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Background

These are benign proliferations of cartilage, most commonly occuring on the feet. Ordinarily, these tumors do not pose a diagnostic difficulty, However, when they occur in unusual locations, such as the hands or jaws, then the pathologist must utilize the radiological studies and histopathology of these tumors to come to a correct diagnosis. In these instances, the differential diagnosis includes a malignant tumor such as a chondrosarcoma.

OUTLINE

Epidemiology  
Disease Associations Popliteal artery pseudoaneurysm
Stem cell transplantation
Pathogenesis Chromosomal Abnormalities
Laboratory/Radiologic/Other Diagnostic Testing  
Gross Appearance and Clinical Variants Bizarre Paraosteal Osteochondromatous Proliferation (Nora's lesion)
Hereditary Multiple Osteochondromas
Mandible
Spine
Subungual
Histopathological Features and Variants  
Differential Diagnosis Tumors of the Foot
Prognosis and Treatment Malignant transformation
Surgery
Commonly Used Terms  
Internet Links  

 

EPIDEMIOLOGY CHARACTERIZATION
PREVALENCE Most common bone tumor of the feet

 

DISEASE ASSOCIATIONS CHARACTERIZATION
POPLITEAL ARTERY PSEUDOANEURYSM  

Osteochondroma as a causal agent in popliteal artery pseudoaneurysms: case report and literature review.

Klebuc M, Burrow S, Organek A, Cole W, Zuker R.

Division of Plastic Surgery, Department of Surgery, Baylor College of Medicine, Houston, TX 77030, USA.

J Reconstr Microsurg 2001 Oct;17(7):475-9 Abstract quote

Osteochondromas are an infrequent, but significant, source of vascular injuries. Popliteal artery pseudoaneurysms are the most common sequelae, with a complex interplay of anatomic and developmental factors accounting for their prevalence.

The authors present a case report, detailed discussion, and literature review of pseudoaneurysm formation in the popliteal artery.

STEM CELL TRANSPLANTATION  


Osteochondroma after pediatric hematopoietic stem cell transplantation: report of eight cases.

Bordigoni P, Turello R, Clement L, Lascombes P, Leheup B, Galloy MA, Plenat F.

Unite de Transplantation Medullaire, Hopital d'Enfants, Nancy, France.


Bone Marrow Transplant 2002 Apr;29(7):611-4 Abstract quote

Eight children developed osteochondroma (OS) at a mean of 88 months after hematopoietic stem cell transplantation (HSCT). The mean age at HSCT was 56 months (12-84).

This represents a cumulative incidence of 20% among patients less than 18 years of age transplanted from 1981 to 1997. These eight patients underwent allogeneic (n = 2) or autologous (n = 6) transplantation for either acute leukemia (n = 6) or neuroblastoma (n = 2) after a conditioning regimen including TBI (n = 7) or a combination of Bu and CY. OS was multiple in seven patients and solitary in one. Eight lesions were resected and all were benign.

Four children received growth hormone before diagnosis of OS, but there was no clinical, radiological or histological difference between those who did not. Univariate analysis showed an increased rate associated only with autologous HSCT, with a 31.7% probability of a new OS at 12 years after HSCT.

Osteochondroma should be added to the other adverse effects of HSCT in children.

 

PATHOGENESIS CHARACTERIZATION
CHROMOSOMAL ABNORMALITIES  

Distinct Chromosomal Rearrangements in Subungual (Dupuytren) Exostosis and Bizarre Parosteal Osteochondromatous Proliferation (Nora Lesion).

Zambrano E, Nose V V, Perez-Atayde AR, Gebhardt M, Hresko MT, Kleinman P, Richkind KE, Kozakewich HP.

*Department of Pathology, daggerOrthopedics, and double daggerRadiology, Children's Hospital Boston, Harvard Medical School, Boston, MA; and section signGenzyme Genetics, Santa Fe, NM.
Am J Surg Pathol. 2004 Aug;28(8):1033-1039 Abstract quote  

BACKGROUND:: Proliferative lesions of the bone surface, such as subungual (Dupuytren) exostosis and bizarre parosteal osteochondromatous proliferation (BPOP, Nora lesion) are currently classified as reactive, proliferative processes that mimic primary neoplasms of bone.

