Modern Rheumatology
ISSN: 1439-7595 (Print) 1439-7609 (Online) Journal homepage: http://www.tandfonline.com/loi/imor20
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report Ryu Terauchi, Toshiharu Shirai, Naoki Mizoshiri, Eiichi Konishi, Keiichiro Ueshima, Hiroyoshi Fujiwara & Toshikazu Kubo To cite this article: Ryu Terauchi, Toshiharu Shirai, Naoki Mizoshiri, Eiichi Konishi, Keiichiro Ueshima, Hiroyoshi Fujiwara & Toshikazu Kubo (2014): Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report, Modern Rheumatology To link to this article: http://dx.doi.org/10.3109/14397595.2014.970341
Published online: 10 Nov 2014.
Submit your article to this journal
Article views: 24
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=imor20 Download by: [Chinese University of Hong Kong]
Date: 10 November 2015, At: 21:03
http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2014; Early Online: 1–3 © 2014 Japan College of Rheumatology DOI: 10.3109/14397595.2014.970341
CASE REPORT
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report Ryu Terauchi1, Toshiharu Shirai1,3, Naoki Mizoshiri1, Eiichi Konishi2, Keiichiro Ueshima1, Hiroyoshi Fujiwara1, and Toshikazu Kubo1 Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
1Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan, 2Department of Surgical
Pathology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan, and 3Department of Orthopaedic Surgery, Kanazawa University, Kanazawa, Japan Abstract
Keywords
We report a case of IgG4-positive inflammatory pseudotumor mimicking malignant bone tumor. Biopsy revealed no tumor cells. Surgical excision was performed and an abscess developing beneath the periosteum was observed with Streptococcus constellatus. Preoperative serum IgG4 value of 120 mg/dl normalized postoperatively to 80.6 mg/dl. It was difficult to distinguish inflammatory pseudotumor from sarcoma because it developed under the periosteum. In such cases, it is important to measure blood IgG4 values and perform tissue staining and culturing.
IgG4-related disease, Serum IgG4 value, Streptococcus constellatus, Subperiosteal inflammatory pseudotumor, Malignant bone tumor History Received 17 June 2014 Accepted 23 September 2014 Published online 3 November 2014
Introduction In many cases, inflammatory pseudotumors are formed in the lungs or the livers, and there has been an increasing focus on this type of tumor as an IgG4-related disease [1,2]. Here we report a case of IgG4-positive inflammatory pseudotumor developing under the periosteum.
Case The patient was a 66-year-old male with no remarkable medical history. He started suffering from pain in the left femur with no known cause and visited a local doctor due to difficulty in walking. The patient was referred to our hospital for examination. At an initial consultation, pressure pain in the left femur, as well as swelling accompanied with pain, was observed. Radiographs revealed dissolving cortical bone in the medial side of the left femur diaphysis, as well as a periosteal reaction in peripheral areas (Figure 1). Magnetic resonance imaging (MRI) showed an extraosseous tumor mainly at the same site with strong edematous change in peripheral soft tissue and intra-medullar areas (Figure 2). Thallium scintigraphy found strong accumulation in the early phase, mainly around the extraosseous tumor and mild accumulation in the late phase. Considering the possibility of malignant tumor, needle biopsy using computed tomography was performed. Tumor cells were not found in the microscopic examination of the specimen but inflammatory granulation tissue with a characteristic of an accumulation Correspondence to: Ryu Terauchi, MD, Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajiicho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan. Tel: ⫹ 81-75-251-5549. Fax: ⫹ 81-75-251-5841. E-mail: [email protected]
of plasma cells was observed. The patient was diagnosed with inflammatory pseudotumor. IgG4 staining in plasma cells found an IgG4/IgG-positive plasma cell proportion of 25% (Figure 3). Since the patient felt better following the biopsy and was not suffering from a tumor, we took a wait-and-see approach. However, inflammatory reaction did not become negative, and an extraosseous tumor did not disappeared on MRI. Therefore, excision was performed on the lesion for the purpose of a radical operation. An abscess was observed intraoperatively underneath the femoral periosteum, and it was immediately excised. No direct continuity was observed between the abscess and the femur bone marrow. Histopathology of the excised specimen found, in the same way as with the biopsy, inflammatory pseudotumor accompanied with an accumulation of IgG4-positive plasma cells. Tissue culture detected Streptococcus constellatus, which are attenuated bacteria. C-reactive protein was 2.96 mg/dl, which was the same as that at initial consultation; however, it normalized to 0.12 mg/dl following excision. The serum IgG4 value directly prior to the operation was high, at 120 mg/dl; however, it normalized to 80.6 postoperatively. Pain completely disappeared postoperatively, and the patient was able to walk independently. There has been no relapse observed approximately 1 year following the operation.
