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1.
Medicine (Baltimore) ; 103(10): e37342, 2024 Mar 08.
Artigo em Inglês | MEDLINE | ID: mdl-38457580

RESUMO

RATIONALE: Conjunctival myxoma is a rare benign tumor, which can mimic more common conjunctival lesions such as a cyst, lymphangioma, amelanotic nevus, neurofibroma, amelanotic melanoma, or lipoma. We describe a patient with the conjunctival myxoma, who was initially misdiagnosed as a conjunctival cyst. This case report includes intraoperative photographs and various immunohistochemical staining images. PATIENTS CONCERNS: A 55-year-old woman presented with a painless mass in the superotemporal conjunctiva of the left eye, which she had noticed 1 month ago. The patient had no previous history of trauma or eye surgery. Slit-lamp examination revealed a well-circumscribed, freely movable, pinkish, semi-translucent mass on the temporal bulbar conjunctiva, suggestive of a conjunctival cyst. DIAGNOSES: Histopathological analysis showed stellate- and spindle-shaped cells within the loose myxoid stroma, confirming a diagnosis of conjunctival myxoma. INTERVENTIONS: The conjunctival lesion was completely excised under local anesthesia. OUTCOMES: After 4 months of follow-up, the patient remained in good health without recurrence of the conjunctival lesion and no evidence of any systemic abnormality. LESSONS: Myxoma is an extremely uncommon benign tumor derived from primitive mesenchyme. Considering the rarity of the tumor and its similarity to other conjunctival tumors, diagnosis can be challenging. Ophthalmologists should consider myxoma as a possible differential diagnosis when encountering conjunctival lesions. Surgical excision is essential to confirm the diagnosis and careful systemic evaluation is required to prevent potentially life-threatening underlying systemic conditions.


Assuntos
Neoplasias da Túnica Conjuntiva , Cistos , Mixoma , Neoplasias Cutâneas , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias da Túnica Conjuntiva/diagnóstico , Neoplasias da Túnica Conjuntiva/cirurgia , Neoplasias da Túnica Conjuntiva/patologia , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Túnica Conjuntiva/patologia
2.
Clin Transl Med ; 14(2): e1581, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38318640

RESUMO

BACKGROUND: Cardiac myxoma (CM) is the most common (58%-80%) type of primary cardiac tumours. Currently, there is a need to develop medical therapies, especially for patients not physically suitable for surgeries. However, the mechanisms that shape the tumour microenvironment (TME) in CM remain largely unknown, which impedes the development of targeted therapies. Here, we aimed to dissect the TME in CM at single-cell and spatial resolution. METHODS: We performed single-cell transcriptomic sequencing and Visium CytAssist spatial transcriptomic (ST) assays on tumour samples from patients with CM. A comprehensive analysis was performed, including unsupervised clustering, RNA velocity, clonal substructure inference of tumour cells and cell-cell communication. RESULTS: Unsupervised clustering of 34 759 cells identified 12 clusters, which were assigned to endothelial cells (ECs), mesenchymal stroma cells (MSCs), and tumour-infiltrating immune cells. Myxoma tumour cells were found to encompass two closely related phenotypic states, namely, EC-like tumour cells (ETCs) and MSC-like tumour cells (MTCs). According to RNA velocity, our findings suggest that ETCs may be directly differentiated from MTCs. The immune microenvironment of CM was found to contain multiple factors that promote immune suppression and evasion, underscoring the potential of using immunotherapies as a treatment option. Hyperactive signals sent primarily by tumour cells were identified, such as MDK, HGF, chemerin, and GDF15 signalling. Finally, the ST assay uncovered spatial features of the subclusters, proximal cell-cell communication, and clonal evolution of myxoma tumour cells. CONCLUSIONS: Our study presents the first comprehensive characterisation of the TME in CM at both single-cell and spatial resolution. Our study provides novel insight into the differentiation of myxoma tumour cells and advance our understanding of the TME in CM. Given the rarity of cardiac tumours, our study provides invaluable datasets and promotes the development of medical therapies for CM.


