Introduction

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare and benign, frequently self-limited histiocytic disorder of unknown etiology.1 The most common clinical findings are broad painless lymphadenopathy accompanied by fever, weight loss, tonsillitis, rhinorrhea, nasal obstruction, and sometimes hepatosplenomegaly.15 Laboratory findings in patients with RDD include an elevated erythrocyte sedimentation rate, anemia, leukocytosis and polyclonal hypergammaglobulinemia.2 RDD classically presents with painless massive cervical lymphadenopathy, which is caused by accumulation of lymphocytes and histiocytes distending the lymph node sinuses. However, the disease may occur at extranodal sites. In most instances, the extranodal sites include the eyes, head and neck, skin, or bone.6,7 

Mandibular cortical grafts are the gold standard in the restoration of intraoral osseous defects. These grafts do not produce immune reactions and are incorporated by osteoclastic resorption with a short healing period when compared with other methods of osseous repair. Intraoral donor sites include the mandibular ramus, angle of the mandible, mandibular symphysis, and mandibular exostoses.

The mandibular ramus provides primarily a dense cortical graft. Other advantages of a mandibular ramus transplant are minimal resorption, maintenance of osseous density, intraoral access, and proximity to the recipient site. Cortical grafts, including the ramus, are reliable donor sites for the reconstruction of the alveolar ridge deformities before dental implant placement. The highest concentrations of promoter proteins (eg, bone morphogenetic proteins) are found in the mandibular cortical grafts.

This report presents a case of an isolated RDD involving the mandible as well as its surgical management, preprosthetic reconstruction, and restoration with dental implants.

Case Report

A 23-year-old woman was referred to our department with a history of pain and swelling in the region of the right mandibular premolars in May 2006. Her dental history revealed second premolar extraction 4 months ago by her general dental practitioner. After tooth extraction, she claimed a persistent pain in the mandibular premolar region for over 2 weeks. She was placed on multiple courses of antibiotic and nonsteroidal anti-inflammatory drug therapy.

Intraoral examination revealed the presence of expansive submucosal mass involving the mandibular premolar region. Lymphadenopathy was not observed in the regional lymph nodes. Routine hematologic and serologic tests did not indicate any abnormal findings, and blood biochemistry was also within normal limits. Panoramic radiography revealed a destructive lytic and ill-defined osseous lesion involving the right mandibular premolar area (Figure 1). An incisional biopsy was performed. Histologic features were consistent with those seen in RDD.

Figures 1–4.

Figure 1. Lytic lesion in the mandibular premolar region. Figure 2. Immunohistochemical staining with anti S-100 protein antibody shows a strong positive reaction in the histiocyte cytoplasms. (S-100 protein antibody, original magnification ×200). Figure 3. Bone scintigraphy and magnetic resonance imaging showing no skeletal involvement of the lesion. Figure 4 . Postoperative panoramic radiograph showing osseous healing at month 16.

Figures 1–4.

Figure 1. Lytic lesion in the mandibular premolar region. Figure 2. Immunohistochemical staining with anti S-100 protein antibody shows a strong positive reaction in the histiocyte cytoplasms. (S-100 protein antibody, original magnification ×200). Figure 3. Bone scintigraphy and magnetic resonance imaging showing no skeletal involvement of the lesion. Figure 4 . Postoperative panoramic radiograph showing osseous healing at month 16.

Treatment consisted of complete enucleation of the residual lesion. All affected teeth were extracted (No. 28 and No. 30), and the lesion was removed with all parts.

Histopathology

Microscopically, the biopsy specimen showed the presence of an infiltrate including histiocytes, plasma cells, and lymphocytes occupying the bone lamellae. Immunoperoxidase staining for S-100 protein was carried out on the majority of the histiocytes were positively stained (Figure 2).

Whole body magnetic resonance imaging revealed no lymph node involvement, and bone scintigraphy showed no skeletal involvement of the lesion (Figure 3). The patient had neither local nor multisystem involvement nor lymphadenopathy.

The patient was followed weekly in the first month and every 2 weeks in the following 3 months. Orthodontic treatment was initiated at the end of 4 months of uneventful healing in order to preserve the edentulous alveolar arch and prepare an appropriate dental alignment for prosthetic restoration by correcting the dental crowding. Control radiographs obtained at 16 months showed new bone formation in the operated mandible (Figure 4). However, prosthetic rehabilitation was not possible without preprosthetic reconstructive surgery. A second surgery was performed for augmenting the horizontal deficiency of the alveolar crest 20 months after the first operation.

Autogenous bone take from the ipsilateral ramus was chosen as a block graft. During the operation a cortical bone block, about 2 cm long, 1.5 cm high, and 3 mm thick, was harvested and placed horizontally to the deficient edentulous alveolar ridge as lateral onlay graft and fixed with titanium osteosynthesis screws (Figures 5 and 6).

Figures 5–8.

