V. VALENTINI', V. TERENZI', A. CASSONI', S. BOSC02, E. BRAUNER I, J. SHAHINAS I

Transcription

1 EUROPEAN JOURNAL OF INFLAMMATION Vol. 10, no. I, (2012) DENTISTRYSECTION GIANT CELL LESION OR LANGERHANS' CELL HISTIOCYTOSIS OF THE MANDIBLE? A CASE REPORT V. VALENTINI', V. TERENZI', A. CASSONI', S. BOSC02, E. BRAUNER I, J. SHAHINAS I andg. POMPN IDepartment oforal and Maxillo-Facial Surgery, "Sapienza" University, Rome, Italy; 2Department of Molecular Medicine, "Sapienza" University, Rome, Italy. ArrivedDecember 29, AcceptedApril 11, 2012 Langerhans' cell histiocytosis (LCH), formerly known as histiocytosis X, is characterized by cell proliferation. The leading clinical symptom of LCH within mandibular and maxillary bones is pain and it may resemble periodontal diseases, apical cysts, ameloblastoma, central giant cell granuloma, vascular malformation, osteomyelitys, bone metastasis and malignancies. In this paper we present the case of a mandibular histiocytosis misdiagnosed as a reparative giant cell granuloma at the first and as an aggressive giant cell tumor at the second biopsy. Definitive diagnosis was made only after examination of surgical specimens following hemimandibulectomy. The most frequent radiolucent lesions involving the mandible include ameloblastoma, odontogenic tumors, central giant cell granuloma, and odontogenic keratocyst, even if other bone lesions, such as plasmocitoma, histiocytosis X, rarely involving jaws, can present in a similar way. Langerhans' cell histiocytosis (LCH), formerly known as histiocytosis X, is characterized by the proliferation of cells causing local or systemic effects. Three classic clinical syndromes considered to be clinical variations ofthe same disease are: a) eosinophilic granuloma (EG, localized benign form, isolated to bone and often monostotic), b) Hand Schuller-Christian disease (classic triad of skull lesions, exophthalmos, and diabetes insipidus), and c) Letterer-Siwe disease (disseminated lesions involving multiple visceral organs) (I). We present the case ofa mandibular histiocytosis misdiagnosed as a giant cell lesion. Case report A 36-year-old male was sent to the Department of Oral and Maxillofacial Surgery, "Sapienza" University of Rome, by his dentist for evaluation of a radiolucent mandibular lesion seen in the Dental Panoramic Radiograph (DPR). It showed a lucent, fairly well-defined area in the body of the left mandible involving the second molar and partially the first molar tooth. Loss of the lamina dura of the second molar that appeared mobile, was observed (Fig. I). Second and third molar teeth extraction and a biopsy of the underlying lesion were performed: after histological examination, diagnosis of giant cell reparative granuloma was made (Fig. 2). Surgical courettage was performed, and pathological examination of the specimens showed fibrous hyperplasia and inflammatory infiltration that confirmed the diagnosis ofreparative granuloma. Two DPRs, consecutively performed Key words: giant cell tumor, giant cell reparative granuloma, Langerans'cell hystiocitosis Mailing address: Prof. Giorgio Pompa, Department oforal and Maxillofacial Surgery, Sapienza University of Rome, Via Caserta 6, Rome, Italy Tel: Fax: giorgio.pompa@uniromal.it X (2012) Copyright by B10LIFE, s.a.s, This publication and/or article is for individual use only and may not be further reproduced without written permission from the copyright holder. Unauthorized reproduction may result in financial and other penalties 159 DISCLOSURE: ALLAUTHORS REPORT NO CONFLICTS OF INTEREST RELEVANT TO THIS ARTICLE.

2 160 V. VALENTINI ET AL. Fig. 1. Dental panoramic radiograph before teeth extraction showing an osteolytic lesion ofthe left mandible. Fig. 2. Hematoxylin eosin stain (40x HPF) ofthe specim en offirst biopsy. The lesion was diagnos ed as a reparative giant cell granuloma. at a distance of 1 month from each other, indicated a growing mass of the mandible (Fig. 3, a, b). CT scan with contrast (non-ionic iodinated contrast) (Fig. 4) showed a 35 mm ostheolytic area in the left mandibular body involving inferior alveolar nerve canal. Reactive lymphadenopathy was observed. The patient presented hyperemic gingival swelling of the left mandible and initial inferior alveolar

