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Central fibromyxoma of the maxilla
CENTRAL
FIBROMYXOMA
OF THE MAXILLA
KARL 1%‘.BRUCE, D.D.S., M.S., AND R. QUES-TIN ROYER, D.D.S., M.S. ROCHESTER, $11~~.
ENTRAL fibromyxoma of the jaws does not occur with great frequency. tumor is composed of mucous connective tissue which is similar trI Wharton’s jelly and the embryonic mesenchyme. Fihromysoma of the jaw appears to have a better prognosis than does fibromyxoma occurring in the long bones of the skeleton. In the long bones the tumor is frequently malignant and tends to recur with great frequency after removal. A fibromyxoma of the jaw is more indolent than those of long bones, and recurrence is not frequent if enucleation is complete. In 1921, Muller’ suggested that central fibromas of the jaw might originate from the mesenchymal portion of the tooth germ. (lonversely, Trauner.” in 1942, maintained that convincing evidence which would link fibromi1.s of the jaw to a dental origin is not available. Ewing3 expressed the belief that mesoblastic tumors such as fibromas anrl lipomas oft,en undergo myxomatous change under the influence of cht80tlicB irritation or degenerative change from diminished blood supply. Willis4 pointed out that this group of tumors probably arises from nerve’ sheaths. Thoma” expressed the belief that the greater portion of myxomas OCCUI*ring in the jaw are derived from embryonic tissue of the dentinal papilla, t.he Some of these t,nmors are dcdental follicle, or the periodontal membrane. rived from retained islands of embryonic undifferentiated tissue which have undergone hyperplasia. This tissue was predestined to form bone but never surpassed a state of differentiation comparable to that of Whartou’s jelly seen in the umbilical cord. Thoma further related that it is difficult t)o differentiate a tumor which develops from an odontogenic source from one whicdh has an osteogenic inception. A clinical m&hod of differentiation was adopted by him. He observed that osteogenic myromas occur in the jaw dissociated i’rom the tooth-hearing areas and are more malignant in behaviot*. 7‘ilp odontogenic type occurs in rather intimate association with the dental system. is benign and seldom recurs if completely rnucleated. Evidence that map support the odontogenic origin of fibromyxoma of I ill jaw was contributed by Thorna in 1934. l”uste and Mena Serra’ in 1931. StraithH in 1942. Millhon and Parkhill” in 1946, Thoma and Goldman’” in 1!)47. Stafne and Parkhill’ in 194$, and EdwardsI’ in 1949. It, has been recognized that the tumor is frequently associated with missirig unerupted or partially formed teeth. Thomn :~nd Goldman, reporting on 11
C The
From the Section of Dentistry. Mayo Clinic. IZii
1279
KARL
IV.
BRITCE
ASD B. BURNTIN
ROYER
cases of myxoma of the jaw, observed that in all but 1 case the tumor was associated with one or more missing or embedded teeth, Fibromyxoma of the jaw is a tumor which occurs in the younger age groups. The tumor appears in the second and third decades most frequently. It affects both jaws, with the mandible being most often involved. The tumor is usually report,ed to be unaccompanied by pain ; however, Thoma and Goldman stated that intense, persistent pain is the chief complaint. Sonesson13 reported 8 cases of central fibromyxoma of the jaw and related that none of these patients registered pain as a complaint. In the majority of cases of fibromysoma reported in the literature there was a history that a tumor had been present for a few months to a year prior to initial examination. In a few cases the tumor was reported to have been present for a longer period. Harbert and co-workers14 reported a case of myxoma of the maxilla and antrum of thirteen years’ duration. There is no typical roentgenographic appearance of fibromyxoma of the jaw. This tumor has been noted to simulat,e polycystic ameloblastoma as well as odontogenic cyst,. The most frequently observed roentgenographic picture is a radiolucent area with scalloped, irregular margins. The central portion is traversed by fine, gracile, straight or angu1a.r trabeculations. The tumor expands the jaw and often causes complete destruction of the cortex. The surgical treatment should consist of complete enucleation or radical excision if possiblse. Resection may be necessary in some cases in which the tumor has completely destroyed a portion of the jaw. NTawro and ReedI’ have described 2 cases of fibromyxoma of the jaws in which wide resection was performed in an effort to irradicate the tumor. Roentgen therapy is generally agreed to be ineffective in the treatment of this tumor. Thomas has stated that recurrence of fibromyxoma is uncommon if enucleation is complete. In Sonesson’s series, the tumor recurred in 4 of 8 cases. One tumor recurred once, 1 recurred twice, and 2 recurred three times after operation. Grossly the tumor is grayish yellow. Its consistency varies. Some portions of the tumor may be sticky, gelatinous, or semisolid, and others may be firmer. The surface of the tumor is shiny and glistening. Histologically the fusiform or stellate cells are elongated, having cytoplasmic processes stretching in various directions. Fine collagen fibers can be seen extending between the aforedescribed cells. The nuclei, which are slender and threadlike, are hyperchromatic, but they vary little in size and shape. Mitotic figures are rarely seen. The tumor is well supplied with fine vessels whose walls have a single layer of endothelium. Plasma cells, lymphocytes, and large mononuclear cells are sometimes present. The report of a case of fibromyxoma of the left maxilla follows.
