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Angioleiomyoma of the hard palate: Report of a case and review of literature
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
Contents lists available at SciVerse ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case report
Angioleiomyoma of the hard palate: Report of a case and review of literature夽 Seiko Tatehara a,∗ , Toru Sato a , Kenji Mishima b , Ichiro Saito b , Kazuhito Satomura a a Department of Oral Medicine and Stomatology, Second Department of Oral and Maxillofacial Surgery, School of Dental Medicine Tsurumi University, 2-1-3 Tsurumi, Tsurumi-ku, Yokohama 230-8501, Japan b Department of Pathology, School of Dental Medicine, Tsurumi University, 2-1-3 Tsurumi, Tsurumi-ku, Yokohama 230-8501, Japan
a r t i c l e
i n f o
Article history: Received 14 March 2012 Received in revised form 12 April 2012 Accepted 7 May 2012 Available online 15 June 2012 Keywords: Angioleiomyoma Magnetic resonance image finding Immunohistochemistry Hard palate
a b s t r a c t Angioleiomyoma is a benign soft tissue tumor rarely observed in oral cavity. We report a case of angioleiomyoma in the midline of the hard palate. A 55-year-old Japanese male was referred to our hospital with a tumor in the midline of the hard palate. The tumor, measuring 10 mm × 7 mm × 6 mm, was observed as a pedunculated and elasticity mass. Magnetic resonance images revealed homogeneously enhanced neoplasm. Under a clinical diagnosis of a benign tumor of the hard palate, the lesion was resected surgically with inclusion of healthy tissue under local anesthesia. Histological examination revealed that the tumor consisted mainly of a proliferation of spindle cells resembling vascular smooth muscle cell. Immunohistochemically, the tumor cells were positive for desmin, ␣-smooth muscle actin and muscle-specific actin (HHF-35) and negative for S-100 protein. From these findings, the tumor was diagnosed as an angioleiomyoma. There has been no recurrence for 1 year after the operation. © 2012 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.
1. Introduction Angioleiomyoma is a benign neoplasm arising from the vascular smooth muscle tunica media and presents commonly in the lower limbs of middle-aged population, rarely occurs in the oral region [1]. Pre-operative diagnosis of oral angioleiomyoma is difficult, because this tumor has no clinically diagnostic characters or symptoms. We report a case of angioleiomyoma in the midline of the hard palate and review the scientific literature from 1980 to 2011. 2. Case report A 55-year-old Japanese male was referred to our clinic for a tumor located on the middle hard palate. He had noticed a small mass without any symptoms in the hard palate for 7 years, and the mass showed gradual growth recently. His medical history was noncontributory. A clinical examination revealed the presence of a pedunculated round elastic soft red-purple mass of 10 mm in diameter in the nearly midline of the hard palate (Fig. 1). The lesion did not
夽 AsianAOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathology; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants. ∗ Corresponding author. Tel.: +81 45 580 8389; fax: +81 45 573 9599. E-mail address: [email protected] (S. Tatehara).
blanch with pressure and negative to diascopy. Magnetic resonance imaging (MRI) showed a well-circumscribed mass on the hard palate, with heterogeneous hypointensity on T1-weighted and hyperintensity on T2-weighted images (Fig. 2). The tumor located on the hard palate displaced slightly the palatal bone (Fig. 2D). With a clinical diagnosis of a benign tumor of the palate, the tumor with the inclusion of peripheral healthy soft tissue including periosteum with 1 mm of surgical margin was resected under local anesthesia. After the removal, bare palatal bone was covered with TERUDERMIS® (Olympus Terumo Biomaterials Corp., Tokyo, Japan). The excised specimen was an elastic soft red-purple mass, 16 mm × 12 mm × 10 mm in size, with encapsulation by fibrous tissue. A histopathological examination revealed a wellcircumscribed mass encapsulated by a thin connective tissue layer. The tumor was composed of abundant blood vessels with thick muscular wall consisting of smooth muscle cells and small clusters of mature fat cells (Fig. 3). Interlacing smooth muscle and collagen fibers were noted between vascular spaces. Immunohistochemical analysis revealed that the spindle smooth muscle cells were positive for desmin, ␣-smooth muscle actin (SMA) and muscle-specific actin (HHF-35) and negative for S-100 protein (Fig. 4). In addition, the endothelial cells of vascular spaces in the tumor were positive for CD34 (Fig. 4D). Taken together, the final diagnosis was angioleiomyoma with an adipocytic component. The patient has showed no functional disturbance or no evidence of local recurrence during 1 year of follow-up.
