Tumoral calcinosis is characterized by the deposition of
calcium phosphate in periarticular tissues. In general, it involves large
joints such as the hip, knee, shoulder, and elbow. The involvement of the hand
is extremely rare and only a few cases have been reported.1-3 This rare
condition may lead to diagnostic confusion and maltreatments.4 The following
case report involves tumoral calcinosis around the third metacarpo-phalangeal
|Figure 1: Standard radiograph showing multiple round to oval, well-demarcated masses of calcifications, located in the periarticular soft tissue of the third metacarpo-phalangeal joint. Figure 2: CT showing calcific focci in the soft tissue around the erosive lesions.
A 43-year-old man presented with periodic attacks of
severe pain, swelling, and redness over the dorsal aspect of the third
metacarpophalangeal joint of his right hand. The pain repeated every 6 months
and was relieved within 10 days with self-oriented antibiotics and nonsteriodal
anti-inflammatory drugs. Post-medical history included a parachuting accident
22 years earlier with no specific involvement of his hand. After a 9-year
interval, a painful and erythemateous mass had appeared. Two years later, the
mass had been excised and diagnosed histopathologically as a benign lesion.
There was neither a history of systemic disease nor a related family history.
During his initial presentation 10 days after the last
attack, physical examination displayed swelling and slight tenderness over the
dorsal aspect of the third metacarpophalangeal joint, just lateral to the
insertion of the extensor tendon of the third finger of his right hand. The
overlying skin was intact. The mobility of the adjacent metacarpophalangeal
joint was unlimited. He had no lymphangitis or regional lymphadenopathy, and
the rest of his hand functioned normally, including the range of movement and
neurovascular status. There were no other such lesions within the body. He was
afebrile and in good general health.
A standard radiograph showed multiple round to oval,
well-demarcated masses of calcifications located in the periarticular soft
tissue of the third metacarpophalangeal joint. An osteolysis of the ulnar
aspects of the subarticular parts of both the third metatarsal and proximal
phalanx had also recently appeared (Figure 1). Computed tomography showed
calcific focci in the soft tissue around the erosive lesions (Figure 2).
Laboratory tests that included the erythrocyte sedimentation rate (ESR), cell
counts, electrolytes (serum calcium and phosphate), and uric acid were all
within the normal range, with only a slight increase in cholesterol and
triglyceride levels. There was no evidence of any underlying disease such as a
metabolic or endocrine abnormality or connective tissue disease. Although
laboratory findings were not completely supportive, the long, periodic, and
relatively benign clinical course of the disease implied that there was a
crystal deposition such as gouty tophus.
Surgical excision of the calcific deposits was performed
through the dorsal approach to the third metacarpophalangeal joint. The excised
material was pink-yellowish and chalky (Figure 3). It consisted of 4 parts,
ranging in size from 2×2×0.4 cm to 0.3×0.2×0.1 cm. The
cut surfaces of the nodules were yellow-white with chalky-white granular
|Figure 3: Intraoperative view of the lesion (A, B). Figure 4: Granular calcium deposits (hematoxylen-eosin 310) (A). Fibroblastic vessel-rich stroma surrounding the deposits (hematoxylen-eosin 320) (B). Brown-black staining of calcium crystals (von kossa 310) (C). Red staining consistent with calcium deposition (alizarin red 320) (D).
In order to rule out gout disease, touch preparations
were prepared and examined in a fresh, unstained state under a polarization
microscope. The samples were also fixed in alcohol to preserve possible uric
acid crystals and processed by formalin-omitted routine tissue protocol. On
histologic examination, granules and deposits of calcium were seen in the soft
tissue (Figure 4A). Deposits were located in a fibroblastic vessel-rich stroma,
without a surrounding foreign body giant cell reaction (Figure 4B). Calcium
deposition was also confirmed by histochemical studies such as von Kossa
(Figure 4C) and alizarin red (Figure 4D) stains. Excised material consisted of
precipitation of calcium salts without any evidence of gouty tophus and was
diagnosed histopathologically as calcinosis.
