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Periosteal chondroma of the rib—report of a case and literature review
Journal of Pediatric Surgery (2005) 40, 1505 – 1507
www.elsevier.com/locate/jpedsurg
Periosteal chondroma of the rib—report of a case and literature review Rouzan Karabakhtsiana, Debra Hellera,*, Meera Hameeda, Colin Bethelb a
Department of Pathology and Laboratory Medicine, UMDNJ–New Jersey Medical School, P.O. Box 1709, Newark, NJ 07103, USA b Department of Surgery, UMDNJ-New Jersey Medical School, P.O. Box 1709, Newark, NJ 07103, USA Index words: Chondroma; Ribs; Cartilage
Abstract Periosteal chondroma is a rare benign tumor of hyaline cartilage. It develops adjacent to the cortex of the bone and is rimmed by an intact periosteal membrane. Periosteal chondromas are most common in the metaphyses of long bones followed by the small tubular bones of the hands and feet. Periosteal chondroma arising in the rib is an extremely rare event. We could only find 10 reported cases in the English literature. We present a case of periosteal chondroma in the rib discovered incidentally on chest x-ray of an 11-year-old girl. D 2005 Elsevier Inc. All rights reserved.
Periosteal (juxtacortical) chondroma is a rare benign neoplasm of hyaline cartilage that develops adjacent to the cortical surface of bone, beneath the periosteal membrane [1,2]. Periosteal chondromas account for less than 2% of all chondromas [1]. Although these tumors can occur both in adults and children [3,4], they predominate in patients younger than 30 years, with the highest frequency in the second decade [3,5,6]. Clinically, periosteal chondromas present as palpable masses which are often swollen and painful [3,5,6], although painless masses have also been described [4]. The most common location for periosteal chondromas are in the metaphyses of long tubular bones, particularly a proximal humerus, although small tubular bones of hands and feet can also be involved [1,3,6]. To our knowledge, periosteal chondroma arising in the rib has been reported only 10 times in the English literature [3,7-10].
T Corresponding author. Tel.: +1 973 972 0751; fax: +1 973 972 5724. E-mail address: [email protected] (D. Heller). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.05.057
We present a case of periosteal chondroma in the rib of an 11-year-old girl discovered incidentally.
1. Case report This was an 11-year-old girl who presented with the asymptomatic finding of a left fourth rib mass on chest x-ray, detected during investigation for conversion of her purified protein derivative status. Imaging studies suggested a mass on the inner portion of the rib, and she underwent a partial rib resection. At surgery, the mass was described as measuring 4.0 3.0 cm.
2. Pathology The rib specimen measured 6.0 cm in length and 1 cm in width. Thickness ranged from 0.3 to 0.6 cm. A centrally located white-gray firm cartilaginous mass was present on the inner pleural surface of the rib, measuring
1506
Fig. 1 Specimen radiograph, showing saucer-shaped erosion of the cortical bone by a lobulated lesion with sclerosis of adjacent cortical bone.
2.5 1.8 0.8 cm. The lesion appeared to arise under the periosteum grossly, eroding into the cortex. Specimen x-ray showed a lytic lesion with saucerization of the underlying cortex (Fig. 1). Histologically, the lesion was seen to tent up the periosteum and was composed of lobules of benign cartilage (Figs. 2 and 3).
3. Discussion Periosteal chondromas are very rare benign tumors of hyaline cartilage accounting for less than 2% of all chondromas [1]. The lesion is predominantly seen in patients younger than 30 years, with the highest frequency in the second decade [3,5,6]. The metaphyses of long bones, particularly a proximal humerus, followed by small tubular bones of hands and feet, are the most common
R. Karabakhtsian et al.
Fig. 3
The lesion is covered by periosteum.
locations [1,3,6]. The rib is an extremely rare location (Table 1). In a review of the literature from 1925 to 1985, Lewis et al [3] found only 2 cases (of 167 cases) arising in the rib. Radiologically, periosteal chondromas appear as sharply marginated radiolucent bone surface tumors often with calcification and mineralization of the chondroid matrix [3,5,6,11], which is seen in about 50% of cases [2,12]. Ossification can also be seen [4]. Classically, there is erosion into the cortex of the bone with saucerization of the underlying bone, with overlapping cortical bone at the edges, often showing sclerosis. On magnetic resonance images, lesions are bordered by a hypointense rim, corresponding to fibrous tissue and an intact periosteum on pathological examination [2]. Macroscopically, periosteal chondromas are well-demarcated tumors without penetration into the underlying cancellous bone. The underlying cortex appears thickened and shows indentation. There is solid periosteal buttressing overlying the tumor. The tumor size is usually less than 6 cm in greatest dimension [3,5,6,9]. Table 1 Cases of periosteal chondroma of the rib in the English literature
Fig. 2 The lesion is composed of low cellular hyaline cartilage and erodes into the cortical bone.
