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Original Report |
1
Department of Diagnostic Radiology, The University of Hong Kong, Queen Mary
Hospital, Rm. 405, Block K, Pokfulam Rd., Pokfulam, Hong Kong.
2
Department of Medicine, The University of Hong Kong, Pokfulam, Hong
Kong.
Received September 8, 2000;
accepted after revision November 13, 2000.
Address correspondence to G. C. Ooi.
Abstract
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CONCLUSION. In patients in our study, the clinical and radiologic manifestations of granulocytic sarcoma were variable and occurred most commonly as disease progression in acute myeloid leukemia (73% of the patients). Granulocytic sarcoma lesions were multiple soft-tissue masses with variable enhancement, recurring in nearly 50% of patients at different sites and points of time during the course of the disease. Lesions in the central nervous system, subcutaneous tissues, and genitourinary system accounted for nearly 52% of all lesions. In general, granulocytic sarcoma masses were isodense to muscle on CT scans, and isointense and hyperintense (mild to moderate) on T1- and T2-weighted MR images, respectively. The presence of peripheral rim enhancement with hypodense or hypointense centers on T1-weighted images may, however, mimic an abscess.
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Granulocytic sarcoma is more than twice as common in children as in adults (13% vs. 5%, respectively) with acute myeloid leukemia, and its overall incidence is 2.5-8% [1,7,8]. Forty percent of the cases occur in children younger than 15 years [1]. Although granulocytic sarcoma can arise anywhere, it appears to have a predilection for appearing in bone and perineural tissue [2,8]. Despite anecdotal case reports describing imaging features of granulocytic sarcoma in various anatomic locations, serial radiologic review of patients with this condition has been limited [8,9]. The most recent published findings were in a group of pediatric patients with leukemia [8]. Our study evaluates the role of imaging of granulocytic sarcoma in adults and aims to summarize pertinent imaging features that may help to differentiate granulocytic sarcoma from the other complications of leukemia, primarily infection and secondary tumors.
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Of the 12 patients with granulocytic sarcoma, 11 had imaging studies available for review. The patients ranged in age from 19-68 years (mean age ± SD, 39.3 ± 12.8 years). Six of the patients were women.
Imaging
Most imaging was performed at the radiology department in our institution.
We retrieved the few CT scans that had been obtained at private institutions.
Over time all patients had undergone multiple imaging. Five patients underwent
at least one enhanced MR imaging examination (gadopentetate dimeglumine,
Magnevist; Schering, Berlin, Germany). In total, 16 MR examinations were
performed to evaluate granulocytic sarcoma at the following sites: brain
(n = 2), conjunctiva (n = 1), spinal cord (n = 1),
lumbar nerve roots (n = 2), and testes (n =1). The remaining
granulocytic sarcoma lesions were evaluated with CT scans, of which there were
14 brain, one orbit, three nasopharynx, 10 thorax, 25 abdomen, 16 pelvis, and
two extremity scans. Iodinated IV contrast medium was used in more than 75% of
these scans. One patient underwent 18fluorine-labeled glucose
positron emission tomography (PET) scanning. Eighteen upper abdominal, five
pelvic, and two extremity sonographic scans were obtained in eight
patients.
Almost all CT scans were obtained on one of two models of scanners (HiSpeed Advantage or 9800; General Electric Medical Systems, Milwaukee, WI). MR imaging was performed with a 1.5-T magnet (General Electric Medical Systems). Two experienced radiologists reviewed all imaging studies, paying particular attention to the site of relapse and imaging features. In this study, granulocytic sarcomas occurring within 2 months of each other were considered multifocal.
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Sites of Granulocytic Sarcoma
A total of 29 granulocytic sarcoma tumors were found in 11 patients over
the 12-year period. The central nervous system, subcutaneous tissues, and
urogenital tract were the most common sites of granulocytic sarcoma, occurring
in five, four, and four patients, respectively. Sites of central nervous
system involvement included the brain (n = 2), spinal cord
(n = 1), nerve root (n = 1), and epidural tissue (n
= 3). In the subcutaneous tissues, skin (n = 2) and breast
(n = 3) were involved, whereas urogenital tract granulocytic sarcoma
occurred in the ovaries (n = 2), testes (n = 1), and bladder
(n = 1). Other sites of involvement included muscle (n = 3),
bone (n = 2), conjunctiva (n = 1), nasopharynx (n =
1), paranasal sinuses (n = 1), thorax (n = 2), external
auditory canal (n = 1), stomach (n = 1), and pancreas
(n = 1).
