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Subungual Extraskeletal Chondroma With Finger Nail Deformity: Case Report
SCIENTIFIC ARTICLE
Subungual Extraskeletal Chondroma With Finger Nail Deformity: Case Report Takayuki Ishii, MD, Masayoshi Ikeda, MD, PhD, Yoshinori Oka, MD, PhD We report a subungual extraskeletal chondroma with a convex nail deformity originating from the index finger in a 39-year-old man. The tumor was excised and its histopathology showed obvious nuclear pleomorphism. However, the Ki-67 (MIB-1) labeling index was less than 1%, indicating low proliferative activity; it was classified as an extraskeletal chondroma with atypical features. There was no recurrence over a 3-year postoperative period. (J Hand Surg 2010;35A:296–299. Copyright © 2010 by the American Society for Surgery of the Hand. All rights reserved.) Key words Extraskeletal chondroma, subungual, distal phalanx, MIB-1.
occurs in soft tissue, is a rare benign tumor relative to other tumors that occur in the distal extremities and lacks continuity with bone or the periosteum.1,2 Although a subungual extraskeletal chondroma has been reported in 3 cases,3–5 each showed only a mild nail deformity without pain and was treated as a dermatologic disease because of negative radiological findings. We treated a case of subungual extraskeletal chondroma of an atypical type with obvious nuclear pleomorphism that occurred in the distal phalanx and caused a severe convex nail deformity. Surgical excision was subsequently performed. We report this case together with immunohistopathological findings.
E
XTRASKELETAL CHONDROMA, WHICH
CASE REPORT A 39-year-old man presented with a 4-year history of persistent swelling in the base of the right index fingernail, which had been neglected because of the absence of specific pain. Since the swelling gradually enlarged and the nail became severely deformed during that From the Department of Orthopaedic Surgery, Tokai University Hachioji Hospital, Tokyo; and the Department of Orthopaedic Surgery, Tokai University Oiso Hospital, Oiso, Kanagawa, Japan. Received for publication July 25, 2009; accepted in revised form October 22, 2009. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Masayoshi Ikeda, MD, PhD, Department of Orthopaedic Surgery, Tokai University Hachioji Hospital, 1838 Ishikawacho, Hachioji, Tokyo 192-0032, Japan; e-mail: [email protected]. 0363-5023/10/35A02-0020$36.00/0 doi:10.1016/j.jhsa.2009.10.026
296 䉬 © ASSH 䉬 Published by Elsevier, Inc. All rights reserved.
period, the patient visited the outpatient office of our hospital. Physical examination demonstrated a 1.5 ⫻ 2.0cm, glossy, extraordinary mass appearing on the dorsal aspect of the right index finger and extending from the distal interphalangeal (DIP) joint through the subungual region (Fig. 1). The mass was hard, elastic, and immobile, with no inflammatory signs. There was no motion restriction of the DIP joint, and a laboratory examination showed no abnormal findings. Plain radiographs demonstrated a soft tissue tumor shadow with clustered calcification, located subcutaneously on the dorsal aspect of the distal phalanx of the right index finger, extending from the distal part of the dorsal DIP joint through the subungual region (Fig. 2). The tumor had no continuity with the bone. There was no destructive change in the distal phalanx and no periosteal reaction. Magnetic resonance imaging showed a soft tissue tumor image with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The tumor was lobulated with linear structures and had a signal intensity thought to be partial segmentation (Fig. 3). The tumor was well demarcated without invasion into the distal phalanx. Based on these findings, the mass was considered to be a cartilaginous tumor and excision was performed as follows. The radial border of the nail fold was incised and the skin was reflected to remove the nail. The thinned nail bed was incised longitudinally on top of the
SUBUNGUAL EXTRASKELETAL CHONDROMA
FIGURE 1: Photograph of the right index finger. A markedly convex nail deformity can be seen arising from the DIP joint and the nail root.
