Skeletal Radiol (2007) 36:985–989 DOI 10.1007/s00256-007-0314-6
CASE REPORT
Generalized intramuscular granulocytic sarcoma mimicking polymyositis Jan Fritz & Wichard Vogel & Claus D. Claussen & Martin Wehrmann & Philippe L. Pereira & Marius S. Horger
Received: 26 December 2006 / Revised: 15 February 2007 / Accepted: 14 March 2007 / Published online: 11 May 2007 # ISS 2007
Abstract We report a case of granulocytic sarcoma exclusively manifesting as diffuse intramuscular infiltration of the proximal upper and lower limb girdle and the torso muscles in a patient with previous history of acute myelogenous leukemia 5a. Whole-body CT showed widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions. On whole-body MRI, lesions were homogeneously hyperintense on fat saturated T2weighted images, isointense on T1-weighted images and strongly enhancing after intravenous gadolinium contrast administration. Histopathology revealed muscular infiltration of blast cells with identical immunochemistry to the initial manifestation of leukemia, diagnostic for an extramedullary relapse manifesting as granulocytic sarcoma. CT and MRI characteristics of this previously undocumented manifestation of granulocytic sarcoma should assist in the identification of such cases. Keywords Chloroma . Granulocytic sarcoma . Myelogenous leukemia . CT . MRI J. Fritz (*) : C. D. Claussen : P. L. Pereira : M. S. Horger Department of Diagnostic Radiology, Eberhard-Karls-University, Hoppe-Seyler-Strasse 3, 72076 Tübingen, Germany e-mail:
[email protected] W. Vogel Department of Internal Medicine-Oncology, Eberhard-Karls-University, Ottfried-Müller-Str. 5, 72070 Tübingen, Germany M. Wehrmann Department of Pathology, Eberhard-Karls-University, Liebermeisterstraße 8, 72076 Tübingen, Germany
Introduction Granulocytic sarcoma is a localized extramedullary tumor composed of malignant cells of myeloid-lineage derivation also known as chloroma, extramedullary cell tumor or myeloid sarcoma [1]. It may be the first manifestation of the disease progression of non-lymphocytic leukemia or secondary in patients with a history of myelodysplastic disorders [2, 3]. Most commonly documented sites of occurrence are the orbits, bones and subcutaneous soft tissues [1, 4, 5]. Other sites that have been described include paranasal sinuses, lymph nodes, spine, brain, pleural and peritoneal cavities, breasts, thyroid gland, salivary glands, small bowel, lungs, and testes. We report, to the best of our knowledge, the first case of granulocytic sarcoma exclusively manifesting as generalized intramuscular infiltration.
Case report A 62-year-old woman was diagnosed with acute myelogenous leukemia in August 2004. Cytogenetic analysis demonstrated a complex aberrant karyotype including deletion of chromosome 7. Based on the WHO criteria the leukemia was classified as acute myeloid leukemia not otherwise categorized (acute monoblastic leukemia of 5A subtype according to the French-American-British classification). Subsequently, the patient received induction and consolidation chemotherapy with total body irradiation, followed by allogeneic peripheral blood stem cell transplantation from a sibling donor. After completion of therapy and achievement of complete remission, the patient was discharged receiving graft-versus-host prophylaxis with cyclosporine and mycophenolate mofetil.
986
Skeletal Radiol (2007) 36:985–989
Fig. 1 Coronal multiplanar reconstruction (a) and axial image on the subtrochanteric level (b) of a whole body 16 MDCT following intravenous administration of 120 ml iodine contrast. Images demonstrate widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions (arrows) of the trunk and pelvic muscles without evidence of rim enhancement. There is neither fascial thickening nor increased fascial enhancement excluding fasciitis. Whole body CT did not show either lymph node enlargement or evidence of an extra muscular infectious focus
In January 2006, she presented with progressive myalgia and subfebrile temperature which she had been experiencing for 10 days. On physical examination, the patient appeared healthy with stable vital signs. Manifestations suggestive of chronic graft versus host disease (GHVD) could be found on the skin, in oral mucosa and in the eyes. CBC and biochemistry profile were normal except for mild leukocytosis (13990/μL, normal range 4500–9000/μL), elevated CFig. 2 Coronal (a) and coronal whole body (b) T2-weighted short-tau inversion recovery MR images (TR/TE/ET=4580/79/ 19) showing multiple intramuscular, ill-defined homogeneously hyperintense lesions without fascial involvement
reactive protein (9.85 mg/dl, normal range