Case Report A 14-year-old white boy was admitted to the Mayo Clinic on Dec. 9, 1950, with the chief complaint of enlargement of the labial alveolus in the maxillary left canine region. Two months previously, the patient The mass was painless and of three months’ duration. had consulted his local dentist because of this swelling and failure of the permanent
CEN![‘RKL
FIBROMYSOMA
I “Y!’
OF RI.\SII.I>.\
maxillary left canine to erupt. The deciduous m;~sillary left cauine had been lost i\\-st,ic lesion iu this region. The Illaxillar~ csninc~ tooth was ~)resent IJII: had not yet erupted. Clinical examination on l)ec. 9, l!)W, revealed a lirnt bull)ous mass, 2.5 i)y 3 cn~.. situated on the iabial aspect of the maxillary alveolus in the left canine region (Fig. 1). The maxillary left canine tooth was partially erupted and t,here was an abnormally large space situated between this tooth and the lateral incisor tooth. The floor of the nose on the left was slightly elevated. The alveolar enlargement was neither painful nor tender The expansive mass yielded to finger pressure but no crepitation was noted. to palpation. Light passed through the tumor on transillumination. The lateral incisor and the firrr premolar tooth adjacent to the lesion reacted negatively to ice vitality tests. The left The czlirrical examination suggested ths.1 canine, however, reacted positively to this test. the tumor was a dentigerous or globulomaxillary cyst.
Fig.
l.-Expansive
growth
in the maxillary left canine region, ttw process of eruption.
The maxillary
canine, is i!i
Roentgenographic examination revealed a radiolucent area with fine angular tra beculations which extended from the maxillary left central incisor to the maxillary let’? second premolar (Fig. 2, a). This exposure. \ras indicative of the fact that the lesion proI,ably was not a cyst of the globulomaxillary suture. An occlusal roentgenogram of the left maxilla also was made to identify further the limits of the lesion (Fig. 8, 7A. The margin of the radiolucent area was undeterminate. The tumor was enucleated by surgical curettage with the patient under intratrachrat anesthesia. The gross specimen appeared as a yellowish, pinkish gray, semisolid, shirk!; gelatinous mass. There were, however, some areas of firm consistency (Fig. 2, L’J. 7’htX tumor was found to extend from the midline to the left first molar region. The floor of the nose and the antrum on the homolateral side were perforated. The excised tumor measure4 3 by 2 cm. On microscopic examination the tissue was ol~nerved to be composed of widely spacetl spindle-shaped and stellate-shaped cells, with long eytoplasmic processes (Fig. 3 1. A I though the nuclei of the cells were somewhat variable in size and shape there was n*) evidence of mitosis. Interspersed between the cellular elements was a reticulum nf sparse, fine, fibrillar material. However, areas were present. in which the retirulum was compose~l of denser collagen strands. The space between the cells and Jillrils was ocru7~ietl 1)~ a 1u111~-
1280
KARL
staining vacuolated capillaries. Round zones of the tumor.
\\r.
