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Lipogenic and fibrous tumors are thought to originate from CD34-positive stromal fibroblastic/fibrocystic cells. Well-differentiated lipogenic tumors typically express CD34, whereas dedifferentiated liposarcoma (DDLPS) often loses it. We conducted survival analyses involving 59 patients with DDLPS. Males comprised 53% of the cohort, and the median age at the time of wide resection of primary DDLPS was 60 years. Loss of CD34 expression was defined as when ≥50% of the dedifferentiated area was immunohistochemically negative for CD34. As a result, 39 of the 59 patients showed loss of CD34 expression during the initial operation for DDLPS. In the univariate analyses, the tumor site in the retroperitoneum/abdominal cavity and loss of CD34 expression were significantly associated with poor overall survival. In the multivariate analyses, loss of CD34 expression (HR = 2.26; 95% CI = 1.02-5.02; p = 0.04) and the tumor site in the retroperitoneum/abdominal cavity (HR = 3.11; 95% CI = 1.09-8.86; p = 0.03) were retained as independent prognostic factors. Six CD34-positive cases lost CD34 expression when they developed metastasis and/or local recurrence, suggesting that the loss was associated with the later stage of the tumor. Therefore, an association existed between the loss of CD34 expression and clinicopathological behaviors such as poorer prognoses and recurrence.
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Antígenos CD34 , Biomarcadores de Tumor , Liposarcoma , Humanos , Masculino , Liposarcoma/patología , Liposarcoma/metabolismo , Persona de Mediana Edad , Femenino , Antígenos CD34/metabolismo , Anciano , Adulto , Biomarcadores de Tumor/análisis , Biomarcadores de Tumor/metabolismo , Pronóstico , Anciano de 80 o más Años , Inmunohistoquímica , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/metabolismo , Neoplasias Retroperitoneales/patología , Neoplasias Retroperitoneales/metabolismoRESUMEN
To improve the efficiency of pathological diagnoses, the development of automatic pathological diagnostic systems using artificial intelligence (AI) is progressing; however, problems include the low interpretability of AI technology and the need for large amounts of data. We herein report the usefulness of a general-purpose method that combines a hyperspectral camera with machine learning. As a result of analyzing bile duct biopsy and bile cytology specimens, which are especially difficult to determine as benign or malignant, using multiple machine learning models, both were able to identify benign or malignant cells with an accuracy rate of more than 80% (93.3% for bile duct biopsy specimens and 83.2% for bile cytology specimens). This method has the potential to contribute to the diagnosis and treatment of bile duct cancer and is expected to be widely applied and utilized in general pathological diagnoses.
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Neoplasias de los Conductos Biliares , Conductos Biliares , Aprendizaje Automático , Humanos , Neoplasias de los Conductos Biliares/patología , Neoplasias de los Conductos Biliares/diagnóstico , Conductos Biliares/patología , Biopsia/métodos , Masculino , Femenino , Anciano , Persona de Mediana Edad , Bilis/citología , Imágenes Hiperespectrales/métodos , Inteligencia Artificial , Citodiagnóstico/métodos , CitologíaRESUMEN
PURPOSE: To evaluate the significant findings of hip periprosthetic joint infection (PJI) using metal-artifact-reduction (MAR) MRI and to compare the MRI results to other clinical markers. METHODS: The results of MRI, including two-dimensional fast-spin echo sequences with increased bandwidth and multi-acquisition variable-resonance image combination selective for hips with orthopedic implants at 1.5T (from April 2014 to November 2021), were retrospectively assessed for imaging findings and diagnostic impressions by two radiologists. Clinical data and courses were also investigated. Univariate and multivariate analyses were performed to identify the significant MRI findings in patients with hip PJI and those who underwent surgical intervention. The MRI impressions were compared with other clinical markers in diagnosing hip PJI. RESULTS: Thirty-seven hip joints in 24 Asian patients (age = 73.9 ± 10.8 years; 18 females) were included. Twelve hip joints (32%) had PJI; seven underwent a surgical intervention. The significant findings for hip PJI included periosteal edema of the acetabulum, intermuscular edema, intramuscular fluid collection, and lymphadenopathy (P < 0.05). In the cases with surgical intervention, the significant findings included capsular distension, capsular thickening, an osteolysis-like pattern of the femur, subcutaneous fluid collection, and lymphadenopathy (P < 0.05). The MRI impressions had high diagnostic significance for both hip PJI cases and those with surgical intervention (P < 0.001). The MRI impression was more significant for hip PJI than the other clinical markers (P < 0.05), while the other clinical markers were more significant in the cases with surgical intervention (P < 0.05). CONCLUSION: The significant findings in the hip PJI cases included acetabular periosteal edema, intermuscular edema, intramuscular fluid collection, and lymphadenopathy. The significant findings in the cases with surgical intervention included capsular distention, capsular thickening, a femoral osteolysis-like pattern, subcutaneous fluid collection, and lymphadenopathy. The utilization of MAR MRI demonstrated great diagnostic significance for hip PJI.
