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
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
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
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.