METHODS:: Cytogenetic analysis was performed on 3 subungual exostoses of the great toe and 2 BPOP lesions of the radius and ulna.

RESULTS:: A balanced translocation t(X;6) was identified in all cases of subungual exostoses. The chromosomal rearrangements observed in 1 case of BPOP differed from those seen in subungual exostosis.

CONCLUSIONS:: The presence of chromosomal abnormalities in subungual exostosis and BPOP suggests that these lesions are neoplastic, with a different molecular pathogenesis, and that each is a distinct clinicopathologic entity.


Recurring breakpoints of 1p13 approximately p22 in osteochondroma.

Sawyer JR, Thomas EL, Lukacs JL, Swanson CM, Ding Y, Parham DM, Thomas JR, Nicholas RW.

Department of Pathology, University of Arkansas for Medical Sciences, 4301 Markham Street, 72205, Little Rock, AR, USA

Cancer Genet Cytogenet 2002 Oct 15;138(2):102-6 Abstract quote

Cytogenetic studies of osteochondromas are scarce but have previously shown recurring clonal aberrations involving chromosome 8.

We have studied a series of eight tumors and have found recurring aberrations not only involving chromosome 8, but also chromosome 1 in five of the seven abnormal tumors. Surprisingly, three of the chromosome 1 aberrations involved pericentric inversions. Four tumors showed aberrations involving the region 1p13 approximately p22 by mechanisms including inversion, insertion, and translocation.

These findings indicate that aberrations of chromosome 1p, in a region spanning 1p13 approximately p22, may be nonrandomly involved in the cytogenetic progression of osteochondroma.


Cytogenetic and molecular cytogenetic evidence of recurrent 8q24.1 loss in osteochondroma.

Feely MG, Boehm AK, Bridge RS, Krallman PA, Neff JR, Nelson M, Bridge JA.

Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE, USA.

 

Cancer Genet Cytogenet 2002 Sep;137(2):102-7 Abstract quote

Osteochondroma most frequently arises sporadically and as a solitary lesion, but may also arise as multiple lesions characterizing the autosomal dominant disorder hereditary multiple exostoses (HME) and the contiguous gene-deletion syndrome, Langer-Giedion syndrome (LGS). Various germline mutations of two putative tumor suppressor genes, EXT1 localized to 8q24.1 and EXT2 localized to 11p11 approximately p12, have been demonstrated in HME families. Constitutional chromosomal deletions or structural rearrangements of 8q24.1 are seen in LGS.

Cytogenetic reports of sporadic and hereditary osteochondromas are few, but have revealed loss or structural rearrangements of 8q24.1 in a small number of tumors. In the current study, karyotypic evaluation of 37 osteochondroma specimens (both sporadic and hereditary lesions) revealed chromosomal anomalies of 8q24.1 in 10 specimens (27%).

In an effort to determine the presence and frequency of submicroscopic deletions, molecular cytogenetic studies were performed on this same set of tumors utilizing a chromosome 8 specific centromeric probe and an 8q24.1 cosmid probe (locus D8S51, within the minimal LGS deletion region). Loss of the 8q24.1 locus was detected by fluorescence in situ hybridization in 27 of 34 (79%) osteochondroma specimens analyzed including all 10 specimens exhibiting chromosome 8 abnormalities cytogenetically.

These findings indicate that a significant subset of osteochondromas harbor genetic aberrations at the EXT1 locus and suggest that loss or mutation of EXT1 plays an important role in the pathogenesis of sporadic as well as hereditary osteochondromas.

 

LABORATORY/
RADIOLOGIC/
OTHER TESTS

CHARACTERIZATION
RADIOLOGIC  
LABORATORY MARKERS  

 

GROSS APPEARANCE/
CLINICAL VARIANTS
CHARACTERIZATION
GENERAL  
VARIANTS  
BIZARRE
PARAOSTEAL
OSTEO-CHONDROMATOUS PROLIFERATION (NORA'S LESION)
 
Molecular cytogenetic characterization of recurrent translocation breakpoints in bizarre parosteal osteochondromatous proliferation (Nora's lesion).

