- Email: [email protected]
Primary leiomyosarcoma of the mandible
Oral Oncology EXTRA (2006) 42, 40–45
http://intl.elsevierhealth.com/journal/ooex
CASE REPORT
Primary leiomyosarcoma of the mandible C. Ries Centeno a, F. Nadini b, R. Adam c, H. Godoy d, P.A. Reichart
e,*
a
Department of Head and Neck Surgery, Hospital I. Pirovano Buenos Aires, Argentina San Martin 890, 1.B. U9000 Comodoro Rivadavia, Chubut, Argentina c Department of Head and Neck Surgery, Hospital A. Roffo, Buenos Aires, Argentina d 4-12 de octubre 655, CP 9410 Ushuaia, Argentina e ´ Campus Virchow Klinikum, University Medicine Berlin, Center for Dentistry, Department of Oral Charite Surgery and Oral Radiology, Germany b
Received 18 August 2005; accepted 19 August 2005
KEYWORDS
Summary Leiomyosarcoma (LMS) is a malignant neoplasm derived from smooth muscle cells. Only between 3% and 10% appear in the head and neck region, and less than 1% is of intraoral location affecting oral soft tissue structures. About 20 cases of primary LMS involving the mandible have been reported. This case report describes the clinical, histological and surgical features of a primary intraosseous mandibular LMS in a 13 year old male patient. The tumour required a subtotal resection of the mandible with immediate reconstruction using a microsurgical fibula flap. Histopathologically, the characteristic features of leiomyosarcoma were revealed. Since the total number of cases of primary LMS of the mandible reported in the literature is very small factors associated with prognosis are difficult to evaluate. Local control of primary LMS of the mandible is achieved by adequate radical surgery and less by radiation therapy. c 2005 Elsevier Ltd. All rights reserved.
Leiomyosarcoma; Mandible; Resection; Microvascular reconstruction; Immunohistochemistry
Introduction
Leiomyosarcoma (LMS) is a malignant neoplasm composed of cells showing distinct smooth muscle
* Corresponding author. Address: Charite ´ Campus Virchow Klinikum, Zentrum fuer Zahnmedizin, Abteilung fu ¨r Oralchirurgie und Zahna ¨rztliche Ro ¨ntgenologie, Augustenburger Platz 1, 13353 Berlin. Tel.: +49 30 450 56 26 02; fax: +49 30 450 56 29 01. E-mail address: [email protected] (P.A. Reichart).
features.1 The majority is located in the retroperitoneum, including the pelvis and the uterus. LMSs represent only between 3% and 10% of all soft tissue sarcomas.2 According to a review of smooth muscle tumours, only 0.06% occurred in the oral region.3 Until 2002 70 cases of LMSs involving oral structures have been reported.4 Primary LMS of the jawbones is exceedingly rare with only 10 published cases until 1999.5 Recently, further cases were published.4,6,7 In Table 1 cases of leiomyosarcoma of the mandible published to date are
1741-9409/$ - see front matter c 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.ooe.2005.08.008
Primary leiomyosarcoma of the mandible Table 1
41
Cases of leiomyosarcoma of the mandible
Authors 8
Carmody et al. Miles et al.12 Dor et al.13 Chomette et al.14 Miettinen et al.15 Abdin et al.16 Bass et al.17 Hilpert et al.10 Soderholm et al.18 Krishnan et al.19 Farole et al.20 Karlis et al.21 Laccourreye et al.9 Ayad et al.6 Carter et al.5 Goldschmidt et al.23 Picard et al.24 Girod et al.22 Nikitakis et al.4 Nikitakis et al.4 Yamaguchi et al.7
Year
Localisation
Age
Gender
Treatment
Recurrence
Metastasis
Survival
1944 1962 1966 1984 1984 1985 1986 1986 1988 1991 1992 1996 1996 1998 1999 1999 2000 2001 2001 2001 2004
Mandible Mandible Mandible Mandible Condyle Mandible Mandible Mandible Condyle Mandible Condyle Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible
18 34 57 24 62 25 62 40 55 27 56 26 34 40 7 24 35 31 35 51 43
M M M M F F F F F M F F M F F F F M M F M
S S + Cg + RT S S S S + Cg S + Cg + RT S + Cg S S + Cg + RT S + RT S + Cg S + Cg S + RT S S S + CT + RT S + Cg + RT S S + RT S
– 1 year – Unknown 4 months – – Unknown 4 months – 1 year – – – – – 4 months – – – –
– 1 year – Unknown – – – Unknown – 1 year 1 year – – – – – 11 months – 7 months 7 months +
6 monthsa 2.5 yearsb 6 monthsa Unknown 2.5 yearsa 7 monthsa 1 yeara Unknown 4 yearsb Unknown 2 yearsb 1 yeara 1 yeara 18 monthsa 16 monthsa 10 yearsa 17 monthsb 3.5 yearsa 17 monthsb 1 yeara 8 yearsa
S: surgery; Cg: curettage; RT: radiotherapy; CT: chemotherapy; –: no metastasis or recurrence. a Living. b Deceased.
