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1.
Dermatol Online J ; 26(4)2020 Apr 15.
Artigo em Inglês | MEDLINE | ID: mdl-32621685

RESUMO

Mid-dermal elastolysis is a rare acquired elastic tissue disorder with about 100 cases reported in the literature. It is characterized by localized patches of finely wrinkled skin on the shoulder and upper extremities and a band-like loss of elastic tissue in the mid-dermal layer on biopsy. Some patients may have symptoms of discomfort, erythema, and/or pruritis. Mid-dermal elastolysis is predominantly seen in young to middle-aged Caucasian females and extensive skin involvement may lead to cosmetic concerns. Furthermore, it is important to rule out other disorders of elastic fiber that are associated with systemic involvement. We present a case of MDE, discuss the differential diagnosis, and describe characteristic clinical features and histology findings of each condition.


Assuntos
Cútis Laxa/patologia , Derme/patologia , Tecido Elástico/patologia , Adulto , Braço/patologia , Biópsia , Feminino , Humanos
2.
Phys Ther ; 100(3): 457-467, 2020 03 10.
Artigo em Inglês | MEDLINE | ID: mdl-32043125

RESUMO

BACKGROUND: There are challenges related to the accurate and efficient measurement of lymphedema in people with breast cancer. The LymphaTech 3D Imaging System (LymphaTech, Atlanta, GA, USA) is a mobile, noninvasive platform that provides limb geometry measurements. OBJECTIVE: The objective of this study was to estimate the reliability and validity of the LymphaTech for measuring arm volume in the context of women seeking care in a specialty breast cancer rehabilitation clinic. DESIGN: This was a cross-sectional reliability and convergent validity study. METHODS: People who had stage I to IV breast cancer with lymphedema or were at risk for it were included. Arm volume was measured in 66 participants using the LymphaTech and perometer methods. Test-retest reliability for a single measure, limb volume difference, and agreement between methods was analyzed for 30 participants. A method-comparison analysis was also used to assess convergent validity between methods. RESULTS: Both LymphaTech and perometer methods displayed intraclass correlation coefficients (ICCs) of ≥0.99. The standard errors of measurement for the LymphaTech and length-matched perometer measurements were nearly identical. Similar intraclass correlation coefficients (0.97) and standard errors of measurement (38.0-40.7 mL) were obtained for the between-limb volume difference for both methods. The convergent validity analyses demonstrated no systematic difference between methods. LIMITATIONS: The sample size was not based on a formal sample size calculation. LymphaTech measurements included interrater variance, and perometer measurements contained intrarater variance. CONCLUSIONS: The LymphaTech had excellent test-retest reliability, and convergent validity was supported. This technology is efficient and portable and has a potential role in prospective surveillance and management of lymphedema in clinical, research, and home settings.


Assuntos
Braço/diagnóstico por imagem , Neoplasias da Mama/terapia , Diagnóstico por Computador/instrumentação , Linfedema/diagnóstico por imagem , Aplicativos Móveis , Adulto , Idoso , Braço/patologia , Neoplasias da Mama/patologia , Estudos Transversais , Diagnóstico por Computador/métodos , Feminino , Humanos , Linfedema/etiologia , Pessoa de Meia-Idade , Tamanho do Órgão , Posicionamento do Paciente , Reprodutibilidade dos Testes , Fatores de Risco , Tamanho da Amostra , Interface Usuário-Computador
3.
Lymphology ; 52(3): 134-142, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31874125

RESUMO

The objective of this study was to evaluate the accuracy of bioimpedance spectroscopy measurements (L-Dex) in the diagnosis of breast cancer-related lymphedema. A retrospective review of a prospectively maintained database was performed of all patients that underwent surgical treatment for breast cancer at a tertiary medical center. Patients who had preoperative and postoperative evaluation for possible lymphedema by limb circumference measurements and bioimpedance were eligible for inclusion in the study. No significant demographic differences were found between the group of patients clinically diagnosed with lymphedema (N=134) and those without a clinical diagnosis of lymphedema (N=261). The ability of bioimpedance to diagnose lymphedema based on the manufacturer's criteria demonstrated low sensitivity, which was 7.5% when lymphedema was defined as an absolute L-Dex value greater than 10, and 24.6% when defined as a relative change of >10 between preoperative and postoperative measurements. This corresponded with a positive predictive value of 61-71% and a negative predictive value of 67-70%. We are unable to recommend the use of bioimpedance as a screening tool or for measurement of breast cancer-related lymphedema.


