Orthopedics

Case Reports 

Chondromyxoid Fibroma Involving the Metacarpophalangeal Joint

Rachel L. Slotcavage, MD; Brendan C. Dickson, MD; Christian M. Ogilvie, MD

Abstract

Chondromyxoid fibroma is a rare benign bony tumor classically occurring in the metaphyseal region of the long bones surrounding the knee, but also found with relative frequency in other long bones, the pelvis, ribs, and small foot bones. The small bones of the hand, however, are rarely involved. The tumor is considered a physeal plate remnant and may involve the epiphysis, diaphysis, or both along with its metaphyseal origin. It may cause cortical expansion and destruction, but consistently respects the periosteal boundary.

We report a case of histopathologically confirmed chondromyxoid fibroma involving a metacarpal, proximal phalanx, and synovial tissues of the metacarpophalangeal joint. A 29-year-old woman presented with pain, weakness, and a mass of the right ring finger metacarpophalangeal joint that had persisted since prior surgery to remove an extraosseous chondroma. Prominent soft tissue swelling and limited range of motion were noted, corresponding to magnetic resonance imaging findings of lytic changes in the right ring finger metacarpal and proximal phalanx, surrounded by an irregularly enhancing mass with soft tissue extension. Open biopsy revealed calcified cartilaginous synovial deposits suspicious for synovial chondromatosis, with erosion into surrounding bones. Histologic examination revealed an unusual chondromyxoid fibroma with joint involvement. The patient is >15 months out from synovectomy, intralesional curettage, and placement of demineralized bone matrix and allograft cancellous chips, and is without signs of recurrence. This benign, curable lesion should be included in the differential diagnosis of a bony lesion, even when a joint and adjacent bones are involved.

Chondromyxoid fibroma is a rare, benign bone lesion accounting for <1% of all bony tumors.1-9 It was first described in 1948 by Jaffe and Lichtenstein,10 who differentiated the histologic findings from that of chondrosarcoma and enchondroma. Since then, approximately 700 cases have been reported in the literature, primarily in long bones and the small bones of the foot. However, <30 reported cases involve the hand, and none of those document joint involvement. We present what we believe to be the first case of chondromyxoid fibroma involving a metacarpal, the adjacent phalanx, and the connecting synovial tissues of the metacarpophalangeal joint.

A 29-year-old woman presented with moderate dull pain, weakness, and a mass of the metacarpophalangeal joint of the right ring finger for over 2 years. She had similar symptoms approximately 3 years prior, at which time she presented to another hospital and underwent excision of a mass via an extended palmar Z incision. Final pathologic diagnosis was reported as “consistent with extraosseous chondroma” and slides were not available for review. The patient reported persistent pain unchanged since this procedure, but increasing weakness and swelling. There was no history of trauma and no symptoms suggestive of infection.

Physical examination revealed no visible mass but prominent soft tissue swelling in the anteroposterior dimension. The patient had tenderness to palpation over the metacarpophalangeal joint and a painful, limited active range of motion of 25° to 45°. Radiographs of the right hand revealed a lobulated but geographic lytic lesion of the metacarpal head of the ring finger and the base of the adjacent proximal phalanx with surrounding sclerosis and cortical destruction (Figure 1). Magnetic resonance imaging showed an irregularly enhancing mass, dark on T1-weighted and bright on T2-weighted images. There were lytic changes in the head of the ring finger metacarpal and the base of the proximal phalanx with extension into the soft tissues of the joint, but no involvement of the surrounding tendons (Figure 2). Laboratory tests documented an elevated erythrocyte sedimentation rate and C-reactive protein with negative rheumatologic antibody titers.