Assuntos
Neoplasias Cardíacas , Mixoma , Humanos , Microambiente Tumoral/genética , Células Endoteliais/patologia , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/patologia , Mixoma/genética , Mixoma/patologia , RNA , Perfilação da Expressão Gênica
3.
Pathol Oncol Res ; 30: 1611553, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38317844

RESUMO

Introduction: Intramuscular myxomas are benign tumors that are challenging to diagnose, especially on core needle biopsies. Acquired chromosomal aberrations and pathogenic variants in codon 201 or codon 227 in GNAS complex locus gene (GNAS) have been reported in these tumors. Here we present our genetic findings in a series of 22 intramuscular myxomas. Materials and methods: The tumors were investigated for the presence of acquired chromosomal aberrations using G-banding and karyotyping. Pathogenic variants in codon 201 or codon 227 of GNAS were assessed using direct cycle Sanger sequencing and Ion AmpliSeq Cancer Hotspot Panel v2 methodologies. Results: Eleven tumors carried chromosomal abnormalities. Six tumors had numerical, four had structural, and one had both numerical and structural chromosomal aberrations. Gains of chromosomes 7 and 8 were the most common abnormalities being found in five and four tumors respectively. Pathogenic variants in GNAS were detected in 19 myxomas (86%) with both methodologies. The detected pathogenic variants were p.R201H in nine cases (seven with abnormal and two with normal karyotypes), p.R201C in five cases, all with normal karyotypes, p.R201S in three cases (two with abnormal and one with normal karyotype), p.R201G in one case with a normal karyotype, and p.Q227E in one case with a normal karyotype. Conclusion: Firstly, our data indicate a possible association between chromosomal abnormalities and GNAS pathogenic variants in intramuscular myxomas. Secondly, the presence of the rare pathogenic variants R201S, p.R201G and p.Q227E in 26% (5 out of 19) of myxomas with GNAS pathogenic variants shows that methodologies designed to detect only the common "hotspot" of p.R201C and p.R201H will give false negative results. Finally, a comparison between Ion AmpliSeq Cancer Hotspot Panel v2 and direct cycle Sanger sequencing showed that direct cycle Sanger sequencing provides a quick, reliable, and relatively cheap method to detect GNAS pathogenic variants, matching even the most cutting-edge sequencing methods.


Assuntos
Neoplasias Musculares , Mixoma , Humanos , Mutação , Aberrações Cromossômicas , Neoplasias Musculares/genética , Códon , Mixoma/genética , Mixoma/patologia
4.
Cardiol Young ; 34(1): 192-193, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37921210

RESUMO

Cardiac tumours are uncommon in the general population and even more so in the paediatric population. Here we present a case of an asymptomatic 7-year-old male with history of high-risk neuroblastoma who underwent 1-year post-treatment surveillance scan with an incidental finding of intracardiac lesion found to be an atrial myxoma.


Assuntos
Neoplasias Cardíacas , Mixoma , Neuroblastoma , Masculino , Humanos , Criança , Achados Incidentais , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/patologia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/patologia , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Neuroblastoma/cirurgia , Neuroblastoma/patologia
5.
Cardiovasc Pathol ; 69: 107599, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38072094

RESUMO

Carney syndrome is an autosomal dominant complex involving endocrinopathy, mucocutaneous hyperpigmentation, and different tumors, including cardiac myxomas. We report on a single family with several members affected with Carney syndrome. Family and individual medical histories were investigated in several Canadian provinces. The histology slides were also reviewed. Four family members (two young women, both sisters, their mother, and maternal grandmother) were found to harbor Carney syndrome. Everyone was presented with multiple and recurrent atrial myxomas of the heart, requiring multiple open cardiac surgeries. Breast myxomas and cutaneous hyperpigmentation were also revealed in one of the sisters and their mother. Interestingly, genetic testing was positive for the female family members and negative for the father and brother. We cannot rule out that the brother may have had a new mutation or harboring a mosaic. The young woman's brother did not have cardiac myxoma but developed a unilateral Sertoli cell tumor of testis. Carney syndrome is a rare complex multisystemic genetic disorder, including multiple and recurrent cardiac myxomas. We strongly suggest that reporting familial Carney syndrome is still critical in the 21st century to augment the awareness of this situation among clinicians and pathologists.


