Jan Lindhe. Clinical Periodontology

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DIFFERENTIAL DIAGNOSES: PERIODONTAL TUMORS AND CYSTS • 303

Fig. 13-8. (a) Hemangioma of alveolar mucosa at right maxillary canine. The lobulated blue lesion is a cavernous

type of hemangioma as shown in (b).

mandibular incisors are the most commonly affected

and the lesions are more frequent in women than in

men. They occur most often between the fourth and

the fifth decade. There are usually no symptoms.

Radiographical findings depend on the develop-

mental phase of the lesion, varying from well-circum-

scribed periapical radiolucencies (Fig. 13-7a), an inter

-mediate phase with irregular radiopacities within the

radiolucent zones, to a late phase with well-circum-

scribed periapical radiopacities bordered by distinct

radiolucent zones. The significance of the lesion is the

differential diagnostic problem in distinguishing it

from periapical granuloma and radicular cyst.

Histopathology

The lesion develops from an osteolytic phase in which

periapical bone is replaced by cellular fibroblastic

tissue through a cementoblastic phase in which a

cementum-like tissue is formed within the fibrous

tissue (Fig. 13-7b). In the late phase the lesion consists

of dense, irregular cementum-like material. The his-

tology resembles that seen in cemento-ossifying fi-

broma and fibrous dysplasia.

Therapy

Periapical cemental dysplasia is innocuous and needs

no surgical treatment as it is usually self-limiting.

Radiographic follow-up examination every fifth year

may be considered.

BENIGN NEOPLASMS OF

PERIODONTAL SOFT TISSUES

Most of the soft tissue tumors that occur in various

sites of the body can be seen in the oral mucosa as well.

Examples are neoplasms derived from peripheral

nerves (neurilemoma ("schwannoma") and neurofi-

broma), blood vessels (hemangioma) and smooth

muscle (leiomyoma) (Pindborg 1992, Neville et al.

1995). None of them, however, have a special propen-

sity to occur on the gingiva and usually they are

clinically indistinguishable from focal fibrous hyper-

plasias or other non-neoplastic lesions. Exceptions are

hemangiomas and nevi, which are described below.

Furthermore, squamous papilloma and verruca vul-

garis are dealt with in this section.

Hemangioma

Clinical features

Hemangiomas show a predilection for the head and

neck region and are rather frequent tumors of the oral

mucosa. There is a predilection for women (Neville et

al. 1995). Several authors doubt whether hemangi-

omas are true neoplasms and suggest they be classi-

fied as hamartomas or developmental anomalies.

Most cases are present at birth or occur shortly there-

after, although some cases develop in adults. Some of

them may undergo regression. Hemangiomas present

as flat or raised, sometimes lobulated, soft lesions of

blue to red color (Fig. 13-8a). They are usually asymp-

tomatic but may bleed when traumatized. Typically,

they blanch on pressure and the color returns shortly

after releasing the pressure. This is not the case with

other bluish lesions such as mucous cysts.

Histopathology

Hemangiomas are divided into capillary and cavern-

ous types (Fig. 13-8b) depending on the size of the

vessels. Mixed types are not uncommon. Especially in

the capillary type, sheets of proliferating endothelial

cells are common. Some hemangiomas resemble pyo-

genic granulomas but ulceration and an inflammatory

component are not typical of hemangiomas.

Treatment

Hemangiomas may give rise to severe bleeding if

treated with conventional surgery and cryosurgery

may be the treatment of choice where treatment is

necessary and possible. Large tumors which are not

suitable for surgery may be embolized or injected with

agents to induce fibrosis. Episodes of bleeding at eat-

ing and toothbrushing are common indications of

treatment.

Fig. 13-10. (a) Gingival papilloma of right maxillary first premolar region. (b) Photomicrograph of lesion in (a).

304 • CHAPTER 13

Fig. 13-9. (a) Nevus of lower right mandibular retromolar area. Histopathologic examination showed that the ne

vus cells were solely located in the subepithelial connective tissue (b), which is why the lesion is an

intramucosal nevus. Note melanin formation by the nevus cells.

