Leukemia & Lymphoma
ISSN: 1042-8194 (Print) 1029-2403 (Online) Journal homepage: http://www.tandfonline.com/loi/ilal20
Disseminated sclerotic bone lesions with normal 18 F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome Xiaofeng Shi, Zhongyi Yang, Lili Wu, Shudong Hu, Qian Jiang, Rong Ba, Qin Zhuang, Xianqiu Yu, Lixia Wang, Xiaodong Xi, Yingjian Zhang & Yan Zhu To cite this article: Xiaofeng Shi, Zhongyi Yang, Lili Wu, Shudong Hu, Qian Jiang, Rong Ba, Qin Zhuang, Xianqiu Yu, Lixia Wang, Xiaodong Xi, Yingjian Zhang & Yan Zhu (2015) Disseminated 18
sclerotic bone lesions with normal F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome, Leukemia & Lymphoma, 56:7, 2215-2217, DOI: 10.3109/10428194.2014.987766 To link to this article: http://dx.doi.org/10.3109/10428194.2014.987766
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Accepted author version posted online: 02 Dec 2014. Published online: 04 Jan 2015.
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Date: 08 November 2015, At: 11:22
Leukemia & Lymphoma, July 2015; 56(7): 2215–2217 © 2015 Informa UK, Ltd. ISSN: 1042-8194 print / 1029-2403 online DOI: 10.3109/10428194.2014.987766
LETTER TO THE EDITOR
Disseminated sclerotic bone lesions with normal 18F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome Xiaofeng Shi1,2, Zhongyi Yang3,4, Lili Wu2, Shudong Hu2, Qian Jiang1, Rong Ba1, Qin Zhuang1, Xianqiu Yu1, Lixia Wang1, Xiaodong Xi2, Yingjian Zhang3,4 & Yan Zhu1
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
1Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, China, 2Ruijin Hospital, Shanghai Jiaotong
University School of Medicine, Shanghai, China, 3Department of Nuclear Medicine, Fudan University Shanghai Cancer Center, Shanghai, China and 4Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
A 47-year-old female presented with a 2-year course of weakness, acrocyanosis, plethora, edema, numbness of the lower extremities and irregular menses. Physical examination showed decreased muscle strength and attenuated tendon reflexes. Further electromyograms displayed a demyelinating polyneuropathy-like change with decreased nerve conduction and prolonged distal latencies. Physical examination and ultrasonography confirmed bilateral adenosis of the breast, hepatosplenomegaly and lymphadenomegaly in the bilateral neck, armpit, groin and supraclavicle, as well as the deep abdominal region. No lymph nodes were sufficiently enlarged to be suitable for excision biopsy. Pulmonary function tests showed restrictive abnormalities and a decreased diffusion capacity of carbon monoxide. Endocrine evaluation indicated subclinical hypothyroidism with thyroid stimulating hormone 6.374 U/mL (0.35–4.94 U/ mL), free triiodothyronine 2.46 pmol/L (2.62–6.49 pmol/L) and normal free thyroxine. Her whole blood counts showed white blood cells 13.4 ⫻ 109/L, hemoglobin 159 g/L, and platelets 535 ⫻ 109/L. A bone marrow smear showed inconspicuous abnormality, except for megakaryocyte hyperplasia and platelet clusters. C-reactive protein was high, with a level of 39.5 mg/L (0–8 mg/L). Serum protein electrophoresis showed no abnormal findings and urine Bence Jones protein was negative. Other biochemical parameters were normal. A routine conventional X-ray survey accidently found multiple plaque-like high-density “thickening” in whole-body bones. Detailed medical history inquiry precluded bone pain in this patient. A further bone window of chest/abdomen computed tomography (CT) displayed clearly disseminated sclerotic bone lesions in vertebrae, pelvis, sternum, humerus and clavicle. These scattered, osteosclerotic lesions took on a high-density plaque appearance with an irregular and spiculate edge surrounded by normal bone marrow or fused with bone cortex, most of which were variably sized with