2212-5558/$ – see front matter © 2012 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ajoms.2012.05.002
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
Fig. 1. Intraoral photograph showing a round red-purple mass in the midline of the hard palate. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
283
Fig. 3. Hematoxylin and eosin staining. Tumor composed of abundant blood vessels with thick muscular wall consisting of smooth muscle cells and small clusters of mature fat cells (original magnification 100×).
3. Discussion Leiomyoma is a benign mesenchymal tumor characterized by the proliferation of smooth muscle cells [1]. They are histologically classified into 3 groups: solid leiomyoma, angioleiomyoma, and epithelioid leiomyoma [2,3]. Angioleiomyoma is usually found in the lower extremities and rarely observed in oral cavity. In a series of 562 angioleiomyoma reviewed by Hachisuga et al. [1], only 15 (2.7%) occurred in the oral cavity. The region of occurrence of oral angioleiomyomas was lips (48.6%), hard palate (9.2%), tongue (9.2%) and buccal mucosa (9.2%) by a retrospective research using 76,412
oral biopsies accessioned from 1963 to 2001 [4]. So as to the origin of oral angioleiomyoma, it has been proposed that blood vessels may be the origin of oral angioleiomyoma, because the oral cavity is rich in blood vessels [5]. We could retrieve 12 cases of angioleiomyoma located in the hard palate reported between 1980 and 2011, including in our case. The age of patients ranged from 24 to 67 years and the mean age was 50.2 years (Table 1). Considering gender, no sex difference was noted in palatal angioleiomyoma. In contrast, oral
Fig. 2. MR image showing a 15 mm × 10 mm mass in the middle of the hard palate, which slightly displaces the palatal bone.
284
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
Fig. 4. Immunohistochemical analysis of the tumor for (A) ␣-smooth muscle actin (SMA), (B) desmin, (C) muscle-specific actin (HHF-35), and (D) CD34 (DAB-hematoxylin, original magnification 200×). The tumor is noted to be positive for SMA and HHF-35; negative for S-100 protein. The endothelial cells in the tumor are positive for CD34.
angioleiomyoma exhibits a slightly higher prevalence in males than females, yielding a man to female ratio of 1.43:1 [6]. It takes long time 12–132 months, 53.6 month on average for patients with angioleiomyoma to be seen in a clinic, because most angioleiomyomas are characterized as only painless and slowly growing mass. In the
present case, the patient has had the lesion in the hard palate for 7 years since he noticed the mass. The clinical differential diagnosis is very difficult because of its non-specific clinical appearance. Angioleiomyomas in the palate appear as painless, slowly enlarging, round, elevated and sessile
Table 1 Clinical data of palate angioleiomyomas reported from 1980 to 2011. Case no. authors
Age
Gender
Size (mm)
Symptoms
Duration
Consistency
Shape
Clinical diagnosis
Recurrence
1 Davis [23]
62
M
11 × 15
Swelling
7 years
Soft
Blue-purple Submucosal swelling
–
2 Natiella [6] 3 Hemani [24]
50 40
F M
12 × 10 × 5 80 × 50
– Swelling
– 4 months
Firm Soft-to-firm
4 Savage [25]
66
M
10
–
–
Firm
Elevated Smooth-surfaced Sessile swelling Mucosa overlying
Pleomorphic adenoma or Mucoepider-moid tumor – –
5 Svane [8]
37
F
10
Lump
9 months
Firm
6 Esguep [26]
48
F
10 × 10 × 6
–
–
–
Erythematous, large semi-pedunculated swelling
7 Esguep [26]
24
F
10 × 10 × 7
–
–
–
swelling
8 Brooks [4]
58
F
–
–
–
9 Al-Amand [27]
60
M
10 × 8 × 5
Occasional ulceration Painful swelling
5 years
–
10 Scheper [7]
67
M
5×5
11 years
Compressible
Raised, slightly pedunculated Raised, slightly pedunculated Raised, round reddish blue
11 Grossmann Sde [3] 12 Present case
35
F
10 × 10 × 5
2 months
Firm
55
M
10 × 7 × 6
7 years
Soft
M: male, F: female.