3 European Journal of Inflammation 161 Fig. 3. A) Dental panoramic radiograph performed after surgery. B) Control dental panoramic radiograph one month later. nerve deficit. Clinical and radiological findings seemed to be in discordance with the diagnosis of reparative granuloma so that a second biopsy was performed. Following histological examination, a diagnosis of aggressive giant cell tumor was made. Left hemimandibulectomy with contemporary reconstruction with an iliac crest free flap was performed (Fig. 5). The histological examination of the surgical specimens revealed diffuse proliferation of large mononuclear histiocytic cells (Langerhans cells) with eosinophilic cytoplasm. Abundant numbers of eosinophils and lymphocytes were observed. Histiocytic cells exhibited nuclear and cytoplasmic immunohistochemical positivity to CDla and to S-IOO protein (partially); Ki67 proliferation index was inferior to I%, and p63 was negative. These cells were found in an adjacent lymph node, so that diagnosis of Langherans' cell hystiocitosis of the mandible with nodal involvement was made (Fig. 6). Total body CT scan was unremarkable for distant metastasis. Patient chemotherapy treatment consisted of vincristine and prednisone. The followup six months after the primary treatment showed no signs of local recurrence. DISCUSSION Giant cell lesions are localized benign but occasionally aggressive osteolytic, vascularized mesenchymal spindle cell proliferations with presence of osteoclast-like multinucleated giant cells. The giant cells are usually clustered in areas of hemorrhage and hemosiderin deposition, while

4 162 V. VALE NTINI ET AL. Fig. 4. C'I scan coronal view (A) CTscan axial view (B) with contrast material. Fig. 5. Surgical specimen: lej i hemimandibulectomy with adjacent lymph node (arrow). trabeculae of woven bone are seen in the periphery or in fibrous septae traversing the stroma (2).The radiographic features of central giant cell granuloma vary. Early lesions are usually small, unilocular areas of lucency that can mimic an odontogenic cyst (3). Giant cell tumors are classified as aggressive or non-aggressive based on clinical and radiographic criteria. The primary criteria are: tumors greater than 5 cm in size or those that recur after treatment, while secondary criteria are root resorption, tooth displacement, rapid growth (noticeable size increase within 2 weeks) or cort ical bone thinning or perforation. To be diagnosed as an aggressive tumor, the tumor would have to have at least I primary or at

5 European Journal of Inflammation 163 Fig. 6. A) Hematoxylin eosin stain (20x HPF) of the mandibular specimen; presence ofgiant cells (black arrows); B) Immunohistochemistry of mandibular specimen (20x HPF) showing positivity to CDla (black arrow); C) Hematoxylin eosin stain (20x HPF) ofthe lymph node; presence ofgiant cells (black arrow); D) Immunohisto chemistry oflymph node (20x HPF) showing positivity to CD Ia (black arrow). least 3 secondary criteria evident (4). Langerhans' cell histiocytosis (LCH), formerly known as histiocytosis X, is characterized by the proliferation of Langherans cells. The leading clinical symptom of LCH within mandibular and maxillary bones is pain which sometimes is misdiagnosed as a marginal peridontal infection. In fact, they may resemble periodontal diseases, apical cysts, ameloblastoma, central giant cell granuloma, vascular malformations, osteomyelitys, bone metastasis and malignancies (5, 6). Final diagnosis of LCH can be made only by histological examination. Langerhans cells are large mononuclear histiocytic cells round or oval in shape, with a vesicular nucleus, a moderate quantity of eosinophilic cytoplasm, and laminated or dispersed distribution. Abundant eosinophils and other inflammatory cells, such as lymphocytes and mononuclear phagocytes, may be found accompanying these cells (7). Multinuclear giant cells are frequently seen (8). Through immunohistochemistry, tumor cells usually express S-IOO and CD Ia. The detection of cytoplasmic inclusion bodies known as Birbeck-Breatnach granules is a typical characteristic of LCH (9). Oral manifestations may be the first signs oflch, and on some occasions the oral cavity may be the only area affected. The incidence of oral lesions in LCH is 77%. There is as yet no clear standard protocol for LCH. Treatment depends upon the severity and progression of clinical signs and symptoms and include s the observation for spontaneous regression, surgical curettage, radiation, topical injection of steroids, systemic steroid therap y and chemotherapy. Surgical curettage may be indicated in easily accessible, monostotic lesions without massi ve destruction of the bone (10). Radiotherapy has been proposed for non-accessible lesions, for those which compromise vital structures such as the optic nerve, and for recurrence of previously resected lesions. When lymphatic nodes are involved, the most adequate treatment is excision of the affected nodes (II). With adequate treatment, the prognosis

