ABSTRACT
Introducción: El linfoma de células T citotóxico/natural killer extranodal de tipo nasal es poco frecuente, pero con alta tasa de mortalidad. Las manifestaciones clínicas de la enfermedad pueden simular una infección de senos paranasales. Objetivo: Presentar las manifestaciones clínicas de un paciente de 34 años de edad con diagnóstico de linfoma de células T citotóxico/natural killer extranodal de tipo nasal. Caso clínico: Se presenta un paciente masculino de 34 años de edad con rinorrea verdosa fétida recurrente y obstrucción en fosa nasal derecha. En la evaluación inicial sugiere sinusitis crónica, sin embargo, debido al empeoramiento de las manifestaciones clínicas se realiza una tomografía computarizada que muestra lesiones sugestivas de infiltración neoplásica, una biopsia de la lesión confirma el diagnóstico de linfoma de células T/natural killer extranodal de tipo nasal. Conclusiones: Los linfomas de células T citotóxico/natural killer extranodal de tipo nasal son considerados neoplasias poco frecuentes, caracterizadas por el patrón rápidamente progresivo con afectación ósea; en su etapa inicial presenta manifestaciones clínicas similares a una sinusitis. La tomografía computarizada y la histopatología, son indispensables en el diagnóstico de la enfermedad.
Introduction: Nasal-type extranodal natural killer/cytotoxic T-cell lymphoma is rare but has a high mortality rate. The clinical manifestations of the disease can mimic a paranasal sinus infection. Objective: To present the clinical manifestations of a 34-year-old patient diagnosed with nasal-type extranodal natural killer/cytotoxic T-cell lymphoma. Clinical case: A 34-year-old male patient with recurrent greenish fetid rhinorrhea and obstruction in the right nostril is presented. In the initial evaluation, it suggests chronic sinusitis, however, due to the worsening of the clinical manifestations, a computed tomography is performed that shows lesions suggestive of neoplastic infiltration, a biopsy of the lesion confirms the diagnosis of T-cell lymphoma/extranodal natural killer. Conclusions: Nasal-type extranodal natural killer/cytotoxic T-cell lymphomas are considered rare neoplasms characterized by a rapidly progressive pattern with bone involvement; in its initial stage it presents clinical manifestations similar to sinusitis. Computed tomography and histopathology are essential in the diagnosis of the disease.
ABSTRACT
Objective@#To report a case of lethal midline granuloma and discuss the diagnostic and treatment dilemma, and management. @*Methods@#Design: Case Report. Setting: Tertiary Government Hospital. Patient: One.@*Results@#A 15-year-old girl under treatment for pulmonary tuberculosis presented to the Emergency Room for epistaxis and a nasopalatine lesion. She was managed as a case of nasopalatine osteomyelitis for one month and discharged on antibiotics. She returned due to bleeding after being lost to follow up for 3 more months. Hemostasis, debridement and biopsy yielded atypical cells, possibly lymphoma. Immunohistochemistry confirmed the diagnosis of NK-cell lymphoma. Unfortunately, she expired prior to initiation of chemotherapy. @*Conclusion@#Clinicians must have a high index of suspicion for lethal midline granuloma in chronic, non-healing midline lesions. Multiple biopsies confirm the diagnosis, and earlier initiation of treatment may improve prognosis.
Subject(s)
Humans , Female , Granuloma , LymphomaABSTRACT
ABSTRACT Extranodal natural killer (NK)/T-cell lymphoma, nasal type, is a rare entity in otorhinolaryngology. Its management requires skilled physicians in order to suspect this disease and making a proper diagnosis at early stages. This paper reports the case of a 31-year-old male patient, with one month of nasal obstruction, recurrent sinusitis, palatal ulceration and a necrotizing lesion. Histopathology reported lymphoid infiltrate polymorph angiocentric growth pattern and extensive areas of necrosis. Immunohistochemistry confirmed the phenotype for T/NK cells: positive CD3, BCL2, CD4 and CD56. IgG for Epstein-Barr virus was also positive. The initial staging was T4, N1, M0, Eastern Cooperative Oncology Group (ECOG) scale was 1, with intermediate risk, and low International Prognostic Index (IPI); based on this results, the patient was referred to oncology to initiate treatment. After a ten-month follow-up, the patient's condition improved, with complete remission of nasal and palate injuries; no relapse has occurred to date. This case is a clear example of the importance of early diagnostic through multiple biopsies in order to establish a specific treatment to decrease complication rates and improve prognosis.
