Aggressive fibromatosis Report
of a case and review
of the maxilla
of the literature
William B. Donohue, DDS, MSc, FRCD (C),a Danny Malexos, Huan Pham, DDS, MSC,~ Montreal, Quebec, Canada FACULTY SURGERY,
OF
DENTAL
ST-MARY’S
MEDICINE, HOSPITAL
UNIVERSIT6 CENTER,
DE
MONTRCAL,
AND
DIVD,~ and
DIVISION
OF
ORAL
MONTREAL
Aggressive fibromatosis is a rare tumor of the jaws. Its occurrence in the maxilla is even more exceptional. The case of a 14-year-old girl with a peripheral fibromatosis of the palatal process of the maxilla is described. The lesion manifested as a painless swelling. On initial excision, a diagnosis of fibroma was made. After recurrence and reevaluation of the microscopic slides, a final diagnosis of aggressive fibromatosis was established. The features of this case and 12 similar lesions of the maxilla previously described in the literature are analyzed and discussed. (ORAL SURC ORAL MED ORAL PATHOL 1990;69:420-6)
F
ibromatosis encompasses a group of soft tissue lesions such as the soft tissue desmoid tumor and the desmoplastic fibroma of bone, which are characterized histologically by fibroblastic proliferation and clinically by the potential to infiltrate and invade locally and to recur after surgical excision, but not metastasize. They must be distinguished, if possible, from other spindle cell fibroblastic tumors of the head and neck such as the histiocytoma, fibrosarcoma, nerve sheath tumors, etc. The most common anatomic locations have been the abdominal muscles and the extremities. Other regions affected are the mesentery, small intestine, iliac fossa, breasts, and head and neck region.‘. 2 In Sweden, the incidence of fibromatoses has been found to be two cases per year per million population.3 The incidence of these lesions in the head and neck is mentioned as from 9.5% to 50% of all desmoid tumors.‘, 4 Within this area, 40% to’ 80% of the tumors are located in the neck.3T 5 The face is mentioned as the second most frequent site for desmoid lesions, with a preponderance in the region of the
aAssociate Professor,
Faculty of Dental Medicine, Universiti de MontrCal; Division of Oral Surgery, St-Mary’s Hospital Center, Montreal. bin OPW~~I nrlcticn Tlinician, Section of Oral Surgery, Universiti de MontrCal; Division of Oral Surgery, St-Mary’s Hospital Center, Montreal. 7/12/11138
420
cheek.5 Other sites are infrequently involved, though fibromatosis has been reported in the alveolar process of the maxilla,5, 7 the inferior turbinates,* the maxillary sinus,8 the tongue,9 the parotid gland,‘O the pharynx,3 the sphenoid bone,12 and the mandible.13 The approximate anatomic location of 73 cases of aggressive fibromatosis of the jaws and adjacent regions is indicated in Fig. 1. It is evident that the mandible is much more frequently involved than the maxilla. We report an additional case of a 14year-old girl with a lesion in the soft tissue of the palate that invaded the underlying bone. CASEREPORT
A 14-year-old girl had a small painless swelling of the left side of the hard palate that had been present for a few months (Fig. 2). The swelling was firm, with a sessile base, and was nontender. The teeth opposite the tumefaction were vital and nonmobile, and radiographs showed no bone destruction at this time. The overlying mucosa appeared normal. The provisional clinical diagnosis was a benign tumor of a minor salivary gland. A 0.8 X 0.6 X 0.4 cm incisional biopsy was done on Aug. 13, 1984. The histologic diagnosis was fibroma. The tumor continued to grow after the first biopsy, suggesting a more aggressive lesion than a simple fibroma. On Sept. 21, 1984, an attempt was made to resect the entire tumor. An elipse of mucosa measuring 2.3 X 1 cm, underlying tissue with submucosal measuring 2.3 X 2 X 1.1 cm, was excised. The latter was gray in color and firm in consistency. Histologically, the tumor consisted of highly cellular connective tissue with immature plump fibroblasts and multidirectional bundles of fibers (Fig. 3). No cellular or nuclear atypia was noted. Occa-
Volume Number
Aggressive jibromatosis
69 4
of the maxilla
42 1
TtiE LOCATION OF 73 CASES OF AGGRESSIVE FIBROMATOSIS (DESMOID TUMORS) OF THE MOUTH AND PERIORAC REGIONS
Fig.
