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Leiomyomatous hamartoma of the tongue: Case report
337
KOBAYASHI, AMAGASA, AND OKADA
J Oral Maxillofac Surg 59:337-340, 2001
Leiomyomatous Hamartoma of the Tongue: Case Report Akiko Kobayashi, DDS, PhD,* Teruo Amagasa, DDS, PhD,† and Norihiko Okada, DDS, PhD‡ First used by Albrecht in 1904,1 the term hamartoma denotes a tumor-like malformation presenting as a *Research Student, Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. †Chief Professor, Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. ‡Associate Professor, Department of Clinical Laboratory, Dental Hospital, Tokyo Medical and Dental University, Tokyo, Japan. Address correspondence and reprint requests to Dr Kobayashi: Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, 5-45 Yushima 1-chome, Bunkyoku, Tokyo, 113-8549 Japan; e-mail: [email protected] © 2001 American Association of Oral and Maxillofacial Surgeons
0278-2391/01/5903-0017$35.00/0 doi:10.1053/joms.2001.21008
mass of disorganized tissue indigenous to a particular site. Hamartomas commonly occur in the liver, spleen, kidneys, lungs, and pancreas, and also in the oral regions. Hemangiomas and lymphangiomas are common types of hamartomas with well-described clinical and histologic features.2 However, leiomyomatous hamartomas are extremely rare in the oral region, and most of those described have occurred in Japanese patients. Although leiomyomatous hamartomas may develop at any site in the oral cavity, they mainly occur on the dorsal tongue or the palate and sometimes more than one can present.3-10 We report a case of leiomyomatous hamartoma on the tongue of a 3-month-old infant.
Report of Case A 3-month-old boy was brought to us in October 1997 for evaluation of a tongue mass. He had been born at term,
Please scroll down for full article.
338
LEIOMYOMATOUS HAMARTOMA OF THE TONGUE The wound healed without complications. No recurrence was noted at the 2-year follow-up examination. The tongue mucosa was normal, and there was no problem with feeding. The surgical specimen consisted of a piece of solid, grayish-white soft tissue with covering mucosa (Fig 2). Tissue sections were stained with hematoxylin and eosin, and immunohistochemical staining was done with the streptavidin-biotinylated-peroxidase complex method using anti-␣smooth muscle actin as a first antibody. Microscopy revealed numerous bundles of fascicular or aggregated smooth muscle fibers under the parakeratinized stratified squamous epithelium (Figs 3, 4). The bundles of smooth muscle were grouped or scattered in the connective tissue, and thus the appearance was not that of a solid tumor such as leiomyoma. The bundles of smooth muscle and striated muscles in deeper regions were completely separated. There were no definite capsular structures. Our final diagnosis was leiomyomatous hamartoma because of the histologic findings, particularly in view of the fact that the lesion was congenital.
Discussion
FIGURE 1. Intraoral view showing pedunculated lesion (10 ⫻ 14 ⫻ 5 mm) on the mid-dorsum of the tongue. The lesion appeared to have a normal mucosal covering without papillae.
weighing 3,034 g, and had shown no apparent difficulty with suckling. The obstetrician noticed the lesion on the mid-dorsum of the tongue and referred the patient to a pediatrician. The patient was then admitted to the First Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tokyo Medical and Dental University for treatment of the lesion. There had been no problem during delivery, and the family history was noncontributory. Physical examination revealed an otherwise healthy infant, who was well-developed, well-nourished, and in no apparent distress. His expression and complexion were good. Intraoral examination revealed a pedunculated lesion (10 ⫻ 14 ⫻ 5 mm) on the mid-dorsum of the tongue at the anterior aspect of the sulcus terminalis linguae (Fig 1). The elastic-soft lesion appeared to have a normal covering mucosa without papillae. Further examination revealed pseudomembranous candidiasis over the entire oral mucosa, for which medical treatment had already been initiated. The results of other clinical examinations were normal. Our first diagnosis was a benign tumor of the tongue, and it was decided to wait until the child was older to remove the lesion. No change was noted in the size of the lesion for 3 months, and in February 1998 it was resected under general anesthesia when the patient was 6 months old. A fusiform surgical incision was made in the healthy normal mucosa around the lesion, which was then resected along with part of the normal tongue muscle. The wound was closed primarily.
