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1.
OBJECTIVETo further understand the characteristics and behavior of malignant fibrous histiocytoma (MFH) in the clinical setting of chronic lymphocytic leukemia (CLL) or non-Hodgkin lymphoma (NHL).PATIENTS AND METHODSThe patient database of MHF diagnosed at our institution from January 1, 1976, to December 31, 2008, was reviewed. For each MFH case with prior NHL or CLL, 3 matched controls from the same cohort without prior CLL or NHL were randomly selected. A retrospective chart review collected patient data, including sex; age; pathologic confirmation of MFH; tumor grade, size, and location; time since MFH diagnosis; history of chemotherapy or irradiation; treatment of MFH; recurrence; metastasis; and death. The Kaplan-Meier method was used to estimate overall survival, survival free of recurrence, and survival free of metastasis. Cox proportional hazards models were fit to evaluate associations between case status and outcomes.RESULTSFifteen MFH cases with prior NHL or CLL were identified. Cases had frequent bone (n=7 [47%]) and cutaneous (n=5 [33%]) involvement. Five cases (33%) had previous irradiation, 6 (40%) had metastases, and 4 (27%) had recurrence. Overall survival and survival free of recurrence were not significantly different between the cases and controls (5-year overall survival, 49.9% and 58.7%; 12-month survival free of recurrence, 79.4% and 90.3%). However, cases were significantly more likely than controls to have metastasis (hazard ratio, 3.79; 95% confidence interval, 1.22-11.79; P=.02). In addition, survival free of metastasis at 12 months was 65.2% and 90.7%, respectively.CONCLUSIONIn the presence of CLL or NHL, MFH seems to behave more aggressively, suggested by the increased likelihood of metastases compared with controls without prior CLL or NHL. However, overall survival does not appear to be worse in cases of MFH and CLL or NHL than in MFH alone.  相似文献   

2.
BACKGROUNDHutchinson-Gilford progeria syndrome (HGPS) is an extremely rare disease characterized by the rapid appearance of aging with an onset in childhood. Serious cardiovascular complications can be life-threatening events for affected patients and the cause of early death. Herein we report a HGPS patient with osteosarcoma hat was successfully managed and is alive 13 years after the diagnosis. This is the first report describing the detailed surgical procedure and long-term follow-up of osteosarcoma in a patient with HGPS. CASE SUMMARYThe patient was diagnosed with HGPS at 5 years of age with typical features and was referred to our department with a suspected bone tumor of the left proximal tibia at the age of 18. Open biopsy of the tibial bone tumor revealed a conventional fibroblastic osteosarcoma. We have developed and performed a freezing technique using liquid nitrogen for tumor reconstruction. This technique overcame the small size of the tibia for megaprosthesis and avoided amputation and limb salvage was achieved 13 years post-operatively. Although the patient had a number of surgical site complications, such as wound dehiscence, and superficial and deep infections due to vulnerable skin in HGPS, no recurrence or metastases were detected for 13 years, and she walks assisted by crutches. Her general health was good at the latest follow-up at 31 years of age.CONCLUSIONA HGPS patient with osteosarcoma was successfully managed and she was alive 13 years after the diagnosis.  相似文献   

3.
BACKGROUNDMalignant solitary fibrous tumors (SFTs) account for 15%-20% of all SFTs, and malignant SFTs arising from the greater omentum are extremely rare. Most malignant SFTs of the greater omentum are diagnosed via pathological examinations after surgery. In this study, we report a case of malignant omental SFT and review the published literature on this rare malignancy.CASE SUMMARYA 64-year-old female presented with an abdominal mass, and underwent exploratory surgery, during which a huge tumor originating from the greater omentum and intraperitoneal implants were identified and resected. The results of the pathological examination, immunohistochemistry staining, and gene sequencing led to the diagnosis of malignant SFT of the greater omentum. The patient died one and a half years later due to tumor recurrence and metastasis.CONCLUSIONThis is the first report of the application of gene sequencing in the diagnosis of malignant SFTs of the greater omentum.  相似文献   

