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Giant cell reparative granuloma in the hand: Report of three cases and review of the literature
Giant cell reparative granuloma in the hand: Report of three cases and review of the literature Occurrence of giant cell reparative granuloma (GCRG) outside the jaw, skull, or facial bones is unusual. Three recent cases of GCRG occurring in the hand are presented. This lesion appears on radiographs as a lucent, expansile defect in bone, similar in appearance to enchondroma, giant cell tumor of bone , aneurysmal bone cyst , or brown tumor of hyperparathyroidism. Histologically, GCRG is characterized by spindle-shaped, fibroblastic stoma with scattered, small, angulated giant cells and areas of osteoid formation around foci of hemorrhage. Establishing the pathologic diagnosis is frequently difficult. Misdiagnosis occurred in approximately half of the reported cases and in one of our cases. The typical clinical presentation is that of localized pain and swelling. GCRG is not a neoplastic disorder; however, it recurred locally in a third of the reported cases. Recommended treatment for GCRG is thorough removal of the diseased tissues and grafting of autologous bone. (J HAND SURG 10A:733-9, 1985.)
Robert L. Merkow, M.D. , Manjula Bansal, M.D ., and Allen E. Inglis , M.D. , New York, N .Y.
A
few reports in the literature describe giant cell reparative granuloma (GCRG) occurring in the hand . 1-5 In 1953 Jaffe" found a group of apparently reactive , intraosseous lesions in the mandible and maxilla that contained prominent giant cells. He coined the term " giant cell reparative granuloma" and differentiated it clinically and histologically from brown tumor of hyperparathyroidism and other giant cell lesion s that occur in bone. Jaffe felt that these lesions repre sented a benign, nonneoplastic reparative response to intraosseous hemorrhage. In 1962 Ackerman and Spjut , I and subsequently others , reported the occurrence of similar " giant cell react ive " lesions in the bones of the hands and feet. In 1980 Lorenzo and Dorfman" reported eight cases of GCRG occurring in the tubular bones of the hands and feet. They described the biologic behavior and distinctive pathologic characteristics of this lesion . Their report emphasizes the differential diagnostic featur es of GCRG in relation to other giant cell-containing lesions known to occur in the hands and feet . Three patients with GCRG were recently seen and treated at the Hospital for Special Surgery. Diagnosis was made in each case by histologic examination of surgically curetted specimens. The first case was initially diagnosed as From the Hospital for Special Surgery, New York, N.Y. Receivedfor publication Aug. 3, 1984; accepted inrevised form Oct. 9,1984.
Reprint requests: Allen E. Inglis, M.D., 535 E. 70th St., New York, NY 10021.
atypical giant cell tumor with features of aneurysmal bone cyst. When the tumor recurred 17 months later and the microscopic specimens were reexamined , the diagnosis was revised to GCRG.
Case reports Case 1. A 16-year-old girl presented with pain and swelling over the metacarpal bone of her right ring finger. The symptoms had been present for 8 weeks and the onset was related to an incident of minor trauma. The swelling, which was located over the dorsum of the ring finger's metacarpal bone, improved with rest and elevation. General physical examination was within normal limits. The right hand was swollen dorsally over the distal half of the metacarpal bone of the ring finger. Minimal tenderness was present. Range of motion, circulation, motor power, and sensibility were normal. Laboratory findings including serum calcium, phosphorus, and alkaline phosphatase values were normal. The initial xray films revealed a clear, lucent, expanded lesion that extended to the end-plate of the fourth metacarpal head (Fig. I , A and B). There were no intralesional calcifications. There was, however, evidence of previous nondisplaced fracture with periosteal reaction along the shaft. Surgical excision, careful curettage , irrigation, and bone grafting with bank allograft were performed. Pathologic interpretation was atypical giant cell tumor with features of aneurysmal bone cyst. The patient' s right hand was immobilized for 5 weeks after the operation, and full motion and normal function were subsequently achieved. X-ray films at 3 months showed the lesion to be "healed in" and at 5 months, completely healed with remodeling of the metacarpal (Fig. I, C). Seventeen months after the operation, the patient again presented with recurrent pain and swelling that was not THE JOURNAL OF HAND SURGERY
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The Journal of HAND SURGERY
Fig. 1. A-B, Initial films, anteroposterior and oblique views, demonstrate a clear, lucent, expanded lesion in the metacarpal head of the ring finger. There is evidence of previous nondisplaced fracture with periosteal reaction along the shaft. C, Radiographic appearance 5 months after curettage and bone grafting of the lesion with apparent healing of the defect. D, X-ray film demonstrates a recurrent, lucent lesion in the ring finger's metacarpal 17 months after surgery. E, Oblique radiograph at most recent follow-up shows healing of the defect.