An open biopsy was performed using a dorsal approach to avoid complications from scar tissue from…

Abstract

Chondromyxoid fibroma is a rare benign bony tumor classically occurring in the metaphyseal region of the long bones surrounding the knee, but also found with relative frequency in other long bones, the pelvis, ribs, and small foot bones. The small bones of the hand, however, are rarely involved. The tumor is considered a physeal plate remnant and may involve the epiphysis, diaphysis, or both along with its metaphyseal origin. It may cause cortical expansion and destruction, but consistently respects the periosteal boundary.

We report a case of histopathologically confirmed chondromyxoid fibroma involving a metacarpal, proximal phalanx, and synovial tissues of the metacarpophalangeal joint. A 29-year-old woman presented with pain, weakness, and a mass of the right ring finger metacarpophalangeal joint that had persisted since prior surgery to remove an extraosseous chondroma. Prominent soft tissue swelling and limited range of motion were noted, corresponding to magnetic resonance imaging findings of lytic changes in the right ring finger metacarpal and proximal phalanx, surrounded by an irregularly enhancing mass with soft tissue extension. Open biopsy revealed calcified cartilaginous synovial deposits suspicious for synovial chondromatosis, with erosion into surrounding bones. Histologic examination revealed an unusual chondromyxoid fibroma with joint involvement. The patient is >15 months out from synovectomy, intralesional curettage, and placement of demineralized bone matrix and allograft cancellous chips, and is without signs of recurrence. This benign, curable lesion should be included in the differential diagnosis of a bony lesion, even when a joint and adjacent bones are involved.

Chondromyxoid fibroma is a rare, benign bone lesion accounting for <1% of all bony tumors.1-9 It was first described in 1948 by Jaffe and Lichtenstein,10 who differentiated the histologic findings from that of chondrosarcoma and enchondroma. Since then, approximately 700 cases have been reported in the literature, primarily in long bones and the small bones of the foot. However, <30 reported cases involve the hand, and none of those document joint involvement. We present what we believe to be the first case of chondromyxoid fibroma involving a metacarpal, the adjacent phalanx, and the connecting synovial tissues of the metacarpophalangeal joint.

Case Report

A 29-year-old woman presented with moderate dull pain, weakness, and a mass of the metacarpophalangeal joint of the right ring finger for over 2 years. She had similar symptoms approximately 3 years prior, at which time she presented to another hospital and underwent excision of a mass via an extended palmar Z incision. Final pathologic diagnosis was reported as “consistent with extraosseous chondroma” and slides were not available for review. The patient reported persistent pain unchanged since this procedure, but increasing weakness and swelling. There was no history of trauma and no symptoms suggestive of infection.

Physical examination revealed no visible mass but prominent soft tissue swelling in the anteroposterior dimension. The patient had tenderness to palpation over the metacarpophalangeal joint and a painful, limited active range of motion of 25° to 45°. Radiographs of the right hand revealed a lobulated but geographic lytic lesion of the metacarpal head of the ring finger and the base of the adjacent proximal phalanx with surrounding sclerosis and cortical destruction (Figure 1). Magnetic resonance imaging showed an irregularly enhancing mass, dark on T1-weighted and bright on T2-weighted images. There were lytic changes in the head of the ring finger metacarpal and the base of the proximal phalanx with extension into the soft tissues of the joint, but no involvement of the surrounding tendons (Figure 2). Laboratory tests documented an elevated erythrocyte sedimentation rate and C-reactive protein with negative rheumatologic antibody titers.

Figure 1: Preoperative AP radiograph of the metacarpophalangeal joint of the patient’s right ring finger shows the lobulated, lytic lesion involving both the metacarpal and proximal phalanx Figure 2A: Preoperative MRI sections show the patient’s right ring finger metacarpophalangeal joint lesion Figure 2B: Preoperative MRI sections show the patient’s right ring finger metacarpophalangeal joint lesion Figure 2C: Preoperative MRI sections show the patient’s right ring finger metacarpophalangeal joint lesion
Figure 1: Preoperative AP radiograph of the metacarpophalangeal joint of the patient’s right ring finger shows the lobulated, lytic lesion involving both the metacarpal and proximal phalanx. Soft tissue involvement is also noted. Figure 2: Preoperative MRI sections show the patient’s right ring finger metacarpophalangeal joint lesion. The lesion appears dark on T1 (A) and bright on fat-suppressed T2 (B), and demonstrates irregular enhancement after administration of gadolinium contrast (C).