Assuntos
Linfedema Relacionado a Câncer de Mama/diagnóstico , Espectroscopia Dielétrica , Impedância Elétrica , Idoso , Braço/patologia , Braço/fisiopatologia , Linfedema Relacionado a Câncer de Mama/etiologia , Neoplasias da Mama/complicações , Neoplasias da Mama/cirurgia , Espectroscopia Dielétrica/métodos , Feminino , Humanos , Pessoa de Meia-Idade , Estudos Retrospectivos , Índice de Gravidade de Doença
4.
Spine (Phila Pa 1976) ; 44(23): 1676-1684, 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-31730573

RESUMO

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: To evaluate the performance and convergent validity of the disabilities of the arm, shoulder, and hand (DASH) in comparison with the visual analog scale (VAS) for pain, and neck disability index (NDI) in patients undergoing cervical spine surgery. SUMMARY OF BACKGROUND DATA: Neck-specific disability scales do not adequately assess concurrent upper extremity involvement in patients with cervical spine disorders. The DASH is a patient-reported outcomes (PRO) instrument designed to measure functional disability due to upper extremity conditions but has additionally been shown to perform well in patients with neck disorders. METHODS: We identified patients who underwent cervical spine surgery at our institution between 2013 and 2016. We collected demographic information, clinical characteristics, and PRO measures-DASH, VAS, NDI-preoperatively, as well as early and late postoperatively. We calculated descriptive statistics and changes from baseline in PROs. Correlation coefficients were used to quantify the association between PRO measures. The analysis was stratified by radiculopathy and myelopathy diagnoses. RESULTS: A total of 1046 patients (52.8% male) with PROs data at baseline were included in the analysis. The mean age at surgery ±â€ŠSD was 57.2 ±â€Š11.3 years, and postoperative follow-up duration 12.7 ±â€Š10.7 months. The most common surgical procedure was anterior cervical discectomy and fusion (71.1%). Patients experienced clinically meaningful postoperative improvements in all PRO measures. The DASH showed moderate positive correlations with VAS preoperatively (Spearman rho = 0.43), as well as early (rho = 0.48) and late postoperatively (rho = 0.60). DASH and NDI scores were strongly positively correlated across operative states (Preoperative rho = 0.74, Early Postoperative rho = 0.78, Late Postoperative rho = 0.82). Stratified analysis by preoperative diagnosis showed similar within-groups trends and pairwise correlations. However, radiculopathy patients experienced larger magnitude early and late change scores. CONCLUSION: The DASH is a valid and responsive PRO measure to evaluate disabling upper extremity involvement in patients undergoing cervical spine surgery. LEVEL OF EVIDENCE: 3.


Assuntos
Vértebras Cervicais/cirurgia , Avaliação da Deficiência , Medidas de Resultados Relatados pelo Paciente , Doenças da Coluna Vertebral/cirurgia , Adulto , Idoso , Braço/patologia , Feminino , Mãos/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Medição da Dor/normas , Reprodutibilidade dos Testes , Estudos Retrospectivos , Ombro/patologia , Doenças da Coluna Vertebral/diagnóstico , Resultado do Tratamento
5.
Indian J Pathol Microbiol ; 62(4): 618-620, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31611455

RESUMO

Opportunistic fungal infections occur predominantly in immunocompromised (IC) patients. Mucormycosis has the highest mortality among fungal infections. The organism is ubiquitous in its presence. The infection is commonly acquired by inhalation of fungal spores or by inoculation by direct trauma. Rhinocerebral and pulmonary mucormycosis present commonly with high mortality rates. Cutaneous mucormycosis (CM) is rarely reported and usually presents in two forms, superficial and disseminated. The superficial infection occurs commonly in immunocompetent hosts, and it can sometimes turn angioinvasive and become a fatal and very aggressive disseminated disease, especially in IC hosts. Coexistant risk factors increase the mortality rate. We report a case of posttraumatic, highly lethal, angioinvasive, and gangrenous type of CM of the arm in an IC patient. Diabetic ketoacidosis and cirrhosis due to chronic alcoholic liver disease were the risk factors.


