181 181 181 181
The Korean Journal of Pathology
2004; 38: 181-3
Angioleiomyoma of the sinonasal area is an extremely rare benign neoplasm. To the best of
our knowledge, only 26 cases have been described. Here, we report a case of angioleiomy-
oma arising in the nasal cavity of a 60-year-old woman. Microscopically, the tumor consisted
of proliferating smooth muscle cells punctuated with thick-walled vessels with slit-like lumina.
The tumor was negative for estrogen and progesterone receptor by immunohistochemical
study. Further studies are needed to clarify whether the growth of this tumor is sex steroid-
Key Words : Angioleiomyoma-Nasal Cavity-Steroid Receptor
Su Jin Kim∙
Mee Sook Roh
∙Sook Hee Hong
Angioleiomyoma of the Nasal Cavity
- A Case Report -
Mee Sook Roh, M.D.
Department of Pathology, Dong-A University College
of Medicine 1, 3-ga, Dongdaeshin-dong, Seo-gu,
Busan 602-713, Korea
Department of Pathology, Dong-A
University College of Medicine, Busan,
Received : January 19, 2004
Accepted : April 27, 2004
Angioleiomyoma (vascular leiomyoma), a solitary form of
leiomyoma that usually occurs in the subcutis, is composed of
numerous thick-walled vessels. Moreover, nasal angioleiomyomas
are extremely rare, and account for less than 1% of all vascular
leiomyoma.1To the best of our knowledge, only 25 cases of angi-
oleiomyomaarising in the nasal cavity and paranasal sinuses have
been described in the English literature.2In Korea, only one case
of nasal angioleiomyoma has been previously reported.3Herein,
we report a case of angioleiomyoma of the nasal cavity, and briefly
discuss the significance of sex steroid receptors and include reviews
of relevant literature.
A 60-year-old woman presented with a two-month history of
intermittent rhinorrhea and sneezing. Endoscopic findings showed
a polypoid tumor originating from the right inferior turbinate.
The tumor was in close contact with the mucosa without evidence
of ulceration. No other abnormalities in the nasopharynx, oral
cavity, larynx or ears were described. Her medical and family
histories were unremarkable. The lesion was completely removed
under endoscopic control and local anesthesia. Grossly, the spec-
imenwas an ovoid, firm, pinkish gray mass, measuring 2×1.2×
0.9 cm; its cut surface was solid and pale gray. Microscopically,
the tumor consisted of a well-demarcated nodule of smooth mus-
cle tissue punctuated with thick-walled vessels with partially
patent lumina (Fig. 1). The smooth muscle cells had rod-like
nuclei and showed no cellular atypia or mitoses. Many vessels
were nested in the major portion of the lesion. The inner layers
of the vessel wall smooth muscle were arranged in an orderly
circumferential fashion whereas the outer layers merged with a
less orderly background muscle fibers. The vessel walls lacked
internal and external elastic laminae by elastic staining. Immuno-
histochemicalstudies produced a positive result for smooth mus-
cle actin (1:300, Dako, Glostrup, Denmark) in the perivascular
proliferating smooth muscle cells and in the muscular wall of
thick blood vessels (Fig. 2A), and for factor VIII-related antigen
(1:3, Dako, Carpinteria, CA, U.S.A.) in the endothelial cells of
thick-walled vessels (Fig. 2B). The perivascular proliferating
smooth muscle cells, the muscular wall of the thick blood vessels
and endothelial cells showed negative immunoreactivity for the
Su Jin Kim∙Sook Hee Hong∙Mee Sook Roh
estrogen receptor (ER) (1:50, Dako, Glostrup, Denmark) and
for the progesterone receptor (PR) (1:50, Dako, Carpinteria, CA,
U.S.A.). There was no evidence of recurrence during the 6-month
Since Maesaka et al.4first reported an intranasal angioleiomy-
oma in 1966, 26 cases have been reported,2,3which demonstrates
the rarity of this condition even taking underreporting into acco-
unt. A review of literature indicated a predominance in middle-
aged women and a predilection for the right side of the nasal
cavity, as in the present case; however, the reasons are unknown.
Though subcutaneous angioleiomyomas are commonly painful,1
tumors occurring in the nasal cavity are not known to be painful.
Patients with nasal angioleiomyomas usually complain of epis-
taxis and nasal obstruction.2These tumors are histologically clas-
sified into three types: capillary or solid, cavernous and venous.
In the extremities the tumors are mainly of the solid type whereas
in the head and neck region they are mainly venous.1Our case,
which arose in the nasal cavity was also of the venous type. Sur-
gical excision is recommended as treatment. The prognosis of
nasal angioleiomyoma is usually favorable. A case of nasal angi-
oleiomyoma extending into the nasopharynx and the anterior
cranial fossa showed no evidence of recurrences until 30 months
after the operation.5
The origin of nasal angioleiomyoma is uncertain. Lesions may
arise from smooth muscle elements in the nasal cavity or from
embryonic tissue remnants. In the nasal cavity, smooth muscles
are present as either piloerector muscle of the vestibule or in the
Fig. 2. (A) Immunohistochemical staining for smooth muscle actin shows a positive reaction in perivascular proliferating spindle cells and
thick blood vessel walls. Thick-walled blood vessel shows a circumferential arrangement of the inner smooth muscle layer and less well-
ordered outer layer blended with the background smooth muscle tissue of the tumor. (B) Immunohistochemical staining for factor VIII-related
antigen shows a positive reaction in the endothelial cells of the thick-walled vessels.
Fig. 1. The nasal tumor consists of a well-demarcated nodule of
smooth muscle tissue punctuated with thick-walled vessels with
Angioleiomyoma of the Nasal Cavity Download full-text
walls of blood vessels elsewhere.6Most of the cases reported in the
sinonasal area have developed from the inferior or middle turbi-
nate.2Therefore, it seems more likely that any leiomyoma devel-
oping in the nasal cavity is of vascular origin, though a hamar-
tomatous basis cannot be excluded. The vessels in these tumors
are difficult to classify because they are not altogether typical of
veins or arteries. Their thick walls and small lumina are reminis-
cent of arteries, but they consistently lack internal and external
elastic laminae, as in our case.
Whether differential incidence is associated with the presence
of ER and/or PR on smooth muscle cells is speculative, although
some evidence exist to show a relationship between the presence
of sex steroid receptors and the proliferation of smooth muscle
cells. The prevalence of female cases of nasal angioleiomyoma
and the increased pain during pregnancy and menstrual cycle
described in cases of angioleiomyoma at several sites suggest that
sex steroid receptors influence nasal angioleiomyoma develop-
ment.1,7Di Tommaso et al.7reported that a focal positivity for
PR was detected in 6 of 10 cases of subcutaneous angioleiomy-
oma and that the same cases showed no positivity for ER. Mar-
ioni et al.2described a case of nasal angioleiomyoma with PR-
positivity but ER-negativity by immunohistochemical study,
and suggested that PR though expressed had no functional role
or, alternatively, that PR was activated by a different pathway in
nasal angioleiomyomas. However, in the present case, the tumor
showed negative immunohistochemical findings for ER and PR.
These discrepancies are probably due to the sex steroid receptors
being expressed in only a small number of copies in mesenchymal
tumors. The reason why nasal angioleiomyomas are not painful
during pregnancy or the menstrual cycle might be related to the
total number of cells expressing sex steroid receptors. Further
studies using more cases are needed to clarify whether the growth
of this tumor is sex steroid-dependent.
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Staffieri A. Progesterone receptor expression in angioleiomyoma of
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myosarcoma - report of 2 cases. Otologia (Fukuoka) 1966; 12: 42-7.
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