4.
目的 观察腹部恶性纤维组织细胞瘤(MFH)的CT表现。方法 回顾性分析16例经病理证实的腹部MFH患者的临床、病理及影像学资料。结果 所有患者均接受CT平扫及增强扫描。11例单发,5例多发;腹膜后7例,肠系膜4例,肝脏3例,十二指肠、肾脏各1例。CT平扫15例病变呈低密度,1例呈等密度。1例病灶内见颗粒状钙化灶,2例周边见大量条索影。增强扫描12例病变不均匀强化,2例均匀强化,2例肝内MFH未见强化,其内可见分隔。病理结果:多型性8例,炎症型2例,黏液型1例,未明确分型MFH 5例。结论 腹部MFH缺乏特征性影像学表现,综合分析有助于术前诊断。  相似文献   

5.
ObjectivesCharacterize the course of depressive symptoms during the first year after dysvascular amputation and identify factors that predict symptom trajectories.DesignProspective cohort study of individuals undergoing lower extremity amputation (LEA), surveyed at 4 time points (perioperative period, 6 weeks, 4 months, and 12 months postamputation). Multilevel modeling was used to describe and predict trajectories.SettingFour Veterans Affairs medical centers, a university hospital, and a level I trauma center.ParticipantsParticipants (N=141; 74% retention) were a consecutive sample, eligible if they were undergoing their first unilateral LEA secondary to dysvascular disease.InterventionsNot applicable.Main Outcome MeasurePatient Health Questionnaire-9.ResultsApproximately 40% of participants endorsed at least moderate depressive symptoms at perioperative baseline. Individuals with greater depressive symptoms in the perioperative period concurrently reported greater pain, poorer self-rated health, and prior mental health treatment. In the first 6 weeks after amputation there was a substantial improvement in depressive symptoms, especially among individuals with greater symptoms at baseline. Depressive symptoms were generally stable after 6 weeks. None of the covariates assessed significantly predicted trajectories of depressive symptom improvement.ConclusionsWatchful waiting may be the most appropriate course of action for many patients in the first 6 weeks after amputation. After 6 weeks, however, symptom levels tend to stabilize, suggesting that active intervention is called for if patients remain depressed at this point. Some patients may benefit from more proactive intervention, such as those with prior mental health treatment histories.  相似文献   

6.
Czerniecki JM, Turner AP, Williams RM, Hakimi KN, Norvell DC. Mobility changes in individuals with dysvascular amputation from the presurgical period to 12 months postamputation.ObjectivesTo describe changes in ambulation among individuals with lower-extremity amputation secondary to peripheral artery disease and/or diabetes prior to surgery through 12 months after surgery. To compare differences in ambulation by amputation level and to examine risk factors for change in ambulation over time.DesignProspective cohort study.SettingTwo Veterans Affairs medical centers, 1 university hospital, and a level I trauma center.ParticipantsPatients with peripheral artery disease or diabetes (N=239) undergoing a first unilateral major amputation were screened for participation between September 2005 and December 2008. Among these, 57% (n=136) met study criteria, and of these, 64% (n=87) participated.InterventionsStandard of care at each facility.Main Outcome MeasuresAmbulatory function measured using the Locomotor Capability Index-5.ResultsSeventy-five of the 87 (86%) subjects enrolled finished their 12-month follow-up interview. Ambulatory mobility declined during the period immediately prior to surgery (premorbid) and remained low at 6 weeks postsurgery. On average, ambulation improved after surgery but did not return to premorbid levels. In the final multivariate model, age and history of lower-extremity arterial reconstruction were significantly associated with a poorer ambulatory trajectory over time, while other factors, such as amputation level, prior alcohol use, and length of disability prior to amputation, were not.ConclusionsThe findings highlight the importance of considering premorbid ambulatory function. Informing providers and patients about the trajectory and time course of changes in ambulation can enhance patient education, patient expectations, and treatment planning.  相似文献   