Assuntos
Braço/microbiologia , Hospedeiro Imunocomprometido , Mucormicose/diagnóstico , Infecções Oportunistas/diagnóstico , Pele/patologia , Adulto , Braço/patologia , Evolução Fatal , Gangrena , Humanos , Masculino , Pele/microbiologia
7.
Rev. esp. patol ; 52(3): 190-193, jul.-sept. 2019. ilus
Artigo em Inglês | IBECS | ID: ibc-191935

RESUMO

Chondroid stromal change is very rare in melanocytic nevi. We present a severely atypical symmetrical Spitz tumor on the right arm of a 24-years-old man. It showed maturation and occasional mitotic figures. The melanocytes were intermingled with cartilaginous stroma. The Ki67 proliferative rate was 5%. It was weakly positive for p16 and negative for BRAF/V600E. BAP1 expression was preserved. PCR for Myriad myPath(R) Melanoma test was also negative


El cambio estromal condroide es muy raro en los nevi melanocíticos. Presentamos un tumor de Spitz simétrico con atipia severa en el brazo derecho de un varón de 24 años. Mostraba maduración y muy ocasionales figuras mitóticas. Los melanocitos estaban entremezclados con estroma cartilaginoso. El índice de proliferación con Ki67 era del 5%. El tumor era débilmente positivo para p16 y negativo para BRAF/V600E. La expresión de BAP1 estaba conservada. El test de PCR para Myriad myPat(R) Melanoma también fue negativo


Assuntos
Humanos , Masculino , Adulto Jovem , Melanócitos/patologia , Neoplasias Cutâneas/patologia , Condroma/patologia , Nevo de Células Epitelioides e Fusiformes/patologia , Nevo Pigmentado/patologia , Metaplasia/patologia , Braço/patologia , Biomarcadores Tumorais/análise
8.
BMJ Case Rep ; 12(8)2019 Aug 20.
Artigo em Inglês | MEDLINE | ID: mdl-31434667

RESUMO

A literature search confirmed no previous cases of necrotising fasciitis (NF) complicating hand,foot and mouth disease (HFMD). This report explores the case of a previously well 55-week-old Caucasian boy who attended accident and emergency with an acutely swollen right hand and atypical viral rash affecting the hands and face. He was admitted under plastic surgery and treated with intravenous antibiotics and fluid resuscitation for sepsis secondary to cellulitis. Following dermatological review of the rash, a clinical diagnosis of atypical HFMD was made. He deteriorated over the first 12 hours with progression of cellulitis despite intervention. Emergency exploration and debridement were performed for suspected NF. NF was subsequently confirmed by laboratory testing. He required 5 days in paediatric intensive care but made a full recovery. Recent reports highlight an increase in atypical cases of HFMD. Clinicians should be aware of the potential for superadded necrotising infection in cases of atypical HFMD.


Assuntos
Antibacterianos/uso terapêutico , Braço/patologia , Celulite (Flegmão)/patologia , Cuidados Críticos , Fasciite Necrosante/diagnóstico , Doença de Mão, Pé e Boca/diagnóstico , Administração Intravenosa , Braço/cirurgia , Celulite (Flegmão)/fisiopatologia , Celulite (Flegmão)/terapia , Desbridamento , Fasciite Necrosante/complicações , Fasciite Necrosante/fisiopatologia , Fasciite Necrosante/terapia , Hidratação , Doença de Mão, Pé e Boca/complicações , Doença de Mão, Pé e Boca/fisiopatologia , Humanos , Lactente , Masculino , Procedimentos Cirúrgicos Reconstrutivos , Resultado do Tratamento
9.
BMJ Case Rep ; 12(8)2019 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-31420428

RESUMO

Soft tissue sarcomas account for about 1% of malignant tumours. More than 50 subtypes of these tumours have been described with some being extremely rare, namely malignant peripheral nerve sheath tumours (MPNST). The authors present a case of a man aged 81 years with a medical history of an adenocarcinoma of the rectum, which was referred to our clinic due to a growing painless mass on the right arm. An MRI showed a well-delimited encapsulated mass involving the long head of the biceps muscle. Biopsy findings revealed a spindle cell tumour with cytonuclear pleomorphism. The patient underwent wide tumour excision and was successfully reconstructed with a latissimus dorsi functional muscle transfer. The presence of two pulmonary nodules on CT scan staging implied a lung biopsy that showed rectum primary tumour metastases. With these additional findings, the pathology department reassessed the case and reclassified the arm tumour as an MPNST, synchronous with pulmonary adenocarcinoma metastases of the rectum.


