Primary central nervous system T-cell lymphoma in children and adolescents: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To study the clinicopathological characteristics, and further understand primary central nervous system T-cell lymphoma (PCNSTCL) in children and adolescents. Methods: Five cases of PCNSTCL in children and adolescents were collected from December 2016 to December 2021 at the First Affiliated Hospital of Zhengzhou University. The clinicopathological characteristics, immunophenotypic, and molecular pathologic features were analyzed, and relevant literatures reviewed. Results: There were two male and three female patients with a median age of 14 years (range 11 to 18 years). There were two peripheral T-cell lymphomas, not otherwise specified, two anaplastic large cell lymphoma, ALK-positive and one NK/T cell lymphoma. Pathologically, the tumor cells showed a variable histomorphologic spectrum, including small, medium and large cells with diffuse growth pattern and perivascular accentuation. Immunohistochemistry and in situ hybridization showed CD3 expression in four cases, and CD3 was lost in one case. CD5 expression was lost in four cases and retained in one case. ALK and CD30 were expressed in two cases. One tumor expressed CD56 and Epstein-Barr virus-encoded RNA. All cases showed a cytotoxic phenotype with expression of TIA1 and granzyme B. Three cases had a high Ki-67 index (>50%). T-cell receptor (TCR) gene rearrangement was clonal in two cases. Conclusions: PCNSTCL is rare, especially in children and adolescents. The morphology of PCNSTCL is diverse. Immunohistochemistry and TCR gene rearrangement play important roles in the diagnosis.

Clinicopathological features and prognosis of cytotoxic T-cell lymphoma: analysis of 134 cases / 中华病理学杂志

Objective: To investigate the clinicopathological features and prognosis of cytotoxic T-cell lymphoma (CTL). Methods: The clinicopathological data of 134 CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University, the 989 Hospital of PLA Joint Logistics Support force (formerly the 152 Hospital) and the Fourth Hospital of Hebei Medical University from 2008 to 2020 were retrospectively collected. Immunophenotype, Epstein-Barr virus infection status and T cell receptor (TCR) clonality of tumor cells were assessed, and clinicopathological features and prognosis of patients were analyzed. Results: Among the 134 CTL patients, the male to female ratio was 1.7∶1.0, the median age was 49.5 years (range 3-83 years), and 100 cases (74.6%) were under 60 years old. Forty-six point nine percent of the patients (53/113) had B symptoms. Most of the patients presented with systemic superficial lymphadenopathy. According to the Ann Arbor staging system, 36.8% (39/106) of the patients were in stage Ⅰ-Ⅱ, and 63.2% (67/106) in stage Ⅲ-Ⅳ. The rate of extranodal involvement was 51.6% (66/128). Spleen was involved in 24.2% (31/128) of the cases. Morphology showed diffuse growth of abnormal lymphocytes, infiltrating and destroying normal tissue structure. Immunohistochemical staining showed that tumor cells expressed T cell antigens (CD2, CD3, CD5, and CD7), and 72.0% (77/107) of them had decreased or lost expression of one or more antigens. According to the numbers of CD4 and CD8 expression in tumor cells, 70 cases (52.2%) were grouped into CD8+>CD4+group. The expression rates of TIA-1 and granzyme B were 99.2% (119/120) and 79.8% (95/119), respectively. CD20 abnormal expression rate was 27.6% (37/134) and CD56 was negative in all cases. The median Ki-67 proliferative index was 45.0% (range 5%-80%). In situ hybridization of small RNA encoded by Epstein-Barr virus was negative. Clonal TCR gene rearrangement analysis was performed on 49 cases and was positive in all cases. Ninety-one patients were followed up for a median of 36 months (range, 1 to 240 months), and 40 of the 91 patients (44.0%) died. The twenty-three patients were in complete remission (including 13 cases with localized single extranodal mass). The 3-year and 5-year overall survival rates were 53.5% and 49.4%, respectively. Univariate analysis showed that B symptom, spleen involvement, extranodal involvement, clinical stage, CD8+>CD4+phenotype, abnormal expression of CD20 and Ki-67 proliferation index (>60%) were associated with overall survival (P<0.05). The multivariate Cox regression analyses showed that spleen involvement and CD8+>CD4+ phenotype were independent prognostic factors for overall survival in CTL patients. Conclusions: CTL are more commonly found in adult males under 60 years old, often accompanied by B symptom, with a high proportion of extranodal involvement and more CD8 positive phenotypes. Spleen involvement and CD8+>CD4+phenotype are independent predictors of CTL overall survival. Some patients with localized extranodal CTL may have a good prognosis.

