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Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2013) xxx–xxx

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Journal of Oral and Maxillofacial Surgery,

Medicine, and Pathology
journal homepage: www.elsevier.com/locate/jomsmp

Case report

A case of angiomyolipoma of the lower lip

Motoki Nakabayashi a,b,∗,1 , Isamu Kodani a , Kazuko Takubo a ,
Kazunori Kidani a , Hiroatsu Sakai a , Kazuo Ryoke a
a
Division of Oral and Maxillofacial Biopathological Surgery, Department of Medicine of Sensory and Motor Organs, Tottori University Faculty of Medicine, Yonago 683-8504, Japan
b
Department of Oral and Maxillofacial Surgery Matsue Red Cross Hospital, 200 Horo-machi, Matsue, Shimane 690-8506, Japan

a r t i c l e i n f o a b s t r a c t

Article history: Angiomyolipoma is a benign tumor that most commonly forms in the kidney, and rarely occurs in the
Received 26 January 2012 oral region. Angiomyolipoma is composed of smooth muscle cells, blood vessels and mature fat cells.
Received in revised form 5 February 2013 Because the three principal components in angiomyolipoma vary greatly in proportion and distribution,
Accepted 13 February 2013
its heterogeneity may cause diagnostic confusions.
We report a case of angiomyolipoma arising in the lower lip of man in his 50s. Histopathological analy-
Keywords:
sis of the tumor revealed three mesenchymal components of smooth muscle, blood vessel and mature fat
Angiomyolipoma
cells. Immunohistochemical examination showed that the tumor cells of the spindle cell were positive for
Lower lip
Immunohistochemistry
alpha smooth muscle actin, desmin, and vimentin, but negative for S-100 protein and HMB-45. The endo-
capillary cells were positive for CD34. The adipose cells were positive for S-100 protein. Angiomyolipoma
occurring in the head and neck do not express HMB-45, in contrast to the usual immunopositivity shown
in angiomyolipomas from the kidney and liver, suggesting that there are differences among them.
These histological and immunohistochemical analyses resulted in a final diagnosis of angiomyolipoma.

© 2013 Asian Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd.
All rights reserved.

1. Introduction 2. Patient report

Angiomyolipoma is a benign tumor that most commonly forms
in the kidney. Angiomyolipoma is a neoplasm composed of varying
admixtures of smooth muscle cells, blood vessels and mature fat
cells [1]. One third or more of patients with angiomyolipomas in the
kidney have the tuberous sclerosis complex (TSC). The association
of tuberous sclerosis with autosomal dominant disorder caused by
mutation of either the TSC1 or TSC2 genes. Angiomyolipoma of the
oral region is rare, and there are only 14 cases reported (Table 1)
[2–14].
We report a case of angiomyolipoma located in the lower lip,
and discuss its histological and immunohistochemical features.
Abbreviation: ␣-SMA, alpha smooth muscle actin.
∗ Corresponding author at: Division of Oral and Maxillofacial Biopathological
Surgery, Department of Medicine of Sensory and Motor Organs, Tottori Univer-
sity Faculty of Medicine, Tottori University, 86 Nishi-cho, Yonago, Tottori 683-8503,
Japan. Tel.: +81 859 38 6687; fax: +81 859 38 6689.
E-mail addresses: m-nakabayashi@med.tottori-u.ac.jp,
nakabayashi motoki@matsue.jrc.or.jp (M. Nakabayashi).
1
Department of Oral and Maxillofacial Surgery Matsue Red Cross Hospital 200
Horo-machi, Matsue, Shimane 690-8506, Japan. Tel: +81 852 24 2111;
Fax: +81 852 31 9783.
2.1. Clinical summary
A Japanese man in his 50s was admitted to the Clinic of Oral
and Maxillofacial Surgeons at Tottori University Hospital due to a
painless swelling in his left lower lip. The lesion was first noticed
20 years ago and had gradually enlarged without pain. A physical
examination revealed a circumscribed nodule of 23 mm × 15 mm.
The tumor surface is smooth and slightly dark red. The tumor is elas-
tic soft overall. However, part of tumor is elastic hard. The tumor
showed a little discoloration in the pressure (Fig. 1). Initially, his
disease was clinically suspected as angioma. However, the proba-
bility of other benign tumors could not be ruled out so the patient
underwent surgery. At operation the mass was well-demarcated
and easily dissected. The excised tumor showed a circular, well-
circumscribed mass measuring 18 mm × 10 mm × 12 mm (Fig. 2).
There has been no recurrence after surgery.
2.2. Pathological findings
Histopathologic examination revealed that the tumor, which
was encapsulated, was composed of thick walled blood vessels,
bundles of intensely eosinophilic spindle-cells, and small group
of mature fat cells in membrane. The blood vessels wall of the

2212-5558/$ – see front matter © 2013 Asian Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ajoms.2013.02.003

Please cite this article in press as: Nakabayashi M, et al. A case of angiomyolipoma of the lower lip. J Oral Maxillofac Surg Med Pathol (2013),
http://dx.doi.org/10.1016/j.ajoms.2013.02.003
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Table 1
Clinical characteristics of oral angiomyolipomas.

