RESUMO
BACKGROUND: Sweet's syndrome (SS) is an acute febrile neutrophilic dermatosis. It can occur as an idiopathic, drug-induced or malignancy-associated entity. SS is also seen in patients with myelodysplastic syndrome (MDS) where it may present atypically, both clinically and histologically. In a few rare cases of MDS, lymphocytic infiltrates are the presenting feature of SS. METHODS: MEDLINE and Scopus were the data sources for our review. RESULTS: A clinicopathological subsetemerged of 12 male SS patients with MDS and a mean age of 67.3 years in which the initial SS lesions were lymphocytic infiltrates. However, from 0.5 to 8 years later, sequential biopsies revealed neutrophilic dermal infiltration typical of SS. CONCLUSION: Initially lymphocytic infiltrates in this subset could be attributed either to an early timing of the biopsy concerning the age of the lesion or to the dysgranulopoiesis syndrome. A possible relationship between the dysfunction of the receptor of the granulocyte-macrophage colony stimulating factor, the gene of which is located on the pseudoautosomal X-Y region, may exist in MDS patients with initially lymphocytic SS. This could explain the male gender of this subset and might establish initially lymphocytic SS as a distinguished clinicopathological entity for predicting the occurrence and even the prognosis of MDS.
Assuntos
Síndromes Mielodisplásicas/complicações , Síndrome de Sweet/etiologia , Idoso , Anemia Refratária com Excesso de Blastos/complicações , Anemia Refratária com Excesso de Blastos/patologia , Biópsia , Cromossomos Humanos X/genética , Cromossomos Humanos Y/genética , Progressão da Doença , Humanos , Linfócitos/patologia , Masculino , Pessoa de Meia-Idade , Modelos Imunológicos , Síndromes Mielodisplásicas/patologia , Infiltração de Neutrófilos , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/genética , Receptores de Fator Estimulador das Colônias de Granulócitos e Macrófagos/fisiologia , Fatores Sexuais , Pele/patologia , Síndrome de Sweet/genética , Síndrome de Sweet/imunologia , Síndrome de Sweet/patologiaRESUMO
PATIENT: Female, 52 FINAL DIAGNOSIS: Myxoma Symptoms: - MEDICATION: - Clinical Procedure: - Specialty: Oncology. OBJECTIVE: Challenging differential diagnosis. BACKGROUND: Intramuscular myxoma is a benign intramuscular neoplasm. However, sometimes it is difficult to distinguish it from soft-tissue sarcomas that underwent myxomatous degeneration. To the best of our knowledge, only 2 cases of intramuscular myxoma in the soleus muscle have been previously reported. CASE REPORT: We present the case of a 52-year-old Caucasian woman who was referred to our tertiary anticancer hospital for magnetic resonance imaging (MRI) diagnosis for suspicion of mesenchymal tumor. Percutaneous core biopsy revealed an intramuscular myxoma (IM). Despite the benign nature of the lesion, the patient desired that the tumor be removed. She underwent wide local excision. The final histopathologic diagnosis was IM. She is doing well 3 years after the operation. CONCLUSIONS: Percutaneous core biopsy is the procedure of choice for providing preoperative tissue diagnosis. The treatment of choice is wide local excision. IM does not usually recur after surgical removal.