|Year : 2018 | Volume
| Issue : 1 | Page : 77-80
Adenoid cystic carcinoma – Solid variant
A Raviprakash, M Rajini Kanth, A Vikram Simha Reddy, JK Sonia Bai
Department of Oral Pathology, G. Pullareddy Dental College and Hospital, Kurnool, Andhra Pradesh, India
|Date of Web Publication||22-Mar-2018|
Dr. J K Sonia Bai
Department of Oral Pathology, G. Pullareddy Dental College and Hospital, Kurnool - 518 007, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Adenoid cystic carcinoma (ACC) accounts for about 1% of all head and neck malignancies and 6–10% of all salivary gland neoplasms. ACC is a rare slow growing salivary gland neoplasm with a high recurrence rate. It can occur in any salivary gland site, but approximately 50–60% develop within the minor salivary glands especially palate. Rarely, ACC arising intraosseously have also been reported. ACC is characterized for its wide local infiltration, perineural spread, local recurrence, and distant metastasis. Pain is a common and important finding, occurring early in the course of the disease before there is a noticeable swelling, which is mainly due to neoplastic cell neurotropism. This report describes a case of ACC in the left posterolateral portion of the hard palate in a 36-year-old woman, along with review of literature on ACC.
Keywords: Adenoid cystic carcinoma, palate, salivary glands
|How to cite this article:|
Raviprakash A, Kanth M R, Reddy A V, Sonia Bai J K. Adenoid cystic carcinoma – Solid variant. J NTR Univ Health Sci 2018;7:77-80
| Introduction|| |
Adenoid cystic carcinoma (ACC) accounts for about 1% of all head and neck malignancies. It is a relatively rare epithelial tumor of the salivary glands  with high recurrence rate and slow rate of growth. ACC is a rare malignant tumor, which has also been referred as “cylindroma,” “basiloma,” “adenoid basiloid carcinoma,” and “adenoepithelioma.” In 1859, it was named as “cylindroma” by Theodor Billroth, as elements of epithelial and connective tissue formed a system of interwining cylinders. In 1954, the term adenoid cystic carcinoma was coined by Ewing. Cases of ACC arising centrally within the jaw bones also have been reported.
ACC is a high-grade malignant tumor and accounts for 8.3% of all palatal salivary gland tumors. The present paper reports a case of ACC in the left posterolateral portion of the hard palate in a 36-year-old woman.
| Case History|| |
A 36-year-old female patient visited private dental hospital in Kurnool, with the chief complaint of swelling on the left side of the palate since 3 months. History revealed that the swelling started insidiously and gradually increased to the present size. The swelling was associated with dull and continuous pain and it was not associated with any discharge. Her past medical and personal histories were non- contributory. On general examination, the patient was moderately built and nourished.
On extra-oral examination, facial asymmetry was seen on the left side of middle third of face. A diffuse swelling [Figure 1] approximately of size 2 × 2 cm was noted anteroposteriorly 3 cm from the corner of the mouth to the zygomatic arch. The overlying skin was normal and not associated with lymphadenopathy.
|Figure 1: Extra-oral view showing a diffuse swelling on the left middle third of the face|
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On intra-oral examination, a solitary ulcerated oval shaped swelling of size 5.5 × 5.5 cm was noted on the left postero-lateral region of hard palate [Figure 2]. Anteriorly, it extends from left side of mid palatal rugae and posteriorly to the soft palate. Medially it extends from the midline of palate, not crossing the midline and laterally to the dentoalveolar process of teeth 24–27 region. Paraesthesia of the upper lip was also noted. On palpation, the swelling was tender and firm in consistency.
|Figure 2: Intra-oral view showing an ulcerated swelling on the left side of palate extending from 24- 27 region|
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A CT scan [Figure 3] of paranasal sinus view was suggestive of hyperdense area in the left maxillary sinus extending medially to the inferior turbinate, laterally contained within left maxillary sinus and posteriorly to the pterygoid plates. The teeth were displaced completely from their socket. Radiological investigations like chest PA X-ray view, and ultrasound abdomen were performed to identify distant organ metastasis. The ultrasound of upper abdomen showed normal liver, gall bladder, pancreas, spleen, and kidneys with no evidence of any metastatic mass and PA X-ray view of chest also confirmed negative metastasis of the tumor. After confirming the nature of lesion and evidence of no distant organ metastasis, the patient was posted for surgery under local anesthesia.
