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OBJECTIVE: Mitotically active cellular fibroma (MACF) of the ovary, characterized by relatively high mitotic activity without severe atypia, was first described in the WHO classification in 2014. However, due to its rarity, the clinicopathological characteristics of ovarian MACF have not been established. This study was performed to describe the clinical, radiological, and pathological features of MACF by analyzing 11 cases of ovarian MACF. MATERIALS AND METHODS: Between 2015 and 2022, 11 patients with ovarian MACFs underwent surgical treatment at our institution. Clinicopathologic data of the patients were retrospectively reviewed from their medical records. RESULTS: Median patient age was 53.7 years (range 21-77 years), and median tumor diameter was 7.8 cm (range 4.3-14.0 cm). Preoperative CA125 was elevated in 4 cases. Four of the eleven patients had abdominal pain, and two presented with vulvar pain or a palpable abdominal mass, respectively. Preoperative radiological impressions included fibroma, fibrothecoma, stromal tumor, and cystadenocarcinoma. A laparoscopic approach was adopted in 7 cases (64%). Intraoperative frozen section was performed in 5 patients, and all demonstrated the presence of a benign, fibromatous stromal tumor. Three patients underwent fertility-sparing surgery, including laparoscopic ovarian cystectomy and unilateral salpingo-oophorectomy. Median follow-up was 37.7 months (range 2-84 months), and no patient experienced disease relapse or died of their disease. CONCLUSION: This study shows that ovarian MACF has a benign clinical course. Fertility-sparing surgery provides a safe therapeutic option for MACF, which can be managed safely by laparoscopy. Imaging findings and final pathological diagnosis were not well matched. Intraoperative frozen section is important for determining surgical extent in mitotically active cellular fibroma of the ovary.

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Ovarian sex cord-stromal tumours (SCSTs) present diagnostic difficulties during frozen section (FS) consultations due to their diverse morphology. This study aimed to evaluate the accuracy of FS evaluation of SCSTs in our institution, as well as to examine the reasons leading to incorrect FS diagnosis. Cases mimicking SCSTs and diagnosed as such during FS were also highlighted. We analysed 121 ovarian SCST cases and their mimics which underwent FS consultations over a 10-year period, to evaluate FS accuracy, reasons for deferrals and discrepancies. FS diagnoses were concordant, deferred and discrepant compared to the final diagnosis in 50 (41.3%), 39 (32.2%) and 32 (26.5%) cases, respectively. Major discrepancies (9/121, 7.4%) were mostly related to the diagnosis of adult granulosa cell tumour (AGCT). A fibromatous AGCT was misinterpreted as fibroma on FS, while a cystic AGCT was called a benign cyst. Conversely, a mesonephric-like adenocarcinoma, a sertoliform endometrioid carcinoma and a thecoma were misinterpreted as AGCT on FS. Another discrepant case was a Krukenberg tumour with prominent fibromatous stroma in which malignant signet ring cells were overlooked and misinterpreted as fibroma. Minor discrepancies were primarily associated with fibroma (21/23, 91.3%), wherein minor but potentially impactful details such as cellular fibroma and mitotically active cellular fibroma were missed due to sampling issues and misinterpretation as leiomyoma. FS evaluation for ovarian SCSTs demonstrated an overall accuracy of 78.5%, 81.0% and 81.8% for benign, uncertain/low malignant potential and malignant categories, respectively. There was no FS-related adverse clinical impact in all cases with available follow-up information (120/121 cases). Intraoperative FS evaluation of ovarian SCSTs is challenging. A small number of cases were misinterpreted, with AGCTs being the primary group where errors occur. Awareness of common diagnostic pitfalls and difficulties, alongside application of a stepwise approach, including (1) obtaining comprehensive clinical information, (2) thorough macroscopic examination and directed sampling, (3) meticulous microscopic examination with consideration of pitfalls and mimics, (4) effective communication with surgeons in difficult cases, and (5) consultation of subspecialty colleagues in challenging cases, will enhance pathologists' reporting accuracy and management of such cases in the future.

