The Journal of Foot & Ankle Surgery 54 (2015) 726–729
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Congenital Extraskeletal Osteochondroma of the Web Space Displacing the Lesser Toes: A Unique Presentation G. Gopisankar Balaji, MS Ortho, DNB Ortho 1, Naveen Kumar Patil, MS Ortho, PG 2, Jagdish Menon, MS Ortho, DNB Ortho 3 1 2 3
Assistant Professor, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India P.G Registrar in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India Professor in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Extraskeletal osteochondroma is a rare benign tumor that occurs predominantly in the soft tissues of the hands and feet. The congenital occurrence of this lesion in children is very rare. We report the case of a 9-yearold male who presented with slowly progressive swelling in his left third web space since birth that was painful with prolonged walking and had displaced his fourth toe further laterally, causing both functional and cosmetic problems. Radiographs and computed tomography revealed a well-circumscribed densely ossified lesion. Excision biopsy of the lesion showed a lobulated extraskeletal osteochondroma. At the end of 1 year of follow-up, the child had had no recurrence. To the best of our knowledge, this is the first report of congenital extraskeletal osteochondroma of the foot in English-language published studies. Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: bone tumor cartilage cap surgical excision toe
Extraskeletal osteochondroma, also referred to as soft tissue osteochondroma, is a rare, benign, slow-growing, soft tissue tumor. Extraskeletal osteochondromas usually arise within the soft tissues of the hands and feet in adults, with a peak occurrence in the third and sixth decades (1). Very rarely, it occurs in children. Congenital occurrence is even rarer. We describe a rare case of congenital extraskeletal osteochondroma in the third web space that caused displacement of the fourth toe and a review of the published data. To the best of our knowledge, this is the first report of congenital extraskeletal osteochondroma of the foot in English-language published studies. Case Report A 9-year-old male presented to our outpatient department, with the informant being his mother, with complaints of swelling over his left foot since birth. The parents had noticed a small swelling between the third and fourth toes that had been insidious in onset and not associated with pain since birth. However, from 3 years of age onward, the parents had noticed an increase in the size of the swelling that Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: G. Gopisankar Balaji, MS Ortho, DNB Ortho, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, No. 67, Easwaran Koil Street, Pondicherry 605001 India. E-mail address: [email protected] (G.G. Balaji).
was gradually progressive until attaining the size at his presentation. The swelling was associated with pain on walking for a long distance. He had no history of trauma or fever, and he had no similar swelling in any other part of his body. He had had no loss of weight or loss of appetite. On examination, a 4 3-cm bony hard swelling was present in the third web space that extended from the dorsal to plantar surface and had displaced the fourth toe further dorsolaterally (Figs. 1 and 2). The swelling was not warm or tender, was freely mobile and lobulated, and the skin over the swelling was normal. The margins were well defined, and no veins were dilated. No distal neurovascular deficit or proximal lymphadenopathy was present. The blood investigations revealed a hemoglobin of 13.1 g/dL, total count of 14,900, and erythrocyte sedimentation rate of 3 mm at 1 hour. The biochemical parameters showed uric acid of 3.6 mg/dL, calcium of 8.3 mg/dL, and alkaline phosphatase of 270 IU/L. A plain radiograph of the foot, both anteroposterior and oblique views, revealed a densely ossified soft tissue mass in the third web space that had displaced the fourth toe (Fig. 3). Computed tomography of the foot showed a soft tissue mass between the third and fourth toes with dense ossification and without any communication with the nearby bones (Fig. 4). Excision biopsy of the mass was planned. The patient was positioned supine under general anesthesia. A dorsal incision was made in the third web space. The mass was exposed and excised in total (Figs. 5 and 6). No communication was found with the adjacent bone or tendons. The wound was closed, and a below the knee splint was
1067-2516/$ - see front matter Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.05.016
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
727
Fig. 1. Clinical photograph of the foot showing a lobulated mass in the third web space displacing the fourth toe.
Fig. 3. Plain radiograph of the left foot showing dense ossification of the mass between the third and fourth toes.
applied. The postoperative period was uneventful. The sutures were removed on the 10th day, and the patient was allowed to ambulate as tolerated. The histopathologic report showed a 3 3 3 cm lobulated mass that was capsulated and covered with cartilage macroscopically. The cut section revealed gray-white firm bony areas. Microscopically, the section showed bony trabeculae covered with a cartilage cap, with fibrocollagenous tissue suggestive of an extraskeletal osteochondroma (Fig. 7). At the end of 1 year of follow-up, he had had no recurrence of the lesion (Fig. 8).
