Fibrocartilaginous mesenchymoma
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Fibrocartilaginous mesenchymomas are very rare locally aggressive mesenchymal bone tumours seen in children and adolescents.
Epidemiology
Fibrocartilaginous mesenchymomas are very rare tumours. They occur in children, adolescents and young adults up to the third decade 1-3. The male gender is slightly more frequently affected 1.
Diagnosis
The diagnosis of osteosarcomafibrocartilaginous mesenchymoma is based on a combination of typical radiographic and pathological features as well as the age of the patient.
Diagnostic criteria
Diagnostic criteria according to the WHO classification of bone tumours (2020 - blue book)1:
- imaging features of a bone tumour
- spindle cells with mild atypia
- areas with growth plate-like hyaline cartilage and trabecular bone
An additional desirable criterium is that the patient is younger than 30 years.
Clinical presentation
The tumours might present as swelling and/or painful lesions or might be entirely asymptomatic and incidentally found 1.
Pathology
Fibrocartilaginous mesenchymomas is a tumour made up of mildly atypical spindle cells and growth plate-like cartilaginous nodules with bony trabeculae 1-5.
Aetiology
The aetiology of fibrocartilaginous mesenchymoma is unknown1.
Location
Fibrocartilaginous mesenchymoma have been most commonly found in the following locations 1-5:
- long bones (often in the metaphysis)
- pelvic bones
- vertebrae
- metatarsal bones
- ribs
Macroscopic appearance
Macroscopically fibrocartilaginous mesenchymomas have a tannish-white appearance with interspersed bluish-grey shimmering cartilaginous areas. There might be cortical destruction in resected specimens 1.
Microscopic appearance
Microscopically fibrocartilaginous mesenchymomas are characterised by the followinghistological features 1:
- hypocellular to a moderately cellular spindle cell component
- spindle cells arranged in bundles or fascicles
- slightly hyperchromatic mildly atypical nuclei
- rare or absent mitoses
- benign
appearing-appearing cartilaginous nodules with chondrocytes arranged in parallel columns - areas of enchondral ossification
- occasional multinucleated giant cells
- infiltration of the host bone
Immunohistochemistry
Immunohistochemistry stains are not helpful in the diagnosis 1.
Radiographic features
Fibrocartilaginous mesenchymomas appear as lytic expansile lesions on imaging often in an eccentric location 1-5.
Plain radiograph
On plain radiographs, fibrocartilaginous mesenchymomas appear as lytic expansile lesions with a hazy internal matrix, ring-like calcifications and features of cortical thinning and/or destruction 1,3.
CT
CT might show an expansile lytic tumour with calcifications, cortical thinning or cortical destruction and extension in the adjacent soft tissues 1.
MRI
MRI show an expansile lesion with dense fibrous areas mixed with epiphyseal chondroid components and features, cortical destruction and soft tissue extension.
Signal characteristics are usually as follows 1:
- T1: low signal intensity
- T2: high signal intensity
- T1 C+ (Gd): strong enhancement
Radiology report
The radiological report should include a description of the following:
- form and location
- tumour margins and transition zone
- endosteal scalloping
- cortical breakthrough
- infiltration of the surrounding tissues
Treatment and prognosis
Management is surgical and excision with wide margins is considered best. Local recurrences might occur after incomplete excision. Metastases or tumour related-related death have not been described 1-3.
History and etymology
The entity was first described by Dahlin et al. in 1984 3-5.
Differential diagnosis
Conditions whichthat can mimic the appearance of a fibrocartilaginous mesenchymoma include 1-5:
-<p><strong>Fibrocartilaginous mesenchymomas</strong> are very rare locally aggressive mesenchymal bone tumours seen in children and adolescents.</p><h4>Epidemiology</h4><p>Fibrocartilaginous mesenchymomas are very rare tumours. They occur in children, adolescents and young adults up to the third decade <sup>1-3</sup>. The male gender is slightly more frequently affected <sup>1</sup>.</p><p>Diagnosis</p><p>The diagnosis of osteosarcoma is based on a combination of typical radiographic and pathological features as well as the age of the patient.</p><p>Diagnostic criteria</p><p>Diagnostic criteria according to the <a href="/articles/who-classification-of-tumors-of-bone">WHO classification of bone tumours (2020 - blue book)</a><sup>1</sup>:</p><ul>- +<p><strong>Fibrocartilaginous mesenchymomas</strong> are very rare locally aggressive mesenchymal bone tumours seen in children and adolescents.</p><h4>Epidemiology</h4><p>Fibrocartilaginous mesenchymomas are very rare tumours. They occur in children, adolescents and young adults up to the third decade <sup>1-3</sup>. The male gender is slightly more frequently affected <sup>1</sup>.</p><h4>Diagnosis</h4><p>The diagnosis of fibrocartilaginous mesenchymoma is based on a combination of typical radiographic and pathological features as well as the age of the patient.</p><h5>Diagnostic criteria</h5><p>Diagnostic criteria according to the <a href="/articles/who-classification-of-tumors-of-bone">WHO classification of bone tumours (2020 - blue book)</a><sup>1</sup>:</p><ul>