First author Reference no. Age Gender Site Size (cm) Duration Recurrence

Gutmann 2 39 M Hard palate 1.0 2y No

Komiya 3 74 F Buccal mucosa 4.0 × 3.0 20y No
Iwai 4 66 F Upper lip 2.2 × 1.2 19y No
Miyazaki 5 66 M Hard palate 1.5 × 1.0 2m No
Yamamoto 6 62 F Hard palate 1.0 Na No
69 F Lower lip 1.0 × 0.7 10y No
Ide 7 60 F Tongue 0.7 × 0.5 1y No
Piattelli 8 43 M Hard palate Na 10m Na
Redman 9 71 M Lower lip 2.0 × 1.0 Na Na
Lopez–Lopez 10 55 F Buccal mucosa and lower lip 0.6 Na Na
Alvarez Alvarez 11 52 M Hard palate 0.6 Na No
da Silva 12 43 F Upper lip 2 6y No
Koizumi 13 23 M Tongue 0.8 × 0.6 2y No
Konstantinos 14 78 M Upper lip 0.7 1y No

M: male; F: female; Na: not available; No: not observed.

tumor demonstrated thickened spindle-cells and elastic fibers. In
addition, spindle-cells were proliferation and scattered around
the blood vessels’ walls. Spindle-cells were many vascular mus-
cle cells than non-vascular muscle cells. Nodules consist of mature
fat cells separated by a branching network of spindle-cells. No
mitotic figures, nuclear pleomorphism, epithelioid cells, or imma-
ture adipocytes were evident (Fig. 3).
Immunohistochemical examination showed that the tumor
cells of the spindle cell were positive for alpha smooth muscle actin
(␣-SMA), desmin, HHF-35 and vimentin, but negative for S-100 pro-
tein and HMB-45. The endocapillary cells were positive for CD34,
␣-SMA, desmin, HHF-35 and vimentin. The adipose cells were pos-
itive for S-100 protein (Fig. 4). From these findings, the tumor was
diagnosed as a angiomyolipoma.
disability. Tuberous sclerosis is caused by a mutation of either of
two genes, TSC1 and TSC2, which code for the proteins hamartin
and tuberin respectively [3,16,17]. Patient was not underwent test-
ing of the electroencephalography. However, patient did not have
past history of nerve disorders and symptoms of facial sebaceous
adenoma. Thus, the patient denied a complication of tuberous scle-
rosis. In previous reports of oral region, tuberous sclerosis did not
develop [2–14].
Previous reports of oral region in 14 cases only, site of
occurrence is diverse as palate, buccal mucosa, lips, tongue. A well-
circumscribed round appearance, no invasion is the most common
clinical findings. However, the difference in the percentage of tissue
components, make a difference in elastic or color [4,5]. In clinical

3. Discussion

Angiomyolipoma is a benign non-epithelial tumor that is com-

posed of smooth muscle cells, blood vessels and mature fat cells.
Angiomyolipoma was considered to represent either a hamartoma
or a choristoma in the past, but is now accept as a neoplasm. The
evidence for a neoplasm includes monoclonality and the presence
of a variety of cytogenetic abnormalities. The perivascular epithe-
lioid cell is considered to be the cell of origin for this and other
related tumors and tumor-like processes [15].
Angiomyolipoma are the most common benign tumor of the
kidney and frequently occurs in people of over 40 years of age [1].
Tuberous sclerosis is a disease characterized by three main symp-
toms of facial sebaceous adenoma, episode epilepsy and learning

Fig. 1. The lesion of the lower lip at first clinical presentation. The tumor surface is Fig. 2. Photograph of a tumor on the lower lip at excised tumor. (A) The excised
smooth surface and slightly dark red. (For interpretation of the references to colour tumor showed a circular, well-circumscribed mass (B) the cut surface of excised
in this figure legend, the reader is referred to the web version of this article.) tumor showed solid and extensive vascular component.

