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Case Report |
1 All authors: Department of Radiology, New York Presbyterian Hospital, Cornell University, Abdominal Imaging Section, 525 E 68th St., New York, NY 10021.
Received July 14, 2003;
accepted after revision September 8, 2003.
Address correspondence to P. Gupta
(pramodvagisha{at}hotmail.com).
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In the last 25 years, only 12 cases of focal intrahepatic extramedullary hematopoiesis have been described in the literature with any imaging diagnostic workup.
We report a rare case of intrahepatic extramedullary hematopoiesis occurring solely in the liver. This case is also unique in that focal extramedullary hematopoiesis presents as multiple fat-containing lesions, which has never been described.
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On physical examination, the patient had a cushingoid appearance with truncal obesity and Noonan facies, was 1.59 m (5 ft 4 inches) tall, and weighed 113 kg (250 lb). The laboratory results included a hemoglobin level of 11.9 g/dL (normal = 13.8–17.2 g/dL), a hematocrit of 37.5% (normal = 41–50%), a platelet count of 147 x 103/µL (normal = 130–400 x 103/m L), a WBC count of 6.5 x 103/µL (normal = 3.8–10.8 x 103/µL), aspartate aminotransferase level of 16 U/L (normal = < 42 U/L), alkaline phosphatase level of 190 U/L (normal = 25–125 U/L), and total bilirubin of 0.3 mg/dL (normal = < 1.3 mg/dL).
The unenhanced CT scan showed multiple well-defined slightly heterogeneous fat density lesions (–30 to –50 H) of variable sizes in both lobes of the liver, with the largest one in the right lobe measuring 3.9 x 5.4 cm (Fig. 1A). On contrast-enhanced CT, none of the lesions showed significant enhancement (Fig. 1B). No lesions or mass were identified in the spleen, adrenal glands, or kidneys. Sonography showed multiple diffusely hyperechoic lesions corresponding to those observed on CT (Fig. 1C).
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Differential possibilities at this point included lipomatous lesions of the liver such as angiomyolipomas, lipomas, and myelolipomas, and less-likely focal fatty infiltration, fatty metamorphosis in hepatomas, and multiple fat-containing adenomas. Because the patient was male without any history of tuberous sclerosis and did not have any predisposing factors for hepatomas, it was considered desirable to obtain a histopathologic diagnosis. Fine-needle aspiration cytology of the largest lesion in the right lobe of the liver was obtained under sonographic guidance and showed extramedullary hematopoiesis. The patient underwent bone marrow biopsy a few days later, which showed hypocellular marrow.
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To our knowledge, only 12 cases of focal intrahepatic extramedullary hematopoiesis have so far been reported with imaging features. Intrahepatic extramedullary lesions were solitary in five patients and multiple in the rest. Most intrahepatic extramedullary lesions were well defined on all imaging techniques. Extrahepatic extramedullary foci were present in six patients, absent in three, and not evaluated in three. The lesions were hypoechoic in five of the nine patients who underwent sonographic examination and inhomogeneous in the rest. In our patient, lesions were hyperechoic reflecting fatty components. On unenhanced CT, lesions were hypodense in six, hyperdense in one, and heterogeneously hypodense in two patients. Contrast-enhanced CT was performed in five patients, with patchy and heterogeneous enhancement in three [4–6] and no enhancement in two [1], including our patient. Final diagnosis was made with core biopsy in five patients; fine-needle aspiration cytology in four, including our patient; by laparotomy in one; and by autopsy in one. Data are not available for two patients. Almost all patients had some underlying hematologic disorder as a predisposing factor for extramedullary hematopoiesis with the exception of one, in which the cause could not be established. In our patient, the predisposing factor is not clear except that he had mild anemia. Also, the extramedullary hematopoiesis lesions with macroscopic fat could not be understood. Our hypothesis is that possibly these lesions, during their development, underwent fatty conversion, which is usually seen in the bone marrow.
In the present case, the correct diagnosis was not originally considered because focal intrahepatic extramedullary hematopoiesis presenting as fat-containing masses has not been described so far and has never been included in the differential diagnosis of lipomatous liver lesions. The differential diagnosis for lipomatous liver lesions includes benign tumors such as lipoma, angiomyolipoma, myelolipoma, sometimes adenomas, and focal fatty infiltration, which usually have poorly defined margins. The malignant lipomatous liver lesions include liposarcoma, hepatocellular carcinoma with fatty metamorphosis, and metastases of a malignant ovarian teratoma containing fat and calcifications [7, 8].
Considering the rarity of this entity, it may not be included in the initial differential of fat-containing liver lesions, but we believe it merits consideration in the appropriate clinical setting. Because the current experience is limited to describing the spectrum of specific radiologic findings, the final diagnosis of intrahepatic extramedullary hematopoiesis necessitates histopathologic diagnosis by core biopsy or fine-needle aspiration cytology.
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