652
LEIOMYOSARCOMA
12. Kember N: Cell division in endochondral ossification. A study of cell proliferation by the method of tritiated thymidine autoradiography. J Bone Joint Surg 42B824, lY60 13. Shuker S: Spontaneous regeneration of the mandible in a child. J Maxillofac Surg 13:70, 1985 14. Kazanjian V: Spontaneous regeneration of bone following excision of section of mandible. Am J Orthod 34:242, 1948 15. Nwoku A: Unusually rapid bone regeneration following mandibular resection, J Maxillofac Surg 8:309, 1980
J Oral Maxillofac 49:652-655.
OF THE MANDIBLE
16. Nagase M, Ueda K, Suzuki I, et al: Spontaneous regeneration of the condyle following hemimandibulectomy by disarticulation. J Oral Maxillofac Surg 43:218, 1985 17. Adekeye E: Rapid bone regeneration subsequent to subtotal mandibulectomy. Oral Med Oral Surg Oral Path01 44:521, 1977 18. Elbeshir E: Spontaneous regeneration of the mandibular bone following hemimandibulectomy. Br J Oral Maxillofat Surg 28:128, 1990
Surg
1991
Leiomyosarcoma
of the Mandible:
A Case Report VEJAYAN KRISHNAN, BDS, MS,* CHARLES M. MIYAJI, DMD,t AND ELGENE G. MAINOUS, DDSS Smooth muscle is the prevalent tissue in the gastrointestinal and the genitourinary systems, but it is present in limited amount in the oral cavity. This may account for the low occurrence of smooth muscle neoplasms in the latter region. This article reports a primary, central leiomyosarcoma in the mandible. Report of Case A 27-year-old white man was seen at the Oral Surgery Clinic of the University of Minnesota on January 31, 1989. His chief complaints were pain and swelling in the right anterior mandible, first noticed in June 1988. The pain was localized to the region and was intermittent at first but was continuous for the past 3 months. He had also noticed loosening of the teeth in the region, but reported no altered sensation in his lower lip or chin. A general dentist, who had taken periapical radiographs, had been seen 3 months previously (Fig 1). A diagnosis of advanced chronic periodontitis was made and periodontal surgery was subsequently performed. However, the patient experienced no relief from his symptoms.
The patient’s past medical history was unremarkable with the exception of a history of drug abuse for the past 10 years. Clinical examination showed the presence of an exophytic lesion on the right parasymphysis of the mandible. The surface mucosa was intact but irregular. Buccolingual expansion was observed in the alveolar segment from the lower right lateral incisor to the right first molar. These teeth exhibited 2 + mobility. The area was tender to palpation. Regional lymph nodes were not palpable. A panoramic radiograph showed an ill-defined, diffuse radiolucency in the symphysis and right body of the mandible (Fig 21, measuring 6 cm in length and spanning the height of the mandible from the alveolar crest to 1 cm short of the inferior border. An incisional biopsy was performed under local anesthesia on January 31, 1989. The lesion was found to be firmly adherent to the overlying mucosa, making it difficult to raise a mucoperiosteal flap. The lesion was whit-
Received from the Department of Oral and Maxillofacial Surgery, University of Minnesota, Minneapolis. * Formerly Clinical Fellow; currently, Resident, Oral and Maxillofacial Surgery, University of Texas Health Science Center at Houston. t Resident. $ Formerly, Chairman; currently Chairman, Oral and Maxillofacial Surgery, University of Texas Health Science Center at Galveston. Address correspondence and reprint requests to Dr Krishnan: Oral and Maxillofacial Surgery, the University of Texas Health Science Center at Houston, 6515 John Freeman Ave. Texas Medical Center, PO Box 20068, Houston, TX 77225. 0 1991 American
Association
geons 0278-2391
I91 /4906-0017$3.00/O
of Oral
and Maxillofacial
Sur-
FIGURE 1. Periapical radiograph in the right parasymphysis of the mandible (September 1988). The radiograph shows an illdefined radiolucency and total loss of bony support for the bicuspid teeth.