Assuntos
Complexo de Carney , Neoplasias Cardíacas , Hiperpigmentação , Mixoma , Masculino , Humanos , Feminino , Complexo de Carney/patologia , Canadá , Mixoma/patologia , Neoplasias Cardíacas/patologia
6.
Dis Colon Rectum ; 67(4): 514-522, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38100620

RESUMO

BACKGROUND: Aggressive angiomyxoma is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. Although many are estrogen and progesterone hormone receptor positive, the pathogenesis is unknown. Due to its rarity, there is a paucity of literature relating to this pathology. This article presents a case series on the management of aggressive angiomyxoma of the pelvis. OBJECTIVE: To present a 35-year experience managing aggressive angiomyxoma of the pelvis. DESIGN: This was a retrospective single-system analysis. SETTINGS: This study was conducted at a quaternary referral academic health care system. PATIENTS: All patients treated for aggressive angiomyxoma of the pelvis. INTERVENTIONS: All patients underwent surgical or medical management of their disease. MAIN OUTCOME MEASURES: The primary outcomes were disease recurrence and mortality. Secondary outcomes included risk factors for recurrence. RESULTS: A total of 32 patients (94% women) were identified with a median follow-up of 65 months. Thirty patients (94%) underwent operative resection and 2 patients were treated solely with medical management. Fifteen achieved an R0 resection (negative microscopic margins) at the index operation, of which 4 (27%) experienced tumor recurrence. There were no mortalities. No risk factors for disease recurrence were identified. LIMITATIONS: Limitations to our study include its nonrandomized retrospective nature, single health care system experience, and small patient sample size. CONCLUSIONS: Aggressive angiomyxoma is a rare, slow-growing tumor with locally invasive features and a high potential for recurrence even after resection with negative margins. Imaging modalities such as CT or MRI should be obtained to aid in diagnosis and surgical planning. Workup should be paired with preoperative biopsy and testing for hormone receptor status, which can increase diagnostic accuracy and guide medical treatment. Close posttreatment surveillance is imperative to detect recurrence. See Video Abstract . ANGIOMIXOMA AGRESIVO DE PELVIS EXPERIENCIA DE AOS: ANTECEDENTES:El angiomixoma agresivo es un tumor mesenquimal muy raro que se encuentra más comúnmente en las regiones pélvica y perineal. Aunque muchos son positivos para los receptores hormonales como el estrógeno y la progesterona, la patogénesis es aún desconocida. Debido a su rareza, existe escasa literatura relacionada con esta patología. Este artículo presenta una serie de casos sobre el tratamiento del angiomixoma agresivo de pelvis.OBJETIVO:Presentar una experiencia de 35 años en el manejo del angiomixoma agresivo de pelvis.DISEÑO:Este fue un análisis retrospectivo de sistema único.AJUSTES:Este estudio se llevó a cabo en un sistema de salud académico de referencia de nivel cuaternario.PACIENTES:Todos los pacientes tratados por angiomixoma agresivo de pelvis.INTERVENCIONES:Todos los pacientes se sometieron a tratamiento quirúrgico y/o médico de su enfermedad.PRINCIPALES MEDIDAS DE RESULTADO:Los resultados primarios fueron la recurrencia de la enfermedad y la mortalidad. Los resultados secundarios incluyeron factores de riesgo de recurrencia.RESULTADOS:Se identificaron un total de 32 pacientes (94% mujeres) con una mediana de seguimiento de 65 meses. Treinta (94%) fueron sometidos a resección quirúrgica y dos fueron tratados únicamente con tratamiento médico. Quince lograron una resección R0 (márgenes microscópicos negativos) en la operación inicial, de los cuales cuatro (27%) experimentaron recurrencia tumoral. No hubo mortalidades. No se identificaron factores de riesgo para la recurrencia de la enfermedad.LIMITACIONES:Las limitaciones de nuestro estudio incluyen su naturaleza retrospectiva no aleatoria, la experiencia de un solo sistema de atención médica y el tamaño pequeño de la muestra de pacientes.CONCLUSIONES:El angiomixoma agresivo es un tumor raro, de crecimiento lento, con características localmente invasivas y un alto potencial de recurrencia incluso después de una resección con márgenes negativos. Se deben obtener modalidades de imágenes como CT y/o MRI para la ayuda diagnóstica y la planificación quirúrgica. El estudio debe combinarse con una biopsia preoperatoria y pruebas del estado de los receptores hormonales, que pueden aumentar la precisión del diagnóstico y guiar el tratamiento médico. Es imperativa una estrecha vigilancia posterior al tratamiento para detectar recurrencia. (Traducción-Dr Osvaldo Gauto ).