Marcove and Huvos [7] Marcove and Huvos [7] Nojima et al [10]
45 y
2 Cases
Lewis et al [3]
2 Cases
Morisaki et al [8] Inoue et al [9] Inoue et al [9]
45 y, Male 5 y, Male 39 y, Male
Current case
11 y, Female
14 y
No other information provided No other information provided No other information provided No other information provided Painful mass Pain and rib swelling 2-mo History of cough, coin lesion on x-ray Incidental finding on x-ray
Periosteal chondroma of the rib—report of a case and literature review Histologically, periosteal chondromas show a lobulated configuration of hyaline cartilage covered by periosteum [2,4]. They are usually hypocellular, although occasionally may focally show increased cellularity with nuclear pleomorphism, binucleation, and multinucleation [3,5,6]. The hypercellularity and features of nuclear atypia can sometimes be misdiagnosed as chondrosarcoma [10]. Recommended methods of treatment of this lesion include intralesional, marginal, and en bloc excisions. All methods appear to be effective, with low recurrence rate [3,6], although some authors have described early postexcisional recurrences [4]. With marginal excision of these tumors, curettage of the underlying cortical bone has been suggested by some authors to avoid the possibility of recurrence [4]. The differential diagnosis of periosteal chondroma includes juxtacortical chondrosarcoma and periosteal osteosarcoma. Here, as in all bone lesions, radiographs are critical to the evaluation. Juxtacortical chondrosarcoma shows popcorn calcifications on radiographs, whereas a periosteal osteosarcoma would be expected to demonstrate perpendicular spicules of calcification [9]. A low-grade chondrosarcoma may be difficult to distinguish histologically from a chondroma; however, chondrosarcoma is exceptionally rare in children. The differential diagnosis of rib lesions in children includes congenital lesions, infections, hemangioma, eosinophilic granuloma, osteochondroma, aneurysmal bone cyst, fibrous dysplasia, chondroblastoma, benign neural lesions, extramedullary hematopoiesis, fracture callus, and primary and metastatic malignancies, including Ewing sarcoma, primative neuroectodermal tumor, osteosarcoma, chondrosarcoma (rare in children), and metastatic neuroblastoma or leukemia [13]. In summary, this case demonstrates the characteristic radiological and histological features of periosteal
1507
chondroma, a rare tumor, which occurred in an unusual location. Suspecting the existence of periosteal chondroma in this location is important to ensure proper surgical treatment.
References [1] Brien EW, Mirra JM, Luck Jr JV. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology II Juxtacortical cartilage tumors. Skelet Radiol 1999; 28:1 - 20. [2] Woertler K, Blasius S, Brinkschmidt C, et al. Periosteal chondroma: MR characteristics. J Comput Assist Tomogr 2001;25:425 - 30. [3] Lewis MM, Kenan S, Yabut SM, et al. Periosteal chondroma. A report of ten cases and review of the literature. Clin Orthop 1990; 256:185 - 92. [4] Molto FL, Lluch DJB, Perales VM. Childhood periosteal chondroma. Arch Orthop Trauma Surg 2000;120:605 - 8. [5] Boriani S, Bacchini P, Bertoni F. Periosteal chondroma. A review of twenty cases. J Bone Joint Surg Am 1983;65:205 - 12. [6] Bauer TW, Dorfman HD, Latham Jr JT. Periosteal chondroma. A clinico-pathologic study of 23 cases. Am J Surg Pathol 1982;6: 631 - 7. [7] Marcove CR, Huvos AG. Cartilaginous tumors of the ribs. Cancer 1971;27:794 - 801. [8] Morisaki Y, Takagi K, Ishii Y, et al. Periosteal chondroma developing in a rib at the site of a chest wall wound from a previous thoracotomy: report of a case. Surg Today 1996;26:57 - 9. [9] Inoue S, Fujino S, Kontani K, et al. Periosteal chondroma of the rib: report of two cases. Surg Today 2001;31:1074 - 8. [10] Nojima T, Unni KK, McLeod RA, et al. Periosteal chondroma and periosteal chondrosarcoma. Am J Surg Pathol 1985;9:666 - 77. [11] Varma D, Kumar R, Carrasco C. MR imaging of periosteal chondroma. J Comput Assist Tomogr 1991;15:1008 - 10. [12] de Santos LA, Spjut HR. Periosteal chondroma: a radiographic spectrum. Skelet Radiol 1981;6:15 - 20. [13] Faro SH, Mahboubi S, Ortega W. CT diagnosis of rib anomalies, tumors, and infections in children. Clin Imaging 1993;17:1 - 7.