summarized. Although the possibility of a LMS metastasizing to the oral cavity is unusual, it has to be considered before establishing a diagnosis of a primary LMS. As the LMS shares histopathological similarities with other sarcomatous tumours, particularly the leiomyoma, detailed studies for a definite diagnosis should be done considering immunohistochemistry and in some cases electron microscopy. The purpose of this report is to present a case of a primary LMS of the mandible in a 13-year old boy, its diagnosis and surgical resolution.
Case report A 13-year old boy was referred to a maxillofacial surgeon in January 2003 because he developed a swelling in the left mandibular molar region of a size of 8 · 3 · 4 cm. The alveolar mucosa covering the region was of normal characteristics. Several teeth, particularly the molars revealed some degree of mobility. Sensitivity of the dental inferior nerve was intact. Periapical radiographs and panoramic radiography revealed loss of cortical bone and an ill-defined radiolucency with irregular oste-
Figure 1 Panoramic radiograph shows extensive bone destruction of the left body of mandible.
olysis in the region of 35–38 (Fig. 1). Computed tomography (CT) scans without contrast medium (axial, coronal) confirmed the radiologic findings of osteodestruction of the left mandible (Fig. 2). Loss of the lingual cortical bone and invasion of the soft tissues of the floor of the mouth exceeding the midline was seen. Examination of the neck did not reveal any lymphadenopathies. In February 2003 an incisional biopsy was made which confirmed a primary intraosseous LMS of low grade malignancy. A left hemimandibulectomy with conservation of the condyle was performed
42
Figure 2 CT scan of mandible revealing osteodestruction of the left mandible with extensive loss of cortical bone.
C. Ries Centeno et al.
Figure 4 Resection specimen of left mandible measuring 12 · 4 · 4 cm.
Figure 3 Reconstruction of left mandible with fibula flap and UniLock plate.
with immediate reconstruction using a microsurgical fibula flap empowered with a UniLock plate of 2.4 mm diameter with 9 screws (Fig. 3). The surgical specimen of 12 · 4 · 4 cm was fixed in 10% formalin (Fig. 4) and routinely processed for histopathological evaluation. Sections were stained with hematoxilin–eosin (H&E). Spindle shaped tumour cells and a fusocellular pleomorphism with a high mitotic activity was observed. Infiltration of the osseous trabeculae presenting in a fascicular pattern with whirls was seen (grade I/III)
Figure 5 High power micrograph of leiomyosarcoma showing spindle-like cells with two mitotic figures (H&E, ·200).
Primary leiomyosarcoma of the mandible
43
Figure 8 Positive immunostaining of LMS-tumour cells for vimentin (streptavidin–biotin–peroxidase; ·250).
Figure 6 Positive reactivity of tumour cells for desmin (streptavidin–biotin–peroxidase; ·250).
smooth-muscle actin and vimentin was noted (Figs. 6–8). Staining for S-100 protein was negative. The diagnosis of primary LMS of the mandible was confirmed. The postoperative course was unremarkable. The patient is now 2 years from initial surgery with no evidence of local recurrence or metastasis.
