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Year : 2013  |  Volume : 14  |  Issue : 1  |  Page : 41-43

Multiple bilateral symmetrical recurrent hyperphosphatemic tumoral calcinosis in a young adolescent girl: A rare entity

1 Department of General Surgery, GSVM Medical College, Kanpur, Uttar Pradesh, India
2 Department of Pathology, GSVM Medical College, Kanpur, Uttar Pradesh, India

Date of Web Publication23-Aug-2013

Correspondence Address:
Sanjay Kala
Department of General Surgery, L 21, GSVM Medical College, Kanpur - 208 002, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.116863

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Tumoral calcinosis is an uncommon disease characterized by periarticular soft-tissue hyperplasia and calcification. Large joint such as hip, shoulder and elbow are most commonly involved. This entity usually manifest in adolescent females as slowly growing, painless, mobile soft-tissue tumor like mass around the large joint that may interfere with joint function when large. A familial predisposition has been reported. Exact cause is not known, but there is deposition of calcium phosphate with normal calcium and elevated phosphorus levels. Here, we report a case of 15-year-old female presented with symmetrical, recurrent swellings on various joints with milk like fluid extrusion and scar mark of previous excision.

Keywords: Calcium, phosphorous, tumoral calcinosis

How to cite this article:
Kala S, Pantola C, Verma AK, Sengar A. Multiple bilateral symmetrical recurrent hyperphosphatemic tumoral calcinosis in a young adolescent girl: A rare entity. Indian J Paediatr Dermatol 2013;14:41-3

How to cite this URL:
Kala S, Pantola C, Verma AK, Sengar A. Multiple bilateral symmetrical recurrent hyperphosphatemic tumoral calcinosis in a young adolescent girl: A rare entity. Indian J Paediatr Dermatol [serial online] 2013 [cited 2020 Oct 29];14:41-3. Available from: https://www.ijpd.in/text.asp?2013/14/1/41/116863

  Introduction Top

Tumoral calcinosis is an uncommon disorder characterized by large periarticular deposition of calcium phosphate that resembles a neoplasm. The first report of tumoral calcinosis was by Duret [1] in 1899 and termed endotheliome calcifie. The term tumoral calcinosis was proposed by Inclan [2] in 1945 and was accepted world-wide.

Tumoral calcinosis is a rare condition, characterized by hyperphosphatemia, normocalcemia and deposits of calcium phosphate, usually adjacent to large joints such as the extensor aspect of the shoulder, hip and elbow. [2],[3],[4],[5] It manifest in childhood and adolescence as painless, firm, mobile tumor like mass in the soft-tissue around the large joint that may interfere with joint function when large. It usually found in Sub-Saharan African countries. [2],[6]

The name indicates calcinosis (calcium deposition), which resembles tumor (like new growth). They are not true neoplasms as they don't have dividing cells. They are just deposition of inorganic calcium with serum extrudate.

  Case Report Top

A 15-year-old female presented with swellings on various joints (left elbow, left shoulder, right shoulder) with scar marks of previous surgical removal at the right elbow and the paraspinal region (near T 12 -L 1 ) of the body [Figure 1]. The swelling on the left shoulder ulcerated onto the skin with the discharge of chalky white material. The swellings were in variable sizes ranging from 3 to 10 cm, hard in consistency, but movable in relation to the underlying bone and skin. The joints were also freely mobile. Patient also gave the history of previous surgical excision of similar swellings from her right elbow and the paraspinal region 1 year back. Serological examination was normal except hyperphosphatemia. X-ray of left shoulder, right shoulder and left elbow were showing opaque shadows surrounding the joints, but present in extracapsular location, suggestive of a hard bony mass?? tumor calcinosis [Figure 2]a-c. Magnetic resonance imaging left shoulder joint showed a large lobulated calcified subcutaneous mass lesion is seen around the left shoulder joint in the region of the deltoid muscle and anterior/superior to clavicle. Multiple fluid-fluid levels were also seen within the lesion suggestive of tumor calcinosis [Figure 2]d. Right elbow mass was excised, which was well-demarcated with chalky white cut surface [Figure 3]. On histology sections show large calcified masses surrounded by dense fibrocollagenous tissue with mild mixed inflammatory infiltrate suggestive of tumor calcinosis [Figure 4].
Figure 1: Tumor like mass in (a) left shoulder, (b) right shoulder, (c) left elbow and (d) right elbow scar of previous excision

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Figure 2: X‑ray of (a) left shoulder, (b) right shoulder, (c) left elbow and magnetic resonance imaging left shoulder show large well‑demarcated calcified mass around joint

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Figure 3: Gross cut surface showing chalky white well‑defined mass

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Figure 4: Histology shows large deposits of calcium separated with fibrocollagenous septa (H and E, ×100)