Discussion
Fig. 2. Clinical photograph of the foot showing the plantar extension of the tumor.
Osteochondromas are benign bone tumors typically arising from the metaphyseal region of the long bones. They usually grow away from the joints and will be continuous with the medullary cavity of the long bone and have a cartilaginous cap. They account for 20% to 50% of all benign bone tumors and 10% to 15% of all tumors (2). The occurrence of an osteochondroma in the soft tissue without any bony contact is very rare. These have been termed “extraskeletal osteochondromas” or “soft tissue osteochondromas.” They have a predilection for the distal part of the extremities, with about 75% occurring in the hands and 25% in the feet (2). A few cases of para-articular osteochondroma have also been reported (3). They usually arise from synovium, tendon sheath, bursa, or infrapatellar fat pad. These tumors have also been reported in the buttocks, thigh, neck, axilla, and so on (2,4–6). Extraskeletal osteochondromas are common in adulthood, most commonly occurring at age 30 to 60 years. Very few cases have been
Fig. 4. Computed tomography scan showing the soft tissue mass in the third web space with dense ossification.
728
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
Fig. 5. Intraoperative photograph showing the soft tissue mass just before excision.
Fig. 7. Photomicrograph showing bony trabeculae covered by a cartilage cap (hematoxylin and eosin stain, original magnification 40).
documented in children. A congenital occurrence is very rare. Miki et al (7) reported a case of congenital chondroma in a newborn between the spinous processes of the D12 and L1 vertebra. These affect both genders equally. The etiology of these lesions is still controversial. Metaplasia of the tendon sheath of the hand and foot has been suggested as the origin of these tumors. Another proposed theory has been that these tumor cells originate from the pleuripotent cell lines derived from joint synovial tissue, tendon sheath, or connective tissue (5). Recently, the role of chromosomes has also been n et al (8) showed involvement of the HMGA2 studied. Dahle gene located at 12q15 in patients with soft tissue chondromas and other cartilaginous tumors. The other chromosomal aberration involved is monosomy of chromosome 6 with rearrangement of chromosome 11 (6). These tumors present as a painless, slowly progressive, soft tissue mass that is usually asymptomatic. In 20% of patients, the lesion will be painful, especially when located on the plantar aspect of the foot (1). Our patient presented with pain on prolonged walking, probably because of extension of the tumor into the plantar surface and possibly owing to the deformed position of the fourth toe secondary to the mass. These lesions have usually been solitary. Radiographically, extraskeletal osteochondromas will show a welldefined soft tissue mass with dense ossification without any contact to the nearby bony structures. Computed tomography will confirm the extraskeletal location and demonstrate the dense ossification of the mass. Magnetic resonance imaging will show an intermediate
signal intensity on T1-weighted images and high-signal intensity on T2-weighted images owing to the high water content of the cartilage (9). Few cases have been reported of bony erosion or cortical scalloping secondary to this tumor (6). The differential diagnosis for extraskeletal osteochondromas include soft tissue chondroma, myositis ossificans, synovial chondromatosis, tumor calcinosis, ossifying fibromyxoid tumor, synovial sarcoma, extraskeletal osteosarcoma, chondrosarcoma, and pseudomalignant osseous tumor (1,5). It is important to differentiate extraskeletal osteochondroma from these other conditions. Histopathologic examination plays a key role in confirming the diagnosis. Cellular atypia might be seen histologically; however, no malignant transformation or metastatic lesions have been demonstrated. Furthermore, malignancy is rare in children. Management should mainly be excision of the tumor in total, along with the cartilage cap and capsule. A local recurrence rate of up to 18% has been reported (1). The unique presentation in our case was its presence since birth and its extent on both the dorsal and the plantar aspects of the third web space, leading to displacement of the fourth toe laterally, which resulted in both functional and cosmetic problems. In conclusion, extraskeletal osteochondroma of the foot should be considered when a benign, slowly growing lesion with dense ossification has been present since birth. Clinical, radiologic, and histopathologic examinations should be used to diagnose this benign entity because a misdiagnosis can result in overtreatment.
Fig. 6. Excised lobulated soft tissue tumor measuring 3 3 3 cm in total.
Fig. 8. Postoperative plain radiograph of the foot.