Subject(s)
Humans , Lymphoma, Extranodal NK-T-Cell , Radiotherapy , Granuloma, Lethal Midline , Antineoplastic ProtocolsABSTRACT
Introducción: El linfoma de células T/Natural Killer (T/NK) extranodal, tipo nasal, es un linfoma extra-ganglionar poco frecuente, con extensión a lo largo de la línea media facial, rápidamente progresivo, catastrófico y de mal pronóstico, por lo que también se le conoce como "granuloma letal de la línea media". Objetivo: El propósito de este artículo es revisar la literatura disponible y actualizada sobre el linfoma de células T/NK extranodal, tipo nasal: manifestaciones clínicas, estándares de enfoque, diagnóstico, pronóstico y tratamiento. Diseño: Revisión Narrativa de la literatura. Metodología: Revisión de la literatura mediante búsqueda selectiva por términos MeSH: Extranodal Natural Killer /T cell lymphoma, nasal type, lethal midline granuloma, de las bases de datos: MEDLINE, Current Contents, Cochrane, Pubmed y Scielo, entre los años 2000 y 2014. Resultados: Se revisaron 36 artículos según los requerimientos de los objetivos. El linfoma de células T/NK es una neoplasia rápidamente progresiva, destructiva y de mal pronóstico excepto en los casos donde el diagnóstico ha sido oportuno. Conclusiones: El diagnóstico y tratamiento tempranos del linfoma de células T/NK extranodal, tipo nasal, son las únicas herramientas para mejorar el mal pronóstico y gran afectación en la calidad de vida de los pacientes con esta enfermedad.
"Introduction: Extranodal nasal Natural Killer (NK) /T-cell lymphoma, nasal type, is an extranodal lymphoma, infrequent, with extension along facial midline, rapidly progressive, catastrophic and with poor prognosis, also known as "lethal midline granuloma". Objectives: The purpose of this article is to review the current literature available about Extranodal nasal NK/T-cell lymphoma, nasal type: Clinical manifestations, approach standards, diagnosis, prognosis and treatment. Methodology: Literature review by selective MeSH search terms: Extranodal Natural Killer / T cell lymphoma, nasal type, lethal midline granuloma of databases: MEDLINE, Current Contents, Cochrane, PubMed and SciELO, between 2000 and 2014. Results: 36 articles were reviewed according to the requirements of the objectives. Extranodal NK/T cell lymphoma, nasal type, is a rapidly progressive neoplasia, destructive and with poor prognosis except in cases where diagnosis has been timely. Conclusions: Early diagnosis and treatment of Extranodal NK/T cell lymphoma, nasal type, are the only tools to improve the poor prognosis and high impairment in quality of life of patients with this disease.
Subject(s)
Lymphoma, Extranodal NK-T-Cell , Otorhinolaryngologic Neoplasms , GranulomaABSTRACT
A rara síndrome do granuloma letal da linha média apresenta difícil diagnóstico, em razão da grande variedade de doenças que podem causá-la e um desconhecimento pela maioria da classe médica. No presente artigo relatamos caso de paciente com esta doença, provocada por carcinoma epidermoide, chamando a atenção para os diagnósticos diferenciais e aspectos clínico-radiológicos que podem auxiliar no diagnóstico.
The rare lethal midline granuloma syndrome is difficult to diagnose because of the wide array of related diseases and lack of knowledge by the majority of physicians. In the present report, the authors describe the case of a patient with this disease, caused by squamous cell carcinoma, drawing attention to differential diagnoses and to clinical and radiological findings that may be useful to define the diagnosis.
