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Revista Portuguesa de Cirurgia... 2017Papillary fibroelastoma is one of the most common types of primary cardiac tumour. Though a rare pathology, its importance relates to its form of presentation, with...
INTRODUCTION
Papillary fibroelastoma is one of the most common types of primary cardiac tumour. Though a rare pathology, its importance relates to its form of presentation, with stroke and sudden death, and the questions regarding its management.
OBJECTIVES
To review the casuistic of cardiac tumours in our service, in special the papillary fibroelastomas.
METHODS
clinical case series overviewing the period from 1st January 2008 to 30th September 2017. We analysed all patients submitted to cardiac surgery due to cardiac masses or tumours and verified all the pathology results to confirm the diagnosis.
RESULTS
In the period selected, around 6500 surgeries were performed. Of those, 59 patients were operated on due to cardiac tumours. 81.4% were myxomas, 13.6% were papillary fibroelastomas, 3.4% were cardiac sarcomas and 1.7% were metastasis. Of the patients that had papillary fibroelastomas (n=8), 5 were male, and the mean age was 51.5 ± 16.2 years. 62.5% (n=5) had origin in the aortic valve and 37.5% (n=3) in the mitral valve. Regarding presentation, 3 patients presented with stroke, 2 patients had episodes of syncope and 3 patients were asymptomatic. After surgery, all patients remained asymptomatic and there was no evidence of recurrence. At the end of follow-up all patients were alive.
CONCLUSION
Although a rare pathology, papillary fibroelastomas remain important due to their potential for embolization and cardiac chamber obstruction, therefore should be primarily treated with surgery.
Topics: Adult; Aged; Echocardiography, Transesophageal; Female; Fibroma; Heart Neoplasms; Heart Valve Diseases; Humans; Male; Middle Aged; Mitral Valve; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 29701392
DOI: No ID Found -
Cardiology in the Young Apr 2024Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent...
Left ventricular tumour is a rare condition in children. The causes include vegetations, thrombus, and fibroma. 2-year-old asymptomatic female presented with an innocent heart murmur at 6 months of age. Subsequent follow-ups at 18 months of age showed left ventricular mass. Surgical pathology revealed "nodular fasciitis." This type of tumour has never been described in the heart before.
Topics: Child; Humans; Female; Child, Preschool; Fasciitis; Heart Neoplasms; Fibroma; Heart Ventricles; Heart Murmurs
PubMed: 38282536
DOI: 10.1017/S1047951124000052 -
Journal of Cutaneous Pathology Jan 2021Histopathologically, scars can mimic superficial fibromatoses. Superficial fibromatoses are known to show nuclear β-catenin immunoexpression, although the tumor types...
BACKGROUND
Histopathologically, scars can mimic superficial fibromatoses. Superficial fibromatoses are known to show nuclear β-catenin immunoexpression, although the tumor types do not harbor CTNNB1 or APC alterations. This study aimed to evaluate nuclear β-catenin immunoexpression in scars compared to that in superficial fibromatoses.
METHODS
Immunostaining with an anti-β-catenin antibody, clone 14, was performed on 8 superficial fibromatoses and 22 scars. The extent of β-catenin nuclear staining was classified as negative (<10%), focally positive (10-49%), or diffusely positive (50-100%). β-catenin staining intensity was semi-quantitatively graded as weak, moderate, or strong.
RESULTS
In 21 (95%) scars, nuclear β-catenin immunoexpression was detected in fibroblasts/myofibroblasts, with mainly diffuse (16/21) and moderate (14/21) to strong (5/21) staining. In contrast, seven (88%) of the eight superficial fibromatoses expressed β-catenin in the nuclei of the lesional spindle cells, at varying levels of staining intensity. Fibroblasts in normal papillary dermis always showed nuclear β-catenin expression to varying degrees but those in the reticular dermis did not.
CONCLUSIONS
Scars typically exhibit nuclear β-catenin expression similar to that in superficial fibromatoses. Thus, β-catenin immunohistochemistry is not suitable for distinguishing superficial fibromatoses from scars.