Non-painful swelling Occasional ulceration Swelling
9 months –
Minor salivary gland tumor Fibroma
–
Pleomorphic adenoma Vascular leiomyoma Fibroma
–
–
Fibroma
–
Excisional biopsy
–
Well-circumscribed
–
–
Well-circumscribed, pedunculated
–
–
2 weeks
–
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
nodules [1,4,7,8]. The clinical appearance of angioleiomyoma resembles fibroma, neurofibroma, neurileimoma, hemangioma and pleomorphic adenoma in shape and symptom [9]. Reports showed that diagnosis before operation were vascular leiomyoma (angioleiomyoma) 8.3% (1/12), fibroma 25.0% (3/12), and minor salivary gland tumor including pleomorphic adenoma 33.3% (4/12) (Table 1). Fibroma is an overgrowth of connective tissue and is a round to ovoid, asymptomatic, smooth-surfaced, and firm sessile or pedunculated mass [10]. Neurilemmoma (schwannoma) is a benign, encapsulated perineural tumor of neuroectodermal derivation that originates from the Schwann cells and round to ovoid, hard or pedunculated mass under mucosa [11]. Neurofibroma is the most common peripheral nerve neoplasm and can arise as a solitary tumor or a component of neurofibromatosis. Solitary neurofibroma appears a slow-growing, soft and painless lesion similar to neurofibroma [12]. Hemangiomas are tumors identified by rapid endothelial cell proliferation. In general, clinical finding shows that hemangiomas are soft and red-purple tumor, following the depth from the surface of mucous membrane and the property of blood vessel [13]. Pleomorphic adenomas are the most common tumor of the salivary glands [3]. The clinical appearance is a round, smooth to firm mass with a thin, delicate, incomplete capsule [9]. In addition, the tumor with cartilaginous tissue formation is hard and the tumor including much mucosa tissue is soft. This present case appeared as a soft red-purple mass, but did not blanch with pressure. From clinical findings, hemangioma was excluded from our differential diagnosis. MRI examination might be helpful to make diagnosis of angioleiomyomas because angioleiomyomas have characteristic findings on MRIs. Images show an isointense to slightly hyperintense signal compared with muscle on T1-weighted images, mixed hyper- and iso-intensity areas with a hypointense rim corresponding to a fibrous capsule on T2-weighed images [14,15]. MRI findings of neurofibroma reported that low-to-intermediate signal intensity on T1-weighted images and heterogeneity on T2-weighted images [16]. Neurilemmomas have very distinctive appearances of a target sign on a peripheral nerve on MRIs. MRI findings enable us to except neurofibroma and neurilemmoma from our differential diagnosis. In contrast, pleomorphic adenomas have findings similar to angioleiomyomas, presenting a predilection for homogeneous intermediate signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted images [17]. Diagnosis of angioleiomyoma is principally histological [18,19]. The angioleiomyomas are classified into 3 subtypes according to their dominant histological pattern: solid, venous, or cavernous [1,20]. The solid-type is composed of compacted bundles of smooth muscle cells and thin-walled vessels, the venous type shows thick walls of the vascular channels and blending of the vascular bundles, and the cavernous-type is characterized by large vascular channels with thin muscular walls [1,15]. There can be also a mixture of patterns, leading to biphasic lesions [1]. This case was wellcircumscribed and sometimes surrounded by a complete capsule and showed the presence of abundant of vascular spaces surrounded by thick muscular walls composed of circumferentially arranged smooth muscle cells (Fig. 3). From these findings, this case was histologically diagnosed as venous–cavernous type. Angioleiomyoma may resemble histologically spindle cell benign tumors such as neurofibroma, palisaded encapsulated neuroma, neurilemmoma, and solitary fibrous tumor [4,21]. The immunohistochemistry might be very important for histopathological evaluation to contribute to differential diagnosis [4,21]. In this case, immunohistochemical analysis for desmin, SMA, HHF35, S-100 protein and CD34 was performed. SMA is a specific immunomarker of smooth muscle. Desmin is a subunit of intermediate filaments in skeletal muscle tissue, smooth muscle tissue, and cardiac muscle tissue and is one of the earliest protein markers
285