7.
Purpose of ReviewMegaprosthesis and Allograft Prosthesis Composite (APC) are the established treatment modalities for massive skeletal defects. There are a handful of studies comparing the use of megaprosthesis and APC in the management of substantial bone loss and it has always been a topic of debate regarding the superiority of one modality over the other. Therefore, we aim to compare the functional outcome and implant survivorship of each modality including complications, revision rates, amputation rate and mortality.Recent FindingsThe Allograft Prosthesis Composite (APC) constitutes a skeletal allograft implanted with a revision type prosthesis in it. The biological environment provided by the allograft allows attachment of the muscles and tendons imparting better stability and function. However, the literature is not kind enough with APC due to associated risk of infection, disease transmission and nonunion at the graft–host junction. The megaprosthesis (MP) on the other hand is a nonbiologic modality with better survivorship but subservient functional outcome. Infection has been a major issue in both the modalities. Advancement in metallurgy using silver coated megaprosthesis also failed to provide strong evidence in preventing infection.SummaryThe functional outcome is better with APC in both the upper and lower limbs. However, the survivorship is better with megaprosthesis, especially in the upper limb when revision rates were compared between the two modalities. Deep infection and mechanical complications were significantly higher in the APC group. There was no significant difference between the two groups in terms of amputation rate, mortality, and local recurrence.Level of Evidence (CEBM)2a  相似文献   

8.
BACKGROUNDKeratinized gingival insufficiency is a disease attributed to long-term tooth loss, can severely jeopardizes the long-term health of implants. A simple and effective augmentation surgery method should be urgently developed.CASE SUMMARYA healthy female patient, 45-year-old, requested implant restoration of the her left mandibular first molar and second molar. Before considering a stage II, as suggested from the probing depth measurements, the widths of the mesial, medial, and distal buccal keratinized gingiva of second molar (tooth #37) were measured and found to be 0.5 mm, 0.5 mm, and 0 mm, respectively. This suggested that the gingiva was insufficient to resist damage from bacterial and mechanical stimulation. Accordingly, modified apically repositioned flap (ARF) surgery combined with xenogeneic collagen matrix (XCM) and platelet-rich fibrin (PRF) was employed to increase the width of gingival tissue. After 1 mo of healing, the widths of mesial, medial, and distal buccal keratinized gingiva reached 4 mm, 4 mm, and 3 mm, respectively, and the thickness of the augmented mucosa was 4.5 mm. Subsequently, through the second-stage operation, the patient obtained an ideal soft tissue shape around the implant.CONCLUSIONFor cases with keratinized gingiva widths around implants less than 2mm,the soft tissue width and thickness could be increased by modified ARF surgery combined with XCM and PRF. Moreover, this surgery significantly alleviated patients’ pain and ameliorated oral functional comfort.  相似文献   

9.
BACKGROUNDMammary analogue secretory carcinoma (MASC) is a rare low-grade malignant salivary gland tumor. The morphological and immunohistochemical features of MASC closely resemble those of breast secretory carcinoma. The key characteristics of the lesion are a lack of pain and slow growth. There is no obvious specificity in the clinical manifestations and imaging features. The diagnosis of the disease mainly depends on the detection of the MASC-specific ETV6-NTRK3 fusion gene. CASE SUMMARYThis report describes a rare case of a 32-year-old male patient who presented with a gradually growing lesion that was initially diagnosed as breast-like secretory carcinoma of the right parotid gland. Imaging and histological investigations were used to overcome the diagnostic difficulties. The lesion was managed with right parotidectomy, facial nerve preservation, biological patch implantation to restore the resulting defect, and postoperative radiotherapy. On postoperative follow-up, the patient reported a mild facial deformity with no complications, signs of facial paralysis, or Frey’s syndrome. CONCLUSIONThe imaging and histological diagnostic challenges for MASC are discussed.  相似文献   

