Isolated granulocytic sarcoma presenting as a large lung mass

Isolated granulocytic sarcoma presenting as a large lung mass

European Journal of Radiology Extra 69 (2009) e61–e63 Contents lists available at ScienceDirect European Journal of Radiology Extra journal homepage...

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European Journal of Radiology Extra 69 (2009) e61–e63

Contents lists available at ScienceDirect

European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex

Isolated granulocytic sarcoma presenting as a large lung mass Ye Lim Kim a , Eun-Young Kang a,∗ , Ok Hee Woo a , Hwan Seok Yong a , Yu-Whan Oh a , Sang Chul Oh b , Han Kyeom Kim c a

Department of Radiology, College of Medicine, Korea University, Korea University Guro Hospital, 97 Guro-dong, Guro-ku, 152-703 Seoul, Republic of Korea Department of Internal Medicine, College of Medicine, Korea University, Korea University Guro Hospital, 97 Guro-dong, Guro-ku, 152-703 Seoul, Republic of Korea c Department of Pathology, College of Medicine, Korea University, Korea University Guro Hospital, 97 Guro-dong, Guro-ku, 152-703 Seoul, Republic of Korea b

a r t i c l e

i n f o

Article history: Received 19 February 2008 Received in revised form 11 July 2008 Accepted 15 July 2008 Keywords: Granulocytic sarcoma Acute myeloid leukemia Lung disease MDCT

a b s t r a c t We report a unique histologically proven case of isolated intrathoracic granulocytic sarcoma in a 37-yearold man who had sustained complete remission for 45 months after receiving allogeneic bone marrow transplantation for acute myeloid leukemia. Chest MDCT scan showed a large lung mass with atelectasis in the right middle lobe and hilar and mediastinal lymphadenopathy which mimicked advanced bronchogenic carcinoma. © 2008 Elsevier Ireland Ltd. All rights reserved.

1. Introduction Granulocytic sarcoma, also known as chloroma, myeloblastoma, or extramedullary myeloid tumor, is a rare extramedullary solid tumor composed of primitive precursors of the granulocytic series of white blood cells, which includes myeloblasts, promyelocytes, and myelocytes [1–3]. It occurs most frequently in conjunction with myeloid leukemia and can involve any organ [1–3]. It can precede, accompany, or complicate the presence of hematologic malignancy. However granulocytic sarcoma as an isolated extramedullary relapse of acute myeloid leukemia (AML) after allogeneic bone marrow transplantation (BMT), and moreover, involvement of the lung, is rare. We report a unique case of isolated granulocytic sarcoma manifesting as a large lung mass mimicking bronchogenic carcinoma on MDCT in a 37-year-old man who sustained complete remission for 45 months after BMT for AML. 2. Case report A 37-year-old man was admitted with blood-tinged sputum, cough, and fever for 10 days. He was previously diagnosed with AML 5 years ago. He remained in complete remission for 45 months after receiving chemotherapy and BMT. At the time of this admis-

∗ Corresponding author. Tel.: +82 2 2626 1342; fax: +82 2 863 9282. E-mail address: [email protected] (E.-Y. Kang). 1571-4675/$ – see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2008.07.013

sion, the white blood count was 5800/␮l, with normocytic and normochromic red blood cells and thrombocytopenia on peripheral blood morphology. His bone marrow specimen was also normal except for a hypocellular marrow. No abnormalities on chromosomal study were revealed. Only the C-reactive protein was elevated. Therefore, systemic relapse of AML was not evident. Previous bone marrow examination at the time of diagnosis of AML 5 years ago, showed 64.2% of the blast cells and the peripheral blood examination showed a white blood count of 2200/␮l with 3% blast cells. On chest radiographs, a mass opacity with atelectasis was noted in the right middle lobe. Ground-glass opacities were also seen in the right lung and a small pleural effusion was suggested in right lower hemithorax (Fig. 1A). Considering radiographic and clinical findings, pneumonia was suspected at first. However, further evaluation was necessary in suspicion of a malignancy since a mass opacity with atelectasis in the RML was recognized. A chest CT scan was taken subsequently for further evaluation. The chest CT scan taken on the same day as the chest radiograph showed an 8.5 cm × 5 cm × 8 cm sized, irregularly shaped mass with atelectasis in the right middle lobe. The mass showed homogeneous enhancement in contrast enhanced CT scan. The mass narrowed and obstructed the bronchus intermedius and right middle lobar bronchus, encased the right pulmonary artery, infiltrated into the mediastinum directly, and protruded into the left atrium (Fig. 1B–D). Extensive lymph node enlargements were also noted in both upper and lower paratracheal, subcarinar, and right hilar areas (Fig. 1B–D). Several nodular consolidations and focal ground-glass attenuation were seen in the periphery of the right lung. There was a

