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Calcium pyrophosphate deposition disease is not an uncommon cause of polyarthritis, especially in the elderly. This disease typically affects the appendicular skeleton but may rarely affect the axial skeleton as well. When the axial skeleton is involved, it can lead to numerous neurological signs and can be disabling. We describe a case in which a 68-year-old male presented with on-and-off myelopathy and was thought to have chronic inflammatory demyelinating polyneuropathy. Magnetic resonance imaging of the spine suggested an inflammatory or infectious lesion at the thoracic level. However, after a surgical biopsy, pathologists concluded that calcium pyrophosphate deposition, or pseudogout, was the cause of this patient's neurological symptoms. Pseudogout in the spine, especially the thoracic spine, is exceptionally rare. There are very few additional cases reported. In this report, we review the current literature on existing similar cases, radiological findings, risk factors, and treatments for this condition in hopes of increasing knowledge and awareness of this rare differential.
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The pathogenesis of intralobar pulmonary sequestration is not completely understood, either representing a congenital or acquired process. The presence of fungal organisms and aspiration has both clinical and etiologic significance.
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Low-grade fibromyxoid sarcoma, also known as Evans tumor, is a low-grade sarcoma that most commonly arises in the deep soft tissue of the proximal extremities or trunk in young adults. It is very rare in the viscera as a primary site, with only a few cases reported in the literature. Here, we present a case of Evans tumor occurring in an unusual and rarely reported location; an intrathoracic mass arising from the diaphragmatic pleura.
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CASE: Extraskeletal myxoid chondrosarcomas (EMCs) are rare soft-tissue malignancies. Intra-articular occurrence is even more rare. To our knowledge, this case is one of only 2 reported intra-articular EMC cases of the knee free of local recurrence and/or amputation at follow-up. This case is also distinctive for being fluorescence in-situ hybridization-negative for the typical EMC-balanced translocation t(9;22) which fuses EWSR1 with NR4A3, harboring instead a variant translocation resulting in fusion of NR4A3 with a less common gene fusion partner. CONCLUSION: This is a unique case of intra-articular EMC of the knee with a rare molecular fingerprint and an unusually positive outcome.
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Condrosarcoma/genética , Proteínas de Unión al ADN/genética , Neoplasias de los Tejidos Conjuntivo y Blando/genética , Proteína EWS de Unión a ARN/genética , Receptores de Esteroides/genética , Receptores de Hormona Tiroidea/genética , Condrosarcoma/diagnóstico por imagen , Condrosarcoma/patología , Condrosarcoma/cirugía , Humanos , Rodilla/patología , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Neoplasias de los Tejidos Conjuntivo y Blando/diagnóstico por imagen , Neoplasias de los Tejidos Conjuntivo y Blando/patología , Neoplasias de los Tejidos Conjuntivo y Blando/cirugía , Colgajos QuirúrgicosRESUMEN
Initial extranodal osseous presentation of Classic Hodgkin lymphoma (CHL) is extremely rare. The present study describes a case of CHL in an 11-year-old male presenting with lower back and right hip pain. Radiologic imaging showed a solitary lytic lesion involving the L5 vertebrae and sacrum. Pathologic examination led to a diagnosis of extranodal osseous CHL and further workup proved bone marrow and nodal disease. Diagnosis of extranodal osseous CHL is challenging, especially in this age group and location. When confronted with a solitary lytic bone lesion in a young patient, more common benign and neoplastic entities enter the differential diagnosis and include osteomyelitis, Langerhans cell histiocytosis, epithelioid hemangioma of bone, leukemia, and anaplastic large cell lymphoma (ALCL) among others, with extranodal osseous CHL being one of the less common entities in the differential diagnosis. This case report raises the awareness of extranodal osseous CHL as one of the entities to consider when confronted with a solitary lytic bone lesion in a young patient.
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Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor most commonly seen in young adults and children. We report a case with morphology and immunohistochemistry supporting the diagnosis of synovial sarcoma. On core biopsy, the tumor had spindle cell and epithelioid morphology with a myxoid background. Staining for transducin-like enhancer of split 1 and CD99 were positive; however, subsequent fluorescence in situ hybridization for SYT (SS18, nBAF chromatin remodeling complex subunit) break apart returned negative. Further study showed fluorescence in situ hybridization for EWSR1 (EWS RNA binding protein 1) gene rearrangement, supporting the diagnosis of AFH. The resected specimen showed a predominant spindle cell proliferation along with histiocytoid areas supporting a diagnosis of AFH. This case report highlights the fact that synovial sarcoma and AFH can overlap morphologically and immunohistochemically. When approaching a biopsy specimen with spindle cell morphology, and transducin-like enhancer of split 1, CD99, and epithelial membrane antigen positivity it is important to include AFH in the differential diagnosis.