FIGURE 2: Plain radiograph. Clustered calcification can be observed in the mass extending from the distal part of the DIP joint through the dorsal part of the distal phalanx.
mass to expose a white solid tumor surrounded by a fibrous capsule. The size of the tumor was 9 ⫻ 15 ⫻ 19 mm, and there was no adhesion to the periosteum or surrounding tissue (Fig. 4). Histopathologically, the tumor had a lobulated structure surrounded by a thin fibrous capsule. The stroma was composed of hyaline chondrocytes and was partially concomitant with myxoid degeneration, calcification, and ossification. The chondrocytes had obvious nuclear pleomorphism. The cellularity was higher than that of typical chondromas (Fig. 5). There were no secondary changes such as inflammation or degeneration, and no continuity with bone tissue. However, the Ki-67 (MIB-1) labeling index was less than 1% (Fig. 6) and P53 immunoreactivity was not seen, indicating low
297
FIGURE 3: Magnetic resonance image showing a well-demarcated mass without invasion into the bone cortex with high signal intensity on T2-weighted images.
FIGURE 4: The nail was removed and the thinned nail bed vertically divided to expose a white solid tumor surrounded by a fibrous capsule. There is no adhesion of the tumor to the periosteum or surrounding tissue.
proliferative activity. Thus, the tumor was diagnosed as an extraskeletal chondroma with atypical features. The nail regenerated without deformity and there was no recurrence over 3 years after the surgey. DISCUSSION The extraskeletal chondroma is rare relative to the other soft tumor, and about 200 cases have been reported in the English literature.2,6 The most common sites of occurrence are the hands, with more than 60% of all extraskeletal chondromas located here.1,6,7 Occurrence in the fingers has been reported in more than 60% of the hands, most of which have occurred on the palmar side.8 –13
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FIGURE 5: Histopathological finding. The chondrocytes had nuclear pleomorphism and the cellularity is higher than that of typical chondromas (hematoxylin– eosin stain, original magnification ⫻200).
FIGURE 6: Immunohistopathological finding. There were few cells expressing MIB-1 positivity. There was a single MIB-1– positive cell nucleus (arrowhead) in this field (MIB-1 antibody, original magnification ⫻200).
Because extraskeletal chondroma is a nodular mass that grows slowly in soft tissue without any continuity with the bone, there are few symptoms and the preoperative duration of symptoms tends to be long. Until now, we are aware of only 3 case reports that describe an extraskeletal chondroma of subungual origin and each had a mild nail deformity.3–5 The present case had a severe convex nail deformity that included the nail root. This created difficulty in diagnosing the extraskeletal chondroma because it grew slowly in the subungual region with few characteristic findings. The only characteristic radiographic feature was a spherically or irregularly calcified soft tissue shadow without invasion into the distal phalanx, as recognized in this case. It is
not difficult to differentiate an extraskeletal chondroma from other tumors such as subungual exostosis, glomus tumor, and so on. Histopathologically, most extraskeletal chondromas show lobulated mature hyaline cartilage tissue accompanied by myxoid degeneration, ossification, and calcification. The others (approximately 15%) show atypical features that should be differentiated from chondrosarcoma. The findings accompany increased cellularity, nuclear pleomorphism, the presence of chondroblastic cells and epithelioid cells, and the proliferation of multinucleated giant cells.1,6 The present case showed atypical findings histopathologically. Recently, it has become possible to overcome diagnostic confusion between chondroma and chondrosarcoma using a Ki-67 (MIB-1) immunohistochemical modality.14 The Ki-67 (MIB-1) immunoreactivity of the present case, whose labeling index was less than 1%, indicated low proliferative activity; it was classified as a chondroma with atypical features. Although few reports have been published on the natural course and prognosis of extraskeletal chondroma, to our knowledge, there has not been a reported case of malignant transformation or of metastasis even if the histopathology revealed undifferentiable malignant features.7 The presence of cosmetic problems such as nail deformation or symptoms such as pain is considered a primary indication for surgical removal. Because the incidence of local recurrence after simple removal of the tumor itself is reported to be 17%,2 it is advisable to remove the tumor completely, including the capsular structure and adhesion sites.
