Caracterización de lesiones letales de la línea media facial en pacientes del Hospital Escuela Universitario, Honduras entre 2011-2014 / Characterization of lethal injuries of the facial midline in patients of the University School Hospital, Honduras between 2011-2014

Objetivo: Caracterizar lesiones letales de línea media facial, signos y síntomas frecuentes, género y procedencia de los pacientes, histopatología e inmunohistoquímica en base a registros médicos institucionales de HEU entre 2011 y 2014.La lesión letal de línea media es un síndrome que inicialmente engloba variadas entidades: Linfomas no Hogdkin de células NK y T, Linfomas no Hogdkin de células B, enfermedades autoinmunes como la Granulomatosis con Poliangeítis, muchas causas infecciosas e idiopáticas con destrucción acelerada y catastrófica de la región nasofaríngea, senos paranasales y septum nasal. Síndromes de difícil diagnóstico con enfoques terapéuticos muy distintos. Metodología. Se realizó un estudio descriptivo, transversal, con revisión de todos los registros de biopsias realizados en el departamento de Anatomía Patológica del HEU desde el año 2011 al 2014. Cumplen criterios de inclusión, 34 casos. Resultados: Mayor prevalencia de lesiones en hombres 59 %, dentro del rango de edad de 19 a 59 años, con predomino de la región central de Honduras. Signo más frecuente: masa obstructiva. Diagnóstico más consignado fue Linfoma No Hodgkin sin especificación. Conclusión: Frecuencia de lesiones letales de la línea media es mayor en varones, procedentes en su mayoría de región central, síntoma y signo más frecuentes son masa obstructiva con ulceración y la rinorrea purulenta; la utilización de marcadores de inmunohistoquímica es deficiente para definir los casos inespecíficos de Linfoma No Hodgkin Nasales.

Objective: To characterize lethal facial midline lesions, frequent signs and symptoms, gender and origin of the patients, histopathology and immunohistochemistry based on HEU institutional medical records between 2011 and 2014. Lethal midline injury is a syndrome that initially encompasses a variety of entities: non-Hogdkin lymphomas of NK and T cells, non-Hogdkin B-cell lymphomas, autoimmune diseases such as granulomatosis with polyangiitis, many infectious and idiopathic causes with accelerated and catastrophic destruction of the nasopharyngeal region, paranasal sinuses and nasal septum. Syndromes which are difficult to diagnose with very different therapeutic approaches.Methodology. A descriptive, crosssectional study was carried out with a review of all biopsy registries performed in the Department of Pathological Anatomy of HEU from 2011 to 2014. 34 cases meet the inclusion criteria. Results: There was a higher prevalence in men 59%, within the age range of 19 to 59 years, with predominance of the central region of Honduras. Most frequent sign: obstructive mass. Most diagnosed was Non-Hodgkin's lymphoma without specification. Conclusion: Frequency of lethal midline lesions is greater in males, mostly from the central region. The most frequent symptoms and signs are obstructive mass with ulceration and purulent rhinorrhea; the use of immunohistochemical markers is deficient to define nonspecific cases of Nasal Non-Hodgkin's Lymphoma.

Chronological changes in incidences of polymorphic reticulosis in Korea and Japan.

Lethal midline granuloma (LMG) is a clinical term, which describes a group of diseases histologically comprising Wegener's granulomatosis, polymorphic reticulosis (PR), and malignant lymphoma of the ordinary type (ML). PR is a variant of ML, and is considered to be a type of NK/T cell lymphoma. Our previous study revealed the clustering of patients with PR in East Asia including China, Korea and Japan. However, the frequency rate of PR varied even among these countries, with that of Korea being approximately 5 times higher than that of Japan. In the present study, we examined the incidences of each type of LMG, especially PR, in Korea (Yonsei University) and Japan (59 university hospitals) over time. A total of 102 cases and 655 cases of LMG admitted to Yonsei University, Korea from 1977 to 1996 and 59 university hospitals in Japan between 1965 and 1996, respectively, were examined. The frequency rate of PR per 100,000 outpatients of ears, nose and throat (ENT) clinics in Korea decreased from 40 to 20 between the periods of 1977-1989 and 1990-1996. However, there were no significant changes in Japan during the period studied.