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OBJECTIVE: Elastofibroma dorsi (ED) is a rare benign tumor located in the subscapular region. The aim of this study was to evaluate our clinical findings, surgical approach, and management of ED patients based on single-center data with the relevant literature. METHODOLOGY: A retrospective evaluation was conducted on 20 patients who were operated on for ED. RESULTS: Of the 16 (80%) female patients and 4 (20%) male patients, the main complaint was swelling (80%), and 10 cases (50%) had unilateral involvement. All patients were operated on using standard surgical procedures. Despite a long follow-up period (6-53 months, mean of 26.6 months), no recurrences were observed. Two patients (10%) required simple needle aspiration due to post-operative seroma, and one patient, due to infection, required evacuation (5%). CONCLUSION: Although rare, ED should not be overlooked in patients with swelling in the back region. Our data suggests that surgery can be safely performed in such patients after a clinical and radiological diagnosis of ED has been established.

OBJETIVO: Evaluar los hallazgos clínicos, el enfoque quirúrgico y el manejo de los pacientes con urgencias a partir de los datos de un solo centro y la literatura relevante. MÉTODO: Se realizó una evaluación retrospectiva de 20 pacientes que fueron operados de ED. RESULTADOS: En los 16 (80%), pacientes del sexo femenino y cuatro (20%) del sexo masculino, la queja principal fue la tumefacción (80%) y 10 casos (50%) tuvieron afectación unilateral. Todos los pacientes fueron operados utilizando procedimientos quirúrgicos estándar. Con un largo periodo de seguimiento (6-53 meses, media de 26.6 meses), no se observaron recurrencias. Dos pacientes (10%) requirieron aspiración con aguja simple por seroma posoperatorio y un paciente (5%) requirió evacuación por infección. CONCLUSIONES: Aunque es raro, el ED no debe pasarse por alto en pacientes con hinchazón en la región de la espalda. Nuestros datos sugieren que la cirugía se puede realizar de manera segura en estos pacientes después de haber establecido el diagnóstico clínico y radiológico de ED.

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BACKGROUND: The aim of this study was to elucidate the histogenesis and genetic underpinnings of fibromatosis-like undifferentiated gastric carcinoma (FLUGC), a rare pathological entity. METHOD: Through a detailed analysis of seven cases, including histopathological evaluation, CTNNB1 gene mutation screening, human epidermal growth factor receptor 2 (HER2) protein level quantification, and HER2 gene amplification assessment to identify the pathological and molecular characteristics of FLUGC. RESULTS: Of the seven patients in this study, five were male and two were female (age: 39-73 years). Four patients presented with lesions in the gastric antrum and three had lesions in the lateral curvature of the stomach. Histopathologically, over 90% of the tumor consisted of aggressive fibromatosis-like tissue, including proliferating spindle fibroblasts and myofibroblasts and varying amounts of collagenous fibrous tissues. Undifferentiated cancer cells, accounting for less than 10%, were dispersed among the aggressive fibromatosis-like tissues. These cells were characterized by their small size and were relatively sparse without glandular ducts or nested mass-like structures. Immunophenotyping results showed positive expression of CKpan, CDX2, villin, and p53 in undifferentiated cancer cells; positive expression of vimentin in aggressive fibromatosis-like tissue; positive cytoplasmic expression of ß-catenin; and focal cytoplasmic positive expression of smooth muscle actin (SMA). Genetic analysis did not reveal any mutations in the CTNNB1 gene test, nor was there amplification in the HER2 gene fluorescence in situ hybridization (FISH) test. Additionally, the Epstein-Barr encoding region (EBER) of in situ hybridization was negative; and the mismatch repair (MMR) protein was positive. Programmed cell death-1 (PD-1) was < 1-5%; programmed cell death ligand 1 (PD-L1): TPS = 1-4%, CPS = 3-8. CONCLUSION: The study highlights the significance of CTNNB1, HER2, EBER, and MMR as pivotal genetic markers in FLUGC, underscoring their relevance for diagnosis and clinical management. The rarity and distinct pathological features of FLUGC emphasize the importance of accurate diagnosis to prevent underdiagnosis or misdiagnosis and to raise awareness within the medical community.

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BACKGROUND: Jaffe-Campanacci syndrome is a rare syndrome, characterized by multiple non-ossifying fibromas (NOF) and cafe-au-lait patches. The name was coined in 1982 by Mirra after Jaffe who first described the case in 1958. Although it's suggested there is a relation with Neurofibromatosis type 1, there is still no consensus on whether Jaffe-Campanacci syndrome is a subtype or variant of neurofibromatosis-1(NF-1). CASE PRESENTATION: In this article, we present a case series of 2 patients. The first case is a 13-year-old male with Jaffe-Campanacci syndrome who presented with a distal femur fracture. His father had positive features of both Jaffe-Campanacci syndrome and NF-1, while his sister only had features of NF-1, so we presented both. CONCLUSION: Jaffe-Campanacci has a clear relationship with type 1 neurofibromatosis, which still has to be genetically established. Due to the presence of several large non-ossifying fibromas of the long bones, it is linked to a significant risk of pathological fractures. We concur with previous authors, that an osseous screening program should be performed for all patients with newly diagnosed type 1 neurofibromatosis, to identify non-ossifying fibromas and assess the potential for pathological fracture. Moreover, siblings of patients with NF-1 should be screened for multiple NOFs that may carry a high risk of pathological fractures.