caused by trauma. Films taken at this time revealed recurrence of a lucent, expanded lesion (Fig . I, D) . A second surgical procedure consisted of curettage and removal of all apparent lesional tissue with a high-speed burr. The defect was packed tightly with autogenous, cancellous , iliac bone graft. Review of the pathologic specimen from both the first and second curettings was consistent with GeRG, and the initial diagnosis was revised. Two years after the second procedure the patient
had no symptoms or evidence of recurrence. An x-ray film showed healing of the defect (Fig . I, E) . Case 2. A 46-year-old, right-handed woman presented with pain and mild swelling in her right thumb . She first noticed these symptoms after playing tennis. She recalled no particular incident of injury. General physical examination was normal. The right thumb was slightly swollen and tender to firm palpation along the dorsoradial aspect of the proximal
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Fig. 2. A·B, Anteroposterior and lateral x-ray films of the proximal phalanx of the thumb show an elongated, eccentric . lucent defect . There are no intralesional calcifications. C·D, Anteroposterior and lateral x-ray films showing healing of the defect after cortical cancellous bone grafting.
phalanx of the right thumb. The muscle strength , sensibility. and circulation were normal. Mobility of the metacarpophalangeal and interphalangeal joints was normal. Laboratory data revealed normal levels of calcium, phosphorus, and alkaline phosphatase. X-ray films of the thumb showed an elongated, eccentric, lucent lesion along the dorsoradial aspect of the proximal phalanx of the thumb (Fig. 2, A and B) . Mild expansion and cortical thinning were present. There were no calcifications or periosteal reaction . The lesion was excised . curetted, and grafted with autologous bone. The histologic examination revealed GCRG. The patient's right thumb was immobilized for 6 weeks after surgery. Pain-free motion was subsequently achieved. Followup at 14 months revealed healing of the bony defect without recurrence (Fig . 2. C and D) . Case 3. A l4-year-old boy twisted his right long finger and thus sustained a pathologic fracture through a lucent bone cyst in the base of the proximal phalanx of his long finger. He was treated with "buddy taping" and plaster immobilization of the minimally displaced fracture. Two months after injury, the fracture had healed, but the lucent lesion had increased in size. Mild pain and swelling persisted and the boy was admitted for biopsy, curettage, and bone grafting. The general physical examination was within normal lim-
its. The right long finger showed fusiform swelling with mild limitation of motion of all three joints . The proximal phalanx was slightly tender. The neurovascular status was normal and laboratory studies were normal. X-ray films taken at the time of surgery showed an expanded lytic lesion occupying the proximal two thirds of the proximal phalanx of the long finger (Fig . 3, A and B). There was some formation of new periosteal bone, but no interlesional calcifications. Surgical excision was performed through a dorsal approach. The lesion was removed with curettes and a power burr. A bank bone allograft was inserted into the defect and the finger was immobilized for 5 weeks. The histologic examination revealed findings consistent with GCRG. Radiographically confirmed bone healing was achieved by 3 months (Fig . 3, C and D) . The patient regained normal function and no recurrence was noted at 2 years follow-up .
Clinical history and examination Typical localized pain and swelling characterized the clinical presentation in these three patients and those reported in the literature (Table I). One quarter of such patients have a history of pain after an incident of minor trauma. The duration of symptoms before recognition
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Fig. 3. A-B, Anteroposterior and oblique x-ray films show an expanded, lucent defect occupying the proximal two thirds of the proximal phalanx of the long finger. There is some formation of new periosteal bone. CoD, Anteroposterior and lateral films demonstrate healing of the defect and remodeling of the phalanx 3 months after surgery.
Table I. Giant cell reparative granuloma in the hand: Summary of clinical data Difficulty making diagnosis
BO
Yes (17)
Yes
+
BO
No
No
FaD at 24 mo after second C + BO FaD at 18 mo
C
+
BO
No
No
FaD at 24 mo
Yes
C
+
BO
Yes (8)
Yes
10 yr
No
C
Yes (15)
Yes
Unknown
No
C
Yes (18)
No
Unknown
No
C
No
Yes
FaD after ray resection' Areas of ABC ray resection" Second C + BO FaD at 5 yr'
6 wk
No
C
+
BO
No
No
FaD at 9 yr'
7 mo
No
C
+
BO
No
No
FaD at 9 yr'
3 mo
No
C
No
Yes
FaD at II mo'
Unknown Unknown
C + BO Distal amputation
No No
Yes No
FaD'
Sex
16
F
Ring finger metacarpal
2 mo
Yes
C
+
2
46
F
6 mo
No
C
3
14
M
2 mo
Yes
4
9'/2
M
Thumb proximal phalanx Long finger proximal phalanx Ring finger proximal phalanx Long finger metacarpal Long finger metacarpal Small finger metacarpal Long finger middie phalanx Long finger proximal phalanx Long finger metacarpal Middle phalanx Distal phalanx
3 mo
Case
5
23
M
6
6
M
7
19
M
8
25
F
9
19
F
10
21
M
11 12
35 10
F F
Location in hand
Recurrence (mo)
Age (yr)
Duration of symptoms
Unknown Unknown
Trauma
Treatment
C + BG. curettage and bone graft; FOD, free of disease; C. curettage; ABC. aneurysmal bone cyst.