Linfoma pericárdico primario a células T/NK: reporte de un caso / Primary pericardial T/NK cell lymphoma: case report / Linfoma pericárdico primário de células T/NK: relato de um caso

Los linfomas cardíacos primarios son un subtipo muy poco frecuente de tumor en los cuales la lesión primaria se encuentra en el corazón. Los tumores suelen ser infiltrantes y se localizan en la aurícula derecha, seguidos del pericardio. Su mortalidad es notablemente alta y el diagnóstico tardío es el principal factor para su mal pronóstico. Describimos el caso de un paciente que presentó shock obstructivo por derrame pericárdico profuso causado por un tipo raro de tumor cardíaco primario, un linfoma pericárdico de células T/NK.

Primary cardiac lymphomas are a rare subtype of lymphomas in which the primary lesion is in the heart. The tumors are usually located in the right atria, followed by the pericardium and are frequently infiltrative. Mortality is remarkably high in this group and the delayed diagnosis is the main factor for its poor prognosis. We describe the case of a patient that presented with obstructive shock due to profuse pericardial effuse caused by a rare kind of primary cardiac tumor, a T/NK cell pericardial lymphoma.

Os linfomas cardíacos primários são um subtipo de tumor muito raro, no qual a lesão primária está no coração. Os tumores geralmente são infiltrativos e localizam-se no átrio direito, seguidos pelo pericárdio. Sua mortalidade é notavelmente alta e o diagnóstico tardio é o principal fator que produz seu mau prognóstico. Descrevemos o caso de um paciente que apresentou choque obstrutivo devido a um derrame pericárdico profuso causado por um tipo raro de tumor cardíaco primário, um linfoma pericárdico de células T/NK.

T-cell lymphoma with rhabdomyolysis: case report and literature review

Rhabdomyolysis refers to the destruction or disintegration of striated muscles. This syndrome is characterized by muscle breakdown and necrosis, resulting in the leakage of intracellular muscle constituents into the circulation and extracellular fluid. We report a rare case of rhabdomyolysis complicating multi-organ failure caused by T-cell lymphoma in a 32-year-old woman. The final diagnosis was rhabdomyolysis caused by peripheral T-cell lymphoma based on bone marrow aspirate and biopsy.

A study of the clinico-histopathological features of erythematous tender nodules predominantly involving the extremities.

Background: Erythematous tender nodules predominantly involving extremities are frequently encountered in dermatology and rheumatology practice. They are diagnosed based on distinct clinical and histopathological features. However, in clinical situations, considerable overlap is observed that poses a diagnostic challenge. We undertook a study on clinico-histological patterns of inflammatory nodules over extremities. Methods: After detailed history and examination, a preliminary clinical diagnosis was made in 43 cases, followed by skin biopsy from representative nodules. Histological diagnosis made was correlated with clinical features. Results: Of 43 cases, a single clinical diagnosis was made in 25 (58.5%) cases while in the remaining cases more than one diagnosis was considered. On correlating with the histopathological diagnosis, concordance was observed in 51% cases while the remaining showed either histological discordance with clinical diagnosis (14% cases) or were kept in the undecided category (35% cases). Conclusion: Considerable clinico-histological overlap was observed in inflammatory nodules over extremities. Histopathology alone was not helpful in differentiating one entity from another at all times since variable histo-pathological patterns were seen.

Subcutaneous Panniculitis-Like T-Cell Lymphoma of the Breast

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of cutaneous lymphoma. There have been a few case reports describing the radiologic imaging findings of SPTCL. We report a case of SPTCL, rarely presented with a breast mass. Here, we review her clinical history and radiologic (mammography and ultrasound) findings.

HTLV 1 associated adult T cell lymphoma/leukemia a clinicopathologic, immunophenotypic tale of three cases from non-endemic region of south India.