Topics: Biomarkers; Cell Nucleus; Cicatrix; Diagnosis, Differential; Fibroma; Humans; Immunohistochemistry; beta Catenin
PubMed: 32652763
DOI: 10.1111/cup.13806 -
In Vivo (Athens, Greece) 2021Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous... (Review)
Review
Desmoplastic fibroblastoma (also known as collagenous fibroma) is an uncommon benign fibroblastic/myofibroblastic neoplasm that primarily arises in the subcutaneous tissue of upper extremity. Magnetic resonance imaging reveals a well-defined mass in intimate association with dense connective tissue and prominent low signal intensity on all pulse sequences. Peripheral and septal enhancement is usually seen after intravenous contrast. Histologically, the lesion is paucicellular and consists of spindle to stellate-shaped cells embedded in a collagenous or myxocollagenous stroma with low vascularity. Diffuse and strong nuclear immunoreactivity for FOS-like antigen 1 seems to be characteristic of desmoplastic fibroblastoma. Cytogenetic studies have demonstrated the presence of 11q12 rearrangements and an identical t(2;11)(q31;q12) translocation. This review provides an updated overview of the clinical, radiological, histological, cytogenetic and molecular genetic features of desmoplastic fibroblastoma and discusses the relationship to fibroma of tendon sheath.
Topics: Fibroma; Fibroma, Desmoplastic; Humans; Magnetic Resonance Imaging; Soft Tissue Neoplasms; Translocation, Genetic
PubMed: 33402451
DOI: 10.21873/invivo.12233 -
Kardiologia Polska 2022
Topics: Aortic Valve; Cardiac Papillary Fibroelastoma; Fibroma; Heart Neoplasms; Humans
PubMed: 35442510
DOI: 10.33963/KP.a2022.0102 -
Journal of the American Podiatric... Mar 2022Ledderhose disease (plantar fibromas) is histologically related to Dupuytren disease, which has been successfully treated for years with radiotherapy. Many conservative...
Ledderhose disease (plantar fibromas) is histologically related to Dupuytren disease, which has been successfully treated for years with radiotherapy. Many conservative treatments have been advanced for plantar fibromas, including accommodative orthotic devices, which help but do not cure the disease. Surgery is considered the mainstay of treatment for this malady, but the failure rate has been as high as 100%, depending on the type of fasciectomy. Radiotherapy is a new, exciting modality that has shown promising results for treating plantar fibromas.
Topics: Fasciotomy; Fibroma; Fibromatosis, Plantar; Foot Diseases; Humans
PubMed: 35324461
DOI: 10.7547/19-008 -
The British Journal of Oral &... Jun 2024The clinical differences between odontogenic myxoma (OM) and odontogenic myxofibroma (OMF), and the clinical significance of their classifications, remain unclear. This...
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.
Topics: Humans; Odontogenic Tumors; Female; Male; Retrospective Studies; Adult; Middle Aged; Myxoma; Fibroma; Aged; Adolescent; Young Adult; Diagnosis, Differential
PubMed: 38702226
DOI: 10.1016/j.bjoms.2024.02.003 -
The Annals of Thoracic Surgery Apr 2022Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior...
BACKGROUND
Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. We aimed to determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence.
METHODS
We retrospectively identified all patients from a single center with pathologically proven PFE, treated from January 1995 through December 2018. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. We compared echocardiographic images obtained intraoperatively (after excision) and at dismissal with those of the most recent examination to assess the possibility of PFE recurrence.
RESULTS
We included 98 patients in the study. The mean duration of follow-up was 5.4 (SD 3.7) years (range, 1 to 17); the median duration of follow-up was 4.3 years (interquartile range, 1.9 to 7.7). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically reexcised, and pathologic analysis showed that two were recurrent PFEs and one was a Lambl excrescence. Initial clinical presentation of stroke or transient ischemic attack was more common for the recurrence group (for the first PFE) than for the nonrecurrence group (83% vs 26%; P < .001).