Assuntos
Adenocarcinoma/patologia , Neoplasias Pulmonares/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias da Bainha Neural/patologia , Neoplasias Retais/patologia , Adenocarcinoma/secundário , Idoso de 80 Anos ou mais , Braço/inervação , Braço/patologia , Humanos , Neoplasias Pulmonares/secundário , Masculino
12.
PLoS One ; 14(8): e0219745, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31386678

RESUMO

OBJECTIVES: This study aims to describe the mortality risk of children in the community who had severe acute malnutrition (SAM) defined by either a mid-upper arm circumference (MUAC) <115mm, a low weight-for-height Z-score (WHZ) <-3 or both criteria. METHODS: We pooled individual-level data from children aged 6-59 months enrolled in 3 community-based studies in the Democratic Republic of the Congo (DRC), Senegal and Nepal. We estimate the mortality hazard using Cox proportional hazard models in groups defined by either anthropometric indicator. RESULTS: In total, we had 49,001 time points provided by 15,060 children available for analysis, summing to a total of 143,512 person-months. We found an increasing death rate with a deteriorating nutritional status for all anthropometrical indicators. Children identified as SAM only by a low MUAC (<115mm) and those identified only by a low WHZ (Z-score <-3) had a similar mortality hazard which was about 4 times higher than those without an anthropometric deficit. Having both a low MUAC and a low WHZ was associated with an 8 times higher hazard of dying compared to children within the normal range. The 2 indicators identified a different set of children; the proportion of children identified by both indicators independently ranged from 7% in the DRC cohort, to 35% and 37% in the Senegal and the Nepal cohort respectively. CONCLUSION: In the light of an increasing popularity of using MUAC as the sole indicator to identify SAM children, we show that children who have a low WHZ, but a MUAC above the cut-off would be omitted from diagnosis and treatment despite having a similar risk of death.


Assuntos
Características de Residência/estatística & dados numéricos , Desnutrição Aguda Grave/epidemiologia , Braço/patologia , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Desnutrição Aguda Grave/mortalidade , Desnutrição Aguda Grave/patologia
14.
PLoS One ; 14(6): e0218193, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31246958

RESUMO

BACKGROUND: The Philippines is ranked 3rd globally for tuberculosis incidence (554/100,000 population). The tuberculosis ward at San Lazaro Hospital, Manila receives 1,800-2,000 admissions of acutely unwell patients per year with high mortality. Objectives of this prospective cohort study were to quantify the association of under-nutrition (primary) and diabetes (secondary) with inpatient mortality occurring between 3-28 days of hospital admission in patients with suspected or previously diagnosed TB. METHODS AND RESULTS: We enrolled 360 adults (≥18 years); 348 were eligible for the primary analysis (alive on day 3). Clinical, laboratory, anthropometric and enhanced tuberculosis diagnostic data were collected at admission with telephone tracing for mortality up to 6 months post-discharge. In the primary analysis population (mean age 45 years, SD = 15.0 years, 70% male), 58 (16.7%) deaths occurred between day 3-28 of admission; 70 (20.1%) between day 3 and discharge and documented total post-day 3 mortality including follow-up was 96 (27.6%). In those in whom it could be assessed, body mass index (BMI) ranged from 11.2-30.6 kg/m2 and 141/303 (46.5%) had moderate/severe undernutrition (BMI<17 kg/m2). A sex-specific cut-off for mid-upper arm circumference predictive of BMI<17 kg/m2 was associated with inpatient Day 3-28 mortality in males (AOR = 5.04, 95% CI: 1.50-16.86; p = 0.009; p = 0.032 for interaction by sex). The inability to stand for weight/height for BMI assessment was also associated with mortality (AOR = 5.59; 95% CI 2.25-13.89; p<0.001) as was severe compared to normal/mild anaemia (AOR = 9.67; 95% CI 2.48-37.76; p<0.001). No TB specific variables were associated with Day 3-28 mortality, nor was diabetes (HbA1c ≥6.5% or diabetes treatment). Similar effects were observed when the same multivariable model was applied to confirmed TB patients only and to the outcome of all post-day 3 in-patient mortality. CONCLUSION: This research supports the use of mid-upper arm circumference for triaging acutely unwell patients and the design and testing of nutrition-based interventions to improve patient outcomes.