Discussion There have been reports of inflammatory pseudotumor accompanied with an accumulation of IgG4 in tissue, and there has been an increasing focus on this disease as an IgG4-related disease [3,4]. Usually, this type of tumor develops in the lungs or the livers, and it is believed that the cause of this pseudotumor is infection with attenuated bacteria [5]. The causal bacteria in the present case were also attenuated bacteria, namely S. constellatus [6]. These are indigenous bacteria in the oral cavity; however, the site of
Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
2
R. Terauchi et al.
Figure 1. Radiographs at the first visit showed dissolving cortical bone in the medial side of the left femur diaphysis and a periosteal reaction in peripheral areas (white arrow). An insert is an enlargement of this area.
development was beneath the periosteum, which is an extremely rare site for it to develop. Also, there have been no reports on pseudotumors developing subperiosteally [5], and the report on the present case is the first on a pseudotumor accompanied by IgG4 aggregation at the same site. If an abscess develops underneath the periosteum due to a bacterial infection, it usually develops into a cyst. However, the present case formed a solid pseudotumor which was difficult to distinguish it from a malignant bone tumor. It was considered that infection caused overexpression of IL 4, 5, 10, 13 and TGFβ mediated by Type 2 helper
Mod Rheumatol, 2014; Early Online: 1–3
Figure 2. MRI, (a) T1, (b) STIR, (c) Gd enhanced MRI revealed an extraosseous tumor mainly at the same site with strong edematous change in peripheral soft tissue and intra-medullar areas.
T cells, leading to a proliferation of acidophils and IgG4, causing the invasion of inflammatory cells and pseudotumor formation [2]. In many IgG4-related diseases, blood IgG4 values tend to be high, and it has been reported that these values are useful in distinguishing such diseases [1]. The present case was also found to have a high preoperative blood IgG4 value, of 120 mg/ dl (normal value: 105 mg/dl). The value normalized to 80 mg/dl following excision, and this can be an index for treatment effects. Although these values do not match the comprehensive IgG4RD diagnostic criteria, we believe, based on changes in preoperative hematology findings as well as pathology findings, that IgG4 has an important role in the pathology of the present case.
Figure 3. Histopathological finding of CT-guided biopsy specimen. (a) Inflamed granulation tissue with lymphoplasmacytic and neutrophilic infiltration is observed. (hematoxylin–eosin: original magnification ⫻ 120). (b) IgG-positive plasma cell infiltration. (Immunostaining of IgG: original magnification ⫻ 240). (c) IgG4-positive plasma cell infiltration in the same region. IgG4/IgG rate was 25%. (Immunostaining of IgG4: original magnification ⫻ 240).
DOI 10.3109/14397595.2014.970341
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor
Conclusion We reported a case of subperiosteal inflammatory pseudotumor that needed to be distinguished from a malignant tumor. If a subperiosteal tumor-appearing lesion is observed, it is important to consider the possibility of an inflammatory pseudotumor, which is an IgG4-related disease, not only malignant bone tumor, and to measure blood IgG4 values and perform tissue staining and culturing.
2. 3. 4.
Conflict of interest None.
5. 6.
References
Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
1. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4-related disease
3
(IgG4RD): general concept and details. Mod Rheumatol. 2011;22(1): 1–14. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51. Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36(7):710–7. Mizushima I, Yamada K, Fujii H, Inoue D, Umehara H, Yamagishi M, et al. Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis. Mod Rheumatol. 2012;22(6):859–70. Narla LD, Newman B, Spottswood SS, Narla S, Kolli R. Inflammatory pseudotumor. Radiographics. 2003;23(3):719–29. Whiley RA, Beighton D, Winstanley TG., Fraser HY, Hardie JM. Streptococcus intermedius, Streptococcus constellatus, Streptococcus anginosus (the Streptococcus milleri group): Association with different body sites and clinical infections. J Clin Microbiol. 1992; 30(1):243–4.