Nilsson M, Domanski HA, Mertens F, Mandahl N.

Department of Clinical Genetics and Pathology, University Hospital, Lund, Sweden.
Hum Pathol. 2004 Sep;35(9):1063-9. Abstract quote  

Bizarre parosteal osteochondromatous proliferation (BPOP), or Nora's lesion, is a rare tumorous lesion with aggressive growth that affects primarily the small tubular bones in the distal extremities and often recurs after excision. No previous cytogenetic data on BPOP are available. In the present study, lesions from 5 patients were investigated by chromosome banding and fluorescence in situ hybridization (FISH) analyses. Patient age ranged from 24 to 46 years, and the lesions were located in the fingers in 4 cases and in a toe in 1 case.

Histological sections from all 5 tumors were characterized by a mixture of hypercellular cartilage, cancellous bone, and spindle cell components. Samples from 2 patients were available for cytogenetic analysis. One of these showed a normal female karyotype, and the other revealed a balanced translocation, t(1;17)(q32;q21), as the sole anomaly. The translocation was further characterized by 3-color metaphase FISH analyses, using 17 1q32-specific and 18 17q21-specific bacterial artificial chromosome probes, to map the precise location of the breakpoints. Split signals were detected by the RP11-99A19 probe in chromosome 1 and by the RP11-219F9 probe in chromosome 17. To determine whether these rearrangements are characteristic features of BPOP, paraffin-embedded tissue sections from all 5 patients were investigated by interphase FISH analyses. All 5 cases had a break in 1q32, and 4 of the 5 cases showed a break in the 17q21 region. The results strongly indicate that t(1;17)(q32;q21), or variant translocations involving 1q32, are recurrent and unique aberrations in BPOP.

Several genes are located within the 2 sequences spanning the breakpoints, and further studies should be performed to determine whether any of these are involved in the formation of a fusion gene.


Bizarre parosteal osteochondromatous proliferation (Nora's lesion): A retrospective study of 12 cases, 2 arising in long bones.

Abramovici L, Steiner GC.

Department of Pathology and Laboratory Medicine, Hospital for Joint Diseases, New York, NY.

Hum Pathol 2002 Dec;33(12):1205-10 Abstract quote

Twelve cases of bizarre parosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, are reported. Ten lesions were located in the small bones of the hands, and 2 were located in long bones (femur and proximal tibia). Patient age ranged from 12 to 63 years (average, 30.3 years).

Radiography of the lesions in the hand bones showed calcific masses attached to the underlying cortex, without interruption of the latter. The long bone lesions revealed unusual findings. In the femur, BPOP presented with extensive cortical destruction and was suggestive of a malignant lesion. This presentation has not been described to date. In the tibia, the lesion was located in the soft tissue without cortical attachment. This type of BPOP probably represents an immature lesion that over time will mature to solid cortical attachment.

On histologic examination, all lesions demonstrated 3 distinct components with variable degrees of representation: (1) hypercellular cartilage with calcification and ossification, with the calcified cartilage having a characteristic basophilic tinctorial quality; (2) cancellous bone undergoing maturation; and (3) spindle cell stroma without cytologic atypia. In 1 case with a long-standing history, the cartilaginous component was minimal.

BPOP, together with florid reactive periostitis and turret exostosis, may represent different stages in the development of a posttraumatic proliferative process. BPOP apparently arises from the periosteal tissues through a process of cartilaginous metaplsia.


MR imaging features of bizarre parosteal osteochondromatous proliferation of bone (Nora's lesion).

Torreggiani WC, Munk PL, Al-Ismail K, O'Connell JX, Nicolaou S, Lee MJ, Masri BA.

Department of Radiology, Vancouver General Hospital, University of British Columbia, 899 West 12th Avenue, Vancouver, BC, Canada V5Z 1M9.

Eur J Radiol 2001 Dec;40(3):224-31 Abstract quote

The purpose of this study was to review the imaging findings of three patients with bizarre parosteal osteochrondromatous proliferation of bone (BPOP). The plain radiographs and MRI images of three patients with BPOP were obtained and retrospectively reviewed.

