Clinical, radiological, and pathological features of mitotically active cellular fibroma of ovary: A review of cases with literature review.

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.

A rare case of ventricular tachycardia caused by an intramyocardial fibroma with successful surgical resection in an adult.

BACKGROUND: Cardiac fibromas are extremely rare in adults. The preferred treatment is surgical resection, but antiarrhythmic medications or heart transplantation have also been used previously. The cardiac imaging, particularly MRI, can be useful to help delineate between primary cardiac tumors, and surgical factors such as the extent/size of the fibroma, involvement of the coronary arteries or mitral apparatus and amount of residual myocardium influence whether surgical resection is feasible. CASE PRESENTATION: A 42-year-old male presented with a wide-complex tachycardia, unresponsive to amiodarone. An echocardiogram was performed which showed a possible posterior wall mass. A cardiac MRI showed a well circumscribed lateral wall intracardiac fibroma, measuring 5.2 × 5.1 × 3.8 cm with preserved function. Surgical resection was successful, and he was discharged without a defibrillator. CONCLUSIONS: Cardiac fibromas are encapsulated tumors which do not infiltrate myocardium and should be surgically resected if possible.

Sclerosing Dermatofibrosarcoma Protuberans: A Possible Diagnosis When a Small Biopsy Shows Sclerotic Fibroma-Like Features.

ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a neoplasm of intermediate malignancy with high local recurrence rates. The sclerosing variant is characterized by the presence of sclerotic areas in more than 50% of tumors and is rarely reported. In this report, we describe a case of sclerosing DFSP with areas histopathologically resembling sclerotic fibroma, where the initial biopsy tissue presented a diagnostic challenge. A 77-year-old man presented with a 2-cm firm, erythematous nodule on the chest. A punch biopsy revealed plywood-like sclerosis and spindle cells with a vaguely storiform pattern. The tumor cells were positive for CD34. Sclerotic fibroma and DFSP were considered differential diagnoses. Subsequent excisional biopsy revealed that the tumor comprised 3 different histopathological areas: classic DFSP, sclerotic fibroma-like, and giant cell fibroblastoma-like. This report highlights the importance of reevaluating the clinical context and excision for further characterization.

Leiomioma intraligamentario degenerado como simulador de patología maligna de ovario. Reporte de un caso / Degenerated intraligamentary leiomioma as a simulator of malignant ovarian pathology. Report of a case

Los leiomiomas son tumores mesenquimatosos benignos que se presentan como la neoplasia uterina más común en mujeres en edad reproductiva. Según su crecimiento, se puede ver comprometido su aporte sanguíneo, ocurriendo cambios degenerativos por la carencia de oxígeno, lo cual le confiere un aspecto atípico que puede generar confusión con el diagnostico. Se describe caso de paciente de 47 años de edad, quien consulta por aumento progresivo de volumen de circunferencia abdominal de 7 meses de evolución, referida a la consulta de ginecología oncológica por gran masa tumoral retrouterina de probable origen ovárico con elevado riesgo de malignidad. Luego de realizar estudios preoperatorios es llevada a quirófano, obteniéndose como diagnóstico definitivo, leiomioma con degeneración hialina e hidrópica focal. Los leiomiomas con cambios degenerativos pueden simular tumores malignos de ovario, por lo cual deben ser considerados como un diagnóstico diferencial antes de intervenciones quirúrgicas por tumores abdominopélvicos de gran tamaño(AU)

Leiomyomas are benign mesenchymal tumors that occur as the most common uterine neoplasm in women of reproductive age. Depending on its growth, its blood supply may be compromised, causing degenerative changes due to lack of oxygen, which gives it an atypical appearance that may cause confusion with the diagnosis. The case of a 47-year-old patient is described, who consults for a progressive increase in the volume of abdominal circumference of 7 months of evolution, referred to the gynecology oncology consultation due to a large retrouterine tumor mass of probable ovarian origin with a high risk of malignancy. After performing preoperative studies, she was taken to the operating room, obtaining as a definitive diagnosis, leiomyoma with hyaline and focal hydropic degeneration. Leiomyomas with degenerative changes can simulate malignant ovarian tumors, which is why they should be considered as a differential diagnosis before surgical interventions for large abdominopelvic tumors(AU)

Elastofibroma Dorsi: experience of a single center.

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.

Histogenetic insights and genetic landscape of fibromatosis-like undifferentiated gastric carcinoma: a focused study.

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.

Intraoperative frozen section evaluation of ovarian sex cord-stromal tumours and their mimics: a study of 121 cases with emphasis on potential diagnostic pitfalls.

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.

Oral microbiome sequencing revealed the enrichment of Fusobacterium sp., Porphyromonas sp., Campylobacter sp., and Neisseria sp. on the oral malignant fibroma surface of giant panda.

Introduction: Microbial community composition is closely associated with host disease onset and progression, underscoring the importance of understanding host-microbiota dynamics in various health contexts. Methods: In this study, we utilized full-length 16S rRNA gene sequencing to conduct species-level identification of the microorganisms in the oral cavity of a giant panda (Ailuropoda melanoleuca) with oral malignant fibroma. Results: We observed a significant difference between the microbial community of the tumor side and non-tumor side of the oral cavity of the giant panda, with the latter exhibiting higher microbial diversity. The tumor side was dominated by specific microorganisms, such as Fusobacterium simiae, Porphyromonas sp. feline oral taxon 110, Campylobacter sp. feline oral taxon 100, and Neisseria sp. feline oral taxon 078, that have been reported to be associated with tumorigenic processes and periodontal diseases in other organisms. According to the linear discriminant analysis effect size analysis, more than 9 distinct biomarkers were obtained between the tumor side and non-tumor side samples. Furthermore, the Kyoto Encyclopedia of Genes and Genomes analysis revealed that the oral microbiota of the giant panda was significantly associated with genetic information processing and metabolism, particularly cofactor and vitamin, amino acid, and carbohydrate metabolism. Furthermore, a significant bacterial invasion of epithelial cells was predicted in the tumor side. Discussion: This study provides crucial insights into the association between oral microbiota and oral tumors in giant pandas and offers potential biomarkers that may guide future health assessments and preventive strategies for captive and aging giant pandas.