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Biomarcadores de Tumor/metabolismo , Proteínas Co-Represoras/metabolismo , Hemangioma/diagnóstico , Histiocitoma Fibroso Maligno/diagnóstico , Inmunoquímica/métodos , Sarcoma Sinovial/diagnóstico , Antígeno 12E7/metabolismo , Adolescente , Diagnóstico Diferencial , Regulación Neoplásica de la Expresión Génica , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , MasculinoRESUMEN
BACKGROUND: Steatosis, as associated with chronic hepatitis C (CHC) and non-alcoholic fatty liver disease (NAFLD), has been considered a risk factor for development of fibrosis. AIMS: Our aims were to determine if correlations existed between the degree of steatosis and fibrosis in treatment-naïve CHC patients, and to compare the accuracy of digital image analysis with semiquantification (manual assessment) to quantify hepatic steatosis. METHODS: We studied 220 treatment-naïve, liver biopsy-proven CHC patients, including a serial biopsy sub-cohort of 37 patients with a mean interval of 3.82 years. Steatosis and fibrosis % were evaluated using digital quantification of steatosis (DQS) and fibrosis contrasted with manual assessment. RESULTS: Most patients had <6% steatosis measured manually and digitally. Overall, manual assessment of steatosis was 3.78 times greater than DQS. Increasing steatosis % was associated with advancing fibrosis stage, both manually and digitally. Intraobserver reliability for DQS showed higher intraclass correlation reproducibility (r = 0.98, P < 0.001) than the manual method (r = 0.81, P < 0.01). Interobserver concordance for DQS had an average measure intraclass correlation of r = 0.99. Cirrhotics were more likely than non-cirrhotics to have grade 2 steatosis. CONCLUSIONS: Increased steatosis was associated with increased fibrosis. DQS was consistently more precise and reproducible than manual assessment of steatosis in grades 1 (1 to <6%) and 2 (6 to <34%), and may prove to be especially preferable in clinical trials of pharmacotherapeutic agents.
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Diagnóstico por Imagen/métodos , Hígado Graso/patología , Hepatitis C Crónica/patología , Procesamiento de Imagen Asistido por Computador , Cirrosis Hepática/patología , Anciano , Antropometría , Biopsia con Aguja , Estudios de Cohortes , Intervalos de Confianza , Hígado Graso/diagnóstico , Femenino , Hepatitis C Crónica/diagnóstico , Hepatitis C Crónica/terapia , Humanos , Inmunohistoquímica , Cirrosis Hepática/diagnóstico , Masculino , Persona de Mediana Edad , Variaciones Dependientes del Observador , Reproducibilidad de los Resultados , Estudios Retrospectivos , Medición de Riesgo , Sensibilidad y Especificidad , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: Patients with acute respiratory distress syndrome (ARDS) are often ventilated with high airway pressure. Brief loss of airway pressure may lead to an extended loss of oxygenation. While using high frequency oscillatory ventilation (HFOV) in a porcine acute lung injury model, two animals became disconnected from the ventilator with subsequent loss of airway pressure. We compared the two disconnected animals to the two animals that remained connected to determine causes for the extended reduction in oxygenation. METHODS: ARDS was induced using 5% Tween. Thirty min of nonprotective ventilation (NPV) followed before placing the pigs on HFOV. Measurements were made at baseline, after lung injury, and every 30min during the 6-h study. Disconnections were treated by hand-ventilation and a recruitment maneuver before being placed back on HFOV. The lungs were histologically analyzed and wet/dry weights were measured to determine lung edema. RESULTS: Hemodynamics and lung function were similar in all pigs at baseline, after injury, and following NPV. The animals that remained connected to the oscillator showed a continued improvement in PaO(2)/FiO(2) (P/F) ratio throughout the study. The animals that experienced the disconnection had a significant loss of lung function that never recovered. The disconnect animals had more diffuse alveolar disease on histologic analysis. CONCLUSIONS: A significant fall in lung function results following disconnection from HFOV, which remains depressed for a substantial period of time despite efforts to reopen the lung. Dispersion of edema fluid is a possible mechanism for the protracted loss of lung function.
