Linfomas cutáneos: serie de casos en México / A case series of cutaneous lymphomas in Mexico

Resumen Introducción: Los linfomas cutáneos primarios son un grupo heterogéneo de neoplasias de células T y B que se presentan en la piel, sin ninguna evidencia de enfermedad extracutánea en el momento del diagnóstico, y muestran diferencias considerables en histologia, fenotipo y pronóstico. Se consideran neoplasias poco frecuentes. Casos clínicos: Se presentan cinco casos de linfomas cutáneos diagnosticados en el Hospital Infantil de México Federico Gómez durante el periodo de 2010 a 2018. Las presentaciones clínicas más frecuentes en estos pacientes fueron dermatitis, costras hemáticas y úlceras necróticas. El inmunofenotipo más común fue el linfoma cutáneo no Hodgkin T/NK extranodal nasal primario. El esquema de tratamiento que se utilizó en la mayoría de los pacientes fue SMILE. El promedio de tiempo al diagnóstico fue de 7 meses. Conclusiones: El pronóstico depende del estadio de la enfermedad al diagnóstico, grado de afectación de la piel y presencia o ausencia de enfermedad extracutánea. Los linfomas cutáneos primarios son neoplasias poco frecuentes. Debido al diagnóstico tardío, el estadio de la enfermedad suele ser avanzado, por lo que, generalmente, el comportamiento es agresivo.

Abstract Background: Primary cutaneous lymphomas are a rare heterogeneous group of T and B cell skin neoplasms without any evidence of extracutaneous disease at the time of diagnosis, which show considerable differences in histology, phenotype and prognosis. Case reports: Five cases of cutaneous lymphomas treated at the Hospital Infantil de México Federico Gómez from 2010 to 2018 are described. The most frequent clinical presentations in these patients were dermatitis, blood scabs, and necrotic ulcers. The most common immunophenotype was non-Hodgkin T/NK primary nasal extranodal cutaneous lymphomas. The treatment scheme used in most patients was SMILE. The average time to diagnosis was 7 months. Conclusions: The prognosis depends on the stage of the disease at diagnosis, the degree of skin involvement, and the presence of extracutaneous disease. As primary cutaneous lymphomas are infrequent neoplasms, the stage of the disease is usually advanced and generally shows an aggressive behavior due to a late diagnosis.

Linfoma de células T/NK extranasal primario en íleon / Natural Killer/T cell primary extranasal ileum lymphoma

RESUMEN El linfoma extranodal de células T/NK extranasal (NKTL) primario del tracto gastrointestinal es poco frecuente y tiene carácter agresivo. Presentamos el caso de un paciente inmunocompetente de 51 años que ingresa por dolor abdominal de dos meses de evolución. En la colonoscopía, se hallaron ulceraciones ileales, por lo que se le realizaron estudios para descartar enfermedad de Crohn y tuberculosis intestinal, posteriormente presentó obstrucción intestinal. En la laparotomía exploratoria, se encontró un conglomerado ganglionar en mesenterio. La anatomía patológica confirmó el diagnóstico de NKTL primario en íleon. Se inició quimioterapia y el paciente pidió alta voluntaria dada su precaria condición. Dos meses después del alta el paciente fallece. Este linfoma ha sido reportado principalmente en Asia y posiblemente sea el primer caso reportado en Perú. Tiene un pronóstico funesto con una supervivencia global de ocho meses. Por ello, es necesario un diagnóstico precoz e iniciar la terapia oportunamente.

ABSTRACT Primary extranodal Natural Killer / T cell lymphoma (NKTL) on gastrointestinal tract is an uncommon and aggressive neoplasm. We present the case of a 51-year-old immunocompetent patient with a 2-month history of abdominal pain. Colonoscopy findings showed ileal ulcerations, so studies were carried out to rule out Crohn's disease and intestinal tuberculosis. Later, he developed intestinal obstruction. Exploratory laparotomy found a nodal conglomerate in the mesentery. Anatomical pathology confirmed the diagnosis of primary NKTL on the ileum. Chemotherapy was initiated but the patient asked for voluntary discharge because of his precarious condition. Two months after discharge the patient died. This lymphoma has been reported mainly in Asia and is possibly the first case in Peru. It has a dismal prognosis with overall survival of 8 months. Therefore, it is necessary to get an early diagnosis and begin therapy in a timely manner.