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In recent years, lesbian, gay, bisexual, and transgender (LGBT) populations have been gaining acceptance in society. However, very few cases of malignancy in the LGBT population have been reported thus far. We herein report a transgender woman receiving estrogen supplementation who developed primary mediastinal large B-cell lymphoma (PMBCL) and was treated with dose-adjusted EPOCH-rituximab (DA-EPOCH-R) therapy. The patient achieved complete remission after the sixth course of DA-EPOCH-R therapy. To help this LGBT patient continue receiving chemotherapy smoothly on admission, adjusting the hospital environment, such as the allocation of rooms, was essential.
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BACKGROUND: Osteoblastic skeletal metastasis is frequently observed in prostate cancer. An effective therapy has not been developed due to the unclear molecular mechanism. The Wnt family is involved in various biological phenomena including bone metabolism. There is no direct evidence that the family causes osteoblastic skeletal metastasis. AIMS: The present study aims to evaluate whether overexpressed Wnt induces osteoblastic bone metastasis in a well-established osteolytic bone metastatic model. METHODS AND RESULTS: The breast cancer-derived 5a-D-Luc-ZsGreen cells were transfected with Wnt1, Wnt3A, and Wnt5A expression vectors, producing stably highly expressing cells. These cells were intracardially transplanted in nude mice. Bone metastasis development was confirmed by fluorescence imaging. Hind-limb bones including metastasis were dissected and visualized through micro-CT imaging. After imaging, sections were stained with hematoxylin and eosin (H&E), and immunohistochemically stained with an anti-SATB2 antibody. Luminescent imaging confirmed mice with bone metastases in the hind limbs. Micro-CT imaging found an osteoblastic change only in bone metastasis of mice transplanted with Wnt1-expressing cells. This was confirmed on H&E-stained sections. SATB2 immunostaining showed differentiated osteoblasts were at the site of bone metastases in the diaphysis. SATB2 in the Wnt/ß-catenin pathway activated by overexpressed Wnt1 could induce osteoblastic change. CONCLUSION: Our findings provided direct evidence Wnt1 is involved in osteoblastic bone metastasis development. Our model would be a powerful tool for further elucidating molecular mechanisms underlying the disease and developing effective therapies.
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Neoplasias Óseas , Neoplasias de la Próstata , Masculino , Ratones , Humanos , Animales , Ratones Desnudos , Neoplasias Óseas/secundario , Neoplasias de la Próstata/patologíaRESUMEN
RATIONALE: Although IgG4-related disease (IgG4-RD) can affect various organs, its association with a cardiac mass is exceptionally rare. Here, we report a case of a woman with IgG4-RD and a cardiac mass and discuss 10 similar cases reported previously. PATIENT CONCERNS: A 65-year-old woman was referred to our hospital for chest discomfort and back pain. DIAGNOSES: In accordance with the 2019 ACR/EULAR diagnostic criteria for IgG4-RD, she was diagnosed with IgG4-RD based on dense lymphocytic infiltration on histopathology, IgG/IgG4-positive cell ratio <40%, >10/hpf IgG4-positive cells on immunostaining, and paraspinal zone soft tissue lesions in the chest. INTERVENTIONS: An external pacemaker was implanted for the complete atrioventricular block on the electrocardiogram. After the diagnosis of IgG4-RD, she was treated with glucocorticoids and rituximab. OUTCOMES: She remains under observation without disease recurrence. LESSONS: IgG4-RD are usually treated with glucocorticoids; however, in cases of a cardiac mass, life-threatening complications may occur and surgery is often needed. Combination therapy with glucocorticoids and rituximab may be effective even in patients with IgG4-RD and cardiac mass, which may avoid the need of invasive treatments, such as surgery.