www.elsevier.com/locate/jpedsurg
Periosteal chondroma of the rib—report of a case and literature review Rouzan Karabakhtsiana, Debra Hellera,*, Meera Hameeda, Colin Bethelb a
Department of Pathology and Laboratory Medicine, UMDNJ–New Jersey Medical School, P.O. Box 1709, Newark, NJ 07103, USA b Department of Surgery, UMDNJ-New Jersey Medical School, P.O. Box 1709, Newark, NJ 07103, USA Index words: Chondroma; Ribs; Cartilage
Abstract Periosteal chondroma is a rare benign tumor of hyaline cartilage. It develops adjacent to the cortex of the bone and is rimmed by an intact periosteal membrane. Periosteal chondromas are most common in the metaphyses of long bones followed by the small tubular bones of the hands and feet. Periosteal chondroma arising in the rib is an extremely rare event. We could only find 10 reported cases in the English literature. We present a case of periosteal chondroma in the rib discovered incidentally on chest x-ray of an 11-year-old girl. D 2005 Elsevier Inc. All rights reserved.
Periosteal (juxtacortical) chondroma is a rare benign neoplasm of hyaline cartilage that develops adjacent to the cortical surface of bone, beneath the periosteal membrane [1,2]. Periosteal chondromas account for less than 2% of all chondromas [1]. Although these tumors can occur both in adults and children [3,4], they predominate in patients younger than 30 years, with the highest frequency in the second decade [3,5,6]. Clinically, periosteal chondromas present as palpable masses which are often swollen and painful [3,5,6], although painless masses have also been described [4]. The most common location for periosteal chondromas are in the metaphyses of long tubular bones, particularly a proximal humerus, although small tubular bones of hands and feet can also be involved [1,3,6]. To our knowledge, periosteal chondroma arising in the rib has been reported only 10 times in the English literature [3,7-10].
T Corresponding author. Tel.: +1 973 972 0751; fax: +1 973 972 5724. E-mail address: [email protected] (D. Heller). 0022-3468/$ – see front matter D 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2005.05.057
We present a case of periosteal chondroma in the rib of an 11-year-old girl discovered incidentally.
1. Case report This was an 11-year-old girl who presented with the asymptomatic finding of a left fourth rib mass on chest x-ray, detected during investigation for conversion of her purified protein derivative status. Imaging studies suggested a mass on the inner portion of the rib, and she underwent a partial rib resection. At surgery, the mass was described as measuring 4.0 3.0 cm.
2. Pathology The rib specimen measured 6.0 cm in length and 1 cm in width. Thickness ranged from 0.3 to 0.6 cm. A centrally located white-gray firm cartilaginous mass was present on the inner pleural surface of the rib, measuring
1506
Fig. 1 Specimen radiograph, showing saucer-shaped erosion of the cortical bone by a lobulated lesion with sclerosis of adjacent cortical bone.
2.5 1.8 0.8 cm. The lesion appeared to arise under the periosteum grossly, eroding into the cortex. Specimen x-ray showed a lytic lesion with saucerization of the underlying cortex (Fig. 1). Histologically, the lesion was seen to tent up the periosteum and was composed of lobules of benign cartilage (Figs. 2 and 3).
3. Discussion Periosteal chondromas are very rare benign tumors of hyaline cartilage accounting for less than 2% of all chondromas [1]. The lesion is predominantly seen in patients younger than 30 years, with the highest frequency in the second decade [3,5,6]. The metaphyses of long bones, particularly a proximal humerus, followed by small tubular bones of hands and feet, are the most common
R. Karabakhtsian et al.
Fig. 3
The lesion is covered by periosteum.
locations [1,3,6]. The rib is an extremely rare location (Table 1). In a review of the literature from 1925 to 1985, Lewis et al [3] found only 2 cases (of 167 cases) arising in the rib. Radiologically, periosteal chondromas appear as sharply marginated radiolucent bone surface tumors often with calcification and mineralization of the chondroid matrix [3,5,6,11], which is seen in about 50% of cases [2,12]. Ossification can also be seen [4]. Classically, there is erosion into the cortex of the bone with saucerization of the underlying bone, with overlapping cortical bone at the edges, often showing sclerosis. On magnetic resonance images, lesions are bordered by a hypointense rim, corresponding to fibrous tissue and an intact periosteum on pathological examination [2]. Macroscopically, periosteal chondromas are well-demarcated tumors without penetration into the underlying cancellous bone. The underlying cortex appears thickened and shows indentation. There is solid periosteal buttressing overlying the tumor. The tumor size is usually less than 6 cm in greatest dimension [3,5,6,9]. Table 1 Cases of periosteal chondroma of the rib in the English literature
Fig. 2 The lesion is composed of low cellular hyaline cartilage and erodes into the cortical bone.