An open biopsy was performed using a dorsal approach to avoid complications from scar tissue from the patient’s prior surgery. The incision was made centrally over the joint with subsequent division of the extensor tendons. The synovium was noted to be involved by grossly calcified cartilaginous deposits. These were removed with care given not to disrupt the collateral ligaments. Secondary to these findings, an intraoperative diagnosis of synovial chondromatosis was suspected. The lesions in the metacarpal head and proximal phalanx were then extensively curetted and irrigated. Both lesions were carefully packed with demineralized bone matrix and cancellous allograft chips, which proved challenging due to the fragility of the remaining cortical bone. Examination of the joint surface revealed articular cartilage loss at the edges of the joint, likely due to the extensive tumor involvement of the synovium, but adequate cartilage coverage remained centrally. Postoperative examination under anesthesia revealed full passive range of motion of all joints of the ring finger. Intraoperative cultures were negative and the patient experienced no complications from the surgery.

On pathologic examination, the specimen grossly consisted of multiple irregular fragments of rubbery tan–yellow to gray–white tissue. Histologic examination revealed a neoplasm that was variably comprised of nodules of maturing cartilage (Figure 3A) and lobules of spindle cells. The latter were admixed with occasional stellate cells and contained a prominent myxoid background (Figure 3B). Within the spindle cell regions were rare moderately enlarged and hyperchromatic cells (Figure 3B, inset). The periphery of the lobules was partially rimmed by areas of osteoclast–type giant cells (Figure 3B, arrows). There was focal dystrophic calcification. There was no evidence of mitotic activity, necrosis, or lymphovascular space invasion. Despite the unusual location, the morphologic features were indicative of the diagnosis of chondromyxoid fibroma.

Figure 3A: Photomicrographs of the patient’s chondromyxoid fibroma demonstrate foci of various stages of cartilaginous differentiation Figure 3B: Photomicrographs of the patient’s chondromyxoid fibroma demonstrate foci of various stages of cartilaginous differentiation Figure 4: AP radiograph of the patient’s right hand was taken 4 months postoperatively
Figure 3: Photomicrographs of the patient’s chondromyxoid fibroma demonstrate foci of various stages of cartilaginous differentiation (A; hematoxylin-eosin 200), lobules of spindle cells rimmed by osteoclast–type giant cells (B; arrows; hematoxylin-eosin 200), and enlarged hyperchromatic cells (B; inset; hematoxylin-eosin 400). Figure 4: AP radiograph of the patient’s right hand was taken 4 months postoperatively. There is no evidence of recurrence or loss of bone graft signal.

At 9-month follow-up, the patient was experiencing occasional sharp, activity-related pain, but reported no night pain or functional limitations. Physical examination revealed a slightly limited but improved and painless range of motion at the metacarpophalangeal joint, measured to be 10° to 70°, and slightly diminished but functional grip strength. Postoperative radiographs taken at 4 months showed no new lytic lesions and no loss of bone graft signal (Figure 4).

Discussion

Accounting for >1% of all biopsy-proven bone lesions, chondromyxoid fibroma occurs classically as a metaphyseal lesion of long bones surrounding the knee, with the proximal tibia being the most common site of occurrence.2,4-8,11-14 The tumor has also been noted with relative frequency in other long bones, the pelvis, ribs, and the small bones of the foot.1,2,5,7,8,11-14 Mandibular involvement has been reported with some frequency, but due to differences in histologic features is thought to be a separate process of odontogenic origin.4 Although small bone involvement accounts for approximately 19% of all lesions,4,9,11-13 chondromyxoid fibroma is rarely found distal to the carpus. Large case series and individual case reports document only 11 metacarpal lesions and 14 cases of upper extremity phalangeal involvement.2,6,7,11-25 An isolated case of carpal involvement in the trapezoid bone also exists.26