RRTJCE
AKD
R. QUENTIN
ROYER
mucoid substance. The tissue was fairly well supplied with blood cells of the lpmphocytic and monocytic varieties were present in some
Fig. Z.--a, Dental roentgenograms revealing a radiolucent area extending from the maxillary left central incisor to the maxillary left second premolar. Fine angular trabeculations are present. The roots of the lateral incisor and the canine tooth are displaced: b, lesion demonstrated on an occlusal roentgenogram of the left maxilla: c, excised flbromyxoma measuring 3 by 2 cm. In a the view is from the lingual side while in b it is from the facial side.
Fig.
3.-Fibt’omyxoma
of
the
maxilla
(hematoxylin
and
eosin,
X100).
CENTRAL
PIBROMYXOMA
OF M.I\XILI..2
Comment It is interesting to speculate as to the genesis of this tumor. It, may have originated in the primitive mesenchymal tissue of bone or odontogenic tissue. It is doubtful that this growth represents a degeneratire process in a previously existing central fibroma of bone. In the evaluation of the possibilities of odontogenic origin the fact that the tumor arose in just,aposition to a11 At the time that the tumor was firsi unerupted tooth must be considered. observed at the Mayo Clinic, however, the left canine had begun to erupt and as a result no definite association could be made between the tumor and t,br follicle of this tooth. It seems unlikely that the tumor developed from a dentinal pa,pilla because the root apices of the teeth in close approximation t,o th(a tumor were well formed. The hypothesis that the tumor had its inception in the periodontal membrane of one of the adjacent teeth cannot be suhstantiated. It is hypothetically possible that the proliferation of mesenchymal rests sit,uated in the alveolar bone of this region could hare accounted for the tumor. Because the roots of the canine and lateral incisor teeth were widely separated it would seem reasonable to assume that the globulomaxillary suture zone may have been the primary site of the growth. If it is possible for epithelial remnants to be located at this site and later proliferate and degenwate to form the so-called globulomaxillary cyst, it would seem just as plausible that primiti\-e embryonic mesentrhymal rests might also frequent, this srrtnre rreiol; and could proliferate to form a tumor such as has hecu descriljed.
References 1. Muller, W.: Quoted by Sonesson, Anders.13 2. Trauner, R.: Quoted by Sonesson, Anders.13 3. Ewing, James: Neoplastic Diseases; a Treatise on Tumors, rd. 4: Philadelphia, 1940, W. B. Saunders Company, 1,160 pp. of Tumors, St. Louis, 1948, The C. V. Mosby Company, 992 pp. 4. Willis, R. A.: Pathology Oral Pathology; a Histological, Roentgenological, and Clinical Stud1 5. Thoma, K. H.: of the Diseases of the Teeth, Jaws, and Mouth, ed. 3, St. Louis, 1950, The (1. T’. ;Mosbr Company! 1,592 pp. Clinical Pathology of Jaws With a Histologic and Roentgen Study of 6. Thoma, K: H.: Practical Cases, Springfield, Ill., 1934, Charles C Thomas, Publisher, 643 pp. Mixoma puro prirnitiro de1 seno mnxilar, Rot. Idip:, 7. Fuste, F., and Mena Serra, R.: contra el c&ncer 16: 19-23, 1941. Myxomatous Degeneration of a Follieular Odontoma, Am. .I. Orthn8. Straith, F. E.: dontics and Oral Surg. (Oral Surg. Sect.) 28: 540-542, 1942. 9. Millhon, J. A., and Parkhill, Edith &I.: A Myxomatous Tumor Simulating a TJWI tigerous Cyst, J. Oral Surg. 4: 129-132, 1946. Central Myxoma of the .Jaw, Am. J. Orthodontic10. Thoma, K. H., and Goldman, H. M.: and Oral Burg. (Oral Surg. Sect.) 33: 532-540, 1947. Myxomatous Tumor Associated With an TJnIl. Stafne, E. C., and Parkhill, Edith M.: erupted Tooth; Report of a Case, Am. .T. Orthodontics and Oral Surg. (Oral Surf. Sect.) 33: 597-598, 1947. Mpxoma of the Maxilla: Report of Case, J. Oral Surg. 7: 167.Ifi!). 12. Edwards, R. W.: 1949.