diameters less than 10 mm. Some osteosclerotic lesions were sporadic while others were confluent. Osteogenous metastatic carcinoma was suspected in this patient. Tumor related antigens, such as carcinoembryonic antigen, α-fetoprotein, carbohydrate antigen (CA)-199, CA125, CA153, CA50 and CA242, were negative. Next, whole-body positron emission tomography/CT (PET/CT) was ordered, but results showed normal 18F-fluorodeoxyglucose (FDG) uptake [Figures 1(A)– 1(C)]. Subsequently, the patient had bone tissue drawn from the sclerotic bone tissue under radiographic guidance. The biopsy showed hyperplasia of chondrocytes and osteocytes, and thickness of bone trabeculae, but no malignant cells in the bone marrow [Figures 1(D) and 1(E)]. A bone tissue printing slice showed a slight increase of plasma cells (1%) [Figure 1(F)]. However, in this site, megakaryocyte hyperplasia and clusters were not found. Fluorosis was suspected, but precluded subsequently due to the absence of contact with fluoride and the normal serum fluorine concentration. Osteopoikilosis, which is a non-symptomatic disease, and osteopetrosis, a congenital or inherited disease, were also outside consideration. Eventually, a rare and complicated disease, POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes), was highly suspected. Then a blood sample was sent to the Center of Multiple Myeloma (MM) in Shanghai and monoclonal immunoglobulin A-λ (IgA-λ) was detected by immunofixation electrophoresis. Finally, the definite diagnosis of POEMS syndrome was achieved. POEMS syndrome, also referred to as osteosclerotic myeloma, is a rare, complicated paraneoplastic disorder due to an underlying plasma cell disorder. POEMS syndrome, related to its memorable acronym, includes the above five characteristic features. There are other important features not included in the POEMS acronym. The major criteria
Correspondence: Dr. Yan Zhu, MD, Affiliated Hospital of Jiangsu University, Department of Hematology, No. 438, North Jiefang Road, Zhenjiang, 212000 China. E-mail: [email protected] Received 3 September 2014; revised 12 October 2014; accepted 10 November 2014
2215
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
2216
X. Shi et al.
Figure 1. (A–C) Whole-body PET/CT reveals normal 18F-FDG metabolism of the disseminated sclerotic bone lesions. PET (upper), CT (middle) and fused PET/CT (lower) images in coronal (A), sagittal (B) and transaxial (C) view. (D, E) Bone biopsy drawn under radiographic localization shows hyperplasia of chondrocytes and osteocytes, thickness of bone trabeculae, but no malignant cells in the bone marrow (hematoxylin and eosin stain). Objective ⫻ 10 (D) and objective ⫻ 60 (E). (F) Bone tissue printing slice from the same site shows slightly increased plasma cells (1%) (Wright stain). Objective ⫻ 100.