Patients with chondromyxoid fibroma are generally in their second or third decade of life, with between 50% to 82% of patients falling in this age range.1,3,4,6,9,13,14 A slight male predominance is normally agreed upon, but male-to-female ratios between 1:1 and 2:1 are found in the literature.1-8,11-14,27 The most common presenting symptoms are pain in up to 100% of cases, and swelling in up to 75%.5,27 Swelling is more likely to be noted when areas of tumor involvement have minimal soft tissue coverage, such as in the hands and feet.

Radiographic findings in chondromyxoid fibroma are consistent with a benign process, but vary depending on the bone involved. Overall, the tumors will appear lytic, round to elongated, and sharply defined with a sclerotic rim and frequently scalloped borders, and may appear to have gross trabeculations.2-5,8,11,14 There is rarely radiologic evidence of calcification, though histologic calcification is not uncommon.2,3,5,9,11,13,27 Reactive new bone formation may occur in up to 39% of cases.12 In long bones, the classic eccentric metaphyseal location is most clearly visible. However, expansile full-width and even full-bone involvement is noted in the small and flat bones.3,5,9,11,12,20,28

Due to its histologic combination of chondroid, fibrous, and myxoid components, it has been suggested that chondromyxoid fibroma is related to physeal development.2,3,28,29 This impression is enhanced by the generally metaphyseal location when present in a long bone. Initial reports documented the tumor’s respect for the epiphyseal plate,3,30 but it was later reported to cross even open growth plates, involving both the metaphysis and epiphysis in a number of cases.1,2,4,5,11,12,28,31 Cases have been documented to extend fully through the epiphysis to the articular cortex.12 This is especially true in the small bones; Wu et al13 documented a rate of epiphyseal involvement in the small tubular bones of 42%. Although the capability of the tumor to involve multiple areas of a bone has now been recognized, the literature is still limited to cases of single bone involvement. Dahlin’s1 case series contained a large metatarsal lesion causing mechanical erosion of the adjacent phalanx. Wilson et al12 described the erosion of another metatarsal lesion into the metatarsophalangeal joint, causing loose body formation. However, true tumor involvement of adjacent bones and the connecting synovial tissues seen in our patient has not heretofore been described.

The gross appearance of the tumor tissue has been described as grayish- to bluish-white, glistening, lobulated, and firm, and may strongly represent its chondroid origins.1-3,5,6,8,11,14,27 Histologically, stellate or spindle-shaped cells are arranged in a lobular pattern on a background of most commonly myxoid, fibrous, or least commonly chondroid matrix.3,5,10,11,13,28 Areas fitting all of these descriptions may be found within a single lesion, in varying proportions.1,4,5,10,11,14,27,28 The lobular appearance is made of hypocellular central areas with a condensation of nuclei at the periphery of each pseudolobule.1,11,13,14,28 Benign-appearing cells with oval nuclei may be present in the interlobular areas, along with blood vessels and multinucleated giant cells.2-4,10,13 Mitotic figures and bizarre cytological features, such as hyperchromatic nuclei, pleomorphism, and nuclear vacuolization, may also be seen,2,4,9-11 with a higher percentage of cellular atypia found in the small bones.13 It is due to these features that chondromyxoid fibroma is sometimes misdiagnosed as a malignant lesion, though it has only rare mitoses.3,11,14 Aneurysmal bone cysts may also be present within the lesion.11,13

