SCIENTIFIC ARTICLE

Chondroblastoma-Like Chondroma of the Hand: Case Report Tomas K. Kuprys, MD, Randip Bindra, MD, Dariusz Borys, MD, Lukas M. Nystrom, MD

Chondroblastoma-like chondroma is a rare tumor that almost exclusively manifests in the hand and presents with a set of unique diagnostic features. This tumor has been described in only a limited number of publications and is thus commonly omitted in the differential diagnosis of hand tumors. The diagnostic imaging and histological specimens may initially appear consistent with a number of relatively more common hand tumors, thereby delaying the diagnosis. We present the case of a 49-year-old woman with chondroblastoma-like chondroma of the hand treated with marginal excision. The physical examination, imaging, and needle biopsy made an initial case for giant cell tumor of tendon sheath until histological analysis of the excised specimen yielded the correct diagnosis. (J Hand Surg Am. 2014;39(5):933e936. Copyright Ó 2014 by the American Society for Surgery of the Hand. All rights reserved.) Key words Case reports, chondroblastoma-like, chondroma, hand, neoplasms.

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calcifying masses that can occur in the hand.1e3 The chondroblastoma-like chondroma (CLC) variant is rare, with only 12 cases reported, 11 of which were in the hand.4e8 Here we describe a case of CLC that presented as a hand mass in a 49-year-old woman. The aim of this report was to highlight this rare cartilaginous tumor that has a range of clinical, radiographic, and histopathological features that may confuse the diagnosis with more aggressive neoplasms.5 OFT TISSUE CHONDROMAS ARE

CASE REPORT A 49-year-old woman presented with a 4-day history of left palm fullness that caused pain with grip. She recalled striking her hand on a door 6 months earlier but From the Departments of Orthopaedic Surgery and Rehabilitation, Loyola University Medical Center, Maywood, IL. Received for publication November 18, 2013; accepted in revised form January 28, 2014. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. The authors acknowledge Dr. Laurie Lomasney of the Department of Radiology, Loyola University Medical Center, for providing invaluable radiographic expertise in the production of this report. Corresponding author: Tomas K. Kuprys, MD, Departments of Orthopaedic Surgery & Rehabilitation, Loyola University Medical Center, Maguire Building 105, Suite 1700, 2160 S 1st Ave., Maywood, IL 60153-3328; e-mail: [email protected]. 0363-5023/14/3905-0014$36.00/0 http://dx.doi.org/10.1016/j.jhsa.2014.01.046

did not notice any swelling or deformity at that time. No constitutional symptoms were present. Her medical history was notable for recently diagnosed diabetes, as well as a remote history of breast cancer that had been treated 12 years before without recurrence. On examination, there was a mass in the first web area with normal overlying skin. The mass was palpable, well-defined, firm, and approximately 4 cm in diameter. The mass was fixed, was not associated with warmth or tenderness, was not pulsatile, and had a negative Tinel sign. Digital motion was unimpaired, but the patient complained of discomfort with thumb opposition and grip. Sensation in the index finger and thumb were preserved. Plain radiographs demonstrated a well-circumscribed 1.8  1.1-cm soft tissue mass containing calcification adjacent to the index metacarpal (Fig. 1). Magnetic resonance T2 fat-saturation (fs) imaging demonstrated a 2-cm mass with heterogeneous, predominantly high T2 signal characteristics deep and just distal to the adductor pollicis with an internal signal void, consistent with calcification, and abundant marginal inflammation (Fig. 2). No marrow changes were present in the adjacent index metacarpal. Following infusion of gadolinium, there was moderate heterogeneous enhancement through the majority of the lesion (Fig. 2). Needle biopsy samples showed spindle cell proliferation with osteoclast-like giant cells favoring a

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Published by Elsevier, Inc. All rights reserved.