A hamartoma is classified histopathologically by its main composite elements (eg, leiomyomatous hamartoma,3-10 cartilaginous hamartoma,11 angiomatous hamartoma,12 etc). Although hemangiomas and lymphangiomas are the most common types of hamartomas seen in the oral cavity,2 other rare forms of hamartoma may arise in the mouth on the gingiva,7-9 palate,10 and tongue,3-6,11,12 particularly in the region of foramen cecum. A review of the Japanese and English literature from 1904 to 1998 yielded only 4
FIGURE 2. The tongue mass was resected with part of the normal tongue muscle.
339
KOBAYASHI, AMAGASA, AND OKADA
cases of histologically defined leiomyomatous hamartoma of the tongue (Table 1). These tongue masses were multiple or solitary and occurred predominantly in males. The predilection of oral hamartomas for the base of the tongue (foramen cecum region) or upper median gingiva may be explained by the fact that these are regions of fusion of several tissues during embryologic development.13 This also explains the histogenesis of thyroglossal duct cysts.14 Nishihara reported evidence for a palatal recess corresponding to a tongue mass.4 This revealed the existence of the mass at 8 or 9 weeks of embryonal development when the secondary palate was formed. Hamartomas grow very slowly or remain almost unchanged in size, or at least do not grow autonomously. On the other hand, hamartomas may be inconspicuous at birth, only becoming apparent when they seem to enlarge rapidly, for example after trauma or during the pubertal growth spurt.10 Excision is not strictly necessary, except in medical emergencies such as dyspnea,15 dysphagia,16 or suckling disability.17 In this patient, the mass was present at birth and did not change in size over 6 months. The patient’s
FIGURE 4. Photomicrograph of the lesion after immunohistochemical staining for smooth-muscle actin. The bundles of smooth muscle are completely separated from the striated muscles (original magnification ⫻ 16).
mother was worried that the mass might hinder feeding and therefore wanted it removed. It is not always easy to distinguish hamartomas from benign neoplasms. Gingival leiomyomatous hamartoma has the clinical features of a congenital epulis, and the corresponding lesion on the tongue is similar to a mesenchymoma. However, consideration of the history and histologic features will allow a definite diagnosis to be established. In this case of leiomyomatous hamartoma, no evidence was noted of nuclear pleomorphism, mitotic
Table 1. FOUR CASES OF LEIOMYOMATOUS HAMARTOMA OF THE TONGUE
Case No. FIGURE 3. Photomicrograph of the lesion showing numerous bundles of smooth muscle with blood vessels. The bundles of smooth muscle are not actively proliferating (hematoxylin-eosin stain, original magnification ⫻ 16).
1. 2. 3. 4.
Author
Year 5
Yamashita et al Tohge et al3 Tamaki et al6 Nishihara et al4
1973 1987 1990 1991
Age/Sex 5 yr/Female 15 yr/Male 1.5 mo/Male 15 d/Male
340 activity, invasion, or necrosis in the microscopic specimens. Numerous bundles of smooth muscle in the connective tissue, were immunohistochemically positive for ␣-smooth-muscle actin. This type of lesion may include salivary gland tissue, fatty tissue, nerves, and blood vessels. Furthermore, only microscopic examination can distinguish between leiomyomatous hamartoma and congenital oral leiomyoma. Active and solid proliferation of smooth muscle tissue is the hallmark of a true leiomyoma of the congenital type; however, this case did not show such active and solid proliferative patterns. The prognosis of leiomyomatous hamartoma of the tongue is good when it is treated by excision with a small surgical margin, and this patient has shown no signs of recurrence for 2 years after surgery.