Treatment options for chondromyxoid fibroma include en bloc resection, simple curettage, and curettage with bone grafting or polymethylmethacrylate (PMMA) placement. Simple curettage offers the least morbidity but the highest risk of recurrence, with rates between 20% and 80% documented in the literature.2,3,5-7 This recurrence is thought to be a result of residual tumor left after surgical removal. En bloc resection has an almost negligible recurrence rate, but is associated with high morbidity and marked functional loss depending on the location of the tumor. Some studies advocate curettage combined with placement of corticocancellous bone graft or PMMA and report a low recurrence rate for these procedures.2,5,7 Overall recurrence rate for chondromyxoid fibroma, independent of surgical technique, is approximately 20%.1,2,4-8,11,13,27 Before presentation to our center, our patient initially underwent excision of a soft tissue lesion, which recurred within 1 year.

Conclusion

Chondromyxoid fibroma is a rare bony tumor which, although commonly found in the foot, involves the bones of the hand infrequently. Initially considered to respect the physeal scar, the tumor is now recognized to cross that boundary and involve the epiphyseal or diaphyseal regions, but is considered not to violate the periosteum. Our case was a previously unreported incident in which a chondromyxoid fibroma crossed the metacarpophalangeal joint of the hand, involving both the metacarpal and proximal phalanx as well as the synovial tissues. This suggests that the behavior of this tumor may not yet be fully defined, and one must thus include this benign, curable lesion on the differential diagnosis even when a joint and adjacent bones are involved.

