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An aneurysmal bone cyst of the mandible
Oral SURGERY Ora MEDtCINE
25
NUMBER
1
JANUARY,
PATHOLOGY
AND&al
VOLUME
1968
Operative oral surgery
An aneurysmal bone cyst of the mandible Report
of a case
W. Hoppe, Prof. Dr. Ned., Dr. Med. Dent., Kiel, West Germany’ DEPARTMENT DENTAL
OF ORAL
POLICLINIC
AND
SURGERY
(CHIEF:
PROF.
DR.DR.
w.
CLINIC
(CHIEF:
PROF.
DR.DR.
SPRETER
HAHN)
OF
THE
v. KREUDES
STEIN),THEUNIVERSITYOFKIEL
A
ncurysmal bone cysts are most common in the long bones and vertebral column. This tumor, which occurs chiefly in children and young adults, is distinguished by rapid growth and, when localized in the maxillofacial area, by its characteristic eccentric, ballooned-out appearance; it causes a noticeable facial asymmetry within a very short time. Gross examination of the tumor tissue shows numerous cystic spaces of varying size, separated by septa and filled with reddish brown liquid. Histologic examination reveals multiform spaces with an endothelial lining filled partly with fresh and partly with older blood. The separating septa contain spindle cells and a varying number of giant cells. The occurrence of a cystic bone tumor in the jaws is extremely rare. Bernier *Address:
23 Kiel-Wik,
Weimarer
Str.
8, W.
Bwmany.
2
Hoppe
O.S., 0-M. January,
& OZ. 1968
and Bhaskar’ mention two cases in the mandible, and both Wang5 and Lucas3 speak of the lesion’s occurrence in the maxilla. The following case is now added to those already on record. CASE REPORT (NO. 591; 813/641 A painless expansile lesion developed in the left lower jaw of a 12-year-old girl within a period of 3 weeks and deformed the face. When the patient was admitted to the clinic, there was a marked swelling of the left mandible in the space between the second premolar and the second molar. Intraorally, an identical extension of the tumor was visible in the buccal vestibule. The consistency was firm and elastic; it was partly compressible without causing pain, but it did cause crepitus. The overlying mucosa did not show any pathologic alteration. The teeth responded positively to the electric test. In the posteroanterior roentgenogram a polycystic, distinctly gross, soap-bubble-like eccentric bulging of the left mandi-
Fig.
2. Eccentric
expansile
Fig.
1. Roentgenogram
swelling
of gross
of left
specimen;
mandible,
polycystie
covered
bone
with
lesion.
thin
layer
of bone.
Volume Number
Anewysmal
25 1
bone cyst of mandible
3
ble was visible. It involved the area between the mandibular second premolar and the second molar. The oblique lateral roentgenogram of the left side of the mandible showed a polycystic, radiolucent area with a coarse, honeycombed, irregular pattern. The root development of the mandibular second premolar and second molar was still unfinished. The mandibular third molar was commensurate with the patient’s age. The left mandibular first molar extended into the radiolueent area; its alveolar lamina dura was not interrupted. Regional lymph nodes were not palpable. Because the tumor was suspected of being an ameloblastoma or a fibromyxoma, a biopsy specimen was taken. The histologic diagnosis was fibrocementoma. Because of the rapid growth and the fact that odontogenic fibromas are known to have become malignant, surgical removal of the tumor by means of partial resection of the mandible under general endotracheal anesthesia was indicated. The hemispherical tumor could be enucleated intraorally. Its surface, from which the periosteum could easily be removed, consisted of a thin layer of bone of variable thickness and a reddish brown color. Accidental incision effected excessive bleeding. Convalescence was uneventful. Gross
findings
The
cut surface of the removed result of the most prominent feature When incised, a brownish red liquid revealed cystlike, multiform, cavernous
Fig.
lin
Fig. and
3. Surface
of cut
section
4. Highly cellular, fibrous, eosin stain. Magnification,
tumor was of a brownish reddish gray color as 2, of grayish red tissue with coarse, irregular elevations. exuded. A cross-section of the formalin-fixed specimen spaces with a distinct formation of septa.
through
tumor
septum-forming x56. Histologic
showing
stroma. section
multiform
cavernous
with calcified 255/64.)
bodies.
spaces.