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Fig. 1. Intrathoracic granulocytic sarcoma in a 37-year-old man, who achieved complete remission after receiving allogeneic bone marrow transplantation for acute myeloid leukemia. (A) Chest PA radiograph shows a mass opacity with complete atelectasis in the middle lobe, ground-glass opacities in the right lung, and pleural effusion in the right hemithorax. (B–D) Chest CT scan with mediastinal window setting demonstrates a large mass with atelectasis in the right middle lobe. The mass obstructs the right middle lobar bronchus, encases the right pulmonary artery, infiltrates into the mediastinum directly, and protrudes into the left atrium. Extensive lymph node enlargement is seen in both upper and lower paratracheal, subcarinar, and right hilar areas. There is a small pleural effusion in the right hemithorax. (E) PET-CT scan shows hot-uptakes only in the lung and mediastinum, corresponding to the lesion on the chest CT scan. (F) Histologic specimen shows diffuse infiltration of leukemic blasts consistent with granulocytic sarcoma (H&E, X400).

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small pleural effusion in the right hemithorax, dependent portion. These findings were suggestive of advanced bronchogenic carcinoma. However, because of the patient’s history of AML and BMT, granulocytic sarcoma involving the lung and mediastinum was the most reasonable diagnosis. Abdominal and pelvic CT scans revealed no pathology. Echocardiogram did not find the mass in left atrium on the following day. F-18-Fluorodeoxyglucose positron emission tomography (PET)-CT scan showed hot-uptakes only in the lung and mediastinum, corresponding to the lesions on the chest CT scan (Fig. 1E). Transbronchial biopsy was performed at the level of the bronchus intermedius which was the obstruction site on bronchoscopy. The histologic specimen showed diffuse infiltration of leukemic blasts consistent with granulocytic sarcoma (Fig. 1F). 3. Discussion Granulocytic sarcoma may be diagnosed in different hematologic malignancies. It occurs most frequently in AML, followed by chronic myeloid leukemia (CML) and myelodysplastic syndromes. Granulocytic sarcoma can arise before, concurrent with, or after the diagnosis of AML or CML [1]. Granulocytic sarcoma occurs in about 5% of adults with a myeloid leukemia [1,2]. However, granulocytic sarcoma as an isolated extramedullary relapse of AML after chemotherapy or following BMT is rare [4–6]. In one study, granulocytic sarcoma was observed in 0.65% of AML patients after allogeneic BMT [4]. However, higher incidences have been reported when transplant-related deaths are excluded [3,5]. The mean time interval between first presentation of granulocytic sarcoma and diagnosis of leukemia was 32.1 ± 20.7 months [2], and granulocytic sarcoma occurred 4–56 months after BMT [4]. In our case, granulocytic sarcoma as an isolated relapse of AML developed in a patient with complete remission after BMT. The interval from complete remission of AML after BMT to the diagnosis of intrathoracic granulocytic sarcoma was 45 months. Granulocytic sarcoma may occur at any part of the body and involve different organs, individually or simultaneously. In general, the breasts, testes, and gut are the most common extramedullary relapse sites for AML [4,5]. Intrathoracic granulocytic sarcoma is uncommon, but it is likely to be detected when it is present, because chest radiographs are usually obtained in leukemic patients [1]. Over a 12-year period of follow-up for 11 adult patients with leukemia who developed a total of 29 granulocytic sarcoma, the central nervous system, subcutaneous tissues, and genitourinary system were the most common sites of disease and account for 52%, and thoracic involvement were found in 2 cases [2]. Intrathoracic granulocytic sarcoma can involve the lung parenchyma, pleura, mediastinum, or airways. The mediastinum is frequently involved relative to the rare pulmonary involvement. Only several cases of granulocytic sarcoma with pulmonary involvement have been reported [1,2,6–8]. In the largest series of intrathoracic granulocytic sarcomas, the mediastinum was the most common site of involvement (6/9 cases), and less commonly, involvement of the lungs (2 cases), the pleura (2 cases), the pericardium (2 cases), and the hilum (2 cases) were observed. Among the 2 cases of pulmonary involvements, 1 was confirmed histologically [1]. Mediastinal involvement of intrathoracic granulocytic sarcoma manifests as a focal mass or generalized mediastinal widening on chest radiographs. Mediastinal widening can be due to lymphadenopathy or diffuse infiltration of mediastinal tissue on CT