10.
BackgroundThe mechanical parameters of the Ilizarov fixator are influenced by many factors related to its spatial configuration. The aim of this study was to experimentally evaluate the impact of the type of implant and the number of distraction rods on the uniformity of loading of distraction rods and stiffness coefficients of the Ilizarov fixator.MethodsThe tests were carried out on a physical model. The model was mounted in a universal loading station MTS 858 Mini Bionix. Forces in distraction rods are measured with the use of strain gauge force transducers. Displacements of bone fragments were measured by means of digital image correction.FindingsIn the case of a fixator with 3 distraction rods, configuration of implants has no statistically significant effect on the forces occurring in distraction rods. In the case of a fixator with 4 distraction rods, there are statistically significant differences in the forces in distraction rods. The highest transverse stiffness coefficients occurs in the system with 4 distraction rods and the configuration of 1K1S implants, while the smallest transverse stiffness coefficients occurs in the fixator with 4 distraction rods and the configuration of 0K1S implants.InterpretationBased on the results of experiments on the physical model, we observed an asymmetry in the distribution of forces transmitted through distraction rods. The configuration with 3 distraction rods provides more uniform distribution of forces in distraction rods and reduces the impact of the implant configuration on transverse stiffness coefficients. The use of a configurations with 4 rods may be advantageous when we want to obtain greater transverse stiffness coefficients of a system consisting of the fixator and fragments of the lengthened bone.  相似文献   

11.
BACKGROUNDMelanoma brain metastasis is a common cause of death in melanoma patients and is associated with a poor prognosis. There are relatively few reports on intracranial infections after brain metastasis resection.CASE SUMMARYHere we report a case of melanoma brain metastases in a patient harboring a BRAF V600E mutation, who experienced intracranial tumor progression despite previous combined treatment with a programmed death (PD)-1 inhibitor, axitinib, and vemurafenib. She repeatedly underwent local therapy, including stereotactic radiosurgery and intracranial surgery, and developed central nervous system infection. Treatment with vemurafenib combined with cobimetinib resulted in an intracranial progression-free survival of 10 mo. During the coronavirus disease 2019 (COVID-19) pandemic, the patient did not visit the hospital for regular vemurafenib treatment, and experienced intracranial progression after involuntary drug reduction for 1 mo. The patient subsequently received various systemic treatments including vemurafenib, PD-1 inhibitor, and chemotherapy, with an overall survival of 29 mo as of September 2020.CONCLUSIONWe report the first case of melanoma brain metastases with co-occurring intracranial infection and unintended drug reduction during the COVID-19 outbreak. Long-term control of the intracranial lesions was achieved with systemic and local therapies.  相似文献   

12.
Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma created from fibroblast cells and characterized by a high rate of metastasis or recurrence with poor prognosis. We report a case of initially well differentiated (G1) MFH of the trunk in a 33-year-old woman. Two years after primary diagnosis, metastases were found in the lung, trunk, gluteus region, upper extremities and brain. Histopathological findings indicated a stromal tumor consisting of spindle cells, and immunohistochemical examination of resected specimens established the definite diagnosis of poorly differentiated MFH (G3). Initial surgery of several solid tumors on the trunk, lung and extremities was performed. There was a high local recurrence and metastasis rate, and the patient was treated with radiotherapy and conventional chemotherapy followed by tandem high-dose chemotherapy and peripheral blood stem-cell transplantation. She is currently well seven years after the transplant, with no signs of metastasis and recurrence. We review the clinical picture of the tumor in this patient and discuss its diagnosis, pathogenesis and treatment.  相似文献   

13.
BACKGROUNDMerkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine neoplasia, with high risk of recurrence and metastasis and poor survival. Immune checkpoint inhibitors, like the anti-programmed death-ligand 1 agent avelumab, were recently approved for the treatment of advanced MCC. We, herein, report the first case of advanced MCC with oligoprogression managed with avelumab and local radical treatment.CASE SUMMARYA 61-year-old man was presented to the hospital with sporadic fever and an exudative malodorous mass (10 cm of diameter), located on the right gluteal region. The final diagnosis was MCC, cT4N3M1c (AJCC, TNM staging 8th edition, 2017), with invasion of adjacent muscle, in-transit metastasis, and bone lesions. Patient started chemotherapy (cisplatin and etoposide), and after six cycles, the main tumor increased, evidencing disease progression. Two months later, the patient started second line treatment with avelumab (under an early access program). After two cycles of treatment, the lesion started to decrease, achieving a major response. Local progression was documented after 16 cycles. However, as the tumor became resectable, salvage surgery was performed, while keeping the systemic treatment with avelumab. Since the patient developed bilateral pneumonia, immunotherapy was suspended. More than 2.5 years after surgery (last 19 mo without systemic therapy), the patient maintains complete local response and stable bone lesions.CONCLUSIONThis report highlights the efficacy and long-term response of avelumab on the management of a chemotherapy resistant advanced MCC, with evidence of oligoprogression, in combination with local radical treatment.  相似文献   