In two cases, BPOP involved the feet. In one case BPOP involved the hand. In all three cases, plain radiographs showed a well-defined calcium containing mass adjacent to the cortical surface of the adjacent bone. The underlying bone appeared normal in all cases. On MRI, the lesion was of low signal intensity on T1 weighted sequences in all cases. On FSE T2 weighted and STIR sequences, the lesion was of high signal in all cases. The cortex, medullary cavity and adjacent soft tissues appeared normal in all cases.

While BPOP is rare and often confused with a variety of both benign and malignant lesions, there are specific radiological findings that may help to distinguish BPOP from many of its mimickers.


Bizarre parosteal osteochondromatous proliferation (Nora's lesion) of the foot.

Horiguchi H, Sakane M, Matsui M, Wadano Y.

Center for Medical Sciences, Ibaraki Prefectural University of Health Sciences, Inashiki, Japan.

Pathol Int 2001 Oct;51(10):816-23 Abstract quote

A 22-year-old man presented with a growing lump on the fifth metatarsal of the right foot. Radiographically, the lesion was a calcified mass stuck on to the bone. The T2-weighted magnetic resonance images showed heterogeneity in intensity. A tumor was suspected and an excisional biopsy was done. The lesion was composed of a cartilaginous cap and bone tissue.

Histological examination revealed characteristic features of bizarre parosteal osteochondromatous proliferation (BPOP), such as hypercellularity, a blue tinctorial quality in the osteocartilaginous interfaces, and a scattering of binucleated or bizarre enlarged chondrocytes. Immunohistochemically, basic fibroblast growth factor was expressed in nearly all chondrocytes within the cartilaginous cap, while vascular endothelial growth factor was expressed only in enlarged chondrocytes near the osteocartilaginous interfaces. Reverse transcription-polymerase chain reaction detected chondromodulin-I transcripts in the tissue of the cartilaginous cap.

These findings indicate that the processes occurring in BPOP are similar to those occurring in endochondral ossification in the growth plate, and they support the concept that BPOP is a reparative process. BPOP is a rare tumorous lesion of the bone and is occasionally confused with other benign or malignant conditions.

Thus, it is important to consider the clinical, radiographical and the gross histological features of the lesion when making a diagnosis.


Bizarre parosteal osteochondromatous proliferation with cortical invasion.

Helliwell TR, O'Connor MA, Ritchie DA, Feldberg L, Stilwell JH, Jane MJ.

Department of Pathology, Royal Liverpool University Hospital, UK.

Skeletal Radiol 2001 May;30(5):282-5 Abstract quote

A 15-year-old male presented with an 18-month history of increasing swelling on the radial aspect of his left forearm. Radiographs and MR images showed a partly calcified mass on the lateral border of the radius with erosion of the underlying cortex.

Following a CT-guided needle biopsy, the mass was excised by segmental resection of the radial shaft with replacement by a vascularised fibular graft. Histological examination of the resected specimen showed many features typical of a bizarre parosteal osteochondromatous proliferation (BPOP) but with destruction of the underlying cortical bone by cellular spindle cell tissue.

Although there were no cytological signs of malignancy, we believe that this unusual disease should be regarded as a particularly aggressive form of BPOP and treated by wide excision. Clinical follow-up of our patient at 2 years shows no sign of local recurrence or metastasis.


Florid reactive periostitis and bizarre parosteal osteochondromatous proliferation: pre-biopsy imaging evolution, treatment and outcome.

Sundaram M, Wang L, Rotman M, Howard R, Saboeiro AP.

Department of Radiology, St. Louis University Health Sciences Center, 3635 Vista at Grand, St. Louis, MO 63110-0250, USA.

 

Skeletal Radiol 2001 Apr;30(4):192-8 Abstract quote

OBJECTIVE: To report on the imaging evolution of florid reactive periostitis (FRP) and bizarre parosteal osteochondromatous proliferation (BPOP) of the phalanges of the hands from prospective diagnosis to operation and on postsurgical outcome.