References 1. Albrecht H: Uber Hamartome. Verh Dtsch Ges Pathol 7:153, 1904 2. Batsakis JG: Tumors of the Head and Neck (ed 2). Baltimore, MD, Williams & Wilkins, 1979, p 291 3. Tohge H, Toratani S, Yoshiga K, et al: A case of hamartoma of the tongue. Jpn J Oral Maxillofac Surg 33:2048, 1987 4. Nishihara K, Nozoe E, Mimura T, et al: A case of multiple leiomyomatous hamartoma on the tongue with palatal recess. Jpn J Oral Maxillofac Surg 37:1506, 1991
5. Yamashita S, Masuda T, Seguchi Y: Hamartoma of the tongue: Report of a case. Jpn J Oral Maxillofac Surg 19:69, 1973 6. Tamaki H, Kitajima T, Yamada H, et al: Congenital tongue mass associated with heterotopic smooth muscle: Report of a case. Jpn J Oral Maxillofac Surg 36:130, 1990 7. Kajiyama M, Iino E, Kurokawa H, et al: Leiomyomatous hamartoma in the region of upper medial alveolar gingiva: Report of a case. Jpn J Oral Maxillofac Surg 29:1520, 1983 8. Mushimoto K, Kakudo K, Ueno S, et al: Leiomyomatous hamartoma of the gingiva: Report of a case. Jpn J Oral Maxillofac Surg 28:493, 1982 9. Semba I, Kitano M, Mimura T: Gingival leiomyomatous hamartoma: Immunohistochemical and ultrastructural observations: Case report. J Oral Pathol Med 22:468, 1993 10. Napier SS, Devine JC, Rennie JS: Unusual leiomyomatous hamartoma of the hard palate: A case report. Oral Surg Oral Med Oral Pathol 82:305, 1996 11. Totsuka M, Yuhki K, Shimizu M: Cartilaginous hamartoma of the tongue: Report of a case. Jpn J Oral Maxillofac Surg 23:418, 1977 12. Nakano Y, Ono K, Shimabara M, et al: A case of angiomatous hamartoma of the tongue. Med J Osaka Univ (Japanese Edition) 43:231, 1984 13. Stamm C, Tauber R: Hamartoma of the tongue. Laryngoscope 55:140, 1945 14. Bhaskar SN, (ed): Orben’s Oral Histology and Embryology. St Louis, MO, Mosby, 1976, p 21 15. Duncan H: Tumor of the tongue. J Tenn Med Assoc 61:48, 1968 16. Perri FA: Myoepithelial hamartoma of the tongue. Arch Otolaryngol 64:289, 1956 17. Hinshaw CT: Unusual lesions of the tongue: Hamartoma. J Kans Med Soc 64:156, 1963
KOBAYASHI, AMAGASA, AND OKADA
J Oral Maxillofac Surg 59:337-340, 2001
Leiomyomatous Hamartoma of the Tongue: Case Report Akiko Kobayashi, DDS, PhD,* Teruo Amagasa, DDS, PhD,† and Norihiko Okada, DDS, PhD‡ First used by Albrecht in 1904,1 the term hamartoma denotes a tumor-like malformation presenting as a *Research Student, Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. †Chief Professor, Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan. ‡Associate Professor, Department of Clinical Laboratory, Dental Hospital, Tokyo Medical and Dental University, Tokyo, Japan. Address correspondence and reprint requests to Dr Kobayashi: Maxillofacial Surgery, Maxillofacial Reconstruction and Function, Division of Maxillofacial and Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, 5-45 Yushima 1-chome, Bunkyoku, Tokyo, 113-8549 Japan; e-mail: [email protected] © 2001 American Association of Oral and Maxillofacial Surgeons
0278-2391/01/5903-0017$35.00/0 doi:10.1053/joms.2001.21008
mass of disorganized tissue indigenous to a particular site. Hamartomas commonly occur in the liver, spleen, kidneys, lungs, and pancreas, and also in the oral regions. Hemangiomas and lymphangiomas are common types of hamartomas with well-described clinical and histologic features.2 However, leiomyomatous hamartomas are extremely rare in the oral region, and most of those described have occurred in Japanese patients. Although leiomyomatous hamartomas may develop at any site in the oral cavity, they mainly occur on the dorsal tongue or the palate and sometimes more than one can present.3-10 We report a case of leiomyomatous hamartoma on the tongue of a 3-month-old infant.
Report of Case A 3-month-old boy was brought to us in October 1997 for evaluation of a tongue mass. He had been born at term,
Please scroll down for full article.