Discussion
Figure 7 Immunohistochemical stain for smooth muscle actin showing positive immunoreactivity of LMStumour cells (streptavidin–biotin–peroxidase; ·250).
(Fig. 5). Areas of necrosis were also observed. Immunohistochemically (streptavidin-biotin-peroxidase method) a positive reactivity for desmin,
The first case of a primary LMS of the mandible was reported by Carmody et al. in 1944.8 At present, about 20 cases of primary LMS of the mandible have been reported (Table 1), in addition about 15 cases of the maxilla have been published. Some authors have excluded earlier cases in which no immunohistochemical or ultrastructural studies have been performed.9 Generally, primary LMSs of the jaws are considered to arise from smooth muscle cells of the tunica of vasculature. Clinically, the loss or mobility of teeth and the increase of volume in the gingival region are the most commonly encountered symptoms of the early stage of a mandibular primary LMS. Radiographically, primary LMSs of the mandible are non-characteristic revealing variable radiologic appearance. Usually, poorly defined lytic lesions with cortical bone destruction and occasional pathologic fractures are seen.10 The extent of the lesion is best demonstrated using CT and MRI. Since the clinical and radiographic findings are nonspecific, the final diagnosis is based on histopathologic and immunohistochemical findings. Histopathologically, LMSs are characterized by spindle-shaped cells with mitotic figures. This morphological feature is by itself not sufficient for a
44 final diagnosis because other spindle-shaped tumours such as fibrosarcoma, osteosarcoma, malignant fibrous histiocytoma, malignant Schwannoma, undifferentiated carcinoma and malignant melanoma. Ultrastructurally, the findings of myofilaments, dense bodies, pinocytic vesicles and basal lamina are characteristic for LMS. Immunohistochemical demonstration of muscle-specific actin and smooth-muscle-specific actin is a strong indicator for LMS. Also, desmin may be helpful in the diagnosis of LMS, since the intermediate filament desmin is present in both smooth muscle and striated muscle. Vimentin is considered a major component of the intermediate filaments of smooth muscle cells derived from vasculature.11 Spindle-shaped cells of LMS should be negative for cytokeratins. Malignant tumours of peripheral nerves show immunoreactivity for protein S-100. The aggressive potential of the LMS is considerable and the prognosis at the site of the primary tumour seems to be related to the extent of the initial surgical resection. This suggests treatment, which includes wide resection with a safety margin. Local control of primary LMS of the mandible was achieved without radiotherapy (see Table 1), suggesting that postoperative radiation may be unnecessary in cases of primary mandibular LMS. Reconstruction is important after the extirpation of the tumour, especially when it is located in the mandible. Reconstruction is achieved by using free grafts, microsurgery and reconstruction plates. In the present case a submandibular incision from gonion to gonion at four centimeters from the border of the mandible was used for exposition of the tumour mass. A UniLock reconstruction plate was contoured and adapted with screws. The plate was taken away and a hemimandibulectomy was performed from the left condylar neck to the midline of the mandible. The UniLock reconstruction plate was reinserted. Microvascular reconstruction using a fibula flap was performed. Final dental restoration using osteo-integrated dental implants is the last stage in the treatment of such intraorally located tumours. Due to the small number of cases of primary LMS involving the mandible the prognosis of this tumour is difficult to predict. However, follow-up should be periodically during the first five years after the surgery. Thereafter, an annual follow-up is advisable to avoid recurrences and metastasis.
C. Ries Centeno et al.
2.
3. 4.
5.
6.
7.
8. 9.
10. 11.
12.
13. 14.
15.
16. 17. 18.
19.
20. 21.