for muscle tissues. HHF-35 is a smooth muscle marker and has been known to be more specific for angioleiomyoma. The immunohistochemical evaluation for these molecules is suggested to be useful to diagnose myogenic tumors. In addition, the negative immunohistochemical staining for S-100 protein could exclude neurogenic tumor, because this marker is positive in tumors of neural lineage [12]. The literature showed that the typical immunohistochemical phenotype of angioleiomyoma is positive for SMA, HHF-35 and desmin, and negative for S-100 protein [4,22]. The treatment of choice for oral angioleiomyoma is surgical excision. In this case, we performed an excisional biopsy because the size of tumor is small and the tumor dose not express any malignant findings. Despite the vascular nature of angioleiomyoma, profuse bleeding during removal has been rarely seen [4]. In this case, the lesion was located in the region with abundant vascularization and the tumor expanded the palate bone. We resected the tumor with safety margins, including periosteum. The bleeding was easily controllable by electrosurgical unit. Although the recurrence of oral angioleiomyoma is rarely seen, 2 cases of angioleiomyoma of the hard palate were reported to have recurrence at 2 weeks and 9 months after surgery (Table 1). This fact suggests that a careful follow up should be taken at least about one year after surgery. References [1] Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126–30. [2] Damm DD, Neville BW. Oral leiomyomas. Oral Surg Oral Med Oral Pathol 1979;47:343–8. [3] Grossmann Sde M, Johann AC, Castro WH, Friedman H, Gomez RS, Mesquita RA. Anterior midline nodule of the hard palate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:808–11. [4] Brooks JK, Nikitakis NG, Goodman NJ, Levy BA. Clinicopathologic characterization of oral angioleiomyomas. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:221–7. [5] Gaitan Cepeda LA, Quezada Rivera D, Tenorio Rocha F, Leyva Huerta ER, Mendez Sánchez ER. Vascular leiomyoma of the oral cavity. Clinical, histopathological and immunohistochemical characteristics. Presentation of five cases and review of the literature. Med Oral Patol Oral Cir Bucal 2008;13:E483–8. [6] Natiella JR, Neiders ME, Greene GW. Oral leiomyoma. Report of six cases and a review of the literature. J Oral Pathol 1982;11:353–65. [7] Scheper MA, Nikitakis NG, Meiller TF. A stable swelling of the hard palate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:461–4. [8] Svane TJ, Smith BR, Cosentino BJ, Cundiff EJ, Ceravolo Jr JJ. Oral leiomyomas. Review of the literature and report of a case of palatal angioleiomyoma. J Periodontol 1986;57:433–5. [9] Cerulli G, Renzi G, Perugini M, Becelli R. Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate. J Craniofac Surg 2004;15:1056–60. [10] Barker DS, Lucas RB. Localised fibrous overgrowths of the oral mucosa. Br J Oral Surg 1967;5:86–92. [11] Williams HK, Cannell H, Silvester K, Williams DM. Neurilemmoma of the head and neck. Br J Oral Maxillofac Surg 1993;31:32–5. [12] Wright BA, Jackson D. Neural tumors of the oral cavity. A review of the spectrum of benign and malignant oral tumors of the oral cavity and jaws. Oral Surg Oral Med Oral Pathol 1980;49:509–22. [13] Ethunandan M, Mellor TK. Haemangiomas and vascular malformations of the maxillofacial region – a review. Br J Oral Maxillofac Surg 2006;44:263–72. [14] Hwang JW, Ahn JM, Kang HS, Suh JS, Kim SM, Seo JW. Vascular leiomyoma of an extremity: MR imaging – pathology correlation. Am J Roentgenol 1998;171:981–5. [15] Ramesh P, Annapureddy SR, Khan F, Sutaria PD. Angioleiomyoma: a clinical, pathological and radiological review. Int J Clin Pract 2004;58:587–91. [16] Hillier JC, Moskovic E. The soft tissue manifestations of neurofibromatosis type 1. Clin Radiol 2005;60:960–7. [17] Hisatomi M, Asaumi J, Yanagi Y, Konouchi H, Matsuzaki H, Honda Y, et al. Assessment of pleomorphic adenomas using MRI and dynamic contrast enhanced MRI. Oral Oncol 2003;39:574–9. [18] McParland H, Warnakulasuriya S, Cook RJ. Angioleiomyoma: an unusual diagnosis for a lump in the cheek. Br J Oral Maxillofac Surg 2009;47:641–2. ˜ Seijas B, Guitián [19] Luaces Rey R, Lorenzo Franco F, Gómez Oliveira G, Patino D, López-Cedrún Cembranos JL. Oral leiomyoma in retromolar trigone. A case report. Med Oral Patol Oral Cir Bucal 2007;12:E53–5. [20] Matsuyama A, Hisaoka M, Hashimoto H. Angioleiomyoma: a clinicopathologic and immunohistochemical reappraisal with special reference to the correlation with myopericytoma. Hum Pathol 2007;38:645–51. [21] Jordan RC, Regezi JA. Oral spindle cell neoplasmas: a review of 307 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:714–24.
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[22] Vered M, Allon I, Buchner A, Dayan D. Clinico-pathologic correlations of myofibroblastic tumors of the oral cavity. II. Myofibroma and myofibromatosis of the oral soft tissues. J Oral Pathol Med 2007;36:304–14. [23] Davis GB. Angiomyoma of the palate. Int J Oral Surg 1980;9:484–5. [24] Hemani DD, Gupta AK, Sharma KK, Sharma SD. Leiomyoma of the palate. J Laryngol Otol 1983;97:471–7.
[25] Savage NW, Adkins KF, Young WG, Chapman PJ. Oral vascular leiomyoma: review of the literature and report of two cases. Aust Dent J 1983;28:346–51. [26] Esguep A, Solar M. Oral vascular leiomyoma – report of 5 cases and review of the literature. J Oral Med 1986;41:126–9. [27] Al-Amad SH, Angel C, O’Grady JF, McCullough MJ. Angioleiomyoma on the hard palate. Oral Oncol Extra 2006;42:244–6.