13. Sonesson, Anders: Odontogenic Cysts and Cystic Tumors of the Jaws; a RoentgenDiagnostic and Patho-anatomic Study, Acta radiol. (Supp.) 81: 104-114! 1950. 14. Harbert, Fred, Gerry, R. G., and Dimmette, R. M.: Myxoma of the Maxilla, 0~1 SURG., ORAL MED., AND ORAL PATH. 2: 1414-1421, 1949. Fibromyxoma of the Mandible; R,eport of Two Cases. 15. Wawro, N. W., and Reed, John: Ann. Surg. 132: 1138-1143, 1950.
FIBROMYXOMA
OF THE MAXILLA
KARL 1%‘.BRUCE, D.D.S., M.S., AND R. QUES-TIN ROYER, D.D.S., M.S. ROCHESTER, $11~~.
ENTRAL fibromyxoma of the jaws does not occur with great frequency. tumor is composed of mucous connective tissue which is similar trI Wharton’s jelly and the embryonic mesenchyme. Fihromysoma of the jaw appears to have a better prognosis than does fibromyxoma occurring in the long bones of the skeleton. In the long bones the tumor is frequently malignant and tends to recur with great frequency after removal. A fibromyxoma of the jaw is more indolent than those of long bones, and recurrence is not frequent if enucleation is complete. In 1921, Muller’ suggested that central fibromas of the jaw might originate from the mesenchymal portion of the tooth germ. (lonversely, Trauner.” in 1942, maintained that convincing evidence which would link fibromi1.s of the jaw to a dental origin is not available. Ewing3 expressed the belief that mesoblastic tumors such as fibromas anrl lipomas oft,en undergo myxomatous change under the influence of cht80tlicB irritation or degenerative change from diminished blood supply. Willis4 pointed out that this group of tumors probably arises from nerve’ sheaths. Thoma” expressed the belief that the greater portion of myxomas OCCUI*ring in the jaw are derived from embryonic tissue of the dentinal papilla, t.he Some of these t,nmors are dcdental follicle, or the periodontal membrane. rived from retained islands of embryonic undifferentiated tissue which have undergone hyperplasia. This tissue was predestined to form bone but never surpassed a state of differentiation comparable to that of Whartou’s jelly seen in the umbilical cord. Thoma further related that it is difficult t)o differentiate a tumor which develops from an odontogenic source from one whicdh has an osteogenic inception. A clinical m&hod of differentiation was adopted by him. He observed that osteogenic myromas occur in the jaw dissociated i’rom the tooth-hearing areas and are more malignant in behaviot*. 7‘ilp odontogenic type occurs in rather intimate association with the dental system. is benign and seldom recurs if completely rnucleated. Evidence that map support the odontogenic origin of fibromyxoma of I ill jaw was contributed by Thorna in 1934. l”uste and Mena Serra’ in 1931. StraithH in 1942. Millhon and Parkhill” in 1946, Thoma and Goldman’” in 1!)47. Stafne and Parkhill’ in 194$, and EdwardsI’ in 1949. It, has been recognized that the tumor is frequently associated with missirig unerupted or partially formed teeth. Thomn :~nd Goldman, reporting on 11
C The
From the Section of Dentistry. Mayo Clinic. IZii
1279
KARL
IV.