References 1. Fletcher CDM, Unni KK, Mertens F, editors. World Health Organization classification of tumours. Pathology and
22.
genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002. Weiss SW, Goldblum JR, editors. Enzinger and Weiss. Leiomyosarcoma soft tissue tumors. 4th ed. St. Louis: Mosby; 2001. p. 727–48. Farman AG. Benign smooth muscle tumors. S Afr Med J 1975;49(33):1333–40. Nikitakis NG, Lopes MA, Bailey JS, Blanchaert Jr RH, Ord JJ, Sauk JJ. Oral leiomyosarcoma: review of the literature and report of two cases with assessment of the prognostic and diagnostic significance of immunohistochemical and molecular markers. Oral Oncol 2002;38: 201–8. Carter LC, Aguirre A, Boyd B, DeLacure MD. Primary leiomyosarcoma of the mandible in a 7-year-old girl. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87(4):477–84. Ayad W, Dieckmann J, Freitag P, Wierich W. Leiomyosarkome im Canalis mandibularis. Mund Kiefer GesichtsChir 1998;2:42–3. Yamaguchi S, Nagasawa H, Suzuki T, Fujii E, Iwaki H, Takagi M, et al. Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years. Clin Oral Invest 2004;8:52–5. Carmody TE, Janney HM, Jsesman HAL. Leiomyosarcoma of the mandible. J Am Dent Assoc 1944;31:1110–4. Laccourreye O, Cauchois R, Laccourreye L. Primary leiomyosarcoma of the mandible. Am J Otolaryngol 1996; 17:415–9. Hilpert PL, Radecki PD, Edmonds P. Primary leiomyosarcoma of the mandible. Skeletal Radiol 1986;15:570–4. Gabbiani Gg, Schmid E, Winter S, et al. Vascular smooth muscle cells differ from other smooth muscle cells: predominance of vimentin filaments and specific alphatype actin. Proc Natl Acad Sci USA 1981;78:298–302. Miles AEW, Waterhouse JP. A leiomyosarcoma of the oral cavity with metastasis to lymph glands. J Path Bact 1962;83:551–5. Dor P, Mayer R, Verlinden W. Le le ´iomyosarcome de la mandibule. Acta Stomatol Belg 1967;64:277–87. Chomette G, Auriol M, Princ G, Guilbert F. Le le ´imyosarcome osseux. Etude ultrastructurale et histoenzymologique. A propos d’un cas de sie `ge mandibulaire. Arch Anat Cytol Path 1984;32:20–5. Miettinen M, Lehto VP, Ekblom P. Leiomyosarcoma of the mandible. Diagnosis as aided by immunohistochemical demonstration of desmin and laminin. J Oral Pathol 1984;13:373–81. Abdin HA, Prabhu SR. Leiomyosarcoma of mandible in a Sudanese female. Int J Oral Surg 1985;14:85–8. Bass B, Archard H, Sussman R. Expansive radiolucent lesion of the mandible. J Oral Maxillofac Surg 1986;44:799–803. So ¨derholm AL, Lindquist C, Teppo L, Wolf J, Sane J. Bone resection in patients with mandibular sarcoma. J CranioMax-Fac Surg 1988;16:224–30. Krishnan V, Miyaji CM, Mainous EG. Leiomyosarcoma of the mandible: a case report. J Oral Maxillofac Surg 1991;49:652–5. Farole A, Manalo AE, Iranpour B. Lesion of the temporomandibular joint. J Oral Maxillofac Surg 1992;50:510–4. Karlis V, Zaslow M, Minkowitz G. Leiomyosarcoma of the mandible: report of a case and literature review. J Oral Maxillofac Surg 1996;54:1127–30. Girod A, Lucas R, Pasquiou C, Isaac S, Freidel M, Breton P. ´ propos Le ´iomyosarcome osseux primitif des maxillaires. A d’un cas. Rev Stomatol Chir Maxillofac 2001;102(6): 319–23.
Primary leiomyosarcoma of the mandible 23. Goldschmidt PR, Goldschmidt JD, Lieblich ST, Eisenberg E. Leiomyosarcoma presenting as a mandibular gingival swelling: a case report. J Periodontol 1999;70(1): 84–9.