Figure 5. Harvesting of corticocancellous ramus block graft. Figure 6. Fixation of the ramus block graft. Figure 7. Panoramic view showing dental implants in the grafted ridge. Figure 8. Intraoral view with final prosthesis.

Figures 5–8.

Figure 5. Harvesting of corticocancellous ramus block graft. Figure 6. Fixation of the ramus block graft. Figure 7. Panoramic view showing dental implants in the grafted ridge. Figure 8. Intraoral view with final prosthesis.

Fixed prosthesis supported by dental implants was the choice of treatment for restoration of the edentulous segment 6 months after grafting surgery. The fixation screw used for stabilization of the bone graft was removed and two dental implants (3.7 × 11.5 mm and 3.7 × 10 mm; Implant Direct LLC, Calif) were placed (Figure 7). Two months thereafter, implant exposure and abutment screw-connections were carried out, and a definitive prosthetic fixed bridge was screwed to the implants (Figure 8). The patient was satisfied with the esthetics and function of the prosthesis.

Three years after surgery the patient was free from recurrences and symptoms, with normal nerve function.

Discussion

RDD is a rare pathological entity, non-neoplastic histiocytosis affecting nodal and extranodal sites. The most common clinical finding is painless, massive cervical lymphadenopathy.1 Patients affected by RDD are usually in the first two decades of life.8,9 The clinical signs and symptoms may be characterized by nonspecific symptoms including fever, weight loss, cellulitis, and neutrophilia.1 The patient in this report complained of localized mandibular pain and swelling and did not demonstrate any other symptoms.

Radiographically, RDD has a variable appearance such as either well or poorly defined radiolucencies; mixed radiolucent-radiopaque and sclerotic lesions have also been described.9,10 

In the head and neck region, RDD has been identified in the nasal cavity, paranasal sinuses, soft tissues, orbit, major salivary glands, larynx, pharynx, tonsils, thyroid gland, ear, maxilla, mandible, and facial skeleton.7,1113 

Foucar et al7 reported 33 cases with osseous manifestations and showed a frequency of bone involvement of less than 10%; only 9 of 33 patients in the RDD has osseous involvement without lymphadenopathy. Three of the 9 patients lacked other sites of extranodal involvement and had isolated osseous disease on presentation.

There is only one paper reporting extranodal mandibular involvement of RDD in a 32-year-old pregnant white woman presenting with ill-defined, lytic lesions in the mesial root of the left mandibular first molar.14 The patient was treated by extraction of the left mandibular first molar tooth and complete enucleation of the residual lesion. No complications or recurrences were reported. This case of ours is the second extranodal mandibular RDD in the literature.

Appropriate treatment of RDD is still controversial. It is believed that the predominance of patients with RDD do not require treatment because the disease usually progresses with spontaneous resolution. For more complicated RDD, or for patients with disseminated disease, more aggressive therapeutic intervention is often required.15 In a recent review of treatment of RDD, chemotherapy with a combination of vinca alkaloid, alkylating agent, and corticosteroid was thought to be the most effective treatment.

Conservative treatment for solitary osseous RDD lesions is generally indicated, consisting of complete enucleation or curettage, with close long-term follow-up. Prognosis for the intraosseous lesions is usually excellent, and recurrences are generally limited. Most patients with solitary extranodal lesions exhibit the best prognosis. In the presented case, enucleation and curettage was the treatment of choice since the patient appeared to have only a solitary mandibular lesion, and enucleation surgery was observed to have been efficient for the treatment of RDD without chemotherapy, radiation therapy, or other medications.

Horizontal alveolar deficiency, which came out as a result of surgery, was reconstructed with autogenous bone graft. Many techniques, such as alloplastic bone or endochondral autogenous bone grafting, ridge splitting, and guided tissue regeneration have been used in alveolar ridge augmentation. However, significant resorption has been reported in alloplastic and endochondral autogenous bone grafting.16 Other disadvantages of endochondral autogenous bone grafting include donor site morbidity and the need for hospitalization and general anesthesia.17 Intramembranous bone grafts undergo accelerated revascularization and consolidation, and thus exhibit less resorption than endochondral grafts.18,19 Mandibular symphysis and ramus are the sources for intraoral bone block grafts. However, compared with the symphysis, the mandibular ramus area provides good bone quality with fewer postoperative complications. In the presented case, autogenous bone graft harvested from the mandibular ramus was preferred. Donor site healing in our patient was uneventful, and dental implants on the grafted site were placed, leading to optimal functional and esthetic results with no recurrence or complications.

It is concluded that enucleation with curettage can be used in patients with extranodal RDD. Though surgery left an edentulous horizontal alveolar ridge defect, there still remained adequate volume of the bone which can be reconstructed by minor grafting procedures. Use of a mandibular ramus donor graft made the surgical protocol easier to manage and was better tolerated by the patient.

Abbreviation

     
  • RDD

    Rosai-Dorfman disease

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