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
References 1. Kho VK, Chen WC. Extraskeletal osteochondroma of the foot. J Chin Med Assoc 73:52–55, 2010. 2. Patrizi A, Ismaili A, Loi C, Fanti PA. Extraskeletal osteochondroma of the left thigh: unique presentation. G Ital Dermatol Venereol 148:154–156, 2013. 3. Ozturan KE, Yucel I, Cakici H, Guven M, Gurel K, Dervisoglu S, Cases J. Patellar tendinopathy caused by a para-articular/extraskeletal osteochondroma in the lateral infrapatellar region of the knee: a case report. Cases J 2:9341, 2009. 4. Lim SC, Kim YS, Kim YS, Moon YR. Extraskeletal osteochondroma of the buttock. J Korean Med Sci 18:127–130, 2003.
729
5. Singh R, Sharma AK, Magu NK, Kaur KP, Sen R, Magu S. Extraskeletal osteochondroma in the nape of the neck: a case report. J Orthop Surg (Hong Kong) 14:192–195, 2006. 6. Smida M, Abdenaji W, Douira-Khomsi W, Nessib N, Bellagha I, Ben Ghachem M. Childhood soft tissue chondroma: two cases report. Tunis Med 89:379–382, 2011. 7. Miki T, Yamamuro T, Shikata J, Iida H, Futami T, Nakashima Y. Congenital soft-part chondroma: a case report. Clin Orthop Relat Res 244:244–248, 1989. n A, Mertens F, Rydholm A, Brosjo € O, Wejde J, Mandahl N, Panagopoulos I. 8. Dahle Fusion, disruption, and expression of HMGA2 in bone and soft tissue chondromas. Mod Pathol 16:1132–1140, 2003. 9. Ueno T, Ansai S, Omi T, Kawana S. Extraskeletal osteochondroma arising on the plantar region. Case Rep Dermatol 3:147–150, 2011.
Contents lists available at ScienceDirect
The Journal of Foot & Ankle Surgery journal homepage: www.jfas.org
Congenital Extraskeletal Osteochondroma of the Web Space Displacing the Lesser Toes: A Unique Presentation G. Gopisankar Balaji, MS Ortho, DNB Ortho 1, Naveen Kumar Patil, MS Ortho, PG 2, Jagdish Menon, MS Ortho, DNB Ortho 3 1 2 3
Assistant Professor, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India P.G Registrar in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India Professor in Orthopaedics, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India
a r t i c l e i n f o
a b s t r a c t
Level of Clinical Evidence: 4
Extraskeletal osteochondroma is a rare benign tumor that occurs predominantly in the soft tissues of the hands and feet. The congenital occurrence of this lesion in children is very rare. We report the case of a 9-yearold male who presented with slowly progressive swelling in his left third web space since birth that was painful with prolonged walking and had displaced his fourth toe further laterally, causing both functional and cosmetic problems. Radiographs and computed tomography revealed a well-circumscribed densely ossified lesion. Excision biopsy of the lesion showed a lobulated extraskeletal osteochondroma. At the end of 1 year of follow-up, the child had had no recurrence. To the best of our knowledge, this is the first report of congenital extraskeletal osteochondroma of the foot in English-language published studies. Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved.
Keywords: bone tumor cartilage cap surgical excision toe
Extraskeletal osteochondroma, also referred to as soft tissue osteochondroma, is a rare, benign, slow-growing, soft tissue tumor. Extraskeletal osteochondromas usually arise within the soft tissues of the hands and feet in adults, with a peak occurrence in the third and sixth decades (1). Very rarely, it occurs in children. Congenital occurrence is even rarer. We describe a rare case of congenital extraskeletal osteochondroma in the third web space that caused displacement of the fourth toe and a review of the published data. To the best of our knowledge, this is the first report of congenital extraskeletal osteochondroma of the foot in English-language published studies. Case Report A 9-year-old male presented to our outpatient department, with the informant being his mother, with complaints of swelling over his left foot since birth. The parents had noticed a small swelling between the third and fourth toes that had been insidious in onset and not associated with pain since birth. However, from 3 years of age onward, the parents had noticed an increase in the size of the swelling that Financial Disclosure: None reported. Conflict of Interest: None reported. Address correspondence to: G. Gopisankar Balaji, MS Ortho, DNB Ortho, Department of Orthopaedics, Jawaharlal Institute of Postgraduate Medical Education and Research, No. 67, Easwaran Koil Street, Pondicherry 605001 India. E-mail address: [email protected] (G.G. Balaji).
