|Year : 2016 | Volume
| Issue : 1 | Page : 40-43
Adenoid cystic carcinoma of the upper lip: A case report and review of literature
Bharat Bhushan Dogra, Gaurav Batra, Chandrashekhar Wahegaonkar, Bhushan Patil
Department of Plastic Surgery, Dr. D. Y. Patil Medical College, Pune, Maharashtra, India
|Date of Web Publication||21-Jul-2016|
Bharat Bhushan Dogra
Department of Plastic Surgery, Dr. D. Y. Patil Medical College, Pimpri, Pune - 411 018, Maharashtra
Source of Support: None, Conflict of Interest: None
Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm of the salivary glands. Although it is a slow-growing malignant tumor, it carries a poor prognosis due to aggressive nature of tumor and a high rate of recurrence. It usually affects elderly patients, and the peak incidence is in the fifth and sixth decades with a female preponderance. Perineural invasion along cranial nerves is a pathognomonic feature of ACC and is believed to be responsible for the high rate of local recurrence. Surgery is the treatment of choice, but some cases may require radiation therapy after surgery. Rarely, chemotherapy may also be required to control the disease. This report pertains to a rare case of ACC of the upper lip managed by excision and primary reconstruction of the lip with gratifying postoperative results.
Keywords: Adenoid cystic carcinoma, perineural invasion, salivary glands
|How to cite this article:|
Dogra BB, Batra G, Wahegaonkar C, Patil B. Adenoid cystic carcinoma of the upper lip: A case report and review of literature. Int J Oral Health Sci 2016;6:40-3
|How to cite this URL:|
Dogra BB, Batra G, Wahegaonkar C, Patil B. Adenoid cystic carcinoma of the upper lip: A case report and review of literature. Int J Oral Health Sci [serial online] 2016 [cited 2020 Oct 25];6:40-3. Available from: https://www.ijohsjournal.org/text.asp?2016/6/1/40/186666
| Introduction|| |
Adenoid cystic carcinoma (ACC) is a rare malignant neoplasm of the salivary glands. Among the head and neck tumors, salivary gland neoplasms contribute about 2-6.5% of tumors and ACCs contribute 5-10% of all salivary gland tumors.  This tumor is frequently located in minor salivary glands (31%) although it may also be found in the submandibular gland (14%) and the parotid gland (2%). 
It usually presents as a painless lump. Occasionally, it may present as an ulcerated lesion on the palate. Characteristic features of ACC are local recurrences, perineural spread, and late distant metastases.
| Case Report|| |
A 62-year-old male patient was referred to the plastic surgery outpatient department, for opinion regarding a slowly growing ulcerative lesion over the inner surface of the upper lip of 1-year duration. The patient happened to be a chronic tobacco chewer for the last 25 years. Local examination revealed a solitary, oval-shaped ulcerative growth with induration all around measuring 1 cm × 1 cm in diameter. The outer skin was, however, not involved [Figure 1]. The swelling was extending neither to the vermilion border of the lip nor to the gingivolabial sulcus superiorly. Regional lymph nodes were not involved. Computed tomography of the thorax ruled out any evidence of pulmonary metastasis.
|Figure 1: Lesion on mucosal surface of the upper lip, outer skin not involved|
Click here to view
Wide local excision with 1 cm margin all around and primary reconstruction using lip switch Abbe flap from mid-lower lip were carried out [Figure 2]a-c and [Figure 3].
|Figure 2: Operative photographs. (a) Ulcerative lesion on mucosal surface of the upper lip, (b) wide local excision of lesion, and (c) abbe flap reconstruction from mid-lower lip|
Click here to view
|Figure 3: Early postoperative picture after showing well-settled flap and well-healed donor site|
Click here to view
Histopathological examination showed tumor tissue growing in large solid sheets, with the cribriform pattern. There was evidence of extensive neural invasion by the tumor cells. Furthermore, cords and tubules of the tumor cells infiltrating into surrounding skeletal muscle with extensive desmoplastic stromal reaction were seen; however, excision margins were free from tumor [Figure 4]. The final diagnosis was ACC of the upper lip. In view of evidence of perineural invasion in excised lip, it was decided to refer the patient to the nearest radiotherapy center for radiotherapy.
|Figure 4: Histopath slide showing tumor in solid sheets, with cribriform pattern, and extensive neural invasion by the tumor cells|
Click here to view
Review after 6 months revealed no evidence of local recurrence or systemic metastasis [Figure 5].
| Discussion|| |
ACC is a malignant salivary gland neoplasm which was first described by Robin and Laboulbene in 1853.
ACC generally arises from the epithelial lining of salivary glands but can also arise from other anatomical sites such as upper aerodigestive tract, eye, and skin. In some reports, ACC from other head and neck sites such as nose, nasal sinuses, nasopharynx, and larynx has also been reported. 
In 1856, Theodor Billroth studied its histological features and described the long amorphous compartments as cylinders and coined the term "cylindroma." 
The World Health Organization in 2005 defined ACC as "a basaloid tumor consisting of epithelial and myoepithelial cells in various morphological configurations including tubular, cribriform, and solid patterns. It has a relentless clinical course and usually a fatal outcome." 