Fig.
1. Anatomic
2. Arrows
distribution
indicate
swelling
of 73 cases of aggressive fibromatosis
of jaws.
of hard palate when patient was first examined.
sional mitotic figures were present (less than 1 per 10 high-power fields). The diagnosis was aggressive fibromatosis, incompletely excised. Instead of healing normally. the biopsy site evolved into an ulcerative lesion. which slowly increased in size. The total lesion measured 5 x 6 cm and involved the mucosa of the left side of the hard and soft palate, encroaching close to the attached gingiva (Fig. 4). In view of the infiltrating nature of the lesion. a resection of the left side of the hard palate extending into the soft palate and including part of the alveolar process and three teeth was carried out on
Dec. 10, 1984 (Fig. 5). The completeness of the resection was confirmed by frozen section. Firm yellowish-gray fibrous tissue occupied the central, anterior, and lateral areas of the submucosa1 tissue. The resultant naso-oral communication was closed with an obturator (Fig. 6). Microscopic sections showed fibrous tissue proliferation consistent with aggressive fibromatosis identical to the previous biopsy specimens. The lesion invaded the superficial underlying palatal bone, but all resection margins were free. Three years later, there has been no recurrence of the tumor.
422
Donohue, Malexos, and Pham
ORAL
SURC
ORAI
Pvlt~
ORAL PATH~L April 1990
Fig. 3. Photomicrographsof representativefields of secondbiopsy specimens.(Hematoxylin and eosin stain.) A, Low-power view of the biopsyspecimenshowingthe cellular arrangement.(Original magnification, X 160.) B, Thin, elongatedfibroblastswith a herring-bonepattern. (Original magnification,X400.)
MATERIALS,
METHODS,
AND RESULTS
All case reports in the English- and Frenchlanguage publications, published before 1987 with respect to aggressive fibromatosis of the jaws, were reviewed. In addition, all cases reported as desmoplastic fibroma or fibromatosis of the jaws were reviewed. Rakower’s case,14 although reported as fasciitis, was included because several of the pathologists who reviewed the case considered it to be a low-grade fibrosarcoma or aggressive fibromatosis. None of the authors differentiated between central and peripheral fibromatosis. Whenever possible, relying on the case history, we have tried to distinguish between the two.
Age
and gender
incidence
Tables I and II list the data from 12 published cases of aggressive fibromatosis of the maxilla, to which our casehas been added.14-i6,6,‘7-23The condition has been reported in nine males and four females. The average age of the patients was 17 years but with a wide range from 48 years to 2 years 9 months. Signs
and symptoms
Eighty-eight percent of the patients with fibromatosis of the jaws were initially seen with hard swellings.l3 Pain, tooth mobility, and limitation of mouth opening are infrequent.
Volume 69 Number 4
Aggressive
Fig.
features
behavior
423
5. Surgical specimenshowingulceration on palatal surface.
On radiographs, well-defined radiolucencies are frequently described; they are often multilocular with a tendency to perforate the cortex. Biologic
of the maxilla
4. Ulcerative, fungating lesionthat developedafter secondbiopsy.
Fig.
Radiographic
jibromatosis
and treatment
Most authors believe that although aggressive fibromatosis is locally invasive, there are no metastases.However, Stout,36 in 1962, was unable to confirm this. One author gives a metastatic incidence of 8%~~’ Extension into adjacent lymph nodes has been reported.’
The treatment of aggressive fibromatosis is tradi-tionally surgical resection with an area of tumor-free tissue. If conservative surgical resection is done, the incidence of local recurrence is high, reported to be from 10% to greater than 90%.26.2’ Of the twelve casesof maxillary fibromatosis, three recurred (Table II). Radiation therapy has not been recommended in the past because of difficulty in controlling the disease.24,27 This has been claimed to be due to the treatment of extensive lesionswith low doses.*sAlso, malignant transformation has been reported years after massive radiotherapy.“j Recent studies indicate
Donohue, Malexos,
424
and Pham
Fig.
Table
1. Features
Case No.