was gradually progressive until attaining the size at his presentation. The swelling was associated with pain on walking for a long distance. He had no history of trauma or fever, and he had no similar swelling in any other part of his body. He had had no loss of weight or loss of appetite. On examination, a 4 3-cm bony hard swelling was present in the third web space that extended from the dorsal to plantar surface and had displaced the fourth toe further dorsolaterally (Figs. 1 and 2). The swelling was not warm or tender, was freely mobile and lobulated, and the skin over the swelling was normal. The margins were well defined, and no veins were dilated. No distal neurovascular deficit or proximal lymphadenopathy was present. The blood investigations revealed a hemoglobin of 13.1 g/dL, total count of 14,900, and erythrocyte sedimentation rate of 3 mm at 1 hour. The biochemical parameters showed uric acid of 3.6 mg/dL, calcium of 8.3 mg/dL, and alkaline phosphatase of 270 IU/L. A plain radiograph of the foot, both anteroposterior and oblique views, revealed a densely ossified soft tissue mass in the third web space that had displaced the fourth toe (Fig. 3). Computed tomography of the foot showed a soft tissue mass between the third and fourth toes with dense ossification and without any communication with the nearby bones (Fig. 4). Excision biopsy of the mass was planned. The patient was positioned supine under general anesthesia. A dorsal incision was made in the third web space. The mass was exposed and excised in total (Figs. 5 and 6). No communication was found with the adjacent bone or tendons. The wound was closed, and a below the knee splint was
1067-2516/$ - see front matter Ó 2015 by the American College of Foot and Ankle Surgeons. All rights reserved. http://dx.doi.org/10.1053/j.jfas.2014.05.016
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
727
Fig. 1. Clinical photograph of the foot showing a lobulated mass in the third web space displacing the fourth toe.
Fig. 3. Plain radiograph of the left foot showing dense ossification of the mass between the third and fourth toes.
applied. The postoperative period was uneventful. The sutures were removed on the 10th day, and the patient was allowed to ambulate as tolerated. The histopathologic report showed a 3 3 3 cm lobulated mass that was capsulated and covered with cartilage macroscopically. The cut section revealed gray-white firm bony areas. Microscopically, the section showed bony trabeculae covered with a cartilage cap, with fibrocollagenous tissue suggestive of an extraskeletal osteochondroma (Fig. 7). At the end of 1 year of follow-up, he had had no recurrence of the lesion (Fig. 8).
Discussion
Fig. 2. Clinical photograph of the foot showing the plantar extension of the tumor.
Osteochondromas are benign bone tumors typically arising from the metaphyseal region of the long bones. They usually grow away from the joints and will be continuous with the medullary cavity of the long bone and have a cartilaginous cap. They account for 20% to 50% of all benign bone tumors and 10% to 15% of all tumors (2). The occurrence of an osteochondroma in the soft tissue without any bony contact is very rare. These have been termed “extraskeletal osteochondromas” or “soft tissue osteochondromas.” They have a predilection for the distal part of the extremities, with about 75% occurring in the hands and 25% in the feet (2). A few cases of para-articular osteochondroma have also been reported (3). They usually arise from synovium, tendon sheath, bursa, or infrapatellar fat pad. These tumors have also been reported in the buttocks, thigh, neck, axilla, and so on (2,4–6). Extraskeletal osteochondromas are common in adulthood, most commonly occurring at age 30 to 60 years. Very few cases have been
Fig. 4. Computed tomography scan showing the soft tissue mass in the third web space with dense ossification.
728
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
Fig. 5. Intraoperative photograph showing the soft tissue mass just before excision.
