The first case of adenoid cystic carcinoma of the cervix was reported as cylindroma in 1949 by Paalman and Counseller [
6]. To date, approximately 150 cases have been reported in the world literature. The most accepted view regarding its origin in the cervix is from "reserve cells" of endocervix. Most of these tumors are found in multiparous women during their post-menopausal life. Though, it has been reported from 24 to 99 years of age, majority of the cases have been reported during 7
th to 9
th decades of life [
1,
5,
7-
9]. Despite the fact that cervical cancer is the most common malignancy in Indian women, only 19 cases of ACC of the cervix have been reported from India and majority of Indian patients were reported during 5
th decade of their life [
10-
14]. This difference in age group is similar to the observation in squamous cell carcinoma of the cervix, which is also seen two decades earlier in Indian patients than in other countries [
10]. Cervical ACC rarely occurs in patients younger than 40 years of age. Including the present patient, only 10 cases of ACC have been found in women under 40 years of age [
7,
10,
12,
15-
17]. Our patient is one of the youngest reported case of ACC of the cervix in the literature. She was 30 years of age when her primary disease in the cervix was diagnosed in 1988.
Metastatic pattern of ACC of the cervix is distinctive. Extensive local infiltration and invasion of lymphatic vessels as well as perineural spaces, with subsequent hematogenous spread are characteristic [8]. ACC originating from various other anatomic sites are known for their slow growth, late local recurrence as well as late distant metastatic presentation [2,18-19]. Maximum interval between a primary treatment and pulmonary metastasis from a cutaneous adenoid cystic carcinoma (ACC) has been reported to be 18 years and the pulmonary metastases was also diagnosed by fine needle aspiration cytology similar to our patient [19]. Our patient had multiple, bilateral lung lesions on chest X-ray, highly suggestive of secondary lesions rather than a primary ACC of the lung, which is usually a solitary mass. Moreover, the patient did not have any clinical or radiological evidence to suggest other location as a second primary site for ACC (parotid and submandibular salivary glands, oral cavity, paranasal sinuses and nasopharynx as well as larynx were negative for the presence of any growth). Examination of the skin and breasts was also negative. Thus, presence of multiple, bilateral lesions in lungs, previous history of similar histology at cervix and absence of lesion at other potential sites led us to be believe that pulmonary lesions were metastatic ACC from the primary cervical lesion.
Surgery and radiation therapy either alone or in combination has been used in the treatment of ACC of the cervix. Chemotherapy has very little role in the management of these tumors but has been used for advanced/recurrent/metastatic disease without much success [4,5]. It is an aggressive tumor and traditionally associated with poor prognosis resulting in death in 50-60% of patients and in almost all individuals with stage II disease or greater [20]. Majority of deaths have been reported within two years of diagnosis. Prior to this report, only Musa et al [9] reported 11 years disease-free survival in one of their patients.
ACC of the cervix is an aggressive but radiosensitive tumor. In the present case, despite an advanced disease (stage III-b) at presentation in 1988, the patient had responded well to radiotherapy and remained asymptomatic for 11 years, when she developed pulmonary metastases. Other organs were not involved and the primary site at cervix was also under control. Among the documented cases, this is the longest interval reported for pulmonary metastasis from adenoid cystic carcinoma of the cervix. It is difficult to explain the very long interval between the treatment of primary disease and diagnosis of pulmonary metastases in our patient. It could be due to the fact that majority of the reported cases of ACC of cervix were found in old age during 7th to 9th decades and did not survive such a long period of time. Only ten patients (including the present case) were young patients. Rarity of this event in cervical ACC deserves attention.
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![Figure 2: (a,b,c). Photomicrograph of USG guided biopsy of the lung lesion showing metastatic adenoid cystic carcinoma [H&E x100 (a), x400 (b,c)]](javascript:openWin('images/figure_TJC_74_1.jpg', 640, 480)){kind=link}