Adult T cell lymphoma/leukemia is a peripheral T-cell neoplasm caused by human T-cell lymphotrophic virus-1, affects mostly adults with systemic involvement and poor prognosis. Diagnosis of adult T-Cell leukemia/Lymphoma is challenging. The clinico-pathologic and immuno-phenotypic features of the three cases will be presented.

T-Cell Lymphomas in South America and Europe

Peripheral T-cell lymphomas are a group of rare neoplasms originating from clonal proliferation of mature post-thymic lymphocytes with different entities having specific biological characteristics and clinical features. As natural killer cells are closely related to T-cells, natural killer-cell lymphomas are also part of the group. The current World Health Organization classification recognizes four categories of T/natural killer-cell lymphomas with respect to their presentation: disseminated (leukemic), nodal, extranodal and cutaneous. Geographic variations in the distribution of these diseases are well documented: nodal subtypes are more frequent in Europe and North America, while extranodal forms, including natural killer-cell lymphomas, occur almost exclusively in Asia and South America. On the whole, T-cell lymphomas are more common in Asia than in western countries, usually affect adults, with a higher tendency in men, and, excluding a few subtypes, usually have an aggressive course and poor prognosis. Apart from anaplastic lymphoma kinase-positive anaplastic large cell lymphoma, that have a good outcome, other nodal and extranodal forms have a 5-year overall survival of about 30 percent. According to the principal prognostic indexes, the majority of patients are allocated to the unfavorable subset. In the past, the rarity of these diseases prevented progress in the understanding of their biology and improvements in the efficaciousness of therapy. Recently, international projects devoted to these diseases created networks promoting investigations on T-cell lymphomas. These projects are the basis of forthcoming cooperative, large scale trials to detail biologic characteristics of each sub-entity and to possibly individuate targets for new therapies.

Intraocular Involvement of a Nasal Natural Killer T-Cell Lymphoma: A Case Report

Herein, we report a case of nasal natural killer T-cell lymphoma (NKTL) with intraocular involvement. A 57-year-old woman was referred due to a three-day history of photophobia and diplopia in the left eye. One-month previously, she was diagnosed with nasal NKTL of the right nasal cavity. Ophthalmic examination revealed conjunctival injection and ptosis. The left pupil was fully dilated and non-reactive to light. Ocular motion was restricted on left-upper gaze. Five days later, anterior uveitis developed and persisted despite topical steroid treatment. An orbital magnetic resonance imaging was without specific findings, however, ophthalmoplegia, vitreous opacity, and an iris mass were observed. A diagnostic anterior chamber aspiration was performed. Aqueous humor aspiration revealed 35% morphologically atypical lymphocytes. After an intravitreal triamcinolone injection, radiotherapy and chemotherapy were administered; this resolved the uveitis and iris mass. When refractory uveitis or orbital pseudotumor occurs in patients with nasal NKTL, ocular and orbital involvement of the NKTL should be considered.

Papulosis linfomatoide / Lymphomatoid papulosis

La papulosis linfomatoide (PL) es considerada en la actualidad una forma indolente de linfoma cutáneo CD30+. Su presentación es más frecuente entre la 4º y 5º décadas de la vida, con un discreto predominio en el sexo masculino (1,5/1). Su mecanismo etiopatogénico es complejo y se ha vinculado principalmente con factores genéticos e inmunitarios. Aunque exhibe características clínicas de benignidad, se manifiesta histológicamente con rasgos de malignidad. Las técnicas inmunohistoquímicas resultan de utilidad a los fines diagnósticos y recientemente han permitido la identificación de un nuevo tipo de PL que remeda un linfoma cutáneo primario agresivo a células T epidermotrópico CD8+ (propuesto como PL tipo D). Puede hallarse asociada a otros trastornos linfoproliferativos y a entidades inflamatorias con perfil de citocinas Th2, entre otros. El diagnóstico diferencial con otros linfomas cutáneos, y especialmente con los casos que presentan el antígeno CD30, puede a veces resultar muy dificultoso. Si bien la PL tiene un curso clínico benigno, quienes la padecen tienen un mayor riesgo de desarrollar una segunda neoplasia. Las opciones terapéuticas disponibles son múltiples; sin embargo, ninguna de ellas ha resultado hasta ahora completamente eficaz.

