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Langerhans Cell Histiocytosis Mimicking Periapical Pathology in A 39-Year-Old Man
Langerhans Cell Histiocytosis Mimicking Periapical Pathology in A 39-Year-Old Man
Langerhans Cell Histiocytosis Mimicking Periapical Pathology in A 39-Year-Old Man
Abstract
Langerhans cell histiocytosis (LCH) is a clonal neoplastic
proliferation of Langerhans-type dendritic cells, with
more than 50% of cases of LCH seen in children younger
L angerhans cells are
dendritic cells of the
skin and mucosa from
Significance
This is a rare presentation of Langerhans cell histio-
cytosis with regard to both its location (posterior
than 15 years of age. The most common clinical presen- which 2 main subgroups
maxilla) and age of onset. Awareness of such pre-
tation of LCH is solitary or multiple bony lesions. The of tumors can arise. The
sentation and consideration of Langerhans cell his-
jaws are affected in approximately 10%–20% of cases, first, Langerhans cell his-
tiocytosis in the differential diagnosis are critical in
with a strong predilection for the mandible. The maxilla tiocytosis (LCH), refers to
patient management.
is involved in only 1% of head and neck cases. When the a clonal neoplastic prolif-
jaws are involved, lesions of LCH may mimic periapical eration of Langerhans-
pathology as seen in patients requiring endodontic ther- type cells, whereas the second, Langerhans cell sarcoma, is a high-grade neoplasm
apy or bone loss as seen in periodontal disease. We with overtly malignant cytologic features (1).
report the case of a 39-year-old man with LCH involving LCH was classically referred to as histiocytosis X; this condition was further sub-
the posterior maxilla. This is a rare presentation of LCH divided into 3 categories depending on the clinical presentation (2). Eosinophilic gran-
with respect to both location and patient age. Clinicians uloma was the term used for a solitary or multiple bony lesions without visceral
should consider LCH when developing a differential involvement. If multiple lesions involving the bone, skin, and viscera were present,
diagnosis of an apical radiolucency of vital teeth or teeth the condition was referred to as Hand-Sch€uller-Christian disease. Prominent cutaneous,
that fail to respond to endodontic therapy and be aware bone marrow, and visceral involvement occurring mainly in infants was termed
of its clinical and radiographic mimics. (J Endod 2017;- Letterer-Siwe disease. These classical designations were often unclear because of over-
:1–6) lapping clinical features, and the generic term of Langerhans cell histiocytosis was later
introduced (3, 4).
Key Words In the current classification system, LCH is categorized on the basis of degree of
Langerhans cell histiocytosis, periapical pathology, organ involvement. According to this system, lesions are first designated as having single
posterior maxilla organ involvement or multiorgan involvement. Those affecting only a single organ, typi-
cally the bone or skin, are further classified as unifocal or multifocal. On the other hand,
multiorgan involvement is further categorized by the presence or absence of organ
From the *Division of Oral and Maxillofacial Pathology, dysfunction. If organ dysfunction is present, the condition is considered to be high
Columbia University College of Dental Medicine, New York; or low risk on the basis of which organs are involved (high risk includes lung, liver,
and †Periodontist, Private practice, Manhattan, New York
Address requests for reprints to Dr Elizabeth M. Philipone,
spleen, and/or bone marrow, and low risk includes skin, bone, lymph nodes, and/
Columbia University Medical Center, 630 West 168th Street, or pituitary gland) (5–8).
PH15W-1562, New York, NY 10032. E-mail address: LCH is a rare disease with an incidence of 5 cases per 1 million per year (9). More
ep2464@columbia.edu than 50% of cases are seen in children younger than 15 years of age. LCH has a definite
0099-2399/$ - see front matter male predilection, with a male to female ratio of 3.7:1 (10). The clinical presentation of
Copyright ª 2017 American Association of Endodontists.
http://dx.doi.org/10.1016/j.joen.2017.05.020 LCH varies, but in more than half of cases (55%) the disease is limited to one organ
(10). The bone is affected most frequently, followed by skin, lymph nodes, liver, spleen,
oral mucosa, lung, and central nervous system (6). Most commonly, bony lesions occur
in the skull, ribs, vertebrae, and mandible (11). In addition, there appears to be a cor-
relation between age of onset of LCH and the bones that are affected by the disease. Chil-
dren younger than 10 tend to have skull and femoral lesions (9), whereas those older
than 20 are more likely to have rib, shoulder girdle, and mandibular lesions (12). Other
clinical presentations depend on the organs involved by the disease process and can
include lymphadenopathy, diabetes insipidus, hepatosplenomegaly, and cytopenia
(13).