Differential diagnosis was given as pyogenic granuloma, low-grade adenocarcinoma, mucoepidermoid carcinoma, and polymorphous low-grade carcinoma.
Excisional biopsy was performed and the grossing specimen was sent to our department. Multiple soft tissue bits and one bit of soft tissue associated with a tooth were received.
The H&E stained soft tissue section showed fibrovascular connective tissue exhibiting numerous islands showing small cuboidal cells with deeply basophilic nucleus and scanty cytoplasm in tumor cells [Figure 4]. The tumor cells demonstrate little tendency for cyst formation. Cellular pleomorphism and mitotic activity was also evident. The histopathological impression was that of an adenoid cystic carcinoma of solid pattern. CD117 is strongly positive of immunohistochemistry, which confirms ACC showing solid pattern [Figure 5] and [Figure 6].
|Figure 4: Hematoxylin & Eosin Section showing numerous islands of tumor cells and with a little tendency for cystic formation denoting solid pattern of Adenoid Cystic Carcinoma (10x view)|
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|Figure 5: Immunohistochemistry - Tumor cells showing strongly positivity to C-KIT (CD 117) (x4)|
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|Figure 6: Immunohistochemistry - Tumor cells showing strongly positivity to C-KIT (CD 117) (x10)|
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After diagnosis of the lesion as solid variant of ACC, the patient underwent surgical excision of the lesion followed by chemotherapy. As solid variant has poor prognosis, the patient is under follow up since 6 months. Uneventful healing is seen at the lesion site.
The patient's consent has been taken to report the case. Patient's consent form has been attached with the article.
| Discussion|| |
Adenoid cystic carcinoma (ACC) is the second most common malignancy arising in the salivary glands following mucoepidermoid carcinoma. ACC was first described as “cylindroma” by Theodar Billroth in 1859 based on its histological features., In 1908, it was named as “Basiloma” by “Krompecher” as the tumor is analogous to the basal cell growths of the skin. ACC occurs at any age and most commonly between 4th to 7th decade with slight female predilection.
Among salivary gland neoplasms, 50% are malignant in which 9% to 23% of tumors occurs intraorally  and have also been found to occur in lacrimal gland, auditory canal, larynx, trachea, lungs, breast, skin, lower respiratory tract, cervix, and vulva, and within the bone have also been reported especially at the posterior body or angle of mandible. Numerous theories were proposed to explain the origin of salivary gland tumors centrally in the bone.
ACC is a slow-growing, indolent tumor but runs a relentless course. Different morphologic configurations of basal epithelial and myoepithelial cells are seen in ACC. Capsule is not seen in most of the cases, and consists of pseudoglandular, “cribriform” structures with small glandular lumens. About 25–50% of cases show distant metastasis even many years after the diagnosis, and lung is the most involved site. The differential diagnosis of ACC includes polymorphous low grade carcinoma, basal cell adenoma (BCA), mixed tumor, and basaloid squamous cell carcinoma.
Three histological patterns of growth have been described: Cribriform, tubular, and solid variants. Solid variants of ACC are high grade lesions with reported recurrence rates of 100% compared to 50–80% for tubular and cribiform variants. Combinations.
Immunohistochemical studies demonstrated that the duct lining cells are positive for C-kit (CD117) and myoepithelial cells are positive for S-100 protein, calponin, p63, smooth muscle actin, and myosin. P53 mutations appear to be involved with tumor progression and recurrence.
The prognosis is found to be better with the cribiform or tubular growth patterns, in contrast to the solid pattern, which indicates an aggressive clinical course. As our present case is of solid variant which is aggressive in nature, the patient was advised for further investigations to rule out metastasis and kept under follow up.
| Conclusion|| |
Adenoid cystic carcinoma is a rare slow growing salivary gland malignancy. It is famous for its peculiar histopathological features, variants, and tendency for perineural invasion. At times, it may metastasize to regional lymphnodes and worsen the prognosis of the case and require prolonged follow-up.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]