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scan [1]. In our case, extensive conglomerated lymphadenopathies were suggested in hilum and mediastinum. These findings are indistinguishable from other causes of mediastinal lymphadenopathy such as lymphoma, metastatic carcinoma, and infections. Other manifestations of intrathoracic granulocytic sarcoma are cardiac enlargement due to cardiac tumor or pericardial effusion, pleural effusions, and lung opacities [1]. Lung manifestations of granulocytic sarcoma consist of alveolar opacities, nodules, masses, or interstitial septal lines [1,3,6–8]. Air bronchograms can be seen in early stages, however in later stages, huge masses typically compressing the adjacent lung parenchyma can be observed [3]. In the nodular form, a large lung nodule or multiple small nodules have been found with or without cavitations, mimicking lung abscesses or fungal infection [1–3]. In our case, a large lung mass with peripheral atelectasis was the main manifestation of pulmonary involvement, which is similar to the usual findings of bronchogenic carcinoma. Intrathoracic granulocytic sarcoma was highly suspected in our case because of the patient’s history of AML and BMT. However, the patient had been in complete remission for 45 months and showed no systemic relapse and no other sites of granulocytic sarcoma involvement. Therefore, transbronchial biopsy of lesion was performed and a histologic diagnosis was made. The proper management of granulocytic sarcoma is controversial and not well established. Radiotherapy or chemotherapy can be still considered for treatment in the case of granulocytic sarcoma [5]. Pulmonary involvement of granulocytic sarcoma may indicate a relatively poor prognosis. However, an isolated extramedullary relapse probably shows a better prognosis than systemic relapse [5]. Unfortunately, this patient died of invasive pulmonary aspergillosis during the follow-up period after chemotherapy. In summary, we report a unique case of histologically proven intrathoracic granulocytic sarcoma following a complete remission state after BMT for AML. Intrathoracic granulocytic sarcoma developed as an isolated relapse of AML. A chest MDCT scan showed a large mass with atelectasis and extensive hilar and mediastinal lymph node enlargements, which are the same findings as bronchogenic carcinoma. Therefore, increased clinical suspicion and tissue biopsy were essential for the diagnosis. References [1] Takasugi JE, Godwin JD, Marglin SI, Petersdorf SH. Intrathoracic granulocytic sarcomas. J Thorac Imag 1996;11:223–30. [2] Ooi GC, Chim CS, Khong PL, et al. Radiologic manifestations of granulocytic sarcoma in adult leukemia. Am J Roentgenol 2001;176: 1427–31. [3] Fritz J, Vogel W, Bares R, Horger M. Radiologic spectrum of extramedullary relapse of myelogenous leukemia in adults. Am J Roentgenol 2007;189: 209–18. [4] Békássy AN, Hermans J, Gorin NC, Gratwohl. A Granulocytic sarcoma after allogeneic bone marrow transplantation: a retrospective European multicenter survey. Acute and chronic leukemia working parties of the European Group for blood and marrow transplantation. Bone Marrow Transplant 1996;17:801–8. [5] Au WY, Kwong YL, Lie AK, Ma SK, Liang R. Extra-medullary relapse of leukemia following allogeneic bone marrow transplantation. Hematol Oncol 1999;17:45–52. [6] Kottaridis PD, Ketley N, Peggs K, et al. An unusual case of intrapulmonary granulocytic sarcoma presenting as interstitial pneumonitis following allogeneic bone marrow transplantation for acute myeloid leukaemia and responding to donor lymphocyte infusion. Bone Marrow Transplant 1999;24:807–9. [7] de Paz R, Canales MA, Hernandez-navarro F. Granulocytic sarcoma (chloroma) of the lung. Br J Haematol 2003;120:176. [8] Avraham S, Akria L, Vlodavsky E, Rowe JM. Granulocytic sarcoma with pulmonary involvement. Am J Hematol 2007;82:222–3.