Contents lists available at SciVerse ScienceDirect
Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology journal homepage: www.elsevier.com/locate/jomsmp
Case report
Angioleiomyoma of the hard palate: Report of a case and review of literature夽 Seiko Tatehara a,∗ , Toru Sato a , Kenji Mishima b , Ichiro Saito b , Kazuhito Satomura a a Department of Oral Medicine and Stomatology, Second Department of Oral and Maxillofacial Surgery, School of Dental Medicine Tsurumi University, 2-1-3 Tsurumi, Tsurumi-ku, Yokohama 230-8501, Japan b Department of Pathology, School of Dental Medicine, Tsurumi University, 2-1-3 Tsurumi, Tsurumi-ku, Yokohama 230-8501, Japan
a r t i c l e
i n f o
Article history: Received 14 March 2012 Received in revised form 12 April 2012 Accepted 7 May 2012 Available online 15 June 2012 Keywords: Angioleiomyoma Magnetic resonance image finding Immunohistochemistry Hard palate
a b s t r a c t Angioleiomyoma is a benign soft tissue tumor rarely observed in oral cavity. We report a case of angioleiomyoma in the midline of the hard palate. A 55-year-old Japanese male was referred to our hospital with a tumor in the midline of the hard palate. The tumor, measuring 10 mm × 7 mm × 6 mm, was observed as a pedunculated and elasticity mass. Magnetic resonance images revealed homogeneously enhanced neoplasm. Under a clinical diagnosis of a benign tumor of the hard palate, the lesion was resected surgically with inclusion of healthy tissue under local anesthesia. Histological examination revealed that the tumor consisted mainly of a proliferation of spindle cells resembling vascular smooth muscle cell. Immunohistochemically, the tumor cells were positive for desmin, ␣-smooth muscle actin and muscle-specific actin (HHF-35) and negative for S-100 protein. From these findings, the tumor was diagnosed as an angioleiomyoma. There has been no recurrence for 1 year after the operation. © 2012 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.
1. Introduction Angioleiomyoma is a benign neoplasm arising from the vascular smooth muscle tunica media and presents commonly in the lower limbs of middle-aged population, rarely occurs in the oral region [1]. Pre-operative diagnosis of oral angioleiomyoma is difficult, because this tumor has no clinically diagnostic characters or symptoms. We report a case of angioleiomyoma in the midline of the hard palate and review the scientific literature from 1980 to 2011. 2. Case report A 55-year-old Japanese male was referred to our clinic for a tumor located on the middle hard palate. He had noticed a small mass without any symptoms in the hard palate for 7 years, and the mass showed gradual growth recently. His medical history was noncontributory. A clinical examination revealed the presence of a pedunculated round elastic soft red-purple mass of 10 mm in diameter in the nearly midline of the hard palate (Fig. 1). The lesion did not
夽 AsianAOMS: Asian Association of Oral and Maxillofacial Surgeons; ASOMP: Asian Society of Oral and Maxillofacial Pathology; JSOP: Japanese Society of Oral Pathology; JSOMS: Japanese Society of Oral and Maxillofacial Surgeons; JSOM: Japanese Society of Oral Medicine; JAMI: Japanese Academy of Maxillofacial Implants. ∗ Corresponding author. Tel.: +81 45 580 8389; fax: +81 45 573 9599. E-mail address: [email protected] (S. Tatehara).
blanch with pressure and negative to diascopy. Magnetic resonance imaging (MRI) showed a well-circumscribed mass on the hard palate, with heterogeneous hypointensity on T1-weighted and hyperintensity on T2-weighted images (Fig. 2). The tumor located on the hard palate displaced slightly the palatal bone (Fig. 2D). With a clinical diagnosis of a benign tumor of the palate, the tumor with the inclusion of peripheral healthy soft tissue including periosteum with 1 mm of surgical margin was resected under local anesthesia. After the removal, bare palatal bone was covered with TERUDERMIS® (Olympus Terumo Biomaterials Corp., Tokyo, Japan). The excised specimen was an elastic soft red-purple mass, 16 mm × 12 mm × 10 mm in size, with encapsulation by fibrous tissue. A histopathological examination revealed a wellcircumscribed mass encapsulated by a thin connective tissue layer. The tumor was composed of abundant blood vessels with thick muscular wall consisting of smooth muscle cells and small clusters of mature fat cells (Fig. 3). Interlacing smooth muscle and collagen fibers were noted between vascular spaces. Immunohistochemical analysis revealed that the spindle smooth muscle cells were positive for desmin, ␣-smooth muscle actin (SMA) and muscle-specific actin (HHF-35) and negative for S-100 protein (Fig. 4). In addition, the endothelial cells of vascular spaces in the tumor were positive for CD34 (Fig. 4D). Taken together, the final diagnosis was angioleiomyoma with an adipocytic component. The patient has showed no functional disturbance or no evidence of local recurrence during 1 year of follow-up.
