Facial swellings are commonly encountered in the dental office, the cause of which could range from a congenital etiology to an acquired one or it may even be a manifestation of an underlying systemic disease. The clinician must have a thorough knowledge of the various clinical and imaging manifestations and the sites of occurrence of the various conditions to arrive at the appropriate diagnosis. Facial swellings can be classified into different groups which include acute swellings with inflammation, nonprogressive swellings, and slowly or rapidly progressive swellings. The various imaging modalities like CT and MRI are useful for assessing the extent of the swelling as well as evaluating the soft tissue and osseous involvement of the swelling. Multiple myeloma represents clonal proliferation of plasma cells and is a condition in which a facial swelling might be present, though not common. This paper reports a case of a patient with a unilateral facial swelling, which on investigation led to a diagnosis of multiple myeloma.
Facial swellings are commonly encountered in the dental office and can be a cause of worry to both the patient and the dentist. They may arise due to a wide range of causes ranging from a congenital etiology to an acquired one [
A 58-year-old female patient presented with a swelling on the left side of face which had evolved over the previous two months. Although the patient was treated with antibiotics, the swelling continued to enlarge to reach its present size. The patient had neither dryness of the mouth nor increased salivation, and the swelling was persistent. The patient did not have any associated fever or paresthesia. She was a diagnosed diabetic patient on oral hypoglycemics for the past 4 years.
On extraoral examination, a diffuse ovoid swelling was seen on the left middle and lower third of the face extending anteroposteriorly from the nasolabial fold to the tragus of the ear and superoinferiorly from the zygomatic arch to two centimeters beneath the lower border of the mandible. Palpation revealed a nontender, noncompressible, and nonfluctuant swelling which was firm to hard in consistency. There was a slight reduction in mouth opening (Figure
A diffuse extraoral swelling in the middle and lower one-third of the face.
Intraoral examination revealed a fixed prosthesis in relation to the upper left posterior teeth. The skin overlying the swelling and the oral mucosa were unaltered and were of normal color (Figure
Intraoral examination reveals a normal mucosa and dentition.
Various hematological tests included routine blood tests and an ANA screen (Table
Hematological investigations.
Blood parameter | Values (SI unit) | Reference range |
---|---|---|
RBC | 4.29 |
4.0–5.2 |
WBC | ||
Total |
|
|
Differential | ||
Neutrophils | 0.57 | 0.44–0.68 |
Lymphocytes | 0.32 | 0.25–0.44 |
Monocytes | 0.66 | 0.0–0.07 |
Basophils | 0.12 | 0.0–0.02 |
Eosinophils | 0.175 | 0.0–0.04 |
Platelets | 2.98 |
1.5–4.5 |
Haemoglobin | 12.3 g/dL | 12.2–18.1 g/dL |
ESR | 64 mm/hr | 8.0–20.0 mm/hr |
An orthopantomograph (OPG) revealed an extensive osteolytic lesion with ill-defined margins involving the left mandibular ramus, body, and coronoid with multiple radiolucent lesions and altered trabecular patterns in the right mandibular ramus, body, and condylar region (Figure
A radiolucent lesion with ill-defined ragged borders involving the left ramus, body, and coronoid process of the mandible. The right side of the mandible shows multiple punched out radiolucencies in the body, ramus, and condylar region with altered trabecular pattern.
Contrast enhanced CT showed an expansile lytic and destructive lesion in the left ramus of the mandible with associated soft tissue component and a few enlarged lymph nodes in level 3 on the left side. A lytic lesion was noted in the left frontal bone as well as the right costochondral joint of the second rib (Figures
Axial CT section: Lytic lesion in left frontal bone and an expansile lytic and destructive lesion in the left ramus of the mandible involving the left infratemporal fossa and masseteric space and indenting the lateral wall of the left maxillary sinus.
Coronal and sagittal sections of CT demonstrating the extent of the lesion.
Based on the extensive skeletal involvement and the FNAC report, a bone marrow biopsy was done to assess if the patient had multiple myeloma. The aspirate revealed a cellular marrow with hypocellular fragments and poor cellular characteristics and 18% of plasma cells of which few were binucleate. This warranted further confirmatory investigations for multiple myeloma. Biochemical tests for serum calcium and serum creatinine were within normal limits. Serum electrophoresis revealed decreased albumin (3.98 g/dL), increased globulin (4.8 g/dL), and increased beta-2 microglobulin (2.8 g/dL) levels (Table
Biochemical investigations.