ISSN: 1439-7595 (Print) 1439-7609 (Online) Journal homepage: http://www.tandfonline.com/loi/imor20
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report Ryu Terauchi, Toshiharu Shirai, Naoki Mizoshiri, Eiichi Konishi, Keiichiro Ueshima, Hiroyoshi Fujiwara & Toshikazu Kubo To cite this article: Ryu Terauchi, Toshiharu Shirai, Naoki Mizoshiri, Eiichi Konishi, Keiichiro Ueshima, Hiroyoshi Fujiwara & Toshikazu Kubo (2014): Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report, Modern Rheumatology To link to this article: http://dx.doi.org/10.3109/14397595.2014.970341
Published online: 10 Nov 2014.
Submit your article to this journal
Article views: 24
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=imor20 Download by: [Chinese University of Hong Kong]
Date: 10 November 2015, At: 21:03
http://informahealthcare.com/mor ISSN 1439-7595 (print), 1439-7609 (online) Mod Rheumatol, 2014; Early Online: 1–3 © 2014 Japan College of Rheumatology DOI: 10.3109/14397595.2014.970341
CASE REPORT
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor: A case report Ryu Terauchi1, Toshiharu Shirai1,3, Naoki Mizoshiri1, Eiichi Konishi2, Keiichiro Ueshima1, Hiroyoshi Fujiwara1, and Toshikazu Kubo1 Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
1Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan, 2Department of Surgical
Pathology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan, and 3Department of Orthopaedic Surgery, Kanazawa University, Kanazawa, Japan Abstract
Keywords
We report a case of IgG4-positive inflammatory pseudotumor mimicking malignant bone tumor. Biopsy revealed no tumor cells. Surgical excision was performed and an abscess developing beneath the periosteum was observed with Streptococcus constellatus. Preoperative serum IgG4 value of 120 mg/dl normalized postoperatively to 80.6 mg/dl. It was difficult to distinguish inflammatory pseudotumor from sarcoma because it developed under the periosteum. In such cases, it is important to measure blood IgG4 values and perform tissue staining and culturing.
IgG4-related disease, Serum IgG4 value, Streptococcus constellatus, Subperiosteal inflammatory pseudotumor, Malignant bone tumor History Received 17 June 2014 Accepted 23 September 2014 Published online 3 November 2014
Introduction In many cases, inflammatory pseudotumors are formed in the lungs or the livers, and there has been an increasing focus on this type of tumor as an IgG4-related disease [1,2]. Here we report a case of IgG4-positive inflammatory pseudotumor developing under the periosteum.
Case The patient was a 66-year-old male with no remarkable medical history. He started suffering from pain in the left femur with no known cause and visited a local doctor due to difficulty in walking. The patient was referred to our hospital for examination. At an initial consultation, pressure pain in the left femur, as well as swelling accompanied with pain, was observed. Radiographs revealed dissolving cortical bone in the medial side of the left femur diaphysis, as well as a periosteal reaction in peripheral areas (Figure 1). Magnetic resonance imaging (MRI) showed an extraosseous tumor mainly at the same site with strong edematous change in peripheral soft tissue and intra-medullar areas (Figure 2). Thallium scintigraphy found strong accumulation in the early phase, mainly around the extraosseous tumor and mild accumulation in the late phase. Considering the possibility of malignant tumor, needle biopsy using computed tomography was performed. Tumor cells were not found in the microscopic examination of the specimen but inflammatory granulation tissue with a characteristic of an accumulation Correspondence to: Ryu Terauchi, MD, Department of Orthopaedics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, 465 Kajiicho, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan. Tel: ⫹ 81-75-251-5549. Fax: ⫹ 81-75-251-5841. E-mail: [email protected]
of plasma cells was observed. The patient was diagnosed with inflammatory pseudotumor. IgG4 staining in plasma cells found an IgG4/IgG-positive plasma cell proportion of 25% (Figure 3). Since the patient felt better following the biopsy and was not suffering from a tumor, we took a wait-and-see approach. However, inflammatory reaction did not become negative, and an extraosseous tumor did not disappeared on MRI. Therefore, excision was performed on the lesion for the purpose of a radical operation. An abscess was observed intraoperatively underneath the femoral periosteum, and it was immediately excised. No direct continuity was observed between the abscess and the femur bone marrow. Histopathology of the excised specimen found, in the same way as with the biopsy, inflammatory pseudotumor accompanied with an accumulation of IgG4-positive plasma cells. Tissue culture detected Streptococcus constellatus, which are attenuated bacteria. C-reactive protein was 2.96 mg/dl, which was the same as that at initial consultation; however, it normalized to 0.12 mg/dl following excision. The serum IgG4 value directly prior to the operation was high, at 120 mg/dl; however, it normalized to 80.6 postoperatively. Pain completely disappeared postoperatively, and the patient was able to walk independently. There has been no relapse observed approximately 1 year following the operation.
