ABSTRACT
Nodal peripheral T-cell lymphoma (PTCL) with a T follicular helper phenotype (PTCL-TFH) is a new type of PTCL. We aimed to define its clinical characteristics and prognosis compared to PTCL not otherwise specified (PTCL-NOS) and angioimmunoblastic T-cell lymphoma (AITL). This retrospective observational study included 175 patients diagnosed with PTCL between 2008 and 2013 in 13 Spanish sites. Patient diagnosis was centrally reviewed, and patients were reclassified according to the World Health Organization (WHO) 2016 criteria: 21 patients as PTCL-NOS, 55 as AITL and 23 as PTCL-TFH. Median follow-up was 56.07 months (95% CI 38.7-73.4). Progression-free survival (PFS) and overall survival (OS) were significantly higher in patients with PTCL-TFH than in those with PTCL-NOS and AITL (PFS, 24.6 months vs. 4.6 and 7.8 months, respectively, p = 0.002; OS, 52.6 months vs. 10.0 and 19.3 months, respectively, p < 0.001). Histological diagnosis maintained an independent influence on both PFS (hazard ratio [HR] 4.1 vs. PTCL-NOS, p = 0.008; HR 2.6 vs. AITL, p = 0.047) and OS (HR 5.7 vs. PTCL-NOS, p = 0.004; HR 2.6 vs. AITL, p = 0.096), regardless of the International Prognostic Index. These results suggest that PTCL-TFH could have more favourable features and prognosis than the other PTCL subtypes, although larger series are needed to corroborate these findings.
Subject(s)
Immunoblastic Lymphadenopathy , Lymphoma, T-Cell, Peripheral , Humans , Immunoblastic Lymphadenopathy/genetics , Prognosis , Phenotype , Retrospective StudiesABSTRACT
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Subject(s)
Humans , Male , Adult , Intestinal Perforation , Intestines/injuries , Celiac Disease , Lymphoma, Large B-Cell, Diffuse/complications , Intestinal Neoplasms , Intestines/diagnostic imaging , Tomography, Spiral Computed/methodsABSTRACT
La neurolinfomatosis es una entidad rara caracterizada por la presencia de linfocitos neoplásicos que infiltran las estructuras del sistema nervioso periférico. Dada su naturaleza y localización puede desarrollar una gran variedad de síntomas, suponiendo un reto diagnóstico. El tratamiento se basa en el uso de metotrexato, aunque, a día de hoy, existen diversos esquemas de quimioterapia para los pacientes con enfermedad sistémica. Presentamos el caso de un varón con neurolinfomatosis a nivel de la cauda equina y agrupamos los casos descritos hasta el momento
Neurolymphomatosis is a rare disorder characterised by infiltration of neoplastic lymphocytes into the peripheral nervous system. A wide variety of symptoms can manifest depending on its nature and location, making its diagnosis a real challenge. Treatment is based on methotrexate, although various chemotherapy regimens are currently available for patients with systemic disease. We present the case of a male patient with neurolymphomatosis of the cauda equina, together with a review of all cases published to date
Subject(s)
Humans , Male , Middle Aged , Polyradiculopathy/diagnosis , Marek Disease/surgery , Biopsy , Peripheral Nervous System/surgery , Lymphoma, Large B-Cell, Diffuse/complications , Lumbar Vertebrae/diagnostic imaging , Lumbar Vertebrae/pathology , Diagnosis, DifferentialSubject(s)
Hodgkin Disease/diagnosis , Thoracic Wall/pathology , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biopsy, Large-Core Needle , Bleomycin/administration & dosage , Dacarbazine/administration & dosage , Doxorubicin/administration & dosage , Female , Hodgkin Disease/drug therapy , Hodgkin Disease/pathology , Humans , Middle Aged , Pleural Effusion, Malignant/etiology , Positron Emission Tomography Computed Tomography , Thoracic Wall/diagnostic imaging , Tomography, X-Ray Computed , Vinblastine/administration & dosageABSTRACT
Neurolymphomatosis is a rare disorder characterised by infiltration of neoplastic lymphocytes into the peripheral nervous system. A wide variety of symptoms can manifest depending on its nature and location, making its diagnosis a real challenge. Treatment is based on methotrexate, although various chemotherapy regimens are currently available for patients with systemic disease. We present the case of a male patient with neurolymphomatosis of the cauda equina, together with a review of all cases published to date.