ORAL SURG ORAL
6. Postsurgical defect resulting from excision of lesion.
of 13 cases of aggressive
Author
Year
fibromatosis
Age
Gender
Rakower14
1971
48 yr
M
2
Pindborg and Hjrting-HansenI
1974
2 Y’
M
3
Sood and Chatterjee16
1975
21 yr
M
4
Wilkins
et aL6
3 yr
M
5
Wilkins
et a1.6
1975 case III 1975 case VI
16 yr
M
6
Summers
21 yr
F
7
Melrose and Abrams’*
1976 case I 1980 case II
5 yr
F
8
Bertrand
9.5 yr
F
9
KrautZo
1981 case III 1981
17 yr
M
1981
41 yr
M
1982 case IV 1985
33 mo
M
22 yr
M
1987
14 yr
F
and Matz17
et a1.19
IO
Osguthorpe
11
Goepfert
12
George
13
Donohue and associates
et al.*’
et al.** et a1.23
MEI) ORAL PATHOI April 1990
Anatomic area (Central = C; Peripheral = P; No precise details = ? Right maxilla CC) Left inferior border of orbit (?) Right lower orbital margin (C) Anterior palate (3 Right maxillary ridge and palate (C) Right maxilla (C) Palate right maxillary ridge (P) Right maxilla (?I Right maxillary sinus (C) Left maxillary sinus region (?) Right upper gingiva (P) Right maxillary alveolus (C) Left palate (P)
Preliminary diagnosis Apical
granuloma
Time before diagnosis (mol 1
Radiographic findings Radiolucency Radiolucency
48
Opacity
Radiolucency Granuloma
3 wks
-
120 1
Radiolucency
Opacity Loss interdental bone
6 Odontogenic keratocyst
Cloudiness, sinus 3
Periapical Fibroma
granuloma
4 3
Variable-opaque
Volume Number
Aggressive jibromatosis
69 4
that it may be possible to treat patients with aggressive fibromatosis with the hope of local control when a dose of 5800 rads or greater is employed.26, 29*3o Radiation alone or in conjunction with surgery has also been advocated.24 Chemotherapy has been suggested as another method of therapy.3’* 32 Hormonotherapy with progesterone is claimed to reduce the size of large lesions and, in some cases, lead to a complete cure.32T 33 DISCUSSION
Not surprisingly, in most cases of aggressive fibromatosis, the tumors were clinically misdiagnosed. Among the preliminary diagnoses mentioned in the literature, the following were considered: fibroma, fibrous histiocytoma, granuloma, cyst, ameloblastama, fibrous dysplasia, and sarcoma. Our case indicates how a misdiagnosis of a fibroma resulted in recurrence after incomplete removal. The precise diagnosis becomes confusing because of a multitude of diagnostic terms. In 1954, Arthur F’urdy Stout reviewed the subject of juvenile fibromatosis. He found that it “was in such a state of confusion that he wondered if there was anything at all to be gained by retaining the older names.“34 He mentioned various forms of generalized and local fibromatosis but included differentiated nonmetastasizing fibrosarcomas in the term jibromatosis. Jaffe,35 in 1958, introduced the term desmoplastic jibroma, not to be confused with desmoid fibromatosis, a lesion of a similar dense fibrous nature occurring in the abdominal wall. He stated that the qualifying term desmoplastic was introduced to clearly distinguish this bone lesion from nonossifying Jibroma of bone and from chondromyxoid jibroma of bone, two lesions that also include the term fibroma. Stout36 reviewed the subject again in 1962 in a study of 241 cases of juvenile fibromatosis. He attempted to distinguish between juvenile fibromatosis and fibrosarcoma and to determine those that were potentially metastatic. He stated that “it must be confessed that the study has failed in both attempts.” Wilkins and Waldron6 in 1975, suggested that the title aggressivefibromatosis was a more appropriate term, reflecting the invasive characteristics of the disease. Synonyms listed were extra-abdominal desmoid, juvenile jibromatosis, aggressive infantile jibromatosis. and congenital jibrosarcoma. Addante and Laskin,37 returning to Jaffe’s term of desmoplastic fibroma, proposed that this diagnosis be restricted to lesions that arise from bone whereas those that arise from the adjacent tissues and then invade bone be termed aggressive jibromatoses.