Discussion There have been reports of inflammatory pseudotumor accompanied with an accumulation of IgG4 in tissue, and there has been an increasing focus on this disease as an IgG4-related disease [3,4]. Usually, this type of tumor develops in the lungs or the livers, and it is believed that the cause of this pseudotumor is infection with attenuated bacteria [5]. The causal bacteria in the present case were also attenuated bacteria, namely S. constellatus [6]. These are indigenous bacteria in the oral cavity; however, the site of
Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
2
R. Terauchi et al.
Figure 1. Radiographs at the first visit showed dissolving cortical bone in the medial side of the left femur diaphysis and a periosteal reaction in peripheral areas (white arrow). An insert is an enlargement of this area.
development was beneath the periosteum, which is an extremely rare site for it to develop. Also, there have been no reports on pseudotumors developing subperiosteally [5], and the report on the present case is the first on a pseudotumor accompanied by IgG4 aggregation at the same site. If an abscess develops underneath the periosteum due to a bacterial infection, it usually develops into a cyst. However, the present case formed a solid pseudotumor which was difficult to distinguish it from a malignant bone tumor. It was considered that infection caused overexpression of IL 4, 5, 10, 13 and TGFβ mediated by Type 2 helper
Mod Rheumatol, 2014; Early Online: 1–3
Figure 2. MRI, (a) T1, (b) STIR, (c) Gd enhanced MRI revealed an extraosseous tumor mainly at the same site with strong edematous change in peripheral soft tissue and intra-medullar areas.
T cells, leading to a proliferation of acidophils and IgG4, causing the invasion of inflammatory cells and pseudotumor formation [2]. In many IgG4-related diseases, blood IgG4 values tend to be high, and it has been reported that these values are useful in distinguishing such diseases [1]. The present case was also found to have a high preoperative blood IgG4 value, of 120 mg/ dl (normal value: 105 mg/dl). The value normalized to 80 mg/dl following excision, and this can be an index for treatment effects. Although these values do not match the comprehensive IgG4RD diagnostic criteria, we believe, based on changes in preoperative hematology findings as well as pathology findings, that IgG4 has an important role in the pathology of the present case.
Figure 3. Histopathological finding of CT-guided biopsy specimen. (a) Inflamed granulation tissue with lymphoplasmacytic and neutrophilic infiltration is observed. (hematoxylin–eosin: original magnification ⫻ 120). (b) IgG-positive plasma cell infiltration. (Immunostaining of IgG: original magnification ⫻ 240). (c) IgG4-positive plasma cell infiltration in the same region. IgG4/IgG rate was 25%. (Immunostaining of IgG4: original magnification ⫻ 240).
DOI 10.3109/14397595.2014.970341
Subperiosteal inflammatory pseudotumor mimicking primary malignant bone tumor
Conclusion We reported a case of subperiosteal inflammatory pseudotumor that needed to be distinguished from a malignant tumor. If a subperiosteal tumor-appearing lesion is observed, it is important to consider the possibility of an inflammatory pseudotumor, which is an IgG4-related disease, not only malignant bone tumor, and to measure blood IgG4 values and perform tissue staining and culturing.
2. 3. 4.
Conflict of interest None.
5. 6.
References
Downloaded by [Chinese University of Hong Kong] at 21:03 10 November 2015
1. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4-related disease
3
(IgG4RD): general concept and details. Mod Rheumatol. 2011;22(1): 1–14. Stone JH, Zen Y, Deshpande V. IgG4-related disease. N Engl J Med. 2012;366(6):539–51. Zen Y, Kitagawa S, Minato H, Kurumaya H, Katayanagi K, Masuda S, et al. IgG4-positive plasma cells in inflammatory pseudotumor (plasma cell granuloma) of the lung. Hum Pathol. 2005;36(7):710–7. Mizushima I, Yamada K, Fujii H, Inoue D, Umehara H, Yamagishi M, et al. Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis. Mod Rheumatol. 2012;22(6):859–70. Narla LD, Newman B, Spottswood SS, Narla S, Kolli R. Inflammatory pseudotumor. Radiographics. 2003;23(3):719–29. Whiley RA, Beighton D, Winstanley TG., Fraser HY, Hardie JM. Streptococcus intermedius, Streptococcus constellatus, Streptococcus anginosus (the Streptococcus milleri group): Association with different body sites and clinical infections. J Clin Microbiol. 1992; 30(1):243–4.
