Cellular Myxoma of the Vocal Cord: A Case Report and Review of the Literature
J. Fernando VAL-BERNAL1 , María MARTINO2 , M. Yolanda LONGARELA3
1Pathology Unit, Medical and Surgical Sciences Department, University of Cantabria and IDIVAL, SANTANDER, SPAIN
2Anatomical Pathology Service, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria and IDIVAL, SANTANDER, SPAIN
3Ear, Nose, and Throat Service, Marqués de Valdecilla University Hospital and IDIVAL, SANTANDER, SPAIN
Keywords: Larynx, Vocal cord, Myxoma, Cellular myxoma, Myxoid sarcoma
Myxomas are rare in the vocal cords. A 69-year-old man was admitted with one-year history of progressive dysphonia. Laryngoscopy revealed a
polypoid mass on the right vocal cord. The diagnosis was cellular myxoma. A review of the literature including the present case revealed eleven
reported cases of myxoma. Ten cases were classic myxoma. To the best of our knowledge, cellular myxoma has not been previously reported in
the vocal cord. Hypercellularity does not affect the behavior of cellular myxoma. However, its recognition is important to prevent confusion with
the group of low-grade myxoid sarcomas. Cellular myxoma should be considered in the differential diagnosis of any vocal cord mass.
Classic myxoma is a benign mesenchymal paucicellular
tumor characterized by bland spindle and stellate
shaped cells embedded in hypovascular, abundant loose
myxoid stroma. The cellular variant of this tumor shows
hypercellularity, more numerous collagen fibers, and
Myxomas of the larynx are very uncommon. The reported
sites of involvement are the vocal cords, the aryepiglottic
fold, and the epiglottis. They are more common in the
vocal cords. As far as we are aware, only ten cases of vocal
cord myxomas have been previously reported. We describe
herein a case of the cellular variant of myxoma in the right
vocal cord and review the literature. To the best of our
knowledge, a cellular myxoma (CM) in a vocal cord has
not been previously reported.
A 69-year-old man was admitted to the hospital with
one-year history of progressive dysphonia. There was no
associated pain, stridor, hemoptysis or weight loss. He
was currently consuming 40 g of alcohol a day and had
quit smoking 20 years ago. The patient was diagnosed
with a polyp of the right vocal cord 20 years ago, but he
refused the surgical treatment. Medical history was also
significant for atrial fibrillation with multiple embolisms
in the vertebrobasilar artery, brachiocephalic trunk, and mesenteric artery and chronic hepatic disease with
thrombosis of the right portal vein. Syndromic associations
were not present. Flexible laryngoscopy revealed a large
polypoid lesion on the right cord with preserved mobility.
The patient underwent phonosurgery under general
The specimen consisted of a glistening white, gelatinous,
polypoid mass measuring 0.9 x 0.6 x 0.3 cm. Histopathological
examination revealed an excrescent tissue fragment
consisting of squamous mucosa that was partially
atrophic and a mesenchymal neoplasm (Figure 1A). The
tumor showed spindled and stellate cells suspended in
a background of loose myxoid matrix. Cell density was
variable throughout the tumor with hypercellular (Figure
1B) and hypocellular areas (Figure 1C). Hypercellular areas
occupied about 90% of the tumor. In these areas, there were
more numerous blood vessels and collagen fibers (Figure
1D). In addition, occasional thick-walled vessels with
smooth muscle in their walls were present (Figure 2A).
Tumor cells were uniform and bland in appearance (Figure
2B). They showed small hyperchromatic nuclei with scant
tapering eosinophilic cytoplasm (Figure 2C). Scattered
muciphages were also observed. Fluid-filled microcystic
spaces were seen occasionally. Cellular pleomorphism,
multinucleated giant cells, mitoses, or necrosis were not
present. The myxoid matrix stained positive with Alcian
blue at pH 2.5 (Figure 2D). Immunohistochemical study revealed diffuse positivity for vimentin (Figure 3A) and
focal positivity for CD34 (Figure 3B) in the constituent cells.
These cells were not reactive for S100 protein, neurofilament
protein, epithelial membrane antigen, claudin-1, GLUT-
1, smooth muscle actin and MUC4. Ki-67 labeled only a
few nuclei of the squamous epithelium. The deep surgical
border was very close to the tumor boundary.
Click Here to Zoom
|Figure 1: A) Vocal cord lesion
showing a squamous mucosa.
Submucosa is occupied by a cellular
myxomatous neoplasm (H&E;
x100). B) Area with variable cell
density (H&E; x200).
