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Resumen ANTECEDENTES: La afectación primaria del aparato genital femenino por un linfoma no Hodgkin es excepcional, se reporta en 0.2 a 1.1% de los casos. Los órganos afectados con más frecuencia son los ovarios, seguidos del cuello uterino; el endometrio y la vagina son los menos aquejados. Debido a su baja frecuencia aún no se llega a un consenso del tratamiento más adecuado de estos linfomas; hasta ahora, por lo general, se ha individualizado para cada caso en concreto. CASO CLÍNICO: Paciente de 29 años que consultó por sangrado genital anómalo e incapacidad para la inserción de tampones vaginales. En la exploración se encontró una masa pélvica, palpable a través de la vagina, que impresionaba al infiltrar toda la luz vaginal y la parte media e izquierda de la vulva. En los estudios de imagen se objetivó una gran masa pélvica de hasta 10 centímetros que parecía depender del cuello del útero y que se extendía e infiltraba el canal vaginal, la vulva y el tercio inferior de la vejiga. El reporte anatomopatológico de la biopsia fue: infiltración de pared vaginal por un linfoma no Hodgkin B difuso de células grandes. Se le indicaron seis ciclos de quimioterapia con ciclofosfamida, vincristina, adriamicina y prednisona con los que se consiguió la remisión metabólica completa. CONCLUSIÓN: El diagnóstico del linfoma genital primario puede resultar complejo por la posibilidad de simular una neoplasia ginecológica. En casos de enfermedad avanzada, la manifestación clínica más frecuente es el sangrado genital anómalo. El esquema de tratamiento más aceptado en la actualidad es con rituximab-ciclofosfamida, vincristina, adriamicina, prednisona seguido de radioterapia de consolidación.
Abstract BACKGROUND: Primary involvement of the female genital tract by non-Hodgkin's lymphoma is exceptional, reported in 0.2 to 1.1% of cases. The most frequently affected organs are the ovaries, followed by the cervix; the endometrium and vagina are the least affected. Due to their low frequency, there is still no consensus on the most appropriate treatment of these lymphomas; until now, it has generally been individualized for each specific case. CLINICAL CASE: A 29-year-old female patient consulted for abnormal genital bleeding and inability to insert vaginal tampons. On examination a pelvic mass was found, palpable through the vagina, which impressed by infiltrating the entire vaginal lumen and the middle and left side of the vulva. Imaging studies showed a large pelvic mass of up to 10 centimeters that appeared to be dependent on the cervix and that extended and infiltrated the vaginal canal, the vulva and the lower third of the bladder. The anatomopathological report of the biopsy was: infiltration of the vaginal wall by diffuse large cell non-Hodgkin's B lymphoma. She was prescribed six cycles of chemotherapy with cyclophosphamide, vincristine, adriamycin and prednisone with which complete metabolic remission was achieved. CONCLUSION: The diagnosis of primary genital lymphoma can be complex because of the possibility of simulating a gynecologic neoplasm. In cases of advanced disease, the most frequent clinical manifestation is abnormal genital bleeding. The most accepted treatment scheme at present is rituximab-cyclophosphamide, vincristine, adriamycin, prednisone followed by consolidation radiotherapy.