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  Discussion Top

In tumoral calcinosis, calcium deposition occurs in the soft-tissue in periarticular locations i.e., around joints. Despite various investigations, the etiology of soft-tissue calcification is still not well-understood. An elevated calcium phosphate product is thought to be the most prominent cause of extraskeletal calcification although inborn error of metabolism or transport of phosphorous, genetically conditioned predisposition of collagen toward calcification, secondary hyperparathyroidism, dynamic bone disease, hypermagnesemia vitamin D and vitamin K overload, aluminum intoxication, metabolic alkalosis and tissue injury may also play a part. [3],[4],[5],[7],[8] They are frequently seen in patients undergoing renal dialysis or renal failure. [9],[10]

Tumoral calcinosis occurs as a well-defined pathologic entity in three heterologous groups of diseases - hyperphosphatemic familial tumoral calcinosis, normophosphatemic tumoral calcinosis and secondary tumoral calcinosis. The histological lesion is stereotypic developing from the concurrence of a juxta-articular injury with elevated calcium - phosphorus product. The reparative response to injury is histiocytic featuring synovial metaplasia forming bursa-like structures that create the characteristic compartmentalization of the lesion. Histiocytic-derived osteoclastogenesis occurs as a response to the calcifying process initiated in the mitochondria of necrotic histiocytes forming the bursa-like structures. These calcifications propelled by a gamut of conditions elevating the serum phosphorus, facilitate the further nucleation of hydroxyapatite in mitochondria, matrical lipidic debris located in the cytoplasm and lysosomes of osteoclasts and in the locular contents and on collagen and other extracellular matrix materials. The lesions enlarge because of new locule formation and failure to reduce the calcified burden by the compartment lining histiocytes and dysmorphic osteoclasts that are unable to solubilize the hydroxyapatite. [11]

  Conclusion Top

Tumoral calcinosis reported here is an extremely rare entity and not so commonly seen in paediatric age group.

Phosphate-binding antacids, together with a diet low in calcium and phosphorus has been used to lower the intestinal absorption of phosphate and treat calcinosis universally. Medical management of this condition is preferable because of the metabolic nature of the disease and the frequent recurrence of the lesions after excision. [12]

  References Top

1.Duret M. Tumeurs multiples et singulaires desbourses sereuses. Bull Mem Soc Anthropal Paris 1899;74:725-32.  Back to cited text no. 1
2.Inclan A. Tumoral calcinosis. JAMA 1943;121:490-5.  Back to cited text no. 2
3.Lafferty FW, Reynolds ES, Pearson OH. Tumoral calcinosis: A metabolic disease of obscure etiology. Am J Med 1965;38:105-18.  Back to cited text no. 3
4.Baldursson H, Evans EB, Dodge WF, Jackson WT. Tumoral calcinosis with hyperphosphatemia. A report of a family with incidence in four siblings. J Bone Joint Surg Am 1969;51:913-25.  Back to cited text no. 4
5.Mozaffarian G, Lafferty FW, Pearson OH. Treatment of tumoral calcinosis with phosphorus deprivation. Ann Intern Med 1972;77:741-5.  Back to cited text no. 5
6.Slavin G, Klenerman L, Darby A, Bansal S. Tumoral calcinosis in England. Br Med J 1973;1:147-9.  Back to cited text no. 6
7.Wilber JF, Slatopolsky E. Hyperphosphatemia and tumoral calcinosis. Ann Intern Med 1968;68:1043-9.  Back to cited text no. 7
8.Mitnick PD, Goldfarb S, Slatopolsky E, Lemann J Jr, Gray RW, Agus ZS. Calcium and phosphate metabolism in tumoral calcinosis. Ann Intern Med 1980;92:482-7.  Back to cited text no. 8
9.Lawrence RA, Ozener C, Kilic S, Akoglu E. Tumoral calcinosis in chronic renal failure: Two case report. J Islam Acad Sci 1994;7:106-10.  Back to cited text no. 9
10.Eisenberg B, Tzamaloukas AH, Hartshorne MF, Listrom MB, Arrington ER, Sherrard DJ. Periarticular tumoral calcinosis and hypercalcemia in a hemodialysis patient without hyperparathyroidism: A case report. J Nucl Med 1990;31:1099-103.  Back to cited text no. 10
11.Slavin RE, Wen J, Barmada A. Tumoral calcinosis - A pathogenetic overview: A histological and ultrastructural study with a report of two new cases, one in infancy. Int J Surg Pathol 2012;20:462-73.  Back to cited text no. 11
12.Alkhooly AZ. Medical treatment for tumoral calcinosis with eight years of follow-up: A report of four cases. J Orthop Surg (Hong Kong) 2009;17:379-82.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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