IMAGING OF TUMORAL CALCINOSIS 

I. CHERIF, M. CHELLI BOUAZIZ, M. ATTIA, MF. LADEBDEPARTMENT OF RADIOLOGY, INSTITUT MT KASSAB D’ORTHOPÉDIE,

KSAR SAÏD, TUNISIA.

MK 22

INTRODUCTION

Idiopathic tumoral calcinosis is an unusual benign condition characterized by the presence of calcified soft tissue masses of varying size around the joints

The purpose of our study is to describe the imaging findings in idiopathic tumoral calcinosis with emphasis on MR imaging

PATIENTS AND METHODS

We retrospectively reviewed nine patients with histopathologically proven tumoral calcinosis explored at two institutions over a ten-years period

Data relative to age, sex, location, clinical presentation, radiological features, histological findings and follow-up are reviewed

PATIENTS AND METHODS

All patients had radiographs and MR scans

Three patients underwent ultrasound studies

Computed tomography (CT) scans were performed in five patients

An additional Tc-99m labelled phosphate bone scintigraphy was performed in two patients

RESULTS

Patients were 6 males and 3 females Age ranged from 2 to 53 years (mean 13

years) Patients had no particular familial history

nor biological abnormalities A history of trauma was found in 2 cases Clinical data are summarized in table 1

CASE AGE GENDER

FIRSTLOCALI-SATION

RECUR-RENCE

OTHER LOCALI-SATION

CLINICALPRESENTATION

1 9Y/Male Elbow R Swelling - PainLimited range of motion

2 10Y/Female

Elbow R Elbow L Swelling - Pain

3 3Y4M/Male Hip L + Elbow LFoot R

Swelling – Pain

4 2Y/Male Foot L + Elbows L+R

SwellingDiaphysitis

5 8Y/Male Elbow R Elbow RHips L+R

Swelling

6 53Y/Female

Foot R Swelling – Pain

7 12Y/Male Hip R Hip L Swelling - Pain Infection

8 9Y/Female Elbow R Swelling

9 13Y/Male Hip L + Swelling

RADIOGRAPHS

Radiographs showed well-demarcated, lobulated and calcified juxta-articular soft tissue masses, unattached to bone in all cases

The masses consisted of conglomeration of multiple small and round opacities with different size and density separated by radiolucent septa

Fluid-calcium levels were observed in 2 cases (fig 1)

Fig. 1. — Anteroposterior radiograph of the left hip in a 12- year-old boy shows well-demarcated lobulated calcified mass with fluid-calcium levels

(arrow) also known as the “sedimentation sign” (case # 7)

Radiographs also showed periosteal reaction associated with ill-defined patchy areas of bone condensation within the medullary cavity of the tibia in one patient

On scintigrams, increased radionuclide uptake was seen in the tibial diaphysis

These lesions completely resolved on follow-up

RADIOGRAPHS

ULTRASOUND (US) US showed well-defined lobulated soft tissue

masses demonstrating heterogeneous echogenicity

Cysts were separated by hyper-echoic thin septa, sometimes partially calcified

Colour Doppler detected blood flow within the septa in 2 cases

Fluid-calcium levels in two cases (fig 2)

Irregular and calcified areas in two cases

Fig.2. — Same patient. Ultrasound study shows multi cystic mass(a). Cysts contain hypo-echoic liquid and are separated by hyper-echoic

vascularized thin septa(b)Fluid-fluid levels are noted (c)

a b c

CT

One lesion showed a nodular calcified component separated by low-attenuation septa

Another lesion consisted of mainly large cystic components with low attenuation centres and thin calcified walls

Three lesions showed both patterns

Fluid-calcium levels were seen in 4 patients (fig 3)

Septal enhancement after contrast injection was seen in one case

Fig. 3. — Same patient. CT image of the left hip : the mass shows a mainly cystic component with low attenuation centres and thin calcified

walls. Fluid-calcium levels are noted

MRI

On T1-weighted MR images, all lesions showed intermediate to low signal with more hypointense round or semi-lunar foci

On T2-weighted images, all lesions were heterogeneous and almost all presented mainly hyperintense multi loculated cystic structure associated to hypointense foci

MRI

Septa separating the cysts were thin, showing low signal on T1-weighted images and variable signal on T2-weighted images

Fluid-calcium levels were noticed in 5 cysts, upper layers being hyperintense and inferior ones hypointense

Peripheral and septal enhancements were noticed in all cases after Gadolinium administration.

MRI

Pseudo-tumoral areas were seen in 4 lesions showing o low signal intensity on T1-weighted imageso heterogeneous and variable intensity on T2-

weighted imageso heterogeneous and marked enhancement after

contrast injection.