Linfoma Extranodal de Células T/NK en Cavidad Oral. Reporte de un Caso en Chile / Extranodal Lymphoma of T / NK Cells in Oral Cavity. Case Report in Chile

RESUMEN: El linfoma extranodal de células T/NK es una neoplasia maligna agresiva que se caracteriza por una destrucción de estructuras de la línea media de la cara como paladar y fosa nasal. Presentamos el caso de un paciente de sexo masculino, 48 años de edad, consumidor de cocaína, que consulta en la Facultad de Odontología de la Universidad de Chile en Septiembre del 2015 por síntomas de disfagia, rinorrea y que presenta al examen clínico un tumor ulcerado que compromete paladar duro y blando, de un mes de evolución. Se confirma diagnóstico de linfoma de células T/NK con una batería de pruebas inmunohistoquímicas. Esta patología, aunque infrecuente, siempre debe ser considerada dentro los diagnósticos diferenciales en tumores ulcerados en esta localización.

ABSTRACT: Extranodal T / NK cell lymphoma is an aggressive malignant neoplasm characterized by destruction of midline structures of the face such as the palate and nasal fossa. We present the case of a male patient, 48 years old, cocaine user, who consults at the Faculty of Dentistry of the Universidad de Chile in September of 2015 due to symptoms of dysphagia, rhinorrhea and presenting to the clinical examination an ulcerated tumor which compromises hard and soft palate, a month of evolution. Diagnosis of T / NK cell lymphoma is confirmed with a battery of immunohistochemical tests. This pathology, although infrequent, should always be considered within the differential diagnoses in ulcerated tumors of this location.

Nasal-type extranodal T-cell/NK lymphoma in association with hemophagocytic syndrome

Abstract: Extranodal NK/T-cell lymphoma nasal type is a rare disease that mainly affects the nasal cavity and paranasal sinuses of males in the fifth decade of life. It has aggressive and locally destructive behaviour, and can be complicated by the hemophagocytic syndrome, conferring high lethality to the disease. This article describes a case of NK/T-cell lymphoma nasal type in a previously healthy patient, exemplifying its rapid and fulminant course.

Linfoma NK/T extra nodal, tipo nasal, con compromiso cutáneo primario: reporte de un caso / Extranodal Natural Killer/T-Cell Lymphoma, nasal-type with primary cutaneous involvement: a case report

INTRODUCCIÓN: El linfoma extranodal natural killer/célula T (NK/T) de tipo nasal, es una neoplasia poco frecuente, con una alta letalidad, caracterizada por destrucción ósea alrededor de los senos paranasales, el septum nasal u obstrucción de la vía aérea. Puede presentar compromiso primario de la piel, vía aérea y otros órganos. OBJETIVO: Presentar un caso ilustrativo de una afección poco frecuente y de curso agresivo en población pediátrica, para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los especialistas. CASO CLÍNICO: Adolescente de 14 años, que consultó por lesiones solevantadas en brazos y piernas, no dolorosas, sugerentes de paniculitis subcutánea, las cuales evolucionaron a máculas violáceas ulceradas. La biopsia de las lesiones fue compatible con linfoma NK/T de tipo nasal. Fue derivada a oncología pediátrica, donde recibió tratamiento quimioterápico. Pese a los esfuerzos médicos, la paciente falleció a los 8 meses producto de una infección pulmonar grave secundaria a inmunosupresión. CONCLUSIONES: El linfoma extranodal NK/T, tipo nasal es una neoplasia poco frecuente, que se comporta de forma agresiva, con una alta mortalidad sin tratamiento. Por lo que su reconocimiento es de gran relevancia para el diagnóstico precoz y rápida derivación a Hemato-Oncología.

INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. OBJECTIVE: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.

Extranodal natural killer/T-cell lymphoma of the tenosynovium of the hand

No abstract available.

