CT characteristics in 24 patients with POEMS syndrome.

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Acta Radiol OnlineFirst, published on January 8, 2015 as doi:10.1177/0284185114564614

Original Article

CT characteristics in 24 patients with POEMS syndrome

Acta Radiologica 0(0) 1–7 ! The Foundation Acta Radiologica 2015 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0284185114564614 acr.sagepub.com

Xiaofeng Shi1,2,*, Shudong Hu3,4,*, Xianfu Luo3, Ming Luo2, Haiyan You2, Yan Zhu2 and Xiaodong Xi1

Abstract Background: POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) syndrome is a complicated and rare disease. Systematic research on computed tomography (CT) imaging characteristics in POEMS syndrome is scanty. The role of CT in diagnosis needs to be assessed. Purpose: To retrospectively analyze the CT imaging features in 24 patients with POEMS syndrome and evaluate the role of CT in diagnosis of this disease. Material and Methods: Twenty-four patients with confirmed POEMS syndrome were included in the study. Chest and abdominal CT images were analyzed. Results: The three minor diagnostic criteria for POEMS syndrome (extravascular volume overload, organomegaly, and bone lesions) can be detected effectively by CT. Extravascular volume overload involved multiple serous cavities: hydrothorax, hydropericardium, and ascites, which were found in 79.2%, 41.7%, and 54.2% patients, respectively. The volume of effusion was small to moderate. Organomegaly involved multiorgans: hepatomegaly was found in 45.8% patients, splenomegaly in 54.2%, and lymphadenopathy in 75% patients. Hepatospleen exhibited moderate homogeneous enlargement without local enhanced signal after injection of contrast material. Bone lesions were classified into three groups: osteosclerotic, osteolytic, and mixed lesions. Osteosclerotic lesions, taking multiple, scattered, and variably sized highdensity plaque-like appearance, were found in 20.8% patients. Osteolytic lesions, exhibiting punched-out low-density image, were found in 4.2% patients. Mixed ones, holding both common characteristics of them, were detected in 8.3% patients. These CT abnormalities disappeared after effective treatment. Conclusion: CT plays vital role in the confirmation of the three minor diagnostic criteria for POEMS syndrome: extravascular volume overload, organomegaly, and bone lesions.

Keywords Computed tomography (CT), POEMS syndrome, extravascular volume overload, organomegaly, bone lesions Date received: 21 July 2014; accepted: 25 November 2014

Introduction POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia, including the following five predominant features by the acronym definition: Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes; as well as some other characteristics: Castleman disease, sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), extravascular volume overload, papilledema, and thrombocytosis/ polycythemia (1–3). Symptoms of POEMS syndrome are related to multiple organs, multiple systems, and

1 Shanghai Institute of Hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, PR China 2 Department of Hematology, Affiliated Hospital of Jiangsu University, Zhenjiang, Jiangsu, PR China 3 Department of Radiology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, PR China 4 Department of Radiology, Affiliated Renmin Hospital of Jiangsu University, Zhenjiang, Jiangsu, PR China

*Equal contributors. Corresponding authors: Yan Zhu, Department of Hematology, Affiliated Hospital of Jiangsu University, No. 438, North Jiefang Road, Zhenjiang, Jiangsu, PR China. Email: [email protected] Xiaodong Xi, Shanghai Institute of Hematology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, No. 197, second Ruijin Road, Shanghai, PR China. Email: [email protected]

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multiple disciplines, so its early diagnosis is challenging. Imaging methods play a vital role in detecting the organ lesions of patients with POEMS. Most of the published materials on POEMS syndrome primarily recorded the clinical manifestations (4,5), laboratory tests (6,7) or histopathological characteristics (8), but with limited emphasis on imaging features. Even though a few of the reports described imaging characteristics of POEMS, the majority of them were either case reports (9,10) or relatively small series (11,12). There is a paucity of systematic research on imaging characteristics among patients with POEMS syndrome. Computed tomography (CT) is the most frequently used clinical imaging method, so, in this study, we aim to analyze the CT imaging features of the 24 cases with POEMS syndrome and assess the role of CT in the diagnosis of this disease.