REFERENCES 1. Weiss SW, Goldblum JR. Cartilaginous soft tissue tumors. In: Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s soft tissue tumors. 4th ed. St. Louis: Mosby, 2001:1361–1388. 2. Dahlin DC, Salvador AH. Cartilaginous tumors of the soft tissues of the hands and feet. Mayo Clin Proc 1974;49:721–726. 3. Ayala F, Lembo G, Montesano M. A rare tumor: subungual chondroma. Report of a case. Dermatologica 1983;167:339 –340. 4. Bauer HI, Kaatz M, Kluge WH, Elsner P. Subungual chondroma, a case report. Z Rheumatol 2002;61:58 – 61. 5. Cho SB, Kim S-C. Subungual extraskeletal chondroma mimicking glomus tumor. J Dermatol 2003;30:492– 494. 6. Chung EB, Enzinger FM. Chondroma of soft parts. Cancer 1978;41: 1414 –1424. 7. Fletcher CD, Krausz T. Cartilaginous tumours of soft tissue. Appl Pathol 1988;6:208 –220. 8. Strong ML Jr. Chondromas of the tendon sheath of the hand: report of a case and review of the literature. J Bone Joint Surg 1975;57A:1164 –1165.
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9. Mahoney JL. Soft tissue chondromas in the hand. J Hand Surg 1987;12A:317–320. 10. DelSignore JL, Torre BA, Miller RJ. Extraskeletal chondroma of the hand: case report and review of the literature. Clin Orthop 1990;254: 147–152. 11. Uchinuma E, Kimata S, Yamashita R, Shioya N. Extraskeletal chondroma with bony destruction: a case report and review of literature. Eur J Plast Surg 1994;17:159 –160.
299
12. Folsom GJ, Lee DH, Lopez-Ben R, Winokur T, Jaffe KA. Hand mass in a 15-year-old boy. Clin Orthop 2003;412:269 –275. 13. Singh P, Mathur SK, Kundu ZS, Singh S, Marwah N, Arora B. Extraskeletal chondroma of the hand: a case report. Indian J Pathol Microbiol 2005;48:206 –208. 14. Scotlandi K, Serra M, Manara MC, Marici D, Benini S, Nini G, et al. Clinical relevance of Ki-67 expression in bone tumors. Cancer 1995;75:806 – 814.
JHS 䉬 Vol A, February
Subungual Extraskeletal Chondroma With Finger Nail Deformity: Case Report Takayuki Ishii, MD, Masayoshi Ikeda, MD, PhD, Yoshinori Oka, MD, PhD We report a subungual extraskeletal chondroma with a convex nail deformity originating from the index finger in a 39-year-old man. The tumor was excised and its histopathology showed obvious nuclear pleomorphism. However, the Ki-67 (MIB-1) labeling index was less than 1%, indicating low proliferative activity; it was classified as an extraskeletal chondroma with atypical features. There was no recurrence over a 3-year postoperative period. (J Hand Surg 2010;35A:296–299. Copyright © 2010 by the American Society for Surgery of the Hand. All rights reserved.) Key words Extraskeletal chondroma, subungual, distal phalanx, MIB-1.
occurs in soft tissue, is a rare benign tumor relative to other tumors that occur in the distal extremities and lacks continuity with bone or the periosteum.1,2 Although a subungual extraskeletal chondroma has been reported in 3 cases,3–5 each showed only a mild nail deformity without pain and was treated as a dermatologic disease because of negative radiological findings. We treated a case of subungual extraskeletal chondroma of an atypical type with obvious nuclear pleomorphism that occurred in the distal phalanx and caused a severe convex nail deformity. Surgical excision was subsequently performed. We report this case together with immunohistopathological findings.
E
XTRASKELETAL CHONDROMA, WHICH
CASE REPORT A 39-year-old man presented with a 4-year history of persistent swelling in the base of the right index fingernail, which had been neglected because of the absence of specific pain. Since the swelling gradually enlarged and the nail became severely deformed during that From the Department of Orthopaedic Surgery, Tokai University Hachioji Hospital, Tokyo; and the Department of Orthopaedic Surgery, Tokai University Oiso Hospital, Oiso, Kanagawa, Japan. Received for publication July 25, 2009; accepted in revised form October 22, 2009. No benefits in any form have been received or will be received related directly or indirectly to the subject of this article. Corresponding author: Masayoshi Ikeda, MD, PhD, Department of Orthopaedic Surgery, Tokai University Hachioji Hospital, 1838 Ishikawacho, Hachioji, Tokyo 192-0032, Japan; e-mail: [email protected]. 0363-5023/10/35A02-0020$36.00/0 doi:10.1016/j.jhsa.2009.10.026
296 䉬 © ASSH 䉬 Published by Elsevier, Inc. All rights reserved.