KRISHNAN ET AL
653
FIGURE 2. Panoramic radiograph (January 1989)showing the radiolucent lesion in the symphysis and right parasymphysis of the mandible.
ish, multinodular, and relatively avascular. It appeared to have perforated the buccal cortical plate. Tissue specimens were obtained from the depth of the lesion and from the surface, including the overlying mucosa. The specimens were submitted for histologic examination. Hematoxylin and eosin-stained sections revealed the presence of a spindle cell neoplasm that had totally replaced the connective tissue underlying the surface epithelium (Fig 3). The cells exhibited a storiform arrangement (Fig 4). Mitotic tigures (approximately one per highpower field) were readily evident (Fig 5). In the depth of the lesion, the cells appeared more histiocytic and less spindle-shaped. Multinucleated giant cells were also evident. Necrotic areas were noted throughout the lesion. Based on these findings, a diagnosis of malignant fibrous histiocytoma was made pending immunohistochemical investigation. The patient was taken to the operating room on February 6, 1989. A right functional neck dissection was performed with removal of the right submandibular salivary gland and the regional lymph nodes. The mandible was resected from the left first premolar to the right second molar. The resected bone and soft tissue were macroscopically free of tumor. Frozen sections also showed the
FIGURE 3. Photomicrograph of the surface of the lesion showing an intact surface epithelium with the tumor having replaced the underlying connective tissue (hematoxylin-eosin, original magnification x10).
resected margins and the nodes to be free of tumor. A Synthes reconstruction plate (Synthes Cot-p, Monument. CO) was used for primary reconstruction of the resected mandible (Fig 6). The patient’s immediate postoperative course was uneventful. Immunohistochemical studies were performed on the original biopsy specimens and the surgical specimens. These studies showed that the tumor cells were reactive for vimentin and muscle-specific actin. In addition, the cells were also reactive for desmin. In concert with the light microscopy findings, a final diagnosis of leiomyosarcoma grade II/III was made. However. this diagnosis would not have changed the treatment, as both the malignant fibrous histiocytoma and the leiomyosarcoma are managed with surgery and adjunctive chemotherapy and1 or radiotherapy. The patient underwent reconstruction with a deltopectoral rotational flap in November 1989. In January 1990, he presented with right cervical nodes and a lung metastasis. At that time a right radical neck dissection was performed. Chemotherapy was started in March 1990. In July 1990, the patient developed altered mental status. Computed tomography scan showed the presence of a right temporal lobe and a mediastinal mass. The patient’s
654
LEIOMYOSARCOMA
OF THE MANDIBLE
FIGURE 4. Low-power photomicrograph demonstrating the storiform arrangement of the spindle-cell neoplasm. Areas of necrosis (N) are ako evident in this section. Multinucleated giant cells (arrow) were scattered throughout the lesion (hematoxylin-eosin, original magnification X40).
prognosis currently astatic disease.
remains
very poor in light of his met-
Discussion Smooth-muscle neoplasms in the oral cavity are rare. A review of the literature by Poon et al’ showed that there have been only 25 reported cases of primary oral leiomyosarcoma. Of these, there appeared to be no specific site predilection in the oral cavity. There is a predilection for males, with the male-to-female ratio being reported as 3 to 1.2,3 The age of occurrence ranges from 1 to 88 years. Some tissues in the oral cavity have been impli-
cated as the origin of this neoplasm: the circumvallate papilla of the tongue,4,5 the undifferentiated mesenchyme,6,7 and the smooth-muscle elements in blood vessel walls.4V8,9In this particular instance we believe the tumor originated either from the neurovascular bundle or from the undifferentiated mesenchymal elements in the region. Leiomyosarcomas pose a diagnostic challenge both clinically and microscopically. Loosening of the teeth in the region has led to a mistaken diagnosis of advanced periodontitis and subsequent mismanagement of patients.“*” At the light microscopic level this neoplasm can be confused with
FIGURE 5. High-power photomicrograph showing an admixture of spindle-shaped cells and histiocytic cells with hyperchromatism, increased nuclear:cytoplasmic ratio, and abnormal mitotic figures (hematoxylin-eosin, original magnification X200).
