8. Case report
8.3 Third case
The following case was obtained through an internet discussion group47. Mrs. J.H., a 41 year-old woman had consulted her dentist for occasional numbness around the left lower lip. Less than 2 months later she had worsening pain which increased at night and was associated with tooth #38. An orthopantogram (Fig 23 and 24) revealed a large radiolucency around the left third molar tooth. The oral mucosa appeared normal.
Radiologically the patient had additional lytic skeletal lesions. Asides the use of anti-inflammatory medication, the patient medical history was unremarkable.
The patient was referred to an oral maxillo facial surgeon that removed the tooth 38 as well as 70 to 80% of the associated fibrous mass which was discovered during surgery.
The blood smear revealed a slight increase in rouleaux formation. The red blood cells were normal in number and normochromic. The bone marrow biopsy showed mild marrow lymphocytosis, adequate sideroblast iron and storage iron and peripheral blood without specific histologic abnormalitites. The histopathological report revealed a malignant B-cell neoplasm consistent with plasmacytoma or MM.
The laboratory results of the SPEP appeared normal and there were no monoclonal immunoglobulins detected in the immunofixation. This lead to the diagnostic of stage III A non-secretory myeloma. The patient underwent first autologous hematopoietic progenitors cells transplants and chemotherapy with melphalan and the second AHPC transplants 6 months later, after conditioning with melphalan and cyclophosphamide. A biopsy taken of a compressed fracture of L5 was negative for malignancy. The metastatic bone survey remained unchanged. She is presently on 90 mg monthly of Aredia ®. The follow-up at 2 years showed that the patient was in hematologic and bone marrow complete remission with minimal abnormality on the MRI with no evidence of any recurrence.
Fig 23 : Orthopantogram showing lower left radiolucency in premolar and molar region.
Fig 24 : Detailed view of osteolytic lesion. Note ill-defined radiolucent borders and associated root resorbtion.
9. Discussion
Oral lesions may be the first manifestation of MM. The potential clinical symptoms, which are listed on Tab 1, are important awareness signs in the diagnosis and patient management. The lesions are more frequent in the mandible than the maxilla as there is a higher hematopoeisis36.
It was estimated that if orthopantograms were systematically taken, the incidence of jaw involvement in MM would be higher23,77. Proper radiographic analysis may prevent an emergency bleeding condition during dental therapy by establishing a proper treatment planning, as bony changes may be pathognomonic of underlying diseases46.
It is important to recognize that lymphoproliferative disorders such as MM may have little or no widespread lesions and may primarily manifest itself in the jaws4.
Oral lesions may originate from the erosion of bone plate by the tumor. The lesions may present itself as a rubbery expansion of the jaw. It may be covered by the mucosal or be ulcerated inflammation due to chronic trauma76. Gingival bleeding associated with lesions may mimic periodontal or dental abscess. Extensive bleeding resulting from a minor surgical procedure was reported to have lead to the diagnosis of MM49.
The diagnosis of MM requires a clinical, hematological, histological, radiological and biochemical examinations. Biopsies and histological examination are also used in differential diagnostic of plasma cell granuloma lesion (rare exophytic lesion of the gingival) from soft-tissue myeloma (plasmacytomas) which may represent early stages of MM1 .
Radiologically, MM resembles a common dental benign inflammatory infection such as a periapical granuloma or dental cyst. When the lesions are more destructive causing large bone resorptions, other intramedullary bone pathologies such as giant cell granuloma or even a malignancy (i.e. metastatic carcinoma) should be considered in the differential diagnosis.
The radiological differential diagnosis should also include, ameloblastoma, osteoporosis, Langerhans’ cell histocytosis, bone lesions of hyperparathyroidism and osteomalacia.
Typical oral manifestations of MM were present in all three case that we have reported. The first patient, Mrs. D.M., suffered from what appeared to be a common palatal abscess by her general physician. Histological examination revealed a gingival location of myeloma. This is apparently an unusual location according to found literature. Numerous described oral lesions seem to be associated with the lower jaws rather than a palatal location. Palatal enlargement or swelling have also been described by others13,27,29,36,70.
Two other buccal lesions were discovered, although asymptomatic, they were related to underlying radiolucencies.
Further investigation of the latter was not completed, as patient general health did not permit it.
The second patient, Mrs. J. D., suffered from macroglossia due to important amyloid infiltration. As seen in Fig 18, 19, 20 and 21, the extent of the affection involved the tongue and buccal mucosa, which provoked frictional keratosis due to local masticatory trauma. The lateral borders of the tongue showed dental indentations, which reproduced adjacent teeth.
Subcutaneous hemorrhages and macroglossia lead to the discovery of amyloidosis and hence the diagnosis of MM. Amyloidosis had evolved for at least 2 to 3 years before the disease’s identification. The patient suffered from undiagnosed carpal tunnel syndrome which may have lead to an earlier discovery of MM if it had been biopsied. The patient’s impaired health did not permit further treatment of lingual amyloidosis. She had deceased shortly after detecting oral lesions. Miyoshi and Tagucchi45 have described a case with the same tongue and cardiac manifestations45. According to the literature cited in Tab 7, it seems that macroglossia due to amyloid infiltration, is regularly found as the most common oral manifestation of MM.
The third case, Mrs. J.H. presented herself to her dentist complaining of a mobile painful tooth40 associated with numbness of lower lip. The patient was referred to a maxillofacial surgeon who removed the tooth and the surrounding tumor. The mass was described as a grey gelatinous mass surrounding the erupted tooth. The molar tooth exhibited apparent resorption of portions of the root apex. The orthopantogram
shows the importance of the lytic lesion (Fig 22). This case is very similar to those reported by Dhanrajani and Abdulkarim11 and Epstein et al.13.
All three cases are of interest as they all show typical manifestations of MM. In which various oral lesions occur separately or side by side and not necessarily associated.
In the two of the presented cases, MM manifested itself through oral mucosal lesions.
10. Conclusion
To date no single cause has been identified in the etiology of MM. The latter may be the result of several risk factors acting together (radiation, chemicals, genetic factors, impaired immune system, etc.). The consequential malignant plasma cells will propagate and provoke systemic and local manifestations.
Upon discovery of oral lesions, the disease’s course is often advanced which renders treatment difficult. It is important to constantly evaluate patients state of health in order to establish proper treatment keeping into consideration the resulting patients quality of life. Two case reports are described in which the oral lesions were not treated as both deceased shortly after diagnosis of their oral manifestations.
As described above, the third case reports a complete remission at a two years evaluation after removal of oral lesion, two AHPC transplants and chemotherapy.
Oral manifestations of MM are discovered in late stages of the disease. Oral manifestations in MM are not uncommon and may be the first sign or expression of the systemic disease leading in some cases, to the discovery of the disease.
The role of the dentist becomes increasingly important as the general practitioner is often unfamiliar with oral lesions. An early diagnosis of MM may increase the patient’s response and outcome of therapy.
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