Large cementifying fibroma in a patient with osteogenesis imperfecta

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LARGE CEMENTIFYING

of thrombin, consolidates the platelet plug, rendering it stable. The use of a fibrin sealant during surgery mimics the last step in the body’s own clotting mechanism. The thrombin spray interacts with the fibrinogen present in the cryoprecipitate to produce fibrin monomers. This in turn undergoes hydrogen bonding to form strands of polymeric fibrin in the presence of factor XIII, fibronectin, and ionized calcium. The clotting mechanism is enhanced when the thrombin is reconstituted with calcium chloride.3 We reconstituted the thrombin with sterile isotonic saline, because the calcium content of the autologous serum appears sufficient to permit the necessary reaction to occur. This coagulum has mild retentive properties, adhering well to soft tissue as well as bone, and hence the term$fibrin glue is sometimes used to describe it. The sealant is incorporated and gradually absorbed through the release of specific proteolytic and fibrinolytic enzymes from neutrophilic granulocytes and macrophages. This duplicates the invasion and breakdown of a clot occurring during natural wound healing. A fibrin sealant is an efficient and cost-effective addition to the hemostatic armamentarium available to

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the oral and maxillofacial surgeon. This method of local hemostasis is recommended when other methods to control bleeding have failed because of either the patient’s coagulopathy or poor tissue conditions. The method is simple, the materials are readily available, and the sealant can be easily used in the operating room. Autologous donation of blood should make the availability of a fibrin sealant routine for those maxillofacial surgical procedures with the possibility of significant blood loss. References 1. Matras H: Fibrin seal: The state of the art. J Oral Maxillofacial Surg 43:605, 1985 2. Gibble JW, Ness PM: Fibrin glue: The perfect operative sealant? Transfusion 30:741, 1990 3. Rousou JA, Engleman RM, Breyer RH: Fibrin glue: An effective hemostatic agent for nonsuturable inoperative bleeding. Ann Thorac Surg 38:409, 1984 4. Lupinetti FM, Stoney WS, Alford WC Jr: Cryoprecipitate-topical thrombin glue. J Thorac Cardiovasc Surg 90502, 1985 5. Martinowitz U, Mazar AL, Taicher S: Dental extraction for patients on oral anticoagulant therapy. Oral Surg Oral Med Oral Path01 70:274, 1985

J Oral Maxillofac Surg 54:1016-1019, 1996

Large Cementifying Fibroma in a Patient With Osteogenesis lmperfecta TAKAHIKO SHIBAHARA, DDS, PHD,* HIROYASU GEN-YUKI YAMANE, DDS, PHD,* AND SADAMITSU Osteogenesis imperfecta is a hereditary disease caused by mesodermal ateliosis and, more specifically, impairment of the collagen maturation process.’ As a result, the principal clinical manifestation is multiple fractures caused by skeletal fragility. The condition is also associated with a variety of other manifestations, including blue sclerae, hypoacusia, dentinogenesis imperfecta, and lax joints and ligaments.2-4 Received from the First Department of Oral and Maxillofacial Surgery, Tokyo Dental College, Chiba-city, Japan. * Assistant Professor. T Professor. $ Associate Professor. § Assistant Professor, Department of Pathology. Address correspondence and reprint requests to Dr Shibahara: The First Department of Oral and Maxillofacial Surgery, Tokyo Dental College, l-2-2 Masago Mihama-Ku, Chiba City, 261 Japan. 0 1996 American

Association

0278-2391/96/5408-0015$3.00/O

of Oral and Maxillofacial

Surgeons

NOMA, DDS, PHD,t HASHIMOTO, DDS, PHD§

Cementifying fibroma is an odontogenic tumor consisting of a proliferation of fibrous tissue that forms cementurn.’ This relatively rare tumor is classified among the cementomas, along with giantiform cementoma, benign cementoblastoma, and periapical cemental dysplasia according to the World Health Organization (WHO) system.(j We report a 40-year-old woman with osteogenesis imperfecta who had large cementifying fibroma of the mandible. Report of Case In 1973, when the patient was 21 years old, she experienced pain in the right molar region. At that time she also noted a bony bulge in the same area, but did not seek treatment. In the summer of 1979 (27 years old), the discomfort increased. A radiograph showed a bone radiolucency, and

the patient was referred to our clinic. Facial deformity, mal-

SHIBAHARA

ET AL

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FIGURE 3. Preoperative panoramic radiograph in June 1992 showing the multilocular radiolucency in the mandibular body.