DESIGN AND PATIENTS: Three patients (2 female, 1 male; age range 11-34 years) presented with a swollen digit of the hand. Following presumptive radiographic diagnosis of FRP, they were closely observed both clinically and radiographically until operation. All three patients had radiographs of the involved digit, and one patient had an MR imaging examination. The interval between presumptive diagnosis and operation ranged from 2 to 8 months. Following operation, the patients have been clinically followed for 9-13 months (mean 10 months).

RESULTS: In each of the patients, maturing of periosteal reaction without bone destruction was observed within 1-2 weeks of the presumptive diagnosis of FRP. Periosteal reaction was initially minimal in relation to the extent of soft tissue swelling and subsequently became more florid. In one patient, the lesion ossified, became adherent to the phalanx, and had an "osteochondromatous" appearance. In another patient, periosteal reaction was seen on both sides of the phalanx with an intact phalanx. In the sole patient who had MR imaging, edema was seen in the phalanx distal to the symptomatic site and the metacarpal proximal to the symptomatic site.

CONCLUSIONS: Close clinical and radiographic correlation permits an accurate pre-biopsy diagnosis of FRP. The first follow-up radiograph taken within 2 weeks usually provides re-assurance of the accuracy of the diagnosis. FRP may progress to BPOP. Arbitrary antibiotic treatment can be avoided, and a planned surgical approach can be adopted.

HERDITARY MULTIPLE EXOSTOSES (OSTEOCHONDROMAS)  


Hereditary multiple exostoses: one center's experience and review of etiology.

Pierz KA, Stieber JR, Kusumi K, Dormans JP.

Children's Hospital of Philadelphia and the University of Pennsylvania School of Medicine, Department of Orthopaedic Surgery and Division of Human Genetics and Molecular Biology, 19104, USA.

Clin Orthop 2002 Aug;(401):49-59 Abstract quote

Hereditary multiple exostosis is a genetic disorder characterized by multiple osteochondromas that can cause pain, deformity, and potential malignant degeneration. Linkage analysis has identified a family of EXT genes which, if mutated, can lose heterozygosity and potentially cause osteochondromas.

A database was established of 43 patients with hereditary multiple exostoses treated at a tertiary pediatric healthcare system. Twenty patients had a known family history of the disorder. All patients were diagnosed between birth and 13 years.

Symptoms or deformity were observed in the forearms of 29 patients, the knees of 37 patients, and the ankles of 28 patients. Valgus knee deformity related to hereditary multiple exostoses, previously reported to be attributable to proximal tibial changes alone, resulted from proximal tibial or distal femoral valgus deformities in this series. Twenty-seven patients required between one and five surgeries to address their lesions. No patient had malignant degeneration of an osteochondroma; however, three patients had first-degree relatives with transformation of an osteochondroma to chondrosarcoma.

This database now may be a resource for additional analysis. By correlating specific genetic mutations with clinical manifestations, it may be possible to stratify patients into subtypes of hereditary multiple exostoses and identify genetic markers associated with malignant degeneration.


Natural history study of hereditary multiple exostoses.

Wicklund CL, Pauli RM, Johnston D, Hecht JT.

University of Texas Health Science Center at Houston 77225, USA.

Am J Med Genet 1995 Jan 2;55(1):43-6 Abstract quote

Hereditary multiple exostosis (EXT) is an autosomal dominant disorder in which the clinical hallmark is the growth of bony protuberances from long bones and which can cause a variety of orthopedic deformities.

This study sought to further delineate the natural history of EXT. In addition, since previous studies have suggested that there are deviations from Mendelian expectations in EXT, including incomplete penetrance and a skewed sex ratio, we attempted to confirm or refute these suggestions. Both portions of the study were carried out through retrospective review of 43 affected probands and 137 of their affected relatives. Data are presented concerning frequency and severity of complications of EXT including short stature, sequelae of exostoses, occurrence of malignant degeneration of exostoses, and problems in pregnancy and delivery of affected females.

Only 2.8% of the total affected population had experienced exostosis-related malignancy, an estimate which is considerably less than earlier reports would suggest. Penetrance was 100%. There was an excess of males within the entire affected population (104:76) and within identified probands (28:15). However, the male to female ratio was unskewed in nuclear families (probands, affected sibs, and parents).

The excess of males appears to be related to males having more severe and more frequent complications of EXT than having any primary genetic origin.