Primary T-cell Lymphoma of the Thyroid Associated with Hashimoto's Thyroiditis, Histologically Mimicking MALT-Lymphoma

Most of thyroid lymphomas are B-lineage, and T-cell lymphomas are rare. Here, we report a case of primary thyroid T-cell lymphoma associated with Hashimoto's thyroiditis. A 48-yr-old woman presented with incidentally found neck mass. Histologically, the resected right lobe of the thyroid was replaced by monomorphic small atypical lymphoid cells with lymphoepithelial lesion-like change, most of which were immunoreactive for CD3, CD8, betaF-1, and TIA-1. Peripheral T-cell lymphoma, unspecified, was finally diagnosed after molecular study for TCR-gamma gene rearrangement. This is the second case of cytotoxic T-cell lymphoma reported in the thyroid gland so far. Unique association between thyroid follicles and neoplastic lymphocytes may be characteristic feature of this type of T-cell lymphoma.

Linfoma subcutâneo de células T paniculite-símile: [relato de caso] / Subcutaneous panniculitis-like T-cell lymphoma: [case report]

Linfoma subcutâneo de células T paniculite-símile foi recentemente reconhecido como entidade clínico-patológica. Paciente do sexo feminino, 17 anos, relatou nodosidades eritêmato-violáceas e depressões nos membros e abdome há três anos e discreta perda ponderal, sem outros sintomas gerais. Adenomegalia, visceromegalias e infiltração da medula óssea estavam ausentes, e a histopatologia da pele mostrou densa infiltração de linfócitos atípicos CD3/CD8 no subcutâneo. A quimioterapia interrompeu o surgimento de novas lesões com remissão das pré-existentes no seguimento de oito meses. Aspectos imunofenotípicos e moleculares são relevantes para elucidação diagnóstica e avaliação do prognóstico.

Subcutaneous panniculitis-like T-cell lymphoma is extremely rare and has recently been recognized as a clinicopathological entity. Young female, 17 years old, has complained of subcutaneous nodules and plaques in the limbs and abdomen for three years, accompanied of mild weight loss without other constitutional symptoms. Nodal, visceral and bone marrow involvement was absent, and subcutaneous CD3/CD8 atypical lymphocyte infiltration was observed in the skin sample. Chemotherapy interrupted the onset of new lesions and led to remission in the 8-month follow-up. Immunophenotypic and molecular aspects were relevant to the diagnosis and as prognosis makers.

Linfoma T primario colónico: reporte de un caso y revisión de la literatura / Primary colonic lymphoma T: report one case and revision of the literature

Los linfomas del tracto gastrointestinal son el tipo más frecuente de linfomas extraganglionares y de estos el 15 por ciento a 20 por ciento son de localización intestinal. Los linfomas colónicos primarios son extremadamente raros, comprenden 0,2 por ciento a 0,6 por ciento de todas las neoplasias malignas de colon. Pueden ser B o T. Los tipo T son menos comunes y pueden estar o no asociados a enteropatía celiaca, enfermedad de Crohn y/o SIDA, siendo más frecuente en hombres adultos en una proporción de 2:1. Son de evolución lenta, clínicamente debutan con dolor abdominal asociado a cuadros obstructivos, seguidos de diarrea, hemorragia, perforación, o masa abdominal. El tratamiento es controvertido, en vista de que solamente el 30 por ciento de los pacientes que van a cirugía tienen la firme presunción de linfoma. Presentamos el caso de un paciente masculino de 62 años, quien consultó por dolor abdominal en hipocondrio izquierdo de 30 días de evolución asociado a anorexia, náuseas, vómitos y melena. Los estudios de extensión gastrointestinales reportaron dentro de lo normal, en la tomografía axial computarizada abdominal se evidenció tumoración en hipocondrio izquierdo que comprimía extrínsecamente al colon descendente; siendo intervenido quirúrgicamente hallándose una gran adherencia entre epiplón mayor, peritoneo, mesocolon, ángulo esplénico, yeyuno y mesenterio, en el cual, se evidenció tumoración de 15 cm de diámetro. Se realizó una colectomía parcial y resección intestinal más anastomosis T-T, con buena evolución posoperatoria.