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Glucocorticoides , Enfermedad Relacionada con Inmunoglobulina G4 , Femenino , Humanos , Anciano , Glucocorticoides/uso terapéutico , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/terapia , Rituximab/uso terapéutico , Inmunoglobulina G , Diagnóstico DiferencialRESUMEN
RATIONALE: Giant cell arteritis (GCA) is an autoimmune vasculitis that affects large and medium-sized blood vessels. The mRNA vaccine against severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) has been associated with the development of immune-mediated diseases. In this article, we present a case of GCA that developed after vaccination against SARS-CoV2. PATIENT CONCERNS: A 77-year-old man developed fever, general fatigue, and headache 1 day after the third dose of vaccination against SARS-CoV2. Nodular swelling and tenderness of the bilateral temporal arteries were observed. DIAGNOSES: Although right temporal artery biopsies were negative, the patient was diagnosed with GCA based on criteria established by the American College of Rheumatology for the classification of GCA. INTERVENTIONS: The patient received methylprednisolone 1000 mg for 3 days. This was followed by prednisolone 1 mg/kg/d, which was decreased by 10 mg every week to 30 mg. From day 16 of hospitalization, the patient received tocilizumab 162 mg/wk every other week. OUTCOMES: There was no occurrence of acute side effects. After 38 days of treatment, the condition improved and the patient was discharged from the hospital; as stated above, the dose of prednisolone was tapered to 30 mg/d. LESSONS: We experienced a case of GCA that occurred immediately after vaccination against SARS-CoV2 with an mRNA vaccine. Early signs of GCA include fever, fatigue, and headache, and often resemble those noted after vaccination against SARS-CoV2. The potential presence of GCA should be determined in individuals with persistent fever and headache after vaccination against SARS-CoV2.
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COVID-19 , Arteritis de Células Gigantes , Masculino , Humanos , Anciano , Arteritis de Células Gigantes/etiología , Arteritis de Células Gigantes/complicaciones , ARN Viral , COVID-19/prevención & control , COVID-19/complicaciones , SARS-CoV-2 , Metilprednisolona/uso terapéutico , Cefalea/etiología , Vacunación/efectos adversosRESUMEN
Cancer stem cells have the capability of self-renewal and multipotency and are, therefore, associated with tumor heterogeneity, resistance to chemoradiation therapy, and metastasis. The hypothesis that multinucleated giant cells, which often emerge following chemo- and/or radiotherapy, serve as cancer stem cells has not been fully evaluated. Although a previous study demonstrated that these cells functioned as stem cells, only low levels of Yamanaka factors were expressed, contrasting with the high expression seen from their gestated first-generation mononuclear cells. Herein, we report a case of a plasmablastic neoplasm with multinucleated giant cells that were analyzed for stemness to test the above hypothesis. The patient was a male in his 80s who had a plasmablastic neoplasm that was not easily distinguishable as plasmablastic lymphoma versus plasma cell myeloma of plasmablastic type. Lymph node biopsy showed predominant mononuclear cell proliferation with admixed multinucleated giant cells. Immunohistochemistry and in situ hybridization showed that both multinucleated and mononuclear cells had the same profile: CD138(+), light chain restriction of κ>λ, cyclin D1(+), CD68(-), EBER-ISH (+). These results suggested that both cell types were neoplastic. In accordance with the previous study, the multinucleated giant cells showed low expression of Yamanaka factors, which were highly expressed in some of the mononuclear cells. Furthermore, the multinucleated giant cells showed a much lower proliferative activity (Mib1/Ki67 index) than the mononuclear cells. Based on these results, the multinucleated giant cells were compatible with cancer stem cells. This case is expected to expand the knowledge base regarding biology of cancer stem cells.
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Treatment-related second malignancies (SMs) remain a major concern in long-term survivors of Hodgkin lymphoma (HL). In this report, the autopsy findings of a patient with HL, who was in complete remission after chemotherapy but expired of pulmonary tumor thrombotic microangiopathy (PTTM) caused by urothelial carcinoma of the renal pelvis (UCRP), were described. A 78-year-old Japanese man with a history of classical HL developed irreversible heart failure about 2.5 years after chemotherapy. The patient expired shortly after being admitted due to ineffective treatment for heart failure. However, the cause of death was not determined. The patient's autopsy findings revealed UCRP in the left kidney, as well as infiltration around the inferior vena cava and lungs, but no HL recurrence. The primary causes of mortality were respiratory and heart failure due to PTTM. Therefore, it is essential to consider the risk of SMs and search for them in patients with HL after chemotherapy.