KRISHNAN
ET AL
655
FIGURE 6. Panoramic radiograph (February 1989) demonstrating the resected area of the mandible with the Synthes reconstruction plate in place.
other lesions, especially when cells are undifferentiated or pleomorphic.‘2.‘3 The literature has also reported an instance of initial misdiagnosis of a malignant fibrous histiocytoma based on the histologic presentation.13 The role that immunohistochemistry plays in the microscopic diagnosis of this neoplasm cannot be overstated. The immunohistochemical reactivity of the tissue to desmin has been demonstrated to be specific for leiomyosarcomas and 13-15Two features serve to difrhabdomyosarcomas. ferentiate the two: the absence of striated-muscle differentiation in leiomyosarcoma and the reactivity to actin by leiomyosarcoma and to myoglobin by rhabdomyosarcoma. l6 Wide surgical excision remains the mainstay of treatment, with irradiation and chemotherapy as adjunctive treatments.’ The prognosis for this neoplasm is guarded. A 50% recurrence and metastatic rate has been reported, with a mortality of 40%.339 Oral leiomyosarcomas appear to metastasize to the cervical lymph nodes and lungs with equal frequency, whereas tumors in other sites metastasize to the lung and liver.’ Because of the relatively high mortality and recurrence rate, a long-term followup of the patient is mandatory. Further adjunctive therapy and surgical management may be necessary.
References 1. Poon CK, Kwan PC, Yin NT, et al: Leiomyosarcoma of gingiva: Report of a case and review of the literature. J Oral Maxillofac Surg 45888, 1987
2. Bass B, Archard H, Sussman R, et al: Expansile radiolucent lesion of the mandible. J Oral Maxillofac Surg 44:799. 1986 3. Abdin HA, Prabhu SR: Leiomyosarcoma of mandible in a Sudanese female. Int J Oral Surg 14:85, 1985 4. Stout AP: Leiomyoma of the oral cavity. Am J Cancer 34:31, 1938 5. Garret JR: Angiomyoma of the palate: report of a case. Oral Surg 27:103. 1969 6. Cheek JH, Nickey WM: Leiomyosarcoma of venous origin. Arch Surg 90:396. 1965 7. Kratochvil FJ. MacGregor SD, Budnick SD, et al: Leiomyosarcoma of the maxilla. Report of a case and review of the literature. Oral Surg 54647, 1982 8. Farman AG, Kay S: Oral leiomyosarcoma: Report of a case and review of the literature pertaining to smooth muscle tumors of the oral cavity. J Oral Surg 43402, 1977 9. Weitzner S: Leiomyosarcoma of the anterior maxillary alveolar ridge. Oral Surg 50:62, 1980 IO. Phillips H, Brown A: Leiomyosarcoma: Report of a case. J Oral Surg 29:194, 1971 11. Mindell RS, Calcaterra TC, Ward PH: Leiomyosarcoma of the head and neck: A review of the literature and report two cases. Laryngoscope 85904, 1975 12. Nishi M. Mimura T, Senba I: Leiomyosarcoma of the maxilla. J Oral Maxillofac Surg 4564, 1987 13. Miettinen M, Lehto V-P, Ekblom P, et al: Leiomyosarcoma of the mandible; diagnosis as aided by immunohistochemical demonstration of desmin and laminin. J Oral Path01 13:373, 1984 14. Gabianni FN, Kapanci Y. Barazzone P, et al: Immunochemical identification of intermediate-sized filaments in human neoplastic cells. A diagnostic aid for the surgical pathologist. Am J Path01 104:206. 1981 15. Denk H, Krepler R, Artlieb U, et al: Proteins of intermediate filaments. An immunohistochemical and biochemical approach to the classification of soft-tissue tumors. Am J Pathol 110:193. 1983 16. Corson J, Pinkus GS: Intracellular myoglobin-A specific marker for skeletal muscle differentiation in soft tissue sarcomas. An immunoperoxidase study. Am J Path01 103:384. 1981
LEIOMYOSARCOMA
12. Kember N: Cell division in endochondral ossification. A study of cell proliferation by the method of tritiated thymidine autoradiography. J Bone Joint Surg 42B824, lY60 13. Shuker S: Spontaneous regeneration of the mandible in a child. J Maxillofac Surg 13:70, 1985 14. Kazanjian V: Spontaneous regeneration of bone following excision of section of mandible. Am J Orthod 34:242, 1948 15. Nwoku A: Unusually rapid bone regeneration following mandibular resection, J Maxillofac Surg 8:309, 1980
J Oral Maxillofac 49:652-655.
