JAW SARCOMAS
Sarcomas are rare malignant tumors that develop in osteocartilaginous tissues and soft connective tissues and are of mesenchymal origin.
Their evolution is rapid, invading the surrounding tissues. They have a high potential for dissemination, far from the primary tumor in the form of metastases.
At the head and neck level they represent a group of very heterogeneous tumors with non-specific symptoms which delays their diagnosis.
- 15 to 20% of sarcomas are located in the cervicofacial region
- The majority of head and neck sarcomas arise in soft tissue.
- They are rare in the jaws, represented mainly by osteosarcomas and chondrosarcomas.
- They mainly affect children and young people under the age of 20, and can also be found after the age of 50.
- ETIOPATHOGENESIS
The etiology is still unknown, however risk factors have been identified:
- Malignant transformation from benign bone tumors (Fibrous dysplasia – ossifying fibroma: especially Osteosarcoma).
- Ionizing radiation: (Osteosarcoma, Fibrosarcoma)
- Viral cause: like EBV
- Other environmental causes (physical-chemical such as herbicides and pesticides) that can cause genetic alterations
- DIAGNOSIS OF SARCOMAS
- Clinical signs
The symptomatology is non-specific, the clinical signs are mild at the beginning, and in advanced forms we find:
- A large, firm, or hard elastic swelling that can distort the face
- Almost always constant pain found in advanced forms
- There is a cutaneous fixity at the beginning, then tumor infiltration with a purplish color and ulceration
- Lymphadenopathy is rare in maxillary sarcomas.
- The mucosa is normal or congestive, or manifested by vestibular filling
- Dental signs are inconstant and late, such as movement, tooth mobility, and spontaneous loss.
- Hypoesthesia or anesthesia in the territory of the infraorbital nerve or the alveolar nerve
- Nasal obstruction, epistaxis, and sometimes exophthalmos may also be observed.
- Radiological signs:
- Standard X-rays ( panoramic, maxillary defilement, Blondeau, Hirtz, etc.) are insufficient to suggest a diagnosis:
- Often appearance of poorly defined irregular osteolysis with blurred contours, with cortical rupture
- Rarely : a mixed image: with an osteocondensing , irregular, poorly defined appearance, sometimes surrounded by a transparent halo
- On the dental level we find an irregular desmodontal widening, root lysis, bone lysis with an appearance of hanging teeth
- Scan, finds a periosteal reaction called:
- Grass fire (osteosarcomas++)
- Rays of sunshine,
- Onion bulb (Ewing’s sarcoma).
- Specifies the topography of the pathological process and its extension.
- MRI : specifies extension at the soft tissue level
- Anatomo-pathological study:
- It is essential to establish the diagnosis of certainty of malignancy. It is done after biopsy or total excision of the tumor,
- The biopsy should be the last diagnostic investigation because it can interfere with the reading of certain radiological examinations.
- Extension report
In sarcomas, metastases are very frequent, the extension assessment is systematic after the positive diagnosis:
- Telethorax
- Abdominal ultrasound
- Bone scan
- ENT examination,
- Thoracoabdominal computed tomography
- Skeletal X-ray;
- THE DIFFERENT TYPES OF SARCOMAS
- Sarcomas of odontogenic origin
Odontogenic sarcomas are rare but serious lesions that develop at the expense of tissue of mesenchymal origin.
- Odontogenic sarcomas
Odontogenic sarcomas are a group of mixed odontogenic tumors in which the epithelial component is benign, and the mesenchymal component is malignant.
Ameloblastic fibrosarcoma , a malignant transformation of ameloblastic fibroma, is the most common type (Wright and Soluk-Tekkeşin, 2017).
Previously, odontogenic sarcomas included three distinct entities:
- Ameloblastic fibrosarcoma, which does not produce hard dental tissue,
- Ameloblastic fibrodentinosarcoma, producing dentin
- Ameloblastic fibroodontosarcoma, producing dentin and enamel.
Today, these malignant tumors are grouped under the name “odontogenic sarcomas” by the WHO, corresponding to the malignant degeneration of an ameloblastic fibroma.
Clinical signs:
- The posterior part of the mandible is more frequently affected than the maxilla (Soluk-Tekkeşin and Wright, 2018).
- Odontogenic sarcomas affect patients of all ages, with an average age of around 30 years, with no predilection for either sex.
- If the presence of an ameloblastic fibroma is a precursor in half of the cases, the tumor appears de novo the rest of the time.
- Despite its locally aggressive appearance, the appearance of metastases is exceptional.
- The swelling appears painful.
