Case Reports

Sarcomatoid Salivary Duct Carcinoma of the

Submandibular Gland
A Case Report

Masaki Mori, C.T., C.M.I.A.C., Makoto Ohta, M.D., Hideki Maegawa, M.T.,
Toshie Hara, M.D., and Yoshiaki Imamura, M.D., F.I.A.C.

Background with findings of typical SDC, sarcomatoid SDC should thus

Sarcomatoid salivary duct carcinoma (sarcomatoid SDC) is be considered. Furthermore, squamous cell carcinoma com-
a rare subtype of SDC. We encountered 1 case of sarcoma- ponent may be involved as in the present case, so it is neces-
toid SDC that developed from a submandibular gland pleo- sary to be aware of the possibility thereof. (Acta Cytol
morphic adenoma, and we herein report our findings. 2010;54:695–700)

Case Keywords: aspiration bi-

A 42-year-old female had tu- opsy, fine-needle; sarcoma-
mentia and pain in the right When polymorphic cells and toid salivary duct carcinoma,
submental area, and therefore multinucleated cells appear in an squamous cell carcinoma,
she underwent a close exami-
nation, wherein a right sub- isolated and scattered manner with submandibular gland.
cytologic findings of conventional arcomatoid salivary duct
mandibular gland neoplasm
was detected. Surgery for re-
moving the right submandib-
SDC, it is necessary to consider the S
carcinoma (sarcomatoid
SDC) is a rare subtype of
ular gland was performed in possibility of the present subtype. SDC that was reported for
addition to neck dissection. Cy- the first time by Henley et
tology showed typical findings al1 in 2000. There have since
of conventional SDC. Moreover, isolated and scattered 1-5
been dozens of reports, but only 1 case report has
large spindle cells and multinucleated cells were also detected described the cytologic findings.4 We encountered
along with atypical epidermal cell clumps showing kera- sarcomatoid SDC with a squamous cell carcinoma
tinization. From a histologic perspective, it appeared to be component that developed from a submandibular
sarcomatoid SDC that developed from a pleomorphic ade- gland pleomorphic adenoma, and we present details of
noma and also involved a squamous cell carcinoma component. the cytologic and histopathologic findings of this case.

Conclusion Case Report

This is the first report on the cytologic findings of a case of The patient was a 42-year-old female. Tumentia and
sarcomatoid SDC with a squamous cell carcinoma compo- pain in the right submental area appeared in addition
nent. When sarcomatoid cells appear in the cytology along to dysgeusia, so she visited a hospital, where a right

From the Division of Surgical Pathology, University of Fukui Hospital, Fukui, Japan.
Mr. Mori is Cytotechnologist.
Drs. Ohta and Hara are Cytopathologists.
Mr. Maegawa is Medical Technologist.
Dr. Imamura is Director.
Address correspondence to: Yoshiaki Imamura, M.D., F.I.A.C., Division of Surgical Pathology, University of Fukui Hospital, 23 Matsuoka-
130.237.165.40 - 4/3/2018 11:45:35 AM

Shimoaizuki, Eiheiji-cho, Yoshida-gun, Fukui, 910-1193, Japan (suki@u-fukui.ac.jp).

Financial Disclosure: The authors have no connection to any companies or products mentioned in this article.
Stockholm University Library

Received for publication April 8, 2009.

Accepted for publication April 9, 2009.
Downloaded by:

0001-5547/10/5405-0695/$21.00/0 © The International Academy of Cytology ACTA CYTOLOGICA 695

Mori et al

Figure 2 Fine needle aspiration cytology. Necrotic materials are

detected in the background, and markedly degenerative neoplastic
cells are detected therein (Papanicolaou stain, ×50).