-</ul><p>An additional desirable criterium is that the patient is younger than 30 years.</p><p>Clinical presentation</p><p>The tumours might present as swelling and/or painful lesions or might be entirely asymptomatic and incidentally found <sup>1</sup>.</p><p>Pathology</p><p>Fibrocartilaginous mesenchymomas is a tumour made up of mildly atypical spindle cells and growth plate-like cartilaginous nodules with bony trabeculae <sup>1-5</sup>.</p><p>Aetiology</p><p>The aetiology of fibrocartilaginous mesenchymoma is unknown.</p><p>Location</p><p>Fibrocartilaginous mesenchymoma have been most commonly found in the following locations <sup>1-5</sup><strong>:</strong></p><ul>-<li>long bones (often in the metaphysis)</li>- +</ul><p>An additional desirable criterium is that the patient is younger than 30 years.</p><h4>Clinical presentation</h4><p>The tumours might present as swelling and/or painful lesions or might be entirely asymptomatic and incidentally found <sup>1</sup>.</p><h5>Pathology</h5><p>Fibrocartilaginous mesenchymomas is a tumour made up of mildly atypical spindle cells and growth plate-like cartilaginous nodules with bony trabeculae <sup>1-5</sup>.</p><h5>Aetiology</h5><p>The aetiology of fibrocartilaginous mesenchymoma is unknown <sup>1</sup>.</p><h5>Location</h5><p>Fibrocartilaginous mesenchymoma have been most commonly found in the following locations <sup>1-5</sup><strong>:</strong></p><ul>
- +<li>
- +<a title="Long bones" href="/articles/long-bones">long bones</a> (often in the metaphysis)</li>
-<li>vertebrae</li>-<li>metatarsal bones</li>-<li>ribs</li>-</ul><p>Macroscopic appearance</p><p>Macroscopically fibrocartilaginous mesenchymomas have a tannish-white appearance with interspersed bluish-grey shimmering cartilaginous areas. There might be cortical destruction in resected specimens <sup>1</sup>.</p><p>Microscopic appearance</p><p>Microscopically fibrocartilaginous mesenchymomas are characterised by the following histological features <sup>1</sup>:</p><ul>-<li>hypocellular to moderately cellular spindle cell component<ul>- +<li><a title="Vertebrae" href="/articles/vertebra">vertebrae</a></li>
- +<li><a title="Metatarsal bone" href="/articles/metatarsals">metatarsal bones</a></li>
- +<li><a title="Ribs" href="/articles/ribs">ribs</a></li>
- +</ul><h5>Macroscopic appearance</h5><p>Macroscopically fibrocartilaginous mesenchymomas have a tannish-white appearance with interspersed bluish-grey shimmering cartilaginous areas. There might be cortical destruction in resected specimens <sup>1</sup>.</p><h5>Microscopic appearance</h5><p>Microscopically fibrocartilaginous mesenchymomas are characterised by the following histological features <sup>1</sup>:</p><ul>
- +<li>hypocellular to a moderately cellular spindle cell component<ul>
-<li>benign appearing cartilaginous nodules with chondrocytes arranged in parallel columns</li>- +<li>benign-appearing cartilaginous nodules with chondrocytes arranged in parallel columns</li>
-</ul><p>Immunohistochemistry</p><p>Immunohistochemistry stains are not helpful in the diagnosis <sup>1</sup>.</p><p>Radiographic features</p><p>Fibrocartilaginous mesenchymomas appear as lytic expansile lesions on imaging often in an eccentric location <sup>1-5</sup>.</p><p>Plain radiograph</p><p>On plain radiographs fibrocartilaginous mesenchymomas appear as lytic expansile lesions with a hazy internal matrix, ring-like calcifications and features of cortical thinning and/or destruction <sup>1,3</sup>.</p><p>CT</p><p>CT might show an expansile lytic tumour with calcifications, cortical thinning or cortical destruction and extension in the adjacent soft tissues <sup>1</sup>.</p><p>MRI</p><p>MRI show an expansile lesion with dense fibrous areas mixed with epiphyseal chondroid components and features, cortical destruction and soft tissue extension. </p><p>Signal characteristics are usually as follows <sup>1</sup>:</p><ul>- +</ul><h5>Immunohistochemistry</h5><p>Immunohistochemistry stains are not helpful in diagnosis <sup>1</sup>.</p><h4>Radiographic features</h4><p>Fibrocartilaginous mesenchymomas appear as lytic expansile lesions on imaging often in an eccentric location <sup>1-5</sup>.