2212-5558/$ – see front matter © 2012 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.ajoms.2012.05.002
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
Fig. 1. Intraoral photograph showing a round red-purple mass in the midline of the hard palate. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of the article.)
283
Fig. 3. Hematoxylin and eosin staining. Tumor composed of abundant blood vessels with thick muscular wall consisting of smooth muscle cells and small clusters of mature fat cells (original magnification 100×).
3. Discussion Leiomyoma is a benign mesenchymal tumor characterized by the proliferation of smooth muscle cells [1]. They are histologically classified into 3 groups: solid leiomyoma, angioleiomyoma, and epithelioid leiomyoma [2,3]. Angioleiomyoma is usually found in the lower extremities and rarely observed in oral cavity. In a series of 562 angioleiomyoma reviewed by Hachisuga et al. [1], only 15 (2.7%) occurred in the oral cavity. The region of occurrence of oral angioleiomyomas was lips (48.6%), hard palate (9.2%), tongue (9.2%) and buccal mucosa (9.2%) by a retrospective research using 76,412
oral biopsies accessioned from 1963 to 2001 [4]. So as to the origin of oral angioleiomyoma, it has been proposed that blood vessels may be the origin of oral angioleiomyoma, because the oral cavity is rich in blood vessels [5]. We could retrieve 12 cases of angioleiomyoma located in the hard palate reported between 1980 and 2011, including in our case. The age of patients ranged from 24 to 67 years and the mean age was 50.2 years (Table 1). Considering gender, no sex difference was noted in palatal angioleiomyoma. In contrast, oral
Fig. 2. MR image showing a 15 mm × 10 mm mass in the middle of the hard palate, which slightly displaces the palatal bone.
284
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
Fig. 4. Immunohistochemical analysis of the tumor for (A) ␣-smooth muscle actin (SMA), (B) desmin, (C) muscle-specific actin (HHF-35), and (D) CD34 (DAB-hematoxylin, original magnification 200×). The tumor is noted to be positive for SMA and HHF-35; negative for S-100 protein. The endothelial cells in the tumor are positive for CD34.
angioleiomyoma exhibits a slightly higher prevalence in males than females, yielding a man to female ratio of 1.43:1 [6]. It takes long time 12–132 months, 53.6 month on average for patients with angioleiomyoma to be seen in a clinic, because most angioleiomyomas are characterized as only painless and slowly growing mass. In the
present case, the patient has had the lesion in the hard palate for 7 years since he noticed the mass. The clinical differential diagnosis is very difficult because of its non-specific clinical appearance. Angioleiomyomas in the palate appear as painless, slowly enlarging, round, elevated and sessile
Table 1 Clinical data of palate angioleiomyomas reported from 1980 to 2011. Case no. authors
Age
Gender
Size (mm)
Symptoms
Duration
Consistency
Shape
Clinical diagnosis
Recurrence
1 Davis [23]
62
M
11 × 15
Swelling
7 years
Soft
Blue-purple Submucosal swelling
–
2 Natiella [6] 3 Hemani [24]
50 40
F M
12 × 10 × 5 80 × 50
– Swelling
– 4 months
Firm Soft-to-firm
4 Savage [25]
66
M
10
–
–
Firm
Elevated Smooth-surfaced Sessile swelling Mucosa overlying
Pleomorphic adenoma or Mucoepider-moid tumor – –
5 Svane [8]
37
F
10
Lump
9 months
Firm
6 Esguep [26]
48
F
10 × 10 × 6
–
–
–
Erythematous, large semi-pedunculated swelling
7 Esguep [26]
24
F
10 × 10 × 7
–
–
–
swelling
8 Brooks [4]
58
F
–
–
–
9 Al-Amand [27]
60
M
10 × 8 × 5
Occasional ulceration Painful swelling
5 years
–
10 Scheper [7]
67
M
5×5
11 years
Compressible
Raised, slightly pedunculated Raised, slightly pedunculated Raised, round reddish blue
11 Grossmann Sde [3] 12 Present case
35
F
10 × 10 × 5
2 months
Firm
55
M
10 × 7 × 6
7 years
Soft
M: male, F: female.
