RESUMO
CASE: Retroperitoneal fibrosis (RPF) is a rare chronic fibroinflammatory disorder with typically unknown etiology (i.e., idiopathic). However, several causes have been identified, including retroperitoneal injury or inflammation. In this study, we describe 2 patients who developed RPF complicated by hydroureteronephrosis after (combined) anterior lower interbody fusion (ALIF) and posterior spine fusion. We also reviewed 6 additional cases from the literature of suspected RPF development after spinal surgery. In these cumulative 8 cases, ALIF was the common denominator. CONCLUSION: RPF may develop after ALIF and should be considered a potential longer term complication of this procedure.
Assuntos
Fibrose Retroperitoneal , Fusão Vertebral , Humanos , Procedimentos Neurocirúrgicos , Fibrose Retroperitoneal/etiologia , Fusão Vertebral/efeitos adversos , Fusão Vertebral/métodosRESUMO
We present a rare case of intimal angiosarcoma arising from the iliac artery with unusual symptoms and signs mimicking retroperitoneal fibrosis (RPF). This 84-year-old male presented with constitutional symptoms, abdominal pain, increased acute-phase reactant levels, impaired renal function and a CT-documented left-sided parailiac soft-tissue mass with unilateral extrinsic ureteric obstruction. Whole-body 18F-fluorodeoxyglucose positron emission tomography scan showed highly increased FDG-uptake in a horseshoe-like pattern surrounding the left common iliac artery, but no pathologic activity elsewhere. Further diagnostic workup revealed no signs of malignancy. Because of its location, CT-guided biopsy of the mass was precluded. A tentative diagnosis of RPF was made and treatment with Tamoxifen 20 b.i.d. was started. However, his condition gradually deteriorated, eventually succumbing to severe pneumosepsis. Autopsy revealed extensive iliac intimal angiosarcoma with infiltrative expansion to the left ureter and tumor emboli in both lungs. The present case suggests that intimal angiosarcoma should be included in the differential diagnosis of suspected RPF.
Assuntos
Hemangiossarcoma , Fibrose Retroperitoneal , Idoso de 80 Anos ou mais , Fluordesoxiglucose F18 , Hemangiossarcoma/diagnóstico por imagem , Hemangiossarcoma/terapia , Humanos , Masculino , Tomografia por Emissão de Pósitrons , Fibrose Retroperitoneal/diagnóstico por imagem , Fibrose Retroperitoneal/tratamento farmacológico , Tomografia Computadorizada por Raios XAssuntos
Doença de Erdheim-Chester/tratamento farmacológico , Inibidores de Proteínas Quinases/uso terapêutico , Serina-Treonina Quinases TOR/antagonistas & inibidores , Doença de Erdheim-Chester/diagnóstico por imagem , Doença de Erdheim-Chester/genética , Seguimentos , Humanos , Mutação , Prednisolona/uso terapêutico , Proteínas Proto-Oncogênicas B-raf/genética , Fatores de Tempo , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
We present a case of atypical idiopathic retroperitoneal fibrosis (iRPF) presenting as a large pelvic tumor, for which it proved difficult to exclude T-cell malignant lymphoma. Histopathological examination of biopsy material showed collagenous tissue and fat with an exuberant and predominant T-cell infiltrate, largely consisting of CD4(+) cells expressing the IL-2 receptor-α chain (CD25). Focal plasma cells were negative for the immunoglobulin G4 (IgG4) isotype. T-cell receptor gene rearrangement (TRGR) pattern showed a Gaussian distribution, in keeping with a polyclonal T-cell population. Awareness of the sometimes exuberant and predominant T-cell infiltrate in iRPF should lead to earlier consideration of this disorder. This is particularly the case where there is an atypically localized and/or extensive mass, for which early exclusion of monoclonality with TRGR may provide helpful. Immunohistochemical findings suggest that CD4(+) CD25(+) cells, which are part of a naturally occurring population of regulatory T-cells, may be involved in the pathogenesis of iRPF.