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CASE REPORT
Idiopathic Tumoral Calcinosis in Hand: A Case
Report
Chris Xu, BMBS,aJosephine Alexa Potter, BMBCh,aChristopher David Carter,
MBBS,band Clayton Miles Cooper Lang, MBBSa
aDepartment of Plastic and Reconstructive Surgery, The Queen Elizabeth Hospital, Woodville South,
South Australia, Australia; and bDepartment of Anatomical Pathology, SA Pathology and Department
of Pathology, Flinders University, South Australia, Australia
Correspondence: chrissunxu@gmail.com
Keywords: hand, tumoral calcinosis, surgery, idiopathic, calciphylaxis
Published July 24, 2014
Objective: Tumoral calcinosis is an uncommon lesion, composed of ectopic calcified
tissue, most commonly seen in the large joints of the hips, knees, shoulders, and elbows.
The involvement of the hand in a healthy patient is extremely rare, and therefore this con-
dition can cause diagnostic confusion. The purpose of this report is to describe one case
of idiopathic tumoral calcinosis that occurred in the left hand of a 35-year-old healthy
female patient. Methods: The patient presented with 2-day history of acutely swelling
and painful left hand middle finger metacarpal phalangeal joint without any precipitants.
Results: All biochemical, radiological, and histopathological evidence suggested idio-
pathic tumoral calcinosis of the hand. Conclusions: In this case, surgery provided the
patient with instant symptomatic relief and full functional recovery of that joint.
Tumoral calcinosis is an uncommon benign condition characterized by large calcified
periarticular soft tissue masses composed of calcium salts, usually located around large
joints. The exact etiology remains unknown. The disease was first recognized in 1898 by
Giard, and then in 1899 by Duret, who reported 2 cases occurring in 2 siblings and named
the process endothelium calcifie. In 1935, Teutschlaender, who investigated the disease for
more than 20 years, labeled the lesion lipocalcinogranulomatosis (Teutschlaender disease).
Unaware of this previous work, Inclan in 1943 described 3 patients with large periarticular
calcified masses, which he called tumoral calcinosis. This name has been accepted by
international community since then.1,2,5-7
There is a primary form (idiopathic or hereditary), but tumoral calcinosis is often found
in a wide variety of conditions secondary to hyperparathyroidism, vitamin D intoxication,
scleroderma, and uremia in the context of chronic renal failure.
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CASE PRESENTATION
A 35-year-old right hand dominant housewife presented to The Queen Elizabeth Hospital
emergency department with spontaneous onset with 2-day history of swollen and tender
left middle finger metacarpophalangeal joint. No history of trauma was recalled. She is a
smoker and had laparoscopic cholecystectomy many years ago secondary to cholelithiasis.
On further questioning, she reported a similar episode 2 weeks prior at the same joint.
However, the symptoms settled within 24 hours and did not prompt her to seek medical
attention. On examination, the affected joint was swollen, exquisitely painful on passive
movement, erythematous, and warm to touch. Biochemical study showed normal calcium,
phosphate, parathyroid hormone, vitamin D, and thyroid function level. X-ray showed a
prominent area of soft tissue calcification projected just dorsolateral to the head of the middle
finger metacarpal bone measuring 12 mm ×4 mm in diameter (Fig 1). Ultrasound-guided
biopsy was unable to aspirate any sample due to the relative solid nature of the lesion.
Initial differential diagnosis was either a crystalline arthropathy or early septic arthritis
and empirical therapy (intravenous cefazolin and oral colchicine) for both conditions were
commenced. Minimal improvement was observed overnight, and the decision was therefore
made to perform an arthrotomy and irrigation of the joint. Perioperatively, access to the
lesion was achieved by a single curvilinear dorsal incision. A single gouty tophus like
structure was found external to the joint, but infiltrating into the collateral ligaments of
the joint (Fig 2). Sharp dissection was done from paratenon to joint capsule. The lesion
was excised and deposits removed from collateral ligament as much as possible. There
was no evidence of ulceration or fistula formation with the superficial skin. Histology
showed a circumscribed ovoid mass of amorphous granular calcific debris containing sparse
macrophages and lymphocytes and having a peripheral rim of dense fibrotic tissue, typical
of tumoral calcinosis at an inactive late stage. In addition, the calcific deposit contained a
moderate neutrophilic inflammatory infiltrate (Fig 3). No crystals or bacteria were identified
in the joint aspirate specimen.
DISCUSSION
Tumoral calcinosis is an uncommon form of ectopic calcification characterized by large,
rubbery, or cystic masses occurring mainly in relation to large joints. Prior studies have
suggested that tumoral calcinosis most commonly occurs around the hip, shoulder, and
elbow while it is rare in the small joints of the hand and foot.1-4
Primary tumoral calcinosis should be differentiated from secondary tumoral calcinosis,
which occurs in association with renal failure and with hypercalcemic disorders. It has been
suggested by Smack et al, tumoral calcinosis should be grouped into 3 categories based
on pathogenesis. The primary familial form of the disease mainly affects young, African
males with elevated serum phosphate and vitamin D. The identified major genes responsible
for the autosomal recessive pattern of inheritance are GALNT3,FGF-23,andKlotho,3
genes whose proteins are important in promoting phosphate excretion and suppressing
vitamin D synthesis.2Patients frequently present with multiple tumoral calcinosis affecting
multiple joints, with a high recurrence rate after surgical excision. In contrast, the primary
idiopathic form of the disease affects patients who have no family history of the condition or
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known disorders of phosphate or calcium metabolism. In this instance, tumoral calcinosis
is normally a single event with low incidence of recurrence.4,7
Figure 1. X-ray. Single 12 mm ×4 mm radio-opaque mass (arrow) can
be visualized alongside the radial border of the affected joint.