of the maxilla
425
II. Features of 13 cases of aggressive fibromatosis
Table
Case
Follow-up h-4
1 2 3 4 5 6 I 8 9 10
38
48 lost 3 9
II 12 13
27 12 36
24 14 12
Recurrence
Complaints Bulging
mass Swelling, vague pain Swelling Pain ulceration Swelling Swelling Swelling Facial pain Anesthesia, nasal stuffiness, swelling
No No No Yes No Yes No Yes No No No No
Swelling Swelling
Histologically, they consist of well-differentiated fibrous tissue that infiltrates neighboring structures. It is generally accepted that soft tissue fibromatoses arise from musculoaponeurotic structures.t2 They are considered to be benign reactive lesions of unknown cause, although trauma” 9, 25,27and hormonal imbalance have been suggested.2 They can be locally invasive and tend to recur after surgical resection. They can be difficult to differentiate from low-grade fibrosarcoma histologically and, therefore in some cases, only the clinical course will give the correct diagnosis. SUMMARY
A case of peripheral aggressive fibromatosis of the left maxilla is reported. The disease was first noted when a painless mass was observed in the hard palate of a ICyear-old white girl. The benign appearance of the tissues when examined microscopically led to an initial diagnosis of fibroma. The rapid recurrence of the lesion led to the final diagnosis. The patient is free of disease 36 months later. A review of the literature indicates a total of 73 cases of aggressive fibromatosis or desmoplastic fibroma of the jaws. If one accepts aggressiveJibromatosis and desmoplastic jibroma as synonyms, a total of 13 cases, including this case, have been reported in the maxilla.
REFEREtdCES 1. Das Gupta desmoids: 170:109-21. 2. Masson JK, Am J Surg
TIC, Brasfield RD, a clinicopathological Soule EH. Desmoid 1966;l 12:615-21.
O’Hara J. Extra-abdominal study. Ann Surg 1969; tumors
of the head and neck.
426
7
8.
9. 10. II.
12.
13. 14.
15. 16. 17. 18.
19.
20.
21.
22.
Donohue,
Malexos,
and Pham
Dahn I, Jonsson N, Lundh G. Desmoid turnouts. Acta Chir Stand 1963;126:305-14. Kauffman SL, Stout AP. Congenital mesenchymal tumors. Cancer 1965;18:460-76. Conley T, Healey WV, Stout AD. Fibromatosis of the head and neck. Am J Surg 1966;112:609-14. Wilkins SA, Waldron CA, Mathews WH, Droulias CA. Aggressive fibromatosis of the head and neck. Am J Surg 1975;130:412-5. Goebfert H, Cangir A, Ayala AG, et al. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Am J Surg 1982;144:437-44. Fu Y, Perzin K. Nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx. Cancer 1976;37:291228. Schwartz H, Ward PH. Aggressive fibromatosis of the tongue. Ann Otol 1979;88: 12-5. May Mudar B, Winiarski N. Desmoid tumor presenting as a parotid mass. JAMA 1978;239:337-9. Close LG, Rosenberg HS, Vogler C, Warshaw HE. Neonatal laryngeal fibromatosis. Otolaryngol Head Neck Surg 1981; 89:992-7. Nieto CS, Forceliedo FF, Cortes JC. Aggressive fibromatosis of the sphenoid. Arch Otolaryngol Head Neck Surg 1986; 112:326-8. Makek M, Lello GE. Desmoplastic fibroma of the mandible. J Oral Maxillofac Surg 1986;44:385-91. Rakower W. Fasciitis, an unusual diagnosis and the clinician’s dilemma: report of a case. J Oral Surg 1971;29:5036. Pindborg JJ, Hjrting-Hansen E. Atlas of diseases of the jaws. Philadelphia: WB Saunders, 1974:64. Sood VP, Chatterjee AK. Desmoplastic fibroma of maxilla. J Laryngol Otol 1975;89:329-33. Summers L, Matz LR. Recurrent desmoplastic fibroma. Int J Oral Surg 1976;5:100-3. Melrose RJ, Abrams AM. Juvenile fibromastosis affecting the jaws. ORAL SURG ORAL MED ORAL PATHOL 1980;49:31724. Bertrand JC, Plautier D, Chanterelle A, Mazza D. Fibromes desmoides maxillo-mandibulaires. Rev Stomatol Chir Maxillofac 1981;82:127-31. Kraut RA. Fibromatosis following enucleation of an odontogenie keratocyst. ORAL SURG ORAL MED ORAL PATHOL 198 1; .51:10-5. Osguthorpe JD, Adkins WY, Rawe SE. Combined extracranial-intracranial resection of a maxillary desmoid tumor. Otolaryngol Head Neck Surg 1981;89:392-7. Goepfert H, Cangir H, Ayala A, Eptekhari F. Chemotherapy