Non-painful swelling Occasional ulceration Swelling
9 months –
Minor salivary gland tumor Fibroma
–
Pleomorphic adenoma Vascular leiomyoma Fibroma
–
–
Fibroma
–
Excisional biopsy
–
Well-circumscribed
–
–
Well-circumscribed, pedunculated
–
–
2 weeks
–
S. Tatehara et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology 25 (2013) 282–286
nodules [1,4,7,8]. The clinical appearance of angioleiomyoma resembles fibroma, neurofibroma, neurileimoma, hemangioma and pleomorphic adenoma in shape and symptom [9]. Reports showed that diagnosis before operation were vascular leiomyoma (angioleiomyoma) 8.3% (1/12), fibroma 25.0% (3/12), and minor salivary gland tumor including pleomorphic adenoma 33.3% (4/12) (Table 1). Fibroma is an overgrowth of connective tissue and is a round to ovoid, asymptomatic, smooth-surfaced, and firm sessile or pedunculated mass [10]. Neurilemmoma (schwannoma) is a benign, encapsulated perineural tumor of neuroectodermal derivation that originates from the Schwann cells and round to ovoid, hard or pedunculated mass under mucosa [11]. Neurofibroma is the most common peripheral nerve neoplasm and can arise as a solitary tumor or a component of neurofibromatosis. Solitary neurofibroma appears a slow-growing, soft and painless lesion similar to neurofibroma [12]. Hemangiomas are tumors identified by rapid endothelial cell proliferation. In general, clinical finding shows that hemangiomas are soft and red-purple tumor, following the depth from the surface of mucous membrane and the property of blood vessel [13]. Pleomorphic adenomas are the most common tumor of the salivary glands [3]. The clinical appearance is a round, smooth to firm mass with a thin, delicate, incomplete capsule [9]. In addition, the tumor with cartilaginous tissue formation is hard and the tumor including much mucosa tissue is soft. This present case appeared as a soft red-purple mass, but did not blanch with pressure. From clinical findings, hemangioma was excluded from our differential diagnosis. MRI examination might be helpful to make diagnosis of angioleiomyomas because angioleiomyomas have characteristic findings on MRIs. Images show an isointense to slightly hyperintense signal compared with muscle on T1-weighted images, mixed hyper- and iso-intensity areas with a hypointense rim corresponding to a fibrous capsule on T2-weighed images [14,15]. MRI findings of neurofibroma reported that low-to-intermediate signal intensity on T1-weighted images and heterogeneity on T2-weighted images [16]. Neurilemmomas have very distinctive appearances of a target sign on a peripheral nerve on MRIs. MRI findings enable us to except neurofibroma and neurilemmoma from our differential diagnosis. In contrast, pleomorphic adenomas have findings similar to angioleiomyomas, presenting a predilection for homogeneous intermediate signal intensity on T1-weighted images, heterogeneous high signal intensity on T2-weighted images [17]. Diagnosis of angioleiomyoma is principally histological [18,19]. The angioleiomyomas are classified into 3 subtypes according to their dominant histological pattern: solid, venous, or cavernous [1,20]. The solid-type is composed of compacted bundles of smooth muscle cells and thin-walled vessels, the venous type shows thick walls of the vascular channels and blending of the vascular bundles, and the cavernous-type is characterized by large vascular channels with thin muscular walls [1,15]. There can be also a mixture of patterns, leading to biphasic lesions [1]. This case was wellcircumscribed and sometimes surrounded by a complete capsule and showed the presence of abundant of vascular spaces surrounded by thick muscular walls composed of circumferentially arranged smooth muscle cells (Fig. 3). From these findings, this case was histologically diagnosed as venous–cavernous type. Angioleiomyoma may resemble histologically spindle cell benign tumors such as neurofibroma, palisaded encapsulated neuroma, neurilemmoma, and solitary fibrous tumor [4,21]. The immunohistochemistry might be very important for histopathological evaluation to contribute to differential diagnosis [4,21]. In this case, immunohistochemical analysis for desmin, SMA, HHF35, S-100 protein and CD34 was performed. SMA is a specific immunomarker of smooth muscle. Desmin is a subunit of intermediate filaments in skeletal muscle tissue, smooth muscle tissue, and cardiac muscle tissue and is one of the earliest protein markers