Nevus

Clinical features

The term "nevus" is used for a variety of lesions of the

skin and oral mucosa. Here it will be used synony-

mously with a pigmented lesion containing nevus

cells (brown or nevocellular nevus) or a lesion contain-

ing melanocytes in the connective tissue (blue nevus).

Nevus cells are derived from the neural crest and are

related to melanocytes. Nevus cells are capable of

producing melanin pigment, although they do not

invariably do so.

Nevi are very common on the skin but rare in the

oral mucosa. They usually develop during childhood

and they are more common in women than in men.

Most intraoral nevi are seen on the palate but the

gingiva is not an uncommon location (Buchner &

Hansen 198Th). They can present as flat, slightly raised

lesions or even as a tumor (Fig. 13-9a). They are usu-

ally brown or black or they may show little or no

pigmentation. The blue nevus should be differenti-

ated from an amalgam tatoo.

Histopathology

Nevocellular or brown nevus contains nests of nevus

cells located along the basal cell layer of the epithelium

(junctional activity, junctional nevus), solely in the

connective tissue (intramucosal nevus) (Fig. 13-9b), or

in both locations (compound nevus). The nevus cells

may or may not contain melanin pigment. In blue

nevus, dendritic, melanin-producing melanocytes oc-

cur in the connective tissue (Neville et al. 1995).

Treatment

Some malignant melanomas of the skin seem to de-

velop from nevi. Especially in adults, nevi with junc-

tional activity should be viewed with caution. In the

oral mucosa little is known about malignant transfor-

mation of nevi. Excision of oral nevi is generally rec-

ommended (Buchner et al. 1990).

Papilloma

Clinical features

The term papilloma covers four or five different types

of benign epithelial proliferations: squamous and fili-

form papilloma, verruca vulgaris, condyloma and fo-

cal epithelial hyperplasia (FEH). Malignant transfor-

mation is very rare or non-existing and usually there

are no symptoms. Human papilloma virus (HPV) is

commonly found in these lesions and a causative role

of HPV in the development of at least some of these

lesions is strongly suggested (Pindborg & Prtorius

1987, Garlick & Taichman 1991). Generally, a relation-

ship between the histomorphology and distinct HPV

DIFFERENTIAL DIAGNOSES: PERIODONTAL TUMORS AND CYSTS • 305

Fig. 13-11. (a) Gingival verruca vulgaris of left maxillary first premolar region. Clinically, it resembles the lesion

shown in Fig. 13-10 but histopathologic examination of the surgically removed lesion showed characteristics typi

cal of verruca (b).

types, of which about 70 are known, can be establ-

ished. Verruca vulgaris is dealt with separately and

condyloma and FEH will not be mentioned further

here.

Squamous papilloma and filiform papilloma can be

distinguished by detailed clinical and histologic fea-

tures (Prxtorius, personal communication). Here,

however, they will be dealt with together. They are

exophytic, pedunculated or sessile lesions with a red-

dish/normal or whitish/gray color and a granu-

lar/moruloid or filiform/digitated surface (Fig. 13-

10a). They are mainly seen in the third to fifth decade,

in contrast to verruca vulgaris, which is usually seen

in younger age groups. The soft palate is the most

common location. It is often difficult to differentiate

clinically between verruca vulgaris and squamous

and filiform papillomas.

Histopathology

Squamous and filiform papillomas are covered by

squamous epithelium with deep crypts in the surface.

The epithelium is keratinized or non-keratinized with

irregular rete ridges which cover sometimes ramified

connective tissue papillae (Fig. 13-10b).

Treatment

Surgical excision, including the base of the lesion, is

the treatment of choice. Recurrence is uncommon.

