Clin Rheumatol (2007) 26: 1142–1144 DOI 10.1007/s10067-006-0269-3
CASE REPORT
Gulgun Yilmaz Ovali . Serdar Tarhan . Selim Serter . Petek Bayindir . Guvenir Okcu . Peyker Demireli . Yuksel Pabuscu
A rare disorder: idiopathic tumoral calcinosis
Received: 21 January 2006 / Revised: 2 February 2006 / Accepted: 2 February 2006 / Published online: 25 March 2006 # Clinical Rheumatology 2006
Keywords Hip . MRI . Tumoral calcinosis
Introduction Tumoral calcinosis is an uncommon disorder characterized with the deposition of calcium and crystals near the large joints. The depositions are presented as calcified soft-tissue masses that are commonly observed around the hips, shoulders, elbows, feet, and buttocks [1]. Epidemiology and etiology of tumoral calcinosis are still under discussion [2]. Renal failure, genetic disorders, and recurrent softtissue microtrauma are considered among the major causes. Idiopathic cases as in our case may occur rarely. The x-ray and MRI findings of a idiopathic tumoral calcinosis case located on right hip are presented.
Case report A 23-year-old woman was admitted to our hospital with a soft-tissue mass on right hip. According to the medical history, she had been operated for similar soft-tissue masses on her left hip and right elbow and had the diagnosis as tumoral calcinosis. Neither family inheritance nor history of dialysis was present. G. Y. Ovali (*) . S. Tarhan . S. Serter . P. Bayindir . Y. Pabuscu Radiology Department, Medical Faculty, Celal Bayar University, 45010 Manisa, Turkey e-mail:
[email protected] Tel.: +90-236-2349070 Fax: +90-236-2370213 G. Okcu Orthopedics Department, Medical Faculty, Celal Bayar University, Manisa, Turkey P. Demireli Pathology Department, Medical Faculty, Celal Bayar University, Manisa, Turkey
Physical examination revealed a swelling on right hip without a change of color or difference in warmth with the range of motion being normal. All of the laboratory findings were normal. No abnormality was detected on left hip and right elbow where she had been operated formerly. Multiple high-density opacities consistent with softtissue calcifications were detected on plain radiographs of the right hip (Fig. 1). MRI demonstrated the extension of the soft-tissue mass from the proximal thigh into the quadriceps musculature. There were no erosions of the femur and iliac bone. The mass lesion was hypointense on T1W images, while it was heterogenously hyperintense with cystic portions on T2W images (Fig. 2). Multiple fluid–fluid levels in cystic parts of the mass lesion were detected on T2W and fat-saturated T2W images. The inferior part of the level which was hypointense represented the calcium component (Fig. 3). The patient was diagnosed with tumoral calcinosis regarding the pathology. On gross pathological examination, nodular calcific yellow-colored material was defined in soft tissue. Histologic findings showed the osteoclastlike giant cells, histiocytes, and wide fibrosis areas (Figs. 4 and 5).
Discussion Tumoral calcinosis is characterized with the development of calcified masses within the soft tissues near the large joints [1]. Only clinical and radiographic findings are universally congruous, whereas the epidemiology and etiology are still under discussion, according to the literature [2]. Renal failure, genetic disorders, and recurrent soft-tissue microtrauma are considered among the major causes. Idiopathic cases as in our case may occur rarely. Blacks are affected more often than whites [3, 4]. Tumoral calcinosis usually appears to be a painless mass lesion as presented in our case. The depositions that are presented as calcified soft-tissue masses are commonly observed around the hips, shoulders, elbows, feet, and buttocks and initiates in the second
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Fig. 4 The macroscopic appearance of excision material Fig. 1 The plain radiographs of the right hip reveals multiple highdensity opacities consistent with soft-tissue calcifications
Fig. 2 On T1-weighted axial MRI, the lesion shows hypointense signal intensity
ta, milk-alkali syndrome, calcifications due to chronic renal failure, and primary hyperparathyroidism of which who have significant laboratory findings and histories [5, 6]. The fluid levels and MRI signal characteristics were described by Smeets et al., Senol et al., Martinez et al. in different locations [5–7]. The appearance of level formation was described as ‘sedimentation sign’ according to their reports. Sedimentation sign is the dependent layer which is seen as hypointense due to the calcium ingredient. Although the fluid levels on MRI are not specific findings for tumoral calcinosis, the levels seen on other pathologies as aneurysmal bone cyst, chondroblastoma, osteosarcoma, giant cell tumor, fibrous dysplasia, osteoblastoma, malignant fibrous histiocytoma, and malignant schwannoma are not calcium sedimentation; hence, they are the degradation of blood products [5–8]. As a result, complementary to plain films, the demonstration of sedimentation sign on MRI is very useful for the diagnosis of tumoral calcinosis.
Fig. 3 On T2-weighted coronal image, the cystic parts of the mass lesion, the fluid levels, and the ‘sedimentation sign’ are demonstrated
decade of life. The initiation age for our patient was 12, and the first lesion was detected on her left hip. The radiographic appearance of tumoral calcinosis typically appears to be a conglomerate of cloudy multiple, rounded dense opacities, some of which show levels [2–4]. There are many reports describing the plain film findings in the literature [4, 5]. Differential diagnoses of these plain film findings include calcinosis universalis or circumscrip-
Fig. 5 Giant cells, histiocytes, and fibrosis around the nodular calcific material. H & E, ×40
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5. Smeets HGW, Lamers RJS, Sastrowijoto SH (1996) Tumoral calcinosis. AJR 167:81–89 6. Senol U, Karaal K, Cevikol C, Dincer A (2000) MR imaging findings of recurrent tumoral calcinosis. Clin Imag 24:154–156 7. Martinez S, Vogler JB III, Harrelson JM, Lyles KW (1990) Imaging of tumoral calcinosis: new observations. Radiology 174:215–222 8. Vilanova JC, Dolz JL, Maestro de Leon JL, Aparicio A, Aldoma ÁJ, Capdevila A (1998) MR imaging of a malignant schwannoma and an osteoblastoma with fluid–fluid levels. Report of two new cases. Eur Radiol 8:1359–1362