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for muscle tissues. HHF-35 is a smooth muscle marker and has been known to be more specific for angioleiomyoma. The immunohistochemical evaluation for these molecules is suggested to be useful to diagnose myogenic tumors. In addition, the negative immunohistochemical staining for S-100 protein could exclude neurogenic tumor, because this marker is positive in tumors of neural lineage [12]. The literature showed that the typical immunohistochemical phenotype of angioleiomyoma is positive for SMA, HHF-35 and desmin, and negative for S-100 protein [4,22]. The treatment of choice for oral angioleiomyoma is surgical excision. In this case, we performed an excisional biopsy because the size of tumor is small and the tumor dose not express any malignant findings. Despite the vascular nature of angioleiomyoma, profuse bleeding during removal has been rarely seen [4]. In this case, the lesion was located in the region with abundant vascularization and the tumor expanded the palate bone. We resected the tumor with safety margins, including periosteum. The bleeding was easily controllable by electrosurgical unit. Although the recurrence of oral angioleiomyoma is rarely seen, 2 cases of angioleiomyoma of the hard palate were reported to have recurrence at 2 weeks and 9 months after surgery (Table 1). This fact suggests that a careful follow up should be taken at least about one year after surgery. References [1] Hachisuga T, Hashimoto H, Enjoji M. Angioleiomyoma. A clinicopathologic reappraisal of 562 cases. Cancer 1984;54:126–30. [2] Damm DD, Neville BW. Oral leiomyomas. Oral Surg Oral Med Oral Pathol 1979;47:343–8. [3] Grossmann Sde M, Johann AC, Castro WH, Friedman H, Gomez RS, Mesquita RA. Anterior midline nodule of the hard palate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2009;108:808–11. [4] Brooks JK, Nikitakis NG, Goodman NJ, Levy BA. Clinicopathologic characterization of oral angioleiomyomas. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:221–7. [5] Gaitan Cepeda LA, Quezada Rivera D, Tenorio Rocha F, Leyva Huerta ER, Mendez Sánchez ER. Vascular leiomyoma of the oral cavity. Clinical, histopathological and immunohistochemical characteristics. Presentation of five cases and review of the literature. Med Oral Patol Oral Cir Bucal 2008;13:E483–8. [6] Natiella JR, Neiders ME, Greene GW. Oral leiomyoma. Report of six cases and a review of the literature. J Oral Pathol 1982;11:353–65. [7] Scheper MA, Nikitakis NG, Meiller TF. A stable swelling of the hard palate. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:461–4. [8] Svane TJ, Smith BR, Cosentino BJ, Cundiff EJ, Ceravolo Jr JJ. Oral leiomyomas. Review of the literature and report of a case of palatal angioleiomyoma. J Periodontol 1986;57:433–5. [9] Cerulli G, Renzi G, Perugini M, Becelli R. Differential diagnosis between adenoid cystic carcinoma and pleomorphic adenoma of the minor salivary glands of palate. J Craniofac Surg 2004;15:1056–60. [10] Barker DS, Lucas RB. Localised fibrous overgrowths of the oral mucosa. Br J Oral Surg 1967;5:86–92. [11] Williams HK, Cannell H, Silvester K, Williams DM. Neurilemmoma of the head and neck. Br J Oral Maxillofac Surg 1993;31:32–5. [12] Wright BA, Jackson D. Neural tumors of the oral cavity. A review of the spectrum of benign and malignant oral tumors of the oral cavity and jaws. Oral Surg Oral Med Oral Pathol 1980;49:509–22. [13] Ethunandan M, Mellor TK. Haemangiomas and vascular malformations of the maxillofacial region – a review. Br J Oral Maxillofac Surg 2006;44:263–72. [14] Hwang JW, Ahn JM, Kang HS, Suh JS, Kim SM, Seo JW. Vascular leiomyoma of an extremity: MR imaging – pathology correlation. Am J Roentgenol 1998;171:981–5. [15] Ramesh P, Annapureddy SR, Khan F, Sutaria PD. Angioleiomyoma: a clinical, pathological and radiological review. Int J Clin Pract 2004;58:587–91. [16] Hillier JC, Moskovic E. The soft tissue manifestations of neurofibromatosis type 1. Clin Radiol 2005;60:960–7. [17] Hisatomi M, Asaumi J, Yanagi Y, Konouchi H, Matsuzaki H, Honda Y, et al. Assessment of pleomorphic adenomas using MRI and dynamic contrast enhanced MRI. Oral Oncol 2003;39:574–9. [18] McParland H, Warnakulasuriya S, Cook RJ. Angioleiomyoma: an unusual diagnosis for a lump in the cheek. Br J Oral Maxillofac Surg 2009;47:641–2. ˜ Seijas B, Guitián [19] Luaces Rey R, Lorenzo Franco F, Gómez Oliveira G, Patino D, López-Cedrún Cembranos JL. Oral leiomyoma in retromolar trigone. A case report. Med Oral Patol Oral Cir Bucal 2007;12:E53–5. [20] Matsuyama A, Hisaoka M, Hashimoto H. Angioleiomyoma: a clinicopathologic and immunohistochemical reappraisal with special reference to the correlation with myopericytoma. Hum Pathol 2007;38:645–51. [21] Jordan RC, Regezi JA. Oral spindle cell neoplasmas: a review of 307 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:714–24.
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