Adenoid Cystic Carcinoma of the Base of the Tongue Metastasizing to the Scalp

Abstract

Adenoid cystic carcinoma is a rare neoplasm that originates from secretory glands, most commonly from the salivary glands. We present a 76 year-old white man with a history of adenoid cystic carcinoma from the base of the tongue diagnosed 15 years prior to the development of the metastatic lesion on his mid-posterior scalp. The present case represents the second reported instance of an extracutaneous adenoid cystic carcinoma metastasizing to the scalp. Differentiating between a primary cutaneous adenoid cystic carcinoma and an extracutaneous adenoid cystic carcinoma metastasizing to cutaneous structures is crucial in determining prognosis and management.

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UC Davis
Dermatology Online Journal
Title
Adenoid Cystic Carcinoma of the Base of the Tongue Metastasizing to the Scalp
Permalink
https://escholarship.org/uc/item/6141j293
Journal
Dermatology Online Journal, 20(3)
Authors
Saco, Michael
Howe, Nicole
Jukic, Drazen M
et al.
Publication Date
2014
License
https://creativecommons.org/licenses/by-nc-nd/4.0/ 4.0
Peer reviewed
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University of California

Volume 20 Number 3
March 2014
Case Presentation
Adenoid Cystic Carcinoma of the Base of the Tongue Metastasizing to the Scalp
Michael Saco BS1, Nicole Howe MD2, Drazen M. Jukic MD PhD2,3,4, Carlos Muro-Cacho MD PhD4,5
Dermatology Online Journal 20 (3): 6
1Medical School, The University of South Florida Morsani College of Medicine, Tampa, Florida, 2The
Department of Dermatology, University of South Florida, Tampa, Florida, 3Chief of Dermatopathology, James
A. Haley Veterans' Hospital, Tampa, Florida, 4Department of Pathology and Cell Biology, University of South
Florida, Tampa, Florida, 5Pathology and Laboratory Medicine Service, James A. Haley Veterans' Hospital,
Tampa, Florida
Correspondence:
Michael Saco BS
University of South Florida Morsani College of Medicine
13330 USF Laurel Drive, Tampa, FL 33612
Tel: (813) 974-4744
Fax: (813) 974-2132
Email: msaco@health.usf.edu
Abstract
Adenoid cystic carcinoma is a rare neoplasm that originates from secretory glands, most commonly from the salivary glands. We
present a 76 year-old white man with a history of adenoid cystic carcinoma from the base of the tongue diagnosed 15 years prior
to the development of the metastatic lesion on his mid-posterior scalp. The present case represents the second reported instance of
an extracutaneous adenoid cystic carcinoma metastasizing to the scalp. Differentiating between a primary cutaneous adenoid
cystic carcinoma and an extracutaneous adenoid cystic carcinoma metastasizing to cutaneous structures is crucial in determining
prognosis and management.
Keywords: adenoid cystic carcinoma; cutaneous metastasis; scalp; tongue; salivary glands
Introduction
Adenoid cystic carcinoma (ACC) is a rare neoplasm that originates from secretory glands and comprises a large portion of
malignant neoplasms originating in both major and minor salivary glands. Origin from other sites, such as excretory glands of the
genital tract, lacrimal glands, and ceruminous glands, has been documented [1]. Adenoid cystic carcinoma is characteristically
associated with a prolonged clinical course and slow, progressive growth. It tends to recur locally and can metastasize regionally
and systemically years after the initial diagnosis. Approximately 35-50% of salivary gland ACCs metastasize distantly 3.1 to 7.6
years after diagnosis. The lungs are the most common sites of distant metastasis. Bone, liver, and brain are also common sites [2,
3]. Adenoid cystic carcinoma is generally treated by surgical excision with attention to margins and the presence of perineural
invasion. This is typically followed by adjuvant external radiation of the surgical bed. Results from studies evaluating the efficacy
of chemotherapy in the treatment of ACC remain inconclusive [1].
Primary cutaneous ACC is best classified as a neoplasm with sweat gland differentiation. These neoplasms can occur anywhere on
the body, but have a predilection for the scalp and chest [4, 5]. Wide local excision with free margins is the treatment choice [6],
and Mohs surgery is frequently employed [7]. Cutaneous ACC is locally invasive and 76% of the cases show perineural invasion,
resulting in local recurrence in 44% of the cases [5, 7]. Primary cutaneous ACC, however, is less aggressive than salivary gland
ACC, and distant metastases, typically to lung, are rare. Cases with metastatic spread to the pleura and lymph nodes have also