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PURPOSE: Osteosarcoma, the most common bone malignancy in children, has a poor prognosis, especially when the tumor metastasizes to the lungs. Therefore, novel therapeutic strategies targeting both proliferation and metastasis of osteosarcoma are required. Podoplanin (PDPN) is expressed by various tumors and is associated with tumor-induced platelet activation via its interaction with C-type lectin-like receptor 2 (CLEC-2) on platelets. We previously found that PDPN contributed to osteosarcoma growth and metastasis through platelet activation; thus, in this study, we developed an anti-PDPN humanized antibody and evaluated its effect on osteosarcoma growth and metastasis. EXPERIMENTAL DESIGN: Nine osteosarcoma cell lines and two osteosarcoma patient-derived cells were collected, and we evaluated the efficacy of the anti-DPN-neutralizing antibody PG4D2 and the humanized anti-PDPN antibody AP201, which had IgG4 framework region. The antitumor and antimetastasis effect of PG4D2 and AP201 were examined in vitro and in vivo. In addition, growth signaling by the interaction between PDPN and CLEC-2 was analyzed using phospho-RTK (receptor tyrosine kinase) array, growth assay, or immunoblot analysis under the supression of RTKs by knockout and inhibitor treatment. RESULTS: We observed that PG4D2 treatment significantly suppressed tumor growth and pulmonary metastasis in osteosarcoma xenograft models highly expressing PDPN. The contribution of PDGFR activation by activated platelet releasates to osteosarcoma cell proliferation was confirmed, and the humanized antibody, AP201, suppressed in vivo osteosarcoma growth and metastasis without significant adverse events. CONCLUSIONS: Targeting PDPN with a neutralizing antibody against PDPN-CLEC-2 without antibody-dependent cell-mediated cytotoxicity and complement-dependent cytotoxicity is a novel therapeutic strategy for PDPN-positive osteosarcoma.
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Neoplasias Óseas , Lectinas Tipo C , Neoplasias Pulmonares , Glicoproteínas de Membrana , Osteosarcoma , Anticuerpos Neutralizantes , Neoplasias Óseas/tratamiento farmacológico , Línea Celular Tumoral , Humanos , Neoplasias Pulmonares/metabolismo , Osteosarcoma/tratamiento farmacológicoRESUMEN
MATERIALS AND METHODS: Magnetic resonance imaging around metal joint prostheses including multiacquisition variable-resonance image combination selective at 1.5 T (from April 2014 to August 2020) was retrospectively evaluated by 2 radiologists for detection of abnormal findings (joint effusion, capsular thickening, pericapsular edema, soft-tissue fluid collection, soft-tissue edema, bone marrow edema pattern around the implant [BME pattern], lymphadenopathy, and others) and overall image impression for PJI. Regarding the soft-tissue fluid collection, presence of communication to the joint or capsular-like structure was evaluated. Clinical assessments were recorded. Positive predictive values (PPVs), negative predictive values (NPVs), and odds ratios (ORs) for PJI were calculated for the abnormal findings. Overall image impression for PJI was evaluated. χ2, Fisher exact, t, and Mann-Whitney U tests and receiver operating characteristic analysis were used. Interobserver agreement was assessed with κ statistics. RESULTS: Forty-three joints in 36 patients (mean ± SD age, 75.4 ± 8.8 years; 30 women; hip [n = 29], knee [n = 12], and elbow [n = 2]) were evaluated. Eighteen joints (42%) were clinically diagnosed as PJI. The findings suggesting PJI were capsular thickening (PPV, 70%; NPV, 90%; OR, 20.6), soft-tissue fluid collection (PPV, 81%; NPV, 81%; OR, 19.1), soft-tissue edema (PPV, 67%; NPV, 89%; OR, 17), pericapsular edema (PPV, 76%; NPV, 81%; OR, 13.7), and joint effusion (PPV, 55%; NPV, 100%; OR, 12). Soft-tissue fluid collection without capsular-like structure (PPV, 83%; NPV, 74%; OR, 14.4) or with communication to the joint (PPV, 75%; NPV, 71%; OR, 7.3) suggested PJI. The combinations of joint effusion, capsular thickening, pericapsular edema, soft-tissue fluid collection, and soft-tissue edema highly suggested PJI. Regarding the BME pattern, the combination with soft-tissue edema raised the possibility of PJI (PPV, 73%; NPV, 69%; OR, 5.9). Regarding the interobserver agreements for each abnormal finding, κ values were 0.60 to 0.77. Regarding the overall image impression, weighted κ value was 0.97 and areas under the receiver operating characteristic curve were 0.949 (95% confidence interval, 0.893-1.005) and 0.926 (95% confidence interval, 0.860-0.991) with no significant difference (P = 0.534). CONCLUSIONS: The findings suggesting PJI were capsular thickening, soft-tissue fluid collection, soft-tissue edema, pericapsular edema, and joint effusion. The combinations of them highly suggested PJI. Regarding the BME pattern, the combination with soft-tissue edema raised the possibility of PJI.