45 24. Picard A, Michalak S, Bonin B, Fassio E, Goga D. Le ´iomyosarcome osseux mandibulaire. A propos d’un cas. Revue de la litte ´rature. Rev Stomatol Chir Maxillofac 2000;101(3): 142–6.
http://intl.elsevierhealth.com/journal/ooex
CASE REPORT
Primary leiomyosarcoma of the mandible C. Ries Centeno a, F. Nadini b, R. Adam c, H. Godoy d, P.A. Reichart
e,*
a
Department of Head and Neck Surgery, Hospital I. Pirovano Buenos Aires, Argentina San Martin 890, 1.B. U9000 Comodoro Rivadavia, Chubut, Argentina c Department of Head and Neck Surgery, Hospital A. Roffo, Buenos Aires, Argentina d 4-12 de octubre 655, CP 9410 Ushuaia, Argentina e ´ Campus Virchow Klinikum, University Medicine Berlin, Center for Dentistry, Department of Oral Charite Surgery and Oral Radiology, Germany b
Received 18 August 2005; accepted 19 August 2005
KEYWORDS
Summary Leiomyosarcoma (LMS) is a malignant neoplasm derived from smooth muscle cells. Only between 3% and 10% appear in the head and neck region, and less than 1% is of intraoral location affecting oral soft tissue structures. About 20 cases of primary LMS involving the mandible have been reported. This case report describes the clinical, histological and surgical features of a primary intraosseous mandibular LMS in a 13 year old male patient. The tumour required a subtotal resection of the mandible with immediate reconstruction using a microsurgical fibula flap. Histopathologically, the characteristic features of leiomyosarcoma were revealed. Since the total number of cases of primary LMS of the mandible reported in the literature is very small factors associated with prognosis are difficult to evaluate. Local control of primary LMS of the mandible is achieved by adequate radical surgery and less by radiation therapy. c 2005 Elsevier Ltd. All rights reserved.
Leiomyosarcoma; Mandible; Resection; Microvascular reconstruction; Immunohistochemistry
Introduction
Leiomyosarcoma (LMS) is a malignant neoplasm composed of cells showing distinct smooth muscle
* Corresponding author. Address: Charite ´ Campus Virchow Klinikum, Zentrum fuer Zahnmedizin, Abteilung fu ¨r Oralchirurgie und Zahna ¨rztliche Ro ¨ntgenologie, Augustenburger Platz 1, 13353 Berlin. Tel.: +49 30 450 56 26 02; fax: +49 30 450 56 29 01. E-mail address: [email protected] (P.A. Reichart).
features.1 The majority is located in the retroperitoneum, including the pelvis and the uterus. LMSs represent only between 3% and 10% of all soft tissue sarcomas.2 According to a review of smooth muscle tumours, only 0.06% occurred in the oral region.3 Until 2002 70 cases of LMSs involving oral structures have been reported.4 Primary LMS of the jawbones is exceedingly rare with only 10 published cases until 1999.5 Recently, further cases were published.4,6,7 In Table 1 cases of leiomyosarcoma of the mandible published to date are
1741-9409/$ - see front matter c 2005 Elsevier Ltd. All rights reserved. doi:10.1016/j.ooe.2005.08.008
Primary leiomyosarcoma of the mandible Table 1
41
Cases of leiomyosarcoma of the mandible
Authors 8
Carmody et al. Miles et al.12 Dor et al.13 Chomette et al.14 Miettinen et al.15 Abdin et al.16 Bass et al.17 Hilpert et al.10 Soderholm et al.18 Krishnan et al.19 Farole et al.20 Karlis et al.21 Laccourreye et al.9 Ayad et al.6 Carter et al.5 Goldschmidt et al.23 Picard et al.24 Girod et al.22 Nikitakis et al.4 Nikitakis et al.4 Yamaguchi et al.7
Year
Localisation
Age
Gender
Treatment
Recurrence
Metastasis
Survival
1944 1962 1966 1984 1984 1985 1986 1986 1988 1991 1992 1996 1996 1998 1999 1999 2000 2001 2001 2001 2004
Mandible Mandible Mandible Mandible Condyle Mandible Mandible Mandible Condyle Mandible Condyle Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible Mandible
18 34 57 24 62 25 62 40 55 27 56 26 34 40 7 24 35 31 35 51 43
M M M M F F F F F M F F M F F F F M M F M
S S + Cg + RT S S S S + Cg S + Cg + RT S + Cg S S + Cg + RT S + RT S + Cg S + Cg S + RT S S S + CT + RT S + Cg + RT S S + RT S
– 1 year – Unknown 4 months – – Unknown 4 months – 1 year – – – – – 4 months – – – –
– 1 year – Unknown – – – Unknown – 1 year 1 year – – – – – 11 months – 7 months 7 months +
6 monthsa 2.5 yearsb 6 monthsa Unknown 2.5 yearsa 7 monthsa 1 yeara Unknown 4 yearsb Unknown 2 yearsb 1 yeara 1 yeara 18 monthsa 16 monthsa 10 yearsa 17 monthsb 3.5 yearsa 17 monthsb 1 yeara 8 yearsa
S: surgery; Cg: curettage; RT: radiotherapy; CT: chemotherapy; –: no metastasis or recurrence. a Living. b Deceased.