Radiographically:
- Fibroodontosarcomas and fibrodentinosarcomas present opacities corresponding to the production of dental hard tissues while ameloblastic fibrosarcoma is completely clear.
- The image is uni or multilocular, of variable size, with poorly defined boundaries, with cortical rupture, periosteal spur and generally associated with a tooth.
- Extension to neighboring soft tissues is very common.
- Odontogenic carcinosarcoma
- Similar to odontogenic fibrosarcomas, this time both components, epithelial and mesenchymal, present malignant cytological aspects.
- Very few cases have been reported so far, covering an age range of 9 to 63 years, which explains the limited data available.
- A high rate of recurrence and metastases will be noted, demonstrating its aggressive nature.
- The radiological image is thus a large and expansive lesion with blurred boundaries (Speight and Takata, 2017).
- Sarcomas of non-odontogenic origin
- Osteosarcoma
They occur more often before the age of 30 but also after the age of fifty. Clinical signs of osteosarcomas:
- The patient’s age (young subject)
- The rapid progression of the lesion
- The importance and precocity of pain On a biological level:
- An accelerated sedimentation rate
- An increase in alkaline phosphatase On the radiological level:
- Bone resorption, poorly defined, with blurred contours.
- The periosteal reaction,
- Cortical rupture
Osteosarcoma of the mandible: Clinical signs:
- This tumor strikes during the period of skeletal growth, therefore in children or adolescents.
- It most often invades the mandibular body, more at the level of the angle than at the level of the symphysis.
- The pains in the corner, dull and increasing at night.
- Hyperesthesia in the territory of the mental nerve.
- Dental mobility
- A swelling may be the alarm symptom: hard, painless, deforming the vestibule, covered with congestive mucous membrane.
The X-ray examination:
- The bone image can be of two types: lysis or condensation
- A blurred outline lysis image
- An irregular, poorly defined condensing image
- The periosteum: the periosteal reaction is rapid, its diagnostic importance is great.
- The cortex: the rupture of the cortex causes the tumor to erupt outside the bone (grass fire or sunbeam image).
Histology:
Highlights atypical osteoblasts with anarchic osteoid tissue. Depending on cell differentiation, the following types are noted:
- Osteoblastic,
- Chondroblastic
- Fibroblastic.
Most often, these different histological types are found within the same tumor
- Chondrosarcoma
It is a rare tumor in its cervicofacial form. It occurs in middle-aged people and particularly affects men.
Characterized by the production by the tumor of tissue reminiscent of the structure of “cartilaginous tissue”.
The most common involvement is the upper jaw (anterior region).
– Clinic:
The symptoms are not specific and are confused with those of osteosarcoma:
Painful swelling.
Dental signs and sensory disturbances
The progression is relatively slower than osteosarcoma
- Radiology:
- Images of poorly defined osteolysis + areas of osteocondensation.
- Periosteal reaction with a sunburst appearance ( nonspecific).
- Periodontal widening,…
– Histology reveals chondrosarcomatous proliferation
- Fibrosarcoma
- It is a well-differentiated tumor that exclusively produces fibroblasts with collagen and reticulin fibers. It is less aggressive than the previous forms,
- Preferentially affects the mandible without distinction of age or sex.
Etiology: It can be:
- Malignant transformation of benign lesions (Fibrous dysplasia)
- Radiation-induced tumor
There are two forms:
- Centromedullary form
- Periosteal (peripheral) form
Radiological aspect found:
- Pure osteolysis, often very limited.
- Absence of periosteal reaction
- The evolution is relatively slow.
The prognosis remains reserved, but more favorable than osteosarcoma.
- Ewing’s sarcoma
- Ewing sarcoma is a high-grade round cell tumor.
- Accounts for 5% of all bone tumors.
- Only 1% of Ewing sarcomas are located in the mandible.
- It mainly affects children between 10 and 15 years old.
- Second most common childhood bone tumor (after osteosarcoma)
Clinical
- The triad of clinical signs, pain, edema and hyperthermia are the most constant signs and represent a source of diagnostic errors suggesting an infectious process delaying the diagnosis
- Other more specific signs are found: dental displacement, paresthesia, exophthalmia, nasal obstruction
- Anemia and pallor
- Hot skin and inconstant lymphadenopathy
X-ray :
- Poorly defined areas of osteolysis
- The periosteal “onion skin” or grass fire reaction
Histologically:
The tumor is composed of small, round blue cells with hyperchromatic nuclei and sparse cytoplasm with numerous foci of necrosis.
- Hematosarcomas:
- Non-Hodgkin’s lymphomas:
The revealing lesions of extra-glandular NHL observed in the cervico-facial region are in the order of 30 to 40% of cases (Haioun et al. 2002) ;
- The average age is 57 years, with a male predominance.