At 4-year follow-up, the patient was pain free with no
evidence of recurrence or similar lesions.
Tumoral calcinosis is an uncommon disorder characterized
by large periarticular deposition of calcium phosphate that resembles a
neoplasm. The first report of tumoral calcinosis was by Duret5 in
1899 and termed endotheliome calcifie. The term tumoral calcinosis was proposed
by Inclan6 in 1945 and was accepted worldwide.
Since Duret’s description, more than 250 cases have
been reported, mostly located around large joints.7 However, the
involvement of the hand in tumoral calcinosis is very rare. Approximately 10
cases have been reported.2,3 Before 1967, Harkess and
Peters8 researched the literature and reported a total of 33 tumoral
calcinosis cases, including 6 of their own. Only 1 of the 33 cases reported
involved the hand. In 2006, Kamath et al4 reported a case of tumoral
calsinosis involving the second metacarpophalangeal joint. Also in 2006, Kim et
al1 reported 2 cases involving the metacarpophalangeal joints in 3
cases of tumoral calsinosis of the hand. The third case involved the fourth
proximal interphalangeal joint.
Tumoral calcinosis is usually seen on the extensor
surfaces of large joints such as the hip, knee, shoulder, and elbow in
adolescents and young adults. The natural course is generally slow, with the
progressive enlargement from a few weeks to many years. The clinical
presentation is generally painless swelling. Chalky discharge and a secondary
infection may arise. The electrolyte levels of these cases are normal. Standard
radiography shows the aggregation of irregularly shaped lobules of varying
sizes located in periarticular soft tissue. The adjacent joint is not involved,
and the joint space is maintained for a long period of time. Our case involving
a 13-year history presented only recently the erosion of adjacent surfaces of
the third metatarsal and proximal phalanx.
The exact etiology of calcinosis is still unknown.
However, an associated molecular abnormality has been shown in some cases of
familial tumoral calcinosis. The elevation of fibroblast growth factor-23 in
patients with a recessive form of familial tumoral calcinosis has been recently
reported.9 A localized soft tissue alteration is proposed in the
pathogenesis because patients with tumoral calcinosis often have a single
calcification with no abnormal findings of mineral homeostasis.1 An
aberrant tissue response to local trauma has been rarely reported as the cause
of tumoral calcinosis.10,11 Although there was a history of a
parachuting accident in our case, the long and asymptomatic interval did not
allow us to claim any relationship. Minor repetitive trauma may also serve as a
trigger mechanism, leading to a chain of events. It begins with a hemorrhage,
fat necrosis, fibrosis, and collagenization, then ends with collagenolysis and
ultimately massive calcification.7
Local inflammatory findings such as painful swelling,
redness, and warmth implied an infection in our patient. However, he was
afebrile and in good general health. White cell counts and ESR were ranged in
normal limits. Sabesta et al2 and Kamath et al4 also
reported 2 cases with infection-like symptoms in the second metacarpophalangeal
Other types of calcium deposition in soft tissues must
be considered in a differential diagnosis. Our case had no laboratory evidence
of any underlying disease such as a metabolic or endocrine abnormality or a
connective tissue disease. Acute calcific deposition is the most common
periarticular inflammatory disease leading to juxta-articular deposits of
calcium hydroxyapatite in the hand. Histological findings are similar to those
of tumoral calcinosis. The main characteristic of acute calcific deposition is
spontaneous and relatively early (<4-month) resolution of the deposition.
The inflammatory symptoms resolve spontaneously within a few days to a week,
and the calcific deposition disappears radiographically within a short time. A
conservative wait is all that is required. Acute calcific deposition is
commonly involved in the shoulder, but the involvement of the hand has been
reported.12 With a 13-year history, periodic character of symptoms
and persistence of deposition, this specific case was not consistent with acute
Tophaceus pseudogout, also called tumoral calcium
pyrophosphate dehydrate deposition disease, is characterized by a deposition of
calcium pyrophosphate dehydrate in the periarticular tissues. Dagregorio and
Saint-Cast13 reported a case with tumoral calcium pyrophosphate
dehydrate deposition disease similar to the localization of our case. Tophaceus
pseudogout is usually seen in elderly patients rather than middle-aged
patients, as in our case. A crystalline appearance under a polarizing
microscope is a characteristic of tophaceus pseudogout. In our case, tophaceus
pseudogout was eliminated by the histological findings of excised material.