14.
《Clinical therapeutics》2023,45(8):797-801
PurposeInfected diabetic foot ulcers can be difficult to treat and, despite appropriate antibiotic therapy, some diabetic foot infections (DFIs) require amputation. Bacteriophages (phages) are viruses that infect and kill bacteria. Phage therapy has been repeatedly used to successfully treat DFIs and other chronic wounds.MethodsThis article reports the provision of topical adjunctive anti-staphylococcal phage therapy to 10 patients with DFI at high risk of amputation at two UK hospitals as part of clinical care; tolerability and efficacy were clinically assessed.FindingsThe opinion of the experienced clinical teams caring for these patients was that 9 of the 10 patients appeared to benefit from adjunctive phage therapy. No adverse effects were reported by clinicians or patients. In 6 of 10 patients the clinical impression was that phage therapy facilitated clinical resolution of infection and limb salvage. Resolution of soft tissue infection was observed in a 7th patient but unresolved osteomyelitis required amputation. An 8th patient demonstrated eradication of Staphylococcus aureus from a polymicrobial infection and a 9th showed signs of clinical improvement before early cessation of phage therapy due to an unrelated event. One patient, with a weakly susceptible S aureus isolate, had no significant response.ImplicationsThis report describes the largest application of phage therapy in the United Kingdom to date and the first application of phage therapy for DFI in the United Kingdom and offers subjective hints toward impressive tolerability and efficacy. Phage therapy has the potential to transform the prevention and treatment of DFIs.  相似文献   

15.
BACKGROUNDSclerosing angiomatoid nodular transformation (SANT) is a rare disease of the spleen. It has unique pathological features and mimics splenic tumor on radiological imaging.CASE SUMMARYA 47-year-old woman was incidentally found to have a splenic mass on abdominal ultrasound. She had a 10-cm postoperative scar in the lower abdomen due to previous cesarean sections. The patient had a past history of anemia of unknown etiology for 20 years. The patient underwent laparoscopic splenectomy. The postoperative course was uneventful, with a hospital stay of 7 d. The histopathological examination of the spleen revealed SANT. At the 6-mo follow-up, the patient remained disease-free.CONCLUSIONSANT is a rare benign disease mimicking a malignant tumor. A definitive diagnosis can be made only on histopathology.  相似文献   

16.
BACKGROUNDInverted papilloma is an uncommon neoplasm in the nasal cavity. It is a histologically benign tumor, but has a high recurrence and local invasion rate. In addition, nasal or skull base lymphoma is another rare neoplasm. The coexistence of these two tumors in one case makes the diagnosis and related treatment difficult.CASE SUMMARYWe report a case of an immunocompetent patient, who had a history of inverted papilloma 20 years ago. The patient presented with an infiltrated mass lesion in the nasal cavity with extension to the frontal base. The repeated biopsies revealed inverted papilloma without any malignant transformation. After the patient underwent a frontobasal craniotomy with total tumor excision, the final pathological examination revealed nasal inverted papilloma coexisting with diffuse large B-cell lymphoma of the skull base.CONCLUSIONBased on this case report, while managing a case of an aggressive recurrent inverted papilloma, not only squamous cell carcinoma transformation, but also other invasive malignancy, such as lymphoma, should be considered.  相似文献   