Subject(s)
Cauda Equina Syndrome/etiology , Lymphoma, Large B-Cell, Diffuse/complications , Neurolymphomatosis/complications , Cauda Equina Syndrome/diagnostic imaging , Cauda Equina Syndrome/pathology , Cauda Equina Syndrome/therapy , Combined Modality Therapy , Dexamethasone/therapeutic use , Fatal Outcome , Humans , Laminectomy , Magnetic Resonance Imaging , Male , Middle Aged , Neuroimaging , RecurrenceSubject(s)
Celiac Disease/complications , Intestinal Perforation/etiology , Jejunal Diseases/etiology , Lymphoma, Large B-Cell, Diffuse/complications , Adult , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Celiac Disease/diet therapy , Colectomy , Combined Modality Therapy , Cyclophosphamide/administration & dosage , Diet, Gluten-Free , Disease Susceptibility , Down Syndrome/complications , Doxorubicin/administration & dosage , Humans , Intestinal Perforation/surgery , Jejunal Diseases/surgery , Jejunal Neoplasms/complications , Jejunal Neoplasms/drug therapy , Jejunal Neoplasms/prevention & control , Jejunal Neoplasms/surgery , Lymphoma, Large B-Cell, Diffuse/drug therapy , Lymphoma, Large B-Cell, Diffuse/prevention & control , Lymphoma, Large B-Cell, Diffuse/surgery , Male , Positron Emission Tomography Computed Tomography , Prednisone/administration & dosage , Rituximab/administration & dosage , Tomography, X-Ray Computed , Vincristine/administration & dosageABSTRACT
Introducción: El Sarcoma histiocítico (SH) es una neoplasiamuy poco frecuente, con mucha controversia respectoa los criterios diagnósticos de esta entidad. Desde que lastécnicas inmunohistoquímicas y citogenéticas presentan unamayor disponibilidad universal, muchos casos que inicialmentese diagnosticaron como sarcoma histiocítico, hansido reclasificados como otras enfermedades. Presentacióndel caso: Describimos el caso de un sarcoma histiocíticoque se presentó como una masa abdominal. En la autopsiatambién se observó afectación tumoral de pulmones, hígado,bazo y múltiples adenopatías. El examen histológicomostró proliferación difusa de células grandes con áreas denecrosis. Las células malignas eran de aspecto histiocitarioy pleomórficas. Inmunohistoquímicamente, las célulastumorales fueron positivas para tinciones contra marcadoreshistiocíticos y negativas para marcadores mieloides, dendríticos,CD30, ALK1, y otros marcadores linfoides. En elestudio ultraestructural las células mostraron extensionescitoplásmicas interdigitantes, pero no gránulos de Birbeck.Conclusiones: El sarcoma histiocítico plantea diagnósticodiferencial con otras neoplasias. El diagnóstico en este caso,se basa en la morfología, técnicas inmunohistoquícas yultraestructurales (AU)
Introduction: Histiocytic sarcoma (HS) is a rare disease.There has been much confusion concerning the diagnosticcriteria for this entity. Since immunohistochemicaland cytogenetic techniques have become more universallyavailable, many cases that were initially diagnosed as histiocyticsarcoma are now being classified as other diseases.Case presentation:We describe a case of HS that began asabdominal mass. At autopsy the tumour also involved lungs,liver, spleen and multiple lymph nodes. Histological examinationshowed proliferation of numerous large histiocyticpleomorphic and malignant cells with areas of necrosis.Immunohistochemically, tumour cells expressed histiocyticmarkers but did not stain with antibodies directed againstmyeloid markers, dendritic markers, CD30, ALK1, or otherlymphoid markers. Ultrastructural examination demostratedinterdigitating cytoplasmic extensions, but not Birbeckgranules. Conclusions: Histiocytic sarcoma raises differentialdiagnosis with other neoplasms. In this case, morphological,immunohistochemical and ultraestructural findingsare necessary for diagnosis (AU)
Subject(s)
Humans , Male , Aged , Histiocytic Sarcoma/diagnosis , Histiocytic Sarcoma/pathology , Abdominal Neoplasms/diagnosis , Abdominal Neoplasms/pathology , Histiocytic Sarcoma/surgery , Diagnosis, Differential , Immunohistochemistry , Autopsy , Abdominal Neoplasms/surgery , Abdomen/anatomy & histologyABSTRACT
OBJECTIVES: To report the 7th case of pararenal angiomyolipoma published in the world literature and to review the international bibliography. METHODS: We report the case of a 46-year-old female with history of renal colic and a complex mass on radiological tests. RESULTS/CONCLUSIONS: Extrarenal retroperitoneal angiomyolipoma is a rare pathology with no more than 7 published cases. The diagnostic difficulty and radiological similarities with liposarcoma make surgery the treatment of choice.