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Respiración Artificial/efectos adversos , Respiración Artificial/métodos , Síndrome de Dificultad Respiratoria/terapia , Anestesia General , Animales , Análisis de los Gases de la Sangre , Presión Sanguínea , Modelos Animales de Enfermedad , Diuresis , Frecuencia Cardíaca , Hemodinámica , Humanos , Lesión Pulmonar/fisiopatología , Modelos Animales , Tamaño de los Órganos , Arteria Pulmonar/fisiología , Arteria Pulmonar/fisiopatología , Pruebas de Función Respiratoria , PorcinosRESUMEN
The purpose of this study is to investigate the relationship between mutation status, copy number, and protein expression of epidermal growth factor receptor (EGFR) in lung adenocarcinoma, and to correlate the genetic status and clinicopathologic features. Forty-nine adenocarcinomas were tested by polymerase chain reaction (PCR) for EGFR gene mutations, by fluorescence in situ hybridization (FISH) for increased EGFR gene copy number, and by immunohistochemistry (IHC) for EGFR protein expression. Terminal respiratory unit (TRU) morphology, tumor grade, mitotic count, and pleural and lymphovascular invasion were also examined. Five of 49 tumors (10%) had EGFR mutation by PCR. Eighteen (36%) had high EGFR gene copy number by FISH, all of which were polysomies without gene amplification. Fifteen (31%) had EGFR protein overexpression by IHC, 13 (87%) of which were also positive by FISH. Of the PCR-positive cases, 4/5 (80%) were positive by FISH and 3/5 (60%) were positive by IHC. TRU morphology was observed in all PCR-positive cases and in about 50% of FISH-positive and IHC-positive cases. Pleural invasion was significantly more common in IHC and FISH-positive cases, and lymphovascular invasion was more often present in FISH-positive cases. No statistically significant differences in mitotic count, age, sex, smoking status, degree of differentiation, or stage were seen. Our findings suggest that a staged approach, with FISH testing as the first step, followed by PCR for the FISH-negative cases would be most effective in the identification of patients with EGFR gene alterations.
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Adenocarcinoma/patología , Receptores ErbB/biosíntesis , Receptores ErbB/genética , Inmunohistoquímica/métodos , Hibridación Fluorescente in Situ/métodos , Neoplasias Pulmonares/patología , Reacción en Cadena de la Polimerasa/métodos , Adenocarcinoma/diagnóstico , Adenocarcinoma/genética , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Dosificación de Gen , Expresión Génica , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/genética , Masculino , Persona de Mediana Edad , MutaciónRESUMEN
Because diffuse alveolar damage, bronchiolitis obliterans-organizing pneumonia, and usual interstitial pneumonia are all related to acute lung injury, we postulated that the proliferative activity of fibroblasts and epithelium would be similar in all 3, and that of fibroblasts would be similar to skin scars. Ki-67 staining was assessed in 16 usual interstitial pneumonia, 9 bronchiolitis obliterans-organizing pneumonia, and 8 diffuse alveolar damage cases, 5 incidental fibroblast foci, 5 skin scars, and 5 keloids. The proliferative activity of alveolar macrophages was also measured and compared with that of 10 respiratory bronchiolitis cases. The greatest proliferative activity was found in fibroblasts and epithelium of diffuse alveolar damage (25.8% and 41.9%), and it was significantly greater (P = .000) than in usual interstitial pneumonia (1.88% and 2.6%), bronchiolitis obliterans-organizing pneumonia (4.07% and 1.55%), and incidental fibroblast foci (2.9% and 0.44%). The proliferative activity in fibroblasts of diffuse alveolar damage was significantly higher than that of fibroblasts in skin scars (P = .024). In contrast, the proliferative rate of fibroblasts in bronchiolitis obliterans-organizing pneumonia, usual interstitial pneumonia, and incidental fibroblast foci was significantly lower than that in skin scars (P = .000, P = .000, and P = .001) but similar to keloids (P = 1.000). Usual interstitial pneumonia macrophages showed an unexpectedly high proliferative rate (19.5%) that was significantly greater than that in bronchiolitis obliterans-organizing pneumonia (5.5%, P = .000), diffuse alveolar damage (9.01%, P = .007), incidental fibroblast foci (9.5%, P = .036), and respiratory bronchiolitis (11.45%, P = .031). Our results suggest different reactions to acute injury in usual interstitial pneumonia and bronchiolitis obliterans-organizing pneumonia compared with diffuse alveolar damage. The similar low proliferative activity of fibroblasts in usual interstitial pneumonia and keloids supports the hypothesis of abnormal wound healing in usual interstitial pneumonia. The high proliferative activity of macrophages in usual interstitial pneumonia suggests a role in the pathogenesis of usual interstitial pneumonia.