Material and Methods The institution review board approved the present retrospective study, and the requirement for informed consent was waived. The study population consisted of 24 patients with POEMS syndrome between 2003 and 2014. All the patients met both two major criteria and one minor criterion for the diagnosis of POEMS syndrome (3). All patients underwent chest and abdominal CT before receiving therapy and were given a non-ionic iodinated contrast material. CT scanning was made from the upper margin of sternum to the lumbar vertebrae with axial consecutive sections (slice thickness was 1.25 mm, 3.37 mm, 5 mm, or 10 mm). All CT images were retrospectively evaluated by two senior radiologists with 15 and 12 years of experience in radiological diagnosis, in a consensus manner. Pleural effusion size was quantitated with a threepoint scale based the anteroposterior quartile: first (0–25%) anteroposterior quartile effusions were small; second (25–50%) anteroposterior quartile effusions were moderate; and third (50–75%) or fourth (>75%) anteroposterior quartile effusions were large (13). Ascites limited in the hepatorenal recess, the clinostatic lowest sites of peritoneal cavity, were small; ascites involved in the whole peritoneal cavity were large; moderate ascites were between both of them (14). Pericardial effusions restricted to the zone behind the posterior wall of left ventricle were defined as small; pericardial effusions occupying the whole pericardial cavity were defined as large; and the moderate was between the small and large (14). IQQA-Liver CT Imaging Analysis System (EDDA Technology, Princeton Junction, NJ, USA) was used to calculate the volume of liver and spleen by summing up the consecutive hepatic or splenic areas after taking into account the slice thickness (15). The average volumes

of liver and spleen were 1070.68 227.52 cm3 (16) and 171.17 24.27 cm3 in normal Chinese adults, respectively. Exceeding 20% from the average value was defined as hepatomegaly or splenomegaly. Lymph nodes measuring greater than 1 cm on the short axis diameters were considered enlarged (17,18). Among these 24 patients, nine patients received chest X-ray scanning, nine patients underwent abdominal ultrasonic examination, and 11 patients underwent superficial ultrasonic test before CT scanning was performed. The available clinical data were also analyzed.

Results Clinical features The average age of patients at the time of diagnosis was 49.4 11.1 years (19 men, 5 women). It took them 6 months to 2 years to get the final definite diagnosis. Most of the patients complained of paresthesia, weakness, skin change, as well as dyspnea, abdominal distension, and pitting edema of extremities. No fracture was present and bone pain was found only in two patients. All but three patients had normal serum albumin levels. These three patients developed hepatic cirrhosis or renal dysfunction finally. Two patients underwent abdominal paracentesis and one received thoracentesis for diagnostic and therapeutic purposes. All the fluids met the criteria for the exudate.

CT imaging characteristics CT imaging features mainly included three aspects: extravascular volume overload, organomegaly, and bone lesions, which are summarized in Table 1. The detailed CT abnormality of each patient is listed in Table 2.

Extravascular volume overload Extravascular volume overload mainly comprised effusion of serous cavities (pleural, pericardial, and peritoneal cavities) and peripheral edema. The detection rates of pleural effusion (hydrothorax), pericardial effusion (hydropericardium), and peritoneal effusion (ascites) are shown in Table 1. The majority of patients had at least one serous cavity involved, and the minority had three involved (Fig. 1). The volume of effusion was small to moderate in the majority of the patients except for three with massive hydrothorax/ascites, who simultaneously had hepatic cirrhosis or renal dysfunction. After injection of non-ionic iodinated contrast material, CT images of effusion showed minimal enhancement. Soft tissue space of chest wall was blurry bilaterally in two patients’ CT images, indicating that



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Table 1. CT imaging features of 24 patients with POEMS syndrome. Characteristics Extravascular volume overload Pleural effusion Pericardial effusion Ascites Any of above Two of above All of above Organomegaly Hepatomegaly Splenomegaly Lymphadenopathy Any of above Two of above All of above Bone lesions Sclerotic Lytic Mixed All of above Multiple Solitary