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OBJECTIVES: This study aimed to review a 35-year experience with chondromyxoid fibroma at our institution. PATIENTS AND METHODS: The study retrospectively analyzed the records of 31 consecutive patients (17 males, 14 females; mean age: 30.5±15.7 years; range, 6 to 63 years) with chondromyxoid fibroma who were treated between January 1988 and December 2021. The clinical and radiological characteristics of lesions, tumor volume, and recurrence rates were assessed using the tumor archive of the hospital. RESULTS: The mean follow-up duration was 65.9±42.0 months. Pelvis, proximal tibia, and distal femur were the most common sites of localization. The initial surgical treatment was performed on 27 patients at our clinic, while four patients were referred to the clinic after recurrence. The overall recurrence rate was 16.1%. Intralesional curettage was applied to 21 (77.8%) out of 27 patients. The cavity created after curettage was filled with bone graft (autograft or allograft) in 15 (55.5%) cases. Bone cement was applied in four (14.8%) cases. Resection was applied to five (18.5%) patients. In two (7.4%) cases, intralesional curettage alone was performed. One of these two patients experienced recurrence, resulting in a recurrence rate of 50% in this patient group. No recurrence was observed in other treatment groups. CONCLUSION: Intralesional curettage and filling the defect with bone graft or cement were effective for local control in most cases. Curettage alone was associated with high recurrence rates.

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Plexiform fibromyxoma (PF), also referred to as plexiform angiomyxoid myofibroblast tumor, is an exceedingly rare mesenchymal neoplasm primarily affecting the stomach. Herein, we present a case of PF diagnosed in a 71-year-old male with a history of lung cancer, initially suspected to have a gastrointestinal stromal tumor (GIST) of the stomach, who subsequently underwent subtotal gastrectomy. The histopathological and molecular features of the tumor, including mutations in ABL1, CCND1, CSF1R, FGFR4, KDR, and MALAT1-GLI1 fusion, are elucidated and discussed in the context of diagnostic, prognostic, and therapeutic considerations.

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A 35-year-old patient presented with a painless, broad-based exophytic lesion in the buccal interdental region between teeth 13 and 14. Despite oral hygiene efforts the lesion persisted for around one year. Radiology excluded bone involvement, and histopathology after excision confirmed a fibromatous epulis, which is characterized by collagen-rich connective tissue. There was no recurrence within one-year follow-up. Surgical removal proved to be efficient.

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The clinical differences between odontogenic myxoma (OM) and odontogenic myxofibroma (OMF), and the clinical significance of their classifications, remain unclear. This study reviewed the clinicopathological characteristics of patients with OM or OMF and evaluated the fibrous component of the specimens. Medical records of 21 patients with OM or OMF who underwent tumour resection were reviewed. The percentage of fibrous tissue on the representative sections was evaluated using haematoxylin and eosin- and Masson's trichrome-stained specimens. Histopathological diagnoses included 11 OMs and 10 OMFs with no tumour recurrence except for two cases in which the dredging method was applied. More cortical bone perforation was observed in OM than in OMF cases, without significant differences. Location-locularity and apparent diffusion coefficient value (ADC)-cortical bone perforation were significantly correlated in all OM and OMF cases. The percentage of fibrous tissue in specimens showed bimodal distribution bordered by 45%. There was a significant association between diagnosis based on 45% fibrous tissue criterion and the final pathological diagnosis. Our study showed a tendency for cortical bone perforation in OM compared to OMF and correlation between ADC and cortical bone perforation. According to the histopathological analyses, the fibrous component of each case was bimodal with 45%, which may be a criterion to distinguish between OM and OMF. Accumulating knowledge, such as significant differences in prognosis, may allow for minimal surgical treatment options based on the diagnosis according to this novel histopathological criterion.