ORAL SURG ORAL
MED ORAL PATHOL April 1990
of locally aggressive head and neck tumors in the pediatric group. Am J Surg 1982;144:437-44. 23. George DI, Gould AR, Milles RL, Strull NJ. Desmoplastic fibroma of the maxilla. J Oral Maxillofac Surg 1985;43:7 1824. Lagace R, Delage C, Bouchard HL, Seemayer TA. Desmoplastic fibroma of bone, an ultrastructural study. Am J Surg Pathol 1979;3:423-30. 25. Sartonis DJ, Arkoff RS, Parker BR. Aggressive fibromatosis of the mandible in childhood. Skeletal Radio1 1983;10:154-6. 26. Krystyna DK, Herman DS. Radiation therapy in the treatment of aggressive fibromatoses (desmoid tumors). Cancer 1984;54:2051-5. 27. Dahn 1, Jonsson N, Goran L. Desmoid tumours. Acta Chir Stand 1963;126:305-14. 28. Chomette G, Tran Baloc P, Avriol M, Guilbert F. Fibrome desmoide osseux. Etude histoenzymologique et ultrastructurale d’un cas de siege mandibulaire. Ann Pathol 1981;1:4751. FM, Weiss SW. Soft tissue tumors. St. Louis: The 29. Enzinger CV Mosby Co, 1983:45. HM, Goebel R, Weichsebaum RR, Greenberger 30. Greenberg JS, Cassady JR. Radiation therapy for the treatment of benign locally aggressive fibromatosis. Rad Oncol Biol Phys 1980; 6:1412. 31. Goebfert H, Cangir A, Ayala AG, Eftekhari F. Chemotherapy of locally aggressive head and neck tumors in the pediatric age group. Am J Surg 1982;144:437-44. 32. Sauven P. Musculoaponeurotic fibromatosis treated by surgery and testolactone. J R Sot Med 1982;75:281-3. 33. Lanari A. Effect of progesterone on desmoid tumors (aggressive fibromatosis). N Engl J Med 1983;24:1523. 34. Stout AP. Juvenile fibromatosis. Cancer 1954;7:953-78. 35. Jaffe HL. Tumors and tumorous conditions of the bones and joints. Philadelphia: Lea & Febiger, 1958:298. 36. Stout AP. Fibrosarcoma in infants and children. Cancer 1962;15:1028-40. 37. Addante RR, Laskin JL. Clinicopathological conferences: case 55, large mandibular mass. J Oral Maxillofac Surg 1985;43:531-6. Reprint requests to: Dr. William B. Donohue Universiti de Montreal Faculty of Dental Medicine P.O. Box 6128-“A” Montreal. Canada H3C 357
of a case and review
of the maxilla
of the literature
William B. Donohue, DDS, MSc, FRCD (C),a Danny Malexos, Huan Pham, DDS, MSC,~ Montreal, Quebec, Canada FACULTY SURGERY,
OF
DENTAL
ST-MARY’S
MEDICINE, HOSPITAL
UNIVERSIT6 CENTER,
DE
MONTRCAL,
AND
DIVD,~ and
DIVISION
OF
ORAL
MONTREAL
Aggressive fibromatosis is a rare tumor of the jaws. Its occurrence in the maxilla is even more exceptional. The case of a 14-year-old girl with a peripheral fibromatosis of the palatal process of the maxilla is described. The lesion manifested as a painless swelling. On initial excision, a diagnosis of fibroma was made. After recurrence and reevaluation of the microscopic slides, a final diagnosis of aggressive fibromatosis was established. The features of this case and 12 similar lesions of the maxilla previously described in the literature are analyzed and discussed. (ORAL SURC ORAL MED ORAL PATHOL 1990;69:420-6)
F
ibromatosis encompasses a group of soft tissue lesions such as the soft tissue desmoid tumor and the desmoplastic fibroma of bone, which are characterized histologically by fibroblastic proliferation and clinically by the potential to infiltrate and invade locally and to recur after surgical excision, but not metastasize. They must be distinguished, if possible, from other spindle cell fibroblastic tumors of the head and neck such as the histiocytoma, fibrosarcoma, nerve sheath tumors, etc. The most common anatomic locations have been the abdominal muscles and the extremities. Other regions affected are the mesentery, small intestine, iliac fossa, breasts, and head and neck region.‘. 2 In Sweden, the incidence of fibromatoses has been found to be two cases per year per million population.3 The incidence of these lesions in the head and neck is mentioned as from 9.5% to 50% of all desmoid tumors.‘, 4 Within this area, 40% to’ 80% of the tumors are located in the neck.3T 5 The face is mentioned as the second most frequent site for desmoid lesions, with a preponderance in the region of the