Verruca vulgaris

Clinical features

Verruca vulgaris is common on the skin. In the oral

mucosa verrucae are less common and are often seen

as a result of autoinoculation in children with warts

on the fingers. The majority are located on the lips and

in the palate and 10-20% are located on the gingiva (

Green et al. 1986, Pindborg & Prxtorius 1987). Ver-

ruca vulgaris seems to be associated with HPV type 2

and 4.

Clinically the lesions are sessile, exophytic or raised

with a whitish surface with angulated verrucous pro-

jections (Fig. 13-11a).

Histopathology

Histologically, they show a papillomatous surface

with hyperkeratinization and elongated rete ridges (

Fig. 13-11b). Characteristically, the rete ridges bend

inward at the margins of the lesion, pointing toward

a center below the lesion (Prtorius-Clausen 1972).

Treatment

Some verrucae disappear spontaneously, especially in

children. Treatment usually consists of surgical exci-

sion. Recurrence occurs in some cases.

Peripheral odontogenic tumors

Clinical features

Odontogenic tumors primarily occur as intraosseous

growths but sometimes they present in a peripheral

location in the gingiva (Buchner & Sciubba 1987, Pind-

borg 1992). The following entities may occur as a

gingival tumor: ameloblastoma, squamous odonto-

genic tumor, ameloblastic fibro-odontoma, adenoma-

toid odontogenic tumor, calcifying odontogenic cyst,

calcifying epithelial odontogenic tumor (Fig. 13-12a,b)

and odontogenic fibroma. Peripheral odontogenic tu-

mors usually present as non-ulcerated sessile or pen-

dunculated gingival lesions. The clinical features,

thus, are nonspecific, resembling focal fibrous hyper-

plasias or other non-neoplastic lesions (Buchner &

Sciubba 1987). Most examples are rather small.

Histopathology

Generally, these lesions show histopathologic features

similar to the intraosseous forms of the tumors (Fig.

13-12b).

Treatment

Due to the rather small number of reported cases and

the varying behavior of the different odontogenic tu-

mors mentioned above, general guidelines will not be

306 • CHAPTER 13

Fig. 13-12. (a) Gingival swelling of left maxillary lateral incisor region. The tumor was surgically removed and the

histopathologic examination revealed a calcifying epithelial odontogenic tumor (b).

Fig. 13-13. (a) Radiograph shows well-demarcated loss of alveolar bone between displaced mandibular incisors. Af

ter surgical removal and histopathologic examination (c) the lesion was diagnosed as an ameloblastoma. (b)

Radio-graph with irregular radiolucency with scalloped periphery in alveolar bone of mandibular premolar and

canine region. The teeth are displaced. Biopsy revealed an ameloblastoma.

given. Apparently an innocuous clinical behavior is

characteristic of most reported cases, showing a dif-

ferent biologic behavior than their intraosseous coun-

terparts (Buchner & Sciubba 1987). However, some

examples of recurrence have been reported.

It should be mentioned here that odontogenic tu-

mors or hamartomas are frequently detected his-

tologically in the soft tissue in operation specimens

from exposure of molars delayed in eruption for no

obvious clinical cause (Philipsen et al. 1992). The fate

of these lesions, had the surgical exposure not been

performed, is unknown.

BENIGN NEOPLASMS OF

PERIODONTAL HARD TISSUES

Ameloblastoma

Clinical features

The ameloblastoma is a benign but locally invasive

neoplasm derived from odontogenic epithelium (Kra-

mer et al. 1992). It occurs most commonly between 20

and 40 years of age and affects women and men

equally. The most common location is the molar re-

gions, with most tumors being localized in the man-

dible (van der Waal 1991, Reichart et al. 1995). The

tumors grow slowly and sometimes reach excessive

sizes before symptoms arise. Such symptoms may be

expansion of cortical bone and displacement of teeth.

The alveolar nerve usually remains undisturbed even

in cases of large tumors in the mandible, but roots of

DIFFERENTIAL DIAGNOSES: PERIODONTAL TUMORS AND CYSTS • 307

Fig. 13-14. (a) Squamous odontogenic tumor demonstrating substantial diffuse radiolucency of alveolar bone in left

maxillary incisor region. The diagnosis was revealed by biopsy showing features presented in (b).

Fig. 13-15. (a) Radiographic presentation of a benign cementoblastoma in a 20-year-old man. The periapical lesion

of the mandibular second molar shows an almost circular radiopacity surrounded by a radiolucent margin. Part of

the root is resorbed and replaced by lesional tissue. The tumor is composed of cementum-like tissue (b).

adjacent teeth are sometimes resorbed. Intraosseous

ameloblastomas rarely demonstrates mucosal in-

volvement in the form of gingival tumor. Radiog-

raphically, the ameloblastoma demonstrate unilocular

or multilocular radiolucency, which is usually well-

circumscribed. The radiographic features may be in-

distinguishable from jaw cysts but may also resemble

loss of bone due to marginal periodontitis (Fig. 13-13a,

b).

Histopathology

The general histopathologic features consist of islands

and/or strands of odontogenic epithelium in a mature

collageneous stroma (Fig. 13-13c). Columnar or cuboi-

dal epithelial cells resembling ameloblasts surround

loosely arranged epithelial cells resembling the stel-

late reticulum of the enamel organ.

Treatment

Conservative and radical surgery are equally common

types of treatment, and recurrences are common fol-

lowing both treatment modalities (Reichart et al.

1995). This is probably due to difficulties in removing

the proliferating cords of tumor tissue in the marrow

spaces at the periphery of the tumor. In general, more

radical treatment of maxillary ameloblastomas than of

mandibular ones is recommended (Reichart et al.

1995).

Squamous odontogenic tumor

Clinical features

This odontogenic tumor was first described in 1975 (

Pullon et al. 1975) and very few examples have been

reported. However, as the tumor seems to develop

from the periodontal ligament, presumably from the

epithelial rests of Malassez, and is most often associ-

ated with the lateral root surface of an erupted tooth,

it is important to include it here as an example of a

periodontal tissue reaction.

Squamous odontogenic tumor has been found in

almost all age groups and does not seem to have a

predilection for any site or gender. Radiographically,

the findings are not specific (Fig. 13-14a). They often

show a triangular radiolucent defect between the

308 • CHAPTER 13

Fig. 13-16. Gingival squamous cell carcinoma of left mandibular canine and premolar region. (a) Facial aspect. (b)

Mesial and lingual aspect. (c) Photomicrograph of the lesion showing carcinoma in right half.

roots of two teeth which may suggest vertical bone

loss (Neville et al. 1995).

Histopathology

The characteristic findings are islands of squamous

epithelium in a mature collageneous stroma (Fig. 13-

14b). There are no columnar peripheral cells or stellate

reticulum-like cells as in the ameloblastoma.

Treatment

Local excision or enucleation is the most common type

of treatment. There seems to be some tendency for

recurrence.

Benign cementoblastoma

Clinical features

Benign cementoblastoma is a slow-growing neoplasm

forming hard tissue around the apex of a tooth (Kra-

mer et al. 1992). It is most common in the mandibular

premolar and molar region, is almost equally common

among men and women, and is most often seen in the

second decade (Ulmansky et al. 1994). The tumor is

slowly growing and may cause swelling and pain.

Radiographically, a periapical radiopacity typically

surrounded by a radiolucent margin of uniform width

is seen (Fig. 13-15a) (Ulmansky et al. 1994). The tumor

may obscure the apex of the involved tooth and root

resorption with fusion of the tumor with the tooth is

characteristic. The involved tooth is usually vital.

Histopathology

The tumor is built up of cementum-like tissue with

numerous reversal lines, the tissue being attached to

the tooth. Between the cementum-like areas the tissue

is cellular, and in the periphery it is unmineralized (

Fig. 13-15b). A small biopsy of this tumor may be

difficult to differentiate from osteosarcoma.