Linfoma extranodal NK de celulas T tipo nasal: reporte de caso / Extranodal NK/T cell lymphoma, nasal type: report of case

El linfoma extranodal de c‚lulas T/NK tipo nasal es una entidad rara, cuya etiolog¡a est  asociada al virus Epstein-Barr, su comportamiento agresivo (20% de sobrevida a 5 a¤os) y es predominante en hombres. Se manifiesta por una lesi¢n tumoral mediofacial destructiva de vasos y necrosis prominente. El diagn¢stico es cl¡nico e inmunohistopatol¢gico y se caracteriza por una marcada agiocentricidad y necrosis tisular, acompa¤ada de infiltrado inflamatorio de linfocitos, histiocitos, c‚lulas plasm ticas y a veces eosfin¢filos. Su inmunofenotipo caracter¡stic es CD2(+), CD56(+), CD3 epsilon (+), CD3 superficie (-), CD7(-), CD16(-), CD30 +/-. A pesar de ser radiosensible, tiene un mal pron¢stico, generalmente, como consecuencia de complicaciones del tratamiento. Se reporta un caso representativo de linfoma extraodal de c‚lulas T/NK tipo nasal de una paciente mujer de 58 anos procedente de Cusco.

Nasal T-cell and natural killer (NK) cell extranodal lymphoma is a rare entity whose etiology is associated with Epstein-Barr, has an aggressive behavior (20% 5-year survival), predominantly in men. Expressed by a midface destructive lesion tumoral of vessels and prominent necrosis. diagnosis is clinical and immunohistochemical is characterized by a marked angiocentricity, and tissular necrosis accompanied by inflamatory infiltrate of lymphocytes, histiocytes, plasma cells and sometimes eosinophils. Its characteristic inmunophenotype is CD2(+), CD56(+), CD3 epsilon (+), CD3 superficie (-), CD7(-), CD16(-), CD30 +/-. Despite being radiosensitive, generally has a poor prognosis due to treatment complications. We report a representative case of nasal T-cell and natural killer cell extranodal lymphoma of a female patient of 58 years from Cusco.

Clinical and otorhinolaryngological aspects of extranodal NK/T cell lymphoma, nasal type / Aspectos clínicos e otorrinolaringológicos do linfoma extranodal de células NK/T tipo nasal,

INTRODUCTION: Extranodal NK/T-Cell lymphoma, nasal type (NKTLN) is a disease that mainly affects the nasal cavity and the paranasal sinuses. Early nasal symptoms are nonspecific, simulating sinus infection. With disease progression, necrosis of the nasal mucosa increases, hindering histological diagnosis. Thus, multiple biopsies may be necessary until definitive diagnosis. Most studies on NKTLN address the hematological and immunological aspects of the disease. OBJECTIVES: To present data from a Brazilian quaternary hospital, with emphasis on the clinical aspects of the disease, and to correlate the findings with the most recent literature data. METHODS: Case study of seven patient files. RESULTS: Patients were evaluated on their medical history, number of biopsies necessary, association with Epstein-Barr virus, treatment, and outcome. All patients had nonspecific nasal complaints and underwent at least three cycles of antibiotic therapy. The earlier a biopsy was performed, the fewer biopsies were required to diagnose the disease and start treatment. However, this fact did not translate into better prognosis. CONCLUSION: The otolaryngologist plays a fundamental role in the prognosis of NKTLN and can shorten time between symptom onset and treatment of the patient. .

INTRODUÇÃO: O linfoma extranodal de células NK/T tipo nasal (LNKTN) é uma doença que acomete preferencialmente a cavidade nasal e os seios paranasais. Os sintomas nasais iniciais são inespecíficos, mimetizando um quadro de infecção nasossinusal. Com a progressão da doença, aumenta a necrose da mucosa nasossinusal, dificultando o diagnóstico histológico e podendo ser necessárias múltiplas biópsias até o diagnóstico definitivo. A maioria dos estudos sobre o LNKTN aborda aspectos imunológicos e hematológicos da doença. OBJETIVO: Apresentar a casuística de um hospital quaternário brasileiro, destacando os aspectos clínicos dos pacientes e correlacionando aos achados mais recentes da literatura. MÉTODO: Estudo de casos de sete pacientes. RESULTADOS: Pacientes foram avaliados quanto a aspectos de sua história clínica, número de biópsias necessárias, associação ao EBV, tratamento e evolução. Todos iniciaram o quadro com queixas inespecíficas nasais e foram submetidos a pelo menos três ciclos de antibióticos. Quanto mais precocemente a biópsia foi realizada, menos biópsias foram necessárias para se obter o diagnóstico e iniciar o tratamento. Entretanto, esta situação não refletiu num melhor prognóstico dos pacientes. CONCLUSÃO: O otorrinolaringologista tem papel fundamental no prognóstico do LNKTN, podendo encurtar o tempo entre o início dos sintomas e o tratamento do paciente. .

