|Year : 2014 | Volume
| Issue : 3 | Page : 198-200
Tumoral calcinosis of scalp: A rare phenomenon
Manish Gutch1, Sukriti Kumar2, Syed Mohd Razi1, Keshav Kumar Gupta1
1 Department of Endocrinology, Lala Lajpat Rai Memorial Medical College, Meerut, Uttar Pradesh, India
2 Department of Radiodiagnosis, Sanjay Gandhi Post Graduate Institute, Lucknow, Uttar Pradesh, India
|Date of Web Publication||17-Aug-2014|
D-15, LLRM Medical College, Meerut, Uttar Pradesh - 250 004
Source of Support: None, Conflict of Interest: None
Tumoral calcinosis is a rare disorder of unknown etiology characterized by the deposition of calcified masses within the peri-articular soft tissues of large joints, and sometimes in small joints but rarely involves the scalp. Masses of soft tissue calcification around the joints sometimes cause limitation of movements and disabilities requiring surgical intervention. Involvement of scalp is extremely rare. We are presenting a case report of a young girl who presented with soft tissue calcification of large joints along with scalp involvement.
Keywords: Chronic renal failure, calcifying lesions, tumoral calcinosis
|How to cite this article:|
Gutch M, Kumar S, Razi SM, Gupta KK. Tumoral calcinosis of scalp: A rare phenomenon. CHRISMED J Health Res 2014;1:198-200
|How to cite this URL:|
Gutch M, Kumar S, Razi SM, Gupta KK. Tumoral calcinosis of scalp: A rare phenomenon. CHRISMED J Health Res [serial online] 2014 [cited 2020 Jul 11];1:198-200. Available from: http://www.cjhr.org/text.asp?2014/1/3/198/138903
| Introduction|| |
Tumoral calcinosis is a rare condition characterized by deposition of tumor-like, hard calcified masses in peri-articular soft tissues of large joints. It was first described by Giard in 1898  and termed "endotheliome calcifie". The term tumoral calcinosis was first used by Inclan et al. in 1943,  and this condition was characterized by normal serum calcium levels and elevated or normal serum phosphate levels. "Tumoral calcinosis" has been divided into primary and secondary type. In the primary type, there is no known cause and in secondary type, one or several responsible etiologies can be attributed, the most common one being chronic renal failure. Other etiologies need to be ruled out before making the diagnosis of primary tumoral calcinosis, like chronic renal insufficiency, hyperparathyroidism, hypervitaminosis D, and milk-alkali syndrome.
The most common locations of tumoral calcinosis are around the hip joint, elbow, shoulder, foot, and wrist joints with a predisposition for extensor surfaces.  Tumoral calcinosis has not been previously reported involving the scalp in a young girl, and to the best of our knowledge, we present the first reported case of a scalp tumoral calcinosis in a young girl.
| Case Report|| |
An 8-year-young girl presented to our hospital for the complaints of multiple progressively enlarging masses over the occipital region of her scalp, lateral side of right foot, and lateral side of left elbow. The first lesion (on lateral side of foot) had appeared when she was 3 years old and subsequently a new lesion would crop up every 2 years. She was never investigated or operated in any hospital. There was no family history of similar illness.
On physical examination, one large swelling was present in the occipital region [Figure 1], 2 nd on lateral side of right foot [Figure 2], and 3 rd on lateral side of elbow. They were non-mobile and had variable consistency. There were no pressure effects over the bone or skin. The rest of the physical examination was unremarkable.
Laboratory investigations for calcium, phosphate, and renal functions were normal. Serum alkaline phosphatase and serum intact parathyroid hormone levels were within normal limits. Serum creatinine was also within normal limits. An X-ray of the skull revealed chunky calcification over the area of the mass with no underlying destruction of bone, periosteal reaction, or soft tissue swelling [Figure 3]. X-ray lateral view right foot showed similar findings [Figure 4].
|Figure 3: X-ray of the skull showing chunky calcifi cation over the occiput|
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|Figure 4: X-ray showing periarticular calcinosis around metatarsophalangeal, cuboid-metatarsal, and interphalangeal joints of left foot|
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An excisional biopsy followed by histopathology was performed on lateral side of foot it revealed; the specimens consisted of fragmented pieces of dense fibrous tissue surrounding nodular aggregates of heavily calcified debris. Focal foreign body giant cells were seen surrounding the calcification. Based on clinical, biochemical, and histological findings, the diagnosis of non-familial sporadic tumoral calcinosis was made.
| Discussion|| |
Tumoral calcinosis is a rare disorder characterized by formation of calcium hydroxyapatite crystals and amorphous calcium phosphate crystal around the soft tissues of large and small joints. In primary type, there is no associated disease (responsible etiology) and a secondary type that follows other disorders, particularly chronic renal failure.
The exact mechanism behind the formation of tumoral calcinosis is still unknown but the probable mechanism appear to be initiated by a localized trigger in the presence of a local or regional deranged metabolic environment. The chain of events begins with local hemorrhage, which progresses to fat necrosis, collagenization, collagenolysis, and ultimately massive calcification. The initial trigger can arise as a result of repetitive microtrauma, peri-articular shear forces, or direct hemorrhage acting as a catalyst in this calcification cascade. 
The treatment of tumoral calcinosis depends largely on its underlying cause. Surgical excision is the mainstay of treatment, but recurrences are common following incomplete excision or in cases involving actively progressing lesions. Phosphate depletion therapy alone for primary normo- and hyperphosphatemia has demonstrated varying success.  Other therapies, including systemic steroid therapy and radiation therapy, are not recommended. 
By far, very few studies have been done on the use of bisphosphonate in the treatment of tumoral calcinosis; however, the study done by Jacob et al. clearly shows successful treatment of tumoral calcinosis by using alendronate. 
Tumoral calcinosis have been described previously around the thigh, large joints, and small joints of the body. Involvement of scalp has been shown in only one case of tumoral calcinosis and the patient was an adult.  There is no reported case of tumoral calcinosis of scalp in a young girl.
| References|| |
|1.||Giard A. On calcification hibernale. C R Soc Biol 1898;10:1013-5. |
|2.||Inclan A, Leon P, Camejo MG. Tumoral calcinosis. JAMA 1943;121:490-5. |
|3.||Olsen KM, Chew FS. Tumoral calcinosis: Pearls, polemics, and alternative possibilities. Radiographics 2006;26:871-85. |
|4.||Weiss SW, Goldblum JR, editors. Enzinger and Weiss's soft tissue tumours. 4 th ed. St. Louis: Mosby; 2001. p. 903-44 |
|5.||Kirk TS, Simon MA. Tumoral calcinosis: Report of a case with successful medical management. J Bone Joint Surg Am 1981;63:1167-9. |
|6.||Thomson JE, Tanner FH. Tumoral calcinosis. J Bone Joint Surg Am 1949;31:132-5. |
|7.||Jacob JJ, Mathew K, Thomas N. Idiopathic sporadic tumoral calcinosis of the hip: Successful oral bisphosphonate therapy. Endocr Pract 2007;13:182-6. |
|8.||Jacob JJ, Thomas N, Seshadri MS. Tumoral calcinosis of the Scalp: An unusual site for a rare tumor. Laryngoscope 2007;117:179-80. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]