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Neumonía en Organización Criptogénica/metabolismo , Antígeno Ki-67/metabolismo , Enfermedades Pulmonares Intersticiales/metabolismo , Alveolos Pulmonares/metabolismo , Fibrosis Pulmonar/metabolismo , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Biomarcadores/metabolismo , Recuento de Células , Proliferación Celular , Niño , Preescolar , Neumonía en Organización Criptogénica/patología , Femenino , Fibroblastos/metabolismo , Fibroblastos/patología , Humanos , Queloide/metabolismo , Queloide/patología , Enfermedades Pulmonares Intersticiales/patología , Masculino , Persona de Mediana Edad , Alveolos Pulmonares/patología , Fibrosis Pulmonar/patología , Adulto JovenRESUMEN
Although pulmonary meningothelial-like nodules (MLNs) have been recognized for decades, their nature and significance remain uncertain. This study was undertaken to evaluate MLNs in a wide range of specimens to clarify their incidence, distribution, relation to age and underlying disease, and histogenesis. Five hundred surgical lung biopsies, 25 extensively sampled lobectomies, 20 resections for pneumothoraces in persons younger than 30 years, and 92 pediatric autopsies were examined. Immunohistochemistry was performed in selected cases. One hundred eighty-six MLNs were identified in 81 cases, including 69 of 500 (13.8%) surgical biopsies and 12 of 25 (48%) lobectomies. No MLNs were found in pneumothorax resections or pediatric autopsies. Patients ranged from 22 to 84 years (mean, 62), with only 4 younger than 40 years. There were 56 women and 25 men (female:male=2.2:1). The highest incidence of MLNs was in thromboembolic disease/infarcts (5/12; 42%) and respiratory bronchiolitis-associated interstitial lung disease/desquamative interstitial pneumonia (9/35; 26%). MLNs were randomly distributed in alveolar septa with no consistent relation to small blood vessels. Immunohistochemistry demonstrated positivity for CD56 (18/18) in addition to progesterone receptor (18/18), epithelial membrane antigen (11/11), and vimentin (2/2). The high incidence of MLNs in our study may be related to underlying chronic lung disease. The finding of MLNs in almost half of extensively sampled lobectomies suggests that they may be present in all abnormal lungs if sufficiently sampled. Their absence in infants and children indicates that they are not congenital rests. The positive staining for CD56 is novel, and as CD56 has been reported in meningiomas, this finding supports meningothelial differentiation.
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Neoplasias Pulmonares/patología , Paraganglioma Extraadrenal/patología , Adolescente , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Biomarcadores de Tumor/análisis , Biopsia , Antígeno CD56/análisis , Niño , Preescolar , Femenino , Humanos , Inmunohistoquímica , Lactante , Recién Nacido , Neoplasias Pulmonares/química , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Paraganglioma Extraadrenal/química , Paraganglioma Extraadrenal/cirugía , Adulto JovenRESUMEN
Differentiating malignant melanoma from benign melanocytic lesions can be challenging. We undertook this study to evaluate the use of the immunohistochemical mitosis marker phospho-Histone H3 (pHH3) and the proliferation markers Ki-67 and survivin in separating malignant melanoma from benign nevi. Sixty-six melanocytic lesions (18 malignant melanomas, 8 Spitz nevi, 20 dysplastic nevi, and 20 compound nevi) were stained with antibodies to pHH3, Ki-67, and survivin. No pHH3 expression was detected in the dermis of compound and dysplastic nevi. Rare mitoses were observed in the superficial dermis in 3 of 8 Spitz nevi (37%). Staining for pHH3 was higher in malignant melanomas [average 25 per 10 high-power field (HPF), range 2-75 per 10 HPF] than in Spitz nevi (average 0.5 per 10 HPF, range 0-2 per 10 HPF) and was heterogeneously distributed in the malignant melanomas compared with a superficial dermal location in Spitz nevi. There was no cytoplasmic staining for survivin in any of the 66 melanocytic lesions and no nuclear staining in any of the benign ones. Survivin nuclear staining was present in 12 of 18 cases of malignant melanoma (67%) with an average index of 7% (range 0%-15%). In benign melanocytic lesions, the Ki-67 index was less than 5% (range 0%-4%) and staining was present close to the dermo-epidermal junction compared with an average index of 27% in melanomas (range 5%-50%) and a generally heterogeneous pattern of staining throughout the dermis. pHH3 and Ki-67 can be useful adjuncts to histopathology to separate malignant melanoma from benign nevi. pHH3 is especially useful to highlight mitoses and to rapidly assess the mitotic activity in melanocytic lesions.