Extranodal Nasal NK/T-Cell Lymphoma: A Rare Oral Presentation and FASN, CD44 and GLUT-1 Expression

Extranodal natural killer (NK)/T-cell lymphoma is an aggressive malignant tumor with distinctive clinicopathological features, characterized by vascular invasion and destruction, prominent necrosis, cytotoxic lymphocyte phenotype and a strong association with Epstein-Barr virus. Here is reported an extranodal nasal NK/T-cell lymphoma case, involving the maxillary sinus, floor of the orbit, and interestingly extending to the oral cavity through the alveolar bone and buccal mucosa, preserving the palate, leading to a primary misdiagnosis of aggressive periodontal disease. Moreover, this work investigated for the first time the immunohistochemical expression of fatty acid synthase (FASN) and glucose transporter 1 (GLUT-1) proteins in this neoplasia. FASN showed strong cytoplasmatic expression in the neoplastic cells, whereas GLUT-1 and CD44 were negative. These findings suggest that the expression of FASN and the loss of CD44 might be involved in the pathogenesis of the extranodal nasal NK/T-cell lymphoma, and that GLUT-1 may not participate in the survival adaptation of the tumor cells to the hypoxic environment. Further studies with larger series are required to confirm these initial results.

O linfoma de células natural killers (NK)/T extranodal é um tumor maligno agressivo com características clinicopatológicas distintas, caracterizadas por invasão e destruição vasculares, necrose proeminente, fenótipo linfocítico citotóxico e uma forte associação com o vírus Epstein-Barr. Relatamos aqui um caso de linfoma de células NK/T nasal extranodal, envolvendo o seio maxilar, assoalho de órbita, e interessantemente estendendo-se para a cavidade oral através do osso alveolar e mucosa vestibular, preservando o palato, levando a um diagnóstico inicial equivocado de doença periodontal agressiva. Ainda, nós investigamos pela primeira vez a expressão imunoistoquímica das proteínas Fatty acid sinthase (FASN) e glucose transporter 1 (GLUT-1) nesta neoplasia. FASN revelou uma forte expressão citoplasmática nas células neoplásicas, enquanto GLUT-1 e CD44 foram negativas. Estes achados sugerem que a expressão de FASN e a perda de CD44 podem estar envolvidas na patogênese do linfoma de células NK/T nasal extranodal, e que GLUT-1 não deve participar da adaptação das células tumorais ao ambiente de hipóxia. Estudos adicionais com séries maiores são necessários para confirmar nossos resultados iniciais.

Les Lymphomes T-NK extraganglionnaires de type nasal : experience du CHU de Nancy. A propos de 9 Cas

Cette etude retrospective et prospective a pour but de decrire les aspects cliniques; biologiques et evolutifs du lymphome T-NK extra-ganglionnaire de type Nasale de stade I et IV chez 9 patients au CHU de Nancy sur une periode de 4 ans (Decembre 2001 a Avril 2005). Chez ces patients ages de plus de 19 ans (19 et 78 ans); le nasosinus etait la localisation la plus frequente (56). Les signes B rencontres chez 6 patients etaient par contre absents chez les 3 autres patients. On note a l'analyse une mediane du taux serique des LDH a 742 UI/l. La recherche du virus d'Epstein Barr par la methode..montre une positivite a Ig G chez 5 patients soit 56 des cas. Le dosage biochimique de la beta2microglobuline montre un taux moyen de .chez 6 patients tandis que 3 patients affichaient un taux normal moyen de. On note a l'immunophenotypage 8 cas l'antigene CD3+; 4 cas de CD7+; 2 cas de CD45 et 3 cas de CD56+. Plusieurs protocoles therapeutiques de la chimiotherapie avec des methodes d'intensification par autogreffe apres irradiation corporelle totale; a la radiotherapie ont ete utilises. Parmi les 9 patients mis sous traitement; 2 etaient en RC; 6 patients etaient consideres en echec therapeutique; un deces lie a la toxicite medicamenteuse a ete constate. La mediane de survie de nos patients etait de 6 mois avec des extremes de 3 a 15 mois. En date de pointe 100 des patients etaient decedes. La recidive est apparue chez 2 patients. Notre etude suggere que la combinaison de la chimiotherapie a haute dose et les methodes d'intensification therapeutique par autogreffe peut etre salvatrice pour ameliorer la survie des patients