aAssociate Professor,
Faculty of Dental Medicine, Universiti de MontrCal; Division of Oral Surgery, St-Mary’s Hospital Center, Montreal. bin OPW~~I nrlcticn Tlinician, Section of Oral Surgery, Universiti de MontrCal; Division of Oral Surgery, St-Mary’s Hospital Center, Montreal. 7/12/11138
420
cheek.5 Other sites are infrequently involved, though fibromatosis has been reported in the alveolar process of the maxilla,5, 7 the inferior turbinates,* the maxillary sinus,8 the tongue,9 the parotid gland,‘O the pharynx,3 the sphenoid bone,12 and the mandible.13 The approximate anatomic location of 73 cases of aggressive fibromatosis of the jaws and adjacent regions is indicated in Fig. 1. It is evident that the mandible is much more frequently involved than the maxilla. We report an additional case of a 14year-old girl with a lesion in the soft tissue of the palate that invaded the underlying bone. CASEREPORT
A 14-year-old girl had a small painless swelling of the left side of the hard palate that had been present for a few months (Fig. 2). The swelling was firm, with a sessile base, and was nontender. The teeth opposite the tumefaction were vital and nonmobile, and radiographs showed no bone destruction at this time. The overlying mucosa appeared normal. The provisional clinical diagnosis was a benign tumor of a minor salivary gland. A 0.8 X 0.6 X 0.4 cm incisional biopsy was done on Aug. 13, 1984. The histologic diagnosis was fibroma. The tumor continued to grow after the first biopsy, suggesting a more aggressive lesion than a simple fibroma. On Sept. 21, 1984, an attempt was made to resect the entire tumor. An elipse of mucosa measuring 2.3 X 1 cm, underlying tissue with submucosal measuring 2.3 X 2 X 1.1 cm, was excised. The latter was gray in color and firm in consistency. Histologically, the tumor consisted of highly cellular connective tissue with immature plump fibroblasts and multidirectional bundles of fibers (Fig. 3). No cellular or nuclear atypia was noted. Occa-
Volume Number
Aggressive jibromatosis
69 4
of the maxilla
42 1
TtiE LOCATION OF 73 CASES OF AGGRESSIVE FIBROMATOSIS (DESMOID TUMORS) OF THE MOUTH AND PERIORAC REGIONS
Fig.
Fig.
1. Anatomic
2. Arrows
distribution
indicate
swelling
of 73 cases of aggressive fibromatosis
of jaws.
of hard palate when patient was first examined.
sional mitotic figures were present (less than 1 per 10 high-power fields). The diagnosis was aggressive fibromatosis, incompletely excised. Instead of healing normally. the biopsy site evolved into an ulcerative lesion. which slowly increased in size. The total lesion measured 5 x 6 cm and involved the mucosa of the left side of the hard and soft palate, encroaching close to the attached gingiva (Fig. 4). In view of the infiltrating nature of the lesion. a resection of the left side of the hard palate extending into the soft palate and including part of the alveolar process and three teeth was carried out on
Dec. 10, 1984 (Fig. 5). The completeness of the resection was confirmed by frozen section. Firm yellowish-gray fibrous tissue occupied the central, anterior, and lateral areas of the submucosa1 tissue. The resultant naso-oral communication was closed with an obturator (Fig. 6). Microscopic sections showed fibrous tissue proliferation consistent with aggressive fibromatosis identical to the previous biopsy specimens. The lesion invaded the superficial underlying palatal bone, but all resection margins were free. Three years later, there has been no recurrence of the tumor.