OF THE MANDIBLE
16. Nagase M, Ueda K, Suzuki I, et al: Spontaneous regeneration of the condyle following hemimandibulectomy by disarticulation. J Oral Maxillofac Surg 43:218, 1985 17. Adekeye E: Rapid bone regeneration subsequent to subtotal mandibulectomy. Oral Med Oral Surg Oral Path01 44:521, 1977 18. Elbeshir E: Spontaneous regeneration of the mandibular bone following hemimandibulectomy. Br J Oral Maxillofat Surg 28:128, 1990
Surg
1991
Leiomyosarcoma
of the Mandible:
A Case Report VEJAYAN KRISHNAN, BDS, MS,* CHARLES M. MIYAJI, DMD,t AND ELGENE G. MAINOUS, DDSS Smooth muscle is the prevalent tissue in the gastrointestinal and the genitourinary systems, but it is present in limited amount in the oral cavity. This may account for the low occurrence of smooth muscle neoplasms in the latter region. This article reports a primary, central leiomyosarcoma in the mandible. Report of Case A 27-year-old white man was seen at the Oral Surgery Clinic of the University of Minnesota on January 31, 1989. His chief complaints were pain and swelling in the right anterior mandible, first noticed in June 1988. The pain was localized to the region and was intermittent at first but was continuous for the past 3 months. He had also noticed loosening of the teeth in the region, but reported no altered sensation in his lower lip or chin. A general dentist, who had taken periapical radiographs, had been seen 3 months previously (Fig 1). A diagnosis of advanced chronic periodontitis was made and periodontal surgery was subsequently performed. However, the patient experienced no relief from his symptoms.
The patient’s past medical history was unremarkable with the exception of a history of drug abuse for the past 10 years. Clinical examination showed the presence of an exophytic lesion on the right parasymphysis of the mandible. The surface mucosa was intact but irregular. Buccolingual expansion was observed in the alveolar segment from the lower right lateral incisor to the right first molar. These teeth exhibited 2 + mobility. The area was tender to palpation. Regional lymph nodes were not palpable. A panoramic radiograph showed an ill-defined, diffuse radiolucency in the symphysis and right body of the mandible (Fig 21, measuring 6 cm in length and spanning the height of the mandible from the alveolar crest to 1 cm short of the inferior border. An incisional biopsy was performed under local anesthesia on January 31, 1989. The lesion was found to be firmly adherent to the overlying mucosa, making it difficult to raise a mucoperiosteal flap. The lesion was whit-
Received from the Department of Oral and Maxillofacial Surgery, University of Minnesota, Minneapolis. * Formerly Clinical Fellow; currently, Resident, Oral and Maxillofacial Surgery, University of Texas Health Science Center at Houston. t Resident. $ Formerly, Chairman; currently Chairman, Oral and Maxillofacial Surgery, University of Texas Health Science Center at Galveston. Address correspondence and reprint requests to Dr Krishnan: Oral and Maxillofacial Surgery, the University of Texas Health Science Center at Houston, 6515 John Freeman Ave. Texas Medical Center, PO Box 20068, Houston, TX 77225. 0 1991 American
Association
geons 0278-2391
I91 /4906-0017$3.00/O
of Oral
and Maxillofacial
Sur-
FIGURE 1. Periapical radiograph in the right parasymphysis of the mandible (September 1988). The radiograph shows an illdefined radiolucency and total loss of bony support for the bicuspid teeth.