Assuntos
Mixoma , Pelve , Humanos , Feminino , Masculino , Estudos Retrospectivos , Pelve/patologia , Períneo/patologia , Imageamento por Ressonância Magnética , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia
7.
BMC Cancer ; 23(1): 1245, 2023 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-38110859

RESUMO

BACKGROUND: Cardiac Myxoma is a primary tumor of heart. Its origins, rarity of the occurrence of primary cardiac tumors and how it may be related to limited cardiac regenerative potential, are not yet entirely known. This study investigates the key cardiac genes/ transcription factors (TFs) and signaling pathways to understand these important questions. METHODS: Databases including PubMed, MEDLINE, and Google Scholar were searched for published articles without any date restrictions, involving cardiac myxoma, cardiac genes/TFs/signaling pathways and their roles in cardiogenesis, proliferation, differentiation, key interactions and tumorigenesis, with focus on cardiomyocytes. RESULTS: The cardiac genetic landscape is governed by a very tight control between proliferation and differentiation-related genes/TFs/pathways. Cardiac myxoma originates possibly as a consequence of dysregulations in the gene expression of differentiation regulators including Tbx5, GATA4, HAND1/2, MYOCD, HOPX, BMPs. Such dysregulations switch the expression of cardiomyocytes into progenitor-like state in cardiac myxoma development by dysregulating Isl1, Baf60 complex, Wnt, FGF, Notch, Mef2c and others. The Nkx2-5 and MSX2 contribute predominantly to both proliferation and differentiation of Cardiac Progenitor Cells (CPCs), may possibly serve roles based on the microenvironment and the direction of cell circuitry in cardiac tumorigenesis. The Nkx2-5 in cardiac myxoma may serve to limit progression of tumorigenesis as it has massive control over the proliferation of CPCs. The cardiac cell type-specific genetic programming plays governing role in controlling the tumorigenesis and regenerative potential. CONCLUSION: The cardiomyocytes have very limited proliferative and regenerative potential. They survive for long periods of time and tightly maintain the gene expression of differentiation genes such as Tbx5, GATA4 that interact with tumor suppressors (TS) and exert TS like effect. The total effect such gene expression exerts is responsible for the rare occurrence and benign nature of primary cardiac tumors. This prevents the progression of tumorigenesis. But this also limits the regenerative and proliferative potential of cardiomyocytes. Cardiac Myxoma develops as a consequence of dysregulations in these key genes which revert the cells towards progenitor-like state, hallmark of CM. The CM development in carney complex also signifies the role of TS in cardiac cells.


Assuntos
Neoplasias Cardíacas , Mixoma , Humanos , Fatores de Transcrição/metabolismo , Miócitos Cardíacos/fisiologia , Diferenciação Celular/genética , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/patologia , Mixoma/genética , Mixoma/metabolismo , Mixoma/patologia , Carcinogênese/genética , Carcinogênese/metabolismo , Microambiente Tumoral
8.
J Comp Pathol ; 207: 50-58, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37944473

RESUMO

This report documents the pathological features of primary cardiac myxoid tumour (MT) in 11 dogs. Macroscopically, all the tumours were located in the tricuspid valve (TV), its septal leaflet being predominantly affected. Therefore, it appears that the TV is the most common site of occurrence for cardiac MT in dogs. Two gross anatomical types of canine valvular MT were evident. Seven of the 11 tumours were round or oval with a smooth or gently lobulated and glistening surface, while the other four were gelatinous, multilobulated and polypoid, with an irregular surface. Microscopically, in nine cases the tumours had an abundant myxoid matrix within which elongated spindle-shaped cells with no remarkable cytological atypia were sparsely embedded, suggesting a benign character (ie, myxoma). In the other two cases the tumours consisted of variably dense, haphazardly arranged, interlacing streams of anaplastic spindle-shaped or polygonal cells containing many mitotic figures, indicative of a malignant form of myxoma (ie, myxosarcoma). Isolated or clustered collections of myxoma cells (eg, cords, rings, syncytia) characteristic of human atrial myxoma were only rarely evident or lacking in all 11 cases, indicating that rarity or absence of such structural features may be specific to valvular MTs. Immunohistochemical findings were indicative of smooth muscle differentiation of the neoplastic cells. Tumour embolization to the intrapulmonary arteries and/or tumour implantation on the endocardium of the right heart chambers was evident only in the four cases of irregular-surfaced MT.