Comments
Vol lOA, No.5 September 1985
Giant cell reparative granuloma in the hand
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Fig. 4. A, High-power photomicrograph of curetted tissue from a GCRG. Small, irregularly shaped giant cells are scattered in a fibrous stroma. A few mononuclear inflammatory cells are present. (Hematoxylin and eosin; original magnification x 200.) B, High-power photomicrograph of a typical giant cell tumor of bone. The giant cells are large, round, multinucleated, and evenly distributed. The stroma consists of scant, plump, ovoid cells. (Hematoxylin and eosin; original magnification x 200.)
of the bone lesion ranged from 6 weeks to 10 years. Persistent signs and symptoms led to the radiographic diagnosis of a lytic bone lesion. Swelling and tenderness were commonly present, but a palpable mass was frequently absent. Nerve and tendon function was normal and motion was only minimally decreased as a result of swelling and/or prior immobilization. None of the patients had any evidence of systemic disease. Demographic and anatomic features In the combined group of reported cases (Table I), the patients' ages ranged from 6 to 46 years (average 20 years). There were equal numbers of female and male patients. Of the 12 reported cases of GCRG occurring in the hand, bony locations numbered five in the metacarpals and seven in the phalanges. Roentgenographic findings All lesions were solitary, lucent defects of the metaphyseal or diaphyseal regions of the metacarpals and phalanges. Expansion and cortical thinning are common features; however, there is little evidence of bone destruction and no surrounding sclerosis. Pathologic fractures may occur, as in one of our cases. Intralesional calcification does not occur. Radiographic differential diagnosis should include giant cell tumor of bone, aneu-
rysmal bone cyst, enchondroma, and brown tumor of hyperparathyroidism. Pathologic features Grossly, tissue from GCRG is friable, hemorrhagic, and grayish-brown in appearance. The microscopic appearance is that of a benign lesion, with the rare presence of mitoses. The stroma is predominately composed of unremarkable spindle-shaped fibroblasts," with scattered lymphocytes, histiocytes, and areas of fibrosis. The giant cells are characteristically seen throughout the lesion, but are more abundant in areas of hemorrhage (Fig. 4, A). The giant cells are small and irregular in contrast to those seen in giant cell tumor of bone, where they are large, round, and evenly distributed amidst plump, ovoid stromal cells (Fig. 4, B). Foci of new bone formation are frequently present in areas of hemorrhage (Table II). Discussion Ackerman and Spjut' initially described giant cell reaction of bone occurring in the hands and feet in 1962. To date 12 cases, including the three in this report, occurring in the hands have been reported. In 1980 Lorenzo and Dorfman" reported eight cases (of which three were in the hand) of GCRG of the short, tubular
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Table II. Histologic differentiation of GCRG from other lesions in bone containing giant cells Giant cell tumor with aneurysmal bone cyst
GCRG*
Giant cell tumor of bone
1. Giant cells are present in clusters around foci of recent and old hemorrhage, creating a granulomatous-like picture 2. Giant cells are small, irregularly shaped, and unevenly distributed; they contain few (4-8) nuclei 3. The stroma is abundant and composed of unremarkable spindle cells, fibrosis, and lymphocytic infiltrates; mitoses are rare 4. Reactive new bone formation is frequent occurrence, appearing as intralesional osteoid bone around areas of hemorrhage
1. Giant cells are uniformly distributed; recent hemorrhage is occasionally present
1. Giant cells are focally clustered
2. Giant cells are large, round, and evenly distributed; they contain many nuclei 3. The stromal cells are plump and oval shaped; usually no lymphocytes are present
2. Giant cells may be round or irreg-
4. No osteoid or new bone formation characteristically occurs
around large blood-filled channels
ular
3. The stromal cells may be slender or plumb with admixed hemosiderin deposits and large vascular channels 4. Reactive bone formation and osteoid bone may be occasionally seen
*Brown tumor of hyperparathyroidism is histologically indistinguishable from OeRO; clinical and laboratory tests are needed to differentiate these lesions.
bones of the hands and feet. They emphasized that the lesion was histologically indistinguishable from GCRG that occurs in the jaw as originally described by Jaffe" in 1953. They pointed out microscopic and ultrastructural differences in this nonneoplastic process as compared with other lesions of bone that contain giant cells. GCRG can be histologically differentiated from giant cell tumor of bone by its prominent, spindle-shaped fibroblastic stroma, granulomatous-like arrangement around foci of hemorrhage, smaller and more irregularly shaped giant cells, and evidence of both recent and old hemorrhage around foci of osteoid (Table 11). Aneurysmal bone cysts may be difficult to differentiate from GCRG. As in other lesions, aneurysmal bone cyst is a diagnosis of exclusion and is considered secondary when foci of GCRG are present. Jaffe, as well as Lorenzo and Dorfman, pointed out that GCRG is histologically indistinguishable from the brown tumor that occurs in hyperparathyroidism. Laboratory determinations of the serum, calcium, phosphorus, and alkaline phosphatase as well as parathyroid hormone should be obtained to rule out hyperparathyroidism. The etiology of GCRG is unknown. However, because its biologic and pathologic features are similar to those of aneurysmal bone cyst, Lorenzo and Dorfman hypothesized that these two lesions may represent related reactive responses to intraosseous hemorrhage. Antecedent trauma was present in only one quarter of the reported cases. Primary treatment of GCRG was curettage alone, or curettage in conjunction with bone grafting in 11 of the reported cases, and distal amputation in the remaining case.
