초록접수 현황
| 14F-229 | 포스터 발표 |
Intrathoracic Extramedullary Hematopoiesis Mimicking Mediastinal Tumor in a Patient with Hereditary Spherocytosis
Jae Bum Park, MD, Jae Joon Hwang, MD, Song Am Lee, MD, Woo Surng Lee, MD, Yo Han Kim, MD. Hwang Jae Joon, MD
Thoracic and Cardiovascular Surgery, Konkuk University Medical Center, Korea
Background : : Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside of the bone marrow. It is usually associated with myeloproliferative disorders or hematologic disorders such as hemolytic anemia. We report a case of intrathoracic EMH, manifesting as a posterior mediastinal tumor in a patient with hereditary spherocytosis.
Methods : A 45-year-old man who presented with anemia, jaundice and abdominal pain was diagnosed with hereditary spherocytosis. Computed tomography (CT) scan disclosed chronic calculous cholecystitis and marked splenomegaly measuring almost 20cm at maximum diameter. It also incidentally identified a 2.6cm homogenous right paravertebral (at the level of T8) mediastinal mass of soft tissue density (Fig 1A). Definite diagnosis was established by video-assisted thoracoscopic surgery (VATS) excisional biopsy.
Results : The patient uneventfully underwent a complete excision by video-assisted thoracoscopic surgery (VATS) to confirm the diagnosis. Concurrently, laparoscopic splenectomy and cholecystectomy surgery was accomplished as well. A 2.6 x 2 x 2 cm ovoid shape soft friable mass was encountered at the upper margin of posterior arc of 8th rib. It was well encapsulated and contained bloody, fragile material. A histopathological examination demonstrated a diffuse hematopoiesis consisting of megakaryocytes, immature granulocytic and myeloid precursor cells (Fig 1B), and confirmed a diagnosis of EMH.
Conclusion : In patients with benign or neoplastic hemolytic disorders presenting an intrathoracic mass, a diagnosis of EMH should be considered in the differential diagnosis and further management. In this respect, we herein present a case of successful treatment of EMH simulating mediastinal neurogenic tumor in which VATS was applied for the diagnosis and complete resection.
Methods : A 45-year-old man who presented with anemia, jaundice and abdominal pain was diagnosed with hereditary spherocytosis. Computed tomography (CT) scan disclosed chronic calculous cholecystitis and marked splenomegaly measuring almost 20cm at maximum diameter. It also incidentally identified a 2.6cm homogenous right paravertebral (at the level of T8) mediastinal mass of soft tissue density (Fig 1A). Definite diagnosis was established by video-assisted thoracoscopic surgery (VATS) excisional biopsy.
Results : The patient uneventfully underwent a complete excision by video-assisted thoracoscopic surgery (VATS) to confirm the diagnosis. Concurrently, laparoscopic splenectomy and cholecystectomy surgery was accomplished as well. A 2.6 x 2 x 2 cm ovoid shape soft friable mass was encountered at the upper margin of posterior arc of 8th rib. It was well encapsulated and contained bloody, fragile material. A histopathological examination demonstrated a diffuse hematopoiesis consisting of megakaryocytes, immature granulocytic and myeloid precursor cells (Fig 1B), and confirmed a diagnosis of EMH.
Conclusion : In patients with benign or neoplastic hemolytic disorders presenting an intrathoracic mass, a diagnosis of EMH should be considered in the differential diagnosis and further management. In this respect, we herein present a case of successful treatment of EMH simulating mediastinal neurogenic tumor in which VATS was applied for the diagnosis and complete resection.
책임저자: Hwang Jae Joon
Konkuk University Medical Center, Seoul, Korea
연락처 : Park Jae Bum, Tel: 02-2030-7590 , E-mail : 20090048@kuh.ac.kr