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Background The outcome of patients with primary extranodal diffuse large B-cell lymphoma (PE-DLBCL) varies according to the primary site involved. Primary gastrointestinal, breast, bone, craniofacial, and testicular DLBCL are rare extranodal manifestations of DLBCL. Objective The objective of the study was to describe the clinical course of patients with PE-DLBCL disease in a referral cancer center. Results From 637 patients, 51 (8.77%) were considered as having PE-DLBCL (25 gastrointestinal, 12 craniofacial, 6 breast, 5 bone, and 3 with primary testicular DLBCL). Complete remission was higher in all PE-DLBCL sites (100% in testicular, 92.6% craniofacial, 83.3% breast, 80% bone, and 80% gastrointestinal) compared with 73.3% in nodal DLBCL. Although 2 cases with breast PE-DLBC relapsed, they achieved a complete response with chemotherapy. The overall survival at 5 years was 100%, 80%, 78%, 58%, 58%, and 62% for patients with primary breast, primary bone, gastrointestinal, primary craniofacial, primary testicular, and nodal DLBCL, respectively. Conclusions PE-DLBCLs constitute rare, primary sites of lymphoproliferative disorders in most cases, with localized disease and good prognosis. They require a combined chemoimmunotherapy with radiotherapy in most cases to improve local and systemic disease.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Aged, 80 and over , Lymphoma, Large B-Cell, Diffuse/pathology , Immunotherapy/methods , Lymph Nodes/pathology , Antineoplastic Agents/administration & dosage , Prognosis , Survival Rate , Retrospective Studies , Cohort Studies , Lymphoma, Large B-Cell, Diffuse/therapy , Treatment Outcome , Combined Modality TherapyABSTRACT
Objective To summarize the imaging features of the primary extranodal lymphoma (PENL)and evaluate the diagnos-tic value.Methods The clinical manifestations,imaging findings and pathological types of 50 patients with PENL were analyzed retrospectively.Results Fifty patients of PENL included 4 cases in orbit (8.0%),1 in middle ear and mastoid (2.0%),5 in thyroid (10.0%),2 in breast (4.0%),8 in lung (16.0%),5 in stomach (10.0%),15 in intestine (30.0%),1 in adrenal gland (2.0%),5 in testis (10.0%),2 in prostate (4.0%)and 2 in soft tissue of shoulder and hip (4.0%).In all patients,the nodular type was 40% (20/50),diffuse type was 34% (17/50)and massive type was 26% (13/50).Most lesions were regular (54%,27/50)and had clear margin (94%, 47/50).26 cases (52%)were non-Hodgkin's lymphoma with diffuse large B cell type.Conclusion The imaging findings of PENL are various and nonspecific,which may be easily misdiagnosed.Various imaging methods have different sensitivity for the diagnosis of lymphoma in different sites.
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La afectación primaria del tracto genital femenino de los linfomas no Hodgkin es poco frecuente (2 por ciento de los linfomas primarios extraganglionares). Los órganos más afectados son los ovarios seguidos del cérvix, siendo la localización endometrial y vaginal extremadamente rara. Presentamos el caso de una paciente de 44 años diagnosticada de linfoma primario de vagina, estadio IE A, con remisión completa tras tratamiento combinado quimioterápico y quirúrgico. Es importante tener presente estas raras aunque posibles localizaciones de linfomas para evitar que se puedan confundir con lesiones inflamatorias u otros tipos de tumores que nos lleven a un fracaso terapéutico.
Primary non-Hodgkin lymphomas rarely involve the female genital tract (2 percent of primary extranodal lym-phomas). The ovaries followed by the cervix are the most common affected sites while the involvement of the endometrium and vagina is extremely rare. We report the case of a 44 year old woman with a primary lymphoma of the vagina, stage IE A, with complete remission after combined chemotherapy and surgical treatment. These rare but possible locations must be kept in mind to avoid misdiagnosis with inflammatory lesions or other types of tumors which may lead us to a therapeutic failure.