(Hematoxy-’
4
O.R., O.M. January,
rroppc
Histologic
& O.P. 196X
ftndings
A tooth-bearing section of the tumor consisted of a well-vascularized, fibrous, highly cellular stroma with a reticular fibrillary network. Dispersed throughout the stromal tissue were calcified bodies of varying size-partly amorphous and partly concentrically stratified. Some places revealed a network of new-formed bone. In the area of the macroscopically visible polycystie swelling of the lower jaw there were irregularly shaped cavernous spaces, either filled with blood, filled with coagulated blood of varying age or empty. The spaces mere lined with a layer of true endothelium, interrupted by multinucleated giant cells. The septa consisted of fibrillar connective tissue, rich in spindle cells; occasionally, multinucleated giant cells were present. In thicker sections fresh or older hemorrhagic foci were noticeable. The grossly spherical thin layer of bone vvhich covered the tumor consisted of a new-formed trabecular network of varying density. Osteoclastic lacunar bone resorption and loose fibrillar tissue with ectatic capillaries predominated the thin zone between the endotheliumlined cavities and the new-formed bone, lined with osteoblasts. All these characteristic signs led to the final diagnosis of an aneurysmal bone cyst.
Fig. 5. Solitary, multinucleated cation, x224. Histologic section
Fig. x224.
6. Focus Histologic
giant 255/64.)
of hemorrhage with section 255/64.)
cells
giant
in septa.
cells.
(Hematoxylin
(Hematoxylin
and toxin
and
eosin
stain.
stain.
Magnifi-
Magnification,
Volume Number
25 1
Aneurysm&!
bone qjst
of mnndiblfz
5
DISCUSStON
The x-ray finding of a unilateral, eccentric, ballooned-out swelling, covered with a grossly hemispherical, thin bone shell is, according to Uehlinger,” pathognomonic for the aneurysmal bone cyst. The macroscopic and histologic findings bear a close resemblance to those described by Bernier and Bhaskar,’ Wang,” and Lucas.3 Only in the smaller amount of multinucleated giant cells is the present case different from the eases reported heretofore. The presence of giant cells is considered to be the expression of reparative activity. Their development and number correlate in most giant-cell-containing tumors with the amount of the hemorrhages in the tissue. The low number of giant cells in the present tumor may be due to the short time of development, which was only 3 weeks. This is in contrast with the cases published by Bernier and Bhaskar’ and Wang,5 in which the tumors developed over a period of several months. The histogenesis of the aneurysmal bone cyst is unknown, although it. is often considered to be the result of a trauma. LichtensteirP suggests that this cyst develops after a thrombosis or another local circulatory disturbance, I’Csulting in dilatation and congestion of the vascular bed. The suggestion by Bernier and Bhaskarl that the aneurysmal bone cyst is a specific entity of the giant cell reparative granuloma is based on t,he supposition that both tumorlikc lesions start with a hemorrhage. The histologic findings of the tumor discussed here, operat,ed on in an early stage, all refer to a mesenchymal matrix, on the one hand tending t.o a fibromatous differentiation and the formation of osteocementum-like structures and trabecular bone, and, on the other hand, angiomatous ilevelopnmnt with a tendency towa,rd hemorrhages. The periosteal reaction caused by the tumor leads to a shell of immature trabecular bone which spans the lesion. The osteoclastic and osteoblastic activities in the bone shell refer to a rapid expansion of the tumor. This is also demonstrated radiographically. SUMMARY
The diagnosis of an aneurysmal bone cyst, which rarely occurs in the maxillofacial area, is based on characteristic, radiographic, macroscopic, and microscopic findings. The described tumor, rernoved at an early stage of development, shows clearly the tendency of the mesenchymal matrix toward differentia.tion in fibromatous structures with multiple hemorrhages while growing rapidly and expansively. RERRENCES
1. Bernier, J. L., and Bhaskar, 5. N.: Aneurysmal Bone Cyst of the Mandible, Oral Surg., Oral Med. & Oral Path. 11: 1018, 1958. 2. Lichtenstein, L.: Aneurysm& Bone Cyst; Further Observations, Cancer 6: 1228, 1953. 3. Lucas, R. W.: Pathology of the Tumors of the Oral Tissues, London, 1964, J. & A. Churchill, Ltd. 4. Uehlinger, E.: Die pathologische Anatomie der Knochengeschwiilste, Helv. chir. acta 26: 597, 1959. 5. Wang, 5. Y.: Aneurysmal Bone Cyst in the Maxilla, Plast. & Reconstruct. Surg. 25: 62, 1960.