In contradistinction to the reported high recurrence rate (greater than 90%) after curettage of giant cell tumors of bone in the hand," the recurrence rate for GCRG was 33% (four of 12 cases). Amputation was required in two of these cases because of extensive recurrence. A second curettage and autologous' bone grafting produced a favorable result in the remaining two cases of recurrence. GCRG is uncommon, but should be considered in the differential diagnosis of a lytic expansile lesion occurring in bones of the hand. It occurs most commonly in the 15 to 25-year-old group, but may appear in younger and older patients. Localized pain and swelling characterize the typical clinical presentation, and a pathologic fracture through a lytic lesion is occasionally encountered. The diagnosis is made on the basis of histologic appearance of the curetted specimen in the absence of hyperparathyroidism. Accurate differentiation from giant cell tumor of bone and brown tumor of hyperparathyroidism is of significant clinical importance because of their different biologic behaviors, prognosis, and treatments. The recurrence rate for GCRG is significant (33%); however, careful curettage and bone grafting or ray amputation for extensive recurrent disease have been curative. REFERENCES 1. Ackerman LV, Spjut HJ: Tumors of bone and cartilage.
In Atlas of tumor pathology, section II, fasicle 4. Washington, DC, 1962, Armed Forces Institute of Pathology 2. Jernstrom P, Stark HH: Giant cell reaction of a metacarpal. Am J Clin Pathol 55:77-81, 1971
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3. D'Alonzo RT, Pitcock JA , Milford LW: Giant cel1reaction of bone . J Bone Joint Surg [Am] 54:1267-71, 1972 4. Lorenzo JD, Dorfman HD: Giant cel1 reparative granuloma of short tubular bones of the hands and feet. Am J Surg PathoI4:551-63 , 1980 5. Bertheussen KJ. Holck S. Schiodt T: Giant cell lesion of bone of the hand with particular emphasis on giant cel1 reparative granuloma . J HAND SURG 8:46-9, 1983
Giant cell reparative granuloma in the hand
6. Jaffe HL: Giant cel1 reparative granuloma, traumatic bone cyst and fibrous (fibro-osseous) dysplasia of the jaw bones . Oral Surg 6:159-75, 1953 7. Averill RM, Smith RJ, Campbell CJ: Giant cel1 tumors of the bone s of the hand. J HAND SURG 5:39-50, 1980 8. Hirschel S, Katz A: Giant cel1 reparative granuloma outside the jaw bone. Hum Pathol 5: 171-81, 1974
Tumoral calcinosis in multiple digits: A case report Tumoral calcinosis usually appears in the vicinity of large joints, and its appearance in the fingers must be quite rare since no reports of such an occurrence have been found. A case report of the presence of tumoral masses of hydroxyapatite in multiple digits is presented. No causative or related metabolic or collagen-vascular disease was identified, and the patient remains in good health. (J HAND S URG 10A:739-43, 1985.)
George P. Bogumill, Ph.D., M.D., and Robert J. Lloyd, M.D. , Washington, D.C.
Tumoral calcinosis is an uncommon condition of unknown etiology that is characterized by soft tissue masses of hydroxyapatite crystals located in periarticular tissues, usuaIly adjacent to larger joints. 1-3 It has been reported in all age-groups and appears to be more prevalent among blacks than among other ethnic groups. We report an unusual case of tumoral calcinosis presenting as multiple large calcified subcutaneous nodules involving the fingers rather than larger joints.
Case report Mrs. D. J. L. . a 50-year-old white woman, noted the gradual enlargement of an indurated mass at the tip of the left small finger in 1977. Radiographs at that time revealed calcific depo sits in the soft tissues of her finger, as wel1 as early depo sits in the left thumb and right long finger. In Augu st 1978, a skin biopsy, which apparently was not deep enough to enter the subcutaneous mass was performed, and the results were negative . A clinical diagnosis of calcinosis From the Department of Orthopaedic Surgeryand the Department of Medicine, Division of Rheumatology, Georgetown University School of Medicine, Washington, D.C. Received for publication May 8, 1984; accepted in revised form Dec. 13, 1984. Reprint requests: George P. Bogumill , Ph.D., M.D., Professor of Orthopaedic Surgery, Georgetown University Schoolof Medicine, 3800 ReservoirRd., N.W., Washington, D.C. 20007.
cutis was made . Further work-up in 1978 showed a weakl y positive antinuclear antibody titer of 1/80 and a sedimentation rate of 22 mm/hr. No therapy was instituted . During the next 5 years , the patient developed additional slowly enlarging, indurated areas on the right thumb, the left long finger, and the right long and small fingers. She stated that when the masses initial1y begin , they swel1 rapidly and are quite tender and inflamed. When the inflammation subsides, the masses remain rather sensitive, but she has no aching. She denied any pain and was unaware of any restriction of motion in the interphalangeal (IP) joints of either hand . She also denied symptoms referable to the metacarpophalangeal joints or wrists. She has not had problems in any of her larger jo ints or feet. The patient had symptoms of lower back pain with stiffne ss and radiation to the left thigh in 1983. This episode was treated with naproxen (Naprosyn) and local steroid injections, and had entirely resolved before she was initial1y evaluated by us in April 1983 . She was unaware of any change in the texture of her skin and had noted no rnyalgias or muscle weakness . She considered her muscle strength normal. On several recent occasions , she stuck a needle into one or more of the nodule s and was able to extrude a white creamy materi al similar to toothpaste. The patient is one of 12 children. One sister has been diagno sed as having scleroderma, and a maternal grandmother had severe deforming arthriti s, possibl y rheumatoid. The family had no history of gout or kidney stones, lupus erythemato sus, abnormalities of calcium metabol ism, or any primary muscular or neurologic disease. Her past medical his-
THE JOURNAL OF HAND SURGERY
739
Robert L. Merkow, M.D. , Manjula Bansal, M.D ., and Allen E. Inglis , M.D. , New York, N .Y.