KRISHNAN ET AL
653
FIGURE 2. Panoramic radiograph (January 1989)showing the radiolucent lesion in the symphysis and right parasymphysis of the mandible.
ish, multinodular, and relatively avascular. It appeared to have perforated the buccal cortical plate. Tissue specimens were obtained from the depth of the lesion and from the surface, including the overlying mucosa. The specimens were submitted for histologic examination. Hematoxylin and eosin-stained sections revealed the presence of a spindle cell neoplasm that had totally replaced the connective tissue underlying the surface epithelium (Fig 3). The cells exhibited a storiform arrangement (Fig 4). Mitotic tigures (approximately one per highpower field) were readily evident (Fig 5). In the depth of the lesion, the cells appeared more histiocytic and less spindle-shaped. Multinucleated giant cells were also evident. Necrotic areas were noted throughout the lesion. Based on these findings, a diagnosis of malignant fibrous histiocytoma was made pending immunohistochemical investigation. The patient was taken to the operating room on February 6, 1989. A right functional neck dissection was performed with removal of the right submandibular salivary gland and the regional lymph nodes. The mandible was resected from the left first premolar to the right second molar. The resected bone and soft tissue were macroscopically free of tumor. Frozen sections also showed the
FIGURE 3. Photomicrograph of the surface of the lesion showing an intact surface epithelium with the tumor having replaced the underlying connective tissue (hematoxylin-eosin, original magnification x10).
resected margins and the nodes to be free of tumor. A Synthes reconstruction plate (Synthes Cot-p, Monument. CO) was used for primary reconstruction of the resected mandible (Fig 6). The patient’s immediate postoperative course was uneventful. Immunohistochemical studies were performed on the original biopsy specimens and the surgical specimens. These studies showed that the tumor cells were reactive for vimentin and muscle-specific actin. In addition, the cells were also reactive for desmin. In concert with the light microscopy findings, a final diagnosis of leiomyosarcoma grade II/III was made. However. this diagnosis would not have changed the treatment, as both the malignant fibrous histiocytoma and the leiomyosarcoma are managed with surgery and adjunctive chemotherapy and1 or radiotherapy. The patient underwent reconstruction with a deltopectoral rotational flap in November 1989. In January 1990, he presented with right cervical nodes and a lung metastasis. At that time a right radical neck dissection was performed. Chemotherapy was started in March 1990. In July 1990, the patient developed altered mental status. Computed tomography scan showed the presence of a right temporal lobe and a mediastinal mass. The patient’s
654
LEIOMYOSARCOMA
OF THE MANDIBLE
FIGURE 4. Low-power photomicrograph demonstrating the storiform arrangement of the spindle-cell neoplasm. Areas of necrosis (N) are ako evident in this section. Multinucleated giant cells (arrow) were scattered throughout the lesion (hematoxylin-eosin, original magnification X40).
prognosis currently astatic disease.
remains
very poor in light of his met-
Discussion Smooth-muscle neoplasms in the oral cavity are rare. A review of the literature by Poon et al’ showed that there have been only 25 reported cases of primary oral leiomyosarcoma. Of these, there appeared to be no specific site predilection in the oral cavity. There is a predilection for males, with the male-to-female ratio being reported as 3 to 1.2,3 The age of occurrence ranges from 1 to 88 years. Some tissues in the oral cavity have been impli-
cated as the origin of this neoplasm: the circumvallate papilla of the tongue,4,5 the undifferentiated mesenchyme,6,7 and the smooth-muscle elements in blood vessel walls.4V8,9In this particular instance we believe the tumor originated either from the neurovascular bundle or from the undifferentiated mesenchymal elements in the region. Leiomyosarcomas pose a diagnostic challenge both clinically and microscopically. Loosening of the teeth in the region has led to a mistaken diagnosis of advanced periodontitis and subsequent mismanagement of patients.“*” At the light microscopic level this neoplasm can be confused with
FIGURE 5. High-power photomicrograph showing an admixture of spindle-shaped cells and histiocytic cells with hyperchromatism, increased nuclear:cytoplasmic ratio, and abnormal mitotic figures (hematoxylin-eosin, original magnification X200).