Fig. 7. Photomicrograph showing bony trabeculae covered by a cartilage cap (hematoxylin and eosin stain, original magnification 40).
documented in children. A congenital occurrence is very rare. Miki et al (7) reported a case of congenital chondroma in a newborn between the spinous processes of the D12 and L1 vertebra. These affect both genders equally. The etiology of these lesions is still controversial. Metaplasia of the tendon sheath of the hand and foot has been suggested as the origin of these tumors. Another proposed theory has been that these tumor cells originate from the pleuripotent cell lines derived from joint synovial tissue, tendon sheath, or connective tissue (5). Recently, the role of chromosomes has also been n et al (8) showed involvement of the HMGA2 studied. Dahle gene located at 12q15 in patients with soft tissue chondromas and other cartilaginous tumors. The other chromosomal aberration involved is monosomy of chromosome 6 with rearrangement of chromosome 11 (6). These tumors present as a painless, slowly progressive, soft tissue mass that is usually asymptomatic. In 20% of patients, the lesion will be painful, especially when located on the plantar aspect of the foot (1). Our patient presented with pain on prolonged walking, probably because of extension of the tumor into the plantar surface and possibly owing to the deformed position of the fourth toe secondary to the mass. These lesions have usually been solitary. Radiographically, extraskeletal osteochondromas will show a welldefined soft tissue mass with dense ossification without any contact to the nearby bony structures. Computed tomography will confirm the extraskeletal location and demonstrate the dense ossification of the mass. Magnetic resonance imaging will show an intermediate
signal intensity on T1-weighted images and high-signal intensity on T2-weighted images owing to the high water content of the cartilage (9). Few cases have been reported of bony erosion or cortical scalloping secondary to this tumor (6). The differential diagnosis for extraskeletal osteochondromas include soft tissue chondroma, myositis ossificans, synovial chondromatosis, tumor calcinosis, ossifying fibromyxoid tumor, synovial sarcoma, extraskeletal osteosarcoma, chondrosarcoma, and pseudomalignant osseous tumor (1,5). It is important to differentiate extraskeletal osteochondroma from these other conditions. Histopathologic examination plays a key role in confirming the diagnosis. Cellular atypia might be seen histologically; however, no malignant transformation or metastatic lesions have been demonstrated. Furthermore, malignancy is rare in children. Management should mainly be excision of the tumor in total, along with the cartilage cap and capsule. A local recurrence rate of up to 18% has been reported (1). The unique presentation in our case was its presence since birth and its extent on both the dorsal and the plantar aspects of the third web space, leading to displacement of the fourth toe laterally, which resulted in both functional and cosmetic problems. In conclusion, extraskeletal osteochondroma of the foot should be considered when a benign, slowly growing lesion with dense ossification has been present since birth. Clinical, radiologic, and histopathologic examinations should be used to diagnose this benign entity because a misdiagnosis can result in overtreatment.
Fig. 6. Excised lobulated soft tissue tumor measuring 3 3 3 cm in total.
Fig. 8. Postoperative plain radiograph of the foot.
G.G. Balaji et al. / The Journal of Foot & Ankle Surgery 54 (2015) 726–729
References 1. Kho VK, Chen WC. Extraskeletal osteochondroma of the foot. J Chin Med Assoc 73:52–55, 2010. 2. Patrizi A, Ismaili A, Loi C, Fanti PA. Extraskeletal osteochondroma of the left thigh: unique presentation. G Ital Dermatol Venereol 148:154–156, 2013. 3. Ozturan KE, Yucel I, Cakici H, Guven M, Gurel K, Dervisoglu S, Cases J. Patellar tendinopathy caused by a para-articular/extraskeletal osteochondroma in the lateral infrapatellar region of the knee: a case report. Cases J 2:9341, 2009. 4. Lim SC, Kim YS, Kim YS, Moon YR. Extraskeletal osteochondroma of the buttock. J Korean Med Sci 18:127–130, 2003.
729
5. Singh R, Sharma AK, Magu NK, Kaur KP, Sen R, Magu S. Extraskeletal osteochondroma in the nape of the neck: a case report. J Orthop Surg (Hong Kong) 14:192–195, 2006. 6. Smida M, Abdenaji W, Douira-Khomsi W, Nessib N, Bellagha I, Ben Ghachem M. Childhood soft tissue chondroma: two cases report. Tunis Med 89:379–382, 2011. 7. Miki T, Yamamuro T, Shikata J, Iida H, Futami T, Nakashima Y. Congenital soft-part chondroma: a case report. Clin Orthop Relat Res 244:244–248, 1989. n A, Mertens F, Rydholm A, Brosjo € O, Wejde J, Mandahl N, Panagopoulos I. 8. Dahle Fusion, disruption, and expression of HMGA2 in bone and soft tissue chondromas. Mod Pathol 16:1132–1140, 2003. 9. Ueno T, Ansai S, Omi T, Kawana S. Extraskeletal osteochondroma arising on the plantar region. Case Rep Dermatol 3:147–150, 2011.