Detection rate

79.2% 41.7% 54.2% 87.5% 54.2% 16.7%

(19/24) (10/24) (13/24) (21/24) (13/24) (4/24)

45.8% 54.2% 75.0% 91.7% 25.0% 29.2%

(11/24) (13/24) (18/24) (22/24) (6/24) (7/24)

20.8% 4.2% 8.3% 33.3% 29.2% 4.2%

(5/24) (1/24) (2/24) (8/24) (7/24) (1/24)

there was edema in subcutaneous tissue. In nine patients receiving chest X-ray examination, pleural effusion was detected in five patients (55.5%), and in nine patients undergoing abdominal ultrasonic examination, ascites were detected in four patients (44.4%).

Organomegaly Organomegaly included hepatomegaly, splenomegaly, and lymphadenopathy, detection rates of which are shown in Table 1. Hepatospleen exhibited moderate enlargement, taking a homogeneous plump appearance. The volumes of liver and spleen were large (Fig. 2). Lymphadenopathy was found in the various sites of the whole body, including armpit (Fig. 3a) (in 37.5% patients), mediastinum (in 37.5% patients), retroperitoneum (in 29.2% patients), and mesenterium (in 12.5% patients). The majority of lymph nodes enlarged slightly. Because of the difficulty in getting tissue, only two patients with obvious enlarged lymph nodes received biopsy examination, which were confirmed as Castleman disease (angiofollicular lymph node hyperplasia). The ultrasonography detected enlarged lymph nodes in armpit, inguen, or supraclavicle in six of 11 patients (54.5%), but missed those in the deep locations including mediastinum, retroperitoneum, and mesenterium.

Bone lesions Bone lesions in CT were classified into three groups: osteosclerotic, osteolytic, and mixed lesions. An osteosclerotic lesion was defined as a lesion with high density surrounded by normal bone marrow or fused with bone cortex, whereas an osteolytic lesion was defined as a lesion with low density in the bone cortex. Although osteosclerotic lesions are distinctive for POEMS syndrome and one of the diagnostic criteria, osteolytic lesions also exist in these patients (detailed detection rates are shown in Table 1). Osteosclerotic lesions on CT exhibited multiple, scattered, and variably sized high-density plaque-like appearance (Fig. 4). Most of the osteosclerotic lesions were less than 10 mm in diameter. Osteolytic lesions on CT exhibited punched-out low density (Fig. 5). The majority of bone lesions were multiple, but focal solitary sclerotic lesions were also present in the minority of the patients (Table 1). Bone lesions were frequently found in vertebrae (in 29.2% patients), and also found in ribs (in 12.5% patients).

Others Adrenal glands were detected thickening and plump in three male patients, whose adrenocorticotropic hormone elevated. Some non-specific frequently-occurring diseases, such as hepatic cyst (5/24), renal cysts (3/24), cholecystitis (4/24), pulmonary infection (3/24), nephrolithiasis (2/24), and disc herniation (1/24) were also found. When adequate treatment was performed, above CT abnormalities would be improved. The lymphadenomegaly, hepatosplenomegaly, and pleural effusion in a patient significantly improved 3 months after he received autologous hematopoietic stem cell transplantation (auto-HSCT) (Fig. 3).

Discussion Because symptoms of POEMS syndrome are related to multiple organs, misdiagnosis is common (4). The time from the onset of symptoms to diagnosis is long, and the departments of the hospitals where the patients admitted are different. It is a challenge for physicians to get accurate diagnosis as early as possible. CT is playing a more and more important role in the diagnosis of the disease and is the most commonly used imaging method. However, there is no systematic description on CT characteristics of POEMS syndrome. In the current study, we analyzed the CT characteristics of 24 patients with POEMS syndrome and assessed the role of CT in diagnosis of this disease. Two major criteria and at least one minor criterion should be met to establish the diagnosis of POEMS



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Table 2. The detailed CT abnormality of each patient. Case no.