cells, large multinucleated cells and spindle cells

showing irregular-shaped nuclei with significant cel-
lular pleomorphism were detected. In the imprint cy-
Figure 1 Neck magnetic resonance imaging contrast-enhanced, tology obtained from a surgical specimen of the right
T1-weighted image. A relatively well-demarcated mass with internal submandibular tumor, the background also appeared
heterogeneity that is 40 × 30 mm is detected in the right submental to be necrotic, and poorly demarcated, overlapping
area.
cell clumps with irregular light-green–stained cyto-
plasm were detected. The neoplastic cells had coarse-
ly granular chromatin, large nucleoli and irregular-
submandibular gland neoplasm was found. The patient shaped nuclei (Figure 4). Moreover, isolated and
visited our hospital after referral for the purpose of re- scattered neoplastic cells with significant cellular pleo-
ceiving treatment. A neck magnetic resonance imag- morphism were detected. They were mostly large cells
ing examination showed a relatively well-demarcated with irregular, light-green, darkly stained cytoplasm
mass with internal heterogeneity that was 40 × 30 mm and coarsely granular chromatin, but naked cells and
and in the right submental area (Figure 1), and a
positron emission tomography examination showed a
significant [F-18]-2-fluoro-2-deoxy-D-glucose accu-
mulation in accordance with the mass in the right sub-
mental area. High grade carcinoma was suspected
from fine needle aspiration cytology, so surgery for re-
moving the right submandibular gland was performed
along with neck dissection. It has been 2.5 years since
radiation therapy (60 Gy) was started after the sur-
gery, but no signs of recurrence or metastasis have
been observed.

Results
Cytologic Findings
In the fine needle aspiration cytology, there were
some cohesive, 3-dimensional clusters in the necrotic
background (Figure 2). The neoplastic cells showed
cellular pleomorphism and had irregular-shaped nu-
Figure 3 Fine needle aspiration cytology. Cohesive,
clei and large nucleoli. Light-green–stained cytoplasm
130.237.165.40 - 4/3/2018 11:45:35 AM

3-dimensional clusters of neoplastic cells are detected. Neoplastic

with a poorly demarcated cell border was observed cells with irregular-shaped, pleomorphic nuclei and large nucleolus
Stockholm University Library

(Figure 3). Furthermore, isolated and scattered oval are observed (Papanicolaou stain, ×100).
Downloaded by:

696 ACTA CYTOLOGICA Volume 54 Number 5 September–October 2010


Figure 4 Imprint cytology showing irregularly overlapping
neoplastic cell clumps. The neoplastic cells have light-green, palely
stained cytoplasm, and the chromatin is coarsely granular, with
large nucleoli (Papanicolaou stain, ×100).
multinucleated cells were also sporadically detected
(Figure 5). In addition, squamous cell clumps with
orange-G–stained cytoplasm and irregular-shaped,
densely stained nuclei were detected (Figure 6). The
cells derived from pleomorphic adenoma were unclear
in the cytologic specimens that we obtained.
Pathologic Findings
A milky, solid neoplasm with hyalinization in the mar-
ginal region that was approximately 4 cm in its great-
est dimension was detected macroscopically. In an
image as viewed via a loupe, the neoplasm appeared to
consist of a site with significant hyalinization and a site
Figure 5 Imprint cytology showing isolated and scattered
neoplastic cells with significant cellular pleomorphism. Some of
them have light-green, darkly stained cytoplasm, but naked cells
are also observed. In addition, multinucleated cells are sporadically
observed (Papanicolaou stain, × 100).
Volume 54
Sarcomatoid SDC
Figure 6 Imprint cytology showing atypical cell clumps with
orange-G–stained cytoplasm and irregular-shaped, densely stained
nuclei (Papanicolaou stain, × 100).
with high cellularity, and it had infiltrated into the
surrounding tissue. From a histologic perspective, the
majority of the neoplasm (about 80%) appeared to
consist of a carcinomatous component (about 30%)
showing an irregular glandular structure with cribri-
form pattern and comedonecrosis and a sarcomatoid
component (about 50%) in which poorly demarcated
polymorphic and spindle cells showing large, polynu-
clear and irregular-shaped nuclei existed (Figure 7).
Both were well demarcated, but a transitional zone
was focally recognized between them. No heterolo-
gous elements such as bone, cartilage or muscle tissue
were detected in the sarcomatoid component. Fur-
thermore, a squamous cell carcinoma component with
Figure 7 Histologic findings. A carcinomatous component of
130.237.165.40 - 4/3/2018 11:45:35 AM
sarcomatoid salivary duct carcinoma showing a cribriform pattern
with comedonecrosis is observed along with the proliferation of a
Stockholm University Library
sarcamotoid component around it (hematoxylin-eosin, × 25).
Downloaded by:
Number 5 September–October 2010 ACTA CYTOLOGICA 697
Mori et al
Figure 8 Histologic findings. A squamous cell carcinoma
component with distinct intercellular bridges is observed
(hematoxylin-eosin, × 50).
distinct intercellular bridges was partially detected
(about 10%) (Figure 8). A squamous cell carcinoma
component was intermingled with a sarcomatoid
component, but not with a carcinomatous component.
In sites with significant hyalinization (about 10%), a
pleomorphic adenoma element remained. All avail-
able 18 lymph nodes showed no evidence of tumor
metastasis.
On immunohistochemical staining, a carcinoma-
tous component was positive for androgen receptor
(AR) (Dako, Glostrup, Denmark, 1:50) (Figure 9),
gross cystic disease fluid protein-15 (GCDFP-15)
(Novocastra, Newcastle-upon-Tyne, U.K., 1:50),
HER-2/neu (Dako, 1:50), cytokeratin 7 (Dako, 1:50),
Figure 9 Immunohistochemical findings. As for androgen
receptor, the neoplastic cell nuclei of a carcinomatous component
of sarcomatoid SDC are positive, but the neoplastic cell nuclei of the
surrounding sarcomatoid component are negative (×50).
698 ACTA CYTOLOGICA Volume 54 Number 5
Figure 10 Immunohistochemical findings. As for vimentin, the
cytoplasm of the neoplastic cells of a sarcomatoid component of
sarcomatoid salivary duct carcinoma is positive, but the cytoplasm
of a carcinomatous component is negative (× 50).
pancytokeratin AE1/AE3 (Dako, 1:100), high-molecular-
weight cytokeratin 34βE12 (Dako, 1:50), epithelial
membrane antigen (EMA) (Dako, 1:50) and carcino-
embryonic antigen (CEA) (Dako, 1:50) but was negative
for cytokeratin 5/6 ( Dako, 1:50), p63 (Dako, 1:50), vi-
mentin (Dako, 1:50) (Figure 10), calponin (Dako,
1:50), α-smooth muscle actin (Dako, 1:50), desmin
(Dako, 1:50) and S-100 protein (Dako, 1:500). A sar-
comatoid component was positive for vimentin (Fig-
ure 10) and was partially positive for high-molecular-
weight cytokeratin 34βE12 and EMA but was negative
for others, such as AR (Figure 9). A squamous cell car-
cinoma component was positive for pancytokeratin
AE1/AE3, high-molecular-weight cytokeratin 34βE12
and p63 and was partially positive for cytokeratin 5/6
and EMA, but was negative for others. The MIB-1
(Dako, 1:50) labeling index was 40.2% for carcinoma-
tous component, 48.9% for sarcomatoid component
and 32.4% for squamous cell carcinoma component,
showing high values for all of these. A summary of the
results of the immunohistochemical staining is shown
in Table I.
Discussion
SDC, which is a salivary gland malignancy with poor
prognosis that frequently occurs in older males, was
reported by Kleinsasser et al6 in 1968 and shows a his-
tologic finding that is similar to that of ductal carcino-
ma of the breast, with intraductal and invasive compo-
nents. Subsequently, sarcomatoid SDC,1-5 mucin-rich
SDC7 and invasive micropapillary SDC8 were report-
ed as subtypes thereof. There have been dozens of re-
130.237.165.40 - 4/3/2018 11:45:35 AM
ports of histologic findings of sarcomatoid SDC.1-5
Stockholm University Library
Histologically, sarcomatoid SDC is characterized by
Downloaded by:
September–October 2010
 Sarcomatoid SDC