for the syndrome are polyneuropathy, monoclonal plasma cell-proliferative disorder, sclerotic bone lesions, vascular endothelial growth factor (VEGF) elevation, and the presence of Castleman disease. Minor features include organomegaly, extravascular volume overload, endocrinopathy, characteristic skin changes, papilledema and thrombocytosis. The diagnosis is made with three of the major criteria, two of which must include polyneuropathy and monoclonal plasma cell disorder, and at least one of the minor criteria [1,2]. The features of this patient fulfilled the above criteria. Although lymph node biopsy and VEGF test were not available due to technological limitations, the patient had peripheral neurogenic damage, monoclonal IgA-λ, osteosclerotic lesions, hepatosplenomegaly and lymphadenopathy, edema, subclinical hypothyroidism, skin changes and an elevated platelet count. Because the symptoms of POEMS
syndrome are related to multiple organs, multiple systems and multiple disciplines, diagnoses are often delayed and misdiagnoses are common [3]. Additionally, because the size of the M-protein is typically small [2,3], it was missed by serum electrophoresis at the first onset, and not detected until use of more sensitive immunofixation electrophoresis in this patient. It took 2 years to arrive at the final diagnosis of POEMS syndrome. Pathogenesis of the POEMS syndrome is not well known, but it is widely thought that proangiogenic (VEGF) and proinflammatory (interleukin-6 [IL-6], IL-12, IL-1β and tumor necrosis factor-α [TNF- α]) cytokines mainly produced by malignant plasma cells play an important role in the pathogenesis of this disease [4]. Sclerotic bone lesions are a defining feature of POEMS syndrome and occur in 27–97% of patients [2]. This patient
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
Letter to the Editor 2217 had disseminated osteosclerotic lesions, the extent of which has rarely been observed previously. Osteosclerotic lesions in the bone window of CT took on a high-density appearance, in line with previous reports [5–7], while osteosclerotic lesions mixed with osteolytic were not found in this patient. It was reported that osteolytic lesions in MM could be well seen with 18F-FDG PET/CT [8], and the activity of bone lesions in POEMS could be defined superiorly by 18F-FDG PET/CT as well [9–11]. However, in this patient, PET/CT showed normal 18F-FDG metabolism in the obviously disseminated sclerotic bone lesions. This finding is consistent with the fact that plasmacytes in the bone marrow of patients with POEMS are less than in those with MM [3,6], and its course is chronic. CT-guided biopsy from the sclerotic bone site showed only slightly increased plasma cells. Furthermore, painless characteristics of the osteosclerotic sites also provided evidence. Some previous reports have noted locally increased 18F-FDG uptake at the sites of bone lesions. However, the so-called hot spots described by these reports were at low-density osteolytic sites, not high-density osteosclerotic sites [1,6,8]. D’Souza et al. [12] reported that in 59 patients with POEMS syndrome who received a PET/CT scan, 31% of patients demonstrated increased FDG uptake, but the “hot” lesions on PET/CT scan were mainly bone/soft tissue plasmacytomas. Even a few osteosclerotic sites displayed hypermetabolic characteristics, the degree of which was light [10,11]. However, the most important factor is that although osteolytic lesions also exist in POEMS syndrome, it is the osteosclerotic lesions that define the characteristics of POEMS. It was also reported that sclerotic bone metastases frequently show no or only a low degree of 18F-FDG uptake in breast or pulmonary cancers, compared to lytic bone metastases [13–15]. Besides the sclerotic/lytic nature of bone lesions, the biologic characteristics of the primary tumor might influence the degree of 18F-FDG uptake [15]. Although FDG PET/CT scan did not show superiority to bone windows of CT whole-body images in detecting osteosclerotic lesions in POEMS syndrome, it had the advantage of providing prognostic information and evaluating the therapeutic response [12]. In the present study, we report a patient with POEMS syndrome who had disseminated sclerotic bone lesions with normal 18F-FDG uptake and slightly increased plasmacytes. We present the clinical picture to increase awareness of POEMS syndrome for radiologists, and provide an objective evaluation of PET/CT in detecting osteosclerotic lesions of this disease.
Acknowledgements This work was supported by Science and Technology Commission of Zhenjiang Municipality (SH2012029), National Natural Science Foundation of China (81270594), and China Postdoctoral Science Foundation (2013M541528).
Potential conflict of interest: Disclosure forms provided by the authors are available with the full text of this article at www.informahealthcare.com/lal.