422
Donohue, Malexos, and Pham
ORAL
SURC
ORAI
Pvlt~
ORAL PATH~L April 1990
Fig. 3. Photomicrographsof representativefields of secondbiopsy specimens.(Hematoxylin and eosin stain.) A, Low-power view of the biopsyspecimenshowingthe cellular arrangement.(Original magnification, X 160.) B, Thin, elongatedfibroblastswith a herring-bonepattern. (Original magnification,X400.)
MATERIALS,
METHODS,
AND RESULTS
All case reports in the English- and Frenchlanguage publications, published before 1987 with respect to aggressive fibromatosis of the jaws, were reviewed. In addition, all cases reported as desmoplastic fibroma or fibromatosis of the jaws were reviewed. Rakower’s case,14 although reported as fasciitis, was included because several of the pathologists who reviewed the case considered it to be a low-grade fibrosarcoma or aggressive fibromatosis. None of the authors differentiated between central and peripheral fibromatosis. Whenever possible, relying on the case history, we have tried to distinguish between the two.
Age
and gender
incidence
Tables I and II list the data from 12 published cases of aggressive fibromatosis of the maxilla, to which our casehas been added.14-i6,6,‘7-23The condition has been reported in nine males and four females. The average age of the patients was 17 years but with a wide range from 48 years to 2 years 9 months. Signs
and symptoms
Eighty-eight percent of the patients with fibromatosis of the jaws were initially seen with hard swellings.l3 Pain, tooth mobility, and limitation of mouth opening are infrequent.
Volume 69 Number 4
Aggressive
Fig.
features
behavior
423
5. Surgical specimenshowingulceration on palatal surface.
On radiographs, well-defined radiolucencies are frequently described; they are often multilocular with a tendency to perforate the cortex. Biologic
of the maxilla
4. Ulcerative, fungating lesionthat developedafter secondbiopsy.
Fig.
Radiographic
jibromatosis
and treatment
Most authors believe that although aggressive fibromatosis is locally invasive, there are no metastases.However, Stout,36 in 1962, was unable to confirm this. One author gives a metastatic incidence of 8%~~’ Extension into adjacent lymph nodes has been reported.’
The treatment of aggressive fibromatosis is tradi-tionally surgical resection with an area of tumor-free tissue. If conservative surgical resection is done, the incidence of local recurrence is high, reported to be from 10% to greater than 90%.26.2’ Of the twelve casesof maxillary fibromatosis, three recurred (Table II). Radiation therapy has not been recommended in the past because of difficulty in controlling the disease.24,27 This has been claimed to be due to the treatment of extensive lesionswith low doses.*sAlso, malignant transformation has been reported years after massive radiotherapy.“j Recent studies indicate
Donohue, Malexos,
424
and Pham
Fig.
Table
1. Features
Case No.
ORAL SURG ORAL
6. Postsurgical defect resulting from excision of lesion.
of 13 cases of aggressive
Author
Year
fibromatosis
Age
Gender
Rakower14
1971
48 yr
M
2
Pindborg and Hjrting-HansenI
1974
2 Y’
M
3
Sood and Chatterjee16
1975
21 yr
M
4
Wilkins
et aL6
3 yr
M
5
Wilkins
et a1.6
1975 case III 1975 case VI
16 yr
M
6
Summers
21 yr
F
7
Melrose and Abrams’*
1976 case I 1980 case II
5 yr
F
8
Bertrand
9.5 yr
F
9
KrautZo
1981 case III 1981
17 yr
M
1981
41 yr
M
1982 case IV 1985
33 mo
M
22 yr
M
1987
14 yr
F
and Matz17
et a1.19
IO
Osguthorpe
11
Goepfert
12
George
13
Donohue and associates
et al.*’
et al.** et a1.23
MEI) ORAL PATHOI April 1990
Anatomic area (Central = C; Peripheral = P; No precise details = ? Right maxilla CC) Left inferior border of orbit (?) Right lower orbital margin (C) Anterior palate (3 Right maxillary ridge and palate (C) Right maxilla (C) Palate right maxillary ridge (P) Right maxilla (?I Right maxillary sinus (C) Left maxillary sinus region (?) Right upper gingiva (P) Right maxillary alveolus (C) Left palate (P)
Preliminary diagnosis Apical
granuloma
Time before diagnosis (mol 1
Radiographic findings Radiolucency Radiolucency
48
Opacity
Radiolucency Granuloma
3 wks
-
120 1
Radiolucency
Opacity Loss interdental bone
6 Odontogenic keratocyst
Cloudiness, sinus 3
Periapical Fibroma
granuloma
4 3
Variable-opaque
Volume Number
