內文：
Background
1. 1953 -Lichtenstein and Jaffe introduced the term Histiocytosis X : a group of uncommon granulomatous proliferative disorders characterized by the presence of histiocytic cells.
2. The three clinical manifestations of Histiocytosis X : Eosinophilic granuloma (EG), Hand- Schuller-Christian and Letterer-Siwe diseases
3. 1985 -The disease changed name to Langerhans Cell Histiocytosis (LCH) 4. LCH is an uncommon disease
5. Only 1 % of the head and neck region cases of EG involves the maxillary bone Case Presentation
1. Patient: 16y/o male ,without any relevant aspects in his medical history 2. Family history:
• Father: Non-Hodgkin lymphoma (being treated)
• Mother: breast cancerin (being treated)
3. The patient was derived from the Hematology Unit for evaluation and definitive treatment of an osteolytic lesion in the left maxillary region
4. A panoramic radiograph in a routine dental exam showed a radiolucent image.
The patient underwent a biopsy of an asymptomatic osteolytic lesion located at apical level of the first upper left molar.
Panoramic radiograph obtained before biopsy, showing an osteolytic lesion in relation to periapex of the first upper left molar and projecting toward the floor of the maxillary sinus (white arrows)
原⽂文題⺫⽬目(出處)：
Spontaneous remission of eosinophilic granuloma of the maxilla after incisional biopsy: a case report
(Vargas et al. Head & Face Medicine (2016) 12:21 DOI 10.1186/
s13005-016-0118-9 )
原⽂文作者姓名： Alex Vargas, Hernán Ramírez, Pablo Ramírez, Camila Foncea, Bernardo Venegas and Paula Astorga
通訊作者學校： Oncology and Maxillofacial Surgery Department, Medicine Faculty, Pontificia Universidad Católica de Chile, Santiago, Chile
報告者姓名(組別)： 劉湘庭 (A組) 報告⽇日期： 105-08-08
5. The clinical examination: the patient only had a slight and generalized gingival inflammation, and no other findings.
6. Biopsy : the sample was consistent with bone monostotic Eosinophilic granuloma (Langerhans histiocytosis).
7. CBCT showed:
• Inflammatory changes in relation to the left maxillary sinus with great opacification
• Osteolysis over the roots of the upper left first molar, causing soil discontinuity of the ipsilateral sinus
• Extensive loss of continuity of the oral bone plate fibrous connective tissue with
lymphoplasmacytic inflammatory infiltrate and strong presence of eosinophils
Histiocyte-like cells with clear cytoplasm and pale rounded or some indented nuclei can be observed. Prominent eosinophilic granules and also some neutrophils are present
8. Bone scintigraphy : increase in osteoblast activity in the left maxillary bone
9. CT images: after the biopsy and before complete surgical removal of the lesion
10.CBCT images of three months after first examination:
Showing recovery of the left maxillary sinus transparency although the mucosal thickening of the floor persists
11.Later studies showed recovery of the left maxillary sinus transparency in spite of the persistence of mucosal thickening of the sinus floor.
• Newly formed bone in the periphery and between the roots
• Decrease of the hypodense areas found in the previous examination
12.Nine months after the biopsy:
It is possible to see the repaired bone in the area initially affected
13. Since the EG is a benign lesion, which developed a spontaneous remission in this case, our patient’s prognosis is excellent, as long he follows his regular checks in order to detect any early recurrence or new outbreaks for at least 5 years.
Discussion
1. Langerhans cell histiocytosis (LCH):
(a)a group of disorders characterized for a clonal proliferation of Langerhans cells (b)a wide spectrum of clinical presentations:
(1)solitary bone lesions with excellent prognosis, known as eosinophilic granuloma (2)with a multi-organic, disseminated and progressive course, previously known as
Hand-Schüller-Christian syndrome and Letterer-Siwe disease 2. The current classification:
(a)single system disease with one organ or system involved
(b)multi-system disease with two or more systems involved that may include ‘risk organs’ (hematopoietic system, spleen and/or liver)
3. A presumptive diagnosis is made when:
(a)typical morphological features of Langerhans cells are found
(b)stains for S-100 protein, CD1a and/or CD207 (Langerin) antigens are positive 4. Bone scan and PET are indicated to evaluate multiple involvements and to discard the
possibility of polyostotic disease
(PET scan shows how organs and tissues are working, the structure of and blood flow to and from organs.) 5. Cause and pathogenesis: still unknown
It has been proposed that a basic immune defect may lead to proliferation of Langerhans cells; or that the Langerhans cell itself may carry a genetic defect leading to abnormal cellular proliferation.