Marcove and Huvos [7] Marcove and Huvos [7] Nojima et al [10]
45 y
2 Cases
Lewis et al [3]
2 Cases
Morisaki et al [8] Inoue et al [9] Inoue et al [9]
45 y, Male 5 y, Male 39 y, Male
Current case
11 y, Female
14 y
No other information provided No other information provided No other information provided No other information provided Painful mass Pain and rib swelling 2-mo History of cough, coin lesion on x-ray Incidental finding on x-ray
Periosteal chondroma of the rib—report of a case and literature review Histologically, periosteal chondromas show a lobulated configuration of hyaline cartilage covered by periosteum [2,4]. They are usually hypocellular, although occasionally may focally show increased cellularity with nuclear pleomorphism, binucleation, and multinucleation [3,5,6]. The hypercellularity and features of nuclear atypia can sometimes be misdiagnosed as chondrosarcoma [10]. Recommended methods of treatment of this lesion include intralesional, marginal, and en bloc excisions. All methods appear to be effective, with low recurrence rate [3,6], although some authors have described early postexcisional recurrences [4]. With marginal excision of these tumors, curettage of the underlying cortical bone has been suggested by some authors to avoid the possibility of recurrence [4]. The differential diagnosis of periosteal chondroma includes juxtacortical chondrosarcoma and periosteal osteosarcoma. Here, as in all bone lesions, radiographs are critical to the evaluation. Juxtacortical chondrosarcoma shows popcorn calcifications on radiographs, whereas a periosteal osteosarcoma would be expected to demonstrate perpendicular spicules of calcification [9]. A low-grade chondrosarcoma may be difficult to distinguish histologically from a chondroma; however, chondrosarcoma is exceptionally rare in children. The differential diagnosis of rib lesions in children includes congenital lesions, infections, hemangioma, eosinophilic granuloma, osteochondroma, aneurysmal bone cyst, fibrous dysplasia, chondroblastoma, benign neural lesions, extramedullary hematopoiesis, fracture callus, and primary and metastatic malignancies, including Ewing sarcoma, primative neuroectodermal tumor, osteosarcoma, chondrosarcoma (rare in children), and metastatic neuroblastoma or leukemia [13]. In summary, this case demonstrates the characteristic radiological and histological features of periosteal
1507
chondroma, a rare tumor, which occurred in an unusual location. Suspecting the existence of periosteal chondroma in this location is important to ensure proper surgical treatment.
References [1] Brien EW, Mirra JM, Luck Jr JV. Benign and malignant cartilage tumors of bone and joint: their anatomic and theoretical basis with an emphasis on radiology, pathology and clinical biology II Juxtacortical cartilage tumors. Skelet Radiol 1999; 28:1 - 20. [2] Woertler K, Blasius S, Brinkschmidt C, et al. Periosteal chondroma: MR characteristics. J Comput Assist Tomogr 2001;25:425 - 30. [3] Lewis MM, Kenan S, Yabut SM, et al. Periosteal chondroma. A report of ten cases and review of the literature. Clin Orthop 1990; 256:185 - 92. [4] Molto FL, Lluch DJB, Perales VM. Childhood periosteal chondroma. Arch Orthop Trauma Surg 2000;120:605 - 8. [5] Boriani S, Bacchini P, Bertoni F. Periosteal chondroma. A review of twenty cases. J Bone Joint Surg Am 1983;65:205 - 12. [6] Bauer TW, Dorfman HD, Latham Jr JT. Periosteal chondroma. A clinico-pathologic study of 23 cases. Am J Surg Pathol 1982;6: 631 - 7. [7] Marcove CR, Huvos AG. Cartilaginous tumors of the ribs. Cancer 1971;27:794 - 801. [8] Morisaki Y, Takagi K, Ishii Y, et al. Periosteal chondroma developing in a rib at the site of a chest wall wound from a previous thoracotomy: report of a case. Surg Today 1996;26:57 - 9. [9] Inoue S, Fujino S, Kontani K, et al. Periosteal chondroma of the rib: report of two cases. Surg Today 2001;31:1074 - 8. [10] Nojima T, Unni KK, McLeod RA, et al. Periosteal chondroma and periosteal chondrosarcoma. Am J Surg Pathol 1985;9:666 - 77. [11] Varma D, Kumar R, Carrasco C. MR imaging of periosteal chondroma. J Comput Assist Tomogr 1991;15:1008 - 10. [12] de Santos LA, Spjut HR. Periosteal chondroma: a radiographic spectrum. Skelet Radiol 1981;6:15 - 20. [13] Faro SH, Mahboubi S, Ortega W. CT diagnosis of rib anomalies, tumors, and infections in children. Clin Imaging 1993;17:1 - 7.