Reticulosis letal de la línea media. Manejo con radiaciones y consideraciones histopatológicas / Lethal midline granuloma. Radiotherapy and pathology

Entre 1973 y 1990 se han tratado 24 pacientes portadores de Reticulosis Letal de la Línea Media (RLLM) mediante el empleo de radiaciones ionizantes en el Departamento de Radioterapia del Instituto Nacional de Enfermedades Neoplásicas en Lima, Perú. Los autores realizan una revisión descriptiva y retrospetiva de dichos casos así como una revisión de la literatura referente a este tipo de neoplasia. Todas las láminas provenientes de las biopsias han sido revisadas para confirmar el diagnóstico de RLLM. Todos los pacientes recibieron tratamiento con radiaciones ionizantes y 12 de ellos algún tipo de quimioterapia durante el tratamiento con radiaciones. La relación según sexo masculino-femenino ha sido de 5:3, la edad promedio de 29:33 años y el rango de edad entre los 6 y 84 años de edad. Los síntomas de presentación más comunes han sido: obstrucción de fosa nasal (45.83 por ciento), aumento de volumen de la fosa nasal (33.33 por ciento), rinorrea (29.16 por ciento) y alza térmica constante mayor de 38.5ºC (29.16 por ciento), principalmente. No se ha podido establecer una dosis de referencia para el tratamiento con radiaciones puesto que las dosis han variado desde 732 cGy hasta 7095 cGy; sí hemos podido observar que todos aquellos que recibieron menos dosis de la planificación fue porque fallecieron durante el tratamiento ya que acudieron con enfermedad muy avanzada y en mal estado general. Asimismo, se ha podido corroborar que si la dosis administrada fue de 50 cGy ó más se obtuvo mejor control local aunque por el número de casos no se ha podido demostrar estadísticamente. Al evaluar la sobrevida se observa que ésta disminuye considerablemente durante el primer año de seguimiento pero a partir del segundo año se mantiene constante (33.33 por ciento); esto en relación al estado en que acudió el paciente a recibir tratamiento. Concluimos que en este tipo de neoplasias el empleo de radiaciones ionizantes juega un rol importante en el control local de la enfermedad pero también es importante la extensión de la misma

Lethal midline granuloma in Okinawa with special emphasis on polymorphic reticulosis.

Lethal midline granuloma (LMG) is a clinical term used to describe a condition which may be manifested histologically as Wegener's granulomatosis (WG), polymorphic reticulosis (PR), and malignant lymphoma (ML). WG is an inflammatory disease, and PR and ML are considered to represent a neoplastic proliferation of lymphoreticular cells. In this report, twenty-two cases of LMG in Okinawa were examined. The frequency of LMG per 100,000 outpatients of the ear, nose and throat clinic in Okinawa was 67, and the higher frequency of PR (27) and ML (34) in Okinawa than in other districts of Japan was characteristic. Polymerase chain reaction, in situ hybridization, and immunohistochemical studies showed that the proliferating cells in PR were CD43+ and simultaneously contained Epstein-Barr viral genome in their nuclei. The higher frequency of PR and ML in Okinawa is discussed in conjunction with a review of pertinent literature: multiple factors including genetic, viral environmental, and socioeconomic factors seem to affect the frequencies of these diseases.

Centrofacial malignant granulomas. Clinicopathologic study of 40 cases and review of the literature.

An important problem in the treatment of centrofacial ulcerations is to establish a precise diagnosis, since similar clinical and microscopic findings can result from many different causes (as in the centrofacial malignant granuloma syndrome [CFMG]). A comprehensive surgical biopsy protocol (known as SNFMI/GMCF), involving microbiology, parasitology, immunology and pathology laboratories, allowed us to evaluate and to treat 40 cases of CFMG, who form the basis of this report. In 13 of them, specific diagnoses were found and curative treatments could be given. In the remaining 27, the optical microscopy pattern met the criteria for CFMG without identifiable origin or the presence of so-called lethal midline granulomas; however, a more precise evaluation with the help of immunofluorescence studies led to the recognition of malignant lymphoma (ulcerative lymphoma of the midface [ULM]). Most of these lymphomas belonged to the T cell lineage; the others were of B lymphoid origin, or, more rarely, of histiocytic origin. Patients with ULM received radiotherapy and chemotherapy with a response rate of 70.3%; however, the toxicity was significant, with frequent occurrence of chemotherapy-induced neutropenia followed by severe infectious facial cellulitis. Six patients were enrolled in a preliminary open trial of treatment with recombinant alpha-2b interferon with little success. Three patients were treated with radiation therapy only, and survived. Thus, CFMG is a syndrome with specific causes and treatments, requiring multiple extensive biopsies to make the correct diagnosis. The recognition of ULM as the cause of the previously called "lethal midline granulomas" leads logically to the use of chemotherapy with growth factors in order to ameliorate its bad prognosis.

Nasal peripheral T-cell lymphoma: a 20-year review of cases treated in Scotland.