summarized. Although the possibility of a LMS metastasizing to the oral cavity is unusual, it has to be considered before establishing a diagnosis of a primary LMS. As the LMS shares histopathological similarities with other sarcomatous tumours, particularly the leiomyoma, detailed studies for a definite diagnosis should be done considering immunohistochemistry and in some cases electron microscopy. The purpose of this report is to present a case of a primary LMS of the mandible in a 13-year old boy, its diagnosis and surgical resolution.
Case report A 13-year old boy was referred to a maxillofacial surgeon in January 2003 because he developed a swelling in the left mandibular molar region of a size of 8 · 3 · 4 cm. The alveolar mucosa covering the region was of normal characteristics. Several teeth, particularly the molars revealed some degree of mobility. Sensitivity of the dental inferior nerve was intact. Periapical radiographs and panoramic radiography revealed loss of cortical bone and an ill-defined radiolucency with irregular oste-
Figure 1 Panoramic radiograph shows extensive bone destruction of the left body of mandible.
olysis in the region of 35–38 (Fig. 1). Computed tomography (CT) scans without contrast medium (axial, coronal) confirmed the radiologic findings of osteodestruction of the left mandible (Fig. 2). Loss of the lingual cortical bone and invasion of the soft tissues of the floor of the mouth exceeding the midline was seen. Examination of the neck did not reveal any lymphadenopathies. In February 2003 an incisional biopsy was made which confirmed a primary intraosseous LMS of low grade malignancy. A left hemimandibulectomy with conservation of the condyle was performed
42
Figure 2 CT scan of mandible revealing osteodestruction of the left mandible with extensive loss of cortical bone.
C. Ries Centeno et al.
Figure 4 Resection specimen of left mandible measuring 12 · 4 · 4 cm.
Figure 3 Reconstruction of left mandible with fibula flap and UniLock plate.
with immediate reconstruction using a microsurgical fibula flap empowered with a UniLock plate of 2.4 mm diameter with 9 screws (Fig. 3). The surgical specimen of 12 · 4 · 4 cm was fixed in 10% formalin (Fig. 4) and routinely processed for histopathological evaluation. Sections were stained with hematoxilin–eosin (H&E). Spindle shaped tumour cells and a fusocellular pleomorphism with a high mitotic activity was observed. Infiltration of the osseous trabeculae presenting in a fascicular pattern with whirls was seen (grade I/III)
Figure 5 High power micrograph of leiomyosarcoma showing spindle-like cells with two mitotic figures (H&E, ·200).
Primary leiomyosarcoma of the mandible
43
Figure 8 Positive immunostaining of LMS-tumour cells for vimentin (streptavidin–biotin–peroxidase; ·250).
Figure 6 Positive reactivity of tumour cells for desmin (streptavidin–biotin–peroxidase; ·250).
smooth-muscle actin and vimentin was noted (Figs. 6–8). Staining for S-100 protein was negative. The diagnosis of primary LMS of the mandible was confirmed. The postoperative course was unremarkable. The patient is now 2 years from initial surgery with no evidence of local recurrence or metastasis.