6. A key feature of this neoplasm is its clonal derivation from a single cell, which does not necessarily mean malignancy
7. Proinflammatory cytokines and chemokines and oncogenic B- type Raf kinase (BRAF) gene mutations are involved in the pathogenesis of EG
8. BRAF mutations are present in several malignant neoplasias Its finding in EG suggests a neoplastic condition of the lesion ->reevaluated considering the existence of spontaneous resolution 9. Differentiation disorders of Langerhans cells have also been studied:
Interleukin 1 and mutations of BRAF genes
->being considered as potential therapeutic targets
10.Associated with other malignancies such as leukemias, lymphomas and other solid tumors Acute lymphoblastic leukemia and lymphoma occur more often prior to the diagnosis of LCH, but they may be diagnosed up to 5 years after LCH
11.In our case: family history of malignancies
(no clear family association for the development of these disorders)
12.Clinical course and prognosis:
(a)age of initial manifestation
(b)the location and the organs involved
13.Multisystem disease (MS-LCH) has a worse prognosis (more complex treatment) 14.skeleton (80 % of cases) and the skin are more frequently involved
15.EG of bone: one per 350,000 to 2 million per year most common in teenagers and adults
patients under 20 years old (75%) and under 40(90%) average age :19
male > female (about twice)
16.In head and neck bone region:(Hicks J. et al., 2005) skull (27 – 43 %)
mandible (7 – 9 %) maxillary bone (1 %) cervical vertebra (2 %) 17.Unifocal: 50 – 75 % 18.Our case : unifocal
alveolar ridge of the maxillary bone -> low incidence especially for its spontaneous regression
19.spectrum of EG in maxillary and mandibular bones is quite varied
20.May mimic a variety of conditions, such as apical cysts, odontogenic tumors (e.g.,
ameloblastoma), non-odontogenic tumors (e.g., central giant cell granuloma), inflammatory diseases (e.g., osteomyelitis), vascular malformations, and malignancies
21.the most common s/s in the oral cavity :
intraoral mass, pain, gingival inflammation, bleeding, mobile teeth, oral mucosal ulcer, impaired healing, and halitosis
-> usually misdiagnosed as marginal periodontal infections 22.Lesion may occasionally remain asymptomatic
-> underdiagnosis
(our case: only referred localized swelling associated with gingival inflammation, with neither pain nor tooth mobility, and the lesion was detected with a routine panoramic radiography.)
23.Single system disease has good prognosis with a high rate of spontaneous remission and negligible mortality
24.unifocal bone disease:
• lesion is small (<2 cm) -> complete excision of bone lesions (curettage)
• lesions with a diameter of 2 to 5 cm -> a biopsy and curettage
25.Our case: apparently simple curettage during the diagnostic biopsy turned out in remission at the control with CT scan -> keep an expectant attitude
26.Other therapeutic combinations:
(a)intra lesional injection of steroids (b)surgery
(c)chemotherapy (d)radiation
(e)monoclonal CD-1a-antibody-therapy and gene transfer
27.Excision (with or without radiation therapy) : > 95 % disease-free survival recurrence rate:
Surgery alone:12 %
Radiation therapy alone: 25 % both combined therapies: 19 %
28.In the present case: more than 1 year of postoperative controls
(bone scintigraphy discards the possibility of polyostotic disease -> maintain an expectant approach of the regression)
29.F/u:
(a)at least 5 years after the end of therapy
(b)5 years after the last disease reactivation, in those who did not receive systemic therapy (c)until final growth and pubertal development have occurred
Our case: since the pubertal growth of the patient is probably finished, a minimum of 5 years follow-up is necessary
Conclusion
1. To diagnosis of LCH: a thorough examination and discard their participation in other systems 2. Our case: conservative approaches in the management of EG solitary maxillary bone
lesions and an expectant attitude toward the possibility of spontaneous regression only performing biopsy, especially in small lesions.