Please cite this article in press as: Nakabayashi M, et al. A case of angiomyolipoma of the lower lip. J Oral Maxillofac Surg Med Pathol (2013),
http://dx.doi.org/10.1016/j.ajoms.2013.02.003
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anisocytosis was described. Groups of mature adipocytes are not

Fig. 3. Histopathology of the angiomyolipoma (hematoxylin and eosin stain). The
tumor was composed of an admixture of spindle-cells, blood vessels, and mature fat
cells (original magnification, 40×).
diagnosis, angiomyolipoma is sometimes confused with Angioma,
angiolipoma, angioleiomyoma, fibrous lipoma, schwannoma and
neurofibroma. Angiomyolipoma is difficult to distinguish by clin-
ical findings, because angiomyolipoma had a broad spectrum of
clinical manifestation. Initially, we diagnosed hemangioma on the
basis of clinical findings. Also previous reports were diagnosed by
histological and immunohistochemical analyses.
Histopathological analysis of the tumor revealed three mes-
enchymal components of blood vessels bundles of spindle cells,
and mature fat cells. The present patient demonstrated fat cells in
tumor. On the contrary, hemangiomas is usually demarcated fatty
tissue from tumor [5]. Angiolipoma is composed of blood vessels
and fat tissue, and does not present bundles of smooth muscle pro-
liferation. Fat cells of previously reported oral angiomyolipomas
were also mature. They were found singly or in small groups and
uncommon in angiomyomas. Angiomyomas reports were possible
that small groups of adipose tissue have been overlooked, as they
should not be of diagnostic importance [14].
Immunohistochemical examination showed that the tumor
cells of the spindle cell were positive for ␣-SMA, desmin, and
vimentin, but negative for S-100 protein and HMB-45. Desmin neg-
ativity in some oral angiomyolipoma was considered indicative
of perivascular origin of the smooth muscle component. However
desmin was positive that was lower than the positive rate of ␣-
SMA. Heterogeneity for desmin is common in smooth muscle cells
of blood vessels and is contributed to the antibodies applied, or the
size and the anatomic location of the vessels [14]. Thus, spindle
cells had been considered smooth muscle cells. Several investiga-
tors have reported that smooth muscle cells of angiomyolipoma
in the kidney show a unique pattern of immunoreactivity, with
coexpression of muscle-specific markers (muscle-specific actin and
smooth muscle actin) as well as melanocytic markers such as
HMB-45 and melan-A [19,20]. In previous reports of oral region,
Angiomyolipoma occurring in the head and neck do not express
HMB-45 [7–14]. The pathogenesis of HMB-45 expression is not
yet clear. In previous reports suggested three hypotheses. First,
HMB-45 expression of angiomyolipomas might show an associ-
ation with tuberous sclerosis [21]. Second, HMB-45 expression
might be useful in distinguishing angiomyolipoma with epithe-
lioid components from other tumors. But it might be not useful in
distinguishing angiomyolipoma without epithelioid components
from other tumors [22]. Third, percentage of fat that might be
affects the HMB-45. Because, clear cells may represent perivascular
epithelioid cells undergoing lipoid degeneration [14,23]. Further
study will be needed to consider the expression of HMB-45 on
angiomyolipoma. The endocapillary cells were positive for CD34.
The adipose cells were positive for S-100 protein. These histological
and immunohistochemical analyses resulted in a final diagnosis of
angiomyolipoma. A surgical excision is considered curative, as this
tumor usually behaves in benign fashion [5–7].

Fig. 4. Immunohistochemistry of the angiomyolipoma. (A) The tumor cells of the spindle cell, myoid cell and endocapillary cell show immunoreactivity for desmin (original
magnification, 100×). (B) The tumor cells of the spindle cell and endocapillary cell show immunoreactivity for Alpha smooth muscle actin (original magnification, 100×). (C)
The tumor cells of the endocapillary cell show immunoreactivity for CD34 (original magnification, 100×). (D) The tumor cells of the adipose cell show immunoreactivity for
S-100 protein (original magnification, 200×).

Please cite this article in press as: Nakabayashi M, et al. A case of angiomyolipoma of the lower lip. J Oral Maxillofac Surg Med Pathol (2013),
http://dx.doi.org/10.1016/j.ajoms.2013.02.003
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4 M. Nakabayashi et al. / Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2013) xxx–xxx
We present a patient with angiomyolipoma classified as a
rare benign tumor that was difficult to differentiate from other
tumors. We emphasize the importance of immunohistochemistry
for obtaining an accurate diagnosis of angiomyolipoma.
Acknowledgments
We are grateful to Kohei Shomori, M.D. (Division of Organ
Pathology, Department of Microbiology and Pathology, Tottori Uni-
versity Faculty of Medicine), for assistance with the histochemistry.
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