C) Hypocellular and hypovascular
area (H&E; x200). D) Cellular
area containing increased collagen
fibers and vessels (H&E; x200).
Click Here to Zoom
|Figure 2: A) Prominent vessels,
some of which are thick-walled
containing smooth muscle
(H&E; x200). B) Uniform and
cytologically bland spindle cells
in a moderately hypercellular
region. Tumor cells are separated
by mucoid matrix and generally
do not touch one another (H&E;
x400). C) High magnification
appearance of a hypercellular
area. Nuclei are uniform and
pyknotic with tapered cytoplasms.
Cells lack nuclear atypia (H&E;
x400). D) Cells are suspended in
abundant mucoid material that
stains positively with Alcian blue at
pH 2.5. A thick-walled vessel can
be seen in the center of the image
(Alcian blue; x200).
Click Here to Zoom
|Figure 3: A) Hypercellular region
showing diffuse reactivity for
vimentin (IHC; x200).
B) Hypocellular region showing
focal positivity for CD34 (IHC;
The patient was discharged in hours. One month later his
voice was much improved. No signs of recurrence were
Pure myxomas are very infrequent in the vocal cords.
A review of the literature revealed only ten previously
reported cases (Table I
. Mean patient age is 58.2
years (SD 13.5; range 36-77 years). The tumor is more
frequent in males (male: female, 4.5:1). The main complaint
varies from hoarseness or dysphonia to dyspnea (Table I
The neoplasm can present with life-threatening dyspnea
requiring tracheotomy 5,11
. One case showing sleep
apnea was cured after removal of the tumor 6
. The average maximum diameter of the tumor was 1.03 cm (SD 0.62;
range 0.4-2.5 cm). The majority of the lesions are located on
the right vocal cord (2:1). Ten cases were classic myxoma.
The present case was a CM. As far as we are aware, a CM
has not been reported in the vocal cord. Excision of vocal
cord myxoma is considered curative. In one case, removal
of the neoplasm was incomplete. Recurrence is possible in
theory but it has never been reported (Table I
Cells of a myxoma originate from modified fibroblastic cells
that lack the ability to polymerize collagen. As an alternative,
they produce an excessive amount of glycosaminoglycans
giving them a gelatinous appearance on gross examination. The process suggests an underlying localized error in tissue
CM is characterized by hypercellular areas that occupy from
10 to 90% of the tumor. These foci have increased number
of cells, more prominent vascularity, increased collagen
content and less extracellular myxoid matrix than classic
myxoma. The hypercellular regions are not associated with
cytologic atypia, multinucleated giant cells, mitotic activity,
or necrosis. Vessels are capillary-sized but occasional thickwalled
vessels with smooth muscle in their walls can be
present. CMs usually show sparse paucicellular areas of
classic myxoma with scant capillary-sized vessels 1,2.
All the cases of CM reported out of the larynx have behaved
in a benign fashion with only a small risk of local nondestructive
recurrence if not excised completely 1,2. Thus,
in general, simple complete local excision is the adequate
The main differential diagnosis includes myxoid
neurofibroma, low-grade myxofibrosarcoma, low-grade
fibromyxoid sarcoma, and myxoid liposarcoma. Myxoid
neurofibroma shows spindled elongated cells with tapering,
wavy or bent nuclei and pale indistinct cytoplasms
embedded in abundant myxoid background. Intralesional
neural fibers are demonstrated with neurofilament protein.
Besides, a considerable number of cells are positive for S100 protein 14,15. CM, unlike low-grade myxofibrosarcoma,
does not show any cytonuclear atypia and does not have
the classical curvilinear vascular architecture, often with
a perivascular increase of cellularity 16. Low-grade
fibromyxoid sarcoma is diffusely more cellular and is
characterized by alternating myxoid and collagenous zones
containing bland spindle cells with a whorled growth
pattern. It may show areas of hyalinizing spindle cells with
giant rosettes. MUC4 immunostaining has been found to be
highly sensitive and specific for the diagnosis 17. Myxoid
liposarcoma has small, bland spindle-shaped or more
rounded cells, lipoblasts and a typically delicate plexiform
or branching “chicken-wire” capillary vasculature 18.
In conclusion, CM of the vocal cord is a benign mesenchymal
tumor that shows foci of increased cellularity and
vascularity, with presence of thick-walled vessels, and
increased collagen content. The recognition of this tumor
is important to avoid a misdiagnosis of any type of lowgrade
myxoid sarcoma. Although very rare, CM should be
considered in the differential diagnosis of any vocal cord
mass to allow for adequate treatment. Surgery is considered
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