A
few reports in the literature describe giant cell reparative granuloma (GCRG) occurring in the hand . 1-5 In 1953 Jaffe" found a group of apparently reactive , intraosseous lesions in the mandible and maxilla that contained prominent giant cells. He coined the term " giant cell reparative granuloma" and differentiated it clinically and histologically from brown tumor of hyperparathyroidism and other giant cell lesion s that occur in bone. Jaffe felt that these lesions repre sented a benign, nonneoplastic reparative response to intraosseous hemorrhage. In 1962 Ackerman and Spjut , I and subsequently others , reported the occurrence of similar " giant cell react ive " lesions in the bones of the hands and feet. In 1980 Lorenzo and Dorfman" reported eight cases of GCRG occurring in the tubular bones of the hands and feet. They described the biologic behavior and distinctive pathologic characteristics of this lesion . Their report emphasizes the differential diagnostic featur es of GCRG in relation to other giant cell-containing lesions known to occur in the hands and feet . Three patients with GCRG were recently seen and treated at the Hospital for Special Surgery. Diagnosis was made in each case by histologic examination of surgically curetted specimens. The first case was initially diagnosed as From the Hospital for Special Surgery, New York, N.Y. Receivedfor publication Aug. 3, 1984; accepted inrevised form Oct. 9,1984.
Reprint requests: Allen E. Inglis, M.D., 535 E. 70th St., New York, NY 10021.
atypical giant cell tumor with features of aneurysmal bone cyst. When the tumor recurred 17 months later and the microscopic specimens were reexamined , the diagnosis was revised to GCRG.
Case reports Case 1. A 16-year-old girl presented with pain and swelling over the metacarpal bone of her right ring finger. The symptoms had been present for 8 weeks and the onset was related to an incident of minor trauma. The swelling, which was located over the dorsum of the ring finger's metacarpal bone, improved with rest and elevation. General physical examination was within normal limits. The right hand was swollen dorsally over the distal half of the metacarpal bone of the ring finger. Minimal tenderness was present. Range of motion, circulation, motor power, and sensibility were normal. Laboratory findings including serum calcium, phosphorus, and alkaline phosphatase values were normal. The initial xray films revealed a clear, lucent, expanded lesion that extended to the end-plate of the fourth metacarpal head (Fig. I , A and B). There were no intralesional calcifications. There was, however, evidence of previous nondisplaced fracture with periosteal reaction along the shaft. Surgical excision, careful curettage , irrigation, and bone grafting with bank allograft were performed. Pathologic interpretation was atypical giant cell tumor with features of aneurysmal bone cyst. The patient' s right hand was immobilized for 5 weeks after the operation, and full motion and normal function were subsequently achieved. X-ray films at 3 months showed the lesion to be "healed in" and at 5 months, completely healed with remodeling of the metacarpal (Fig. I, C). Seventeen months after the operation, the patient again presented with recurrent pain and swelling that was not THE JOURNAL OF HAND SURGERY
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The Journal of HAND SURGERY
Fig. 1. A-B, Initial films, anteroposterior and oblique views, demonstrate a clear, lucent, expanded lesion in the metacarpal head of the ring finger. There is evidence of previous nondisplaced fracture with periosteal reaction along the shaft. C, Radiographic appearance 5 months after curettage and bone grafting of the lesion with apparent healing of the defect. D, X-ray film demonstrates a recurrent, lucent lesion in the ring finger's metacarpal 17 months after surgery. E, Oblique radiograph at most recent follow-up shows healing of the defect.
caused by trauma. Films taken at this time revealed recurrence of a lucent, expanded lesion (Fig . I, D) . A second surgical procedure consisted of curettage and removal of all apparent lesional tissue with a high-speed burr. The defect was packed tightly with autogenous, cancellous , iliac bone graft. Review of the pathologic specimen from both the first and second curettings was consistent with GeRG, and the initial diagnosis was revised. Two years after the second procedure the patient
had no symptoms or evidence of recurrence. An x-ray film showed healing of the defect (Fig . I, E) . Case 2. A 46-year-old, right-handed woman presented with pain and mild swelling in her right thumb . She first noticed these symptoms after playing tennis. She recalled no particular incident of injury. General physical examination was normal. The right thumb was slightly swollen and tender to firm palpation along the dorsoradial aspect of the proximal
Vol lOA, No.5
September 1985
Giant cell reparative granuloma in the hand
735
Fig. 2. A·B, Anteroposterior and lateral x-ray films of the proximal phalanx of the thumb show an elongated, eccentric . lucent defect . There are no intralesional calcifications. C·D, Anteroposterior and lateral x-ray films showing healing of the defect after cortical cancellous bone grafting.
