RESUMEN
Mixed histiocytoses are a rare and recently recognized subset of histiocytic disorders that may involve the skin, characterized by the synchronous or metachronous development of lesions with Langerhans and/or non-Langerhans cell histiocytosis histopathological features. Around 10% of patients diagnosed with histiocytosis may develop a hematological malignancy, often with dramatic prognostic consequences. We hereby describe the exceptional case of a patient developing a MAP2K1-driven mixed histiocytosis with Langerhans cell histiocytosis, Rosai-Dorfman-Destombes disease, and Erdheim-Chester disease features and cutaneous involvement, progressing to a fatal and clonally-related acute myeloid leukemia. We reviewed the literature on similar cases and discussed the histopathological difficulties in their diagnosis and their clinical-pathological features.
Asunto(s)
Enfermedad de Erdheim-Chester/genética , Histiocitosis de Células de Langerhans/genética , Histiocitosis Sinusal/genética , Leucemia Mieloide Aguda/patología , MAP Quinasa Quinasa 1/genética , Anciano , Biopsia , Diagnóstico Diferencial , Enfermedad de Erdheim-Chester/complicaciones , Enfermedad de Erdheim-Chester/patología , Resultado Fatal , Femenino , Histiocitos/patología , Histiocitosis de Células de Langerhans/complicaciones , Histiocitosis de Células de Langerhans/tratamiento farmacológico , Histiocitosis de Células de Langerhans/patología , Histiocitosis Sinusal/complicaciones , Histiocitosis Sinusal/patología , Humanos , Leucemia Mieloide Aguda/diagnóstico , Leucemia Mieloide Aguda/etiología , Masculino , Persona de Mediana Edad , Neoplasias Primarias Secundarias/patología , Piel/patologíaRESUMEN
BACKGROUND: The aim of this study was to evaluate the outcomes of patients suffering prostate cancer (PCa) treated conservatively using 1064 nm laser energy for focal laser ablation (FLA). The patients included in the study were unsuitable for surgery or unwilling to receive external beam radiotherapy because they were afraid of the possible side effects of whole-gland therapies. METHODS: This study included patients with a diagnosis of nonmetastatic PCa who underwent FLA using SoracteLiteTM system. Tissue ablation was performed at a fixed power of 5 W by the diode multichannel laser system EchoLaser X4 that uses laser light transmitted through optical fibres causing the target tissue to undergo irreversible thermal damage. Functional outcomes were evaluated with the International Prostatic Symptoms Score (IPSS) and 5-item version of the International Index of Erectile Function (IIEF-5) before the treatment and one year later. RESULTS: Ten patients suffering non-metastatic PCa were included. Four decided upon a conservative treatment because of reduced performance status and for six patients the procedure was chosen electively. All patients underwent multiparametric magnetic resonance imaging at 3 and 12 months and eight out of ten patients underwent prostate biopsy at 6 months. Persistent disease was detected in 3 patients who underwent a second ablation. In these patients at the biopsy following the second ablation none harbored residual disease. At follow-up, no patient suffered urinary incontinence requiring the use of pads. No significant worsening in sexual potency measured with IIEF-5 (p = 0.356) or prostatic symptoms measured at IPSS (p = 0.462) were recorded comparing pre-treatment condition vs one-year follow-up. Compared with baseline, prostate-specific antigen was significantly reduced at one-year follow-up (3.7 ± 1.1 vs 7.9 ± 4.1 ng/mL; p = 0.008). CONCLUSIONS: Although whole gland therapies remain the gold standard treatment for PCa, our results indicate that the SoracteLiteTM system for focal laser ablation, as a very preliminary step, appears to offer a short-term oncologic control of PCa with negligible side effects.