Aggressive jibromatosis
69 4
that it may be possible to treat patients with aggressive fibromatosis with the hope of local control when a dose of 5800 rads or greater is employed.26, 29*3o Radiation alone or in conjunction with surgery has also been advocated.24 Chemotherapy has been suggested as another method of therapy.3’* 32 Hormonotherapy with progesterone is claimed to reduce the size of large lesions and, in some cases, lead to a complete cure.32T 33 DISCUSSION
Not surprisingly, in most cases of aggressive fibromatosis, the tumors were clinically misdiagnosed. Among the preliminary diagnoses mentioned in the literature, the following were considered: fibroma, fibrous histiocytoma, granuloma, cyst, ameloblastama, fibrous dysplasia, and sarcoma. Our case indicates how a misdiagnosis of a fibroma resulted in recurrence after incomplete removal. The precise diagnosis becomes confusing because of a multitude of diagnostic terms. In 1954, Arthur F’urdy Stout reviewed the subject of juvenile fibromatosis. He found that it “was in such a state of confusion that he wondered if there was anything at all to be gained by retaining the older names.“34 He mentioned various forms of generalized and local fibromatosis but included differentiated nonmetastasizing fibrosarcomas in the term jibromatosis. Jaffe,35 in 1958, introduced the term desmoplastic jibroma, not to be confused with desmoid fibromatosis, a lesion of a similar dense fibrous nature occurring in the abdominal wall. He stated that the qualifying term desmoplastic was introduced to clearly distinguish this bone lesion from nonossifying Jibroma of bone and from chondromyxoid jibroma of bone, two lesions that also include the term fibroma. Stout36 reviewed the subject again in 1962 in a study of 241 cases of juvenile fibromatosis. He attempted to distinguish between juvenile fibromatosis and fibrosarcoma and to determine those that were potentially metastatic. He stated that “it must be confessed that the study has failed in both attempts.” Wilkins and Waldron6 in 1975, suggested that the title aggressivefibromatosis was a more appropriate term, reflecting the invasive characteristics of the disease. Synonyms listed were extra-abdominal desmoid, juvenile jibromatosis, aggressive infantile jibromatosis. and congenital jibrosarcoma. Addante and Laskin,37 returning to Jaffe’s term of desmoplastic fibroma, proposed that this diagnosis be restricted to lesions that arise from bone whereas those that arise from the adjacent tissues and then invade bone be termed aggressive jibromatoses.
of the maxilla
425
II. Features of 13 cases of aggressive fibromatosis
Table
Case
Follow-up h-4
1 2 3 4 5 6 I 8 9 10
38
48 lost 3 9
II 12 13
27 12 36
24 14 12
Recurrence
Complaints Bulging
mass Swelling, vague pain Swelling Pain ulceration Swelling Swelling Swelling Facial pain Anesthesia, nasal stuffiness, swelling
No No No Yes No Yes No Yes No No No No
Swelling Swelling
Histologically, they consist of well-differentiated fibrous tissue that infiltrates neighboring structures. It is generally accepted that soft tissue fibromatoses arise from musculoaponeurotic structures.t2 They are considered to be benign reactive lesions of unknown cause, although trauma” 9, 25,27and hormonal imbalance have been suggested.2 They can be locally invasive and tend to recur after surgical resection. They can be difficult to differentiate from low-grade fibrosarcoma histologically and, therefore in some cases, only the clinical course will give the correct diagnosis. SUMMARY
A case of peripheral aggressive fibromatosis of the left maxilla is reported. The disease was first noted when a painless mass was observed in the hard palate of a ICyear-old white girl. The benign appearance of the tissues when examined microscopically led to an initial diagnosis of fibroma. The rapid recurrence of the lesion led to the final diagnosis. The patient is free of disease 36 months later. A review of the literature indicates a total of 73 cases of aggressive fibromatosis or desmoplastic fibroma of the jaws. If one accepts aggressiveJibromatosis and desmoplastic jibroma as synonyms, a total of 13 cases, including this case, have been reported in the maxilla.
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