Lymphomas of the gastrointestinal tract are the most frequent type of primary extranodal lymphomas and of these, the 15 % to 20 % are of intestinal location. The primary colonic lymphomas is extremely rare, they include 0.2 % to 0.6 % of all the colonic malignant neoplasm. They can be B or T. Intestinal T-cell lymphomas are much less common and can be or no associate to enteropathy celiac, Crohn’s disease and/or AIDS. They are more frequent in adult men in a proportion of 2:1. They are of slow evolution, clinically they make debut with abdominal pain associate to obstructive squares, followed by diarrhea, hemorrhages, perforation or abdominal mass. The treatment is controverted, in view of which only 30 % of the patients who go to surgery have the firm presumption of lymphoma. We present the case of a 62 year-old male patient who consulted by abdominal pain in left hypochondriac of 30 days of evolution associated to anorexia, sick feeling, vomits and manes. The gastrointestinal extension studies reported normal; the Computerized Axial Tomography showed a tumor in left hypochondriac that compressed extrinsically the descending colon. The patient was under surgery finding in a great adherence among great omentun, peritoneum, mesocolon, splenic flexure, jejunum and mesentery surgically, a 15 cm diameter tumor in jejune and great omentun. We realized a partial colectomy and small intestine resection with a T-T anastomosis with a well post-operative evolution.

Expresión inmunohistoquímica de metaloproteasas (MMP-1, 2 y 11) e inhibidor de metaloproteasas de tejido-1 (TIMP-1), y expresión de p53 en linfomas angiocéntricos de células T/NK tipo nasal / Metalloproteinase (MMP-1, 2 and 11), tissue inhibitor of metalloproteinase-1 (TIMP-1), and p53 expression in nasal-type angiocentric T/NK-cell lymphoma. An immunohistochemical study

Se analizan 20 casos de linfomas extraganglionares de células T/NK de tipo nasal, estudiados en el Instituto Nacional de Cancerología, México, D. F., para su expresión inmunohistoquímica de las células neoplásicas, expresión nuclear de la proteína supresora de tumor p53, así como de enzimas que participan en invasión, destrucción tisular y metástasis: metaloproteasas. Material y métodos: Se estudió el material quirúrgico de estos casos y se efectuó tinción con hematoxilina y eosina analizando sus características histopatológicas: tamaño celular y detalle citológico. Se realizó estudio de inmunohistoquímica para corroborar el tipo celular, así como CD3 (células T), CD56 (células NK), expresión nuclear de la proteína supresora de tumor p53, y la expresión de metaloproteasas tipo 1, 2, 11 (MMP-1, 2, 11) y un inhibidor de metaloproteasas 1 (TIMP-1). Se analizaron variables demográficas, como edad del paciente, sexo, localización del tumor primario, etapa clínica, tratamiento en general y seguimiento. Estudio estadístico: Se analizó la prueba exacta de Fisher para correlacionar la expresión entre las metaloproteasas y su diferencial entre las células epiteliales, tumorales, estromales, necrosis y células endoteliales. Resultados: Los 20 casos fueron positivos CD3 citoplásmico, CD56, 19 de ellos positivos a p53, cinco de ellos con positividad nuclear mayor al 50% de las células neoplásicas. Hubo una mayor expresión citoplásmica tumoral de MMP-1; mayor expresión citoplásmica en el epitelio de TIMP1 y MMP-11. Los pacientes con sobreexpresión de p53 tuvieron un curso clínico fatal. Tres de ellos recibieron únicamente radioterapia falleciendo dentro del primer mes del tratamiento. Discusión: Los linfomas angiocéntricos de células T/NK tipo nasal son neoplasias frecuentes en los países de Asia, Latinoamérica, incluyendo a México. Frecuentemente esta patología se asocia a VEB con expresión fenotípica de células T/NK, cuyas características histológicas son: atipia celular linfoide, angioinvasión y necrosis, reflejado en los pacientes con destrucción progresiva de los tejidos blandos del macizo facial y curso clínico fatal.