Treatment

Surgical enucleation is the treatment of choice, al-

though this may be difficult due to the relationship

between the tumor and the related tooth.

MALIGNANT NEOPLASMS OF

PERIODONTAL SOFT TISSUES

Squamous cell carcinoma

Clinical features

In the literature it is rare to see a distinction main-

tained between gingival cancer and cancer developed

on the edentulous alveolar ridge. Thus, many reports

deal with cancer of the "gums". In most of these cases

men have dominated, and only older people are af-

DIFFERENTIAL DIAGNOSES: PERIODONTAL TUMORS AND CYSTS • 309

Fig. 13-17. Metastasis from adenocarcinoma of lung.

The neoplasm affects lingual gingiva of right mandibu

lar second molar and mucosa covering semi-

impacted third molar.

Fig. 13-18. (a) Gingival Kaposi's sarcoma of the maxilla in 33-year-old man with AIDS. (b) Histopathologic features

of Kaposi's sarcoma shown in (a).

fected (Pindborg 1980). In England a marked shift in

male/female ratio has been observed, since the ratio

was 5:1 in 1932-39, but 1:1 by 1960-69 (Easson & Pal-

mer 1976). Most gingival cancers affect the mandible

and 60% are located posterior to the premolars. In an

American study it has been shown that dentists play

an important part in the early recognition of gingival

cancer; 60% of these patients are first seen by a dentist

(Cady & Catlin 1969). Gingival cancer commonly be-

gins as a nodular lesion (Fig. 13-16a,b) often with

ulceration and surrounding leukoplakia. It infiltrates

rapidly in depth, frequently extending along the peri-

odontal membrane with osseous involvement and

destruction of the supporting bone. Pain and loosen-

ing of teeth is common. In the American study, gingi-

val cancer had exceeded 3 cm at admission in 44% of

the referred cases and regional lymph-node metasta-

sis on admission was found in 37%.

Histopathology

Squamous cell carcinoma of the gingiva does not dif-

fer histologically from squamous cell carcinomas else-

where in the oral mucosa. Islands and cords of malig-

nant epithelial cells are seen infiltrating underlying

tissues (Fig. 13-16c). Varying amounts of horn pearls

are formed and usually a strong inflammatory reac-

tion is found in the stroma.

Treatment

Gingival squamous cell carcinomas are usually

treated by surgery, irradiation or combinations of

these. When irradiation therapy is required, extraction

of involved teeth is often necessary before irradiation

is instituted if the teeth suffer from severe periodonti-

tis. This is due to an increased risk of osteoradione-

crosis after extraction of teeth situated in irradiated

bone as the result of permanently reduced vasculari-

zation (Beumer et al. 1984).

Metastasis to the gingiva

The majority of metastases to the oral region are in-

traosseous. The gingiva (including the alveolar mu-

cosa) is most often the seat of soft tissue metastasis in

the mouth. In the oral regions, soft tissue metastases

from lung cancer (Fig. 13-17) is encountered most

frequently in men, while metastases from breast can-

cer account for most soft tissue metastases in women.

About 20% of oral soft tissue metastases were mani-

fested before the primary tumor was diagnosed. Fur-

thermore, in 90% of the cases the clinical manifestation

resembled a hyperplastic or reactive lesion (Hirshberg

et al. 1993). These observations emphasize the need

for a histologic examination of all such tumors.

Histopathology

The histology resembles the tumor of origin. Most

cases are carcinomas and sarcomas rarely metastasize

to the oral region.

310 • CHAPTER 13

Fig. 13-19. (a) Malignant lymphoma of HIV-infected 36-year-old man. The gingival neoplasm in the right mandibu-

lar molar region resembles a pyogenic granuloma, but histopathologic examination revealed a malignant lym-

phoma shown in (b).

Kaposi's sarcoma

Clinical features

In 1872, Kaposi described what he called "multiple

idiopathic hemorrhagic sarcoma", which later came to

bear his name. Most of his patients were Jews, espe-

cially Ashkenazim. Later it was reported that Kaposi's

sarcoma occurred among people living around the

Mediterranean.

