Brief articleDetection of Extramedullary Infiltrates in Acute Myelogenous Leukemia with Whole-Body Positron Emission Tomography and 2-Deoxy-2-[18F]-Fluoro-D-Glucose
Introduction
E
xtramedullary leukemic infiltrates of acute myelogenous leukemia (AML) are also known as granulocytic sarcoma, chloroma or “green cancer” and have been reported for any body site.1, 2 The frequency of extramedullary leukemia (EML) is about 5–10% of all patients with AML varying very much for its different onset forms. EML may appear in the absence of medullary AML (primary EML), at the time of presentation of AML, as an isolated recurrence of AML or with concurrent bone marrow relapse of AML.2 Primary EML is not always followed by AML, conversely, EML after AML is commonly followed by a systemic relapse.3, 4 Primary EML is misdiagnosed in about 50%, most commonly as malignant lymphoma.2 Possible predisposing risk factors for extramedullary infiltrates include chromosomal abnormalities, blast expresssion of neural-cell adhesion molecule or the expression of several T-cell markers.2, 5 It seems necessary that the blast cells have acquired at least some degree of differentiation to migrate into the tissues. Depending on the morphologic subtype or the patient's age, some locations seem to be more typical than others. In AML FAB M4 and M5, the EML presents itself most commonly as gingival hypertrophy or as infiltration of the skin (leukemia cutis).2 In children with AML, lymphadenopathy and splenomegaly appear more often than in adults.2, 3 In adults, involvement of the skeleton, predominantly affecting the metaphyseal regions of the long bones of the lower extremities, is very infrequent. Therapy of EML depends primarily on its time of appearance. The recommended treatment of primary EML is with induction chemotherapy alone, whereas in combined EML/AML, the standard AML chemotherapy protocol should be applied. Isolated EML may be treated with local irradiation or surgery, and possibly with continued intensification chemotherapy.2 To our knowledge, we are the first to report the detection of EML by 2-deoxy-2-[18F]-fluoro-D-glucose whole-body positron emission tomography (FDG-PET).
Section snippets
Case Report
In May 1997, a 25-year-old female patient developed intermittent, later constant pain in the right upper ankle, both knees, as well as the left elbow and wrist. Simultaneously, painless subcutaneous nodules appeared. In October 1997, the patient consulted the clinic of Rheumatology at the University Hospital Zurich because of persistent arthralgias. There, the observation of anemia and thrombocytopenia prompted a bone marrow examination that resulted in the diagnosis of AML FAB M5a. The
Discussion
To our knowledge this is the first report of a detection of extramedullary manifestations of AML by FDG-PET. The role of the FDG-PET examination in other hematologic malignancies, such as non-Hodgkin lymphoma (NHL) or Hodgkin's disease (HD), is increasingly important. Due to the high FDG-uptake, even small manifestations of the lymphoma may be detected, which improves the initial staging of the disease and the follow-up of either chemotherapy or irradiation. Compared with computed tomography
References (6)
- et al.
Cancer Medicine
(1997) - et al.
Extramedullary myeloid cell tumors in acute nonlymphocytic leukemiaa clinical review
J. Clin. Oncol.
(1995) - et al.
Granulocytic sarcoma in nonleukemic patients
Cancer
(1986)
Cited by (27)
Myeloid sarcoma, chloroma, or extramedullary acute myeloid leukemia tumor: A tale of misnomers, controversy and the unresolved
2021, Blood ReviewsCitation Excerpt :18Fluorodeoxy-Glucose Positron Emission Tomography/CT (FDG-PET/CT) appears to be the best imaging modality to assess the presence of eAML. Early reports noted the ability of FDG-PET/CT to both detect eAML and monitor its response to therapy [67,68]. Small case series of FDG-PET/CT when paired with contemporaneous biopsies noted an 80–90% rate of specificity with a maximum standardized uptake value (SUVmax) ranging from 2.1 to 9.7 [69–71].
Diagnostic Imaging: Pediatrics
2017, Diagnostic Imaging: PediatricsFDG-PET imaging in hematological malignancies
2016, Blood ReviewsCitation Excerpt :FDG-PET/CT is not regularly used in the assessment of leukemia [1]. However, several case reports have demonstrated the potential of FDG-PET/CT in diagnosis and follow-up of leukemic bone marrow infiltration [123–125]. In a preliminary study [126], utility of FDG-PET/CT to diagnose extramedullary disease in de novo or relapsed AML patients was described.
Extramedullary Relapse of Acute Myeloid Leukemia after Allogeneic Hematopoietic Stem Cell Transplantation: An Easily Overlooked but Significant Pattern of Relapse
2012, Biology of Blood and Marrow TransplantationCitation Excerpt :Thus, the diagnosis of an EM relapse is often delayed until patients develop the symptoms of a large mass, such as abdominal pain or constipation. However, a number of recent reports have shown that EM infiltration of AML can be detected by 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) or FDG-PET/computed tomography (CT) [43-53]. Stölzel et al. [54] recently reported the results of FDG-PET/CT in 10 patients with AML with histologically proven EM disease at diagnosis (n = 5), relapse after alloSCT (n = 4), or relapse after chemotherapy (n = 1).
Utilization of FDG PET/CT in the management of inflammation and infection in patients with malignancies
2012, PET ClinicsCitation Excerpt :Therefore the uptake cannot be automatically attributed to infection or tumor infiltration, and further investigations are warranted if clinically indicated.22 In patients with leukemia, PET/CT may be useful in detecting extramedullary involvement; however, studies in this area are limited.43–45 The modality has already proved its value in diagnosing Richter transformation of chronic lymphoid leukemia to diffuse large-cell lymphoma, with a reported 91% sensitivity and 80% specificity.46
Utility of 18F-FDG-PET for detecting acute lymphoblastic leukemia: a case series of pediatric acute lymphoblastic leukemia without hematological symptoms
2022, International Journal of Hematology