MANDIBLE  


Osteochondroma of the mandibular condyle: a case report and review of the literature.

Saito T, Utsunomiya T, Furutani M, Yamamoto H.

Department of Pathology, Nihon University School of Dentistry at Matsudo, Chiba, Japan.

J Oral Sci 2001 Dec;43(4):293-7 Abstract quote

Osteochondroma is rarely found in the oral and maxillofacial regions. A rare case of osteochondroma affecting the mandibular condyle of a 46-year-old Japanese woman is reported.

Clinical examination revealed facial asymmetry, malocclusion, and a palpable hard mass in the right temporomandibular joint (TMJ). Radiologically, the lesion was visualized as a radiopaque mass in the same region, but no destructive features were evident. Three-dimensional computed tomography was employed for estimating the stereographic extension of the lesion, which seemed to develop from the anterior portion of the condylar neck, and extend to the condylar head. The patient underwent tumor excision and condyloplasty under a clinical diagnosis of benign TMJ tumor.

The histopathological diagnosis was osteochondroma of the mandibular condyle, and the lesion consisted of proliferative bony and hyalinized cartilage-like tissues. Moreover, a cartilage cap, a characteristic feature of osteochondroma, was also observed.

Thirty-eight cases of osteochondroma of the mandibular condyle described in the English literature, including the present case, were reviewed. The mean patient age was 39.7 years with a peak in the fourth decade, which was older than patients with tumors in the axial skeleton. There was no sexual predominance for tumors in either the mandibular condyle or axial skeleton. The histopathogenesis of this tumor developing in the mandibular condyle was also discussed.

SPINE  


Osteochondroma of the spine: an enigmatic tumor of the spinal cord. A series of 10 cases.

Sharma MC, Arora R, Deol PS, Mahapatra AK, Mehta VS, Sarkar C.

Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.

 

J Neurosurg Sci 2002 Jun;46(2):66-70 Abstract quote

BACKGROUND: The aim of this study was to evaluate clinical, radiological and pathological features of vertebral osteochondromas with compressive myelopathy and to review the relevant English literature. Osteochondro-mas are common benign bony lesions of long bones but involvement of spine by solitary osteochondroma and its presentation as compressive myelopathy is rare. Most of the literature is in the form of case reports.

METHODS: During a period of 20 years (1980-1999), 10 cases of osteochondromas of the spine were encountered. Clinical, radiological and pathological features were reviewed.

RESULTS: The age ranged from 13 to 45 years (mean 25.3 years) and all except 1 were males. In 8 cases the pathology involved the cervical spine and in two cases dorsal spine was involved. All patients presented with progressive motor sensory deficit of 6 months to 30 years duration (mean 3.9 years). Decompressive laminectomy was carried out in all the patients. Fortunately, gradual and complete recovery was observed in all of them.

CONCLUSIONS: Osteochondromas of the spine are not as rare as reported in the literature. In a young patient of compressive myelopathy this possibility should be considered. Magnetic resonance imaging, computed tomography and CT myelogram are useful in evaluating the size and extent of the lesion for subsequent surgical planning.

SUBUNGUAL  

Subungual osteochondroma: a case report.

Bostanci S, Ekmekci P, Ekinci C, Akcaboy B, Gurgey E.

Department of Dermatology, Ankara University Medical School, 06100 Sihhiye, Ankara, Turkey.

Dermatol Surg 2001 Jun;27(6):591-3 Abstract quote

Osteochondromas are among the most common bone tumors of the foot. However, they rarely occur in subungual locations. Clinically they appear as slow-growing masses causing deformity of the overlying nail.

Here we present a case of subungual osteochondroma with characteristic clinical, radiologic, and histopathologic features. The lesion was successfully treated by total excision.

 

HISTOLOGICAL TYPES CHARACTERIZATION
GENERAL Characteristic cartilage cap of mature cartilage

 

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
GENERAL  

Bone and soft tissue tumours of the foot: review of 83 cases.

Kinoshita G, Matsumoto M, Maruoka T, Shiraki T, Tsunemi K, Futani H, Maruo S.