period, the patient visited the outpatient office of our hospital. Physical examination demonstrated a 1.5 ⫻ 2.0cm, glossy, extraordinary mass appearing on the dorsal aspect of the right index finger and extending from the distal interphalangeal (DIP) joint through the subungual region (Fig. 1). The mass was hard, elastic, and immobile, with no inflammatory signs. There was no motion restriction of the DIP joint, and a laboratory examination showed no abnormal findings. Plain radiographs demonstrated a soft tissue tumor shadow with clustered calcification, located subcutaneously on the dorsal aspect of the distal phalanx of the right index finger, extending from the distal part of the dorsal DIP joint through the subungual region (Fig. 2). The tumor had no continuity with the bone. There was no destructive change in the distal phalanx and no periosteal reaction. Magnetic resonance imaging showed a soft tissue tumor image with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The tumor was lobulated with linear structures and had a signal intensity thought to be partial segmentation (Fig. 3). The tumor was well demarcated without invasion into the distal phalanx. Based on these findings, the mass was considered to be a cartilaginous tumor and excision was performed as follows. The radial border of the nail fold was incised and the skin was reflected to remove the nail. The thinned nail bed was incised longitudinally on top of the
SUBUNGUAL EXTRASKELETAL CHONDROMA
FIGURE 1: Photograph of the right index finger. A markedly convex nail deformity can be seen arising from the DIP joint and the nail root.
FIGURE 2: Plain radiograph. Clustered calcification can be observed in the mass extending from the distal part of the DIP joint through the dorsal part of the distal phalanx.
mass to expose a white solid tumor surrounded by a fibrous capsule. The size of the tumor was 9 ⫻ 15 ⫻ 19 mm, and there was no adhesion to the periosteum or surrounding tissue (Fig. 4). Histopathologically, the tumor had a lobulated structure surrounded by a thin fibrous capsule. The stroma was composed of hyaline chondrocytes and was partially concomitant with myxoid degeneration, calcification, and ossification. The chondrocytes had obvious nuclear pleomorphism. The cellularity was higher than that of typical chondromas (Fig. 5). There were no secondary changes such as inflammation or degeneration, and no continuity with bone tissue. However, the Ki-67 (MIB-1) labeling index was less than 1% (Fig. 6) and P53 immunoreactivity was not seen, indicating low
297
FIGURE 3: Magnetic resonance image showing a well-demarcated mass without invasion into the bone cortex with high signal intensity on T2-weighted images.
FIGURE 4: The nail was removed and the thinned nail bed vertically divided to expose a white solid tumor surrounded by a fibrous capsule. There is no adhesion of the tumor to the periosteum or surrounding tissue.
proliferative activity. Thus, the tumor was diagnosed as an extraskeletal chondroma with atypical features. The nail regenerated without deformity and there was no recurrence over 3 years after the surgey. DISCUSSION The extraskeletal chondroma is rare relative to the other soft tumor, and about 200 cases have been reported in the English literature.2,6 The most common sites of occurrence are the hands, with more than 60% of all extraskeletal chondromas located here.1,6,7 Occurrence in the fingers has been reported in more than 60% of the hands, most of which have occurred on the palmar side.8 –13
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SUBUNGUAL EXTRASKELETAL CHONDROMA
FIGURE 5: Histopathological finding. The chondrocytes had nuclear pleomorphism and the cellularity is higher than that of typical chondromas (hematoxylin– eosin stain, original magnification ⫻200).
FIGURE 6: Immunohistopathological finding. There were few cells expressing MIB-1 positivity. There was a single MIB-1– positive cell nucleus (arrowhead) in this field (MIB-1 antibody, original magnification ⫻200).
