|Year : 2022 | Volume
| Issue : 1 | Page : 19-22
Clinicoepidemiological profile of adenoid cystic carcinoma: Experience from a tertiary care oncology center in Kashmir, India
Mushtaq Ahmad Sofi, Mohmad Hussain Mir, Kaneez Fatima, Asifa Andleeb, Farhana Siraj, Nazir Ahmad Dar
Radiation Oncology, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
|Date of Submission||24-Sep-2021|
|Date of Acceptance||16-Nov-2021|
|Date of Web Publication||15-Feb-2022|
Dr. Mohmad Hussain Mir
Radiation Oncology, Sheri Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Background: Adenoid cystic carcinoma (ACC) is a relatively rare tumor mostly involving head and neck region of the body. Due to lack of high-quality evidence, optimal treatment is still controversial and continues to evolve over time. Aim and Objectives: To analyze the clinicoepidemiological profile of ACC in Kashmir. Materials and Methods: Data of 30 patients, who were treated in the Regional Cancer Center at She-i-Kashmir Institute of Medical Sciences (Deemed University, Srinagar) between January 2015 and December 2019, were reviewed with respect to their demographic and clinical profile. Results: The study included 30 patients with ACC. The mean age at presentation was 55 ± 12.15 years. There were 10 (33.3%) males and 20 (66.6%) females. Among salivary gland ACC s, sublingual gland (06/30, 20%) was the most common site followed by parotid gland (05/30, 16.7%). Nonhead and neck primary ACC cases were reported from lung (6.7%) and breast (6.7%). Majority of the patients presented in stage-III (43.3). Only 5 patients presented with distant metastasis. All patients were subjected to surgery. Adjuvant radiotherapy was given in 18 patients (60%). Three patients received all three modalities (surgery, radiotherapy, and chemotherapy). The overall one year survival was 96%. However, the mean & median survivals reported were 24 & 18 months respectively. Surgery with negative surgical margin is the first choice of treatment for the patients with ACC. Our findings show that the prognosis of patients with ACC in our center is comparable to other parts of country. Conclusion: ACC has locally aggressive behavior. Adjuvant radiotherapy improves local control in locally advanced disease. Longer follow-up is mandatory in view of incidence of late metastasis.
Keywords: Adenoid cystic carcinoma, natural history, salivary gland neoplasm, survival
|How to cite this article:|
Sofi MA, Mir MH, Fatima K, Andleeb A, Siraj F, Dar NA. Clinicoepidemiological profile of adenoid cystic carcinoma: Experience from a tertiary care oncology center in Kashmir, India. J Radiat Cancer Res 2022;13:19-22
|How to cite this URL:|
Sofi MA, Mir MH, Fatima K, Andleeb A, Siraj F, Dar NA. Clinicoepidemiological profile of adenoid cystic carcinoma: Experience from a tertiary care oncology center in Kashmir, India. J Radiat Cancer Res [serial online] 2022 [cited 2022 May 17];13:19-22. Available from: https://www.journalrcr.org/text.asp?2022/13/1/19/337774
| Introduction|| |
Adenoid cystic carcinoma (ACC) is a rare form of adenocarcinoma, a type of cancer that begins in glandular tissues. This entity was first described by Theodar Billroth in 1859. It commonly arises in the salivary glands (minor as well as major salivary glands) of the head and neck; however, it can arise in any site lined with mucous glands. These tumors account for 10% of salivary gland tumors and <1% of head and neck neoplasms. Among salivary glands, ACC commonly involves submandibular gland. ACC has also been seen to arise from other sites such as lungs, breast, nasopharynx, lacrimal glands, esophagus, trachea, prostate, cervix, and external auditory canal.,,
Most of the ACC patients are diagnosed in fourth to sixth decade of life and have slight female predisposition (male-to-female ratio, 2:3).,,, There are no known risk factors that can lead to this type of malignancy. Although some studies have reported that P53 suppressor gene is inactivated in advanced and aggressive forms of this type of cancer, no specific molecular abnormalities that underlie this disease process are known.
ACC is a slow-growing tumor and most patients do well. The diagnosis is made by histological analysis of a biopsy, and treatment is decided after proper staging workup that includes history and physical examination, imaging, and tissue diagnosis with immunohistochemistry. Taking into consideration the nature of tumor, there are many controversies regarding the treatment of ACC. However, surgery is considered the standard of care with or without radiotherapy. After treatment, long-term follow-up is needed in view of late recurrences (both local and distant relapses). It has been reported that distant metastasis occurs quite earlier (within first 5 years posttreatment) than local recurrences. Long-term follow-up regardless of site of tumor is, thus, needed, in view of late local recurrences. The goal of this study was to analyze the clinicodemographic profile, management, and outcome of ACC patients treated at a tertiary care cancer center in Kashmir valley (Sher-i-Kashmir Institute of Medical Sciences, SKIMS, Soura, Srinagar).
| Materials and Methods|| |
This was a retrospective study where medical records of 30 biopsy-proven ACC patients who were registered in the regional cancer center, SKIMS, Srinagar, from January 2015 to December 2019 were reviewed to analyze the data on demographics, presenting symptoms, tumor characteristics, treatment protocols, and outcome. Overall survival was calculated from date of biopsy or surgery to the date of death or last follow-up.