Department of Orthopedic Surgery, Takarazuka Municipal Hospital, Takarazuka, Japan.

 

J Orthop Surg (Hong Kong) 2002 Dec;10(2):173-8 Abstract quote

PURPOSE. To review cases of bone and soft tissue tumours of the feet managed at the Hyogo College of Medicine, Nishinomiya and Takarazuka Municipal Hospital, Takarazuka, Japan.

METHODS. Retrospective analysis of 83 patient records treated for bone and soft tissue tumours of the feet between 1974 and 2000.

RESULTS. There were 33 benign bone tumours, one primary malignant bone tumour, and 2 metastatic bone cancer. Marginal resection was performed in cases of osteochondroma and curettage in cases of other benign bone tumour. Despite below-knee amputation in the case of chondrosarcoma, the patient died because of pulmonary metastasis. Two patients with metastatic cancer also died, and 2 cases of osteochondroma and one of benign chondroblastoma recurred. There were 47 cases of soft tissue tumour. Treatment for benign soft tissue tumours was marginal resection; no cases recurred. In contrast, all patients with soft tissue sarcoma died after surgery. The majority of bone tumours were located in the toe and hindfoot areas, in the first and second decades of life, whereas soft tissue tumours occurred mainly in the midfoot area and in patients aged between 20 and 50 years. The sex distribution was almost even for bone tumours (male: female ratio, 19:17), whereas about half as many males as females had soft tissue tumours (14:33).

CONCLUSION. Bone and soft tissue tumours of the feet are uncommon. Most bone tumours are chondrogenic, but differential diagnosis of malignant from benign disease is difficult and prognosis is poor. Management of benign tumours by marginal resection has good prognosis, whereas prognosis of soft tissue sarcomas is very poor.

 

PROGNOSIS AND TREATMENT CHARACTERIZATION
PROGNOSTIC FACTORS  
MALIGNANT TRANSFORMATION  


Chondrosarcoma in a family with multiple hereditary exostoses.

Kivioja A, Ervasti H, Kinnunen J, Kaitila I, Wolf M, Bohling T.

Department of Orthopaedics and Traumatology, Helsinki University Central Hospital, Finland.

J Bone Joint Surg Br 2000 Mar;82(2):261-6 Abstract quote

Multiple hereditary exostoses is an autosomal dominant skeletal disorder in which there are numerous cartilage-capped excrescences in areas of actively growing bone. The condition is genetically heterogeneous, and at least three genes, ext1, ext2 and ext3 are involved. The reported risk for malignant transformation to chondrosarcoma has been from 0.6% to 2.8%.

We have reviewed six generations of a family with 114 living adult members, 46 of them with multiple exostoses. Four have had operations for chondrosarcoma, giving the risk for malignant transformation as 8.3% in this family. Clinical and radiological examination revealed two additional patients with a suspicion of malignancy, but in whom the histological findings were benign. Reported elsewhere in detail, genetic linkage analysis mapped the causative gene to chromosome 11 and molecular studies revealed a guanine-to-thymine transversion in the ext2 gene.

Patients with multiple hereditary exostoses carry a relatively high risk of malignant transformation. They should be informed of this possibility and regularly reviewed.

TREATMENT  
SURGERY  


Arthroscopic resection of an osteochondroma of the knee.

Schmoyer S, Ciullo JV.

Sports Medicine Center of Metro Detroit, P.C., Troy, Michigan, U.S.A.

Arthroscopy 2001 Sep;17(7):765-7 Abstract quote

We present a case of arthroscopic resection of a symptomatic periarticular osteochondroma of the knee. A competitive tennis athlete presented to our clinic at the beginning of the season with a contact lesion, a distal femoral osteochondroma, with a 3-year history of painful synovial inflammation and lateral patellar maltracking. She was treated symptomatically throughout the season until it was decided to perform surgery.

The benign bone tumor, a sessile osteochondroma, was arthroscopically resected, which led to complete relief of symptoms and return to full competitive activity within 8 weeks. There has been no return of symptoms in 32 months of follow up.

An arthroscopic resection of a symptomatic osteochondroma may be less painful, more cosmetically accepted, and result in quicker recovery than the traditional open incision approach.

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