References

  1. Dahlin DC. Chondromyxoid fibroma of bone, with emphasis on its morphological relationship to benign chondroblastoma. Cancer. 1956; 9(1):195-203.
  2. Schajowicz F, Gallardo H. Chondromyxoid fibroma (fibromyxoid chondroma) of bone. A clinico-pathological study of thirty-two cases. J Bone Joint Surg Br. 1971; 53(2):198-216.
  3. Rahimi A, Beabout JW, Ivins JC, Dahlin DC. Chondromyxoid fibroma: a clinicopathologic study of 76 cases. Cancer. 1972; 30(3):726-736.
  4. Beggs IG, Stoker DJ. Chondromyxoid fibroma of bone. Clin Radiol. 1982; 33(6):671-679.
  5. Gherlinzoni F, Rock M, Picci P. Chondromyxoid fibroma. The experience at the Istituto Ortopedico Rizzoli. J Bone Joint Surg Am. 1983; 65(2):198-204.
  6. Unni KK. Chondromyxoid fibroma. In: Unni KK, ed. Dahlin’s Bone Tumors: General Aspects and Data on 11,087 Cases. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1996:59-69.
  7. Lersundi A, Mankin HJ, Mourikis A, Hornicek FJ. Chondromyxoid fibroma: a rarely encountered and puzzling tumor. Clin Orthop Relat Res. 2005; (439):171-175.
  8. Dorfman HD, Czerniak B. Benign cartilage lesions. In: Dorfman HD, Czerniak B, eds. Bone Tumors. St. Louis, MO: CV Mosby; 1998:253-352.
  9. Feldman F, Hecht HL, Johnston AD. Chondromyxoid fibroma of bone. Radiology. 1970; 94(2):249-260.
  10. Jaffe HL, Lichtenstein L. Chondromyxoid fibroma of bone; a distinctive benign tumor likely to be mistaken especially for chondrosarcoma. Arch Pathol (Chic). 1948; 45(4):541-551.
  11. Zillmer DA, Dorfman HD. Chondromyxoid fibroma of bone: thirty-six cases with clinicopathologic correlation. Hum Pathol. 1989; 20(10):952-964.
  12. Wilson AJ, Kyriakos M, Ackerman LV. Chondromyxoid fibroma: radiographic appearance in 38 cases and in a review of the literature. Radiology. 1991; 179(2):513-518.
  13. Wu CT, Inwards CY, O’Laughlin S, Rock MG, Beabout JW, Unni KK. Chondromyxoid fibroma of bone: a clinicopathologic review of 278 cases. Hum Pathol. 1998; 29(5):438-446.
  14. Campanacci M. Chondromyxoid fibroma. In: Campanacci M, ed. Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. New York, NY: Springer; 1999:265-277.
  15. Daghfous M, Charfi H, Turki M, Maalla R, Baccari S, Tarhouni L. Chondromyxoid fibroma. A case report of location in the phalanx of the thumb [in French]. Chir Main. 2007; 26(3):159-164.
  16. Atalar H, Basarir K, Uras I, Yildiz Y, Erekul S, Saglik Y. Chondromyxoid fibroma: an evaluation of 11 patients [in Turkish]. Acta Orthop Traumatol Turc. 2007; 41(1):31-35.
  17. Yamamoto T, Mizuno K. Chondromyxoid fibroma of the finger. Kobe J Med Sci. 2000; 46(1-2):29-32.
  18. Milliez PY, Thomine JM. Rare benign bone tumors and dystrophy in the hand. Review of the literature and report of four cases. Ann Chir Main. 1988; 7(3):189-201.
  19. Anderson WJ, Bowers WH. Chondromyxoid fibroma of the proximal phalanx. A tumor that may be confused with chondrosarcoma. J Hand Surg Br. 1986; 11(1):144-146.
  20. Nalbantoglu U, Aktas S, Peker KR. Chondromyxoid fibroma involving the entire metacarpal: a case report. J Hand Surg Am. 2005; 30(5):1083-1086.
  21. Hau MA, Fox EJ, Rosenberg AE, Mankin HJ. Chondromyxoid fibroma of the metacarpal. Skeletal Radiol. 2001; 30(12):719-721.
  22. Strauch RJ, Kleinman WB. Chondromyxoid fibroma of a metacarpal: a case report and review of the literature. J Hand Surg Am. 1996; 21(2):293-295.
  23. Declerck GM, Rawlings ID, Hunt AC. Chondromyxoid fibroma in the metacarpal bone of the thumb. Acta Orthop Belg. 1992; 58(2):216-220.
  24. Patryn A. Chondromyxoid fibroma of the metacarpal bone [in Polish]. Chir Narzadow Ruchu Ortop Pol. 1974; 39(5):655-658.
  25. Fourquet F, Pusel J, Jouffroy J, Forest G. A case of metacarpal chondro-myxoid fibroma [in French]. Chirurgie. 1973; 99(10):749-750.
  26. Blair WF, Robinson RA, Buckwalter JA. Chondromyxoid fibroma in a carpal bone. Clin Orthop Relat Res. 1983; (188):199-202.
  27. Desai SS, Jambhekar NA, Samanthray S, Merchant NH, Puri A, Agarwal M. Chondromyxoid fibromas: a study of 10 cases. J Surg Oncol. 2005; 89(1):28-31.
  28. Schutt PG, Frost HM. Chondromyxoid fibroma. Clin Orthop Relat Res. 1971; (78):323-329.
  29. Steiner GC. Ultrastructure of benign cartilaginous tumors of intraosseous origin. Hum Pathol. 1979; 10(1):71-86.
  30. Jaffe HL. Tumors and Tumorous Conditions of the Bones and Joints. Philadelphia, PA: Lea & Febiger; 1961.
  31. Ralph LL. Chondromyxoid fibroma of bone. J Bone Joint Surg Br. 1962; (44):7-24.

Authors

Drs Slotcavage and Ogilvie are from the Department of Orthopedic Surgery, University of Pennsylvania, and Dr Dickson is from the Department of Pathology and Laboratory Medicine, Pennsylvania Hospital, Philadelphia, Pennsylvania.

Dr Slotcavage received a research fellowship from Stryker Orthopaedics. Drs Dickson and Ogilvie have no relevant financial relationships to disclose.

Correspondence should be addressed to: Christian M. Ogilvie, MD, Garfield Duncan Bldg, Ste 2C, 301 S 8th St, Philadelphia, PA 19106-6192.

10.3928/01477447-20090401-15

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