Parameter | Values (SI unit) | Reference range |
---|---|---|
Serum calcium | 8.4 mg/dL | 8.5–11.5 mg/dL |
Serum creatinine | 1.37 mg/dL | 0.84–1.4 mg/dL |
Total protein | 8.75 g/dL | 6.6–8.3 g/dL |
Serum albumin | 3.98 g/dL | 3.5–5.2 g/dL |
Serum globulin | 4.8 g/dL | 2.5–4.0 g/dL |
Immunoglobulin assessment.
Parameter | Value (SI) | Reference range |
---|---|---|
IgG | 617 mg/dL | 751–1560 mg/dL |
IgM | 69.3 mg/dL | 40.0–230.0 mg/dL |
IgA | 3000 mg/dL | 82.0–453.0 mg/dL |
Kappa free light chain | 3.7 mg/L | 3.3–19.4 mg/L |
Lambda free light chain | 16.9 mg/L | 5.7–26.3 mg/L |
Kappa lambda ratio | 1 : 5 | 1 : 1.5 |
Serum beta-2 microglobulin | 2.74 mg/L | 0.8–2.4 mg/L |
A final diagnosis of multiple myeloma was made based on the clinical presentations and investigations, which satisfied the revised diagnostic criteria of International Myeloma Working Group (IMWG) [
PET CT (Figure
PET CT images showing extensive FDG uptake in frontal bone, maxilla, and right costochondral junction of second rib.
Following 16 weeks of chemotherapy, the patient’s IgA levels normalized and she is currently scheduled for autologous stem cell transplantation.
The term “multiple myeloma” was introduced by J. Von Rustizky in 1873 when he found eight separate tumors of the bone marrow which he designated as “multiple myeloma.” The first well-documented case of multiple myeloma was reported by Samuel Solley in 1844 [
Multiple myeloma is a malignant disease characterized by multifocal proliferation of atypical plasma cells and by the presence in the serum of monoclonal gamma globulins, often referred to as “M” or myeloma proteins [
Multiple myeloma involves destructive lesions affecting the bone and the patients present with persistent pain in the bone, especially in the affected areas with a history of recurrent infection, fever, fatigue, and multisystem involvement [
In our case, the patient was a fifty-eight-year-old female with manifestations in the maxillofacial region. Primary manifestation in the maxillofacial region, though not common, usually manifests as pain, swelling, paresthesia, mobility of tooth, hemorrhage, and pathological fractures [
In the present case, the patient had a swelling on the left side of face which was not associated with pain, paresthesia, or mobility or displacement of the teeth.
Radiographic examinations usually reveal osteolytic lesions with irregular, noncorticated margins and multiple punched out radiolucencies with altered trabecular pattern which was seen in the present case too [
The diagnosis of multiple myeloma in our case was established by a multidisciplinary diagnostic protocol involving routine investigations of both serum and urine followed by quantification of immunoglobulins. This was accompanied by cytological examination of bone marrow aspirate and a detailed radiological survey in the form of contrast enhanced CT, PET CT to assess the extent of skeletal involvement. Shah et al. have emphasized the importance of 18F-FDG PET/CT in myeloma patients as it enables detection of unsuspected sites of bone involvement, which upstages the disease [
The patient was staged as stage 1 as per the International Staging System (ISS) (Table
ISS staging of multiple myeloma.
Stage | Median survival time | |
---|---|---|
Stage 1 | Serum beta-2 microglobulin is less than 3.5 (mg/L) and the albumin level is 3.5 (g/dL) or greater | 62 months |
|
||
Stage 2 | The beta-2 microglobulin level is between 3.5 and 5.5 (with any albumin level) |
44 months |
|
||
Stage 3 | Serum beta-2 microglobulin is 5.5 or greater. | 29 months |
Radiotherapy for the symptomatic lesion followed by chemotherapy is the treatment of choice [
The present case reports maxillofacial swelling as the primary manifestation of multiple myeloma, reinforcing the role of the dentist in identification of systemic conditions contributing to maxillofacial manifestations. This facilitates an early and prompt diagnosis, which in turn affects the treatment and prognosis of the patient.
The authors declare that there is no conflict of interests regarding the publication of this paper.