Because extraskeletal chondroma is a nodular mass that grows slowly in soft tissue without any continuity with the bone, there are few symptoms and the preoperative duration of symptoms tends to be long. Until now, we are aware of only 3 case reports that describe an extraskeletal chondroma of subungual origin and each had a mild nail deformity.3–5 The present case had a severe convex nail deformity that included the nail root. This created difficulty in diagnosing the extraskeletal chondroma because it grew slowly in the subungual region with few characteristic findings. The only characteristic radiographic feature was a spherically or irregularly calcified soft tissue shadow without invasion into the distal phalanx, as recognized in this case. It is
not difficult to differentiate an extraskeletal chondroma from other tumors such as subungual exostosis, glomus tumor, and so on. Histopathologically, most extraskeletal chondromas show lobulated mature hyaline cartilage tissue accompanied by myxoid degeneration, ossification, and calcification. The others (approximately 15%) show atypical features that should be differentiated from chondrosarcoma. The findings accompany increased cellularity, nuclear pleomorphism, the presence of chondroblastic cells and epithelioid cells, and the proliferation of multinucleated giant cells.1,6 The present case showed atypical findings histopathologically. Recently, it has become possible to overcome diagnostic confusion between chondroma and chondrosarcoma using a Ki-67 (MIB-1) immunohistochemical modality.14 The Ki-67 (MIB-1) immunoreactivity of the present case, whose labeling index was less than 1%, indicated low proliferative activity; it was classified as a chondroma with atypical features. Although few reports have been published on the natural course and prognosis of extraskeletal chondroma, to our knowledge, there has not been a reported case of malignant transformation or of metastasis even if the histopathology revealed undifferentiable malignant features.7 The presence of cosmetic problems such as nail deformation or symptoms such as pain is considered a primary indication for surgical removal. Because the incidence of local recurrence after simple removal of the tumor itself is reported to be 17%,2 it is advisable to remove the tumor completely, including the capsular structure and adhesion sites.
REFERENCES 1. Weiss SW, Goldblum JR. Cartilaginous soft tissue tumors. In: Weiss SW, Goldblum JR, eds. Enzinger and Weiss’s soft tissue tumors. 4th ed. St. Louis: Mosby, 2001:1361–1388. 2. Dahlin DC, Salvador AH. Cartilaginous tumors of the soft tissues of the hands and feet. Mayo Clin Proc 1974;49:721–726. 3. Ayala F, Lembo G, Montesano M. A rare tumor: subungual chondroma. Report of a case. Dermatologica 1983;167:339 –340. 4. Bauer HI, Kaatz M, Kluge WH, Elsner P. Subungual chondroma, a case report. Z Rheumatol 2002;61:58 – 61. 5. Cho SB, Kim S-C. Subungual extraskeletal chondroma mimicking glomus tumor. J Dermatol 2003;30:492– 494. 6. Chung EB, Enzinger FM. Chondroma of soft parts. Cancer 1978;41: 1414 –1424. 7. Fletcher CD, Krausz T. Cartilaginous tumours of soft tissue. Appl Pathol 1988;6:208 –220. 8. Strong ML Jr. Chondromas of the tendon sheath of the hand: report of a case and review of the literature. J Bone Joint Surg 1975;57A:1164 –1165.
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SUBUNGUAL EXTRASKELETAL CHONDROMA
9. Mahoney JL. Soft tissue chondromas in the hand. J Hand Surg 1987;12A:317–320. 10. DelSignore JL, Torre BA, Miller RJ. Extraskeletal chondroma of the hand: case report and review of the literature. Clin Orthop 1990;254: 147–152. 11. Uchinuma E, Kimata S, Yamashita R, Shioya N. Extraskeletal chondroma with bony destruction: a case report and review of literature. Eur J Plast Surg 1994;17:159 –160.
299
12. Folsom GJ, Lee DH, Lopez-Ben R, Winokur T, Jaffe KA. Hand mass in a 15-year-old boy. Clin Orthop 2003;412:269 –275. 13. Singh P, Mathur SK, Kundu ZS, Singh S, Marwah N, Arora B. Extraskeletal chondroma of the hand: a case report. Indian J Pathol Microbiol 2005;48:206 –208. 14. Scotlandi K, Serra M, Manara MC, Marici D, Benini S, Nini G, et al. Clinical relevance of Ki-67 expression in bone tumors. Cancer 1995;75:806 – 814.
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