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Year : 2006  |  Volume : 16  |  Issue : 3  |  Page : 349-352
Tumoral calcinosis in child having turner syndrome - A case report


Department of Radio-diagnosis, G. R. Medical College, Gwalior - 474009, M.P., India

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Keywords: Tumoral calcinosis, Turner syndrome

How to cite this article:
Mukund A, Rawat L, Kumar A, Sharma G L. Tumoral calcinosis in child having turner syndrome - A case report. Indian J Radiol Imaging 2006;16:349-52

How to cite this URL:
Mukund A, Rawat L, Kumar A, Sharma G L. Tumoral calcinosis in child having turner syndrome - A case report. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Dec 10];16:349-52. Available from: http://www.ijri.org/text.asp?2006/16/3/349/29014

   Introduction Top


Tumoral calcinosis or lipocalcinogranulomatosis is an autosomal dominant condition with variable clinical expressivity [1,2]. It is relatively rare disorder presenting during 1st or 2nd decade and most commonly seen in people of African descent [3]. The disease presents as juxta-articular soft tissue masses of varying sizes containing calcific fluid (calcium hydroxyapatite) enclosed by fibrous tissue. Here we present a case of tumoral calcinosis in a thirteen year child having Turner syndrome.


   Case report Top


A thirteen year female child presented with the complaint of gradually growing painless swelling present in the anterior aspect of the right hip joint [Figure - 1]a and similar swelling on the left gluteal region [Figure - 1]b.

On general physical examination the patient was seen having micrognathia [Figure - 1]c, low posterior hair line and cubitus valgus deformity. Abdominal ultrasonography was performed using 3.5 MHz probe (Toshiba Just vision 400), revealed infantile uterus and the ovaries could not be demonstrated. Rest of the abdominal organs were normal except for splenomegaly. On local examination the swelling was non tender, firm, immobile, soft tissue mass. The overlying skin was stretched over the lesion and shiny in appearance.

The patient was sent for X-ray examination of pelvic region, which showed a large, lobulated radio-opaque mass placed in the lateral aspect of right hip joint within the soft tissue [Figure - 2]a, b. Bony involvement could not be ruled out. A similar lesion, smaller in size was seen on the contralateral side. X- ray examination of other joints were done with informed consent, which were normal. Ultrasonography of the lesion demonstrated lobulated mass permitting no sound transmission causing throughout posterior acoustic shadowing [Figure - 3].

Then the patient was sent for CT examination (GE CT/e) after explaining about the radiation factor and due consent. Considering the age of the patient minimum possible mAs was applied and only few sections were taken. Findings were similar as on X-ray showing lobulated bony dense mass placed exclusively in the soft tissue without any bony involvement [Figure - 4]a,b.

Prior to surgery for exact localization of the mass MRI was performed on 0.2 T open magnet MR scanner (GE signa profile 4). MR images showed bilateral juxta-articular lobulated masses, larger on right side, displaying uniformly hypointense signal intensity on T1, T2 and fat suppressed sequences [Figure - 5]a,b. Fat suppressed images also showed mild soft tissue edema surrounding the lesion [Figure - 5]c. No bony involvement was seen.

Other biochemical investigations were normal except for slightly raised phosphorus level. Calcium metabolism was normal. F.N.A.C. of the lesion yielded white cheesy material containing multinucleated giant cells [Figure - 6]a. The gross specimen [Figure - 5]b showed lobulated cystic mass which on histology [Figure - 6]c proved to be tumoral calcinosis.

So finally the patient was diagnosed as having Turner syndrome with bilateral tumoral calcinosis affecting anterolateral aspect of right hip region and left gluteal region. Till date no association of tumoral calcinosis with turner syndrome is known or reported.


   Discussion Top


Tumoral calcinosis is charecterised by tumor like periarticular deposits of calcium, seen affecting regions of hip, shoulder, elbow and feet [1]. The disorder occurs predominantly in otherwise healthy children, adolescents, and young adults and is more often multiple than solitary and is frequently seen affecting two or more siblings of same family [4] and also it is seen more commonly in African American population [3],[5]. More strikingly the calcium metabolism is normal. Association with calcifying skin lesions, dental abnormalities and angioid retinal streaks has been reported [6],[7],[8] but no association with Turner syndrome is known and this is the only case of its kind.

It presents as a large, firm, subcutaneous mass, being generally asymptomatic and slow growing. The lesion predominantly occurs in the soft tissues and is unrelated to bone. It is attached to fascia, muscle or tendon and may infiltrate them [4].

Pathologic findings disclose unencapsulated rubbery mass of dense fibrous tissue having cystic spaces filled with white creamy material consisting of hydroxyapatite crystals and non crystalline calcific deposits [9]. The wall is formed of florid proliferating mono or multinucleated macrophages, osteoclast like giant cells, fibroblasts and chronic inflammatory elements [5].

On imaging the lesion is seen as large, nodular, smoothly marginated juxta-articular masses of calcium density / intensity. Fluid fluid level may be seen sometimes due to lesions filled with cacific fluid [2]. Bones are not involved.

Differential diagnosis includes patients with chronic renal failure on dialysis, secondary hyperparathyroidism and hypervitaminosis D.

 
   References Top

1.Dahnert W, editor. Radiology review manual, 5th edition. Philadelphia, USA: Lippincott Williams & Wilkins, 2003:170.  Back to cited text no. 1    
2.Davis AM. Imaging of soft tissues. In: Grainger & Allison's Diagnostic radiology, 4th edition. London, UK: Churchill Livingstone, 2001: 2085.  Back to cited text no. 2    
3.Jain SP. Tumoral calcinosis in Somalia and Ethiopia: a report of 21 cases and brief review of literature. East Afr Med J 66:476, 1989.  Back to cited text no. 3    
4.Weiss SW, Goldblum JR. Benign soft tissue tumors and pseudotumors of miscellaneous type. In: Strauss M, editor. Soft tissue tumors. St louis, USA: Mosby, 2001: 1419-1482.  Back to cited text no. 4    
5.Pasaka NM, Kalengayi RM. Tumoral calcinosis: a clinicopathological study of 111 cases with emphasis on earliest changes. Histology 31:18, 1997.  Back to cited text no. 5    
6.Abraham Z, Rozner I, Rozenbaum M. Tumoral calcinosis: report of a case and brief review of literature. J Dermatol 23:545, 1996.  Back to cited text no. 6    
7.Gall G, Metzker A, Garlick J, ET AL. Head and neck manifestations of tumoral calcinosis. Oral Surg Oral Med Oral Pathol 77:158, 1994.  Back to cited text no. 7    
8.Ghanchi F, Ramsay A, Coupland S, et al. Ocular tumoral calcinosis: a clinicopathologic study. Arch Ophthalmol 114:341, 1996.  Back to cited text no. 8    
9.Hatori M, Oomamiuda K, Kokubun S. Hydroxyapatite crystals in tumoral calcinosis: a case report. Tohoku J Exp Med 180:359, 1996.  Back to cited text no. 9    

Top
Correspondence Address:
A Mukund
Room No. 3, P.G. Hostel, G.R. Medical College, Gwalior - 474009, M.P.
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.29014

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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]



 

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