338
LEIOMYOMATOUS HAMARTOMA OF THE TONGUE The wound healed without complications. No recurrence was noted at the 2-year follow-up examination. The tongue mucosa was normal, and there was no problem with feeding. The surgical specimen consisted of a piece of solid, grayish-white soft tissue with covering mucosa (Fig 2). Tissue sections were stained with hematoxylin and eosin, and immunohistochemical staining was done with the streptavidin-biotinylated-peroxidase complex method using anti-␣smooth muscle actin as a first antibody. Microscopy revealed numerous bundles of fascicular or aggregated smooth muscle fibers under the parakeratinized stratified squamous epithelium (Figs 3, 4). The bundles of smooth muscle were grouped or scattered in the connective tissue, and thus the appearance was not that of a solid tumor such as leiomyoma. The bundles of smooth muscle and striated muscles in deeper regions were completely separated. There were no definite capsular structures. Our final diagnosis was leiomyomatous hamartoma because of the histologic findings, particularly in view of the fact that the lesion was congenital.
Discussion
FIGURE 1. Intraoral view showing pedunculated lesion (10 ⫻ 14 ⫻ 5 mm) on the mid-dorsum of the tongue. The lesion appeared to have a normal mucosal covering without papillae.
weighing 3,034 g, and had shown no apparent difficulty with suckling. The obstetrician noticed the lesion on the mid-dorsum of the tongue and referred the patient to a pediatrician. The patient was then admitted to the First Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, Tokyo Medical and Dental University for treatment of the lesion. There had been no problem during delivery, and the family history was noncontributory. Physical examination revealed an otherwise healthy infant, who was well-developed, well-nourished, and in no apparent distress. His expression and complexion were good. Intraoral examination revealed a pedunculated lesion (10 ⫻ 14 ⫻ 5 mm) on the mid-dorsum of the tongue at the anterior aspect of the sulcus terminalis linguae (Fig 1). The elastic-soft lesion appeared to have a normal covering mucosa without papillae. Further examination revealed pseudomembranous candidiasis over the entire oral mucosa, for which medical treatment had already been initiated. The results of other clinical examinations were normal. Our first diagnosis was a benign tumor of the tongue, and it was decided to wait until the child was older to remove the lesion. No change was noted in the size of the lesion for 3 months, and in February 1998 it was resected under general anesthesia when the patient was 6 months old. A fusiform surgical incision was made in the healthy normal mucosa around the lesion, which was then resected along with part of the normal tongue muscle. The wound was closed primarily.
A hamartoma is classified histopathologically by its main composite elements (eg, leiomyomatous hamartoma,3-10 cartilaginous hamartoma,11 angiomatous hamartoma,12 etc). Although hemangiomas and lymphangiomas are the most common types of hamartomas seen in the oral cavity,2 other rare forms of hamartoma may arise in the mouth on the gingiva,7-9 palate,10 and tongue,3-6,11,12 particularly in the region of foramen cecum. A review of the Japanese and English literature from 1904 to 1998 yielded only 4
FIGURE 2. The tongue mass was resected with part of the normal tongue muscle.
339
KOBAYASHI, AMAGASA, AND OKADA
cases of histologically defined leiomyomatous hamartoma of the tongue (Table 1). These tongue masses were multiple or solitary and occurred predominantly in males. The predilection of oral hamartomas for the base of the tongue (foramen cecum region) or upper median gingiva may be explained by the fact that these are regions of fusion of several tissues during embryologic development.13 This also explains the histogenesis of thyroglossal duct cysts.14 Nishihara reported evidence for a palatal recess corresponding to a tongue mass.4 This revealed the existence of the mass at 8 or 9 weeks of embryonal development when the secondary palate was formed. Hamartomas grow very slowly or remain almost unchanged in size, or at least do not grow autonomously. On the other hand, hamartomas may be inconspicuous at birth, only becoming apparent when they seem to enlarge rapidly, for example after trauma or during the pubertal growth spurt.10 Excision is not strictly necessary, except in medical emergencies such as dyspnea,15 dysphagia,16 or suckling disability.17 In this patient, the mass was present at birth and did not change in size over 6 months. The patient’s
FIGURE 4. Photomicrograph of the lesion after immunohistochemical staining for smooth-muscle actin. The bundles of smooth muscle are completely separated from the striated muscles (original magnification ⫻ 16).
mother was worried that the mass might hinder feeding and therefore wanted it removed. It is not always easy to distinguish hamartomas from benign neoplasms. Gingival leiomyomatous hamartoma has the clinical features of a congenital epulis, and the corresponding lesion on the tongue is similar to a mesenchymoma. However, consideration of the history and histologic features will allow a definite diagnosis to be established. In this case of leiomyomatous hamartoma, no evidence was noted of nuclear pleomorphism, mitotic
Table 1. FOUR CASES OF LEIOMYOMATOUS HAMARTOMA OF THE TONGUE
Case No. FIGURE 3. Photomicrograph of the lesion showing numerous bundles of smooth muscle with blood vessels. The bundles of smooth muscle are not actively proliferating (hematoxylin-eosin stain, original magnification ⫻ 16).