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Infecciones Relacionadas con Prótesis , Anciano , Anciano de 80 o más Años , Artefactos , Edema , Femenino , Humanos , Imagen por Resonancia Magnética/métodos , Infecciones Relacionadas con Prótesis/diagnóstico por imagen , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
A 50's woman was diagnosed with left local-advanced breast cancer(pT4bN2aM0, stage â ¢B, estrogen-receptor positive and human epidermal growth factor-2 negative)in 2016. Neoadjuvant therapy consisting of 4 courses of epirubicin plus cyclophosphamide and 4 courses of docetaxel were administered. After neoadjuvant therapy, a mastectomy with axillary node dissection was performed. And after surgery, she was received radiation and endocrine therapy. In May 2019, multiple bone metastases were detected. We administered endocrine therapy. In February 2020, she developed leg paralysis and malignant cells were collected from the cerebrospinal fluid. She was diagnosed with meningeal carcinomatosis without brain metastasis from breast cancer. To improve quality of life, we started radiation therapy, intrathecal chemotherapy and systemic chemotherapy. After 3 months of these therapies, leg paralysis was improved and quality of life was maintained for 9 months. Herein, we report a case of meningeal carcinomatosis without brain metastasis from breast cancer which is improved by radiation therapy, intrathecal chemotherapy and systemic chemotherapy.
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Neoplasias Encefálicas , Neoplasias de la Mama , Carcinomatosis Meníngea , Femenino , Humanos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias Encefálicas/cirugía , Neoplasias de la Mama/tratamiento farmacológico , Neoplasias de la Mama/patología , Mastectomía , Carcinomatosis Meníngea/tratamiento farmacológico , Parálisis , Calidad de Vida , QuimioradioterapiaRESUMEN
It is controversial whether patients with myxofibrosarcomas (MFSs) have better prognoses than those with undifferentiated pleomorphic sarcomas (UPSs). No useful prognostic factors have been established to date. We therefore aimed to evaluate the prognostic value of CD34 expression status in 192 patients with MFSs and UPSs. Using the log-rank test, we showed that patients with MFSs had a significantly better overall survival than did those with UPSs when defining the former as having a > 10% myxoid component (p = 0.03), but not when defining it as having a > 50% myxoid component (p = 0.1). Under the definition of MFSs as > 10% myxoid component, the log-rank test revealed that the diagnosis of the UPS and the CD34 loss (p < 0.001) were significant adverse predictors of overall survival. As per the Cox model, the CD34 loss remained an independent prognostic factor (hazard ratio = 3.327; 95% confidence interval 1.334-8.295), while the diagnosis of the UPS was a nonsignificant confounding factor (hazard ratio = 1.084; 95% confidence interval 0.679-1.727). In conclusion, CD34 expression status is a useful prognostic factor in patients with MFS and UPS, and it should be incorporated into grading systems that are used to predict outcomes.