Then, in the middle of the 1950s, reports came from

Sub-Saharan Africa that Kaposi's sarcoma was a

prevalent malignant tumor in that area, but oral mani-

festations of Kaposi's sarcoma in Jews and Africans

were rare.

It was later recognized that Kaposi's sarcoma some-

times occurred in individuals who had been treated

with immunosuppressive drugs after kidney trans-

plantation.

The latest phase in the occurrence of Kaposi's sar-

coma is among individuals suffering from the ac-

quired immunodeficiency syndrome (AIDS). The ap-

pearance of a Kaposi's sarcoma is one of the criteria

for the development of AIDS in patients infected with

human immunodeficiency virus (HIV). In most in-

stances, Kaposi's sarcoma will first manifest as skin

lesions followed by oral lesions. In rare cases, oral

manifestations may be the first sign of Kaposi's sar-

coma. Gingival location is the second most frequently

observed after the palate (Pindborg & Reichart 1995).

The typical appearance is that of single or multiple

blue, violet or red slightly raised lesions. Ulceration is

common and with time the tumors can reach mon-

strous proportions (Fig. 13-18a).

Histopathology

The typical features are a lesion with bundles of spin-

dle-shaped cells and many thin-walled vascular lumi-

nae, often lined by plump endothelial cells (Fig. 13-

18b). There are usually a number of mitotic figures of

which some are atypical. There is general agreement

that the cell of origin of Kaposi's sarcoma is the endo-

thelial cell.

Treatment

There is no curative treatment but palliative treatment

includes both cytostatics and irradiation.

Malignant lymphoma

Clinical features

A primary malignant lymphoma is rare in the oral

cavity. When it occurs, it is most commonly seen in the

palate and gingiva (Takahashi et al. 1989). It presents

as a diffuse swelling which is usually ulcerated. The

diagnosis may be quite difficult to arrive at as the first

manifestations may resemble a non-specific periodon-

titis, pyogenic granuloma (Fig. 13-19a) or pericoroni-

tis (Maxymiw et al. 1991).

In HIV-infected patients non-Hodgkin's lymph-

omas occur with increased frequency (Holmstrup &

Westergaard 1994). Occasionally, a gingival tumor

may be the first manifestation of a non-Hodgkin's

lymphoma in an HIV-infected patient (Fig. 13-19a).

Histopathology

Histomorphologic features, immunologic and genetic

markers are used to diagnose and classify malignant

lymphomas. The lesions contain lymphocytic-appear-

ing cells (Fig. 13-19b); in low-grade tumors the cells

are well-differentiated small lymphocytes, whereas

high-grade tumors contain less differentiated cells.

Common to all lymphomas are infiltrative growth as

characteristically seen in all malignant tumors.

Treatment

Depending on the extension and spread of the tumor,

surgical removal, irradiation, cytostatics and combi-

nations of these may be the treatment of choice.

DIFFERENTIAL DIAGNOSES: PERIODONTAL TUMORS AND CYSTS • 311

Fig. 13-20. (a) A swelling between

the right mandibular canine and

lateral incisor was associated with

tooth mobility, displacement, and

loss of supporting periodontal

bone as seen in the radiograph (b).

The biopsy showed osteosarcoma

(c).

MALIGNANT NEOPLASMS OF

PERIODONTAL HARD TISSUES

Osteosarcoma

Clinical features

Osteosarcoma is the most common primary malig-

nant tumor of bone. About 7% of all osteosarcomas

occur in the jaws.

The first symptoms of a jaw osteosarcoma appear

when the patients are, on average, about 30 years old,

which is a decade older than for osteosarcomas in

other bones. The maxilla and mandible are affected

with about equal frequency. Men are affected more

commonly than women. Symptoms include mobile

teeth, swelling (Fig. 13-20a), anesthesia or paresthesia,

toothache and nasal obstruction (van der Waal 1991).