phalanx of the right thumb. The muscle strength , sensibility. and circulation were normal. Mobility of the metacarpophalangeal and interphalangeal joints was normal. Laboratory data revealed normal levels of calcium, phosphorus, and alkaline phosphatase. X-ray films of the thumb showed an elongated, eccentric, lucent lesion along the dorsoradial aspect of the proximal phalanx of the thumb (Fig. 2, A and B) . Mild expansion and cortical thinning were present. There were no calcifications or periosteal reaction . The lesion was excised . curetted, and grafted with autologous bone. The histologic examination revealed GCRG. The patient's right thumb was immobilized for 6 weeks after surgery. Pain-free motion was subsequently achieved. Followup at 14 months revealed healing of the bony defect without recurrence (Fig . 2. C and D) . Case 3. A l4-year-old boy twisted his right long finger and thus sustained a pathologic fracture through a lucent bone cyst in the base of the proximal phalanx of his long finger. He was treated with "buddy taping" and plaster immobilization of the minimally displaced fracture. Two months after injury, the fracture had healed, but the lucent lesion had increased in size. Mild pain and swelling persisted and the boy was admitted for biopsy, curettage, and bone grafting. The general physical examination was within normal lim-
its. The right long finger showed fusiform swelling with mild limitation of motion of all three joints . The proximal phalanx was slightly tender. The neurovascular status was normal and laboratory studies were normal. X-ray films taken at the time of surgery showed an expanded lytic lesion occupying the proximal two thirds of the proximal phalanx of the long finger (Fig . 3, A and B). There was some formation of new periosteal bone, but no interlesional calcifications. Surgical excision was performed through a dorsal approach. The lesion was removed with curettes and a power burr. A bank bone allograft was inserted into the defect and the finger was immobilized for 5 weeks. The histologic examination revealed findings consistent with GCRG. Radiographically confirmed bone healing was achieved by 3 months (Fig . 3, C and D) . The patient regained normal function and no recurrence was noted at 2 years follow-up .
Clinical history and examination Typical localized pain and swelling characterized the clinical presentation in these three patients and those reported in the literature (Table I). One quarter of such patients have a history of pain after an incident of minor trauma. The duration of symptoms before recognition
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Fig. 3. A-B, Anteroposterior and oblique x-ray films show an expanded, lucent defect occupying the proximal two thirds of the proximal phalanx of the long finger. There is some formation of new periosteal bone. CoD, Anteroposterior and lateral films demonstrate healing of the defect and remodeling of the phalanx 3 months after surgery.
Table I. Giant cell reparative granuloma in the hand: Summary of clinical data Difficulty making diagnosis
BO
Yes (17)
Yes
+
BO
No
No
FaD at 24 mo after second C + BO FaD at 18 mo
C
+
BO
No
No
FaD at 24 mo
Yes
C
+
BO
Yes (8)
Yes
10 yr
No
C
Yes (15)
Yes
Unknown
No
C
Yes (18)
No
Unknown
No
C
No
Yes
FaD after ray resection' Areas of ABC ray resection" Second C + BO FaD at 5 yr'
6 wk
No
C
+
BO
No
No
FaD at 9 yr'
7 mo
No
C
+
BO
No
No
FaD at 9 yr'
3 mo
No
C
No
Yes
FaD at II mo'
Unknown Unknown
C + BO Distal amputation
No No
Yes No
FaD'
Sex
16
F
Ring finger metacarpal
2 mo
Yes
C
+
2
46
F
6 mo
No
C
3
14
M
2 mo
Yes
4
9'/2
M
Thumb proximal phalanx Long finger proximal phalanx Ring finger proximal phalanx Long finger metacarpal Long finger metacarpal Small finger metacarpal Long finger middie phalanx Long finger proximal phalanx Long finger metacarpal Middle phalanx Distal phalanx
3 mo
Case
5
23
M
6
6
M
7
19
M
8
25
F
9
19
F
10
21
M
11 12
35 10
F F
Location in hand
Recurrence (mo)
Age (yr)
Duration of symptoms
Unknown Unknown
Trauma
Treatment
C + BG. curettage and bone graft; FOD, free of disease; C. curettage; ABC. aneurysmal bone cyst.
Comments
Vol lOA, No.5 September 1985
Giant cell reparative granuloma in the hand
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Fig. 4. A, High-power photomicrograph of curetted tissue from a GCRG. Small, irregularly shaped giant cells are scattered in a fibrous stroma. A few mononuclear inflammatory cells are present. (Hematoxylin and eosin; original magnification x 200.) B, High-power photomicrograph of a typical giant cell tumor of bone. The giant cells are large, round, multinucleated, and evenly distributed. The stroma consists of scant, plump, ovoid cells. (Hematoxylin and eosin; original magnification x 200.)