6 164 V.VALENTINI ET AL. of unifocal LCH lesion is generally excellent, but, in very rare instances, the lesion may recur rapidly within a short period and lead to a fatal outcome (12) The most important factors that may worsen the prognosis are: firstly, visceral involvement (liver, lung, bone marrow), as this has a negative effect on survival; secondly, where age at first presentation is under two years since the mortality rate rises to 50%; thirdly, when the disease spreads to various bones or soft tissues. In general, it is considered that the younger the patient, the worse the prognosis (13). In our case, surgical resection was performed in order to treat an aggressive giant cell tumor of the mandible. In fact, these tumors are locally aggressive with the possible recurrence at or near the site of presentation; metastases are described in only 5% to 10% of cases, usually in lungs. The rapid growth ofthe lesion seemed to be in agreement with the histological diagnosis of aggressive giant cell tumor, but only after pathological examination of the surgical specimen definitive diagnosis of mandibular LCH was done. During histological evaluation of specimens from the periradicular inflamed granulation tissue, the oral pathologist must always consider Langerhans' cell histiocytosis before making a definitive diagnosis (14). REFERENCES 1. Azouz EM, Saigal G, Rodriguez MM, Podda A. Langerhans' cell histiocytosis: pathology, imaging and treatment ofskeletal involvement. PediatrRadiol 2005; 35: Jundt G. Central giant cell lesion. In: Barnes L, Eveson JW, Reichert P, Sidransky D, editors. World health organization classification of tumors. Pathology and genetics. Head and neck tumors. Lyon: IARC Press; p Scholl RJ, Kellett HM, Neumann DP, Lurie AG. Cysts and cystic lesions of the mandible: clinical and radiologic-histopathologic review. Radiographies 1999; 19(5): Chuong R, Kaban LB, Kozakewich H, Perez Atayde A. Giant cell lesions of the the jaws: A clinicopathologic study. J Oral Maxillofac Surg 1986; 44: Jones LR, Toth BB, Cangir A. Treatment for solitary eosinophilic granuloma of the mandible by steroid injection: report of a case. J Oral Maxillofac Surg 1989; 47: A. Eckardt, A. Schultze Maxillofacial manifestations of Langerhans cell histiocytosis: a clinical and therapeutic analysis of 10 patients. Oral Oncology 2003; 39: Madrigal-Martinez-Pereda C, Guerrero-Rodriguez V, Guisado-Moya B, Meniz-Garcia e. Langerhans cell histiocytosis: Literature review and descriptive analysis oforal manifestations. Med Oral Patol Oral Cir Buca12009; 14:E Muramatsu T, Hall GL, Hashimoto S. Clinico Pathologic Conference: Case 4. Head and Neck Pathol201O; 4: Liebermann PH, Steinmann RM, Smith J, Garin Chesa P, Urmacher C, Sperber M. Langerhans cell (eosinophilic) granulomatosis. A clinicopathologic study encompassing 50 years. Am J Surg Pathol 1996; 20: Esen A, Dolanmaz D, Kalayci A, Gunhan 0, Avunduk Me. Treatment of localized Langerhans' cell histiocytosis ofthe mandible with intralesional steroid injection: report of a case. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010; 109:e53-e58. II. Garcia de Marcos JA, Dean Ferrer A, Alamillos Granados F. Langerhans cell histiocytosis in the maxillofacial area in adults. Report of three cases. Med Oral Patol Oral Cir Buca12007; 12:EI Pacino GA, Serrat A, Redondo LM, Verrier A. Langerhans cell histiocytosis: clinical diagnostic features and current concepts. Med Oral 1999; 4: Rovelo MO, Kim R, Mascarenhas L, Hammoudeh J, Urata MM. Recurrent and refractory giant cell tumor ofthe jaw. J Craniofac Surg 2009; 20(6): Murray M, Dean J, Slater L. Multifocal oral Langerhans cell histiocytosis. J Oral Maxillofac Surg 2011; 69: