ABSTRACT
El Hamartoma Condromesenquimal Nasal (HCMN) es una neoplasia descrita por McDermott et al en 1998 en pacientes pediátricos, con pocos casos reportados en adultos. Este tumor está constituido por tejido condroide o cartilaginoso así como por tejidos mesenquimales. Es una lesión expansiva, localmente destructiva y en algunos casos está descrita la extensión intracraneal. El comportamiento es habitualmente benigno y el tratamiento consiste en la resección quirurgica. Se presenta un caso de un paciente masculino de 8 meses de edad, quien presentaba aumento de volumen en región periorbitaria derecha, con pseudoptosis palpebral, paresia del nervio oculomotor derecho y rinorrea hialina. Mediante la Tomografia Computarizada (TC) craneal y la Resonancia Magnética (RM) cerebral se observó lesión ocupante de espacio naso fronto etmoidal. El estudio histopatológico e inmunohistoquimico determinó la presencia de tumor mesenquimal fibrocartilaginoso osificante: Hamartoma Condromesenquimal. Se revisan las características y el tratamiento de este tumor poco frecuente(AU)
The Chondromesenchymal Nasal Hamartoma (CMNH) is a rare neoplasm described in 1998 by McDermott et al in the pediatric age, with few cases reported in adults. This tumor is composed by chondroid or cartilaginous tissue as well as mesenchymal tissues. It is an expansive lesion, locally destructive and in some cases intracranial extension is described. Its behavior is usually benign and its treatment consists of surgical resection. The authors present an 8-month-old male patient with increase volume in the right periorbital region, palpebral ptosis, paresis of the right oculomotor nerve and hyaline rhinorrhea. Computed Tomography (CT) and Cerebral Magnetic Resonance Imaging (MRI) were perfomed and revealed a nasofrontoethmoidal space occupying lesion; a histopathological and inmunohistochemical study resulted in osseous fibrocartilaginous mesenchymal tumor: chondromesenchymal hamartoma. We review the characteristics and treatment of this rare tumor(AU)
Subject(s)
Humans , Male , Infant , Nose Neoplasms/diagnosis , Nose Neoplasms/physiopathology , Hamartoma/surgery , Hamartoma/pathology , Pediatrics , Magnetic Resonance Spectroscopy , TomographyABSTRACT
BACKGROUND: Contact endoscopy (CE) was initially described as a method used in the analysis of uterine and vocal folds histology. The first nasal cavity CE studies achieved promising results regarding its use for the differentiation between benign and malignant lesions, considering that biopsy might cause some complications, especially bleeding. This study described and compared the findings of CE on inverted papilloma and nasosinusal squamous cell carcinoma (SCC) and tested the effectiveness of this exam as a noninvasive method for in vivo differentiation between these tumors. METHODS: The patients included in this study were divided into group A, patients diagnosed with inverted papilloma, and group B, patients diagnosed with SCC. CE results were compared among themselves. CE images were presented to examiners not experienced with the method. RESULTS: Twenty-two patients were examined, 13 in group A and 9 in group B. The main relevant differences in CE findings between those two groups were corkscrew vessels, presence of mitoses, keratinization and nuclear pleomorphism in carcinoma, and vacuolated cells in papilloma. The examiners were capable of defining the diagnosis of these nasal tumors only based on CE images. CONCLUSION: CE may be a useful noninvasive exam to be used in the in vivo diagnosis of inverted papilloma and nasosinusal SCC, which may enable better preoperative planning.
Subject(s)
Carcinoma, Squamous Cell/diagnosis , Endoscopy/methods , Nasal Cavity/pathology , Nose Neoplasms/diagnosis , Papilloma, Inverted/diagnosis , Aged , Aged, 80 and over , Carcinoma, Squamous Cell/pathology , Carcinoma, Squamous Cell/physiopathology , Carcinoma, Squamous Cell/surgery , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Mitosis , Nasal Cavity/surgery , Nose Neoplasms/pathology , Nose Neoplasms/physiopathology , Nose Neoplasms/surgery , Papilloma, Inverted/pathology , Papilloma, Inverted/physiopathology , Papilloma, Inverted/surgery , Reproducibility of Results , Sensitivity and SpecificityABSTRACT
We describe 8 patients affected with Costello syndrome including an affected sib pair and review the literature on 29 previously reported cases. We emphasize an association with advanced parental age, which is consistent with autosomal dominant inheritance with germline mosaicism. The pathogenesis appears to involve metabolic dysfunction, with growth disturbance, storage disorder appearance, acanthosis nigricans, hypertrophic cardiomyopathy, and occasional abnormalities of glucose metabolism. Although the cause is currently unknown, Costello syndrome is interesting because of a potential genetic-metabolic etiology.
Subject(s)
Dwarfism/pathology , Facies , Intellectual Disability/pathology , Acanthosis Nigricans/pathology , Acanthosis Nigricans/physiopathology , Adolescent , Adult , Age Factors , Carbohydrate Metabolism, Inborn Errors/pathology , Carbohydrate Metabolism, Inborn Errors/physiopathology , Cardiomyopathy, Hypertrophic/pathology , Cardiomyopathy, Hypertrophic/physiopathology , Child , Child, Preschool , Diagnosis, Differential , Dwarfism/diagnosis , Dwarfism/etiology , Dwarfism/genetics , Dwarfism/physiopathology , Female , Genes, Dominant/genetics , Germ-Line Mutation/genetics , Glucose/metabolism , Humans , Intellectual Disability/diagnosis , Intellectual Disability/etiology , Intellectual Disability/genetics , Intellectual Disability/physiopathology , Male , Metabolic Diseases/pathology , Metabolic Diseases/physiopathology , Mosaicism/genetics , Nose Neoplasms/diagnosis , Nose Neoplasms/etiology , Nose Neoplasms/genetics , Nose Neoplasms/pathology , Nose Neoplasms/physiopathology , Papilloma/diagnosis , Papilloma/etiology , Papilloma/genetics , Papilloma/pathology , Papilloma/physiopathology , Parents , Phenotype , SyndromeABSTRACT
El presente artículo expone la experiencia del departamento de Otorrinolaringología del Hospital General Dr. Manuel Gea González en el diagnóstico de las masas nasales desde Enero de 1986 a Diciembre de 1995. El fue de 122 pacientes; los tumores fueron clasificados según su tipo histológico en lesiones benignas, lesiones de malignidad intermedia, lesiones malignas y lesiones tumoroides (pseudotumores). Se comunica la distribución por edad y sexo, así como los signos y síntomas que se presentaron en estos pacientes
Subject(s)
Child , Adolescent , Adult , Middle Aged , Humans , Male , Female , Hypersensitivity/etiology , Nose Neoplasms/physiopathology , Nose/physiopathology , Nasal Obstruction/epidemiology , PapillomaABSTRACT
Este es un informe de la frecuencia por sexo y edad de tumores malignos de nariz y senos paranasales, tomados de los archivos del Departamento de Anatomía Patológica del Hospital de Especialidades "Dr. Bernardo Sepúlveda", Centro Médico Nacional Siglo XXI, registrados en el protocolo de investigaciión 92-766-691 del Comité de Investigación de dicho hospital. Se realizaron 20,937 estudios, de los cuales 356 biopsias fueron de nariz y senos paranasales, siendo un total de 23 casos de tumores malignos de nariz y senos paranasales, encontrándose al carcinoma de células escamosas como la neoplasia más frecuente
Subject(s)
Humans , Male , Female , Paranasal Sinus Neoplasms/physiopathology , Nose/physiopathology , Nose Neoplasms/physiopathology , Magnetic Resonance Spectroscopy/methodsABSTRACT
Three patients seen at the Medicine Department of Del Salvador Hospital between 1986 and 1990 are reported. All had a history of purulent or bloody nasal discharge and recurrent sinusitis, before the appearance of progressive and painful destructive phenomena that affected the rhino faucial region. The diagnosis was made with the histopathological study that disclosed and angiocentric polymorphic infiltrates and perivascular necrosis. The three patients received similar treatment consistent in prednisone 1 mg/kg/day per os, cyclophosphamide 2 mg/kg/day per os and local radiotherapy. The response to therapy was bad and survival was less than three months. It is concluded that although this entity is infrequent, its severity requires and aggressive workup and management by a multidisciplinary team. Notwithstanding the mortality remains to be high.
Subject(s)
Lymphatic Diseases , Nose Neoplasms , Adult , Aged , Humans , Lymphatic Diseases/diagnosis , Lymphatic Diseases/physiopathology , Lymphatic Diseases/therapy , Male , Nose Neoplasms/diagnosis , Nose Neoplasms/physiopathology , Nose Neoplasms/therapyABSTRACT
Se revisaron 34 casos de cancer de fosas nasales y senos paranasales en tres hospitales de La Paz, en cuanto a su epidemiologia, formas histoliticas y en 12 casos, los datos clinicos mas importantes. Llama la atencion el mnumero elevado de melanoma maligno, al parecer, primarios de fosas nasales y la presencia de un liposarcoma en una fosa nasal. Por ultimo, se comenta sobre la elevada incidencia de cancer de fosas nasales en nuestra poblacion, de acuerdo al Registro de Cancer de La Paz, en relacion a la incidencia de otras poblaciones consideradas de alta frecuencia para este grupo de neoplasias.