ABSTRACT
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.
Subject(s)
Hematopoiesis, Extramedullary , Myelodysplastic Syndromes , Hematopoiesis, Extramedullary/genetics , Humans , Male , Middle Aged , Mutation , Myelodysplastic Syndromes/genetics , Myelodysplastic Syndromes/pathology , Phosphoproteins/genetics , RNA Splicing Factors/geneticsABSTRACT
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.
Subject(s)
Cervical Vertebrae/pathology , Muscle Weakness/diagnosis , Muscular Diseases/diagnosis , Neck Muscles/pathology , Aged , Aged, 80 and over , Biopsy , Cervical Vertebrae/diagnostic imaging , Female , Humans , Magnetic Resonance Imaging/methods , Male , Middle Aged , Muscle Weakness/pathology , Muscle Weakness/physiopathology , Muscular Diseases/pathology , Muscular Diseases/physiopathology , Neck Muscles/diagnostic imaging , Neck Muscles/physiopathology , Retrospective Studies , SyndromeABSTRACT
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.