Assuntos
Braço/patologia , Tuberculose/mortalidade , Adulto , Diabetes Mellitus/epidemiologia , Feminino , Mortalidade Hospitalar , Humanos , Pacientes Internados , Modelos Logísticos , Masculino , Desnutrição/epidemiologia , Pessoa de Meia-Idade , Análise Multivariada , Alta do Paciente , Filipinas/epidemiologia , Probabilidade , Estudos Prospectivos , Fatores de Risco
16.
Ann Surg Oncol ; 26(11): 3446-3454, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31240591

RESUMO

BACKGROUND: Controversy in axillary reverse mapping in axillary lymph node dissection (ALND) possibly results from incomplete recognition of the arm lymphatic system (ALS) and its compromise to oncological safety. The iDEntification and Preservation of ARm lymphaTic system (DEPART) technique facilitates complete identification of ALS; therefore, its use may decrease the occurrence of arm lymphedema. This study aimed to examine the arm lymphedema rate, locoregional recurrence, and feasibility to perform DEPART in ALND. METHODS: Patients from February 2013 to October 2017 from two tertiary referral centers were randomly assigned to two groups. In the study group, indocyanine green and methylene blue (MB) were utilized to identify arm sentinel nodes, and 0.1 ml MB was injected into the arm sentinel nodes to reveal the subsequent-echelon nodes and lymphatics. Gross arm lymph nodes were examined by intraoperative partial frozen section and were removed if positive. Arm lymphedema, local recurrence, regional recurrence, and distant metastasis were recorded at different follow-up examinations. RESULTS: Arm sentinel nodes were identified in 573 (83.2%) patients. Subsequent-echelon nodes and lymphatics were visualized in 558 (97.4%) patients. Metastatic arm nodes were identified in 38 (6.8%) patients. The arm lymphedema rate was 3.3% (18/543) in the study group versus 15.3% (99/648) in the control group (p < 0.001) after 37-month median follow-up. Regional recurrence showed no difference between the two groups (1.4% and 1.2%, respectively) (p = 0.392). CONCLUSIONS: DEPART can benefit breast cancer patients who undergo ALND, reducing the arm lymphedema rate without adversely affecting the morbidity of regional recurrence.


Assuntos
Braço/cirurgia , Neoplasias da Mama/cirurgia , Linfonodos/cirurgia , Sistema Linfático , Linfedema/prevenção & controle , Recidiva Local de Neoplasia/cirurgia , Tratamentos com Preservação do Órgão/métodos , Adulto , Idoso , Braço/patologia , Axila , Neoplasias da Mama/patologia , Carcinoma Ductal de Mama/patologia , Carcinoma Ductal de Mama/cirurgia , Carcinoma Lobular/patologia , Carcinoma Lobular/cirurgia , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Excisão de Linfonodo , Linfonodos/patologia , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Prognóstico , Estudos Prospectivos , Procedimentos Cirúrgicos Reconstrutivos
17.
Cardiol Young ; 29(7): 856-861, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31218968

RESUMO

OBJECTIVES: Concerns exist over the long-term consequences of subclavian artery ligation in subclavian flap repair for coarctation of the aorta. We sought to analyse upper limb structural and functional performance in adults who have had surgery in childhood for coarctation of the aorta, using either subclavian flap repair or end to end aortic anastomosis. METHODS: Two-group observational design using anatomical and upper limb functional performance measures. Purposive sampling from our specialist adult congenital heart disease database of patients who received subclavian flap repair or end to end anastomosis for coarctation of the aorta as children. Upper limb measurements were completed using MRI and blood flow velocity with ultrasound imaging. Bilateral standardised upper limb functional testing of assessment of strength, dexterity and a standardised self-report of upper limb disability was completed. RESULTS: Eighteen right-handed patients, 9 with subclavian repair, (38 ± 12 years, 78% males) were studied. Age at repair was 4.7 ± 5.9 years; mean time from initial repair 32 ± 9 years. The subclavian group had a larger difference between right and left when compared the end to end anastomosis group in: lower arm muscle mass (94.5 ± 42.3 mls versus 37.8 ± 94.5 mls, p = 0.008), lower arm maximal cross-sectional area, (5.9 ± 2.8 cm2 versus 2.9 ± 2.6 cm2, p = 0.038) and grip strength (14.7 ± 8.3 lbs versus 5.9 ± 5.3 lbs, p = 0.016) There were no significant functional differences between groups. CONCLUSIONS: In adults with repaired coarctation of the aorta, those with subclavian flap repair had a greater right to left arm muscle mass and grip strength differential when compared to those with end to end anastomosis repair.


