Joint Bone Spine 73 (2006) 202–204 http://france.elsevier.com/direct/BONSOI/
Case report
Multiple parosteal lipoma associated to polyarthritis José Luis Fernández-Sueiro a,*, José Antonio Pinto a, Francisco J. Blanco a, Mercedes Freire a, José A. Veiga b, Fausto Galdo a, Miguel A. González-Gay c a
Division of Rheumatology, Hospital CHU Juan Canalejo, c) Xubias de Arriba no. 84, 15006 La Coruña, Spain b Division of Pathology, Hospital CHU Juan Canalejo, La Coruña, Spain c Division of Rheumatology, Xeral-Calde, Lugo, Spain Received 9 March 2004; accepted 18 March 2005 Available online 08 June 2005
Abstract Parosteal lipoma is a benign adipose tissue tumor situated directly in the bone cortex. All cases previously reported in the literature were described as solitary lesions. We describe a patient who presented with polyarthritis associated with multiple parosteal lipomatous involvement. A tissue sample from the distal portion of the forearm confirmed the presence of cumulative fat tissue with nodes of esteatonecrosis. To the best of our knowledge this is the first case of multiple parosteal lipoma associated to polyarthritis. © 2005 Elsevier SAS. All rights reserved. Keywords: Multiple parosteal lipoma; Polyarthritis; Periostic reaction; Benign neoplasm bone; Magnetic resonance imaging
1. Introduction Parosteal lipoma is a benign adipose tissue tumor situated directly in the bone cortex. It is an unusual neoplasm that appears to emerge from multidirectional mesenchymal “modulation” within the periosteum [1]. To the best of our knowledge, all cases previously reported in the literature were described as solitary lesions. We describe a patient who presented with polyarthritis associated with multiple parosteal lipomatous involvement.
2. Case report A 52-year-old man was sent to the hospital because of a 6-month-history of pain and swelling in his wrists, hands, ankles and feet, without morning stiffness. The patient medical history included smoking (20 cigarettes per day), a prostatectomy at the age of 47 due to prostatic adenoma and painless soft tissue swelling on distal forearms, fingers and calves. On physical examination there was swelling warmth and ten* Corresponding author. Tel.: +34 981 17 8149/8272; fax: +34 981 17 8273. E-mail address:
[email protected] (J.L. Fernández-Sueiro). 1297-319X/$ - see front matter © 2005 Elsevier SAS. All rights reserved. doi:10.1016/j.jbspin.2005.03.002
derness in wrists and ankles, and soft tissue swelling in the distal portion of forearms, calves and dorsum of the phalanges of the fingers of both hands (Fig. 1A, B). Full blood cell count, erythrocyte sedimentation rate, C-reactive protein and routine blood and urine biochemistry profile were normal. The rheumatoid factor and antinuclear antibodies were negative. Skin tuberculin test was also negative. Chest radiograph was normal. A radiograph disclosed a radiolucent mass around the distal ulnar epiphysis in both wrists. However, bone erosions were not found. A 99mtechnetium bone scan revealed light increased isotope uptake in both wrists. Magnetic resonance images (MRI) of both wrists showed a well-delimited polylobulated neoformation with the same signal as subcutaneous fat and minimal periostic reaction of the ulnar bone (Fig. 2A). No erosions within carpal bones were seen. Computed tomography (CT) suggested the presence of fatty masses around the distal ulnar epiphysis and the dorsal area of the distal radius epiphysis as well as a periostic reaction of the distal ulnar and radius epiphysis in both hands, with minimal joint effusion close to the radius and trapezium of the left hand (Fig. 2B). A tissue sample from the distal portion of the forearm confirmed the presence of cumulative fat tissue with nodes of esteatonecrosis (Fig. 3). The patient was diagnosed of multiple parosteal lipoma and polyarthritis being treated with NSAIDs to control joint symptoms, however, due to
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Fig. 3. Low power view of the excised specimen showing a predominantly adipose tissue.