been reported. Metastases have been diagnosed from 8.2 to 16 years after diagnosis of the primary tumor [4-6]. Even though
primary cutaneous ACC has a predilection for the scalp and chest, to the authors’ knowledge, only one case of metastases from
extracutaneous ACCs to the scalp has been reported [8].
Methods
A search of PubMed performed on November 2, 2013, with the following terms – “adenoid,” “cystic,” “carcinoma,” “cutaneous,”
“metastasis,” “metastases,” “scalp” yielded only one report of metastases to the scalp from a primary ACC of lacrimal gland
origin.
Case synopsis
A 76 year-old white man presented to the dermatology clinic with a firm, pink papule on the mid-posterior scalp that had been
slowly growing for an indeterminate period of time (Figure 1). He had a history of non-melanoma skin cancer (NMSC) and right
base of tongue ACC with pulmonary metastases. The primary ACC was diagnosed 15 years prior to this visit and the tumor had
peri- and intraneural invasion (Figure 2). The initial treatment was surgical excision, followed one month later by a re-excision to
obtain negative margins. He received no further treatment and was followed by his oncologist. A computed tomographic (CT)
scan of the thorax with contrast, performed 2 years prior to the current date, revealed diffuse pulmonary metastases, bilaterally,
confirmed by biopsy. The biopsied metastatic lesion was diffusely positive for CD117 (c-kit). A new thoracic CT scan showed
that one of the metastases was possibly increasing in size and that the other nodules were relatively stable. The patient deferred
oncologic treatment, but agreed to be monitored via thoracic CT scans. During his visit to the dermatology clinic for an unrelated
reason, a firm, pink papule was discovered on the mid-posterior scalp and a shave biopsy was performed
Upon histopathology examination (Figures 3A and 3B), the lesion resembled a basal cell carcinoma, but a closer inspection
revealed that the neoplasm formed multiple ductal structures expressing carcinoembryonic antigen (CEA), cytokeratin (CK) 8/18
(Figure 3C), epithelial membrane antigen (EMA) (Figure 3D), and pankeratin AE1/AE3. No connection to the epidermis was
identified. At the time of this diagnosis, the pathologist was unaware of the patient’s clinical history and the diagnosis considered
was primary ACC of sweat gland differentiation. Once the pathologist learned about the patient’s history of base of tongue ACC,
the current tumor was compared to the tumor from the patient’s previous excision. The histopathologic features of the original
tumor and the scalp metastasis were almost identical, except that the metastasis lacked intraluminal globules of basement
membrane material. Accordingly, a diagnosis of metastatic ACC was made.
The tumor measured 0.8 cm in greatest dimension and was excised with 0.4 cm negative margins. After the patient had a
discussion with his oncologist, he decided that he wanted to defer workup for further metastatic lesions pending the results of
future repeat CT scans.
Figure 1. A 5 mm erythematous, slightly scaly papule on the mid-posterior scalp.

Figure 2. Primary adenoid cystic carcinoma at base of tongue; hematoxylin and eosin, original magnification x 10.
Figure 3. Metastatic adenoid cystic carcinoma to scalp. (A) No connection to epidermis observed; hematoxylin and eosin, original
magnification x 5. (B) Cellular detail; hematoxylin and eosin, original magnification x 20. (C) Ductal components outlined with cytokeratin
(CK) 8/18, original magnification x 10. (D) Ductal components outlined with epithelial membrane antigen (EMA), original magnification x 20.
Discussion

Differentiating between primary cutaneous ACC and cutaneous metastases originating from an extracutaneous ACC can be
difficult, but it has important implications for management, specifically whether chemotherapy should be added to surgical
excision [1, 6]. Distant cutaneous metastases from extracutaneous ACCs are rare and carry a poor prognosis [4, 5]. Table 1
presents all 16 cases of extracutaneous adenoid cystic carcinoma metastasizing to cutaneous sites that are documented in the
available literature, including the present case. The present case is the second reported case of an ACC metastatic to the scalp.
In general, there are no histopathological differences between a primary cutaneous ACC and a cutaneous metastasis from an
extracutaneous ACC [9]. Immunohistochemical results in cutaneous ACC closely resemble those of salivary gland ACC. One
noteworthy difference is that CEA is consistently positive in extracutaneous ACC, whereas it is only positive in half of the cases
of cutaneous ACC [5]. As is typically observed in ACC, the present case was positive for CEA, EMA and cytokeratins AE1/AE3,
CK7, and CK 8/18 [9, 10]. Interestingly, although basal cell carcinomas can stain positively for most of the aforementioned
immunohistochemical markers, basal cell carcinomas are typically negative for EMA, except for focal staining of
keratinizing/squamous areas. Thus, EMA can be very useful in differentiating between a basal cell carcinoma and ACC [11].

CD117 and focal expression of vimentin and S100 can also be observed in ACC [4, 5, 9]. Because primary cutaneous ACCs can
be misclassified as metastases, one could argue that the present case is a primary cutaneous ACC rather than a metastasis, even in
light of the nearly identical histopathology in the tumors examined. Primary cutaneous ACC, however, should only be diagnosed
with certainty if the patient does not have a history of extracutaneous ACC [5].
Conclusion
In conclusion, the present case demonstrates a rare yet relevant scenario that clinicians must consider when dealing with patients
who have a history of ACC. The distinction between primary cutaneous ACC and a metastasis originating from an extracutaneous
ACC is difficult, but it has critical implications for prognosis and selection of therapy. Primary cutaneous ACC should only be
diagnosed with certainty if the patient has no history of extracutaneous ACC. Furthermore, in addition to being monitored closely
by oncologists, patients with a history of ACC should also be followed by dermatologists with regularly scheduled total body skin
exams.
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