1. 2. 3. 4.
Author
Year 5
Yamashita et al Tohge et al3 Tamaki et al6 Nishihara et al4
1973 1987 1990 1991
Age/Sex 5 yr/Female 15 yr/Male 1.5 mo/Male 15 d/Male
340 activity, invasion, or necrosis in the microscopic specimens. Numerous bundles of smooth muscle in the connective tissue, were immunohistochemically positive for ␣-smooth-muscle actin. This type of lesion may include salivary gland tissue, fatty tissue, nerves, and blood vessels. Furthermore, only microscopic examination can distinguish between leiomyomatous hamartoma and congenital oral leiomyoma. Active and solid proliferation of smooth muscle tissue is the hallmark of a true leiomyoma of the congenital type; however, this case did not show such active and solid proliferative patterns. The prognosis of leiomyomatous hamartoma of the tongue is good when it is treated by excision with a small surgical margin, and this patient has shown no signs of recurrence for 2 years after surgery.
References 1. Albrecht H: Uber Hamartome. Verh Dtsch Ges Pathol 7:153, 1904 2. Batsakis JG: Tumors of the Head and Neck (ed 2). Baltimore, MD, Williams & Wilkins, 1979, p 291 3. Tohge H, Toratani S, Yoshiga K, et al: A case of hamartoma of the tongue. Jpn J Oral Maxillofac Surg 33:2048, 1987 4. Nishihara K, Nozoe E, Mimura T, et al: A case of multiple leiomyomatous hamartoma on the tongue with palatal recess. Jpn J Oral Maxillofac Surg 37:1506, 1991
5. Yamashita S, Masuda T, Seguchi Y: Hamartoma of the tongue: Report of a case. Jpn J Oral Maxillofac Surg 19:69, 1973 6. Tamaki H, Kitajima T, Yamada H, et al: Congenital tongue mass associated with heterotopic smooth muscle: Report of a case. Jpn J Oral Maxillofac Surg 36:130, 1990 7. Kajiyama M, Iino E, Kurokawa H, et al: Leiomyomatous hamartoma in the region of upper medial alveolar gingiva: Report of a case. Jpn J Oral Maxillofac Surg 29:1520, 1983 8. Mushimoto K, Kakudo K, Ueno S, et al: Leiomyomatous hamartoma of the gingiva: Report of a case. Jpn J Oral Maxillofac Surg 28:493, 1982 9. Semba I, Kitano M, Mimura T: Gingival leiomyomatous hamartoma: Immunohistochemical and ultrastructural observations: Case report. J Oral Pathol Med 22:468, 1993 10. Napier SS, Devine JC, Rennie JS: Unusual leiomyomatous hamartoma of the hard palate: A case report. Oral Surg Oral Med Oral Pathol 82:305, 1996 11. Totsuka M, Yuhki K, Shimizu M: Cartilaginous hamartoma of the tongue: Report of a case. Jpn J Oral Maxillofac Surg 23:418, 1977 12. Nakano Y, Ono K, Shimabara M, et al: A case of angiomatous hamartoma of the tongue. Med J Osaka Univ (Japanese Edition) 43:231, 1984 13. Stamm C, Tauber R: Hamartoma of the tongue. Laryngoscope 55:140, 1945 14. Bhaskar SN, (ed): Orben’s Oral Histology and Embryology. St Louis, MO, Mosby, 1976, p 21 15. Duncan H: Tumor of the tongue. J Tenn Med Assoc 61:48, 1968 16. Perri FA: Myoepithelial hamartoma of the tongue. Arch Otolaryngol 64:289, 1956 17. Hinshaw CT: Unusual lesions of the tongue: Hamartoma. J Kans Med Soc 64:156, 1963