Aggressive nasal-type natural killer/-T-cell lymphoma associated with Epstein barr virus presenting as testicular tumor

Testicular lymphoma is a lethal disease with a median survival of approximately 12 to 24 months. Nasal-type natural killer/T-cell lymphoma of the testis is exceptional whether as a primary or secondary tumor. The authors report on the comprehensive histopathologic, immunohistochemical and molecular analysis of a case of primary testicular nasal type NK/T cell lymphoma and review the features of previously reported cases. We report a case of primary nasal-type natural killer/T-cell lymphoma of testis in a 28-year-old male. The histopathological examination of the surgical specimen, showed a large lymphoma cells with angioinvasion expressing CD 3 [cytoplasmic], CD2, CD8, CD43, CD45, CD45Ro, CD56, T-cell intracellular antigen-1, perforine, Mib1 and granxyme. In situ hybridation for Epstein-Barr-virus-encoded mENA was positive. Polymerase chain reaction study of formalin-fixed tissue showed lack of T-cell receptor gene rearrangements. The initial stage was I [EA] of Ann Arbor. This lymphoma was refractory to chemotherapy. The patient developed lymph node metastases in the out iliac and in the subclavicular region two months later. He died of disease after eight months. This study confirms that testicular NK/T-cell lymphoma deserves to be distinguished from the other testicular lymphomas. In fact, this lymphoma tends to occur at young age, to disseminate early, to have an aggressive course, and is strongly associated with EBV

A Case of CD56+ Extranodal NK/T-cell Lymphoma, Nasal Type, Presenting as a Duodenal Ulcer Bleeding / 대한소화기학회지

Extranodal NK/T-cell lymphoma is a recently recognized distinct entity within the World Health Organization classification of lymphoid tumors. It is relatively prevalent in Asian and South American populations. It most commonly occurs in the nasal or paranasal areas and less frequently in the skin, the soft tissue, and the gastrointestinal tract. Among these, extranodal NK/T-cell lymphoma of the gastrointestinal tract has shown an aggressive clinical course. We report a case of CD56+ extranodal NK/T-cell lymphoma presenting as a duodenal ulcer bleeding. A 62-year-old male patient presented with melena and abdominal pain. Endoscopic examination of the upper gastrointestinal tract showed the duodenal ulcer covered by blood clot. Pathologic examination revealed the diffuse infiltration of atypical lymphocytes with an angiocentric growth pattern, which was positive for CD3, CD56, and granzyme. The patient showed rapid deteriorating clinical course and died on day 14 after admission. Thus, we report this case with the review of literatures.

Mediastinal Single Nodal Relapse of a Nasal Nk/T cell Lymphoma

A nasal NK/T cell lymphoma is a very aggressive form of lymphoma. Patterns of relapse after treatment have not been systematically evaluated, and mediastinal nodal relapse at a primary site has never been documented. We describe here a 40-year old man who presented with a nasal obstruction caused by a protruding mass that was identified as a nasal NK/T cell lymphoma. The initial work-up, including chest and abdominopelvic computed tomography (CT) and positron emission tomography (PET), showed no regional or distant metastasis. A CT scan performed following three cycles of chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) showed that the mass had nearly disappeared. Radiation therapy undertaken following chemotherapy was given to the primary site. However, PET performed following radiotherapy revealed a single mediastinal lymphadenopathy, with no evidence of residual tumor in the nasal cavity. A biopsy using video-assisted thoracoscopy (VATS) showed the presence of a recurrent NK/T cell lymphoma with an immunophenotype identical to that of the primary nasal lymphoma. An additional three cycles of CHOP chemotherapy were administered, and the patient remains alive, with no evidence of disease 30 months after the initial relapse. These findings indicate that early detection with PET and prompt surgical excision with the use of VATS can lead to successful treatment of a relapsed nasal NK/T cell lymphoma.