References [1] Dispenzieri A . How I treat POEMS syndrome. Blood 2012;119: 5650–5658. [2] Dispenzieri A . POEMS syndrome: 2014 update on diagnosis, riskstratification, and management. Am J Hematol 2014;89:213–223. [3] Li J, Zhou DB, Huang Z, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 2011;90:819–826. [4] D’Souza A , Hayman SR, Buadi F, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 2011;118:4663–4665. [5] Chong ST, Beasley HS, Daffner RH. POEMS syndrome: radiographic appearance with MRI correlation. Skeletal Radiol 2006;35:690–695. [6] Dispenzieri A . POEMS syndrome. Blood Rev 2007;21:285–299. [7] Shibuya K , Misawa S, Horikoshi T, et al. Detection of bone lesions by CT in POEMS syndrome. Intern Med 2011;50:1393–1396. [8] Walker RC, Brown TL, Jones-Jackson LB, et al. Imaging of multiple myeloma and related plasma cell dyscrasias. J Nucl Med 2012;53: 1091–1101. [9] Minarik J, Scudla V, Bacovsky J, et al. Comparison of imaging methods in POEMS syndrome. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2012;156:52–57. [10] Montoriol PF, Cachin F, Michel JL, et al. Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. Eur J Radiol 2011;80:861–864. [11] Alberti MA , Martinez-Yelamos S, Fernandez A , et al. 18FFDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol 2010;76:180–182. [12] D’Souza A , Lacy M, Gertz M, et al. Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience. Blood 2012;120:56–62. [13] Kruger S, Buck AK , Mottaghy FM, et al. Detection of bone metastases in patients with lung cancer: 99mTc-MDP planar bone scintigraphy, 18F-fluoride PET or 18F-FDG PET/CT. Eur J Nucl Med Mol Imaging 2009;36:1807–1812. [14] Nakai T, Okuyama C, Kubota T, et al. Pitfalls of FDG-PET for the diagnosis of osteoblastic bone metastases in patients with breast cancer. Eur J Nucl Med Mol Imaging 2005;32:1253–1258. [15] Koolen BB, Vegt E, Rutgers EJ, et al. FDG-avid sclerotic bone metastases in breast cancer patients: a PET/CT case series. Ann Nucl Med 2012;26:86–91.
ISSN: 1042-8194 (Print) 1029-2403 (Online) Journal homepage: http://www.tandfonline.com/loi/ilal20
Disseminated sclerotic bone lesions with normal 18 F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome Xiaofeng Shi, Zhongyi Yang, Lili Wu, Shudong Hu, Qian Jiang, Rong Ba, Qin Zhuang, Xianqiu Yu, Lixia Wang, Xiaodong Xi, Yingjian Zhang & Yan Zhu To cite this article: Xiaofeng Shi, Zhongyi Yang, Lili Wu, Shudong Hu, Qian Jiang, Rong Ba, Qin Zhuang, Xianqiu Yu, Lixia Wang, Xiaodong Xi, Yingjian Zhang & Yan Zhu (2015) Disseminated 18
sclerotic bone lesions with normal F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome, Leukemia & Lymphoma, 56:7, 2215-2217, DOI: 10.3109/10428194.2014.987766 To link to this article: http://dx.doi.org/10.3109/10428194.2014.987766
View supplementary material
Accepted author version posted online: 02 Dec 2014. Published online: 04 Jan 2015.