Subject(s)
Humans , Male , Female , Adult , Carcinoma, Squamous Cell/diagnosis , Granuloma, Lethal Midline , Head and Neck Neoplasms , Lymphoma, Non-Hodgkin , Magnetic Resonance Spectroscopy , Skull , Tomography, X-Ray ComputedABSTRACT
El linfoma nasal de células T asesinas naturales, también conocido como granuloma letal de la línea media, es una entidad poco frecuente. Se trata de un linfoma extraganglionar de comportamiento agresivo que, usualmente, se origina en la cavidad nasal, prevalente en países orientales y en hombres en la tercera década de la vida. Su asociación con infección por el virus de Epstein-Barr actualmente está bien documentada. Su tratamiento es aún controvertido; la tendencia actual está a favor de la radioterapia. A continuación se presenta un caso de linfoma nasal de células T asesinas naturales y la revisión de la literatura, con el fin de proveer un mayor entendimiento de la enfermedad...
The nasal natural killer T-cell lymphoma, also known as lethal midline granuloma, is an uncommon entity. It consists of an extranodal lymphoma of aggressive behavior that generally arises from de nasal cavity. Ii is prevalent in eastern countries and in males in the third decade. Epstein-Barr virus infection in association with this lymphoma is well documented. Current practice is in favor of radiotherapy. We present a case report, and a review of the literature of nasal natural killer T-cell lymphoma was done as an initiative to provide a better understanding of this pathology...
Subject(s)
Granuloma , LymphomaABSTRACT
Lethal midline granuloma (Midline granuloma, Midline reticulosis, Polymorphic reticulosis, and Angiocentric immunoproliferative lesion) is relatively a rare entity of unknown etiology characterized by a massive destruction, erosion and mutilation of the tissues of nose and upper respiratory passages. Unlike Wagner’s granulomatosis which is known for its multi organ involvement, lethal midline granuloma remains usually localized. Without therapeutic intervention it has a very high mortality. Radiotherapy is the treatment of choice which considerably improves both quality and quantity of life. Addition of chemotherapy gives additional benefit. Sporadic cases have appeared in literature from time to time under various synonyms.
ABSTRACT
Idiopathic midline destructive disease(IMDD) is a subgroup of midline granuloma syndrome, which is a clinical entity characterized by a relentless uilcer ation of the upper air way involving the nose, the palate, and the face, with a fatal outcorne. A 32 year-old male had symptoms of nasal obstruct.ion and rhinorrhea for about 4 months prior to visiting the hospital ad was referred to our Department for evaluation of a verely destruct.ive skin lesions on his face. The patient, revealed unique clinopatholcgic featurs compat with IMDD. These were : 1) prescence of locally destructive lesion which were restricted to the upper respiratory tract. 2) abscence of systemic disease durin the follow up peroid, 3) The histologic features consisted of acute and chronic inflarnmation with a variable dearel of necrosis on the examination of repeated biopsy specimens, 4) inabiliy to demonst rate an infectious causative organisms on repeated laboratory tests.
Subject(s)
Adult , Humans , Male , Biopsy , Follow-Up Studies , Granuloma , Necrosis , Nose , Palate , Respiratory System , SkinABSTRACT
This study reviewed 65 cases of polymorphic reticulosis (PR) with respect to clinical and histopathologic bases, and immunohistochemical studies were done using MT1 and UCHL as T-cell markers, MB2 as a B-cell marker and alpha-1-antichymotrypsin as a histiocytic marker. The results obtained were as follows: 1. The male to female ratio was 2.4:1 and the mean age of patients was 44.5 years. The sites involved primarily were the nasal cavity, tonsil and pharynx and about one-fourth of the total cases showed extensive involvement of two anatomical sites at initial presentation. 2. Almost all cases showed characteristic histologic features similar to those of peripheral T-cell lymphoma and showed positive reaction to the T-cell marker. The above immunohistochemical findings suggest strongly that quite a significant portion of PR is in fact T-cell lymphoma.