BRITCE
ASD B. BURNTIN
ROYER
cases of myxoma of the jaw, observed that in all but 1 case the tumor was associated with one or more missing or embedded teeth, Fibromyxoma of the jaw is a tumor which occurs in the younger age groups. The tumor appears in the second and third decades most frequently. It affects both jaws, with the mandible being most often involved. The tumor is usually report,ed to be unaccompanied by pain ; however, Thoma and Goldman stated that intense, persistent pain is the chief complaint. Sonesson13 reported 8 cases of central fibromyxoma of the jaw and related that none of these patients registered pain as a complaint. In the majority of cases of fibromysoma reported in the literature there was a history that a tumor had been present for a few months to a year prior to initial examination. In a few cases the tumor was reported to have been present for a longer period. Harbert and co-workers14 reported a case of myxoma of the maxilla and antrum of thirteen years’ duration. There is no typical roentgenographic appearance of fibromyxoma of the jaw. This tumor has been noted to simulat,e polycystic ameloblastoma as well as odontogenic cyst,. The most frequently observed roentgenographic picture is a radiolucent area with scalloped, irregular margins. The central portion is traversed by fine, gracile, straight or angu1a.r trabeculations. The tumor expands the jaw and often causes complete destruction of the cortex. The surgical treatment should consist of complete enucleation or radical excision if possiblse. Resection may be necessary in some cases in which the tumor has completely destroyed a portion of the jaw. NTawro and ReedI’ have described 2 cases of fibromyxoma of the jaws in which wide resection was performed in an effort to irradicate the tumor. Roentgen therapy is generally agreed to be ineffective in the treatment of this tumor. Thomas has stated that recurrence of fibromyxoma is uncommon if enucleation is complete. In Sonesson’s series, the tumor recurred in 4 of 8 cases. One tumor recurred once, 1 recurred twice, and 2 recurred three times after operation. Grossly the tumor is grayish yellow. Its consistency varies. Some portions of the tumor may be sticky, gelatinous, or semisolid, and others may be firmer. The surface of the tumor is shiny and glistening. Histologically the fusiform or stellate cells are elongated, having cytoplasmic processes stretching in various directions. Fine collagen fibers can be seen extending between the aforedescribed cells. The nuclei, which are slender and threadlike, are hyperchromatic, but they vary little in size and shape. Mitotic figures are rarely seen. The tumor is well supplied with fine vessels whose walls have a single layer of endothelium. Plasma cells, lymphocytes, and large mononuclear cells are sometimes present. The report of a case of fibromyxoma of the left maxilla follows.
Case Report A 14-year-old white boy was admitted to the Mayo Clinic on Dec. 9, 1950, with the chief complaint of enlargement of the labial alveolus in the maxillary left canine region. Two months previously, the patient The mass was painless and of three months’ duration. had consulted his local dentist because of this swelling and failure of the permanent
CEN![‘RKL
FIBROMYSOMA
I “Y!’
OF RI.\SII.I>.\
maxillary left canine to erupt. The deciduous m;~sillary left cauine had been lost i\\
Fig.
l.-Expansive
growth
in the maxillary left canine region, ttw process of eruption.
The maxillary
canine, is i!i
Roentgenographic examination revealed a radiolucent area with fine angular tra beculations which extended from the maxillary left central incisor to the maxillary let’? second premolar (Fig. 2, a). This exposure. \ras indicative of the fact that the lesion proI,ably was not a cyst of the globulomaxillary suture. An occlusal roentgenogram of the left maxilla also was made to identify further the limits of the lesion (Fig. 8, 7A. The margin of the radiolucent area was undeterminate. The tumor was enucleated by surgical curettage with the patient under intratrachrat anesthesia. The gross specimen appeared as a yellowish, pinkish gray, semisolid, shirk!; gelatinous mass. There were, however, some areas of firm consistency (Fig. 2, L’J. 7’htX tumor was found to extend from the midline to the left first molar region. The floor of the nose and the antrum on the homolateral side were perforated. The excised tumor measure4 3 by 2 cm. On microscopic examination the tissue was ol~nerved to be composed of widely spacetl spindle-shaped and stellate-shaped cells, with long eytoplasmic processes (Fig. 3 1. A I though the nuclei of the cells were somewhat variable in size and shape there was n*) evidence of mitosis. Interspersed between the cellular elements was a reticulum nf sparse, fine, fibrillar material. However, areas were present. in which the retirulum was compose~l of denser collagen strands. The space between the cells and Jillrils was ocru7~ietl 1)~ a 1u111~-
1280
KARL
staining vacuolated capillaries. Round zones of the tumor.
\\r.
RRTJCE
AKD
R. QUENTIN
ROYER
mucoid substance. The tissue was fairly well supplied with blood cells of the lpmphocytic and monocytic varieties were present in some
Fig. Z.--a, Dental roentgenograms revealing a radiolucent area extending from the maxillary left central incisor to the maxillary left second premolar. Fine angular trabeculations are present. The roots of the lateral incisor and the canine tooth are displaced: b, lesion demonstrated on an occlusal roentgenogram of the left maxilla: c, excised flbromyxoma measuring 3 by 2 cm. In a the view is from the lingual side while in b it is from the facial side.
Fig.