Submit your article to this journal
Article views: 34
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ilal20 Download by: [University of Pennsylvania]
Date: 08 November 2015, At: 11:22
Leukemia & Lymphoma, July 2015; 56(7): 2215–2217 © 2015 Informa UK, Ltd. ISSN: 1042-8194 print / 1029-2403 online DOI: 10.3109/10428194.2014.987766
LETTER TO THE EDITOR
Disseminated sclerotic bone lesions with normal 18F-fluorodeoxyglucose uptake in polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome Xiaofeng Shi1,2, Zhongyi Yang3,4, Lili Wu2, Shudong Hu2, Qian Jiang1, Rong Ba1, Qin Zhuang1, Xianqiu Yu1, Lixia Wang1, Xiaodong Xi2, Yingjian Zhang3,4 & Yan Zhu1
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
1Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, China, 2Ruijin Hospital, Shanghai Jiaotong
University School of Medicine, Shanghai, China, 3Department of Nuclear Medicine, Fudan University Shanghai Cancer Center, Shanghai, China and 4Department of Oncology, Shanghai Medical College, Fudan University, Shanghai, China
A 47-year-old female presented with a 2-year course of weakness, acrocyanosis, plethora, edema, numbness of the lower extremities and irregular menses. Physical examination showed decreased muscle strength and attenuated tendon reflexes. Further electromyograms displayed a demyelinating polyneuropathy-like change with decreased nerve conduction and prolonged distal latencies. Physical examination and ultrasonography confirmed bilateral adenosis of the breast, hepatosplenomegaly and lymphadenomegaly in the bilateral neck, armpit, groin and supraclavicle, as well as the deep abdominal region. No lymph nodes were sufficiently enlarged to be suitable for excision biopsy. Pulmonary function tests showed restrictive abnormalities and a decreased diffusion capacity of carbon monoxide. Endocrine evaluation indicated subclinical hypothyroidism with thyroid stimulating hormone 6.374 U/mL (0.35–4.94 U/ mL), free triiodothyronine 2.46 pmol/L (2.62–6.49 pmol/L) and normal free thyroxine. Her whole blood counts showed white blood cells 13.4 ⫻ 109/L, hemoglobin 159 g/L, and platelets 535 ⫻ 109/L. A bone marrow smear showed inconspicuous abnormality, except for megakaryocyte hyperplasia and platelet clusters. C-reactive protein was high, with a level of 39.5 mg/L (0–8 mg/L). Serum protein electrophoresis showed no abnormal findings and urine Bence Jones protein was negative. Other biochemical parameters were normal. A routine conventional X-ray survey accidently found multiple plaque-like high-density “thickening” in whole-body bones. Detailed medical history inquiry precluded bone pain in this patient. A further bone window of chest/abdomen computed tomography (CT) displayed clearly disseminated sclerotic bone lesions in vertebrae, pelvis, sternum, humerus and clavicle. These scattered, osteosclerotic lesions took on a high-density plaque appearance with an irregular and spiculate edge surrounded by normal bone marrow or fused with bone cortex, most of which were variably sized with
diameters less than 10 mm. Some osteosclerotic lesions were sporadic while others were confluent. Osteogenous metastatic carcinoma was suspected in this patient. Tumor related antigens, such as carcinoembryonic antigen, α-fetoprotein, carbohydrate antigen (CA)-199, CA125, CA153, CA50 and CA242, were negative. Next, whole-body positron emission tomography/CT (PET/CT) was ordered, but results showed normal 18F-fluorodeoxyglucose (FDG) uptake [Figures 1(A)– 1(C)]. Subsequently, the patient had bone tissue drawn from the sclerotic bone tissue under radiographic guidance. The biopsy showed hyperplasia of chondrocytes and osteocytes, and thickness of bone trabeculae, but no malignant cells in the bone marrow [Figures 1(D) and 1(E)]. A bone tissue printing slice showed a slight increase of plasma cells (1%) [Figure 1(F)]. However, in this site, megakaryocyte hyperplasia and clusters were not found. Fluorosis was suspected, but precluded subsequently due to the absence of contact with fluoride and the normal serum fluorine concentration. Osteopoikilosis, which is a non-symptomatic disease, and osteopetrosis, a congenital or inherited disease, were also outside consideration. Eventually, a rare and complicated disease, POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes), was highly suspected. Then a blood sample was sent to the Center of Multiple Myeloma (MM) in Shanghai and monoclonal immunoglobulin A-λ (IgA-λ) was detected by immunofixation electrophoresis. Finally, the definite diagnosis of POEMS syndrome was achieved. POEMS syndrome, also referred to as osteosclerotic myeloma, is a rare, complicated paraneoplastic disorder due to an underlying plasma cell disorder. POEMS syndrome, related to its memorable acronym, includes the above five characteristic features. There are other important features not included in the POEMS acronym. The major criteria
Correspondence: Dr. Yan Zhu, MD, Affiliated Hospital of Jiangsu University, Department of Hematology, No. 438, North Jiefang Road, Zhenjiang, 212000 China. E-mail: [email protected] Received 3 September 2014; revised 12 October 2014; accepted 10 November 2014