</p><h5>Plain radiograph</h5><p>On plain radiographs, fibrocartilaginous mesenchymomas appear as lytic expansile lesions with a hazy internal matrix, ring-like calcifications and features of cortical thinning and/or destruction <sup>1,3</sup>.</p><h5>CT</h5><p>CT might show an expansile lytic tumour with calcifications, cortical thinning or cortical destruction and extension in the adjacent soft tissues <sup>1</sup>.</p><h5>MRI</h5><p>MRI show an expansile lesion with dense fibrous areas mixed with epiphyseal chondroid components and features, cortical destruction and soft tissue extension. </p><p>Signal characteristics are usually as follows <sup>1</sup>:</p><ul>
-</ul><p>Radiology report</p><p>The radiological report should include a description of the following:</p><ul>- +</ul><h4>Radiology report</h4><p>The radiological report should include a description of the following:</p><ul>
-</ul><p>Treatment and prognosis</p><p>Management is surgical and excision with wide margins is considered best. Local recurrences might occur after incomplete excision. Metastases or tumour related death have not been described <sup>1-3</sup>.</p><p>History and etymology</p><p>The entity was first described by Dahlin et al. in 1984 <sup>3-5</sup>.</p><p>Differential diagnosis</p><p>Conditions which can mimic the appearance of a fibrocartilaginous mesenchymoma include <sup>1-5</sup>:</p><ul>-<li>fibrous dysplasia or </li>-<li>giant cell tumour of bone</li>-<li>chondroblastoma</li>- +</ul><h4>Treatment and prognosis</h4><p>Management is surgical and excision with wide margins is considered best. Local recurrences might occur after incomplete excision. Metastases or tumour-related death have not been described <sup>1-3</sup>.</p><h4>History and etymology</h4><p>The entity was first described by Dahlin et al. in 1984 <sup>3-5</sup>.</p><h4>Differential diagnosis</h4><p>Conditions that can mimic the appearance of a fibrocartilaginous mesenchymoma include <sup>1-5</sup>:</p><ul>
- +<li>
- +<a title="Fibrous dysplasia" href="/articles/fibrous-dysplasia">fibrous dysplasia</a> or <a title="Osteofibrous dysplasia" href="/articles/osteofibrous-dysplasia-2">osteofibrous dysplasia</a>
- +</li>
- +<li><a title="Giant cell tumour of bone" href="/articles/giant-cell-tumour-of-bone">giant cell tumour of bone</a></li>
- +<li><a title="Chondroblastoma" href="/articles/chondroblastoma">chondroblastoma</a></li>
-<li>aneurysmal bone cyst</li>-<li>dedifferentiated chondrosarcoma</li>- +<li><a title="Aneurysmal bone cyst" href="/articles/aneurysmal-bone-cyst">aneurysmal bone cyst</a></li>
- +<li><a title="Dedifferentiated chondrosarcoma" href="/articles/dedifferentiated-chondrosarcoma">dedifferentiated chondrosarcoma</a></li>
-<li>low-grade osteosarcoma</li>- +<li><a title="Teleangiectatic osteosarcoma (TOS)" href="/articles/telangiectatic-osteosarcoma">teleangiectatic osteosarcoma</a></li>
References changed:
- 1. Gambarotti M, Inwards CY. Fibrocartilaginous mesenchymoma. In: WHO Classification of Tumours Editorial Board. Soft tissue and bone tumours. Lyon (France): International Agency for Research on Cancer; 2020. (WHO classification of tumours series, 5th ed.; vol. 3). <a href="https://publications.iarc.fr/Book-And-Report-Series/Who-Classification-Of-Tumours/Soft-Tissue-And-Bone-Tumours-2020">https://publications.iarc.fr</a>
- 2. Gambarotti M, Righi A, Vanel D et al. Fibrocartilaginous Mesenchymoma of Bone: A Single-Institution Experience with Molecular Investigations and a Review of the Literature. Histopathology. 2017;71(1):134-42. <a href="https://doi.org/10.1111/his.13201">doi:10.1111/his.13201</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28239886">Pubmed</a>
- 3. Lin J, Shulman S, Steelman C et al. Fibrocartilaginous Mesenchymoma, a Unique Osseous Lesion: Case Report with Review of the Literature. Skeletal Radiol. 2011;40(11):1495-9. <a href="https://doi.org/10.1007/s00256-011-1189-0">doi:10.1007/s00256-011-1189-0</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/21560005">Pubmed</a>
- 4. Oh S. Fibrocartilaginous Mesenchymoma with an Unusual Location in the Rib. J Pathol Transl Med. 2021;55(1):75-8. <a href="https://doi.org/10.4132/jptm.2020.10.08">doi:10.4132/jptm.2020.10.08</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/33260287">Pubmed</a>
- 5. Dahlin D, Bertoni F, Beabout J, Campanacci M. Fibrocartilaginous Mesenchymoma with Low-Grade Malignancy. Skeletal Radiol. 1984;12(4):263-9. <a href="https://doi.org/10.1007/bf00349507">doi:10.1007/bf00349507</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/6505732">Pubmed</a>
Systems changed:
- Musculoskeletal
- Oncology
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