3. Discussion
Fig. 1. View of soft tissue swelling in writs, dorsal view (A) and lateral view (B).
increased pain the patient was started on low dose prednisone (10 mg daily) with poorly symptoms control. On follow up for more than 6 years the patient goes on with an asymmetric polyarthritis as confirmed by 99mtechnetium bone scan and MRI (data not showed) which could not be attributed to any known rheumatic condition such as rheumatoid arthritis, spondyloarthritis or granulomatous disease.
Parosteal lipoma is a benign adipose tissue tumor situated directly in the bone cortex. It is an unusual neoplasm that appears to emerge from multidirectional mesenchymal “modulation” within the periosteum. To the best of our knowledge, all cases previously reported in the literature were described as solitary lesions. However, in our case a multiple parosteal lipomatous involvement was associated with seronegative polyarthritis [1]. Lipoma is one of the most common soft tissue tumors. Its osseous location is rather unusual (0.3%). Three types have been described according to their relationship to bony structures, namely; intraosseous, cortical or parosteal. Parosteal lipoma [1] is situated directly on the bone cortex. It is thought to arise from mesenchymal cells in the periosteum [2]. Parosteal lipoma share histopathologic features with the com-
Fig. 2. T1-weighted MRI of the patient right wrist, showing (arrows) a fat mass around the ulnar bone (A). Direct transversal CT image of the patient left wrist, showing periostic reaction (B) in ulnar and radial bone (arrows).
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monly occurring soft tissue lipomas, and cytogenetic evidence suggest a common histopathogenesis [3]. According to the grade of chondroid modulation and enchondral ossification [4], parosteal lipomas may rest directly on the cortex without cartilage or bone elements; may have a narrow bony stalk with a lucent lipomatous cap, mimicking a pedunculated exostosis [5]; have a densely ossified broad-based osteochondromatous element beneath a thin lipomatous cap, simulating a sessile exostosis; or patches of chondroid and bone scattered throughout the lipomatous mass. The lesion is solitary and preferably affects the metaphysis of the long bones (femur, tibia and humerus). The tumor is mostly asymptomatic and it does not have any preference according to age or sex. It presents as a slowly growing mass with an average of 8–10 years of evolution. Clinical symptoms are related to compression of adjacent neurovascular and muscular structures and depend on the size and location of the lesions and cause motor and sensory function deficits [6]. Common sites of involvement include the proximal forearm and sciatic nerve. On plain radiographs parosteal lipomas present as well defined areas of lucency located adjacent to a long bone. Bone destruction is absent. Radiologic evaluation, including CT [7] and MRI [8,9], is essential to evaluate the localization and nature of the lesion. Both techniques are the methods of choice for diagnosis of osseous and non-osseous lipomas, showing a homogeneous lobulated appearance and are adherent to the surface of the adjacent bone. On MRI the image presents as high signal intensity on T1-WI, intermediate on SI or T2-WI and they loose signal on fat suppressed sequences. They do not enhance after intravenous gadolinium injection. MRI imaging may show low-signal-intensity strands in the lesion, corresponding to fibrovascular strands that are commonly found in lipomatous lesions. Bone reactive changes resemble osteochondromatous exostosis but they lack contiguity of adjacent cortex and medulla as described in osteochodromas. Differential diagnosis with low-grade liposarcoma can be difficult but neither primary parosteal liposarcoma nor degenerated benign parosteal liposarcoma has been described in patients with these tumors. Parosteal lipomas are treated surgically when there are involved vascular or neurologic structures; no malignant potential has been reported.
Although parosteal lipoma is an extremely rare benign primary neoplasm of bone, the occurrence of polyarthritis in our case was closely associated to the presence of the multiple parosteal lipoma. Even though pain over subcutaneous lipomas has been described such in Dercum’s disease [10], parosteal lipomas characteristically are painless. Pain over the joints is usually considered to be of a mechanical origin [10], however, as in the present case it seems that arthritis can develop as well. Even though this feature has not been described as well as multiple parosteal lipoma [11], perhaps we might hypothesized that the strong periostic reaction closed to the joints, may induce that inflammatory cytokines reach the joint provoking inflammatory arthritis. To the best of our knowledge this is the first case of an association between this tumor and polyarthritis.
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