2215
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
2216
X. Shi et al.
Figure 1. (A–C) Whole-body PET/CT reveals normal 18F-FDG metabolism of the disseminated sclerotic bone lesions. PET (upper), CT (middle) and fused PET/CT (lower) images in coronal (A), sagittal (B) and transaxial (C) view. (D, E) Bone biopsy drawn under radiographic localization shows hyperplasia of chondrocytes and osteocytes, thickness of bone trabeculae, but no malignant cells in the bone marrow (hematoxylin and eosin stain). Objective ⫻ 10 (D) and objective ⫻ 60 (E). (F) Bone tissue printing slice from the same site shows slightly increased plasma cells (1%) (Wright stain). Objective ⫻ 100.
for the syndrome are polyneuropathy, monoclonal plasma cell-proliferative disorder, sclerotic bone lesions, vascular endothelial growth factor (VEGF) elevation, and the presence of Castleman disease. Minor features include organomegaly, extravascular volume overload, endocrinopathy, characteristic skin changes, papilledema and thrombocytosis. The diagnosis is made with three of the major criteria, two of which must include polyneuropathy and monoclonal plasma cell disorder, and at least one of the minor criteria [1,2]. The features of this patient fulfilled the above criteria. Although lymph node biopsy and VEGF test were not available due to technological limitations, the patient had peripheral neurogenic damage, monoclonal IgA-λ, osteosclerotic lesions, hepatosplenomegaly and lymphadenopathy, edema, subclinical hypothyroidism, skin changes and an elevated platelet count. Because the symptoms of POEMS
syndrome are related to multiple organs, multiple systems and multiple disciplines, diagnoses are often delayed and misdiagnoses are common [3]. Additionally, because the size of the M-protein is typically small [2,3], it was missed by serum electrophoresis at the first onset, and not detected until use of more sensitive immunofixation electrophoresis in this patient. It took 2 years to arrive at the final diagnosis of POEMS syndrome. Pathogenesis of the POEMS syndrome is not well known, but it is widely thought that proangiogenic (VEGF) and proinflammatory (interleukin-6 [IL-6], IL-12, IL-1β and tumor necrosis factor-α [TNF- α]) cytokines mainly produced by malignant plasma cells play an important role in the pathogenesis of this disease [4]. Sclerotic bone lesions are a defining feature of POEMS syndrome and occur in 27–97% of patients [2]. This patient
Downloaded by [University of Pennsylvania] at 11:22 08 November 2015
Letter to the Editor 2217 had disseminated osteosclerotic lesions, the extent of which has rarely been observed previously. Osteosclerotic lesions in the bone window of CT took on a high-density appearance, in line with previous reports [5–7], while osteosclerotic lesions mixed with osteolytic were not found in this patient. It was reported that osteolytic lesions in MM could be well seen with 18F-FDG PET/CT [8], and the activity of bone lesions in POEMS could be defined superiorly by 18F-FDG PET/CT as well [9–11]. However, in this patient, PET/CT showed normal 18F-FDG metabolism in the obviously disseminated sclerotic bone lesions. This finding is consistent with the fact that plasmacytes in the bone marrow of patients with POEMS are less than in those with MM [3,6], and its course is chronic. CT-guided biopsy from the sclerotic bone site showed only slightly increased plasma cells. Furthermore, painless characteristics of the osteosclerotic sites also provided evidence. Some previous reports have noted locally increased 18F-FDG uptake at the sites of bone lesions. However, the so-called hot spots described by these reports were at low-density osteolytic sites, not high-density osteosclerotic sites [1,6,8]. D’Souza et al. [12] reported that in 59 patients