A 17-year-old woman presented with a painless, bony hard mass in the gluteal region around the left hip joint, and in the posterior region of the left elbow joint. The central part of the overlying skin of the gluteal lesion eroded, with discharge of a chalky white material.
The patient's serum calcium, phosphate, uric acid, alkaline phosphatase, creatinine and blood urea nitrogen levels were within normal limits. Complete hemogram showed no abnormality. Radiographs of the left hip and elbow joints (Figure 1) showed irregularly round to oval, radio-dense, juxta-articular calcifications. CT scan (Figure 2) showed in the left gluteal region a large, lobulated calcified mass. There are fluid–fluid levels in several gluteal lobules consistent with ‘milky calcium’. In the left elbow joint CT scan showed less voluminous amorphous soft-tissue calcifications. The adjacent joints and the contiguous bones were unaffected. Fine needle aspiration cytology of the gluteal lesion showed amorphous granular material with occasional histiocytes. The gluteal lesion was surgically resected, the excised mass was whitish in color and milky fluid came out during sectioning. Histopathological examination revealed deeply basophilic amorphous granular material consistent with calcium deposits surrounded by dense fibrous tissue. Histiocytes were seen occasionally, there were no tumor cell and the histologic diagnosis was tumoral calcinosis. The lesions did not recur during a one year follow-up after excision.
Tumoral calcinosis (TC) is a rare benign disease, characterised by deposits of calcic material in peri-articular soft tissues. TC mostly affects young, African non-white adults males. The clinical manifestation of the disease is the presence of a spontaneously soft tissue mass in the periarticular areas. The mass usually is asymptomatic and rarely causes discomfort, tenderness, pain or discharge of calcium salts from the lesions. The trochanteric and gluteal areas of the hip joint being the most common site in reported series. In the majority of cases the patient's serum calcium, alkaline phosphatase, and the urine calcium are within normal limits. The phosphatemia is almost always elevated. The pathogenesis of TC is not clear in most cases, and several theories have been advanced on this day. Familial tumoral calcinosis is an autosomal recessive metabolic disorder associated with elevated levels of serum phosphate. The disease can be caused by recessive mutations in at least two different genes: GalNAc transferase 3 (GALNT3), encoding a glycosyltransferase that initiates mucin-type O-glycosylation, and fibroblast growth factor 23 (FGF23). Radiological examination plays a key role in the diagnosis. On plain radiographs, tumoral calcinosis has a typical cloudy and multilobulated appearance. Calcified lumps can grow up to 20 cm in diameter and usually have a smooth or nodular structure. A Pseudocysts and a pseudocapsule may be seen. The cystic appearance shows fluid-fluid levels caused by calcium layering and commonly termed the sedimentation sign. CT scan better delineates the calcific mass and shows patchy increased attenuation of affected areas, erosion or osseous destruction by adjacent soft-tissue masses is typically absent. MR imaging demonstrates focal increased signal intensity with multiple small foci of decreased T2 signal intensity surrounded by a high-signal-intensity ring in the periosseous subcutaneous tissue. Fluid–fluid levels described on MR fat-suppressed T2-weighted images favour the diagnosis. There are many conditions with similar appearances, including the calcinosis of chronic renal failure, calcinosis universalis, calcific tendonitis, synovial osteochondromatosis, osteosarcoma, myositis ossificans, tophaceous gout, and calcific myonecrosis. Rule out metabolic diseases, checking phosphate and calcium levels, as well as renal failure. Radiological examination plays a key role in differentiation as marked nicely in the discussion calcinosis universalis, that affects muscle and fascia planes; calcinosis circumscripta, which is less extensive and located within the subcutaneous tissue; synovial osteochondromas can associate bone erosion, has intraarticular locations and a typical rings and arcs calcifications; osteosarcoma shows bone destruction and soft tissue mass; myositis ossificants is usually a delimitated mass with rapid evolution in organized cartilage and bone with lack of loculated appearance; and calcified tophi are less dense do not show sedimentation, and patients with tumoral calcinosis don’t have hyperuricemia. The treatment of choice is the early complete surgical excision. Spontaneous regression of tumoral calcinosis in an infant was described once.
Tumoral calcinosis
Based on the provided images (X-ray and CT scan), there are relatively large, lobulated or mass-like calcifications around the left hip joint (gluteal region) and in the soft tissues behind the left elbow. They appear “cloud-like” or multi-cystic in nature. Local skin may show ulceration with white, powder-like exudate.
On the X-ray, the lesion shows high-density, lobulated calcification with distinct layers. Some areas reveal fluid-fluid levels (layered calcification sign). CT scan demonstrates a mass-like calcified lesion with well-defined borders and heterogeneous high density, without obvious bone destruction or bone erosion.
Overall, the lesion mainly involves the periarticular soft tissues, with a focal nature of calcification. Considering the large size, multinodular presentation, and clinical lack of significant pain (except for discharge from areas of ulceration), it is consistent with “tumoral calcinosis.”
Considering the patient’s age (17 years), clinical presentation (painless mass, chalky discharge at ulceration site), imaging features (large lobulated mass, presence of fluid-fluid level on imaging), and routine clinical findings (blood calcium, phosphorus levels possibly within or slightly outside normal ranges), the most likely diagnosis is:
Tumoral Calcinosis.
Further confirmation can be obtained by checking serum phosphorus, calcium levels, and relevant genetic tests (if there is a family history or high clinical suspicion). If uncertainty remains, a biopsy or pathological examination can be considered to rule out other sarcomatous lesions.
Since the lesion primarily involves soft tissue, the effect on joint range of motion depends on the location and size of the lesion. Typically, after surgery or once wounds are well-healed, an individualized rehabilitation program is recommended:
Throughout the rehabilitation process, a gradual approach following the FITT-VP principles (Frequency, Intensity, Time, Type, Volume, Progression) and individualization is crucial. Adjust exercise intensity and frequency according to the patient’s condition (bone strength, pain threshold, wound healing) to ensure both safety and efficacy.
This report is based on the limited information and imaging data provided. It is for reference only and does not replace in-person consultations or professional medical advice. If clinical symptoms worsen or potential complications arise, please seek timely medical attention from a specialist for further diagnosis and treatment.
Tumoral calcinosis