Radiographically, the neoplasm demonstrates a vari-

ety of patterns ranging from radiolucent (Fig. 13-20b)

to radiopaque changes. Sometimes the formation of

bony trabeculae results in a sunray appearance in a

direction perpendicular to the outer surface. A num-

ber of jaw osteosarcomas will have, as their first mani-

festation, a widening of the periodontal membrane (

Garrington et al. 1967).

Histopathology

A variety of histologic appearances can be seen (

Neville et al. 1995). Some osteosarcomas are primar-

ily osteoblastic, with production of irregular osteoid

and bone (Fig. 13-20c). In others, production of carti-

lage (chondroblastic osteosarcomas) or the presence

of fibroblastic cells (fibroblastic osteosarcomas) are the

dominant histologic features. Common to all these

types are the malignant-appearing cells with pleo-

morphic nuclei and mitotic figures. The prognosis is

not dependent on the histologic subtype.

Treatment

Resection of involved and surrounding bone is the

common treatment. Sometimes supplementary che-

motherapy and/or radiotherapy are used.

Langerhans cell disease

Clinical features

Langerhans cell disease, formerly named histiocytosis

X, is a proliferation of histiocyte-like cells that have

312 • CHAPTER 13

Fig. 13-21. (a) Eosinophilic granuloma of alveolar bone in the mandibular premolar region showed progressive loss

of supporting periodontal tissue in a 33-year-old woman. The lesion was biopsied and characteristic features

of Langerhans cell disease were revealed (b). Note the numerous eosinophils.

been shown in recent years to be Langerhans cells.

Langerhans cells are blood marrow-derived cells nor-

mally present in the skin, mucosa, lymph nodes and

blood marrow. The disease is difficult to classify as the

etiology and pathogenesis are unknown and due to

the fact that it is characterized by clinical heterogene-

ity and an unpredictable course. Some classify this

disease, or at least some of its manifestations, as a

malignant systemic disorder/neoplastic disease while

others classify it as a reactive, proliferative disease.

Traditionally, the disease has been divided into three

subtypes: a disease with solitary or multiple bone

lesions (eosinophilic granuloma), a chronic multi-

organ disease associated with considerable morbidity

(Hand-Schuller-Christian syndrome) and an acute

multi-organ disease occurring mainly in infants and

with a common fatal outcome (Letterer-Siwe disease).

There are overlapping clinical features between

these subtypes.

Manifestations in the jaws are seen in 10-20% of all

cases. Swelling, tenderness, pain and loosening of

teeth (Fig. 13-21a) are frequent symptoms. The jaw

lesions often appear as punched-out radiolucencies

but ill-defined radiolucencies are also seen, sometimes

mimicking periodontal disease (Neville et al. 1995,

van der Waal 1991).

Histopathology

The lesions are characterized by a diffuse infiltration

of rather large mononuclear cells representing Lang-

erhans cells with varying numbers of eosinophilic

granulocytes and other inflammatory cells (Fig. 13-

21b). Identification of Langerhans cells by electron

microscopy or immunohistochemical methods is

sometimes used to confirm the diagnosis.

Treatment

The prognosis for patients with jaw lesions in the

absence of visceral involvement is generally good.

Such lesions are usually treated surgically, sometimes

with supplementary radiotherapy. Recurrence and

progression of the disease can be seen. Chronic and

acute disease with multiorgan involvement is usually

treated with chemotherapeutic agents and the prog-

nosis is generally moderate or poor.

CYSTS OF THE PERIODONTIUM

The origin of jaw cysts is either developmental or

inflammatory and the background of the frequent

occurrence of these cysts is the abundance of epithelial

remnants residing within the jaws (Shear 1992, 1994).

Fig. 13-22. (a) Gingival cyst between right mandibular first premolar and canine. (b) Characteristic histopathologic

features of a cyst lined by a thin non-keratinized squamous epithelium.