Jaffe-Campanacci syndrome; a case series and review of the literature.

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.

[Surgical treatment of chondromyxoid fibroma of ribs via thoracoabdominal access]. / Khirurgicheskoe lechenie khondromiksoidnoi fibromy reber iz kombinirovannogo torakoabdominal'nogo dostupa.

Surgical treatment of chondromyxoid fibroma of ribs is described. The diagnosis was verified after histological analysis. The patient underwent resection of multinodular tumor of anterolateral thoracic wall invading abdominal cavity via thoracoabdominal access. Postoperative period was uneventful. This case demonstrates the need for total en-bloc resection of tumor with surrounding tissues. Surgery is the only effective method for these patients.

Fibroma bilateral de ovario en mujer posmenopáusica. Reporte de un caso y revisión de la literatura / Bilateral ovarian fibroma in postmenopausal woman. Case report and literature review

Los fibromas ováricos son tumores benignos poco frecuentes, generalmente unilaterales, que se presentan principalmente en mujeres perimenopáusicas y posmenopáusicas. Los síntomas pueden variar y en algunos casos están asociados a ascitis y derrame pleural, conocido como síndrome de Meigs. Se presenta un caso de una paciente de 55 años de edad con antecedentes de hipertensión arterial y asma bronquial, quién acude a ginecólogo por dolor en fosa ilíaca derecha de tres meses de evolución. Se realizan diferentes estudios incluyendo ecografía y tomografía abdomino-pélvica, los cuales revelan una tumoración sólida bilateral de ovarios. Se realiza histerectomía abdominal total más salpingo-oforectomía bilateral y a pesar de sus características, el estudio histopatológico hace el diagnóstico de fibroma bilateral. La presentación clínica de los fibromas de ovario es inespecífica, por lo que suelen confundirse con patología maligna del ovario u otras patologías benignas de útero u ovario, constituyendo un reto para el diagnóstico preoperatorio(AU)

Ovarian fibromas are rare tumors that mainly occur in perimenopausal and postmenopausal women. Symptoms can vary, and in some cases, they may be associated with ascites and pleural effusion, known as Meigs Syndrome. The article presents the case of a 55-year-old patient with a history of hypertension and bronchial asthma, who visit a gynecologist due to pain in the right iliac fossa of three months duration. Various studies were performed, including ultrasound and abdominal and pelvic CT, which reveal the presence of solid bilateral ovarian tumors. The patient undergoes a total hysterectomy and bilateral salpingo-oophorectomy and despite it's characteristics, the histopathological study makes the diagnosis of bilateral ovarian fibroma with no signs of malignancy. The clinical presentation of ovarian fibromas is nonspecific, often leading to confusion with malignant ovarian pathology or other benign uterine or ovarian conditions, representing a challenge for preoperative diagnosis(AU)

Chondromyxoid fibroma: A retrospective evaluation of 31 cases.

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.

Incidental diagnosis of cardiac fibroma in an infant with acute viral bronchiolitis.

OBJECTIVE: Pediatric cardiac tumors are rare and, among them, 90% are benign. Cardiac fibroma is the second most frequent tumor, after rhabdomyoma. The objective of this study is to report a case of cardiac fibroma diagnosed incidentally in a patient admitted with acute viral bronchiolitis. CASE DESCRIPTION: A 5-month-old male infant was admitted to the pediatric emergency department with acute viral bronchiolitis requiring hospitalization. He presented a detectable respiratory syncytial virus in oropharyngeal swab, blood test with lymphocytosis and a chest radiography revealed cardiomegaly. Further cardiologic testing was performed detecting elevation of cardiac biomarkers, an electrocardiogram with alteration of left ventricular repolarization and echocardiogram with a heterogeneous mass in the left ventricular, with areas of calcification. A chest angiotomography suggested rhabdomyosarcoma or cardiac fibroma and a magnetic resonance showed a mass, with characteristics suggesting fibroma. The final diagnosis was made after two cardiac catheterizations for biopsy of the lesion, confirming cardiac fibroma by anatomopathological examination. Because the patient had moderate to severe systolic dysfunction, he was submitted to heart transplant. COMMENTS: One third of cardiac fibromas are asymptomatic, generally diagnosed late through tests ordered for other reasons. The gold-standard test for definitive diagnosis is biopsy. Cardiac fibroma usually does not present spontaneous regression and, in most cases, partial or total surgical resection is necessary. When tumors are unresectable, heart transplantation should be indicated. It is essential to have detailed characterization of the cardiac mass to establish the most appropriate therapeutic approach for each patient.

[Fibromatous Epulis: A Clinical Case Report].

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.

Retrospective analysis of odontogenic myxoma and odontogenic myxofibroma in the oral and maxillofacial region: a fibrous tissue-related differentiation.

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.

Superficial fibromas with CTNNB1 mutation.

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.