Discussion
Figure 7 Immunohistochemical stain for smooth muscle actin showing positive immunoreactivity of LMStumour cells (streptavidin–biotin–peroxidase; ·250).
(Fig. 5). Areas of necrosis were also observed. Immunohistochemically (streptavidin-biotin-peroxidase method) a positive reactivity for desmin,
The first case of a primary LMS of the mandible was reported by Carmody et al. in 1944.8 At present, about 20 cases of primary LMS of the mandible have been reported (Table 1), in addition about 15 cases of the maxilla have been published. Some authors have excluded earlier cases in which no immunohistochemical or ultrastructural studies have been performed.9 Generally, primary LMSs of the jaws are considered to arise from smooth muscle cells of the tunica of vasculature. Clinically, the loss or mobility of teeth and the increase of volume in the gingival region are the most commonly encountered symptoms of the early stage of a mandibular primary LMS. Radiographically, primary LMSs of the mandible are non-characteristic revealing variable radiologic appearance. Usually, poorly defined lytic lesions with cortical bone destruction and occasional pathologic fractures are seen.10 The extent of the lesion is best demonstrated using CT and MRI. Since the clinical and radiographic findings are nonspecific, the final diagnosis is based on histopathologic and immunohistochemical findings. Histopathologically, LMSs are characterized by spindle-shaped cells with mitotic figures. This morphological feature is by itself not sufficient for a
44 final diagnosis because other spindle-shaped tumours such as fibrosarcoma, osteosarcoma, malignant fibrous histiocytoma, malignant Schwannoma, undifferentiated carcinoma and malignant melanoma. Ultrastructurally, the findings of myofilaments, dense bodies, pinocytic vesicles and basal lamina are characteristic for LMS. Immunohistochemical demonstration of muscle-specific actin and smooth-muscle-specific actin is a strong indicator for LMS. Also, desmin may be helpful in the diagnosis of LMS, since the intermediate filament desmin is present in both smooth muscle and striated muscle. Vimentin is considered a major component of the intermediate filaments of smooth muscle cells derived from vasculature.11 Spindle-shaped cells of LMS should be negative for cytokeratins. Malignant tumours of peripheral nerves show immunoreactivity for protein S-100. The aggressive potential of the LMS is considerable and the prognosis at the site of the primary tumour seems to be related to the extent of the initial surgical resection. This suggests treatment, which includes wide resection with a safety margin. Local control of primary LMS of the mandible was achieved without radiotherapy (see Table 1), suggesting that postoperative radiation may be unnecessary in cases of primary mandibular LMS. Reconstruction is important after the extirpation of the tumour, especially when it is located in the mandible. Reconstruction is achieved by using free grafts, microsurgery and reconstruction plates. In the present case a submandibular incision from gonion to gonion at four centimeters from the border of the mandible was used for exposition of the tumour mass. A UniLock reconstruction plate was contoured and adapted with screws. The plate was taken away and a hemimandibulectomy was performed from the left condylar neck to the midline of the mandible. The UniLock reconstruction plate was reinserted. Microvascular reconstruction using a fibula flap was performed. Final dental restoration using osteo-integrated dental implants is the last stage in the treatment of such intraorally located tumours. Due to the small number of cases of primary LMS involving the mandible the prognosis of this tumour is difficult to predict. However, follow-up should be periodically during the first five years after the surgery. Thereafter, an annual follow-up is advisable to avoid recurrences and metastasis.
C. Ries Centeno et al.
2.
3. 4.
5.
6.
7.
8. 9.
10. 11.
12.
13. 14.
15.
16. 17. 18.
19.
20. 21.