Bone lesions, either solitary or multiple, are the most common clinical presenta-
tion of LCH (14). Radiographically, the lesions appear as punched out radiolucencies
without cortication. The jaws are affected in 10%–20% of all cases (15). The most
frequently affected intraoral site is the posterior mandible, and here lesions may appear
scooped out or scalloped as a result of the destruction of superficial alveolar bone (16).
With advanced bone destruction, clinical symptoms may mimic those of severe chronic
Case Presentation
A 39-year-old man presented to his periodontist with a chief
complaint of pain and swelling associated with tooth #3 (maxillary right
first molar). The patient reported that he had previously experienced
similar symptoms at that site 2 years ago. At that time he was seen by
an endodontist who prescribed antibiotics that were ineffective at alle-
viating the pain or swelling. A periapical radiograph was taken that
showed a possible area of decreased bone density around the distal
root of tooth #3, which may have been interpreted as periapical pathol-
ogy (Fig. 1). The tooth tested vital with both Endo Ice (Coltene/Whale-
dent Inc, Cuyahoga Falls, OH) and electric pulp testing; however, it was
subsequently treated endodontically via RCT.
The patient’s medical history is significant for an isolated lesion of
LCH diagnosed within the skull approximately 1 year ago. With regard to
the patient’s history of LCH, he first presented to his neurologist with
complaints of headaches refractory to nonsteroidal anti-inflammatory
Figure 1. Pretreatment radiograph of tooth #3. Possible area of decreased drugs. A magnetic resonance imaging scan was performed that showed
bone density, which may have been interpreted as periapical pathology, is pre- a radiolucent lesion within the skull (Fig. 2). On the basis of the patient’s
sent around the distal root. Tooth tested vital with both electric pulp testing and symptoms and radiographic presentation, a presumptive clinical diag-
Endo Ice; however, RCT was performed. Triangular-shaped radiolucency ex-
tending from center of crown to coronal portion of root is a radiographic
nosis of a meningioma was made. The lesion was then excised and sent
artifact. for pathologic analysis, at which time a final diagnosis of LCH was
rendered. No other lesions of LCH were observed on imaging at this
time.
periodontitis, and the teeth are described as ‘‘floating in air’’ after the On presentation to his periodontist, the patient was not in acute
loss of alveolar bone (16, 17). Patients may complain of dull pain or distress. Clinical examination revealed a gingival swelling in the area
tenderness as a result of intraosseous oral lesions. Patients may also of tooth #3. A radiograph was taken that showed a radiolucency at
develop proliferative or ulcerative mucosal or gingival lesions if the the apex of the RCT-treated tooth (Fig. 3). Different treatment
Figure 2. Sagittal (A) and coronal (B) magnetic resonance imaging scans showing lytic lesion at right skull base. Lesion is indicated with an asterisk (*).
Figure 4. (A) Low-power image showing pieces of edematous fibrous connective tissue infiltrated by acute and chronic inflammatory cells (hematoxylin-eosin;
original magnification, 20). (B) On higher magnification, abundant eosinophils and atypical histiocytes with indented (kidney bean shaped) nuclei can be appre-
ciated (hematoxylin-eosin; original magnification, 400). (C) Also identified within the specimen are small and thin-walled blood vessels and pieces of non-vital
bone exhibiting loss of osteocytes from lacunae (sequestrum formation) and peripheral resorption. Abundant eosinophils are present ( hematoxylin-eosin; original
magnification, 200). (D) LCH, diffusely positive for CD1a (hematoxylin-eosin; original magnification, 20).