17.
Czerniecki JM, Turner AP, Williams RM, Hakimi KN, Norvell DC. The effect of rehabilitation in a comprehensive inpatient rehabilitation unit on mobility outcome after dysvascular lower extremity amputation.ObjectivesTo (1) compare the total volume of rehabilitation therapy for patients ever attending a comprehensive inpatient rehabilitation unit (CIRU) versus never during the 12 months after amputation; (2) determine whether rehabilitation in a CIRU at any time in the first year after amputation results in greater mobility success compared with other types of rehabilitation environments of care; and (3) determine for those patients treated in a CIRU, which specific patient characteristics were associated with improved mobility outcome.DesignProspective cohort study.SettingTwo Veterans Affairs medical centers.ParticipantsPatients (N=199) with peripheral vascular disease or diabetes undergoing a first unilateral major amputation were screened for participation between September 2005 and December 2008. Among these, 113 (57%) met study criteria; of these, 72 (64%) participated.InterventionEver attending a CIRU versus never attending a CIRU in first 12 months after amputation.Main Outcome MeasuresNumber of rehabilitation therapy visits, Locomotor Capability Index scores, and mobility success.ResultsThe mean number of all therapy visits for patients ever attending a CIRU was significantly greater than that for those never attending over a 12-month period (48.6 vs 22.6; P=.001). Mean total time per any rehabilitation visit was .83±.27 hours for those ever attending and .60±.20 hours for those never attending (P<.001). Patients who ever were treated in a CIRU were 17% more likely to achieve mobility success than those who were not, controlling for amputation level, major depressive episode, alcohol use, social support, total number of rehabilitation visits, and hospital site (risk difference=.17; 95% confidence interval, .09–.25; P<.001).ConclusionsRehabilitation in a CIRU resulted in improved mobility success for veterans undergoing major lower extremity amputation secondary to peripheral vascular disease or diabetes. Among those admitted to a CIRU, younger patients with greater social support, healthy weight, and without chronic obstructive pulmonary disease had the greatest probability of mobility success.  相似文献   

18.
19.
BackgroundBody Center Of Mass velocity assessment is a prerequisite for several applications in prosthetic control and rehabilitation monitoring. Force plate data integration is a promising alternative to full-body quantitative analysis of segmental kinematics to estimate the velocity. Still, it remains to be implemented and validated for people with transfemoral amputation.MethodsTwo methods were used (force plate based and pelvic markers based) for Body Center Of Mass velocity estimation in a clinical context. The two methods were comparatively assessed on overground walking data of eight people with transfemoral amputation in a laboratory equipped with a motion capture system and force plates compared to reference estimation derived from a full body segmental gait analysis. The ‘Methods’ agreement with the reference was quantified from the Bland and Altman procedure.FindingsThe estimation of Body Center Of Mass velocity from force plate data integration was considered acceptable in terms of limits of agreement. In addition, the hypotheses used to determine integration constants were evaluated and shown to be reasonable as far as the walking direction is well controlled.InterpretationResults demonstrate the possibility to use the force plate method to assess the Body Center Of Mass velocity of people with transfemoral amputation for straight walking on level ground. An estimation from the velocity of pelvic markers can also be a relevant alternative as soon as the walking velocity remains low. Further investigation will deal with the impact of the errors on the computation of derived parameters such as individual limb power.  相似文献   

20.
BACKGROUNDPlexiform fibromyxoma (PF) is a rare mesenchymal tumor of the stomach. The clinical features of PF frequently include upper abdominal pain, abdominal discomfort, hematemesis, melena, pyloric obstruction and an upper abdominal mass. We herein report a case of PF resected by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.CASE SUMMARYThe patient was admitted to hospital, due to a 1-wk history of an abdominal space-occupying lesion identified during a health examination. He underwent complete resection by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy. During the operation, the tumor was located in the anterior wall of the gastric antrum (approximately 7 cm × 6 cm × 5.5 cm) and did not show evidence of invasion of the serosa. Histology showed that the tumor cells were oval fibroblast-like and spindle-shaped cells, with numerous thin-walled blood vessels and abundant myxoid stroma. Cellular atypia and mitosis were both rare. Immunohistochemistry showed that the tumor cells were immunoreactive for smooth muscle actin, S-100 and CD-10, but were negative for CD-117, CD-34, DOG-1, and ALK. In this case, S-100 was positive and no significant disease was observed during the follow-up period.CONCLUSIONThe fact that PF is a rare tumor with only a few cases in this region can lead to misdiagnosis of this entity and pose a real diagnostic challenge for general surgeons and pathologists when encountering such patients and differentiating PF from other primary tumors of gastric mesenchymal origin. Our report may help increase awareness of this rare, but important new disease entity.  相似文献   

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