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.

Combined Chemotherapy and Radiotherapy versus Radiotherapy alone in the Management of Localized Angiocentric Lymphoma of the Head and Neck / 대한방사선종양학회지

PURPOSE: To clarify the clinical benefit derived from the combined modality therapy (CMT) consisting of chemotherapy (CT) and involved field radiotherapy (RT) for stage I and II angiocentric lymphomas of the head and neck. MATERIALS AND METHODS: Of 143 patients with angiocentric lymphoma of the head and neck treated at our hospital between 1976 and 1995, 104 patients (RT group) received involved field RT alone with a median dose of 50.4 Gy (range : 20-70 Gy), while 39 patients (CMT group) received a median 3 cycles (range : 1-6 cycles) of CT before involved field RT. The response rate, patterns of failure, complications, and survival data of the RT group were compared with those of the CMT group. RESULTS: Despite a higher response rate, local failure was the most common pattern of failure in patients of both groups. The patterns of failure, including the systemic relapse rate were not influenced by the addition of combination CT. Although both modalities were well tolerated by the majority of patients, aberrant immunologic disorders or medical illnesses, such as a hemophagocytic syndrome, sepsis, intractable hemorrhage, or the evolution of second primary malignancies were more frequently observed in patients of the CMT group. The prognosis of patients in the RT group was relatively poor, with a 5-year overall actuarial survival rate of 38% and disease-free survival rate of 32%, respectively. However, their clinical outcome was not altered by the addition of systemic CT. Achieving complete remission was the most important prognostic factor by univariate and multivariate analyses, but treatment modality was not found to be a prognostic variable influencing survival. Conclusions : Involved field RT alone for angiocentric lymphoma of the head and neck was insufficient to achieve an improved survival rate, but the addition of CT to involved field RT failed to demonstrate any therapeutic advantage over involved field RT alone.

Nasal angiocentric lymphoma with hemophagocytic syndrome

OBJECTIVES: Hemophagocytic syndrome (HS) is a fatal complication of nasal angiocentric lymphoma (AL) and difficult to distinguish from malignant histiocyosis. Epstein-Barr virus (EBV)-associated HS is frequently observed in lymphoma of T-cell lineage and EBV is highly associated with nasal AL. Clinicopathologic features of 10 nasal ALs with HS were reviewed to determine the clinical significance and the pathogenetic association with EBV. METHODS: Ten patients of HS were identified from a retrospective analysis of 42 nasal ALs diagnosed from 1987 to 1996. Immunohistochemical study and in situ hybridization were performed on the paraffin-embedded tumor specimens obtained from 10 patients. Serologic study of EBV-Ab was performed in 3 available patients. RESULTS: Five patients had HS as initial manifestation, 3 at the time of relapse and 2 during the clinical remission of AL. Four patients were treated by combination chemotherapy (CHOP) and others had only supportive care. The median survival of all patients with HS was 4.1 months (range 2 days-36.5 months) and all had fatal outcome regardless of the treatment-modality. All cases were positive for UCHL1 (CD45RO) and EBV by EBER in situ hybridization. The data of serologic tests indicated the active EBV infection. CONCLUSIONS: HS is a fatal complication of nasal AL and has a high association with EBV. Reactivation of EBV may contribute to HS and further investigation of predictive factors and effective treatment of HS should be pursued in the future.

A case of primary pulmonary angiocentric lymphoma manifested as a mass / 결핵

The primary pulmonary lymphomas are uncommon, accounting for 0.5% of primary lung tumor and 0.4% of all malignant lymphomas. The majority of primary pulmonary lymphomas are of B-cell originating from bronchus associated lymphoid tissue(BALT). Angiocentric lymphoma is a rare type of primary pulmonary lymphomas characterized by polymorphic lymphoid infiltrates, which make it even more difficult to differentiate from benign infiltration. The radiographic findings are variable, depending on the stage of evolution of the disease. The prognosis of angiocentric lymphoma is poor, nearly two-thirds of the patients with grade 2 or 3 angiocentic lymphomas were died within a year of diagnosis. We report a case of primary pulmonary angiocentric lymphoma manifested as a mass of right lower lobe.

A Case of Angiocentric Lymphoma Mimicking Behcet's Disease

We report a case of angiocentric lymphoma in a 41-year-old female who complained chiefly of a genital ulcer and a bulging conjunctiva on the right eye. She had a history of a re-current oral ulcer over a 5 year period and a genital ulcer over 3 years. The above manifested clinical findings may suggest Behcet's disease. However, a biopsy from the upper vaginal wall and the inferior turbinate showed that atypical small and large lymphocytes with hyper-chromatic, elongated and convoluted nuclei had aggregated around the thickened blood vessels, which is consistent with angiocentric lymphoma. Immunophenotypically, the atypical cells were positive for pan T-cell markers not B-cells. Serologically, the patient showed a high titer of i?B virus viral capsid antigen(VCA) IgG.

A Case of Angiocentric Lymphoma

Angiocentric lymphoma is a lymphoma like lesion which has been known as a lethal midline granuloma, lymphomatoid granulomatosis and polymorphic reticulosis. It involves predominantly lungs, and other extranodal sites such as upper respiratory tract, skin, kidneys, peripheral nervous system and CNS. The authors experienced a case of angiocentric lymphoma which was misdiagnosed as orbital cellulitis because there were persistent epiphora, erythematous swelling in dacryocyst area and diffuse edema in upper and lower eyelids which occurred after functional endoscopic sinus surgery. This case was diagnosised as angiocentric lymphoma through multiple tissue biopsies. This disease is rare, especially in orbit and eyelid. It leads to the destruction and inflammation of tissue. To avoid misdiagnosis to orbital cellulitis, accurate biopsy is needed with the interchange of clinical informations with pathologist. We report this case with the review of literatures.

Immunohistochemical Study of Upper Respiratory Tract Malignant Lymphomas with Features of Polymorphic Reticulosis

Malignant lymphomas of the upper respiratory tract are relatively common in the Orient including Korea. They have a marked clinicopathologic and immunophenotypic diversity. We retrospectively reviewed 20 cases of malignant lymphomas with features of polymorphic reticulosis in the upper respiratory tract. Histopathologic findings consisted of polymorphism of lymphoid cells in 17 cases(85%), angiocentricity in 14 cases(70%), coagulation necrosis in 12 cases(60%), and epitheliotropism in 8 cases(40%). By Working Formulation, 14 cases(70%) were classified as diffuse large cell type, 4 cases(20%) as diffuse mixed small cleaved and large cell type, one case as immunoblastic type, and one case as anaplastic large cell type. On immunostaining, one showed B-cell phenotype and 19 cases showed T-cell phenotype. The B-cell type was histologically diffuse large cell type. From these results, we concluded that polymorphic reticulosis is a type of peripheral T-cell lymphoma with angiocentricity.