|We would like to report an unusual presentation of giant cell tumor
of tendon sheath (GCTTS) at the distal interphalangeal (DIP) joints
of the hand. Giant cell tumors of tendon sheath are the second most
common benign tumors of hand, simple ganglion cysts being the most
common. Usual presentation is painless masses presenting for a long
time. Occasionally they can cause distal numbness or mild disability
secondary to the size of lesion. Peak age of occurrence is between 30
and 50 years, often seen in women than in men . The etiology is
unknown. There is a 50% association with trauma and the most widely
accepted theory is that GCTTS is a reactive or regenerative hyperplasia
associated with an inflammatory process . Surgical excision is the
treatment of choice. Symptoms of the disease are non specific; hence
clinical knowledge of the presentation of GCTTS may be beneficial to
|A 51-year old Indian male, a heavy vehicle driver, was referred to
rheumatology clinic from General Practitioner with history of painless
swellings at the distal interphalangeal (DIP) joints of both hands with a
referral diagnosis of nodal osteoarthritis. The swellings were insidiously
growing for the past six years and did not interfere with hand
movements and functions. There was no history of preceding trauma
and there were no similar swellings elsewhere in the body. There were
no constitutional symptoms such as night sweats, fever, loss of appetite
or weight loss. The patient was not obese and there was no history of
diabetes or dislipidemia. His systemic review was normal. Examination
of hand revealed 3x4 mm in size lobulated, nodular swellings located
on dorsal aspect of all digits adjacent to DIP joints (Figure 1) [Patient’s
consent was obtained for publication]. Skin over the swelling was
normal. Nodules were smooth, soft to firm, nontender, not adherent to
the overlying skin, but held in place by the basement structures. There
was no transillumination through the swelling. There was normal
sensory perception in the digit and range of motion was normal in the related joints. The patient’s blood parameters (complete blood count,
blood sugar, indicators of acute phase response, lipid profile, renal
profile, liver profile and serology) were normal. Plain radiography of
hands showed soft tissue swellings, with no erosion or calcification with
preserved joint space. The differential diagnosis at this point was nodal
osteoarthritis. The patient was referred for excision biopsy.
|Pathology showed a firm yellowish white piece of tissue measuring
4x5x5 mm. Paraffin blocks were made, glass slides were prepared
and processed with Hematoxylin and Eosin stains. Histopathology
showed a lobulated well circumscribed partially encapsulated tumor.
The bulk of the tumor is composed of small, round to spindle shaped
mononuclear cells with pink cytoplasm and round, often grooved
nuclei with inconspicuous nucleoli. Many osteoclast-like giant cells
were present containing a variable number of nuclei from 4 to 20 nuclei
per cell. The nuclear characteristics of the giant cells were similar to
the mononuclear cells. Haemosiderin deposits were focally present. The
background stroma showed collagen bands in areas and hyalinization
in other areas (Figure 2). No mitotic figures or necrosis were identified.
A diagnosis of GCTTS was made. Because of the benign nature of the lesion and preserved hand functions, patient refused to undergo
surgical excision for rest of the lesions. Patient was followed up for a
period of three years. There was no sign recurrence of excised tumor.
|The localized form of giant cell tumors is the most common subset
of giant cell tumors. It has been also called tenosynovial giant cell tumor
and nodular tenosynovitis and consists principally of a proliferation of
synovial cells arising from a tendon sheath. It occurs at any age, but
usually between 30 and 50 years, with 2:1 female predominance .
They occur predominantly in the hand were they probably represent
the second most common benign tumors of hand, simple ganglion
cysts being the most common. The majority occur in the fingers in close
proximity to the synovium of the tendon sheath of interphalangeal joint.
The lesions may infrequently erode or infiltrate the nearby bone  or
rarely involve the skin. Other sites of involvement include the wrist,
ankle, foot, knee and very rarely the elbow and the hip . The tumors
develop gradually over a long period of time. Antecedent trauma
is reported in a variable number of cases (1 to 50%) [2,3]. The most
common presenting symptom is that of a painless swelling, however
GCTTS might cause compression neuropathy .
|Our patient had a unique presentation of GCTTS by the involvement
at all the DIP joints of both hands. He had no history of trauma and
the disease process started at the age of 45 years which is usual age of
onset in this lesion. The clinical differential diagnosis of a swelling of
DIP includes ganglion cysts, foreign body granuloma, epidermoid cyst,
mucous cyst, sebaceous cyst, lipoma, nodal osteoarthritis, sarcoidosis,
psoriatic arthritis, multicentric reticulohistiocytosis, tendinous
xanthoma, fibroma of tendon sheath, infection, rheumatoid nodule,
gout, cavernous hemangioma and amyloidoma [5,6].
|The tumor is a benign condition with a capacity of local recurrence.
Local excision is the treatment of choice. Complete excision is difficult
as the mass is associated with the tendon sheath of the joint. Four
to thirty percent of cases recur [7,8] but these recurrences are nondestructive
and are controlled by surgical excision. It has been suggested
the recurrences develop most often in highly cellular tumors or lesions
with a high mitotic activity. Al- Qattan , in his review found that
encapsulated tumors do not recur, where as uncapsulated tumors have a tendency to recur. Other factors such as poor surgical technique and
bony invasion of the tumors also favor recurrences. Kotwal et al. 
reported the importance of a classification system to identify patients
with high risk of recurrence and a favorable outcome of postoperative
radiotherapy for those groups of patients with high risk of recurrence.
The patients with high risk either showed mitotic figures in histology or
were those with possible incomplete excision. The patient in our report
didn’t come under this category.
|Presentation of GCTTS involving all the DIP joints of both
hands simulating nodal OA is unusual. Clinicians should include the
possibility of GCTTS among differential diagnosis of swellings in
interphalangeal joints that resemble osteoarthritis of hands.
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- Kotwal PP, Gupta V, Malhotra R (2000) Giant-cell tumour of the tendon sheath. Is radiotherapy indicated to prevent recurrence after surgery? J Bone Joint Surg Br 82: 571-573.