SURGICAL ONCOLOGY AND RECONSTRUCTION

Salivary Duct Carcinoma Ex Pleomorphic

Adenoma of the Palate: A Case Report
Lauren G. Bourell, DDS, MD,* King Chong Chan, DMD, MS,y
and David L. Hirsch, DDS, MDz

Carcinoma ex pleomorphic adenoma is a rare malignancy of the head and neck, particularly in the minor
salivary glands. Most cases arise in the major salivary glands, most commonly in the parotid gland, followed
by the submandibular gland. The malignant component of the tumor varies, but can be salivary duct car-
cinoma, adenoid cystic carcinoma, mucoepidermoid carcinoma, squamous cell carcinoma, or adenocarci-
noma, not otherwise specified. Primary salivary duct carcinoma is also a rare malignancy of the head and
neck. Similar to carcinoma ex pleomorphic adenoma, it is more common in the major salivary glands, with
the parotid gland accounting for 88% and the submandibular gland for 10% of cases. To date, only 25
known cases of primary salivary duct carcinoma arising in the minor salivary glands have been docu-
mented, with most arising in the palate. Salivary duct carcinoma ex pleomorphic adenoma of the minor
salivary glands appears to be even rarer. Our case of salivary duct carcinoma ex pleomorphic adenoma
of the palate is the first complete report, to our knowledge, in the English-language scientific literature.
Ó 2015 American Association of Oral and Maxillofacial Surgeons
J Oral Maxillofac Surg 73:370.e1-370.e7, 2015

Carcinoma ex pleomorphic adenoma is a rare malig- pleomorphic adenoma of the palate is the first
nancy of the head and neck, in particular, in the minor complete report, to our knowledge, in English-
salivary glands. Most cases arise in the major salivary language scientific studies.
glands, most commonly, the parotid gland followed
by the submandibular gland. The malignant compo-
Case Report
nent of the tumor varies, but it can be salivary duct car-
cinoma, adenoid cystic carcinoma, mucoepidermoid We present the case of a 58-year-old man who was
carcinoma, squamous cell carcinoma, or adenocarci- referred to our hospital’s oral and maxillofacial surgery
noma, not otherwise specified.1 clinic for evaluation of an exophytic mass of the right
Similarly, primary salivary duct carcinoma is also a palate. The patient reported that the mass had been
rare malignancy of the head and neck. Just as with car- noted by a dentist ‘‘a few years ago’’ but had not both-
cinoma ex pleomorphic adenoma, it is more common ered him until 6 months earlier, when it began to
in the major salivary glands, with the parotid gland ac- enlarge and prevented him from wearing his maxillary
counting for 88% and the submandibular gland, 10%.2 partial denture. One month before his presentation,
To date, only 25 known cases of primary salivary duct the patient had also noticed numbness over his right
carcinoma arising in the minor salivary glands have upper lip and nose.
been documented, with most arising in the palate.3,4 He denied any chronic health problems or previous
Salivary duct carcinoma ex pleomorphic adenoma of surgeries and was taking no medications. His social his-
the minor salivary glands appears to be even more tory was significant for cigarette smoking of 1 pack per
rare. Our case of salivary duct carcinoma ex day for 30 years.

*Chief Resident, Department of Oral and Maxillofacial Surgery, Address correspondence and reprint requests to Dr Bourell: LEAP
New York University/Bellevue Hospital Center, New York, NY. Foundation, 7777 Forest Lane B326, Dallas, TX 75230; e-mail:
yClinical Assistant Professor, Departments of Oral and lgb231@nyu.edu
Maxillofacial Pathology, Radiology, and Medicine, New York Received July 16 2014
University College of Dentistry, New York, NY. Accepted August 19 2014
zClinical Assistant Professor, Department of Surgery and Plastic Ó 2015 American Association of Oral and Maxillofacial Surgeons
Surgery, New York University Langone Medical Center, New York, 0278-2391/14/01434-7
NY. http://dx.doi.org/10.1016/j.joms.2014.08.040