3.-Fibt’omyxoma
of
the
maxilla
(hematoxylin
and
eosin,
X100).
CENTRAL
PIBROMYXOMA
OF M.I\XILI..2
Comment It is interesting to speculate as to the genesis of this tumor. It, may have originated in the primitive mesenchymal tissue of bone or odontogenic tissue. It is doubtful that this growth represents a degeneratire process in a previously existing central fibroma of bone. In the evaluation of the possibilities of odontogenic origin the fact that the tumor arose in just,aposition to a11 At the time that the tumor was firsi unerupted tooth must be considered. observed at the Mayo Clinic, however, the left canine had begun to erupt and as a result no definite association could be made between the tumor and t,br follicle of this tooth. It seems unlikely that the tumor developed from a dentinal pa,pilla because the root apices of the teeth in close approximation t,o th(a tumor were well formed. The hypothesis that the tumor had its inception in the periodontal membrane of one of the adjacent teeth cannot be suhstantiated. It is hypothetically possible that the proliferation of mesenchymal rests sit,uated in the alveolar bone of this region could hare accounted for the tumor. Because the roots of the canine and lateral incisor teeth were widely separated it would seem reasonable to assume that the globulomaxillary suture zone may have been the primary site of the growth. If it is possible for epithelial remnants to be located at this site and later proliferate and degenwate to form the so-called globulomaxillary cyst, it would seem just as plausible that primiti\-e embryonic mesentrhymal rests might also frequent, this srrtnre rreiol; and could proliferate to form a tumor such as has hecu descriljed.
References 1. Muller, W.: Quoted by Sonesson, Anders.13 2. Trauner, R.: Quoted by Sonesson, Anders.13 3. Ewing, James: Neoplastic Diseases; a Treatise on Tumors, rd. 4: Philadelphia, 1940, W. B. Saunders Company, 1,160 pp. of Tumors, St. Louis, 1948, The C. V. Mosby Company, 992 pp. 4. Willis, R. A.: Pathology Oral Pathology; a Histological, Roentgenological, and Clinical Stud1 5. Thoma, K. H.: of the Diseases of the Teeth, Jaws, and Mouth, ed. 3, St. Louis, 1950, The (1. T’. ;Mosbr Company! 1,592 pp. Clinical Pathology of Jaws With a Histologic and Roentgen Study of 6. Thoma, K: H.: Practical Cases, Springfield, Ill., 1934, Charles C Thomas, Publisher, 643 pp. Mixoma puro prirnitiro de1 seno mnxilar, Rot. Idip:, 7. Fuste, F., and Mena Serra, R.: contra el c&ncer 16: 19-23, 1941. Myxomatous Degeneration of a Follieular Odontoma, Am. .I. Orthn8. Straith, F. E.: dontics and Oral Surg. (Oral Surg. Sect.) 28: 540-542, 1942. 9. Millhon, J. A., and Parkhill, Edith &I.: A Myxomatous Tumor Simulating a TJWI tigerous Cyst, J. Oral Surg. 4: 129-132, 1946. Central Myxoma of the .Jaw, Am. J. Orthodontic10. Thoma, K. H., and Goldman, H. M.: and Oral Burg. (Oral Surg. Sect.) 33: 532-540, 1947. Myxomatous Tumor Associated With an TJnIl. Stafne, E. C., and Parkhill, Edith M.: erupted Tooth; Report of a Case, Am. .T. Orthodontics and Oral Surg. (Oral Surf. Sect.) 33: 597-598, 1947. Mpxoma of the Maxilla: Report of Case, J. Oral Surg. 7: 167.Ifi!). 12. Edwards, R. W.: 1949.
13. Sonesson, Anders: Odontogenic Cysts and Cystic Tumors of the Jaws; a RoentgenDiagnostic and Patho-anatomic Study, Acta radiol. (Supp.) 81: 104-114! 1950. 14. Harbert, Fred, Gerry, R. G., and Dimmette, R. M.: Myxoma of the Maxilla, 0~1 SURG., ORAL MED., AND ORAL PATH. 2: 1414-1421, 1949. Fibromyxoma of the Mandible; R,eport of Two Cases. 15. Wawro, N. W., and Reed, John: Ann. Surg. 132: 1138-1143, 1950.