Twenty cases of extraganglionar Nasal type T/NK cell lymphomas were analyzed at the National Cancer Institute of Mexico. We studied immunophenotype of neoplastic cells, nuclear p53 expression, and enzymes as matrix metalloproteinases participating in invasion, tissular destruction and metastases. Material and Methods: Paraffin blocks from all cases were retrieved and analyzed by hematoxilin and eosin. Histopathological features included cellular size and cytologic characteristics. We performed immunohisto chemistry to determine CD3, CD56, p53 cellular type and expression of (MMPs-1, 2,11) matrix metalloproteinases and one tissue inhibitor of TIMP 1 metalloproteinase. Demographic variables included, age, sex, primary location, clinical stage, treatment and follow up. Statistical analysis: The association of different matrix metalloproteinases in epithelial and tumoral cells, stroma, necrosis and endothelial cells were found to be significant using Fisher s exact test. Results: All studied cases were positive to cytoplasmic CD3, CD56 (NK cells), 19 of them were positive to p53, five of them with nuclear overexpression of p53 in more than 50% of neoplastic cells. There was significant expression of MMP-1 in tumoral cells; the epithelium displayed significant expression of TIMP 1 and MMP-11. Patients with p53 overexpression displayed a poorer prognosis. Three of them had undergone radiotherapy and died within the first month of treatment. Discussion: This type of lymphoma is a common neoplasm in Asia, Latin America and Mexico. It is worth noting it has has been linked to Epstein Barr virus with T/NK-cell phenotype, which often displays cellular atypia, an angiocentric growth pattern and necrosis. It is clinically expressed by progressive destruction of midline facial soft tissue and has a poor prognosis.

Primary esophageal T-cell non-Hodgkin's lymphoma.

Primary non-Hodgkin's lymphoma of the esophagus is a rare disease. We report a 52-year-old man who had a polypoid mass in the esophagus at endoscopy. Histology was suggestive of non-Hodgkin's lymphoma; immunohistochemistry was positive for CD3, CD45 RO, LCA. He was treated with 6 cycles of CHOP and is disease-free 14 months later.

Epidemiología y patología de la leucemia/linfoma de células T del adulto en Cali y el suroccidente colombiano / Epidemiology and pathology of the adult T-cell Lymphoma in Cali and Colombian Southwest

Introducción: Este estudio se realizó con el fin de obtener un acercamiento con la epidemiología geográfica de las neoplasias linfoide en Cali y el suroccidente colombiano, con atención especial en la leucemia linfoma de células T del adulto. Materiales y métodos: Se utilizó información del Registro Poblacional de Cáncer de Cali, de las personas con linfoma no Hodgkin y leucemia linfoides entre 1987 y 1996 procedentes de la costa pacífica. Se obtuvo material incluido en parafina de los pacientes con linfoma no Hodgkin del departamento de Patología, del Hospital Universitario del Valle, con el próposito de investigar secuencias provirales para HTLV-I usando técnicas biomoleculares. Además, se obtuvieron muestras de sangre de voluntarios nativos de Tumaco que representan niveles socioecónomicos bajo, medio y alto, y se analizaron para anticuerpos contra HTLV-I/II con una prueba de imunoabsorción ligada con enzimas (ELISA, Abbott®) y confirmados por Western blot. Resultados: La tasa de incidencia global para linfoma no Hodgkin es al menos dos veces mayor para leucemias en ambos sexos y no ha mostrado cambios notables, mientras la leucemia linfoide ha aumentado en ambos sexos, de manera más notoria en mujeres. La tasa de incidencia para linfoma no Hodgkin aumenta con la edad en ambos sexos. Cinco de 75 casos de linfoma fueron positivos para secuencia de HTLV-I por PCR, en los especímenes tisulares y ninguno de los pacientes tenía prueba serológica para HTLV; 18 (5.1/100) de las 356 personas fueron positivas serológicamente para anticuerpos contra el HTLV-I muestreadas en Tumaco.Conclusiones: Varios casos de ATL pueden pasar desapercibidos en pacientes con linfoma no Hodgkin en quienes la asociación no había sido sospechada. La seroprevalencia de HTLV-I en Tumaco es más alta en mujeres. Merece la pena continuar investigando la intercurrencia con infecciones u otros factores medioambientales que facilitarían una progresión rápida de portadores ATL en habitantes del área

A Case of NK/T-Cell Lymphoma Complicated by a Squamous Cell Carcinoma of Hard Palate during Combination Chemotherapy and Radiation Therapy