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Superficial fibromas are a group of mesenchymal spindle cell lesions with pathomorphological heterogeneity and diverse molecular backgrounds. In part, they may be indicators of an underlying syndrome. Among the best-known entities of superficial fibromas is Gardner fibroma, a plaque-like benign tumor, which is associated with APC germline mutations and occurs in patients with familial adenomatosis polyposis (Gardner syndrome). Affected patients also have an increased risk to develop desmoid fibromatosis (DTF), a locally aggressive neoplasm of the deep soft tissue highly prone to local recurrences. Although a minority of DTFs occur in the syndromic context and harbor APC germline mutations, most frequently their underlying molecular aberration is a sporadic mutation in Exon 3 of the CTNNB1 gene. Up to date, a non-syndromic equivalent to Gardner fibroma carrying a CTNNB1 mutation has not been defined. Here, we present two cases of (sub-)cutaneous tumors with a hypocellular and collagen-rich Gardner fibroma-like appearance and pathogenic, somatic CTNNB1 mutations. We aim to differentiate these tumors from other fibromas according to their histological appearance, immunohistochemical staining profile and underlying somatic CTNNB1 mutations. Furthermore, we distinguish them from locally aggressive desmoid fibromatosis regarding their biological behavior, prognosis and indicated therapeutic strategies. Consequently, we call them CTNNB1-mutated superficial fibromas as a sporadic counterpart lesion to syndromic Gardner fibromas.

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INTRODUCTION: Ischaemic stroke results from a sudden interruption of cerebral blood flow, often caused by thrombosis, embolism or hypoperfusion. Rarely, cardiac tumours are the cause of an embolic event. We report on an 80-year-old woman with a cerebral stroke. Successful thrombectomy revealed a histopathologically confirmed papillary fibroelastoma as the embolic source. Although it was not detectable on initial echocardiography, the tumour was discovered by transesophageal echocardiography. This case emphasises the importance of considering primary cardiac tumours as potential sources of emboli in ischaemic stroke. Keywords.

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PURPOSE: To explore the frequency of superior scapular elastofibroma dorsi in a large patient series with elastofibroma dorsi. METHODS: 136 chest CTs from January 2016 to July 2022 reporting elastofibroma dorsi were retrospectively analyzed. Three radiologists assessed the number, size, and location of elastofibroma dorsi. Continuous variables underwent two-tailed t-tests with p < 0.05. Inter-observer agreement was assessed by using Cohen's Kappa values. RESULTS: In 136 patients (mean age, 75.9 +/- 9.8 years; 117 female), 330 elastofibroma dorsi were found. Six (4.4 %) patients had single, 87 (64 %) double, 22 (16.2 %) triple and 21 (15.4 %) quadruple lesions. All single and double lesions were in the inferior scapular regions. 43 (31.6 %) patients had superior scapular lesions in addition to inferior scapular elastofibroma dorsi. Inferior scapular elastofibroma dorsi was significantly larger than superior scapular elastofibroma dorsi. The probability of a right superior lesion was significantly higher in patients with a larger right inferior lesion. Inter-observer agreement was very good for experienced radiologist (κ = 94.1) and good for other radiologists (κ = 79.4 and κ = 78). CONCLUSION: In contrast to current belief, superior scapular elastofibroma dorsi accompanying the typical inferior scapular lesions is not uncommon and can even manifest bilaterally. To the best of our knowledge, this is the first case series reporting prevalence of quadruple elastofibroma dorsi.

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BACKGROUND: Ovarian fibromas are benign tumors that can present peculiar morphological features not studied sufficiently. MATERIAL AND METHODS: In this retrospective study, 75 consecutive cases of ovarian fibroma were morphologically compared with 46 thecomas, 16 granulosa cell tumors, and 5 sclerosing stroma tumors for the following factors: the growth pattern as diffuse or nodular, the presence of hyaline plaques, necrosis, keloid-like sclerosis, calcifications, cystic degeneration, fibrous or edematous stroma, prominent vascularity, lutein cells, cellularity, scant or abundant cytoplasm, prominent cell membranes, nuclear grooves, atypia, and mitotic activity. RESULTS: The tumors differed significantly in terms of hyaline plaques presence, nuclear grooves, growth pattern, stroma type, tumor cellularity, cytoplasm, prominence of cell membranes, atypia, mitotic activity, and prominent vascularity. CONCLUSION: Ovarian fibromas can present some maybe unexpected features rather frequently, such as cystic degeneration, hyaline plaques, prominent vascularity, increased cellularity, and some mitotic activity, thus their presence should not always prompt to an alternative diagnosis.

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