Finally, soft tissue chondromas are commonly seen in the
hand, especially in middle-aged adults. These tumors are usually solitary and
may adhere to tendons or joint capsules, but soft tissue chondromas, as in our
case, can be easily differentiated by histological findings.
Surgical excision in tumoral calcinosis is the
mainstream treatment; recurrence is rare. Kirk and Simon14 and
Harkess and Peters8 reported the recurrence of tumoral calcinosis
around the shoulder and elbow. These recurrences may have originated from
incomplete excision. Researchers advocate meticulous excision. In our case, the
reason for failure of the previous excision may have been an incomplete
Tumoral calcinosis should be considered in differential
diagnoses of patients with painful masses in the hand. Its cure is possible
with early and complete surgical excision.
- Kim HS, Suh JS, Kim YH, Park SH. Tumoral calcinosis of the hand:
three unusual cases with painful swelling of small joints. Arch Pathol Lab
Med. 2006; 130(4):548-551.
- Sebesta A, Kamineni S, Dumont CE. Idiopathic tumoral calcinosis of
the index finger. Case report. Scand J Plast Reconstr Surg Hand Surg.
- Murai S, Matsui M, Nakamura A. Tumoral calcinosis in both index
fingers: a case report. Scand J Plast Reconstr Surg Hand Surg. 2001;
- Kamath BJ, Pinto D, Sharma C. Tumoral calcinosis of hand: a rare
location with unusual presentation. Int J Orthop Surg. 2006; 3(3):22.
- Duret M. Tumeurs multiples et singulaires desbourses sereuses.
Bull Mem Soc Anat Paris. 1899; 74:725-732.
- Inclan A. Tumoral calcinosis. JAMA. 1943; 121:490-495.
- Weiss SW, Goldblum JR. Soft Tissue Tumors. 4th ed. St Louis,
MO: CV Mosby; 2001.
- Harkess JW, Peters HJ. Tumoral calcinosis. A report of six cases.
J Bone Joint Surg Am. 1967; 49(4):721-731.
- Topaz O, Shurman DL, Bergman R. Mutations in GALNT3, encoding a
protein involved in O-linked glycosylation, cause familial tumoral calcinosis.
Nat Genet. 2004; 36(6):579-581.
- Smack D, Norton SA, Fitzpatrick JE. Proposal for a pathogenesis-based
classification of tumoral calcinosis. Int J Dermatol. 1996;
- Chen WS, Eng HL. Tumoral calcinosis after thumb tip injury: case
report. J Trauma. 1995; 38(6):952-954.
- Foose TE, Simon AE, Strauch RJ. Acute calcific deposition adjacent to
the metacarpal head: report of two cases and review of the literature.
Orthopedics. 2005; 28(8):798-800.
- Dagregorio G, Saint-Cast Y. Tumoral calcium pyrophosphate dihydrate
crystal deposition in the fifth metacarpophalangeal region. Orthopedics.
- Kirk TS, Simon MA. Tumoral calcinosis. Report of a case with
successful medical management. J Bone Joint Surg Am. 1981;
Drs Ozcelik, Aydogdu, and Sur are from the Department of
Orthopedics and Traumatology, and Dr Doganavsargil is from the Pathology
Department, Ege University Hospital, Izmir, Turkey.
Drs Ozcelik, Aydogdu, Doganavsargil, and Sur have no
relevant financial relationships to disclose.
Correspondence should be addressed to: Semih Aydogdu,
MD, Ege University Medical School Department of Orthopedic Surgery and
Traumatology, Bornova, 35100, Izmir, Turkey.