18 January 2006 Case of the Week #34

 

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We thank Dr. Juan Jose Segura Fonseca, Departamento de Patologia, Hospital San Juan de Dios, San Jose, Costa Rica, for contributing this case. We invite you to contribute a Case of the Week by sending an email to

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Case of the Week #34

 

Clinical History

 

A 24 year old Caucasian man presented to the outpatient clinic complaining of a slow growing, painless soft tissue mass over the right inguinal area for 8 months. The skin over the mass was tense, and over the past 2 months, a small ulcerated area discharged yellow-white chalky fluid and sometimes small, white, solid granules (Figure 1). A fine needle aspiration was performed and white chalky material was aspirated. A pelvic Xray showed small calcified nodules and a large, dense periarticular calcified mass in the soft tissue around the hip joint (Figure 2). The soft tissue mass was partially resected.

 

The tissue grossly was composed of white, granular, calcified chalky material surrounded by a fibrous tissue capsule (Figure 3).

 

Microscopic images: Figure 4, Fig 5, Fig 6, Fig 7, Fig 8 and Fig 9.

 

What is your diagnosis?

 

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Diagnosis

 

Tumoral calcinosis

 

Discussion

 

Tumoral calcinosis was described by Giard in 1888, Duret in 1889 and named by Inclan in 1943 (OMIM 211900). Its main feature is large, subcutaneous calcified masses in teenagers and young adults that are usually asymptomatic and slow growing. The masses are always near a large joint, commonly the hip, shoulder or posterior elbow. A few cases have been reported near the hands and feet, but the knee joint is always spared. Intraosseous deposits are rare.

 

Occasionally, there is ulceration of the overlying skin with discharge of chalky material. The deposits contain amorphous calcium phosphate and calcium carbonate with associated hydroxyapatite crystals (Clin Imaging 2003;27:184). The differential diagnosis includes dystrophic calcification, which is associated with necrosis.

 

Tumor calcinosis has its onset during the first and second decades of life, and is rare after age 50 years. It is more common in blacks, and males and females are equally affected. It is usually inherited as an autosomal dominant trait with variable clinical expression. Mutations have been recently described in familial cases (J Clin Endocrinol Metab 2005;90:2424, J Clin Endocrinol Metab 2005;90:5523, Hum Genet 2005;118:261. Typically there is a mild elevation of serum phosphate and Vitamin D, and normal serum calcium, alkaline phosphatase and uric acid levels (J Clin Endocrinol Metab 1985;6:1093).

 

Microscopically, an active cellular phase is characterized by a central mass of amorphous or granular calcified material surrounded by hyalinized fibrous tissue separating several cavities. The fibrous tissue is bordered by a granulomatous and chronic inflammatory infiltrate. There may be prominent small psammoma-like bodies or calcospherites (Histopathology 1997;31:18).

 

Treatment is complete surgical excision, due to a high recurrence rate. Fine needle aspiration may be helpful in diagnosis (Diagn Cytopathol 1995;13:339)

 

Additional references: Am J Surg Pathol 1993;17:788.

 

 

 

Nat Pernick, M.D.
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