370.e1
BOURELL, CHAN, AND HIRSCH 370.e2

The physical examination revealed no facial asym- nus malignancy were also considered. The specimen
metry and no palpable cervical lymphadenopathy. from the incisional biopsy was described as a salivary
His cranial nerves were grossly intact, except for hypo- gland neoplasm with basaloid features arranged in
esthesia of the right cheek, upper lip, and ala of tubular and reticular patterns (Fig 2). These findings
the nose. No nasal obstruction or mass was noted. In- alone were consistent with a cellular pleomorphic ad-
traorally, a 2  4-cm, smooth, exophytic, nonulcer- enoma; however, given the radiographic findings of
ated, firm, blue-red mass was present in the right bone destruction, suspicion was raised for malignant
hard palate, extending posteriorly to the soft palate transformation.
(Fig 1A). A panoramic image was acquired and showed The patient underwent contrast-enhanced
destruction of the posterior right maxilla (Fig 1). computed tomography (CT) of the head and neck for
Given the clinical and radiographic findings, the top surgical treatment planning. The CT scan showed an
2 differential diagnoses of the palatal mass in our pa- invasive, heterogeneously enhancing, soft tissue
tient were salivary gland malignancy and squamous mass measuring approximately 6 cm  3.5 cm that
cell carcinoma. Lymphoma, sarcoma, and maxillary si- was centered on the hard palate. The internal pattern

FIGURE 1. A, Intraoral photograph showing the tumor at the initial presentation, before biopsy. B, Panoramic image showing destruction (star)
of the maxillary right molar region extending posteriorly and superiorly to involve the maxillary tuberosity, sinus, and pterygomaxillary fissure.
Bourell, Chan, and Hirsch. Salivary Duct Carcinoma Ex Pleomorphic Adenoma. J Oral Maxillofac Surg 2015.
370.e3 SALIVARY DUCT CARCINOMA EX PLEOMORPHIC ADENOMA

were reported positive for metastatic carcinoma.

The patient then underwent a completion right
neck dissection of lymph node levels I to III.
Because of the tumor location, cervical metastases
in levels IV and V were considered to be significantly
less likely, and these were not included in our neck
dissection.
The histologic findings of the surgical specimen
showed 2 distinct morphologic patterns of the
neoplasm. The pattern reminiscent of the biopsy spec-
imen had transformed to a high-grade, cytologically
malignant neoplasm, showing marked nuclear atypia,
mitotic figures, comedonecrosis, and bone destruc-
tion (Fig 5A). Foci of the malignant epithelial cells
had ‘‘roman bridging’’ arrangement, a feature charac-
FIGURE 2. A hematoxylin and eosin–stained section of the biopsy teristic of salivary duct carcinoma (Fig 5B). Perineural
specimen at 200 magnification showing tubular and reticular
morphology of a hypercellular neoplasm with cytologically bland and lymphovascular invasion was also present. Of the
and basaloid features, consistent with a cellular pleomorphic 15 lymph nodes, 4 were positive for carcinoma with
adenoma. extracapsular spread. Immunohistochemical staining
Bourell, Chan, and Hirsch. Salivary Duct Carcinoma Ex Pleomor- of select sections of the specimen for Her2/neu was
phic Adenoma. J Oral Maxillofac Surg 2015.
strongly positive (3+) in the carcinoma, a character-
istic of salivary duct carcinomas that has been associ-
ated with a poor prognosis.5 The 5-year overall
of the mass was predominantly of muscle-like attenua- survival of salivary duct carcinoma has been reported
tion, with scattered calcifications that represented re- to range from 11 to 30%, with 40 to 45% locoregional
sidual bone. Extensive destruction of the hard palate recurrence and 55 to 65% distant metastasis.6,7
and invasion into the floor of the nose were present. Our patient underwent adjuvant radiotherapy
The mass extended posteriorly and laterally, destroy- within 6 weeks of surgery and was followed monthly
ing the alveolar process of the posterior right maxilla, for 9 months with no signs of recurrence. A PET/CT
right maxillary tuberosity, and right pterygoid plates of scan was performed at 3 months postoperatively,
the sphenoid bone. The mass had invaded the pterygo- with no signs of recurrent or new disease. He will
palatine fossa and was suspicious for perineural spread continue to be followed monthly for the first year,
(Fig 3). The right jugulodigastric lymph node was every 2 to 3 months for the second and third year,
enlarged, measuring approximately 2 cm in the great- and every 6 months for the fourth and fifth years and
est dimension, but had no other features of malignancy will undergo additional imaging, as indicated.
such as central necrosis or loss of fatty hilum (Fig 4).
Also, smaller, cervical nodes were positive. The highly
Discussion
aggressive radiographic features of the mass were
consistent with a high-grade malignancy, such as squa- Primary salivary duct carcinoma histologically re-
mous cell carcinoma or adenocarcinoma of minor sali- sembles intraductal carcinoma of the breast and is a
vary gland origin; however, the biopsy specimen did relatively recent addition to the diagnostic spectrum.
not demonstrate high-grade features. Consequently, The World Health Organization recognized it in
the patient underwent the standard imaging workup, 1991,8 although it was first reported in 1968 by
including a neck CT scan and chest radiograph, Kleinsasser et al.9 Tumors can display calcifications
without consideration for positron emission tomogra- on CT or magnetic resonance imaging,10,11 just as
phy (PET). the tumor in our patient did. Overexpression of C-
After presentation of the case at the hospital’s ErbB-2 and/or Her2/neu is common.12,13 In the
multidisciplinary head and neck tumor board, a major salivary glands, the rate of lymph node
total maxillectomy with microvascular free flap metastasis has been reported to be as great as 80%,
reconstruction was performed. An anterior lateral although in the minor salivary glands, the rate has
thigh flap was chosen to provide sufficient soft tis- been approximately 4 times lower.1 The prognosis
sue bulk and to minimize donor site morbidity, for patients with salivary duct carcinoma of the mi-
because the patient desired the quickest possible re- nor glands and lymphatic spread is poor.14 However,
turn to mobility. Intraoperatively, the right jugulodi- whether the prognosis of minor gland tumors is bet-
gastric lymph node and several additional level IIb ter, worse, or the same as that of major gland tumors
nodes were sent for frozen section analysis and is currently unknown.14-17
BOURELL, CHAN, AND HIRSCH 370.e4