1

2

3

4

5

6

7

8

9

10

11

12

Gender/age* (years) Extravascular volume overload Pleural effusion Ascites Pericardial effusion Organomegaly Hepatomegaly Splenomegaly Lymphadenomegaly Bone lesions Osteosclerotic Osteolytic Case no. Gender/age (years) Extravascular volume overload Pleural effusion Ascites Pericardial effusion Organomegaly Hepatomegaly Splenomegaly Lymphadenomegaly Bone lesions Osteosclerotic Osteolytic

M/64 þ

M/32 þ

M/50 þ

M/61 þ

F/47 þ

M/58 þ

M/31 þ

M/54 þ

M/49 þ

M/42 þ

M57 þ

M40 þ

þ

þ þ þ þ

þ

þ þ

þ þ

þ þ

þ þ

þ þ þ þ –

þ þ þ þ –

þ þ þ þ þ þ þ –

þ

þ

þ þ

þ

þ þ þ

18 M/47 þ þ þ

19 M/62 þ þ

22 F/46 þ þ

23 F/46 þ þ

þ –

þ þ

–

þ þ –

þ þ

þ þ þ 13 M/45 þ þ þ þ þ þ þ –

þ þ

–

þ þ þ þ –

þ 14 M/33 –

15 M/38 –

16 M/41 –

þ

þ

þ

þ þ þ

þ –

þ –

þ þ

þ þ þ 17 M/54 þ þ þ þ þ þ þ –

þ þ þ –

þ þ

þ þ þ

þ

þ þ þ þ 20 F/62 þ þ þ þ þ þ –

þ þ þ þ þ þ þ 21 M/76 þ þ þ þ þ þ þ þ

þ –

–

24 F/50 þ þ þ þ þ þ þ þ –

*Age is at time of diagnosis. þ, positive; –, negative; F, female; M, male.

Fig. 1. A 40-year-old man with POEMS syndrome who complained of slowly progressive dyspnea and general malaise. Unenhanced (a) and contrast-enhanced (b) CT images show small pleural effusion bilaterally (black arrows), massive ascites (gray arrows), and probable pericardial effusion (white arrows) (adipose tissue cannot be precluded).

syndrome in 2003 (3). The major criteria include polyneuropathy and clonal plasmaproliferative disorder. The minor criteria include extravascular volume overload, organomegaly, osteosclerotic lesions, Castleman disease, papilledema, endocrinopathy, and skin changes. CT plays a vital role in detecting three

abnormalities: extravascular volume overload, organomegaly, and bone lesions. Extravascular volume overload is a general feature of POEMS syndrome. The incidence of it, when assessed by ultrasonography, varied greatly in the range of 29–87% according to previous reports



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Fig. 2. A 46-year-old female with POEMS syndrome who had hepatosplenomegaly. (a) Three-dimensional reconstruction of liver (pink) and spleen (orange) was provided using IQQA-Liver CT Imaging Analysis System during the portal phase after injection of contrast material. The volume of liver and spleen was calculated as 2003.2 cm3 and 825.7 cm3, respectively. The sagittal (b), coronal (c), and transverse views (d) are shown.

Fig. 3. A 49-year-old man with POEMS syndrome before and after autologous hematopoietic stem cell transplantation (auto-HSCT). (a) Enlarged lymph nodes in the bilateral armpits before auto-HSCT; (b) enlarged hepatospleen and small bilateral pleural effusion before auto-HSCT; (c) lymph nodes 3 months after auto-HSCT; (d) hepatospleen and lung 3 months after auto-HSCT (with arrows).



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Fig. 4. A 64-year-old man with POEMS syndrome who had paresthesia and weakness. Sclerotic bone lesions in the thoracic vertebrae were displayed on bone window (with arrows).

Fig. 5. A 32-year-old man with POEMS syndrome who complained of dyspnea and constant bone pain. Lytic bone lesions in the ribs were shown on (a) mediastinum window and (b) bone window (with arrows). Small to moderate pleural effusion is also shown bilaterally.