Table I Summary of Immunohistochemical Results sis in this case was carcinosarcoma. Based on im-
Immunohistochemical CC SC SCC munohistochemical demonstration of positivity in the
sarcomatoid areas of sarcomatoid SDC for cytokeratin
AR + − −
GCDFP-15 + − − and EMA, several authors came to the conclusion that
HER-2/neu + − − these tumors should be termed sarcomatoid SDC rather
Cytokeratin 5/6 − − F+ than carcinosarcoma.1-4 Our own immunohistochemi-
Cytokeratin 7 + − − cal findings are in accordance with this point of view.
Pancytokeratin AE1/AE3 + − +
Based on these findings, the use of the term sarcoma-
High-molecular-weight cytokeratin
34βE12 + F+ + toid SDC seems more appropriate than carcinosarco-
p63 − − + ma for the tumor presented here.
EMA + F+ F+ In the cytology of a typical example of convention-
CEA + − − al SDC, epithelial clumps with a cribriform to papil-
Vimentin − + −
lary structure appear along with a large amount of
Calponin − − −
α-Smooth muscle actin − − − necrotic material. It is said that individual neoplastic
Desmin − − − cells have abundant granular cytoplasm and eccentric
S-100 protein − − − nuclei with delicate chromatin and large nucleoli.10-13
MIB-1 labeling index (%) 40.2 48.9 32.4 In the cytology of the present case, neoplastic cells
+ = Positive, − = negative, CC = carcinomatous component, F = focally, SC = with significant degeneration and neoplastic cell
sarcomatoid component, SCC = squamous cell carcinoma. clumps that had a light-green–stained and poorly de-
marcated cytoplasm, irregular-shaped pleomophic
nuclei and large nucleoli were detected in the necrot-
ic background. These findings are consistent with the
the presence of both a carcinomatous and a sarcoma- cytologic findings of conventional SDC that have pre-
toid component. A carcinomatous component shows viously been reported, and it is believed that they re-
the cytomorphologic aspect of conventional SDC flect a carcinomatous component of sarcomatoid
with cribriform or solid cell nests, ductal carcinoma in SDC. In the present case, isolated and scattered spin-
situ,and comedonecrosis, whereas a sarcomatoid com- dle cells and large, multinucleated giant cells with sig-
ponent is composed of mitotically active, pleomorphic nificant cellular pleomophism were detected along
spindle cells and anaplastic tumor giant cells.1-5 In the with naked cells. We believe that these cells are de-
present case, the histologic findings of sarcomatoid rived from a sarcomatoid component of sarcomatoid
SDC described above were detected. Furthermore, in SDC. One case reported by Padberg et al4 is the only
the present case, a squamous cell carcinoma compo- report of the cytologic findings of sarcomatoid SDC,
nent was also found. Sarcomatoid SDC with a squa- and they reported that spindle cells derived from a sar-
mous cell carcinoma component was detected in only comatoid component existed in an aggregated man-
1 of the 3 cases reported by Henley et al,1 so it is be- ner, showing cytologic findings different from the pat-
lieved that it is very rare. tern of the isolated and scattered appearance of the
An immunohistochemical study of sarcomatoid sarcomatoid component in the present case. It will be
SDC has shown that it is characterized by the fact that necessary to study this point after the further accumu-
a carcinomatous component is positive for cytokera- lation of cases. In the cytology of the present case,
tin, a sarcomatoid component is diffusely positive for atypical cell clumps with orange-G–stained cytoplasm
vimentin, and a sarcomatoid component shows partial and irregular-shaped, densely stained nuclei that are
positive reactions for cytokeratin and EMA.1-5 In the believed to have been derived from a squamous cell
present case, results of immunohistochemical staining carcinoma component were also observed. No reports
similar to those in previous reports were also obtained. state that cells derived from a squamous cell carcino-
A carcinomatous component of the present case was ma component were observed in the cytology of sar-
positive for AR, GCDFP-15, HER-2/neu, EMA and comatoid SDC.
CEA9 as well, which are known as frequently showing When polymorphic cells and multinucleated cells
positive by conventional SDC other than cytokeratin, appear in an isolated and scattered manner with cyto-
but a sarcomatoid component was negative for all of logic findings of conventional SDC, it is necessary to
these. This is also a finding that is consistent with consider the possibility of the present subtype. Fur-
those of previous reports,1,3,4 and the fact that a carci- thermore, considering that a squamous cell carcinoma
nomatous component and a sarcomatoid component component may be observed in sarcomatoid SDC, it is
had high MIB-1 labeling indexes is also consistent thus considered important to differentiate such an oc-
130.237.165.40 - 4/3/2018 11:45:35 AM