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Biomarcadores de Tumor/análisis , Histonas/análisis , Antígeno Ki-67/análisis , Melanoma/química , Proteínas Asociadas a Microtúbulos/análisis , Proteínas de Neoplasias/análisis , Nevo de Células Epitelioides y Fusiformes/química , Nevo Pigmentado/química , Neoplasias Cutáneas/química , Adolescente , Adulto , Anciano , Biopsia con Aguja , Distribución de Chi-Cuadrado , Preescolar , Estudios de Cohortes , Diagnóstico Diferencial , Femenino , Humanos , Inmunohistoquímica , Lactante , Proteínas Inhibidoras de la Apoptosis , Masculino , Melanoma/patología , Persona de Mediana Edad , Nevo de Células Epitelioides y Fusiformes/patología , Nevo Pigmentado/patología , Probabilidad , Pronóstico , Estudios Retrospectivos , Sensibilidad y Especificidad , Neoplasias Cutáneas/patología , SurvivinRESUMEN
Enteric cysts result from an anomaly of embryonic foregut development. In adults, these are frequently asymptomatic. The following is a case of an enteric cyst identified during routine coronary artery bypass attached directly to the epicardium of the left ventricle, a finding not previously reported. Approaches to the treatment of this rare lesion vary. Due to the patient's history of breast cancer, the lesion was excised for histopathological diagnosis.
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Quiste Esofágico/patología , Ventrículos Cardíacos/patología , Quiste Mediastínico/patología , Anciano , Quiste Esofágico/cirugía , Femenino , Humanos , Quiste Mediastínico/cirugíaAsunto(s)
Neoplasias Meníngeas/secundario , Neoplasias Ováricas/patología , Antígeno Ca-125/sangre , Femenino , Humanos , Neoplasias Meníngeas/inmunología , Neoplasias Meníngeas/terapia , Persona de Mediana Edad , Estadificación de Neoplasias , Neoplasias Ováricas/inmunología , Neoplasias Ováricas/terapia , Derrame Pleural Maligno/etiologíaRESUMEN
Inflammation of ileal reservoir mucosa ("pouchitis") is a common sequelae in ulcerative colitis (UC) patients who have had a colectomy with ileal pouch anal-anastomosis (IPAA). Although several clinical, genetic, and laboratory parameters have been evaluated, reliable pathologic predictors for the development of pouchitis are lacking. The purpose of this case-control study was to determine whether there are any pathologic features in UC colectomy specimens that may help predict the subsequent development of pouchitis after an IPAA procedure. The study group consisted of 39 UC patients (male/female ratio: 21/18, mean age: 35 years), who had at least 1 episode of pouchitis after an IPAA procedure during the follow-up period (mean: 57 months, range: 12-121 months). There were 26 control patients (male/female ratio: 11/15, mean age: 37 years), all of whom also underwent a total colectomy and IPAA procedure for UC, but did not develop pouchitis during the follow-up period (mean: 78 months, range: 14-223 months). Routinely processed tissues from each colectomy specimen were evaluated for a variety of histologic features, such as extent of colitis, severity of colitis, extent of severe colitis, type and extent of ulceration, presence and severity of appendiceal inflammation, and the presence of active ileitis, and compared between the study and control patients. Pathologic features that were associated with the subsequent development of pouchitis included the presence of severe colitis that extended into the cecum (severe pancolitis), which was present in 7/39 (18%) pouchitis patients, but in none (0%) of the control patients (P = 0.03), early fissuring ulcers [9/39 (23%) pouchitis cases versus 1/26 (4%) controls (P = 0.04)], active inflammation of the appendix [20/32 (63%) pouchitis patients versus 7/19 (31%) controls (P = 0.03)], and appendiceal ulceration [13/32 (41%) pouchitis patients versus none (0%) of the controls (P = 0.002)]. No significant differences in patient gender or age, depth or extent of ulceration, or the presence or absence of "backwash ileitis" were identified between the 2 groups. In conclusion, there are several histologic features in colectomy specimens from UC patients who have undergone an IPAA procedure that may help predict the subsequent development of pouchitis. Of these features, appendiceal ulceration is highly associated with pouchitis.