These areas corresponded to mainly calcified zones on CT scans in 2 cases (figures4a,b,c&d)

No bone or joint involvement was seen on imaging

Fig.4. — Same patient. Pseudo-tumoral area showing low signal intensity on T1-weighted MR images (a), heterogeneous variable intensity T2-

weighted MR images (5) and fluid-calcium levels(c)

a b c

Fig.4d.— Gross examination of this area shows multiple small and empty cysts. We speculate that the particular pattern of collapsed cysts walls

accounts for the pseudo-tumoral feature on MRI

Evolution

All patients underwent surgical resection

On gross pathological examinations, the masses were multiloculated and exuded yellowish creamy to chalky fluid

Microscopic analysis showed granulomas with calcified central area and separated by large fibrous walls

DISCUSSION

Idiopathic tumoral calcinosis is an unusual benign condition characterized by the presence of calcified soft tissue masses of varying size around the joints

Previously described by Giard in 1898, Duret in 1899 and Teutschländer in 1935

The term “tumoral calcinosis” was used, and the first radiographs of the condition were published for the very first time by Inclan in 1943 (Figure 5) (1)

Fig.5. : First published radiographs of tumoral calcinosis lesions(a) Tumoral calcinosis of the hip(b) Lateral and (c) anteroposterior views show tumoral calcinosis of the

elbow (Inclan A, Leon P, Camejo MG. Tumoral calcinosis.J Am Med Assoc 1943;121:490–495.)

a b c

DISCUSSION

Idiopathic tumoral calcinosis is a hereditary metabolic dysfunction of phosphate regulation associated with massive periarticular calcinosis.

Occurs in the first three decades of life

There is apparently no sex predominance (2)

Predilection for black people and people from tropical climates has been reported (3)

DISCUSSION

Slow growing soft tissue mass, painless in most cases, arising in the vicinity of large joints

Most common locations in descending order are the hip, elbow, shoulder, foot, and wrist (2)

In our series, the most affected site was the elbow (n = 9), with the hip coming in second position (n = 6)

Two thirds of patients have multiple lesions (4)

DISCUSSION

The tumor may : o interfere with joint motion, o cause pain by nerve compression, o Ulcerations and secondary infection occasionally

appear

Laboratory analysis usually indicates : o Normal calcemia, parathyroid hormone level, renal

function, alkaline phosphatase and uremiao Phosphatemia and Vitamin D levels are normal or

slightly elevated

DISCUSSION On radiographs typical appearance : Amorphous, cystic, and multilobulated calcification

located in a periarticular distribution CT better delineates the calcified mass The cystic appearance shows fluid-calcium levels

caused by calcium layering and commonly termed the sedimentation sign (5)

No adjacent bone erosion or destruction

DISCUSSION

MR imaging : T1-weighted sequences usually show

inhomogeneous lesions with low signal intensity (6)

T2-weighted sequences generally shows inhomogeneous high signal intensity even though there is a large amount of calcification

DISCUSSION The diagnosis of idiopathic tumoral calcinosis

is one of exclusion

The differential diagnosis includes : chronic renal failure, primary hyperparathyroidism, calcinosis universalis, calcinosis circumscripta, chronic vitamin D intoxication, milk-alkali syndrome and collagen vascular diseases (6,7)

Such diseases are excluded by history and laboratory findings in our patients

These latter diseases are associated with a high serum calcium level while normocalcemia is seen in idiopathic tumoral calcinosis

The diagnosis of tumoral calcinosis has to be refuted if an elevation of the blood levels of urea, calcium or non-protein nitrogen is found (7)

DISCUSSION

DISCUSSION

Complete surgical excision is the optimum treatment

In some patients, masses tend to recur easily despite repeated complete surgical resections

It is thought that recurrences are quite common in cases with hyperphosphatemia or with predisposing genetic abnormality (8)

Management will be easier once all mechanisms of the disease are elucidated

CONCLUSION

Because many conditions mimic idiopathic tumoral calcinosis, diagnosis is difficult with diagnostic imaging alone

The radiologist plays a critical role in :o Avoiding unnecessary invasive procedures

o Guiding the selection of appropriate biological

testso Making conclusive diagnosis of tumoral

calcinosis

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Orthop 1984 ; 185 : 83-86.3. Barnacle AM, Gower PE, Mitchell AW. Ultrasonography of acute and chronic tumoral

calcinosis. Clin Radiol 2002 ;57 : 146-149.4. Fujii T, Matsui N, Yamamoto T, Yoshiya S, Kurosaka M. Solitary intra-articular tumoral

calcinosis of the knee.Arthroscopy 2003 ; 19 : 1-4.5. Hug I, Guncaga J. Tumoral calcinosis with sedimentation sign. Br J Radiol 1974;47:734–736.6. Idiopathic tumoral calcinosis Skander CHAABANE, Mouna CHELLI-BOUAZIZ, Helmi JELASSI

and al Acta Orthop. Belg., 2008, 74, 00-007. Tumoral Calcinosis: Pearls, Polemics, and Alternative Possibilities Kathryn M.

Olsen,Felix S. Chew, RadioGraphics 2006; 26:871–885.8. Hammoud S, McCarthy EF, Weber K. Tumoral calcinosis in infants : A report of three

cases and review of the literature. Clin Orthop 2005 ; 436 : 261-264.