Subject(s)
Humans , Adult , Female , Lymphoma, Non-Hodgkin/pathology , Lymphoma, Non-Hodgkin/therapy , Vaginal Neoplasms/pathology , Vaginal Neoplasms/therapy , Antineoplastic Agents/therapeutic use , Hysterectomy, Vaginal , Lymphoma, B-Cell , Lymphoma, Non-Hodgkin/diagnosis , Neoplasm Staging , Vaginal Neoplasms/diagnosis , Antineoplastic Combined Chemotherapy Protocols/therapeutic useABSTRACT
BACKGROUND: The addition of rituximab to cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy (R-CHOP) has significantly improved clinical outcomes for patients with diffuse large B-cell lymphoma (DLBCL). However, new predictors of patient response to R-CHOP have not been established. We aimed to evaluate the impact of R-CHOP compared with CHOP in patients with DLBCL and to establish clinical predictors of better outcomes in these patients. METHODS: We retrospectively identified 177 patients diagnosed with CD20-positive DLBCL and treated with CHOP (N=82) or R-CHOP (N=95). The response rate, event-free survival (EFS), and overall survival (OS) rates were compared between the 2 treatment groups. All patients were classified into primary extranodal lymphoma (PENL) or nodal lymphoma (NL) subgroups, and the clinical parameters of each subgroup were analyzed. RESULTS: The overall response rate was higher in R-CHOP group (95% vs. 84%, P=0.07). The 3-year EFS rate was significantly higher in R-CHOP group (71% vs. 52%, P=0.013), but the OS rate was comparable between the 2 groups (79% vs. 69%, P=0.23). A significant survival benefit was seen with R-CHOP compared to CHOP therapy in NL patients (P=0.002 for EFS and 0.04 for OS). Multivariate analyses confirmed that R-CHOP therapy is an independent prognostic factor for EFS (hazard ratio of 0.32 [0.17-0.62], P=0.001) and OS (hazard ratio of 0.4 [0.18-0.87], P=0.02) in NL patients. CONCLUSION: Patients in the PENL group did not benefit from R-CHOP chemotherapy.
Subject(s)
Humans , Antibodies, Monoclonal, Murine-Derived , Antineoplastic Combined Chemotherapy Protocols , B-Lymphocytes , Cyclophosphamide , Disease-Free Survival , Doxorubicin , Lymphoma , Lymphoma, B-Cell , Multivariate Analysis , Prednisolone , Prednisone , Retrospective Studies , VincristineABSTRACT
Objective: To evaluate the incidence, clinical features, diagnosis and treatment of primary ex-tranodal non-Hodgkin' s lymphoma (PE-NHL). Methods: The data of 110 patients diagnosed as PE-NHL be-tween January 2001 and May 2008 were reviewed. Results: These PE-NHL patients counted 60.11% of the 183 malignant lymphoma patients at the same period. The primary sites affected were the gastrointestinal tract 21.82% (24/110), Waldeyer ring 10.91% (12/110), nasal cavity 9.10% (10/110), soft tissue 9.10% (10/110), mediastinum 7.27% (8/110) and other unusual sites 41.82% (46/110). Symptoms and signs of PE-NHL were not specific, and 77.27% of these cases had a swelling organ or lump of the primary organ affected. Ac-cording to the International Prognosis Index (IPI), the percentage of patients in low, intermediate, and high group was 41.11%, 44.44% and 14.44%, respectively. Immunophenotype was assayed in 93 cases. The per-centage of B-cell lymphoma was 69.90% while that of T-cell lymphoma was 30.10%. For those 95 cases treat-ed, the effective rate including complete remission (61.05%) and part remission (16.84%) was 77.89%, the median survival was 30 months, and the 5-year overall survival (OS) was 27%. While, for patients with prima-ry gastrointestinal non-Hodgkin' s lymphomas (PGIL), the complete remission rate, part remission rate and the effective rate was 65.21%, 17.39% and 82.80%, respectively. The median survival was 24 months, and the 5-year overall survival (OS) was 30%. Conclusion: PE-NHL is more common than nodal lymphoma. The symptoms and signs of PE-NHL of different sites are quite different. To improve the curative strategies of PE-NHL, it is important to make an allround understanding of PE-NHL and follow reasonable mode of diagno-sis and therapy.
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Objective:To detect the changes on the ratio of T lymphocyte subgroup and immunoglobulin(Ig) in peripheral blood of patients with Primary extranodal lymphoma(PENL).Methods:The peripheral blood T lymphocyte subgroup ratio and immunoglobulin(Ig) complement C3,C4 content were detected by Flow Cytometry(FCM) and Immunoturbidimetry.Results:The numbers of CD3,CD4 or CD8 was lower(P