FIGURE 1. Forty-year-old woman with firm right-sided enlargement of the mandibular body.

occlusion, and blue sclerae were noted (Fig 1). Intraorally, a bone-hard, nontender, protruberance approximately 3 cm in diameter was noted on the buccal and lingual side of the right molar region. A panoramic radiograph showed a welldemarcated multilocular radiolucency containing the lower right canine extending from the lateral incisor to the molar region (Fig 2). The pulp chambers of all of the teeth were narrow, and the dentin appeared hypoplastic. Surgery exploration showed a bony cavity without an apparent lining, and microscopic examination of a bone biopsy specimen resulted in a diagnosis of simple bone cyst. When the bony expansion recurred in 198 1, the protruding area was excised, and the same microscopic diagnosis was made. In 1989 (37 years old), a radiograph showed that the bone cavity was enlarging and, when a biopsy was performed, a

FIGURE 2. Pretreatment panoramic radiograph in November 1979 showing the lesion in the right premolar region.

diagnosis of cementifying fibroma was made. Surgery was planned, but the patient’s consent could not be obtained. However, the right lower first and second premolar and molar tooth were extracted because of their looseness. At that time, the impacted canine was also extracted. In 1992, when the patient turned 40 years old, the bony expansion had increased considerably, and surgery was performed because of the risk of pathologic fracture. On the preoperative panoramic radiograph, the radiolucency was seen to have expanded both anteriorly to the midline and posteriorly to the anterior portion of the mandibular ramus (Fig 3). A computed tomography (CT) scan showed that the tumor had expanded buccally and lingually, and areas could even seen where the cortical bone was extremely thin and even perforated (Fig 4). Tumor resection was performed under general anesthesia. The procedure consisted a mandibular resection from the right canine region anteriorly to the angle of the mandible posteriorly. Because the ilium was very poorly developed, and there were no other sites where bone could be harvested, immediate jaw reconstruction was performed using an A-O reconstruction plate (Fig 5). Microscopically, numerous cystlike cavities of various sizes were seen within the tumor, but no epithelial lining was detected on the cavity walls (Fig 6). The tumor was composed of fibrous tissue rich in spindle-shaped mesenchyma1 cells, and numerous, roundish, hard tissue structures

FIGURE 4. Computed tomographic scan showing that the expanding lesion destroyed a portion of the mandibular body.

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FIGURE 5. Panoramic radiograph three years postsurgery

were scattered throughout it. The hard tissue lesions had a homogeneous matrix and possessed only a few inclusion bodies (Fig 7). There was an uncalcified zone around them, and structures that were radially arranged like cementicles could be observed with argentaffin stain. Based on these findings, a diagnosis of cementifying fibroma was made.

Discussion

The cause of osteogenesis imperfecta is thought to be a generalized defective development of mesodermal tissue. There are also reports claiming that the cause is largely a defect in collagen metabolism. Abnormalities in the synthesis of the a-chain and pro-a chain of type I collagen, the major component of bone collagen, have been reported.7X8 The three principal clinical manifestations of osteogenesis imperfecta are fragile bones, blue sclerae, and hypoacusia. Osteogenesis imperfecta also damages mesoderm-derived tissue in general, and is associated with a diversity of other clinical manifestations. Osteogenesis imperfecta is an extremely rare disease, affecting only one person in 8,000, and many of

FIGURE 6. Photomicrograph showing the numerous cyst-like cavity walls within (hematoxylin-eosin stain, original magnification X75).

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FIGURE 7. Photomicrograph of fibrous tissue rich in spindleshaped mesenchymal cells containing round, calcified structures (hematoxylin-eosin stain, original magnification ~125).

those with the congenital form are stillborn or die shortly after birth. This patient displayed the clinical manifestations of congenital osteogenesis imperfecta, including multiple fractures in the hip area, blue sclerae, hypoacusia, dentinogenesis imperfecta, cranial deformity, deformation of the pectoral region, and scoliosis. Cementifying fibroma is an odontogenic tumor in which formation of cementum is observed within fibromatous tissue.g It is a relatively rare tumor, accounting for 2% of the odontogenic tumors and 33% of the cementomas encountered in our clinic.” This patient’s cementifying fibroma was composed of fibromatous tissue with abundant spindle-shaped mesenchymal cells, and containing numerous, round, hard tissue formations. Because no bone tissue was detected, a diagnosis of cementifying fibroma was made. However, a few cystic cavities were also present in the tumor. This raises the possibility of a relationship to the aneurysmal bone cyst. Struthers and Shear” hypothesized that the aneurysmal bone cyst was a secondary phenomenon that occurred in a primary lesion of bone. El Deeb et al” reported that 21% of 50 aneurysmal bone cysts in the jaws had developed in association with various primary lesions of bone, including fibroosseous lesions. This patient’s tumor was considered to be of that type. Changes in radiolucency according to the degree of maturation are claimed to be a characteristic of cementifying fibromas, with the degree of maturation being classified into a bone liquefaction stage, a cementum-forming stage, and a mature stage. However, in this patient there were well-demarcated multilocular radiolucencies that were clearly different from the surrounding bone. The patient’s course was monitored continuously for 3 years after the diagnosis of cementifying fibroma was made from the biopsy specimen in 1989, but none of the characteristic changes in radiolucency were observed. The fact that this patient exhib-