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FIGURE 1: Radiograph demonstrates a well-circumscribed lesion with a stippled and chondroid matrix pattern of calcification (dashed outline).

diagnosis of giant cell tumor of tendon sheath (GCTTS). The patient underwent a marginal excision for presumed GCTTS through a palmar oblique incision made directly over the mass. The mass was closely associated with the index finger metacarpal periosteum, metacarpophalangeal joint capsule, and radial sesamoid bone (Fig. 3). There was a distinct capsular plane around the tumor with a prominent deep palmar arch branch vessel supplying the tumor. The blood vessel was ligated, and the tumor measuring 2.5  2.4  2.4 cm was excised en bloc. Gross analysis revealed a yellow-tan well-circumscribed solid nodule. Histological sections showed cellular areas of polygonal to oval chondroblast-like cells with eosinophilic cytoplasm and peripheral nuclei that were mostly oval in shape with some notching and prominent nucleoli. Whereas these cells surrounded lobules of immature and mature hyaline cartilage, chondrocytes forming mainly hyalinized cartilage were round, were less eosinophilic, and had elliptical and hyperchromatic nuclei without nucleoli. Peripheral metaplastic ossification and scant osteoclast-like giant cells bordered a number of cartilaginous nodules. Focal calcium pyrophosphate dihydrate crystal deposits in hyaline cartilage were identified. No cytological atypia, necrosis, or increased mitotic figures were present. All these findings were consistent with a diagnosis of CLC (Figs. 4e7). Following surgery, the patient was placed into a soft dressing, encouraged to use her hand as tolerated, and at 10 days, was allowed light activity. The wound healed, the patient regained full mobility of the hand, J Hand Surg Am.

FIGURE 2: A Magnetic resonance T2 fs coronal and axial images of the thenar eminence confirms demarcated heterogeneous high T2 signal lesion (arrow) with regional nonspecific inflammation. B Magnetic resonance T1 fs axial image following intravenous infusion of contrast confirms a lesion with moderate heterogeneous enhancement (arrow).

and 6 weeks after surgery, she had returned to fullduty nursing. Fifteen months following the excision, there was no clinical or radiographic evidence of recurrence of the tumor, and the patient remained free of symptoms. DISCUSSION Soft tissue chondromas represent 1.5% of benign soft tissue tumors and predominantly occur in the extremities with up to 64% occurring in fingers.8,9 No prevalence data exist for the CLC subtype although 1 series reported immature chondroblastoma-like cellular elements in 47% of soft tissue chondroma specimens, all of which occurred in the hand.5 The average age of patients was 46 years (range, 28e71 y) without a sex predilection; all reported cases were treated with marginal excision.5e8 Although no cohort recurrence rate data are published, a case of local hand recurrence at 9 months following excision exists.5 A skull-based CLC was found to have local r

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FIGURE 5: Immature cartilage lobules (arrows) juxtaposed to osteoclast-like giant cells in the setting of spindle to oval chondroblastoma-like cells (magnification  100).

FIGURE 3: Intraoperative demonstration of the tumor during marginal excision.

FIGURE 6: Maturing cartilage lobules with focal calcification (arrow; magnification  200).

FIGURE 4: Proliferation of polygonal to oval chondroblastomalike cells surrounding immature cartilage (dashed outline) with scant osteoclast-like giant cells and peripheral metaplastic ossification (asterisks; magnification  50).

recurrence after 7 months; but given differences in skull and hand anatomy, it should not be generalized to hand-based CLC.4 Metastasis is not a documented attribute of CLC. Soft tissue chondromas have been described with and without attachment to periosteum, joint capsule, and tendon sheath, although these associations as defining characteristics are debated. 9 CLC attachments to surrounding tissue are not described in depth, but authors note subcutaneous location without invasion into J Hand Surg Am.

FIGURE 7: Polygonal to oval chondroblastoma-like cells without atypia (magnification  400).