Assuntos
Coartação Aórtica/cirurgia , Braço/fisiopatologia , Complicações Pós-Operatórias/epidemiologia , Artéria Subclávia/cirurgia , Retalhos Cirúrgicos/efeitos adversos , Adulto , Anastomose Cirúrgica/efeitos adversos , Braço/irrigação sanguínea , Braço/patologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Força da Mão , Humanos , Lactente , Ligadura , Masculino , Pessoa de Meia-Idade
18.
Ann Surg Oncol ; 26(10): 3250-3259, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31054038

RESUMO

BACKGROUND: Breast cancer-related lymphedema (BCRL) represents a major source of morbidity among breast cancer survivors. Increasing data support early detection of subclinical BCRL followed by early intervention. A randomized controlled trial is being conducted comparing lymphedema progression rates using volume measurements calculated from the circumference using a tape measure (TM) or bioimpedance spectroscopy (BIS). METHODS: Patients were enrolled and randomized to either TM or BIS surveillance. Patients requiring early intervention were prescribed a compression sleeve and gauntlet for 4 weeks and then re-evaluated. The primary endpoint of the trial was the rate of progression to clinical lymphedema requiring complex decongestive physiotherapy (CDP), with progression defined as a TM volume change in the at-risk arm ≥ 10% above the presurgical baseline. This prespecified interim analysis was performed when at least 500 trial participants had ≥ 12 months of follow-up. RESULTS: A total of 508 patients were included in this analysis, with 109 (21.9%) patients triggering prethreshold interventions. Compared with TM, BIS had a lower rate of trigger (15.8% vs. 28.5%, p < 0.001) and longer times to trigger (9.5 vs. 2.8 months, p = 0.002). Twelve triggering patients progressed to CDP (10 in the TM group [14.7%] and 2 in the BIS group [4.9%]), representing a 67% relative reduction and a 9.8% absolute reduction (p = 0.130). CONCLUSIONS: Interim results demonstrated that post-treatment surveillance with BIS reduced the absolute rates of progression of BCRL requiring CDP by approximately 10%, a clinically meaningful improvement. These results support the concept of post-treatment surveillance with BIS to detect subclinical BCRL and initiate early intervention.


Assuntos
Antropometria/instrumentação , Braço/patologia , Linfedema Relacionado a Câncer de Mama/diagnóstico , Linfedema Relacionado a Câncer de Mama/prevenção & controle , Neoplasias da Mama/complicações , Sobreviventes de Câncer/estatística & dados numéricos , Espectroscopia Dielétrica/métodos , Idoso , Linfedema Relacionado a Câncer de Mama/etiologia , Feminino , Seguimentos , Humanos , Pessoa de Meia-Idade , Prognóstico
19.
Medicine (Baltimore) ; 98(13): e15017, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30921223

RESUMO

RATIONALE: Malignant peripheral nerve sheath tumor occurring in the context of neurofibromatosis type I (NF1) is relatively rare. Herein, we report a case of NF1 with malignant peripheral nerve sheath tumor in the upper arm. PATIENT CONCERNS: A 24-year-old man presented with a mass in the medial part of the left upper arm that had been present for more than 20 years. In the previous 1 year prior to admission, the mass had grown significantly. Physical examination showed cafe-au-lait spots of variable sizes throughout the body and multiple masses in the medial part of the left upper arm. Three months later after the resection of the masses, the patient was readmitted to our department due to tumor recurrence. Two months after the extended resection, in situ recurrence of the tumor was noted again. Four months after the operation and the administration of radiotherapy, a mass was found in the outside of the left upper arm. DIAGNOSIS: Immunohistochemical staining showed the masses were positivity for vimentin, CD34, and S100; the tumor cells were negative for PGP9.5, CD57, EMA, and SMA. The Ki-67 labeling index was approximately 40%. A diagnosis of malignant peripheral nerve sheath tumor was made. INTERVENTIONS: Surgical resection was performed for both the primary tumors and the 2 subsequent recurrence tumors. The patient underwent radiotherapy with 60 Gy in 30 fractions after the third operation. Four months after the administration of radiotherapy, the patient underwent tumorectomy of a mass in the outside of the left upper arm. OUTCOMES: During the 4-month follow-up after the fourth operation, the patient's condition was stable. LESSONS: Malignant peripheral nerve sheath tumor in NF1 is an exceedingly rare entity that poses a great diagnostic challenge. High-frequency ultrasound can support the diagnosis.


Assuntos
Neurofibromatose 1/patologia , Neoplasias do Sistema Nervoso Periférico/patologia , Braço/inervação , Braço/patologia , Humanos , Masculino , Adulto Jovem
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