Finally, a secondary form of tumoral calcinosis results from a concurrent disease
that is capable of causing excessive calcium deposition conditions such as chronic renal
failure ±dialysis, hyperparathyroidism, vitamin D intoxication, milk alkali syndrome,
collagen vascular disease (ie, scleroderma), and bony destruction secondary to malignancy.2
Secondary tumoral calcinosis is best controlled by effective management of the primary
underlying condition.
Tumoral calcinosis has a typical appearance of amorphous, cystic, and multilobulated
periarticular calcification on plain radiographs. Tumoral calcinosis in the large joints is
often reported as a dense “chicken wire” type of lucencies that give the calcification a
“cobblestone” appearance. Computed tomography delineates the calcific mass, whereas the
fluid level seen within is termed a “sedimentation sign.” Similar findings can be found in
magnetic resonance imaging as a hypointense-dependent layer on T2 images. Erosions and
osseous destruction by the adjacent soft tissue mass is usually absent.7,8 These findings
help to distinguish tumoral calcinosis from other diseases causing periarticular soft tissue
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calcification such as synovial osteochondromatosis, neoplasms, connective tissue diseases,
or degenerative changes.7,8 Our case’s hand X-ray showed the lesion to be a rounded, dense,
periarticular opacity that was typical of tumoral calcinosis of the hand. It was relatively
homogenous, which is suggestive of a reduced metabolic activity with a reduced chance
of growth. Tumoral calcinosis is frequently recognized in preoperative imaging before
definitive histological examination.3
Figure 2. Intraoperative aspect. Single white tophus-like structure was excised. The structure was
adjacent to metacarpophalangeal joint, but not penetrating into the joint capsule. Some collateral
ligament attachment was observed.
Histologically, periarticular calcification is an obligatory finding for tumoral
calcinosis.7Other diagnostic criteria for this condition are the amorphous granular na-
ture of the calcific material and the absence of crystal deposits. An unusual finding in
our case was the presence of a moderate neutrophilic inflammatory infiltrate within the
calcific debris, raising the possibility of superimposed infection. However, other case re-
ports describe a mild neutrophilic component found on histology, without evidence of an
infective component.1This acute inflammatory response in our case may simply relate to
traumatization of the lesion although there remains the possibility of sepsis, particularly
in view of the preoperative needle aspiration procedure. Cartilage and synovium are not
normally found in the lesion itself in cases of tumoral calcinosis.
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Figure 3. Histopathological aspect. There is amorphous calcific material with an infiltrate of neu-
trophils and macrophages.
The treatment of tumoral calcinosis should be tailored to the clinical setting. For
patients with secondary tumoral calcinosis, managing the underlying calcium/phosphate
homeostasis is the primary goal. In patients with end-stage renal failure, it is important
to differentiate secondary tumoral calcinosis from calciphylaxis due to high mortality rate
of the latter one. The clinical presentation and radiological and histological appearances
of calciphylaxis significantly differ from secondary tumoral calcinosis. Clinically, patients
with calciphylaxis develop superficial violaceous skin lesions that are painful, hyperes-
thetic, or pruritic. More widespread ischemia can produce a mottled or serpiginous skin
appearance similar to livedo reticularis. With tissue infarction, the lesions become intensely
necrotic, hemorrhagic, and painful and spread to contiguous areas. Radiologically, calci-
phylaxis presents with small and medium vessel calcifications, and a netlike pattern of soft
tissue calcifications. Histologically, calciphylaxis is characterized by microvascular medial
calcification and intimal hypertrophy associated with cutaneous ischemia and ulceration.9
Overall, calciphylaxis can be confidently diagnosed clinically and from skin biopsy.
Surgical excision of tumoral calcinosis is indicated if there is significant skin break-
down or joint involvement. Early excision should be undertaken while the masses are small
to avoid operative difficulties and ulceration of the overlying skin with secondary infection
that occur with very large masses.6Excision may be difficult if no true capsule is present
as the mass may invade adjacent muscle tissue. Recurrence in idiopathic tumoral calcinosis
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is uncommon. Spontaneous regression of the mass of tumoral calcinosis has never been
reported. The prognosis is good particularly in the absence of secondary infection.
Increased awareness of tumoral calcinosis is essential. Complete surgical resection is
the treatment of choice in most idiopathic cases. Alternative treatments using radiotherapy
and systemic steroids have been unsuccessful.7Finally, the role of the surgeon consists
not only in the proper excision of the mass but also in reconstruction of any resulting
tissue defect or functional deficit. With the additional help of early hand therapy, complete
functional return of the affected digits should be expected.
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