FIGURE 3. A, Axial bone window of the contrast-enhanced computed tomography (CT) study of the head and neck showing the tumor has
destroyed the hard palate (star), the right maxillary tuberosity (asterisk), and right pterygoid plates of the sphenoid bone (vertical arrow).
B, Coronal bone window of the CT study showing the bone destruction extending superiorly to the mid-pterygoid level (horizontal arrow).
Bourell, Chan, and Hirsch. Salivary Duct Carcinoma Ex Pleomorphic Adenoma. J Oral Maxillofac Surg 2015.
370.e5 SALIVARY DUCT CARCINOMA EX PLEOMORPHIC ADENOMA

FIGURE 4. A, Axial soft tissue window of the computed tomography (CT) study showing a large, destructive, heterogeneously enhancing mass
(star) of muscle-like attenuation that has invaded the nasal cavity and extended posterolaterally to involve the pterygopalatine fossa (arrow). B,
Coronal soft tissue window of the CT study showing enlarged and matted right jugulodigastric lymph nodes (asterisk).
Bourell, Chan, and Hirsch. Salivary Duct Carcinoma Ex Pleomorphic Adenoma. J Oral Maxillofac Surg 2015.

It is widely acknowledged that pleomorphic ade- been present for many years. Carcinoma ex pleomor-
noma has potential for malignant transformation, phic adenoma can be challenging to diagnosis for
although the reported range has varied widely from several reasons. It is a rare tumor, and the 2 compo-
as low as 5% to as high as 25%4 for lesions that have nents can be difficult to discern in the tumor
BOURELL, CHAN, AND HIRSCH 370.e6

FIGURE 5. A, Hematoxylin and eosin–stained sections of the surgical specimen at 200 magnification showing malignant transformation
(star) of the basaloid specimen (vertical arrow), reminiscent of the biopsy specimen, to a high-grade malignancy (horizontal arrow). B, The pres-
ence of ‘‘roman bridging’’ morphology (brackets), comedonecrosis (star), numerous mitotic figures, nuclear atypia, and bone destruction
(asterisk) is characteristic of salivary duct carcinoma.
Bourell, Chan, and Hirsch. Salivary Duct Carcinoma Ex Pleomorphic Adenoma. J Oral Maxillofac Surg 2015.

specimen. The carcinoma can overtake the residual References

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