of the bone lesion ranged from 6 weeks to 10 years. Persistent signs and symptoms led to the radiographic diagnosis of a lytic bone lesion. Swelling and tenderness were commonly present, but a palpable mass was frequently absent. Nerve and tendon function was normal and motion was only minimally decreased as a result of swelling and/or prior immobilization. None of the patients had any evidence of systemic disease. Demographic and anatomic features In the combined group of reported cases (Table I), the patients' ages ranged from 6 to 46 years (average 20 years). There were equal numbers of female and male patients. Of the 12 reported cases of GCRG occurring in the hand, bony locations numbered five in the metacarpals and seven in the phalanges. Roentgenographic findings All lesions were solitary, lucent defects of the metaphyseal or diaphyseal regions of the metacarpals and phalanges. Expansion and cortical thinning are common features; however, there is little evidence of bone destruction and no surrounding sclerosis. Pathologic fractures may occur, as in one of our cases. Intralesional calcification does not occur. Radiographic differential diagnosis should include giant cell tumor of bone, aneu-
rysmal bone cyst, enchondroma, and brown tumor of hyperparathyroidism. Pathologic features Grossly, tissue from GCRG is friable, hemorrhagic, and grayish-brown in appearance. The microscopic appearance is that of a benign lesion, with the rare presence of mitoses. The stroma is predominately composed of unremarkable spindle-shaped fibroblasts," with scattered lymphocytes, histiocytes, and areas of fibrosis. The giant cells are characteristically seen throughout the lesion, but are more abundant in areas of hemorrhage (Fig. 4, A). The giant cells are small and irregular in contrast to those seen in giant cell tumor of bone, where they are large, round, and evenly distributed amidst plump, ovoid stromal cells (Fig. 4, B). Foci of new bone formation are frequently present in areas of hemorrhage (Table II). Discussion Ackerman and Spjut' initially described giant cell reaction of bone occurring in the hands and feet in 1962. To date 12 cases, including the three in this report, occurring in the hands have been reported. In 1980 Lorenzo and Dorfman" reported eight cases (of which three were in the hand) of GCRG of the short, tubular
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Table II. Histologic differentiation of GCRG from other lesions in bone containing giant cells Giant cell tumor with aneurysmal bone cyst
GCRG*
Giant cell tumor of bone
1. Giant cells are present in clusters around foci of recent and old hemorrhage, creating a granulomatous-like picture 2. Giant cells are small, irregularly shaped, and unevenly distributed; they contain few (4-8) nuclei 3. The stroma is abundant and composed of unremarkable spindle cells, fibrosis, and lymphocytic infiltrates; mitoses are rare 4. Reactive new bone formation is frequent occurrence, appearing as intralesional osteoid bone around areas of hemorrhage
1. Giant cells are uniformly distributed; recent hemorrhage is occasionally present
1. Giant cells are focally clustered
2. Giant cells are large, round, and evenly distributed; they contain many nuclei 3. The stromal cells are plump and oval shaped; usually no lymphocytes are present
2. Giant cells may be round or irreg-
4. No osteoid or new bone formation characteristically occurs
around large blood-filled channels
ular
3. The stromal cells may be slender or plumb with admixed hemosiderin deposits and large vascular channels 4. Reactive bone formation and osteoid bone may be occasionally seen
*Brown tumor of hyperparathyroidism is histologically indistinguishable from OeRO; clinical and laboratory tests are needed to differentiate these lesions.
bones of the hands and feet. They emphasized that the lesion was histologically indistinguishable from GCRG that occurs in the jaw as originally described by Jaffe" in 1953. They pointed out microscopic and ultrastructural differences in this nonneoplastic process as compared with other lesions of bone that contain giant cells. GCRG can be histologically differentiated from giant cell tumor of bone by its prominent, spindle-shaped fibroblastic stroma, granulomatous-like arrangement around foci of hemorrhage, smaller and more irregularly shaped giant cells, and evidence of both recent and old hemorrhage around foci of osteoid (Table 11). Aneurysmal bone cysts may be difficult to differentiate from GCRG. As in other lesions, aneurysmal bone cyst is a diagnosis of exclusion and is considered secondary when foci of GCRG are present. Jaffe, as well as Lorenzo and Dorfman, pointed out that GCRG is histologically indistinguishable from the brown tumor that occurs in hyperparathyroidism. Laboratory determinations of the serum, calcium, phosphorus, and alkaline phosphatase as well as parathyroid hormone should be obtained to rule out hyperparathyroidism. The etiology of GCRG is unknown. However, because its biologic and pathologic features are similar to those of aneurysmal bone cyst, Lorenzo and Dorfman hypothesized that these two lesions may represent related reactive responses to intraosseous hemorrhage. Antecedent trauma was present in only one quarter of the reported cases. Primary treatment of GCRG was curettage alone, or curettage in conjunction with bone grafting in 11 of the reported cases, and distal amputation in the remaining case.