Contiguous perineural invasion is a hallmark of ACC, which partly explains its tendency to spread. Tumor surrounds and invades nerve sheaths, causing cranial or peripheral neuropathy. Perineural involvement occurs in approximately 22-46% of cases of ACC. 
Assessment of the histological grade is of significance to predict the likelihood of tumor recurrence and survival. In one series, 5-year recurrence rates of 100%, 89%, and 59% were reported for tumors with solid, cribriform, or tubular growth patterns, respectively. 
Surgery is the treatment of choice for ACC by carrying out wide local excision with a minimum of 1 cm margin all around to achieve tumor-free excision margins.
Sung et al. studied the predictive factors and the impact of distant metastases of ACC in a total of 94 patients. In this study, 25 had distant metastases while three had regional metastasis.  Other studies have shown similar results where the most common sites of metastasis were lungs (80%), bone (15%), liver and other sites (5%). The metastases may appear even 20 years after primary tumor resection, which represents the major cause of treatment failure.
Radiosensitivity of these tumors has now been firmly established despite early reports to the contrary.  Studies have shown that patients exhibiting perineural invasion require radiotherapy. Radiation therapy is also recommended when the surgical margins are not tumor free or in cases where tumor is present over surgically nonaccessible region. Radiation therapy consists of daily treatments, five times a week for 6 weeks. Each treatment involves 2 Gray (Gy) of radiation and total radiation exposure to the target area of 60-70 Gy. Silverman et.al reported his study of 75 cases, where 25 cases were managed only with surgery and 50 were treated with surgery and post operative radiotherapy. There was no significant difference in outcome between two treatment groups when correlated with tumor site. However, postoperative radiation was associated with improved overall survival for advanced T-stage (T4) tumors and patients with microscopically positive margins. There was no demonstrable benefit of postoperative radiation for patients with microscopically negative margins. There was no significant difference in outcome between two treatment groups when correlated with tumor site. However, postoperative radiation was associated with improved overall survival for advanced T-stage (T4) tumors. There was no demonstrable benefit of postoperative radiation for patients with microscopically negative margins. 
Response to chemotherapy in the management of recurrent and metastatic disease is found to be too low and usually only for a short duration. Dodd and Slevin reviewed 114 publications where chemotherapy as well as role of molecular target therapy, epidermal growth factor receptor, human epidermal growth receptor-2, etc., were studied, but response rates were found to be low and duration short lived.  The survival rate varies among the various studies. DeAngelis et al. reported overall survival rates of 92% at 5 years, 72% at 10 years, and 54% in 20 years. 
| Conclusion|| |
ACC of minor salivary glands in the upper lip is quite rare tumor, which presented in the case report being presented here as an ulcerative mass on the mucosal surface of the upper lip. Wide local excision with primary reconstruction of lip was carried out. Excised specimen showed tumor-free excision margins; however, perineural invasion was noticed. Adjuvant radiotherapy should be given postsurgery in cases showing microscopic evidence of tumor in excision margins or if perineural invasion is noticed to achieve better control of disease.
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.
| References|| |
Santos GC, Martins MR, Pellacani LB, Vieira AC, Nascimento LA, Abrahão M. Neoplasms of Salivary Glands: Study of 119 casess. J Bras Patol Med Lab 2003;39:371-5.
Huber PE, Debus J, Latz D, Zierhut D, Bischof M, Wannenmacher M, et al.
Radiotherapy for advanced adenoid cystic carcinoma: Neutrons, photons or mixed beam? Radiother Oncol 2001;59:161-7.
Abdul-Hussein A, Morris PA, Markova T. An unusual presentation of adenoid cystic carcinoma of the minor salivary glands with cranial nerve palsy: A case study. BMC Cancer 2007;7:157.
Al-Sukhun J, Lindqvist C, Hietanen J, Leivo I, Penttilä H. Central adenoid cystic carcinoma of the mandible: Case report and literature review of 16 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006;101:304-8.
Barrett AW, Speight PM. Perineural invasion in adenoid cystic carcinoma of the salivary glands: A valid prognostic indicator? Oral Oncol 2009;45:936-40.
Perzin KH, Gullane P, Clairmont AC. Adenoid cystic carcinomas arising in salivary glands: A correlation of histologic features and clinical course. Cancer 1978;42:265-82.
Sung MW, Kim KH, Kim JW, Min YG, Seong WJ, Roh JL, et al.
Clinicopathologic predictors and impact of distant metastasis from adenoid cystic carcinoma of the head and neck. Arch Otolaryngol Head Neck Surg 2003;129:1193-7.
Parsons JT, Mendenhall WM, Stringer SP, Cassisi NJ, Million RR. Management of minor salivary gland carcinomas. Int J Radiat Oncol Biol Phys 1996;35:443-54.
Dodd RL, Slevin NJ. Salivary gland adenoid cystic carcinoma: A review of chemotherapy and molecular therapies. Oral Oncol 2006;42:759-69.
DeAngelis AF, Tsui A, Wiesenfeld D, Chandu A. Outcomes of patients with adenoid cystic carcinoma of the minor salivary glands. Int J Oral Maxillofac Surg 2011;40:710-4.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]