Statistical analysis was done using SPSS software v25 (IBM, SPSS, Chicago, Illinois, USA). Overall survival was done using Kaplan–Meier Survival method. All results were discussed at 5% level of significance, i.e., P < 0.05.
| Results|| |
The study included 30 patients with ACC. The mean age at presentation was 55 ± 12.15 years. There were 10 (33.3%) males and 20 (66.6%) females [Table 1]. Among salivary gland ACCs, sublingual gland (06/30, 20%) was the most common site followed by parotid gland (05/30, 16.7%). Nonhead and neck primary ACC cases were reported from lung (6.7%) and breast (6.7%) [Table 2]. During pretreatment evaluation, most of the patients had been staged with computed tomography (60%) followed by magnetic resonance imaging (40%). Histopathological examination by tissue biopsy (incisional as well as excisional) was done in all cases. Most of the patients in our study presented in locally advanced stage (43.3%) followed by 40% cases with localized disease. Only 16.7% of cases (05/30) had distant metastasis (stage IV) at presentation. It has also been noticed that majority of the patients had grade-I tumors (73.3%) followed by 23.3% (Grade-II) [Table 3]. All patients were subjected to surgery. Adjuvant radiotherapy was given in 18 patients (60%). Three patients received all three modalities (surgery, radiotherapy, and chemotherapy) [Table 4]. The overall one year survival was 96%. However, the mean & median survivals reported were 24 & 18 months respectively.
| Discussion|| |
ACC is an uncommon and usually an indolent type of malignancy arising within glands and occurring mainly in the head and neck, but it can also arise in the lungs, breast, trachea, lacrimal glands, skin, and vulva. Although, a slow-growing tumor, ACC behaves as locally aggressive tumor and postoperative, it has high propensity of local recurrence due to the tumor's ability disseminate widely through submucosal and fibrous tissue planes around the primary tumor site and extend via the perineural route through major and minor nerves. The risk of local recurrence is decreased with a surgical procedure with adequate resection margins followed by adjuvant radiotherapy.
ACCs arising in the head and neck have poor prognosis as compared to tumors arising from other sites (especially breast) that may have a better prognosis.
In this study, we studied demographics, clinical features, treatment, and outcome of ACC patients diagnosed from January 2015 to December 2019. A total of 30 ACC patients were analyzed in this study. ACC has preponderance for the elderly, with the highest number of cases in the head and neck areas, which is consistent with our study, in which most patients presented in the fourth and fifth decades of life. There was a slight female predominance among the cases, which was similar to the available literature.,,
In the present study, major salivary glands were the most commonly affected. The most common major salivary gland was the sublingual gland followed by parotid gland, which was in contrast to the literature where submandibular gland was commonly involved., Besides salivary glands, cases of ACC were also reported from breast (6.7%), lung (6.7%), orbit (3.3%), ear (6.7%), hard palate (6.7%), and others (23.3%). As per a study done by Glazebrook KN et al., primary ACC breast accounts for 0.056% of ACC cases, which is in contrast to our study and may be due to small sample size.
The clinical presentation largely depends on the site of tumor. Most patients, especially head and neck ACC cases, present with painless nonulcerated mass lesions, and hence, diagnosis is often delayed. Advanced tumors may present with pain/nerve paralysis.,,, Similarly, in our study, majority of the patients presented with painless swellings (83.3% cases), followed by diplopia (3.3%), ptosis (3.3%), bleeding through ear (3.3%), and cough (3.3%).
During pretreatment evaluation, most of the patients had been staged with computed tomography (60%) followed by magnetic resonance imaging (40%). Histopathological examination by tissue biopsy (incisional as well as excisional) was done in all cases. Most of the patients in our study presented in locally advanced stage (43.3%). Only 16.7% of cases (5/30) had distant metastasis at presentation. It has also been noticed that majority of the patients had Grade-I tumors (73.3%) followed by 23.3% (Grade-II).
Identification of c-Kit mutation was done in 13% of cases (06/30) and all patients demonstrated strongly positive for Kit expression on IHC. The results of our study are similar to that of the study done by Lizette Vila et al., in which fourteen patients of salivary gland ACC were assessed for c-Kit mutation and demonstrated strong Kit mutation.
The mainstay of treatment is surgical resection. Role of radiation therapy in adjuvant setting is to decrease the locoregional recurrence. Postoperative radiotherapy significantly improves regional control in the pathologically lymph node positive (pN + ve) neck. Hence, adjuvant radiotherapy is recommended in patients with T3/T4 tumors, incomplete or close resection, pathologically node positive disease, and perineural or bone involvement (Terhaad et al.). In our study, majority of the patients (50.0%) had received both surgery and radiotherapy. Only three patients had received all the three modalities (surgery + radiotherapy + chemotherapy).
The overall 5-year survival as reported by Shane Lloyed et al. was 77.3%. In view of the short follow-up of less than 5 years in this study, long-term survival as well as progression free survival could not be evaluated. However, in our study, the overall one year survival was 96%. The mean & median survivals reported were 24 & 18 months respectively. The study done by Oplatek A. et al; has reported 71 months overall median survival in their cohort of patients. It was a retrospective review of head and neck ACC treated between January 1990 & March 2007.
| Conclusion|| |
We conclude from this study that ACC is predominantly a disease of the middle-aged population with a slight female predominance. Most patients present with painless mass lesions. Complete surgical excision is the treatment of choice. Postoperative radiotherapy is indicated in cases of close or positive margins, lymph node involvement, perineural spread, as well as very large primary. These cases generally have a poorer prognosis, hence, the need for postoperative radiation therapy. Multiagent chemotherapy offers palliation in patients with advanced, recurrent, and metastatic disease. Long-term survival in these patients is generally excellent even in patients with metastatic disease due to the indolent nature of both primary and metastatic lesions. However, in this study, in view of the short follow-up of <5 years, long-term survival as well as progression-free survival could not be evaluated. Further longer-term follow-up is required.
Financial support and sponsorship
Radiation Oncology, Medical Oncology, Internal Medicine.
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3], [Table 4]