The nasal peripheral (post-thymic) T-cell lymphoma is an important cause of the midline granuloma syndrome (MGS), in which ulceration and destruction of the tissues of the nose and paranasal sinuses occurs. We reviewed the histology of 9 cases of the MGS treated with radiotherapy, and, using immunocytochemistry, showed 8 cases to be peripheral T-cell lymphomas (PTCL) and 1 a B-cell lymphoma. All patients received radiotherapy and 2 died shortly after treatment from unrelated causes. Two patients with T-cell lymphoma and the solitary case of B-cell lymphoma achieved long-term disease-free survival. The 4 remaining cases of T-cell lymphoma relapsed locally at a median interval of 3.5 months despite megavoltage irradiation of 45-50 Gy (in 3 cases) and inclusion of uninvolved paranasal sinuses and the nasopharynx in the field (in 2 cases). All patients with local relapse achieved, and remain in, remission after treatment with alkylating agents and prednisolone. The disappointing response of some cases of nasal T-cell lymphoma to radiotherapy has been reported by others, and this may be due partly to the heterogeneity of nasal lymphomas. We are unable to provide clear guidelines for treatment but suggest that a role exists for initial treatment with oral alkylating agents and steroids in newly diagnosed cases.

[Lethal midline granuloma in Senegal. (Apropos of 15 cases)]. / Le granulome médio-facial au Sénégal (A propos de 15 observations).

African authors, other than senegalese, have not paid enough attention to lethal granuloma, a terrible disease. This series is about a series collected between 1975 and 1990. It is particularly high compared to other series in the world. If diagnosis is easy for the clinician, much care must surround the pathological answer. Those diagnosis problems and difficulties of treatment (represented by 11 deaths) are studied.

[Midline granuloma and Wegener's granulomatosis]. / Il "granuloma centro-facciale" e la granulomatosi di Wegener. "Midline granuloma" and Wegener's granulomatosis.

Necrotizing lesions of upper respiratory tract have always been among the most enigmatic diseases of the head and neck region. Nowadays a great deal of nosographic confusion still remains along with numerous doubts concerning diagnostic and therapeutic strategies to be followed in dealing with many diseases often erroneously defined "midline granuloma". In fact, a large variety of diseases appear or may appear as midline destructive lesions in the upper respiratory tract. Each of these, including infections, immune and neoplastic disorders, obviously requires a different therapy. The clinician must have a very good knowledge of the problem in order to make a rational approach to diagnosis and therapy. Wegener's granulomatosis is quite different from "midline granuloma" and must be diagnosed promptly so that an appropriate therapy may be determined as soon as possible. Unlike in the case of "midline granuloma", a prompt therapy is often very effective and gives long periods of remission. In this disease, nevertheless, the etiopathogenesis of both diseases is unknown, precise protocols for diagnosis or treatment do not exist (every case must be considered separately) and prognosis is very poor. On the basis of their personal experience and of an accurate review of Literature, the Authors present a systemic and up-to-date monographic study focusing particular attention on the most recent diagnostic techniques, such as immunohistochemical techniques which utilize monoclonal antibodies, indispensable in the cases of "midline granuloma", and immunofluorescent techniques searching antibodies to cytoplasmic antigens of neutrophil granulocytes, of great value in dealing with Wegener's granulomatosis not only in establishing an initial diagnosis, but also in making prognosis and in controlling the evolution of the disease. The paper also discusses differential diagnosis of midline destructive disorders, highly important for a correct and rational initial approach in diagnosis. Every disease described in the section concerning differential diagnosis must be excluded in order to make an accurate diagnosis of "midline granuloma" in that no typical histopathologic or clinical signs of this particular, destructive disorder. Finally, the authors focus their attention on new etiopathogenetic hypotheses and their therapeutic implications. The most interesting of them are surely those that consider "midline granuloma" the manifestation of a malignant lymphoma as well as those that explain the recent success obtained in treatment of Wegener's granulomatosis using trimethoprim and sulfamethoxazole asserting a possible essential role of infections in the etiology of the disease.

Nation-wide study of lethal mid-line granuloma in Japan: frequencies of wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions.

Lethal mid-line granuloma (LMG) is a clinical term, and it is histologically composed of Wegener's granulomatosis (WG), polymorphic reticulosis (PR), and malignant lymphoma (ML). WG is an inflammatory disease, and PR and ML are considered to represent a neoplastic proliferation of lymphoreticular cells. In the present report, a nation-wide study on LMG in Japan is compared with a study from the UK, to examine differences in frequencies of each disease in Eastern and Western countries. A total of 340 cases of LMG were examined. On the basis of histological and clinical findings, these cases were categorized as 68 WG, 129 PR, 92 ML, 44 chronic inflammation not specified, and 7 other related conditions. The crude frequencies of WG, PR, and ML per 100,000 outpatients of ENT clinics in Japan and England were 4, 8 and 6, respectively and 8, 4 and I, respectively. The predominance of malignant lymphoproliferative diseases to WG in Japan (3.5:1) was in marked contrast to the situation in the UK (1:1.6). A review of the pertinent literature suggests a clustering of malignant lymphoproliferative diseases of the nose among Mongolian ethnic groups.