Ann Thorac Surg 1996;61:1003-1005
© 1996 The Society of Thoracic Surgeons
Case Report
Extramedullary Hematopoiesis Simulating Posterior Mediastinal Tumors
Chun K. Loh, MD, PhD,
Carlos Alcorta, MD,
A. James McElhinney, MD
Department of Cardiothoracic Surgery, Palisades General Medical Center, North Bergen, New Jersey
Accepted for publication September 8, 1995.
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Abstract
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We report the case of a patient with intrathoracic extramedullary hematopoiesis presenting as a posterior mediastinal tumor in response to chronic hemolytic anemia. Noninvasive studies including chest roentgenograms, computed tomographic scans, magnetic resonance images, and nuclear scans can establish the diagnosis in most cases. In equivocal cases, transthoracic needle biopsy and open biopsy should be considered. Surgical resection is recommended only for patients with symptoms of compression.
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Introduction
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Extramedullary hematopoiesis is generally considered to be the result of a compensatory response to either chronic hemolytic conditions or disturbance of bone marrow [1, 2]. Here we present the case of a patient with extramedullary hematopoiesis presenting as a posterior mediastinal tumor in response to chronic hemolytic anemia.
An 86-year-old man of Hispanic ancestry was admitted to the hospital because of a 1-month history of pleuritic left chest pain radiating to the back and a large posterior mediastinal mass. Medical history included cholecystectomy for gallstones of unknown composition (1920), splenectomy for hemolytic jaundice resulting from hereditary spherocytosis (1978), and normal chest radiographs (1989, 1991).
On physical examination, the patient had decreased breath sounds bilaterally and an enlarged left axillary node. He had no motor weakness of the extremities. Laboratory studies showed no evidence of any hematologic abnormality other than anemia (hemoglobin, 11.5 g/dL; hematocrit, 32.8%) and a slight reticulocytosis. Radiologic evaluation included a chest radiograph (Fig 1
), a computed tomographic scan of the chest (Fig 2), and a technetium 99m–labeled bone scan (an intense uptake at the T-5 or T-6 level). The results showed evidence of bilateral paravertebral heterogeneous necrotic masses with bony erosion at the level of T-5 or T-6. A needle biopsy performed under computed tomographic control yielded cytologic findings suggestive of malignant lymphoma. As subsequent results of an open biopsy of the axillary lymph node and a bone marrow biopsy were negative, an exploratory left thoracotomy was undertaken.
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Fig 1. . (A) Posteroanterior and (B) lateral chest radiographs showing posterior mediastinal mass. The chest radiologic features of the mass are its multiple lobular and well-circumscribed shape with rounded margins [1–3].
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Fig 2. . Computed tomographic scan of chest showing (A) vertebral-body erosion at T-5 and (B) bilateral paravertebral masses at T-7. Computed tomographic images are characterized by the presence of solid, uncalcified masses with uneven and bilateral distribution in the paravertebral region between T-2 and T-12 [2, 3]. Smaller masses are usually located more proximally [3], as seen here.
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At exploration, a large, hourglass-shaped, soft, dark-red tumor extending from T-5 to T-9 was noted. It was covered with parietal pleura and overlay the descending thoracic aorta. A wedge biopsy was performed for diagnosis. During this procedure, profuse low-pressure bleeding was encountered. Usual measures to control the hemorrhage were unsuccessful. To obtain control, the mass had to be completely and expeditiously extirpated. This was accomplished by debulking most of the vascular amorphous mass using blunt dissection with the fingers and the hand passed around the mass after the parietal pleura had been incised along the lateral edge of the tumor. The residual fragments of the tumor were removed with either pledgets or a curette. Bleeding from the tumor bed and vertebral bone marrow was controlled using a combination of a hemostatic agent, bone wax, and electrocoagulation.
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Comment
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To date, 89 cases of intrathoracic extramedullary hematopoiesis have been reported in the literature. Contrary to the findings in the majority of previous cases [1–3], the mass in our patient grew relatively fast (normal chest radiograph 2 years before admission) and was associated with erosion of vertebral bone [4].
As for the diagnosis, the presence of extramedullary hematopoiesis should be suspected in any patient with known chronic hemolytic disease who is seen with the characteristic chest radiograph (see Fig 1
) [1–3]. Computed tomography of the chest alone (see Fig 2) or possibly in combination with magnetic resonance imaging (high signal intensity rim) or technetium 99m–labeled sulfur colloid scan (increased uptake) will probably confirm the diagnosis in most cases [2–8]. In atypical cases, transthoracic needle biopsy and open biopsy should be considered [4, 6, 7]. Using a needle biopsy to forestall thoracotomy, however, may not yield sufficient tissue for diagnosis [6]. Further, it should be noted that either procedure can cause dangerous hemorrhage from the tumor [2, 3, 6]. Bleeding can be controlled by the usual surgical measures, including complete resection of the mass if necessary. The hemorrhagic complications in our patient required complete extirpation of the mass. Finally, it is generally agreed that surgical decompression, followed by radiotherapy if necessary, is indicated only in patients with symptoms of compression [1–3].
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Footnotes
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Address reprint requests to Dr Loh, Department of Cardiothoracic Surgery, Veterans Affairs Medical Center, 130 W Kingsbridge Rd, Bronx, NY 10468.
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References
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- Andras C, Klassen KP, Holaday WJ. Tumor-simulating intrathoracic extramedullary hematopoiesis. Ann Thorac Surg 1970;10:75–80.[Medline]
- Elbers H, Stadt JVD, Wagenaar SS. Tumor-simulating thoracic extramedullary hematopoiesis. Ann Thorac Surg 1980;30:584–7.[Abstract]
- Falappa P, Danza FM, Leone G, Pincelli G, Marin AW, Bock E. Thoracic extramedullary hematopoiesis: evaluation by conventional radiology and computed tomography. Diagn Imaging 1982;51:19–24.[Medline]
- Hines GL. Paravertebral extramedullary hematopoiesis (as a posterior mediastinal tumor) associated with congenital dyserythropoietic anemia. J Thorac Cardiovasc Surg 1993;106:760–1.[Medline]
- Savader SJ, Otero RR, Savader BL. MR imaging of intrathoracic extramedullary hematopoiesis. J Comput Assist Tomogr 1988;12:878–80.[Medline]
- Catinella FP, Boyd AD, Spencer FC. Intrathoracic extramedullary hematopoiesis simulating anterior mediastinal tumor. J Thorac Cardiovasc Surg 1985;89:580–4.[Abstract]
- Fielding JR, Owens M, Naimark A. Intrathoracic extramedullary hematopoiesis secondary to B12 and folate deficiency: CT appearance. J Comput Assist Tomgr 1991;15:308–10.
- Gumbs RV, Higginbotham-Ford EA, Teal JS, Kletter GG, Castro O. Thoracic extramedullary hematopoiesis in sickle-cell disease. Am J Radiol 1987;149:889–93.[Abstract/Free Full Text]
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Abstract
Introduction