with the previous reports.3 currence from a salivary gland neoplasm in which
Stockholm University Library

The most important histologic differential diagno- other squamous cell carcinoma components appear.
Downloaded by:

Volume 54 Number 5 September–October 2010 ACTA CYTOLOGICA 699

Mori et al
References
1. Henley JD, Seo IS, Dayan D, Gnepp DR: Sarcomatoid salivary
duct carcinoma of the parotid gland. Hum Pathol 2000;31:
208–213
2. Ide F, Mishima K, Saito I: Sarcomatoid salivary duct carcinoma
of the oral cavity. Virchows Arch 2003;443:686–689
3. Nagao T, Gaffey TA, Serizawa H, Iwaya K, Watanabe A,
Yoshida T, Yamazaki K, Sageshima M, Lewis JE: Sarcomatoid
variant of salivary duct carcinoma: Clinicopathologic and im-
munohistochemical study of eight cases with review of the lit-
erature. Am J Clin Pathol 2004;122:222–231
4. Padberg BC, Sasse B, Huber A, Pfaltz M: Sarcomatoid salivary
duct carcinoma. Ann Diagn Pathol 2005;9:86–92
5. Jeong HS, Son YI, Ko YH, Kim SY: Sarcomatoid salivary duct
carcinoma of the larynx. J Laryngol Otol 2006;120:154–157
6. Kleinsasser O, Klein HJ, Hubner G: Salivary duct carcinoma: A
group of salivary gland tumors analogous to mammary duct car-
cinoma. Arch Klin Exp Ohren Nasen Kehlkopfheilkd 1968;
192:100–105
7. Simpson RH, Parasad AR, Lewis JE, Skalova A, David L:
Mucin-rich variant of salivary duct carcinoma: A clinicopatho-
logic and immnohistochemical study of four cases. Am J Surg
700 ACTA CYTOLOGICA Volume 54 Number 5 September–October 2010
Pathol 2003;27:1070–1079
8. Nagao T, Gaffey TA, Visscher DW, Kay PA, Minato H, Ser-
izawa, H, Lewis JE: Invasive micropapillary salivary duct carci-
noma: A distinct histologic variant with biologic significance.
Am J Surg Pathol 2004;28:319–326
9. Ellis GL, Auclair PA: Salivary duct carcinoma. In AFIP Atlas of
Tumor Pathology. Fourth series, fascicle 9: Tumors of the sali-
vary glands. Washington, DC, Armed Forces Institute of
Pathology, 2008, pp 322–332
10. Fyrat P, Camer H, Feczko JD, Kratzer S, Layfield LJ, Eisenhut
CC, Glant MD: Fine-needle aspiration biopsy of salivary duct
carcinoma: Report of five cases. Diagn Cytopathol 1997;16:
526–530
11. Khurana KK, Pitman MB, Powers CN, Korourian S, Bardales
RH, Stanley MW: Diagnostic pitfalls of aspiration cytology of
salivary duct carcinoma. Cancer 1997;81:373–378
12. Gilcrease MZ, Guzman-Paz M, Froberg K, Pambuccian S:
Salivary duct carcinoma: Is a specific diagnosis possible by fine
needle aspiration cytology? Acta Cytol 1998;42:1389–1396
13. Klijanienko J, Vielh P: Cytologic characteristics and histomor-
phologic correlations of 21 salivary duct carcinoma. Diagn Cy-
topathol 1998;19:333–337
130.237.165.40 - 4/3/2018 11:45:35 AM
Stockholm University Library
Downloaded by: