Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report

Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report

Journal of Orthopaedic Science xxx (2016) 1e4 Contents lists available at ScienceDirect Journal of Orthopaedic Science journal homepage: http://www...

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Journal of Orthopaedic Science xxx (2016) 1e4

Contents lists available at ScienceDirect

Journal of Orthopaedic Science journal homepage: http://www.elsevier.com/locate/jos

Case Report

Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report Yu Toda a, Motoki Sonohata a, *, Kazuyoshi Uchihashi c, Hiroko Mine b, Toshiyuki Tsuruta b, Masaaki Mawatari a a b c

Departmet of Orthopaedic Surgery, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan Tsuruta Orthopaedic Clinic, 1241-6, Ushidu, Saga 849-0306, Japan Departmet of Pathology, Faculty of Medicine, Saga University, Japan

a r t i c l e i n f o Article history: Received 2 March 2016 Received in revised form 2 August 2016 Accepted 5 August 2016 Available online xxx

1. Introduction The clinical manifestations of calcium pyrophosphate dehydrate crystal deposition disease (CPPD) vary widely. Many cases are found incidentally and may be asymptomatic [1]. In symptomatic patients, the lesions may be misdiagnosed as pseudorheumatoid arthritis, pseudoosteoarthritis or pseudoneuropathic joints [2,3]. CPPD is defined as a joint disease that is characterized by the presence of calcium pyrophosphate dehydrate crystals in the intraarticular and periarticular tissue [4,5]. CPPD is divided into “diffuse or non-tumoral deposition CPPD” and “massive or tumoral CPPD”. Non-tumoral CPPD is easily diagnosed by an X-ray, however, it is often difficult to make a proper diagnosis for tumoral CPPD because tumoral CPPD is the rarest clinical form of CPPD deposition. We herein report the case of a unique clinical form of tumoral CPPD in the elbow. The study protocol adhered to the ethical guidelines of the 1975 Declaration of Helsinki, and the study was approved by the institutional review board of Tsuruta Orthopaedic Clinic. The patient was informed that this case study would be submitted for publication, and she provided her informed consent.

* Corresponding author. Fax: þ81 952 34 2509. E-mail address: [email protected] (M. Sonohata).

2. Case report A 77-year-old female had been diagnosed with elbow osteoarthritis at another hospital 8 years prior to admission to our hospital. Radiographs of her elbow taken at another hospital showed a slight degree of osteoarthritis with some calcareous depositions in the elbow joint (Fig. 1). She subsequently underwent an operation on her right elbow at the previous hospital (details unknown). After the operation, the pain in her right elbow improved for a few years. However, she began to notice a limited range of motion in her right elbow six years after the operation, and gradually felt pain and swelling in her right elbow. She presented to our clinic with a chief complaint of right elbow pain and swelling. The preliminary examination at the first visit to our clinic revealed swelling and pain at the right elbow. The range of motion of the right elbow was markedly decreased. The ranges of flexion/extension of the elbow motion and pronation/supination of the forearm rotation were restricted to 90 /40 and 10 /10 , respectively. There were no subjective or objective symptoms or disorders at any other joints. The ulnar nerve disturbance was not recognized. The patient had no family history of CPPD, and no history of CPPD in other joint. The patient had no history of kidney or thyroid gland disease, and the laboratory findings, including the alkaline phosphatase and calcium levels, were within normal limits. Radiographs of the elbow at the first visit to our clinic showed a massive mass involving the entire proximal radius, proximal ulna, and adjacent soft tissues. However, the humerus was intact. A welldefined, punctate lesion of calcification was noted in the mass. Some free bodies were observed on the anterior side of the elbow (Fig. 2). Computed tomography (CT) showed sclerotic lesions around the coronoid process of the ulna and proximal radius. Additionally, a lucent rim was observed near the lesion of the ulna and radius (Fig. 3). Magnetic resonance imaging (MRI) confirmed the presence of a space occupied lesion around the coronoid process of the ulna and proximal radius with a low-isointensity heterogeneous signal on T1-weighted and fat-saturated T2-weighted images. The patient underwent surgical mobilization of the right elbow and resection of the calcification under general anesthesia. We

http://dx.doi.org/10.1016/j.jos.2016.08.004 0949-2658/© 2016 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.

Please cite this article in press as: Toda Y, et al., Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report, Journal of Orthopaedic Science (2016), http://dx.doi.org/10.1016/j.jos.2016.08.004

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Fig. 1. Anteroposterior (A) and lateral (B) roentgenograms of the right elbow at 8 years before our operation show osteoarthritis with calcareous depositions on the anterior side of the elbow joint.

Fig. 2. Anteroposterior (A) and lateral (B) roentgenograms before our operation show a tumoral mass in the soft tissues and invading bone. A radio-opaque mass with a fluffy pattern is observed.

initially used a posterolateral approach between the anconeus and extensor carpi ulna muscles. The posterior capsule calcification was removed. The surface of the radial head and capitellum had adopted a milky white hue, due to calcium deposition. Additionally, the inside of the radial head had been filled with a chalky-white materials. As a result, we resected the radial head. The calcification on the radial side of ulna was curetted, and the cortex of radial wall of the ulna was preserved as far as possible. We then employed a medial approach. The ulna nerve was released and retracted, and the posterior oblique ligament and medial osteophytes, which had suffered from calcium deposition, were resected, while the anterior oblique ligament was preserved. Instability of the elbow was not observed after surgery. The anterior capsule was released. A milky-

white mass consisting of crystals was recognized to extend from the intra capsule to the extra capsule. This mass was entirely removed. Finally, autologous bone from the ilium and artificial bone were grafted to the cavity of the proximal ulnar. Following the operation, ROM exercise was performed after 2 weeks fixation by long arm cast. Grossly the tumor contained numerous chalky materials (Fig. 4A). Microscopically, sections showed abundant calcification and rhomboid-shaped crystals that revealed positive birefringence by polarized microscopy (Fig. 4B). The feature was compatible with CPPD. The patient was followed up for 4 years after the operation. At the last follow-up, the range of motion of the right elbow had

Please cite this article in press as: Toda Y, et al., Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report, Journal of Orthopaedic Science (2016), http://dx.doi.org/10.1016/j.jos.2016.08.004

Y. Toda et al. / Journal of Orthopaedic Science xxx (2016) 1e4

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Fig. 3. Computed tomography (CT) shows sclerotic lesions invading into ulna (A) and radius (B) with sharp margins.

Fig. 4. Excised specimen. (A) Macroscopically, the tumor contains numerous chalky-white materials. (B) Polarized microscopy shows crystals with positive birefringence (arrow heads).

improved, with ranges of flexion/extension of the elbow motion and pronation/supination of the forearm rotation restricted to 105 /15 and 65 /70 , respectively. The radiographs showed no sign of recurrence and remodeling of the grafted bone was observed (Fig. 5). 3. Discussion CPPD is a benign crystalline arthropathy of unknown etiology. It is typically a monoarticular condition characterized by crystal deposition of calcium pyrophosphate in synovial membranes and joint cartilages [4]. Tumoral CPPD as a form of CPPD is rarely diagnosed in clinical practice. Yamakawa et al. reported 5 cases of tumoral CPPD and reviewed 54cases of CPPD. However, many case reports of tumoral CPPD have appeared as single case reports [6]. CPPD occurs most commonly on the knee or wrist joint, however, the most common anatomic location of tumoral CPPD is the temporomandibular joint, followed by the cervical spine and hand [3]. Other less common locations included the hip, toe, wrist, shoulder, elbow, and parotid gland [6]. Involvement of the adjacent bone was noted in 38% patients [6], and the most common pattern of bony involvement of tumoral

CPPD was pressure erosion [7]. In the current case, huge pressure erosion into the ulna and radius was observed in the radiographs. To the best of our knowledge, there are no previous reports of tumoral CPPD with such massive involvement of the adjacent bone. Unfortunately, the detail of the patient's course following surgery at another hospital were unknown. However, the CPPD may have been a disseminated aberrant lesion caused by handling from the previous procedures. Additionally, the lesions in the radius and ulnar had grown a great deal over the previous 8 years, while the growth of the periarticular lesion was very slow and limited. Both lesions had possibility to be other pathological findings, however, the lesion of periarticular was not pathologically examined. Tumoral CPPD should be differentiated from tumoral calcinosis, hydroxyapatite deposition disease (including calcium pyrophosphate dehydrate crystal deposition disease or calcium hydroxyapatite crystal deposition disease), and malignant disease [8]. The clinical and radiographic features of reported patients with tumoral CPPD initially led to a provisional diagnosis of malignant tumors in 14 patients. Seven of these cases were diagnosed as having - or suspected of being - chondrosarcomas [7]. In some patients, the preoperative misdiagnosis resulted in more radical surgery than was appropriate.