Imaging Characteristics
On both CT and MR imaging, most granulocytic sarcoma lesions were discrete
solid masses with variable enhancement. Primary bone involvement was found in
two patients (skull base and sacrum) in whom lytic destruction was noted. A
large soft-tissue component was associated with the sacral lesion.
Granulocytic sarcoma sited in the conjunctiva, muscle, testes, and bladder
tended to cause diffuse thickening rather than discrete masses. On MR imaging,
conjunctiva and bone lesions were isointense and mildly hyperintense to normal
muscle on T1- and T2-weighted images, respectively (Fig.
1A,1B,1C).
Testicular granulocytic sarcoma was mildly hyperintense to muscle on
T1-weighted images, with a loss of normal testicular hyperintensity on
T2-weighted images (Fig.
1A,1B,1C).
Thickened enhancing tunica vaginalis was noted with bilateral hydrocele.
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Brain parenchymal lesions were slightly hyperdense on CT scans and isointense to gray matter on both T1- and T2-weighted MR images. One patient had a lesion that had a peripheral enhancing rim indistinguishable from an abscess (Fig. 2A,2B,2C). A spinal intramedullary granulocytic sarcoma at the conus medullaris was isointense to muscle and enhanced after the patient received an IV gadolinium injection. Granulocytic sarcoma in the cauda equina and spinal epidura were isointense and hyperintense to muscle on T1- and T2-weighted MR images, respectively, and enhanced markedly (Fig. 3A,3B,3C). On an 18fluorine-labeled glucose PET scan, strong focal uptake was seen in an intracranial dural granulocytic sarcoma, which was excised and shown to have permeated the adjacent skull bone without obvious erosive changes. On CT scans, this lesion shared imaging features similar to parenchymal lesions.
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Nasopharynx, external auditory canal, breast, and skin lesions were isodense to muscle on CT (Figs. 2A,2B,2C and 4). In the skin lesions, moderate enhancement with and without rim enhancement was also noted. One patient had recurrence of breast granulocytic sarcoma at the same site 2 years after the excision biopsy of the initial tumor (Fig. 4). A large cavitating lung nodule with irregular rim enhancement and smaller lung nodules were found in one patient with intrathorax granulocytic sarcoma (Fig. 4). A paraspinal mass with rim enhancement was found in the other patient.
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In two patients, muscle granulocytic sarcoma was multifocal and showed heterogeneous enhancement (Fig. 4). In a third patient, the lesion was hypodense with a thick enhancing rim, reminiscent of a soft-tissue abscess (Fig. 5A,5B). On sonography, the mass was heterogeneously hypoechoic with a capsule. CT-guided aspiration biopsy findings excluded the possibility of an abscess. Other relevant sonographic findings in this series included dilated intrahepatic ducts in a patient with a granulocytic sarcoma mass in the head of the pancreas. Ovarian granulocytic sarcoma caused ipsilateral hydronephrosis in two patients.
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A review of the radiographs of 31 pediatric patients with leukemia with granulocytic sarcoma treated over a 30-year period revealed that 48% of all granulocytic sarcoma tumors occurred in the subcutaneous tissues and orbits [8]. Other common sites were the paranasal sinuses, lymph nodes, and bones. Central nervous system and genitourinary system involvement contributed to only 3.8% of the total number of granulocytic sarcoma lesions compared with 38% of the lesions in our study. However, in reviews of the pathologic findings of granulocytic sarcoma patients, the bone and periosteum are invariably cited as the most frequent sites of involvement [1, 2]. In a series of 338 patients with myeloid leukemia with granulocytic sarcoma who were autopsied between 1949 and 1969 in Hiroshima and Nagasaki, granulocytic sarcoma of the bone was found in 91.3% of the patients, whereas lymph node, kidney, and dural disease was found in 56.1%, 47.8%, and 39.1% of the patients, respectively [1]. The pathogenesis of bone involvement has been postulated to be via trans-haversian canal migration of leukemia cells from the bone marrow to the periosteum and dura [10]. Similarly, central nervous system involvement has been theorized to occur via perivascular or perineural routes from direct dural extension or through capillary migration [11]. Granulocytic sarcoma in the gastrointestinal or genitourinary system may arise de novo from nests of hematopoietic cells [11].