(3,5,19), but was 87.5% when assessed by CT in the current study. By ultrasonography ascites were reported in 39.6% patients from a Chinese report (5) and were detected in 44.4% patients in this study, but ascites were detected by CT in 54.2% patients in the current study. The differences between detection rates by ultrasonography and by CT are probably due to the small volume of effusion which can only be detected by more sensitive CT. Moreover, CT also exhibited higher sensitivity than chest X-ray in detecting pleural effusion with a detection rate of 79.2% to 55.5%. Multiple serous cavities were involved suggesting that the etiological factors are from the whole body but not the local lesions. Microvascular hyperpermeability induced by elevated VEGF, which has become one of the new criteria for the diagnosis of POEMS syndrome (19), may be a causative factor of the extravascular volume overload, according to the literature (6,7,20). Increased immune activation and inflammatory status could also contribute to the pathogenesis of extravascular volume overload (5). Organomegaly is one of the five characteristics by the acronym definition of POEMS syndrome. The incidence of organomegaly (91.7%) in the current study was slightly higher than that from clinical and ultrasound evidence previously (range, 45–85%) (21). The increased detection rate may result from the superiority of CT to

ultrasonography in detecting the deep enlarged lymph nodes including mediastinal, and retroperitoneal lymph nodes. The extent of hepatosplenomegaly in POEMS was mild-to-moderate with the characteristics of homogeneity. Overproduction of VEGF is considered to be responsible for the hepatosplenomegaly (22), which can be regarded as a kind of tissue edema. Whether there are the same Castleman-like pathological change in the liver and spleen as in the bone marrow and lymph node is not confirmed. Enlargement of lymph nodes is disseminated and develops almost at any part of body. The lymph node histopathology displays Castleman-like change with the hyperplasia of lymphocytes and plasmacytes, which is one of the new major criteria for POEMS syndrome (19). Typical osteosclerotic or mixed lesions have become one of the new major diagnostic criteria for POEMS syndrome (1,2,19). The finding of an osteosclerotic lesion highly suggests the diagnosis of POEMS. In this study, 33.3% patients were found with bone lesions, while the reports from the Western countries showed bone lesions occur in approximately 95% patients (19). Two reports from China showed bone lesions were detected in 27% patients (4) and 41% patients (23). The disparities in the rates may originate from race difference between the Eastern and Western (4,12,23). Chest/abdomen CT is sensitive in detecting the small



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lesions in the vertebrae, which often are missed by conventional X-ray (24). On CT, osteosclerotic lesions took a high-density plaque-like appearance and osteolytic lesions exhibited punched-out low-density change, consistent with the previous reports (10,12,19), while osteolytic lesions with a sclerotic rim or lesions with a mixed soapbubble appearance described by previous literature (19) were not found in the current study. The majority of bone lesions were found multiple, while focal solitary sclerotic lesions were also present in the minority of the patients, which must be differentiated from other disorders (19). The overwhelming majority of bone lesions were sclerotic or mixed, while only a few were lytic, consistent with the literature (19,24). Pain and fractures associated with the osteosclerotic lesions in POEMS syndrome are rare. CT findings contributed not only to the diagnosis of POEMS syndrome, but also to the assessment of therapeutic results. In conclusion, when the combination of above CT abnormalities exists, POEMS syndrome should be highly suspected by radiologists. The three minor diagnostic criteria for POEMS syndrome: extravascular volume overload, organomegaly, and bone lesions confirmed by CT help to establish the final diagnosis of it. Acknowledgements The authors thank Chenchen Hua for his help in 3D-reconstruction. We also feel indebted to Xianqiu Yu, Lixia Wang, Qin Zhuang, Rong Ba, Yilong Lu, and Xiaoming Fei for their invaluable contributions.

Conflict of interest None declared.

Funding This work was supported by the Science and Technology Commission of Zhenjiang Municipality [SH2012029]; the National Natural Science Foundation of China [81270594]; and the China Postdoctoral Science Foundation [2013M541528].

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