CONCLUSIONS
Contrary to findings from previous studies, PFEs do recur after surgical excision. These findings emphasize the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses.
Topics: Cardiac Papillary Fibroelastoma; Echocardiography; Fibroma; Heart Neoplasms; Humans; Retrospective Studies
PubMed: 34022212
DOI: 10.1016/j.athoracsur.2021.03.114 -
Seminars in Thoracic and Cardiovascular... 2022Papillary fibroelastomas (PFE) are benign neoplasms, mostly located on valvular surfaces with high embolic potential. This study presents a 27-year single institutional...
Papillary fibroelastomas (PFE) are benign neoplasms, mostly located on valvular surfaces with high embolic potential. This study presents a 27-year single institutional experience on surgical treatment of PFE in an adult patient- cohort with long-term follow-up. This study was approved by the institutional review board. Date and number of IRB approval: 11/23/2017, Institutional Review Board approval number A2014-0149. The need for individual patient consent was waived. We retrospectively evaluated all patients who underwent cardiac surgery for suspected space-occupying lesions in the observation period between June 1991 and June 2018 at our hospital. Clinicopathological features, imaging characteristics, surgical procedures and disease outcome were analyzed. 120 patients were diagnosed with various primary/secondary cardiac tumors and histology confirmed 21 PFEs were found in 16 patients. There was no significant age difference between patients with valvular vs nonvalvular PFEs (P = 0.26). Valvular lesions were found in aortic valve (n = 6), mitral valve (n = 2) and tricuspid valve (n = 1). Nonvalvular PFEs were found in right atrium (n = 2), left ventricle (n = 2), left atrial appendage (n = 2) and aortic wall (n = 1). Valvular lesions were significantly smaller in size compared to non-valvular lesions (P = 0.0013). Left-side PFEs were associated with a high embolization episodes (10/13 patients, 77%) not related to the size. One patient died in-hospital. All other patients were discharged out of the hospital postoperative. Follow-up was performed regularly for a median of 2.8 years (range 0.1-11 years) postoperative. Nonvalvular PFE tended to be larger in size and at least when located on the left sided heart had equally high propensity to embolize compared to valvular PFE. We strongly advocate surgical excision in all left-sided PFE.
Topics: Adult; Cardiac Papillary Fibroelastoma; Fibroma; Heart Neoplasms; Humans; Retrospective Studies; Treatment Outcome
PubMed: 34022368
DOI: 10.1053/j.semtcvs.2021.03.037 -
Annals of Diagnostic Pathology Dec 2022Calcifying aponeurotic fibroma (CAF) is a very rare benign entity that principally affects the volar fascia, tendons, and aponeuroses of the hands and feet with a peak... (Review)
Review
Calcifying aponeurotic fibroma (CAF) is a very rare benign entity that principally affects the volar fascia, tendons, and aponeuroses of the hands and feet with a peak incidence of between 5 and 15 years, although there have been cases found for a wide age range and at various anatomical sites. We present ten CAF cases; consisting of eight children and two adults. CAF occurred in the extremities in nine of the cases and in the chest wall in one case. CAF ultrasound and radiological findings are nonspecific but may help orientate diagnosis. Magnetic resonance imaging should be performed when there are doubtful cases, when occurring in nontypical sites, and when there are cases of nontypical clinical presentation. Histologically, all cases showed two components, a fibromatosis-like component and a nodular component. Chondroid areas were present in five cases. Calcifications were observed in nine cases. ERG immunostaining showed the same patterns in all the cases; diffuse positivity in pericalcified areas, and patchy positivity in areas away from calcifications. CAF has distinctive histopathological features which should aid in the differential diagnoses with other entities.
Topics: Child; Adult; Humans; Soft Tissue Neoplasms; Fibroma, Ossifying; Fibroma; Calcinosis
PubMed: 36371890
DOI: 10.1016/j.anndiagpath.2022.152056