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Antígenos CD34/biosíntesis , Fibroma/diagnóstico , Fibrosarcoma/diagnóstico , Perfilación de la Expresión Génica , Histiocitoma Fibroso Maligno/diagnóstico , Sarcoma/diagnóstico , Anciano , Supervivencia sin Enfermedad , Femenino , Fibroma/metabolismo , Fibrosarcoma/metabolismo , Histiocitoma Fibroso Maligno/metabolismo , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Recurrencia Local de Neoplasia , Pronóstico , Modelos de Riesgos Proporcionales , Sarcoma/metabolismo , Resultado del TratamientoRESUMEN
Giant cell tumors of soft tissue (GCT-ST) arising in the breast are extremely rare. We report a unique case of breast GCT-ST coincident with ductal carcinoma in situ (DCIS), diagnosed with histological, immunohistochemical, and H3F3A (Histone H3.3) mutation analyses. A 59-year-old woman preoperatively diagnosed with DCIS underwent total mastectomy for a cystic mass. Histology revealed a tumor composed of mononuclear cells interspersed with numerous osteoclast-like giant cells, resembling giant cell tumor of bone (GCT-B), with apocrine DCIS in proximity to the tumor. The mononuclear and giant cells were immunoreactive for CD68 and negative for cytokeratins. Granulomatous diseases, carcinomas with giant cells, and giant cell-type sarcomas were excluded by histological and immunophenotypic features. Lack of H3F3A mutation eliminated the possibility of GCT-B metastasizing to the breast. These findings were consistent with GCT-ST of the breast. To our knowledge, this is the ninth reported case of breast GCT-ST, but the first case that accompanied DCIS or involved H3F3A mutation status investigation. For correct diagnosis of this rare tumor, it is important for pathologists to raise the possibility of GCT-ST when encountering giant cell-rich breast lesions and to exclude other differential diagnoses by combining the results of histological, immunohistochemical, and genetic analyses.
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Neoplasias de la Mama/diagnóstico , Carcinoma Intraductal no Infiltrante/diagnóstico , Histonas/genética , Neoplasias de los Tejidos Blandos/diagnóstico , Mama/patología , Mama/cirugía , Neoplasias de la Mama/patología , Neoplasias de la Mama/cirugía , Carcinoma Intraductal no Infiltrante/patología , Carcinoma Intraductal no Infiltrante/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Mastectomía , Persona de Mediana Edad , Mutación , Neoplasias de los Tejidos Blandos/patología , Neoplasias de los Tejidos Blandos/cirugíaRESUMEN
A 57-year-old woman with a history of uterine endometrial carcinoma underwent PET/CT examinations for initial staging and posttreatment survey. Multiple patchy accumulations were noted in the muscles, particularly in both thighs. These accumulations resolved spontaneously 6 months after the follow-up examination. However, 3.5 years after the surgery, the multiple patchy accumulations reappeared in the muscle of the upper and lower extremities showing an increase in signal intensity from previous examination. A biopsy of the right thigh revealed epithelioid cell granuloma without necrosis. We therefore consider that this case might be "idiopathic" granulomatous myositis.
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Fluorodesoxiglucosa F18 , Granuloma/complicaciones , Miositis/complicaciones , Miositis/diagnóstico por imagen , Tomografía Computarizada por Tomografía de Emisión de Positrones , Neoplasias Endometriales/complicaciones , Neoplasias Endometriales/diagnóstico por imagen , Neoplasias Endometriales/patología , Neoplasias Endometriales/terapia , Femenino , Humanos , Persona de Mediana Edad , Miositis/patología , Estadificación de NeoplasiasRESUMEN
Nodular fasciitis (NF) is a self-limiting benign disease that is characterized by rapid proliferation of fibroblastic and myofibroblastic cells. The characteristic gene fusion containing the USP6 gene is a genetic hallmark of NF and MYH9-USP6 is the most frequent fusion, suggesting that NF is not a reactive condition but a neoplastic disease. Malignant transformation of NF has been reported rarely as a single case associated with the PPP6R3-USP6 fusion. Here we report a case of soft part tumor of which the histological feature was a typical NF but showed aggressive and non-regressing growth with local invasion. Targeted RNA sequencing and fluorescence in situ hybridization analysis identified PPP6R3-USP6 with gene amplification. These findings indicate that the present case is the second case of malignant NF, and we suggest potential malignant transformation in certain NF cases.
