ABSTRACT
The case of 70-year-old man with mantle cell lymphoma (MCL) carrying t(11;14) translocation that relapsed as nodal lymphoma combining MCL and classic Hodgkin lymphoma (cHL) 9 years after autologous peripheral blood stem cell transplant (auto-PBSCT) is reported. Lymph nodes contained two separate areas of MCL and cHL-like components. Hodgkin and Reed-Sternberg (HRS)-like cells were accompanied by a prominent histiocyte background. HRS-like cells were CD5- , CD15+ , CD20- , CD30+ , PAX5+ , Bob.1- , Oct2- and EBER+ . The MCL component expressed cyclin D1 and SOX11, whereas cyclin D1 and SOX11 expressions were reduced and lost, respectively, in HRS-like cells. Polymerase chain reaction results showed a single clonal rearrangement of the IGH gene in MCL and cHL-like components. CCND1 break apart fluorescence in situ hybridization showed split signals in both MCL and HRS-like cells, suggesting that MCL and cHL-like components were clonally related. Acquisition of p53 expression and Epstein-Barr virus (EBV)-positivity was seen in HRS-like cells. The patient died of disease progression with elevated hepatobiliary enzymes. The autopsy showed both MCL and cHL-like components around the bile ducts, splenic white pulp and bone marrow. The two components were phenotypically distinct, but genetically related, suggesting that transformation of MCL to HRS-like cells during the course of MCL in association with EBV infection.
Subject(s)
Lymphoma, Mantle-Cell , Aged , Autografts/abnormalities , Biomarkers, Tumor/analysis , Cyclin D1/analysis , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human/isolation & purification , Hodgkin Disease/pathology , Humans , In Situ Hybridization, Fluorescence , Lymph Nodes/pathology , Lymphoma, B-Cell/diagnosis , Lymphoma, B-Cell/pathology , Lymphoma, Mantle-Cell/diagnosis , Lymphoma, Mantle-Cell/pathology , Male , Reed-Sternberg Cells/cytology , Tumor Suppressor Protein p53/analysisABSTRACT
INTRODUÇÃO: As reconstruções dos ossos do crânio podem ser especialmente desafiadoras. Existem três indicações para se realizar uma cranioplastia: readquirir proteção contra traumas, recuperação do contorno craniano e tratamento da síndrome de trefinado. Este trabalho tem como objetivo mostrar a experiência do autor com cranioplastias e discutir as estratégias cirúrgicas de reconstrução. MÉTODOS: Foi feita uma análise retrospectiva de 16 casos consecutivos de reconstrução craniana operados na saúde pública (INTO - RJ) e na prática privada em 2013 e 2014. RESULTADOS: De janeiro de 2013 a janeiro de 2014, 16 pacientes foram operados. Treze eram homens. A idade foi de 10 a 72 anos. Doze pacientes tiveram sua reconstrução feita com enxerto de parietal, 2 com prótese customizada, 1 com redução e fixação da fratura, e 1 com reconstrução de partes moles. A maioria dos defeitos estavam localizados na região fronto-orbital. As próteses customizadas foram usadas em defeitos de 192 e 22,5 cm2. Tivemos complicações em 5 pacientes: lesão de seio sagital, irregularidade no contorno, seroma, não integração do enxerto, vazamento de líquor e extrusão da prótese. Todas as complicações foram resolvidas sem prejuízo à reconstrução. O seguimento variou de 10 a 22 meses. Todos mostraram-se satisfeitos com as reconstruções e houve melhora de funções neurológicas. CONCLUSÃO: A cranioplastia é uma cirurgia primariamente reparadora para recuperar a função protetora do crânio e tratar a síndrome do trefinado. O enxerto autólogo de parietal segue sendo a primeira escolha. A prótese está indicada quando há um grande defeito ou quando a captação do enxerto parietal não é possível.
INTRODUCTION: Reconstruction of the skull bones can prove challenging. There are three indications for carrying out cranioplasty: (1) recovery of protection against trauma, (2) recovery of the cranial contour, and (3) treatment of the syndrome of the trephined. The objective of this report is to present the experience of the author with cranioplasties, and discuss surgical reconstruction strategies. METHODS: This report presents a retrospective analysis of 16 consecutive cases of cranial reconstruction, which were operated in 2013 and 2014 in the public health service (INTO - RJ) and in the private practice. RESULTS: From January 2013 to January 2014, 16 patients underwent surgery. Thirteen were men. Their age ranged from 10 to 72 years. Twelve patients underwent reconstruction with a parietal graft, two with custom prosthesis, one with fracture reduction and fixation, and one with reconstruction of soft parts . Most of the defects were located in the fronto-orbital region. The customized prostheses were used in defects of 192 cm2 and 22.5 cm2. Complications occurred in five patients: lesion of the sagittal sinus, irregularity in the contour, seroma, failure in graft integration, leakage of cerebrospinal fluid, and extrusion of the prosthesis. All the complications were resolved without impairment to the reconstruction. The follow-up time ranged from 10 to 22 months. All the patients were pleased with the reconstructions, and improvement of the neurological functions was reported. CONCLUSION: Cranioplasty is a primarily restorative surgical procedure that is used to restore the protective function of the skull and to treat the syndrome of the trephined. The autologous parietal graft is the surgeons' first choice. The prosthesis is indicated when there is a major defect or when the harvesting of a parietal graft is not possible.
