RESUMO
We present the case of a 56-year-old male patient with paravertebral extramedullary hematopoiesis (EMH) secondary to myelodysplastic syndrome with ring sideroblasts and multilineage dysplasia. In a routine health checkup over 5 years prior, he presented with asymptomatic mild anemia and a posterior mediastinal mass. Pathological and cytomorphological findings of the resected paravertebral mass were similar to those of his bone marrow specimen, and included cellularity with erythroid hyperplasia, multilineage dysplastic changes, and the presence of ring sideroblasts. A concordant SF3B1 mutation was detected in both bone marrow and paravertebral mass samples, suggesting that the EMH cells were derived from the bone marrow.
Assuntos
Hematopoese Extramedular , Síndromes Mielodisplásicas , Hematopoese Extramedular/genética , Humanos , Masculino , Pessoa de Meia-Idade , Mutação , Síndromes Mielodisplásicas/genética , Síndromes Mielodisplásicas/patologia , Fosfoproteínas/genética , Fatores de Processamento de RNA/genéticaRESUMO
BACKGROUND: To date, the histopathologic characteristics of dropped head syndrome (DHS) have not been reported sufficiently. The present study investigates the histopathology of biopsy specimens from the cervical paravertebral region in patients with DHS. METHODS: Histopathological parameters were evaluated in biopsy specimens of the cervical paravertebral soft tissue from 15 patients with DHS. RESULTS: Among the 15 cases of DHS examined, skeletal muscle was identified in 7 cases, all of which showed necrosis, microvessel proliferation and atrophy. The ligament was identified in 12 cases, 8 of which showed degeneration. The lag time between the onset of symptoms and the performance of a biopsy in all 8 cases, which showed degeneration was over 3 months. Microvessel proliferation in the ligament was observed in 1 of the 4 cases, in which the lag time between the onset of symptoms and the performance of a biopsy was less than 3 months (acute or subacute phase), and in 7 of the 8 cases, in which the lag time between the symptoms and the performance of a biopsy was over 3 months (chronic phase). Chronic inflammation in the ligament was identified in 1 of the 12 cases. CONCLUSIONS: The identification of necrosis, microvessel proliferation, and atrophy in the skeletal muscle of patients with DHS and the presence of ligament degeneration and microvessel proliferation in the chronic but not acute or subacute phases may suggest that persistent skeletal muscle damage of the cervical paravertebral region causes subsequent ligament damage in patients with DHS.
Assuntos
Vértebras Cervicais/patologia , Debilidade Muscular/diagnóstico , Doenças Musculares/diagnóstico , Músculos do Pescoço/patologia , Idoso , Idoso de 80 Anos ou mais , Biópsia , Vértebras Cervicais/diagnóstico por imagem , Feminino , Humanos , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/patologia , Debilidade Muscular/fisiopatologia , Doenças Musculares/patologia , Doenças Musculares/fisiopatologia , Músculos do Pescoço/diagnóstico por imagem , Músculos do Pescoço/fisiopatologia , Estudos Retrospectivos , SíndromeRESUMO
Extraskeletal Ewing's sarcoma (EES) is a rare tumor. Paravertebral Ewing's sarcoma requires more extensive therapy as compared to Ewing's sarcoma of bone. Fine needle aspiration cytology (FNAC) plays an important role in the early diagnosis of these cases. We present a case where paravertebral extraosseous Ewing's sarcoma was diagnosed on FNAC in a 19-year-old girl.