Spontaneous regression of multiple tumoral calcinosis in a child

ARTICLE

Auteur(s) : Tomoyoshi OKADA, Hiroyuki HARA, Hiroyuki SHIMOJIMA, Hiroyuki SUZUKI

Department of Dermatology, Nihon University School of Medicine, 30-1 Oyaguchi-kamimachi, Itabashi-ku, Tokyo, 173-8610, Japan

Article accepted on 25/5/2004

Tumoral calcinosis is a rare benign tumor with unidentified etiology. It is characterized by large, calcified, painless subcutaneous masses. The calcium deposits are usually encapsulated, multilobulated and induce a foreign body reaction with histiocytes and osteoclast-like giant cells [1]. However, spontaneous regression quite rarely occurs.
This article reports a case of multiple tumoral calcinosis which spontaneously regressed after an excisional biopsy in a one-year-old Japanese boy.

Case report

A one-year-old Japanese boy was referred for investigation of the painless multiple masses on his occipital and back regions, which were first noticed 2 months before presentation. There was no history of trauma. The child’s history included neonatal hepatitis. The viruses such as hepatitis B, hepatitis C, rubella, coxsackie, cytomegalovirus and parainfluenza were not implicated in the etiology of this case.
Physical examination showed multiple, rubbery to bony-hard subcutaneous masses measuring 2 to 8 cm in diameter on the occipital region (Figure 1a). In addition, there were many blue-gray, irregular, rubbery subcutaneous masses, the largest measuring 8 cm in diameter, scattered on the back (Figure 1b). No chalky material could be discharged from the lesions. There was no limitation of joint motion. Neurological examination findings were normal.
Laboratory investigations disclosed a serum calcium content of 18.1 (normal level for infants is 8.5 to 10.3 mg/dL). The corresponding serum phosphorous level was 4.8 (normal for infants is 4.0 to 7.0 mg/dL). His Ca X P serum product was 87 units (normal < 60). Serum alkaline phosphatase, 1,25 dihydroxycholecalcifererol and parathyroid hormone levels were within the normal limits. Levels of urea nitrogen, creatinine, uric acid were all within normal limits. Asparate aminotransferase (AST), alanine aminotransferase (ALT), and γ-glutamyl transpeptidase (γ-GTP) levels were 120 U/l (normal for 11-35), 62 U/l (normal 4-30) and 882 U/l (normal 4-62), respectively.
X-rays showed a large lobulated calcified mass both on the occipital and back regions with well-rounded calcifications separated by linear radiolucencies. A computed tomography (CT) scan demonstrated the amorphous calcification within the subcutaneous tissues and separate from the bone and joint (Figure 2a).
An excisional biopsy was performed from one of the tumors on the occipital region. Histopathological findings showed masses of calcification situated in the deep dermis and the subcutaneous fat (Figure 3). These deposits were not encapsulated. The surrounding dermal tissue showed some increase in vascularity and in the number of fibroblasts. Multiple cystic spaces with irregular contours were observed. The cystic lumen contained eosinophilic debris giving a speckled granular appearance, which was strongly positive with the von Kossa stain. The calcified masses were surrounded by lymphocytes, plasma cells, macrophages and giant cells with an adjacent fibrous tissue. A diagnosis of multiple tumoral calcinosis was made.
The subcutaneous masses both on the occipital and back regions were gradually reduced after an excisional biopsy. Three months later, all masses spontaneously regressed without any medical treatment. Six months after the initial presentation, there was no clinical or radiological recurrence (Figure 2b). All the parameters mentioned previously had returned to within normal limits, and the serum calcium level decreased from 18.1 to 10.5 mg/dL. His serum Ca X P product also declined 86.7 units to 54.6. The levels of AST, ALT and γ-GTP also decreased to within normal range.

Discussion

In the present study, multiple tumoral calcinosis occurs under one year old and this disorder spontaneously regresses after an excisional biopsy from one of the lesions. There is only one report in the literature which demonstrated spontaneous resolution of a painful mass on the right supraclavicular region after only incisional biopsy [2].
Sporadic or familial cases have been described. The familial pattern suggests an autosomal recessive inheritance [3]. Although most patients are affected in the first and second decades of life, the youngest patient was 3 months old [4]. The calcified masses vary in size and predisposition sites are above the joints for the buttocks and hips [3]. The recommended treatment is complete surgical excision before the invasion of surrounding structures takes place. The lesions of this condition often recur after inadequate excision.
The pathogenesis of tumoral calcinosis remains obscure. The most plausible theory is an inborn error in the phosphate metabolism [5] or of transport [6]. One of the important factors associated with this disorder is an elevation of the serum Ca X P product. The net calcium and phosphate balance, for most cases reported, shows over 60. Therefore, one of the recommended treatments of the condition is a phosphorus-restricted diet. There are several reports of successful treatment with a phosphorous- and calcium-restricted diet, where lesions completed resolution after several months [5, 7]. In our case, the serum Ca X P product decreased to beyond 60 units without a phosphorus- and calcium-restricted diet as the tumoral calcinosis regressed spontaneously.
The present case also had neonatal hepatitis with unknown etiology. Laboratory examination indicated that there was considerable improvement in the liver function as well as the course of the spontaneous regression of the tumoral calcinosis, although we cannot fully explain whether these findings represent cause or effect. n

References

1. Krueger-Franke M, Siebert ChH, Weiss M, Rosemeyer B, Gokel M. Tumoral calcinosis of the ischium. Arch Orthop Trauma Surg 1992; 111: 284-6.

2. Niall DM, Fogarty EE, Moore DP. Spontaneous regression of tumoral calcinosis in an infant: A case report. J Pediatr Surg 1998; 33: 1429-31.

3. Metzker A, Eisenstein B, Oren J, Samuel R. Tumoral calcinosis revisited – common and uncommon features. Report of ten cases and review. Eur J Pediatr 1988; 147: 128-31

4. Bostrum B. Tumoral calcinosis in an infant. Am J Dis Child 1981; 135: 246-7

5. Mozaffarian G, Lafferty FW, Pearson O. Treatment of tumoral calcinosis with phosphate deprivation. Ann Intern Med 1972; 77: 741-5.

6. Wilber JF, Slatopolsky E. Hyperphosphatemia and tumoral calcinosis. Ann Intern Med 1968: 39: 1043-9.

7. Gregosiewicz A, Warda E. Tumoral calcinosis: Successful medical treatment. J Bone and Joint Surg 1989; 71-A: 1244-9.