25
NUMBER
1
JANUARY,
PATHOLOGY
AND&al
VOLUME
1968
Operative oral surgery
An aneurysmal bone cyst of the mandible Report
of a case
W. Hoppe, Prof. Dr. Ned., Dr. Med. Dent., Kiel, West Germany’ DEPARTMENT DENTAL
OF ORAL
POLICLINIC
AND
SURGERY
(CHIEF:
PROF.
DR.DR.
w.
CLINIC
(CHIEF:
PROF.
DR.DR.
SPRETER
HAHN)
OF
THE
v. KREUDES
STEIN),THEUNIVERSITYOFKIEL
A
ncurysmal bone cysts are most common in the long bones and vertebral column. This tumor, which occurs chiefly in children and young adults, is distinguished by rapid growth and, when localized in the maxillofacial area, by its characteristic eccentric, ballooned-out appearance; it causes a noticeable facial asymmetry within a very short time. Gross examination of the tumor tissue shows numerous cystic spaces of varying size, separated by septa and filled with reddish brown liquid. Histologic examination reveals multiform spaces with an endothelial lining filled partly with fresh and partly with older blood. The separating septa contain spindle cells and a varying number of giant cells. The occurrence of a cystic bone tumor in the jaws is extremely rare. Bernier *Address:
23 Kiel-Wik,
Weimarer
Str.
8, W.
Bwmany.
2
Hoppe
O.S., 0-M. January,
& OZ. 1968
and Bhaskar’ mention two cases in the mandible, and both Wang5 and Lucas3 speak of the lesion’s occurrence in the maxilla. The following case is now added to those already on record. CASE REPORT (NO. 591; 813/641 A painless expansile lesion developed in the left lower jaw of a 12-year-old girl within a period of 3 weeks and deformed the face. When the patient was admitted to the clinic, there was a marked swelling of the left mandible in the space between the second premolar and the second molar. Intraorally, an identical extension of the tumor was visible in the buccal vestibule. The consistency was firm and elastic; it was partly compressible without causing pain, but it did cause crepitus. The overlying mucosa did not show any pathologic alteration. The teeth responded positively to the electric test. In the posteroanterior roentgenogram a polycystic, distinctly gross, soap-bubble-like eccentric bulging of the left mandi-
Fig.
2. Eccentric
expansile
Fig.
1. Roentgenogram
swelling
of gross
of left
specimen;
mandible,
polycystie
covered
bone
with
lesion.
thin
layer
of bone.
Volume Number
Anewysmal
25 1
bone cyst of mandible
3
ble was visible. It involved the area between the mandibular second premolar and the second molar. The oblique lateral roentgenogram of the left side of the mandible showed a polycystic, radiolucent area with a coarse, honeycombed, irregular pattern. The root development of the mandibular second premolar and second molar was still unfinished. The mandibular third molar was commensurate with the patient’s age. The left mandibular first molar extended into the radiolueent area; its alveolar lamina dura was not interrupted. Regional lymph nodes were not palpable. Because the tumor was suspected of being an ameloblastoma or a fibromyxoma, a biopsy specimen was taken. The histologic diagnosis was fibrocementoma. Because of the rapid growth and the fact that odontogenic fibromas are known to have become malignant, surgical removal of the tumor by means of partial resection of the mandible under general endotracheal anesthesia was indicated. The hemispherical tumor could be enucleated intraorally. Its surface, from which the periosteum could easily be removed, consisted of a thin layer of bone of variable thickness and a reddish brown color. Accidental incision effected excessive bleeding. Convalescence was uneventful. Gross
findings
The
cut surface of the removed result of the most prominent feature When incised, a brownish red liquid revealed cystlike, multiform, cavernous
Fig.
lin
Fig. and
3. Surface
of cut
section
4. Highly cellular, fibrous, eosin stain. Magnification,
tumor was of a brownish reddish gray color as 2, of grayish red tissue with coarse, irregular elevations. exuded. A cross-section of the formalin-fixed specimen spaces with a distinct formation of septa.
through
tumor
septum-forming x56. Histologic
showing
stroma. section
multiform
cavernous
with calcified 255/64.)
bodies.
spaces.
