Year : 2009 | Volume
: 52 | Issue : 4 | Page : 543--545
Adenoid cystic carcinoma of uterine cervix in a young patient
Ankit Seth1, Asha Agarwal2,
1 Department of Pathology, Kasturba Hospital, Daryaganj, Delhi-2, India
2 Department of Pathology, GSVM Medical College, Kanpur, UP, India
80, Thomson Road, MCD Officers«SQ» Colony, Near Shivaji Park, Minto Road, New Delhi-110 002
Adenoid cystic carcinoma of uterine cervix is a rare tumor. Its origin is debatable. It has a high incidence in postmenopausal women but rarely can develop in patients under 40. An association with squamous cell carcinoma has been described. We report a case of adenoid cystic carcinoma of the endocervical canal with foci of squamous cell carcinoma in a 34-year-old suffering from menorrhagia associated with blood-stained vaginal discharge. Per vaginum and per speculum examination revealed a growth. Cervical biopsy showed bits of tissue, suggesting adenoid cystic carcinoma. Patient was operated upon and uterus with cervix sent for histopathological examination. We report this case because of its rarity, particularly in young patients, with description of illustrative pathology and discussion on the histological diagnosis.
|How to cite this article:|
Seth A, Agarwal A. Adenoid cystic carcinoma of uterine cervix in a young patient.Indian J Pathol Microbiol 2009;52:543-545
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Seth A, Agarwal A. Adenoid cystic carcinoma of uterine cervix in a young patient. Indian J Pathol Microbiol [serial online] 2009 [cited 2020 Feb 24 ];52:543-545
Available from: http://www.ijpmonline.org/text.asp?2009/52/4/543/56158
Adenoid cystic carcinoma is more common in the salivary glands and respiratory tract glands. Occasionally, it is found in the mucus membrane of head and neck, skin, and breast. In the uterine cervix, it is a rare and peculiar variant of adenocarcinoma and is mostly seen in postmenopausal women. This tumor represents 3% of all primary cervical adenocarcinomas,  and it is locally aggressive and capable of metastasis to other organs even in its early stage. This case is being reported because of its rarity, especially in women of less than forty years of age.
A 34-year-old woman presented with the complaints of continuous vaginal bleeding for two months, associated with blood-stained vaginal discharge. Her past medical history including menstrual history was normal. Except anaemia related findings, nothing significant was found on her general examination. On per vaginum examination, an exophytic friable growth was found arising from cervix and occupying the upper part of vaginal cavity, without involving its wall. Per speculum examination showed a large growth present on cervix that bled on touch. Her hemoglobin level was 10 gm%. Other blood profile and biochemical investigations were within normal limits. Sonography revealed an echogenic mass in the region of cervix. Lungs were radiologically clear. Wertheim's extended abdominal hysterectomy was done and uterus with cervix was sent for histopathological examination.
Gross examination of the operated specimen showed a large grayish-white growth, measuring 3x3 cms, involving both lips of the cervix and obliterating the endocervical canal.
Microscopic examination of tissue sections of the tumor showed tumor cells disposed in cribriform as well as in solid pattern. The cribriform pattern manifested as a punched-out or "Swiss cheese" arrangement of tumor cells [Figure 1], surrounding acellular spaces containing hyaline material, the latter being the reduplicated basement membrane [Figure 2]. The tumor cells were composed of dense basophilic nuclei containing very inconspicuous nucleoli. Mitotic figures were rarely found. The solid pattern was characterized by large masses or nests of uniform basaloid tumor cells. Occasional prominent areas of necrosis were seen within the central portions of solid nests. Foci of moderately differentiated squamous cell carcinoma were also identified, distinct and separate from the adenoid cystic carcinoma [Figure 3]. The adenoid cystic component was strongly positive for smooth muscle actin while hyaline stroma was periodic acid-Schiff reaction [PAS] positive. A histopathological diagnosis of adenoid cystic carcinoma of cervix with foci of squamous cell carcinoma was made.
Adenoid cystic carcinoma (ACC) is more common in salivary and lacrimal glands. It is a rare but highly specific undifferentiated cervical neoplasm due to its distinct morphology and different biological behavior.  Previously, adenoid cystic carcinoma of cervix was considered as the disease of the postmenopausal women, with average age of presentation found to be 20 years older than squamous cell carcinoma of cervix. However, later reviews mentioned it in below 40 years of age also , but such examples are very few. ,
Etiology of the tumor is unknown. However, Yang and Gordon  reported a case of cervical adenoid cystic carcinoma coexisting with a variety of human papilloma virus (HPV) - related lesions including condyloma acuminata, vulvar intraepithelial neoplasm, cervical intraepithelial neoplasm, and invasive basaloid squamous cell carcinoma which raises the speculation that HPV may also be the causative factor for adenoid cystic carcinoma of uterine cervix.
The tumor probably originates from the cervical multi-potent reserve cells.  It is most often associated with foci of squamous cell carcinoma,  as in our case. Vaginal cytology is abnormal in most cases comprising of small cells arranged in cribriform pattern and dsysplastic or malignant squamous cells with presence of hyaline material in the background (suggestive of remnants of lamina densa of basement membrane of malignant cells). Despite the tumors' architectural similarity to adenoid cystic carcinoma of the salivary gland, the cervical adenoid cystic carcinomas show necrosis, a high mitotic rate, and greater nuclear pleomorphism than its salivary gland counterpart. 
The most important differential diagnosis of cervical adenoid cystic carcinoma is adenoid basal carcinoma (ABC). However, cervical adenoid cystic carcinoma has several clinico-pathologic features that will allow distinction from ABC. The differentiation is important as unlike ACC, the overwhelming majority of cases of ABC are benign owing to the low potential for recurrence and metastasis.  Both tumors are common in the postmenopausal women but patients with ABC are usually asymptomatic, without a gross abnormality of the cervix. Histologically, ABC has been described as a proliferation of nested uniform, bland basaloid cells with a peripheral palisading pattern. Mild chromatin abnormalities are present with small nucleoli and rare mitoses.  Cellular pleomorphism, mitoses, necrosis, stromal hyalinization, and metastasis are commonly identified in ACC but are rare or absent in ABC. Immunohistochemical studies show positive reactivity to actin, collagen IV, and laminin for ACC whereas these stains do not highlight the tumor cells of ABC though both show positive reactivity to CEA, EMA, CK7, CK20, and CAM 5.2. 
Adenoid cystic carcinoma of uterine cervix is traditionally associated with poor prognosis due to widespread lymph node and vascular metastases, especially to lungs, which is the commonest site of metastasis.  Cause of early metastasis is unclear. In one study,  it was shown that the expression of cyclo-oxygenase-2 (COX-2) in the tumor might induce the expression of vascular endothelial growth factor (VEGF), increase angiogenesis (as proved by expression of CD34), and enhance tumor growth and invasion, with the result that patients with more than II B do not have survival rate over five years. Treatment is a judicious combination of radical pelvic surgery, radiotherapy and chemotherapy except in the patients with stage I (especially those with histological features associated with a higher risk of recurrence) who should receive aggressive local therapy alone. 
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