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periosteum or tendon sheath.6,7 The presented CLC did not demonstrate frank invasion but did have structural affiliations similar to soft tissue chondromas, and thus, on a clinical inspection level, could not be differentiated from a soft tissue chondroma. The general gross appearance of CLC is described as yellow, tan, or brown with a rubber or firm consistency similar to our findings.6,7 Previous reviews have described CLC characteristics as part of a spectrum from prototypical soft tissue chondroma to a purely chondroblast-like growth, whereas more recent papers presented CLC as a unique variant of soft tissue chondroma.5,6 The meaning of the chondroblastoma-like cell proportion as a measure of tumor development or as a stable idiosyncratic characteristic is not yet clear. Radiographic calcification of soft tissue hand masses is rare and, thus, an important diagnostic factor.2,9 CLC plain radiographic findings are sparsely described. One report explicitly mentioned radiographic presence of calcification.6 Soft tissue tumors with radiographic calcification may represent benign masses such as gout tophi or myositis ossificans; however, a variety of sarcomas may present this way as well.3 The magnetic resonance imaging findings in this case were problematic, given the mostly low T1 and heterogeneous high T2 signal characteristics, with markedly heterogeneous enhancement and abundant perilesional inflammatory changes following contrast administration. Chondromas usually show less perilesional inflammatory change and more consistent high T2 signal (possibly related to high water content of hyaline cartilage), although large areas of mineralization may contribute to low signal.2 Similarly, the appearance is not classical for GCTTS, because these lesions may have relatively low T2 signal characteristics and uniform enhancement following contrast administration.1 We found 1 report of CLC magnetic resonance imaging, and it did not elaborate beyond noting the presence of a defined tumor border.6 Standard soft tissue chondromas typically appear as more uniformly benign hyaline cartilage on pathological evaluation. In contrast, CLC histopathology may contain any number of findings that could signify a more aggressive chondroid tumor. In particular, these soft tissue chondroma variants have hypercellular areas containing immature chondroblasts, osteoclastlike multinucleated giant cells, both fine and coarse calcifications, myxoid degeneration, and metaplastic osseous tissue.4e7 However, these same specimens lack

J Hand Surg Am.

aggressive histological features such as considerable mitotic spindle activity, necrosis, or cellular atypia. Alternatively, discovery of osteoclast-like multinucleated giant cells and epithelioid mononuclear cells in a mass with easily discernible borders and lobules might be confused with a benign GCTTS, as has been reported and as was initially suspected after our patient’s core biopsy.5 Hemosiderin and lipid-containing macrophages would be key differentiators commonly seen in GCTTS but not reported in CLC.5,7 In all of the cited cases, definitive CLC diagnosis necessitated histopathological analysis. CLC is a rare tumor of the hand, and this case illustrates how it can represent a diagnostic challenge owing to variable radiographic and pathological findings. We have highlighted the clinical examination, diagnostic imaging, and surgical findings in addition to the more commonly highlighted histopathology. This perspective is specifically valuable to the hand surgeon who is responsible for the patient from presentation through treatment. Magnetic resonance imaging of CLC may help differentiate it from more common hand tumors, but an excisional tissue diagnosis is clarifying. Diagnosis on core biopsy alone should be avoided because it will likely fail to show the varied microarchitecture that is indicative of CLC. Based on review of the literature and our experience, this tumor does not appear malignant and can be successfully treated with marginal excision. REFERENCES 1. Teh J, Whiteley G. MRI of soft tissue masses of the hand and wrist. Br J Radiol. 2006;80(949):47e63. 2. Chandramohan M, Thomas NB, Funk L, Muir LT. MR appearance of mineralized extra skeletal chondroma: a case report and review of literature. Clin Radiol. 2002;57(5):421e423. 3. Nakamura R, Ehara S, Nishida J, Shiraishi H, Sato T, Tamakawa Y. Diffuse mineralization of extraskeletal chondroma: a case report. Radiat Med. 1997;15(1):51e53. 4. Raparia K, Lin JW, Donovan D, et al. Chondroblastoma-like chondroma of soft tissue: report of the first case in the base of skull. Ann Diagn Pathol. 2013;17(3):298e301. 5. Cates JM, Rosenberg AE, O’Connell JX, Nielsen GP. Chondroblastoma-like chondroma of soft tissue: an underrecognized variant and its differential diagnosis. Am J Surg Pathol. 2001;25(5):661e666. 6. Yamada T, Irisa T, Nakano S, Tokunaga O. Extraskeletal chondroma with chondroblastic and granuloma-like elements. Clin Orthop Relat Res. 1995;315:257e261. 7. Isayama T, Iwasaki H, Kikuchi M. Chondroblastomalike extraskeletal chondroma. Clin Orthop Relat Res. 1991;268:214e217. 8. Lee JW, Kim DS, Kim MK, et al. Chondroblastoma-like extraskeletal chondroma: a case report. Korean J Pathol. 1999;33(1):55e58. 9. Rouleau PA, Sundaram M. Radiologic case study. Soft-tissue chondroma. Orthopedics. 2003;26(10):1008. 1088e1090.

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Chondroblastoma-like chondroma of the hand: case report.

Chondroblastoma-like chondroma is a rare tumor that almost exclusively manifests in the hand and presents with a set of unique diagnostic features. Th...
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