with POEMS syndrome who received a PET/CT scan, 31% of patients demonstrated increased FDG uptake, but the “hot” lesions on PET/CT scan were mainly bone/soft tissue plasmacytomas. Even a few osteosclerotic sites displayed hypermetabolic characteristics, the degree of which was light [10,11]. However, the most important factor is that although osteolytic lesions also exist in POEMS syndrome, it is the osteosclerotic lesions that define the characteristics of POEMS. It was also reported that sclerotic bone metastases frequently show no or only a low degree of 18F-FDG uptake in breast or pulmonary cancers, compared to lytic bone metastases [13–15]. Besides the sclerotic/lytic nature of bone lesions, the biologic characteristics of the primary tumor might influence the degree of 18F-FDG uptake [15]. Although FDG PET/CT scan did not show superiority to bone windows of CT whole-body images in detecting osteosclerotic lesions in POEMS syndrome, it had the advantage of providing prognostic information and evaluating the therapeutic response [12]. In the present study, we report a patient with POEMS syndrome who had disseminated sclerotic bone lesions with normal 18F-FDG uptake and slightly increased plasmacytes. We present the clinical picture to increase awareness of POEMS syndrome for radiologists, and provide an objective evaluation of PET/CT in detecting osteosclerotic lesions of this disease.
Acknowledgements This work was supported by Science and Technology Commission of Zhenjiang Municipality (SH2012029), National Natural Science Foundation of China (81270594), and China Postdoctoral Science Foundation (2013M541528).
Potential conflict of interest: Disclosure forms provided by the authors are available with the full text of this article at www.informahealthcare.com/lal.
References [1] Dispenzieri A . How I treat POEMS syndrome. Blood 2012;119: 5650–5658. [2] Dispenzieri A . POEMS syndrome: 2014 update on diagnosis, riskstratification, and management. Am J Hematol 2014;89:213–223. [3] Li J, Zhou DB, Huang Z, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 2011;90:819–826. [4] D’Souza A , Hayman SR, Buadi F, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 2011;118:4663–4665. [5] Chong ST, Beasley HS, Daffner RH. POEMS syndrome: radiographic appearance with MRI correlation. Skeletal Radiol 2006;35:690–695. [6] Dispenzieri A . POEMS syndrome. Blood Rev 2007;21:285–299. [7] Shibuya K , Misawa S, Horikoshi T, et al. Detection of bone lesions by CT in POEMS syndrome. Intern Med 2011;50:1393–1396. [8] Walker RC, Brown TL, Jones-Jackson LB, et al. Imaging of multiple myeloma and related plasma cell dyscrasias. J Nucl Med 2012;53: 1091–1101. [9] Minarik J, Scudla V, Bacovsky J, et al. Comparison of imaging methods in POEMS syndrome. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2012;156:52–57. [10] Montoriol PF, Cachin F, Michel JL, et al. Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. Eur J Radiol 2011;80:861–864. [11] Alberti MA , Martinez-Yelamos S, Fernandez A , et al. 18FFDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol 2010;76:180–182. [12] D’Souza A , Lacy M, Gertz M, et al. Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience. Blood 2012;120:56–62. [13] Kruger S, Buck AK , Mottaghy FM, et al. Detection of bone metastases in patients with lung cancer: 99mTc-MDP planar bone scintigraphy, 18F-fluoride PET or 18F-FDG PET/CT. Eur J Nucl Med Mol Imaging 2009;36:1807–1812. [14] Nakai T, Okuyama C, Kubota T, et al. Pitfalls of FDG-PET for the diagnosis of osteoblastic bone metastases in patients with breast cancer. Eur J Nucl Med Mol Imaging 2005;32:1253–1258. [15] Koolen BB, Vegt E, Rutgers EJ, et al. FDG-avid sclerotic bone metastases in breast cancer patients: a PET/CT case series. Ann Nucl Med 2012;26:86–91.