NK/T-cell lymphoma, which often shows an angiocentric growth pattern, is a distinct clinicopathologic entity highly associated with Epstein-Barr virus. The disease is characterized by a destruction of the upper respiratory tract, particularly the nasal cavity, palate and paranasal sinuses. Interestingly, NK/T-cell lymphoma is closely linked to a variety of complications, such as hemophagocytic syndrome, second primary cancer, sepsis and bleeding. Here we report a case of a 50-year-old man diagnosed initially as NK/T-cell lymphoma of the oropharynx and who developed a second primary carcinoma of the hard palate during combination chemotherapy and radiation therapy.

Linfoma gama-delta: consideraçöes clínicas e métodos diagnósticos correlacionados a um caso clínico / Gama-delta lymphoma: case report with discussion of clinical aspects and diagnostic methods

O Linfoma gama-delta (çÙ), classificado como uma linfoproliferaçäo T periférica ou pós-tímica, foi descrito pela primeira vez nos anos 90. Trata-se de uma entidade rara, identificada pelo rearranjo de dois genes alelos, envolvendo as cadeias gama e delta do receptor de célula T (TCR). Esse grupo distinto de linfoma caracteriza-se pela clínica, imunofenotipagem, citigenética e histopatologia peculiares. Clinicamente, cursa com volumosa esplenomegalia, hepatomegalia moderada e ausência de linfonodomegalias. A histopatologia dos orgäos acometidos é marcada pela distensäo de sinusóides secundária à infiltraçäo por linfócitos de imunofenótipo T, expressando CD2, CD7, CD3, CD43 e CD45RO. A trissomia do cromossomo 8 (+8) e o aparecemimento de um isocromossomo 7i (7q) säo alteraçöes cariotípicas mais frequentes - em até 50 por cento dos pacientes. Apenas as técnicas de biologia molecular, como a reaçäo da polimerase em cadeia (PCR) e o southerm blot, säo eficientes para o reconhecimento dos rearranjos clonais destes linfócitos T. Este tipo de linfoma manifesta-se em adultos jovens, entre os 20 e 40 anos, predominando no sexo masculino e na raça branca. Até o momento, pouco é conhecido sobre seu comportamento biológico, mas se observa uma doença agressiva e refratária aos tratamentos quimioterápicos convencionais usados nos linfomas T em geral

A Case of Primary Intestinal T-Cell Lymphoma Involving Entire Gastrointestinal Tract: Esophagus to Rectum

Primary intestinal T-cell lymphoma is a rare disease entity, which is approximately 10% to 25% of intestinal lymphomas, and most of the lymphomas occur in the small intestine. We report here a case of a 56-year-old woman who has been suffering from chronic diarrhea and weight loss for 6 months. Abdominal CT scan and small bowel series showed diffuse wall thickening of the small bowel. Gastroscopic examination showed diffuse erythematous lesions on the esophagus and small gastric ulcerations on the antrum of the stomach, and colonoscopic examination also showed multiple punched-out ulcerations and erosions on the entire colon, including the sigmoid colon to the terminal ileum. Diffuse infiltration of CD 3 positive lymphoma cells was found on biopsy. The patient was diagnosed as primary intestinal T-cell lymphoma with diffuse involvement of the entire gastrointestinal tracts from the esophagus to the rectum. Although the patient received systemic combination chemotherapy and achieved partial response initially, the lymphoma relapsed repeatedly.

Extranasal T/NK-cell lymphoma presenting as intestinal diverticulum

A case of intestinal angiocentric T/NK-cell lymphoma in a 58-year-old man is reported. The patient presented initially with panperitonitis because of perforation of sigmoid colon diverticulum. He underwent segmentectomy of involved bowel. Histologically, the intestinal wall showed diffuse infiltration of medium or large size lymphoma cells with angiocentric growth and necrosis. The lymphoma cells were CD56+, CD45RO+, CD3+, CD4-, CD8-, CD20-, and CD30- in paraffin sections with germline configuration of TCR-gamma gene, consistent with T/NK-cell lymphoma. Further staging revealed splenomegaly. Intestinal angiocentric T/NK cell lymphoma represents a distinct etiology of diverticulum with perforation.