Assuntos
Doenças do Cão , Neoplasias Cardíacas , Mixoma , Mixossarcoma , Humanos , Cães , Animais , Neoplasias Cardíacas/veterinária , Mixoma/veterinária , Mixoma/patologia , Endocárdio/patologia , Mixossarcoma/veterinária
10.
Mult Scler ; 29(13): 1688-1692, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37712415

RESUMO

Cortical lesions (CLs) detected with double inversion recovery (DIR) magnetic resonance imaging (MRI) are very helpful in differentiating multiple sclerosis (MS) from other neuroinflammatory diseases of the central nervous system (CNS), that is, neuromyelitis optica spectrum disorders (NMOSDs). Furthermore, CLs are closely related to motor and cognitive impairment. We report a case of a 48-year-old female MS patient who developed several CLs during anti-CD20 therapy. Some CLs disappeared during follow-up MRIs. In the suspicion of a treatment failure, the screening for the autologous hematopoietic stem cell transplant (AHSCT) was performed with the evidence of an atrial myxoma. In MS patients with new CLs, a comorbid ischemic pathology should be considered and carefully investigated.


Assuntos
Fibrilação Atrial , Esclerose Múltipla , Mixoma , Neuromielite Óptica , Feminino , Humanos , Pessoa de Meia-Idade , Fibrilação Atrial/patologia , Córtex Cerebral/patologia , Esclerose Múltipla/patologia , Neuromielite Óptica/patologia , Imageamento por Ressonância Magnética/métodos , Mixoma/diagnóstico por imagem , Mixoma/patologia
11.
J Cutan Pathol ; 50(10): 861-863, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37455589

RESUMO

Carney complex is a rare genetic disorder associated with a number of cutaneous lesions, especially cutaneous myxomas. We present a rare case of cutaneous myxoma (superficial angiomyxoma) with trichofolliculoma-like features in a patient with Carney complex, and explore how the associated histopathology provides critical context for elucidating the etiology of this benign neoplasm.


Assuntos
Complexo de Carney , Mixoma , Neoplasia de Células Basais , Neoplasias Cutâneas , Humanos , Complexo de Carney/patologia , Mixoma/patologia , Neoplasias Cutâneas/patologia , Doenças Raras
13.
BMC Cardiovasc Disord ; 23(1): 219, 2023 04 28.
Artigo em Inglês | MEDLINE | ID: mdl-37118677

RESUMO

BACKGROUND: Primary cardiac tumors are rare, and cardiac myxoma (CM) accounts for the majority of these tumors. Most of the reports in the literature are case reports. This study summarizes our clinical experience in the surgical treatment of CM over the past 12 years. METHODS: We retrospectively analyzed the clinical data of 23 children with CM(8 boys, 15 girls; median age: 8.92 months, range: 2 years 5 months-12 years 9 months; body weight: 11-45 kg, median body weight: 28.21 kg) admitted to our hospital in the previous 12 years, and we statistically analyzed their clinical manifestations and surgical methods. RESULTS: 23 cases underwent myxoma excision under cardiopulmonary bypass(CPB). The follow-up period was 0.2 to 12.6 years (mean:7.2 years). Two patients could not be traced, and the follow-up completion rate was 91.30%. One patient (4.35%) died of myocardial infarction early after surgery with low continuous cardiac output. There were no cerebral embolism, acute heart failure, atrioventricular block and other related complications in 19 cases. A patient with cerebral infarction complicated with right hemiplegia recovered well after rehabilitation treatment. There was no recurrence of CM in 19 cases and all patients recovered after surgery. One patient relapsed 5 years after surgery, and no tumor recurrence was observed after the second surgery. Among the 20 long-term survivors, 13 (65.00%) were NYHA Class I patients and 7(35.00%) were NYHA Class II patients. CONCLUSIONS: Although CM in children is rare, it may cause cerebral infarction and other multi-organ embolism. Once CM is found and removed as soon as possible, it can reduce serious complications. If the complete resection is possible, surgery provides better palliation. Follow-up echocardiographic should be paid attention to after surgery.