KRISHNAN
ET AL
655
FIGURE 6. Panoramic radiograph (February 1989) demonstrating the resected area of the mandible with the Synthes reconstruction plate in place.
other lesions, especially when cells are undifferentiated or pleomorphic.‘2.‘3 The literature has also reported an instance of initial misdiagnosis of a malignant fibrous histiocytoma based on the histologic presentation.13 The role that immunohistochemistry plays in the microscopic diagnosis of this neoplasm cannot be overstated. The immunohistochemical reactivity of the tissue to desmin has been demonstrated to be specific for leiomyosarcomas and 13-15Two features serve to difrhabdomyosarcomas. ferentiate the two: the absence of striated-muscle differentiation in leiomyosarcoma and the reactivity to actin by leiomyosarcoma and to myoglobin by rhabdomyosarcoma. l6 Wide surgical excision remains the mainstay of treatment, with irradiation and chemotherapy as adjunctive treatments.’ The prognosis for this neoplasm is guarded. A 50% recurrence and metastatic rate has been reported, with a mortality of 40%.339 Oral leiomyosarcomas appear to metastasize to the cervical lymph nodes and lungs with equal frequency, whereas tumors in other sites metastasize to the lung and liver.’ Because of the relatively high mortality and recurrence rate, a long-term followup of the patient is mandatory. Further adjunctive therapy and surgical management may be necessary.
References 1. Poon CK, Kwan PC, Yin NT, et al: Leiomyosarcoma of gingiva: Report of a case and review of the literature. J Oral Maxillofac Surg 45888, 1987
2. Bass B, Archard H, Sussman R, et al: Expansile radiolucent lesion of the mandible. J Oral Maxillofac Surg 44:799. 1986 3. Abdin HA, Prabhu SR: Leiomyosarcoma of mandible in a Sudanese female. Int J Oral Surg 14:85, 1985 4. Stout AP: Leiomyoma of the oral cavity. Am J Cancer 34:31, 1938 5. Garret JR: Angiomyoma of the palate: report of a case. Oral Surg 27:103. 1969 6. Cheek JH, Nickey WM: Leiomyosarcoma of venous origin. Arch Surg 90:396. 1965 7. Kratochvil FJ. MacGregor SD, Budnick SD, et al: Leiomyosarcoma of the maxilla. Report of a case and review of the literature. Oral Surg 54647, 1982 8. Farman AG, Kay S: Oral leiomyosarcoma: Report of a case and review of the literature pertaining to smooth muscle tumors of the oral cavity. J Oral Surg 43402, 1977 9. Weitzner S: Leiomyosarcoma of the anterior maxillary alveolar ridge. Oral Surg 50:62, 1980 IO. Phillips H, Brown A: Leiomyosarcoma: Report of a case. J Oral Surg 29:194, 1971 11. Mindell RS, Calcaterra TC, Ward PH: Leiomyosarcoma of the head and neck: A review of the literature and report two cases. Laryngoscope 85904, 1975 12. Nishi M. Mimura T, Senba I: Leiomyosarcoma of the maxilla. J Oral Maxillofac Surg 4564, 1987 13. Miettinen M, Lehto V-P, Ekblom P, et al: Leiomyosarcoma of the mandible; diagnosis as aided by immunohistochemical demonstration of desmin and laminin. J Oral Path01 13:373, 1984 14. Gabianni FN, Kapanci Y. Barazzone P, et al: Immunochemical identification of intermediate-sized filaments in human neoplastic cells. A diagnostic aid for the surgical pathologist. Am J Path01 104:206. 1981 15. Denk H, Krepler R, Artlieb U, et al: Proteins of intermediate filaments. An immunohistochemical and biochemical approach to the classification of soft-tissue tumors. Am J Pathol 110:193. 1983 16. Corson J, Pinkus GS: Intracellular myoglobin-A specific marker for skeletal muscle differentiation in soft tissue sarcomas. An immunoperoxidase study. Am J Path01 103:384. 1981