The nasopharynx, external auditory canal, conjunctiva, conus medullaris, and cauda equina were unusual sites of presentation in this series. The latter three occurred in a single patient. Orbital granulocytic sarcoma usually arises from adjacent bone and, less commonly, from the lacrimal gland or intraorbital muscles [8, 12]. The conjunctiva is rarely involved. Our patient complained of a painful, reddened eye, lower limb weakness, and urinary incontinence, in that order. MR imaging confirmed diffuse conjunctival thickening, an enhancing spinal intramedullary lesion, and enlarged and markedly enhancing cauda equina. In our patient with external auditory canal granulocytic sarcoma, fine-section CT performed through the temporal bone revealed soft-tissue masses obliterating both canals with bulging of the tympanic membranes. Complete resolution of these masses was confirmed on MR images a few weeks after treatment.
The CT and MR imaging characteristics of central nervous system lesions in our patients were fairly constant; intracranial granulocytic sarcoma lesions were hyperdense to normal brain tissue on CT and isointense to gray matter on both T1- and T2-weighted MR imaging sequences. Marked enhancement of these lesions was noted on both contrast-enhanced CT and MR imaging. Extracranial central nervous system lesions, including spinal epidural granulocytic sarcoma, were largely isointense on T1-weighted MR images and mildly hyperintense to muscle on T2-weighted images with homogeneous enhancement. These imaging findings, in general, agree with other published studies of central nervous system granulocytic sarcoma and are indistinguishable from those finding of meningioma or lymphoma, particularly findings of epidural lesions [3, 4, 8, 13]. Other soft-tissue granulocytic sarcoma masses were largely isodense to muscle on CT, and isointense and mildly hyperintense on T1- and T2-weighted MR imaging, respectively, with variable degrees of enhancement. In isolation, these granulocytic sarcoma masses may be difficult to differentiate from other focal malignancies, such as lymphoma or carcinoma. However, the multisited and recurrent nature of granulocytic sarcoma lesions in a patient with hematologic malignancy may help with the diagnosis. Imaging features akin to those seen in abscesses were noted in one case each of muscle, brain, paraspinal, skin, and lung granulocytic sarcoma masses. In all these masses, irregular peripheral rim enhancement with hypodense or hypointense centers on T1-weighted images was present. The presence of only mild hyperintensity on T2-weighted images suggesting the lack of a cystic center is, however, unusual in an abscess and may help in the differential diagnosis. Nevertheless, in these patients, imaging alone is insufficient, and aspiration biopsies are warranted to exclude the possibility of an abscess. The prompt diagnosis of granulocytic sarcoma lesions will facilitate appropriate treatment and disease control, preferably before the development of frank systemic leukemia.
In conclusion, granulocytic sarcoma in adults usually occurs as extramedullary relapse in patients with acute myeloid leukemia with concurrent bone marrow disease. The clinical presentations are variable and may include compressive or obstructive symptoms. Despite the variable nature of the disease, there are a few features, particularly of central nervous system granulocytic sarcoma, that may help to distinguish these lesions from other common complications of leukemia. Signs strongly suggestive of granulocytic sarcoma are multiple, enhancing, solid masses occurring at different sites and time points during the course of disease in a patient with either acute myeloid leukemia or myeloproliferative or myelodysplastic disorders. One caveat is that a mass with an enhancing peripheral rim and a hypodense or hypointense center may be indistinguishable from an abscess. In such cases, aspiration biopsy is unavoidable for determining the definitive diagnosis.
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