Assuntos
Embolia , Neoplasias Cardíacas , Mixoma , Masculino , Feminino , Humanos , Criança , Lactente , Estudos Retrospectivos , Recidiva Local de Neoplasia , Neoplasias Cardíacas/diagnóstico por imagem , Neoplasias Cardíacas/cirurgia , Neoplasias Cardíacas/patologia , Mixoma/diagnóstico por imagem , Mixoma/cirurgia , Mixoma/patologia , Peso Corporal
14.
J Cardiothorac Surg ; 18(1): 143, 2023 Apr 17.
Artigo em Inglês | MEDLINE | ID: mdl-37069679

RESUMO

A 33-year male patient presented with a 6-month history of cough and shortness of breath upon physical activity. Echocardiography demonstrated right ventricular space-occupying lesions. Contrast-enhanced computed tomography of the chest showed multiple emboli in the pulmonary artery and its branches. Right ventricle tumor (myxoma) resection, tricuspid valve replacement, and clearance of the pulmonary artery thrombus were performed under cardiopulmonary bypass. Minimally invasive forceps and balloon urinary catheters were used to clear the thrombus. Clearance was confirmed by direct visualization using a choledochoscope. The patient recovered well and was discharged. The patient was prescribed oral warfarin 3 mg/day, and the international normalized ratio for prothrombin time was maintained between 2.0 and 3.0. Pre-discharge echocardiogram showed no lesion in the right ventricle or pulmonary arteries. The 6-month follow-up echocardiography indicated that the tricuspid valve was functioning well and showed no thrombus in the pulmonary artery.


Assuntos
Neoplasias Cardíacas , Mixoma , Embolia Pulmonar , Trombose , Humanos , Masculino , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/etiologia , Embolia Pulmonar/cirurgia , Neoplasias Cardíacas/cirurgia , Artéria Pulmonar/patologia , Ventrículos do Coração , Trombose/diagnóstico por imagem , Trombose/etiologia , Trombose/cirurgia , Mixoma/complicações , Mixoma/cirurgia , Mixoma/patologia
15.
World J Surg Oncol ; 21(1): 99, 2023 Mar 20.
Artigo em Inglês | MEDLINE | ID: mdl-36941612

RESUMO

BACKGROUND: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma. METHODS: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was "pulmonary valve myxoma." Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively. RESULTS: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient's median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively. CONCLUSIONS: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.


Assuntos
Neoplasias Cardíacas , Mixoma , Valva Pulmonar , Masculino , Humanos , Criança , Idoso , Pessoa de Meia-Idade , Valva Pulmonar/cirurgia , Valva Pulmonar/patologia , Ecocardiografia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirurgia , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Átrios do Coração/patologia
16.
Am J Surg Pathol ; 47(3): 296-306, 2023 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-36607731

RESUMO

Superficial angiomyxoma is an uncommon benign mesenchymal neoplasm that usually arises in dermis/subcutis of the extremities or trunk. Some tumors are associated with Carney complex. When arising in breast, these tumors are not well-recognized, mainly due to a lack of uniform nomenclature in the literature. This study therefore aims to improve recognition of angiomyxomas of the breast region. Forty cases were identified: demographics, presence of Carney complex, imaging and histologic features, PRKAR1A expression, and outcomes were evaluated. There were 22 female and 18 male patients (median age 40 years, range: 14 to 72). Most tumors presented as slowly-growing masses (77%). All but one were solitary, and median size was 1.5 cm. Tumors were superficial (dermal/subcutaneous) in 52.5% and deep/parenchymal in 47.5%. Nine involved the nipple-areola complex. All showed characteristic features of superficial angiomyxoma: poorly circumscribed, hypocellular, myxoid neoplasms with lobulated (55%) or infiltrative (45%) architecture, bland spindled fibroblasts, prominent thin-walled vessels, and admixed neutrophils. Tumors involving the nipple-areola complex infiltrated through areolar smooth muscle, and deep/parenchymal tumors showed entrapment of lobules mimicking myxoid fibroadenoma. Mitoses were typically absent, as was significant atypia. Cystic change was common. Two-thirds showed loss of PRKAR1A expression by immunohistochemistry. Two patients had Carney complex (7%). Recurrence after incomplete excision occurred in 1 patient. Angiomyxoma of breast may arise at superficial, nipple-areola or deep/parenchymal locations, where it can be difficult to recognize classic histologic features. Loss of expression of PRKAR1A is not invariable, but may be a helpful diagnostic clue. Recognizing angiomyxoma is important for 2 reasons: first, the recurrence rate is low and therefore wide excision is not essential, and second, it may allow detection of Carney complex in some patients.


