion: In cases with long standing ulnar wrist pain, one should consider osteoid osteoma of the pisiform in differential diagnosis. If an OO is suspected, thin slice CT scan in initial evaluation would be valuable in preventing the patient from misdiagnosis or delay in diagnosis., (Copyright © 2016 Turkish Association of Orthopaedics and Traumatology. Production and hosting by Elsevier B.V. All rights reserved.)

Helicobacter Pylori was previously demonstrated at gastric patch after gastrocystoplasty and a possible relationship with acid-haematuria syndrome was established after symptomatic relief by medical treatment. We present the long term outcome of a male bladder exstrophy patient after gastrocystoplasty. There was past history of bladder perforation, acid haematuria syndrome and treatment of HP and recurrent urinary tract infections, noncompliance on regular follow-up and cadaveric renal transplantation. At the preoperative evaluation for renal transplantation HP was present in the biopsy samples collected during gastroscopy and cystoscopy. The significance of persistant HP after gastrocystoplasty in the long term follow-up was discussed.

The objective of this study was to investigate the effects of selective inducible nitric oxide synthase and neuronal nitric oxide synthase inhibitors on cartilage regeneration. The study involved 27 Wistar rats that were divided into five groups. On Day 1, both knees of 3 rats were resected and placed in a formalin solution as a control group. The remaining 24 rats were separated into 4 groups, and their right knees were surgically damaged. Depending on the groups, the rats were injected with intra-articular normal saline solution, neuronal nitric oxide synthase inhibitor 7-nitroindazole (50 mg/kg), inducible nitric oxide synthase inhibitor amino-guanidine (30 mg/kg), or nitric oxide precursor L-arginine (200 mg/kg). After 21 days, the right and left knees of the rats were resected and placed in formalin solution. The samples were histopathologically examined by a blinded evaluator and scored on 8 parameters. Although selective neuronal nitric oxide synthase inhibition exhibited significant (P = 0.044) positive effects on cartilage regeneration following cartilage damage, it was determined that inducible nitric oxide synthase inhibition had no statistically significant effect on cartilage regeneration. It was observed that the nitric oxide synthase activation triggered advanced arthrosis symptoms, such as osteophyte formation. The fact that selective neuronal nitric oxide synthase inhibitors were observed to have mitigating effects on the severity of the damage may, in the future, influence the development of new agents to be used in the treatment of cartilage disorders.

Objective: An increasing number of studies indicate that pepsin and bile acid cause damage to the ear, nose, and throat structures as a result of extraesophageal reflux. The aim of this study was to evaluate and compare the damaging effect of bile acids and pepsin on the middle ear mucosa., Material and Methods: Twenty-nine healthy rats were included in this study. The animals were divided into 5 groups. A single daily dose of 40 μmol/L chenodeoxycholic acid, 40 μg/mL pepsin, and saline were injected separately into the right middle ear of the rats. On day 30, all rats were decapitated, and formalin-fixed, paraffin-embedded samples of the middle ear both from the control and experimental rats were prepared. A semiquantitative analysis was performed., Results: Inflammatory response was seen in all middle ear mucosa of rats except control group 1. The degree of inflammatory response was higher in the bile acid group when compared with the other groups. Epithelial metaplastic changes with varying number of goblet cells were observed in both the bile acid- and pepsin-injected groups. These metaplastic changes were also higher in the bile acid-induced group than in the pepsin-injected group., Conclusions: This is the first study on the middle ear mucosal damage of both pepsin and bile acid. Our results demonstrate that bile acids were associated with more extensive mucosal injury at pH 7 in comparison to pepsin in a rat animal model. Inflammatory response and metaplastic changes may play an important role in the etiology of middle ear pathologies.

Aims and Background: There is limited data regarding outcomes of Ewing's sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing's sarcoma family of tumors., Methods and Study Design: From 1992-2008, 90 adolescents and adults with Ewing's sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics., Results: The median age was 21 years (range, 13-50). Most patients (74%) were >17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis., Conclusions: We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

Purpose: Alterations in patellar height after high tibial osteotomy are found in many instances. Fibrosis of the tendon is implicated as the cause of the mechanism of patella lowering. This study aimed to determine the relationship between the position of the patella and the histopathological findings at the patellar tendon after high tibial osteotomy., Methods: Nineteen knees in seventeen patients who were consecutively hospitalised for implant extraction are studied. All of the patients had previously undergone closing wedge osteotomy by the same surgeon at the same department. The median follow-up time is 15 months (range: 11-35). Five patients who all underwent high tibial osteotomy at the same time are also included in the study as a control group for histopathological evaluation. All of the patients are evaluated radiologically, patellar tendon biopsies are taken during the operation, and histopathological analyses are performed., Results: The shortening of the patellar tendon is statistically significant (P < 0.05). The severity of the vascularisation, inflammation, and fibrotic change observed at the distal part of the tendon is evident. However, there is no statistically significant correlation between these findings and the degree of shortening., Conclusions: The shortening of the tendon occurs as a result of adherence in the distal part of the tendon. It would appear that it is this shortening that causes the difficulties encountered during arthroplasty surgery of osteotomy patients, and not patella infera.