In contradistinction to the reported high recurrence rate (greater than 90%) after curettage of giant cell tumors of bone in the hand," the recurrence rate for GCRG was 33% (four of 12 cases). Amputation was required in two of these cases because of extensive recurrence. A second curettage and autologous' bone grafting produced a favorable result in the remaining two cases of recurrence. GCRG is uncommon, but should be considered in the differential diagnosis of a lytic expansile lesion occurring in bones of the hand. It occurs most commonly in the 15 to 25-year-old group, but may appear in younger and older patients. Localized pain and swelling characterize the typical clinical presentation, and a pathologic fracture through a lytic lesion is occasionally encountered. The diagnosis is made on the basis of histologic appearance of the curetted specimen in the absence of hyperparathyroidism. Accurate differentiation from giant cell tumor of bone and brown tumor of hyperparathyroidism is of significant clinical importance because of their different biologic behaviors, prognosis, and treatments. The recurrence rate for GCRG is significant (33%); however, careful curettage and bone grafting or ray amputation for extensive recurrent disease have been curative. REFERENCES 1. Ackerman LV, Spjut HJ: Tumors of bone and cartilage.
In Atlas of tumor pathology, section II, fasicle 4. Washington, DC, 1962, Armed Forces Institute of Pathology 2. Jernstrom P, Stark HH: Giant cell reaction of a metacarpal. Am J Clin Pathol 55:77-81, 1971
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3. D'Alonzo RT, Pitcock JA , Milford LW: Giant cel1reaction of bone . J Bone Joint Surg [Am] 54:1267-71, 1972 4. Lorenzo JD, Dorfman HD: Giant cel1 reparative granuloma of short tubular bones of the hands and feet. Am J Surg PathoI4:551-63 , 1980 5. Bertheussen KJ. Holck S. Schiodt T: Giant cell lesion of bone of the hand with particular emphasis on giant cel1 reparative granuloma . J HAND SURG 8:46-9, 1983
Giant cell reparative granuloma in the hand
6. Jaffe HL: Giant cel1 reparative granuloma, traumatic bone cyst and fibrous (fibro-osseous) dysplasia of the jaw bones . Oral Surg 6:159-75, 1953 7. Averill RM, Smith RJ, Campbell CJ: Giant cel1 tumors of the bone s of the hand. J HAND SURG 5:39-50, 1980 8. Hirschel S, Katz A: Giant cel1 reparative granuloma outside the jaw bone. Hum Pathol 5: 171-81, 1974
Tumoral calcinosis in multiple digits: A case report Tumoral calcinosis usually appears in the vicinity of large joints, and its appearance in the fingers must be quite rare since no reports of such an occurrence have been found. A case report of the presence of tumoral masses of hydroxyapatite in multiple digits is presented. No causative or related metabolic or collagen-vascular disease was identified, and the patient remains in good health. (J HAND S URG 10A:739-43, 1985.)
George P. Bogumill, Ph.D., M.D., and Robert J. Lloyd, M.D. , Washington, D.C.
Tumoral calcinosis is an uncommon condition of unknown etiology that is characterized by soft tissue masses of hydroxyapatite crystals located in periarticular tissues, usuaIly adjacent to larger joints. 1-3 It has been reported in all age-groups and appears to be more prevalent among blacks than among other ethnic groups. We report an unusual case of tumoral calcinosis presenting as multiple large calcified subcutaneous nodules involving the fingers rather than larger joints.
Case report Mrs. D. J. L. . a 50-year-old white woman, noted the gradual enlargement of an indurated mass at the tip of the left small finger in 1977. Radiographs at that time revealed calcific depo sits in the soft tissues of her finger, as wel1 as early depo sits in the left thumb and right long finger. In Augu st 1978, a skin biopsy, which apparently was not deep enough to enter the subcutaneous mass was performed, and the results were negative . A clinical diagnosis of calcinosis From the Department of Orthopaedic Surgeryand the Department of Medicine, Division of Rheumatology, Georgetown University School of Medicine, Washington, D.C. Received for publication May 8, 1984; accepted in revised form Dec. 13, 1984. Reprint requests: George P. Bogumill , Ph.D., M.D., Professor of Orthopaedic Surgery, Georgetown University Schoolof Medicine, 3800 ReservoirRd., N.W., Washington, D.C. 20007.
cutis was made . Further work-up in 1978 showed a weakl y positive antinuclear antibody titer of 1/80 and a sedimentation rate of 22 mm/hr. No therapy was instituted . During the next 5 years , the patient developed additional slowly enlarging, indurated areas on the right thumb, the left long finger, and the right long and small fingers. She stated that when the masses initial1y begin , they swel1 rapidly and are quite tender and inflamed. When the inflammation subsides, the masses remain rather sensitive, but she has no aching. She denied any pain and was unaware of any restriction of motion in the interphalangeal (IP) joints of either hand . She also denied symptoms referable to the metacarpophalangeal joints or wrists. She has not had problems in any of her larger jo ints or feet. The patient had symptoms of lower back pain with stiffne ss and radiation to the left thigh in 1983. This episode was treated with naproxen (Naprosyn) and local steroid injections, and had entirely resolved before she was initial1y evaluated by us in April 1983 . She was unaware of any change in the texture of her skin and had noted no rnyalgias or muscle weakness . She considered her muscle strength normal. On several recent occasions , she stuck a needle into one or more of the nodule s and was able to extrude a white creamy materi al similar to toothpaste. The patient is one of 12 children. One sister has been diagno sed as having scleroderma, and a maternal grandmother had severe deforming arthriti s, possibl y rheumatoid. The family had no history of gout or kidney stones, lupus erythemato sus, abnormalities of calcium metabol ism, or any primary muscular or neurologic disease. Her past medical his-
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