Assuntos
Neoplasias da Mama , Complexo de Carney , Mixoma , Humanos , Masculino , Feminino , Adulto , Imuno-Histoquímica , Mixoma/patologia , Mitose
18.
Lasers Med Sci ; 38(1): 38, 2023 Jan 11.
Artigo em Inglês | MEDLINE | ID: mdl-36630018

RESUMO

It was to report a rare case of peripheral odontogenic myxoma removed with high-power diode laser and to do an extensive review of studies of odontogenic cysts and tumors treated with high-power laser (HPL). This is a rare case of a 63-year-old male patient with a peripheral odontogenic myxoma measuring approximately 10 cm in the attached gingiva region of tooth 16 removed with a high-power diode laser (808 nm, 3 W, in continuous mode, under constant suction, with 400-µm optical fiber). A literature review was also carried out looking for articles that involved the use of HPL in the treatment of odontogenic cysts and tumors, without restriction of year or language. In the present case, there was no need for suturing, no postoperative discomfort, and minimal bleeding during the procedure. In a 12-month follow-up period, there were no signs of recurrence. Only two cases of intra-osseous odontogenic myxomas treated with HPL and 10 cases involving other odontogenic cysts and tumors were found. All studies showing HPL to be effective in treating these lesions. Despite the different types of lasers used and different parameters, it is observed that lasers are effective in the treatment of odontogenic lesions.


Assuntos
Mixoma , Tumores Odontogênicos , Masculino , Humanos , Pessoa de Meia-Idade , Lasers Semicondutores/uso terapêutico , Mixoma/cirurgia , Mixoma/diagnóstico , Mixoma/patologia , Tumores Odontogênicos/radioterapia , Tumores Odontogênicos/cirurgia , Tumores Odontogênicos/diagnóstico
19.
Eur J Ophthalmol ; 33(5): NP130-NP136, 2023 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-36083167

RESUMO

PURPOSE: The purpose of this study was to report the clinicopathological features and management of the first case of bilateral synchronous conjunctival myxoma. METHODS: This study was a case report and literature review. RESULTS: A 66-year-old Chinese male with past ocular history of uncomplicated bilateral phacoemulsification and intraocular lens (IOLs) 3 years ago prior to presentation presented with bilateral red and swollen conjunctiva for over a year. On examination his corrected distance visual acuity (CDVA) was 25/20 in the right eye 20/20 in the left eye. Slit lamp examination revealed swollen temporal conjunctiva bilaterally which appeared as painless, well-circumscribed, salmon-pink, fleshy patches. The lesion in the right eye was subsequently excised, followed by excision of the lesion in the left eye at 3-week interval. Microscopically, histopathological examination of both excised specimens revealed hypocellular conjunctival mucosa covered by non-dysplastic epithelium, with presence of myxoid degeneration in the subepithelial stroma and immunostaining findings consistent with conjunctival myxoma. At his latest follow-up at 24 months, there were no recurrences of the conjunctival masses and the CDVA was the same as preoperatively.


Assuntos
Neoplasias da Túnica Conjuntiva , Mixoma , Masculino , Humanos , Idoso , Neoplasias da Túnica Conjuntiva/diagnóstico , Neoplasias da Túnica Conjuntiva/cirurgia , Neoplasias da Túnica Conjuntiva/patologia , Túnica Conjuntiva/patologia , Mixoma/diagnóstico , Mixoma/cirurgia , Mixoma/patologia , Microscopia com Lâmpada de Fenda , Acuidade Visual
20.
Ear Nose Throat J ; 102(6): 405-408, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33829881

RESUMO

Head and neck tumors are rare in pediatric patients but should be kept in the differential when a patient presents with a new swelling or mass. One of these tumors is a myxoma, which is an insidiously growing, benign mass originating from the mesenchyme. They most commonly arise in the myocardium but can also develop in facial structures, particularly in the maxilla and mandible. When arising in facial structures, ocular, respiratory, and digestive systems can be affected based on local invasion. Complete surgical resection is curative but can lead to significant morbidity as well. Here, we present a case of a 15-month-old toddler presenting with a paranasal mass, which was ultimately diagnosed as a maxillary myxoma. This tumor is very rare in the pediatric population, especially in the toddler age-group, reminding clinicians to broaden the differential diagnosis when a patient's course is atypical.


Assuntos
Neoplasias de Cabeça e Pescoço , Mixoma , Humanos , Masculino , Criança , Pré-Escolar , Lactente , Maxila/patologia , Neoplasias de Cabeça e Pescoço/diagnóstico , Face/patologia , Diagnóstico Diferencial , Mixoma/patologia
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