(Hematoxy-’
4
O.R., O.M. January,
rroppc
Histologic
& O.P. 196X
ftndings
A tooth-bearing section of the tumor consisted of a well-vascularized, fibrous, highly cellular stroma with a reticular fibrillary network. Dispersed throughout the stromal tissue were calcified bodies of varying size-partly amorphous and partly concentrically stratified. Some places revealed a network of new-formed bone. In the area of the macroscopically visible polycystie swelling of the lower jaw there were irregularly shaped cavernous spaces, either filled with blood, filled with coagulated blood of varying age or empty. The spaces mere lined with a layer of true endothelium, interrupted by multinucleated giant cells. The septa consisted of fibrillar connective tissue, rich in spindle cells; occasionally, multinucleated giant cells were present. In thicker sections fresh or older hemorrhagic foci were noticeable. The grossly spherical thin layer of bone vvhich covered the tumor consisted of a new-formed trabecular network of varying density. Osteoclastic lacunar bone resorption and loose fibrillar tissue with ectatic capillaries predominated the thin zone between the endotheliumlined cavities and the new-formed bone, lined with osteoblasts. All these characteristic signs led to the final diagnosis of an aneurysmal bone cyst.
Fig. 5. Solitary, multinucleated cation, x224. Histologic section
Fig. x224.
6. Focus Histologic
giant 255/64.)
of hemorrhage with section 255/64.)
cells
giant
in septa.
cells.
(Hematoxylin
(Hematoxylin
and toxin
and
eosin
stain.
stain.
Magnifi-
Magnification,
Volume Number
25 1
Aneurysm&!
bone qjst
of mnndiblfz
5
DISCUSStON
The x-ray finding of a unilateral, eccentric, ballooned-out swelling, covered with a grossly hemispherical, thin bone shell is, according to Uehlinger,” pathognomonic for the aneurysmal bone cyst. The macroscopic and histologic findings bear a close resemblance to those described by Bernier and Bhaskar,’ Wang,” and Lucas.3 Only in the smaller amount of multinucleated giant cells is the present case different from the eases reported heretofore. The presence of giant cells is considered to be the expression of reparative activity. Their development and number correlate in most giant-cell-containing tumors with the amount of the hemorrhages in the tissue. The low number of giant cells in the present tumor may be due to the short time of development, which was only 3 weeks. This is in contrast with the cases published by Bernier and Bhaskar’ and Wang,5 in which the tumors developed over a period of several months. The histogenesis of the aneurysmal bone cyst is unknown, although it. is often considered to be the result of a trauma. LichtensteirP suggests that this cyst develops after a thrombosis or another local circulatory disturbance, I’Csulting in dilatation and congestion of the vascular bed. The suggestion by Bernier and Bhaskarl that the aneurysmal bone cyst is a specific entity of the giant cell reparative granuloma is based on t,he supposition that both tumorlikc lesions start with a hemorrhage. The histologic findings of the tumor discussed here, operat,ed on in an early stage, all refer to a mesenchymal matrix, on the one hand tending t.o a fibromatous differentiation and the formation of osteocementum-like structures and trabecular bone, and, on the other hand, angiomatous ilevelopnmnt with a tendency towa,rd hemorrhages. The periosteal reaction caused by the tumor leads to a shell of immature trabecular bone which spans the lesion. The osteoclastic and osteoblastic activities in the bone shell refer to a rapid expansion of the tumor. This is also demonstrated radiographically. SUMMARY
The diagnosis of an aneurysmal bone cyst, which rarely occurs in the maxillofacial area, is based on characteristic, radiographic, macroscopic, and microscopic findings. The described tumor, rernoved at an early stage of development, shows clearly the tendency of the mesenchymal matrix toward differentia.tion in fibromatous structures with multiple hemorrhages while growing rapidly and expansively. RERRENCES
1. Bernier, J. L., and Bhaskar, 5. N.: Aneurysmal Bone Cyst of the Mandible, Oral Surg., Oral Med. & Oral Path. 11: 1018, 1958. 2. Lichtenstein, L.: Aneurysm& Bone Cyst; Further Observations, Cancer 6: 1228, 1953. 3. Lucas, R. W.: Pathology of the Tumors of the Oral Tissues, London, 1964, J. & A. Churchill, Ltd. 4. Uehlinger, E.: Die pathologische Anatomie der Knochengeschwiilste, Helv. chir. acta 26: 597, 1959. 5. Wang, 5. Y.: Aneurysmal Bone Cyst in the Maxilla, Plast. & Reconstruct. Surg. 25: 62, 1960.