Please cite this article in press as: Toda Y, et al., Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report, Journal of Orthopaedic Science (2016), http://dx.doi.org/10.1016/j.jos.2016.08.004

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Y. Toda et al. / Journal of Orthopaedic Science xxx (2016) 1e4

Fig. 5. Anteroposterior (A) and lateral (B) roentgenograms at the last follow-up show remodeling of the grafted autologous and artificial bones.

Synovial chondromatosis is another type of lesion that can be confused with tumoral CPPD, especially cases in which a heavily calcified cartilage matrix may obscure their cartilaginous nature [9]. However, the calcified areas of synovial chondromatosis do not contain rhomboid crystals, which are characteristic of CPPD deposition. Tumoral CPPD may recur after complete or incomplete surgical excision. Six cases recurring from 11 months to 6 years after surgery have been reported [10]. Multiple recurrences (three times) were noted in one patient [10]. Fortunately, there is no report of malignant change of recurrent tumoral CPPD, however, recurrence of tumoral CPPD can aggravate the joint function. We have thus far conducted only 4 years of follow-up examination after the operation, and a further long-term follow-up study is thus warranted. 4. Conclusions Our report described a case of massive CPPD of the proximal ulna and proximal radius. The clinical manifestations of CPPD and tumoral CPPD vary widely, and it is challenging to make a proper diagnosis of tumoral CPPD due to its infrequent incidence. A greater accumulation of case reports of tumoral CPPD is needed to establish the diagnostic criteria and define disease characteristics. The present case illustrates the importance of considering another type of tumoral CPPD from the previously reported tumoral CPPD types.

Conflict of interest The authors declare that they have no conflict of interest. References [1] Resnick D, Niwayama G. Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease. In: Resnick D, Niwayama G, editors. Diagnosis of bone and joint disorders. 2nd ed. Philadelphia: Saunders; 1998. p. 1672e732. [2] McCarty D. Crystals, joints, and consternation. Ann Rheum Dis 1983 Jun;42(3): 243e53. [3] McCarty DJ. Calcium pyrophosphate dehydrate crystal deposition disease1975. Arthritis Rheum Dis 1976 May-Jun;19(Suppl. 3):275e85. [4] Kishore B, Khare P, Gupta RJ, Gupta C, Khare V. Tumoral calcium pyrophosphate dihydrate crystal deposition disease: a rare diagnosis by fine-needle aspiration. Diagn Cytopathol 2010 Jan;38(1):47e50. [5] Meul B, Ernestus K, Neugebauer J, Kuebler AC. A case of chronic calcium pyrophosphate dehydrate crystal disease in the temoporomandibulara joint. Oral Dis 2005 Mar;11(2):113e5. [6] Yamakawa K, Iwasaki H, Ohjimi Y, Kikuchi M, Iwashita A, Isayama T, Naito M. Tumoral calcium pyrophosphate dihydrate crystal deposition disease. A clinicopathologic analysis of five cases. Pathol Res Pract 2001;197(7):499e506. [7] Ishida T, Dorfman HD, Bullough PG. Tophaceous pseudogout (tumoral calcium pyrophosphate dihydrate crystal deposition disease). Hum Pathol 1995 Jun;26(6):587e93. [8] Resnick CS, Resnick D. Crystal deposition disease. Semin Arthritis Rheum 1983 May;12(4):390e403. [9] Lagier R. Case report 354: chondroma of soft tissues with dystrophic calcification. Skelet Radiol 1986;15(2):179e83. [10] Ling D, Murphy WA, Kyriakos M. Tophaceous pseudogout. Am J Roentgenol 1982 Jan;138(1):162e5.

Please cite this article in press as: Toda Y, et al., Massive osteolysis in the proximal radius and ulna due to calcium pyrophosphate dehydrate deposition disease: A case report, Journal of Orthopaedic Science (2016), http://dx.doi.org/10.1016/j.jos.2016.08.004