References 1. Fletcher CDM, Unni KK, Mertens F, editors. World Health Organization classification of tumours. Pathology and
22.
genetics of tumours of soft tissue and bone. Lyon: IARC Press; 2002. Weiss SW, Goldblum JR, editors. Enzinger and Weiss. Leiomyosarcoma soft tissue tumors. 4th ed. St. Louis: Mosby; 2001. p. 727–48. Farman AG. Benign smooth muscle tumors. S Afr Med J 1975;49(33):1333–40. Nikitakis NG, Lopes MA, Bailey JS, Blanchaert Jr RH, Ord JJ, Sauk JJ. Oral leiomyosarcoma: review of the literature and report of two cases with assessment of the prognostic and diagnostic significance of immunohistochemical and molecular markers. Oral Oncol 2002;38: 201–8. Carter LC, Aguirre A, Boyd B, DeLacure MD. Primary leiomyosarcoma of the mandible in a 7-year-old girl. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87(4):477–84. Ayad W, Dieckmann J, Freitag P, Wierich W. Leiomyosarkome im Canalis mandibularis. Mund Kiefer GesichtsChir 1998;2:42–3. Yamaguchi S, Nagasawa H, Suzuki T, Fujii E, Iwaki H, Takagi M, et al. Sarcomas of the oral and maxillofacial region: a review of 32 cases in 25 years. Clin Oral Invest 2004;8:52–5. Carmody TE, Janney HM, Jsesman HAL. Leiomyosarcoma of the mandible. J Am Dent Assoc 1944;31:1110–4. Laccourreye O, Cauchois R, Laccourreye L. Primary leiomyosarcoma of the mandible. Am J Otolaryngol 1996; 17:415–9. Hilpert PL, Radecki PD, Edmonds P. Primary leiomyosarcoma of the mandible. Skeletal Radiol 1986;15:570–4. Gabbiani Gg, Schmid E, Winter S, et al. Vascular smooth muscle cells differ from other smooth muscle cells: predominance of vimentin filaments and specific alphatype actin. Proc Natl Acad Sci USA 1981;78:298–302. Miles AEW, Waterhouse JP. A leiomyosarcoma of the oral cavity with metastasis to lymph glands. J Path Bact 1962;83:551–5. Dor P, Mayer R, Verlinden W. Le le ´iomyosarcome de la mandibule. Acta Stomatol Belg 1967;64:277–87. Chomette G, Auriol M, Princ G, Guilbert F. Le le ´imyosarcome osseux. Etude ultrastructurale et histoenzymologique. A propos d’un cas de sie `ge mandibulaire. Arch Anat Cytol Path 1984;32:20–5. Miettinen M, Lehto VP, Ekblom P. Leiomyosarcoma of the mandible. Diagnosis as aided by immunohistochemical demonstration of desmin and laminin. J Oral Pathol 1984;13:373–81. Abdin HA, Prabhu SR. Leiomyosarcoma of mandible in a Sudanese female. Int J Oral Surg 1985;14:85–8. Bass B, Archard H, Sussman R. Expansive radiolucent lesion of the mandible. J Oral Maxillofac Surg 1986;44:799–803. So ¨derholm AL, Lindquist C, Teppo L, Wolf J, Sane J. Bone resection in patients with mandibular sarcoma. J CranioMax-Fac Surg 1988;16:224–30. Krishnan V, Miyaji CM, Mainous EG. Leiomyosarcoma of the mandible: a case report. J Oral Maxillofac Surg 1991;49:652–5. Farole A, Manalo AE, Iranpour B. Lesion of the temporomandibular joint. J Oral Maxillofac Surg 1992;50:510–4. Karlis V, Zaslow M, Minkowitz G. Leiomyosarcoma of the mandible: report of a case and literature review. J Oral Maxillofac Surg 1996;54:1127–30. Girod A, Lucas R, Pasquiou C, Isaac S, Freidel M, Breton P. ´ propos Le ´iomyosarcome osseux primitif des maxillaires. A d’un cas. Rev Stomatol Chir Maxillofac 2001;102(6): 319–23.
Primary leiomyosarcoma of the mandible 23. Goldschmidt PR, Goldschmidt JD, Lieblich ST, Eisenberg E. Leiomyosarcoma presenting as a mandibular gingival swelling: a case report. J Periodontol 1999;70(1): 84–9.
45 24. Picard A, Michalak S, Bonin B, Fassio E, Goga D. Le ´iomyosarcome osseux mandibulaire. A propos d’un cas. Revue de la litte ´rature. Rev Stomatol Chir Maxillofac 2000;101(3): 142–6.