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ited idiosyncratic manifestations appeared to be in some way related to the patient having osteogenesis imperfecta. No differences have been found between the cementurn and bone in biochemical analysis of organic protein components.13 Eight types of peptide chains, differing in amino acid sequence, are generally present in collagen, and six different molecules exist, depending on how they are combined.14 The collagen found in both bone and cementum is type I collagen. This, together with the abnormal synthesis of type I collagen attributable to osteogenesis imperfecta, is thought to be why there were also associated abnormalities in the cementurn. Embryologically, bone, dentin, and cementum are all mesodermal tissues. This suggests that cementum abnormalities are likely to occur in osteogenesis imperfecta, which has a high rate of complication by dentinogenesis imperfecta. Because bone tissue and cementum are fundamentally similar to each other, both embryologically and biochemically, there seems to be some connection between osteogenesis imperfecta and cementoma. Because the histologic studies performed at the time of the patient’s initial examination resulted in a diagnosis of simple bone cyst, the course of the disease was followed for 10 years. It is unknown whether the cementifying fibroma arose as a new lesion in the same site during that lo-year period or whether a cementifying fibroma was already present in a portion of the bone cyst in the initial stage. Furthermore, numerous cavities were observed within the cementifying fibroma, but it is unclear whether this was because the patient had osteogenesis imperfecta, or because the growth of this tumor was idiosyncratic. Long-term follow-up will be necessary, taking into account impair-

ment of the bone healing process in osteogenesis imperfecta patients and the unknown cause of cementifying fibroma. References 1. Looser E: Zur kenntnis der Qsteogenesis Imperfecta Congenita et tarde. Mitt Grenzged Med Chir 15:161, 1937 2. Cohen-Solal L, Bonaventure J, Maroteaux P: Dominant mutations in familial lethal and severe osteogenesis imperfecta. Hum Genet 87:297, 1991 3. Mundlos S, Spranger J: Genetic disorders of connective tissues. Curr Opin Rheumatol 3:832, 1991 4. Ugoljewa S, Herrmann C, Hock S: Disorders of growth and development in patients with osteogenesis imperfecta. Arztl Jugendkd 82:115, 1991 5. Shager WG, Hine MK: A Textbook of Oral Pathology (ed 3). Philadelphia, PA, Saunders, 1974, p 268 6. Pindborg JJ, Krammer IR: Histological Typing of Odontogenie Tumor, Jaw Cyst. Geneva, Switzerland, WHO, 1971, p 31 7. Willing MC, Deschenes SP, Scott DA, et al: Osteogenesis imperfecta type I. Am J Hum Genet 55:638, 1994 8. Steinmann B, Westerhausen A, Constantinou CD, et al: Substitution of cysteine for glycine-alpha l-169 in the pro alpha l(1) chain of type I procollagen in a proband with lethal osteogenesis imperfecta destabilizes the triple helix at a site C-terminal to the substitution. Biochem J 279:147, 1991 9. Kramer IRH, Pindborg JJ, Shear M: Histological Typing of Odontogenic Tumours (ed 2). New York, NY, Springer-Verlag, 1992, p 27 10. Eda S, Shimono M: A case report of cementifying fibroma. Shikwa Gakuho 71:2014, 1971 11. Struthers PJ, Shear M: Aneurysmal bone cyst of the jaws. Int J Oral Surg 13:92, 1984 12. El Deeb M, Sedan0 HO, Waite DE: Aneurysmal bone cyst of the jaws. Int J Oral Surg 9:301, 1980 13. Lukinmaa PL, Vaheri A: ED-A region-containing isoform of cellular fibronectin is present in dentin matrix in dentinogenesis imperfecta associated with osteogenesis imperfecta. J Dent Res 73:1187, 1994 14. Deak SB, Scholz PM, Amenta PS, et al: The substitution of arginine for glycine 85 of the alpha l(1) procollagen chain results in mild osteogenesis imperfecta. J Biol Chem 266:21827, 1991

J Oral Maxillofac Surg 54:1019-1020, 1996

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LJISCUSSlOn Large Cementifying Fibroma in a Patient With Osteogenesis Imperfecta Ellen Eisenberg, DMD University of Connecticut Health Center, Farmington, Connecticut The authors present a case of what they believe is a benign neoplasm, specifically, a cementifying$broma of the mandible in a young woman with osteogenesis imperfecta (01). If this diagnosis is correct, it reflects the occurrence of two relatively uncommon conditions in a single patient. Given the interesting history, complex clinical and radiographic features, plus the composite microscopic findings that indicate a benign fibroosseous process, the precise nature of this case actually presents a formidable diagnostic challenge worthy of brief discussion.

Is this truly a cementifying

fibroma? Apparently

the au-

thors’ diagnostic impression is based primarily on just one aspect of the lesion’s microscopic appearance, as narrowly illustrated in Figure 7, a high-power photomicrograph that shows fibroosseous elements: sparsely cellular cementumlike bone “droplets” within a highly cellular fibrous stroma. Although these features admittedly are among the microscopic elements one may encounter in “cementifying” (or ossifying) fibromas,’ in isolation from an abundance of other attendant relevant data, they are at best diagnostically inconclusive for any specific fibroosseous entity, neoplastic or otherwise. Should one ignore the other concomitant histologic features of this lesion as depicted in Figure 6, a low-power photomicrograph, which demonstrates striking traumatic bone cystlike changes among the aforementioned fibroosseous tissue elements? Microscopic cystlike changes