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Fascitis/diagnóstico , Neoplasias de los Tejidos Conjuntivo y Blando/diagnóstico , Fosfoproteínas Fosfatasas/genética , Ubiquitina Tiolesterasa/genética , Adulto , Transformación Celular Neoplásica , Fascitis/genética , Fascitis/patología , Fusión Génica , Reordenamiento Génico , Humanos , Hibridación Fluorescente in Situ , Masculino , Miofibroblastos/patología , Neoplasias de los Tejidos Conjuntivo y Blando/genética , Neoplasias de los Tejidos Conjuntivo y Blando/patologíaRESUMEN
Complex aortic atheroma is a high-risk factor for recurrent embolic stroke. An accurate identification of stroke etiology is clinically important; however, it can be challenging. A 91-year-old man with atrial fibrillation was diagnosed with cardioembolic stroke and treated with mechanical thrombectomy. The removed thrombus microscopically contained foamy cells, suggesting an atheroembolism. An autopsy revealed an atherosclerotic lesion with ulceration, located in the aortic arch. At the lesion, the plaque had microscopically ruptured into the lumen. We therefore concluded that the aortic atherosclerotic lesion was the embolic source. Removed thrombi should be pathologically examined even if a cardioembolic stroke is clinically suspected.
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Enfermedades de la Aorta/complicaciones , Fibrilación Atrial/complicaciones , Embolia por Colesterol/diagnóstico , Embolia Intracraneal/diagnóstico , Placa Aterosclerótica/complicaciones , Anciano de 80 o más Años , Enfermedades de la Aorta/diagnóstico , Autopsia , Angiografía Cerebral , Embolia por Colesterol/etiología , Embolia por Colesterol/cirugía , Procedimientos Endovasculares/métodos , Resultado Fatal , Humanos , Embolia Intracraneal/etiología , Embolia Intracraneal/cirugía , Imagen por Resonancia Magnética , Masculino , Placa Aterosclerótica/diagnósticoRESUMEN
Carcinosarcomas with elements of cholangiocarcinoma and sarcoma are rare and have a poor prognosis. The spreading pattern and radiological findings of these lesions remain unclear. A 74-year-old man presented with a high γ-glutamyl transferase level. Magnetic resonance imaging revealed dilation of the right intrahepatic and common bile ducts, consistent with an intraductal papillary neoplasm of the bile duct (IPNB), and diffusion-weighted imaging (DWI) indicated an area of high signal intensity in the intrahepatic bile duct. Bile duct biopsy yielded a small amount of atypical spindle cells, and the patient underwent a right hepatectomy. Microscopically, the tumor contained cholangiocarcinoma and sarcomatous components, including osteosarcoma and leiomyosarcoma, leading to a diagnosis of intrahepatic carcinosarcoma. The tumor spread primarily through the intrahepatic bile duct. An accurate radiological diagnosis of carcinosarcoma was challenging, given the apparent similarities with IPNB. The findings from DWI and pathology of a bile duct biopsy may assist with preoperative diagnosis.
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Neoplasias de los Conductos Biliares/diagnóstico , Conductos Biliares Intrahepáticos/patología , Carcinosarcoma/diagnóstico , Colangiocarcinoma/diagnóstico , Anciano , Enfermedades Asintomáticas/terapia , Neoplasias de los Conductos Biliares/sangre , Neoplasias de los Conductos Biliares/patología , Neoplasias de los Conductos Biliares/terapia , Conductos Biliares Intrahepáticos/diagnóstico por imagen , Conductos Biliares Intrahepáticos/cirugía , Biopsia , Carcinosarcoma/sangre , Carcinosarcoma/patología , Carcinosarcoma/terapia , Colangiocarcinoma/sangre , Colangiocarcinoma/patología , Colangiocarcinoma/terapia , Colangiopancreatografia Retrógrada Endoscópica , Embolización Terapéutica , Hepatectomía , Humanos , Pruebas de Función Hepática , Imagen por Resonancia Magnética , Masculino , Vena Porta , Ultrasonografía , gamma-Glutamiltransferasa/sangreRESUMEN
Synovial sarcoma is a malignant tumor, it accounts for approximately 5-10% of the soft tissue sarcoma, and mainly develops in the para-articular regions in adolescents and young adults. We reported a case of synovial sarcoma extending into the vastus intermedius muscle beyond the intermuscular septum which is considered to be the greatest barrier to ensuring a safety margin in musculoskeletal surgery. Preoperative sequential MRI showed the intermuscular-septum line and direct tumoral invasion beyond the intermuscular septum. A histopathological examination showed the direct invasion to the intermuscular septum. Synovial sarcoma seldom shows such direct invasive pattern to barriers, much less the robust barrier like an intermuscular septum. To our knowledge, no previous study has reported such unique extension of a synovial sarcoma beyond the intermuscular septum. Surgeons should be aware of the potential vulnerability of the intermuscular septum.