- NHL can affect both bone and soft tissue; most commonly in the palate and mandible, gingival involvement is rare.
- The symptoms are not specific:
- Exo- or endo-oral (vestibular) mandibular swellings
- Firm, painless gingival lesions (lumps)
- Cervical lymphadenopathy is rarely found
- Paresthesia
- Later, trismus, earache, gum ulceration and invasion of soft tissues.
X-ray :
- Poorly defined osteolysis, the lamina dura is often destroyed; as are the dental roots
- Enlargement of the inferior alveolar canal.
- Burkitt’s lymphoma: bone lymphoma = reticulosarcoma = lymphosarcoma
It is a B-cell lymphoma; it is very undifferentiated and aggressive;
The definition of Burkitt’s lymphoma is histological , it is a diffuse proliferation of monoclonal B-type lymphocytes, that is to say from a single cell of origin.
Etiopathogenesis:
- In Africa, the appearance of the tumor is the consequence of several successive infections of the subject, leading to a stimulation of his immune system and more particularly of B lymphocytes = It is the disease of the poor who are often exposed to infections.
- There is an infection by the Epstein Barr virus , generally contracted by the mother, then malaria by plasmodium falciparum , the tumor would then occur, during genetic accidents leading to the translocation (exchange) of chromosomes 8 and 14;
The Clinical Table:
- The evolution is very rapid.
- Within a few months, a mildly painful tumor develops in the upper jaw, with rapid spread to other bone structures, especially the orbit.
- Non-African Burkitt lymphomas are more painful and more common in the mandible;
- Vincent’s sign ++
- dental mobility ++
The pathognomonic histological aspect
There is a diffuse proliferation of B-cell lymphocyte cells, with signs of malignancy, the arrangement of the cells is said to be in a “starry sky”.
Prognosis
- The prognosis is fatal in the absence of treatment,
- Cure can be up to 100% in localized forms and early diagnosis (responds well to treatment).
- We are talking about a curable chemo lymphoma
- Isolated Plasmacytoma and Multiple Myeloma Definition:
- Solitary plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma are different manifestations of the same disease characterized by a monoclonal proliferation of abnormal plasma cells.
- They are part of malignant proliferations of B lymphocytes.
Isolated plasmacytoma:
Clinic:
- Maxillary location is rare; the preferred location is the mandible at body level.
- The tumor may be accompanied by a biological syndrome;
- It can manifest itself through peripheral neurological symptoms or through a
painful or painless swelling ; sometimes tooth mobility.
- Sometimes it is a chance discovery.
X-ray:
We find a multilocular image, with clear limits, not surrounded by a border of osteocondensation; the osteolytic image is punched out.
The diagnosis of isolated bone plasmacytoma is made based on four criteria:
- Biopsy positive
- Absence of other homes
- Bone puncture result
- Biology result (absence or disappearance of Bence Jones protein after treatment)
Histology:
Typical characterized by the presence of more or less mature plasma cells, aligned in single file;
The prognosis : is better for isolated forms than for disseminated forms.
Multiple myeloma (Kahler’s disease):
- Formerly called “Kahler’s disease”;
- Both sexes are affected equally frequently between the ages of 60 and 70 ;
- Black people are said to be affected twice as often as white people;
- This involves the proliferation of clones that synthesize and secrete Ig;
- Maxillofacial localization is found in 1/3 of cases with a preference for the mandible in its angular region.
- The clinical symptomatology is not very specific.
- These tumors are painful, inflammatory in appearance, and sometimes cause peripheral neurological disturbances.
Radiologically :
Appearance of generalized osteoporosis , or multiple osteolytic tumor lesions;
Histology:
Presence of inflammatory cells and plasma cells with nuclear abnormalities.
The prognosis is very reserved despite therapeutic progress.
CONCLUSION
Jaw sarcomas develop in a complex anatomical region with a nonspecific clinical picture and late expression.
Their prognosis remains reserved despite the evolution of anticancer therapies.
Standard X-rays provide little information on the state of the lesion, so it is imperative for any atypical lesion to be supplemented by CT and/or MRI, which allow a reliable diagnostic approach and an extension assessment to implement an appropriate treatment plan as early as possible.
JAW SARCOMAS
Wisdom teeth can cause pain if they erupt crooked.
Ceramic crowns offer a natural appearance and great strength.
Bleeding gums when brushing may indicate gingivitis.
Short orthodontic treatments quickly correct minor misalignments.
Composite dental fillings are discreet and long-lasting.
Interdental brushes are essential for cleaning narrow spaces.
A vitamin-rich diet strengthens teeth and gums.