Rare case of primary adenoid cystic carcinoma of cervix: A case report

Abstract

Introduction and importance

Adenoid cystic carcinoma (ACC) is an uncommon malignant tumor with histological features commonly associated with salivary glands. Although it is thought that human papillomavirus infection is a required cause of most cases of cervical cancer, little is known about its function in the pathophysiology of ACC. Patients with ACC usually present with vaginal bleeding, and physical examination may reveal a firm, palpable mass that may be friable or ulcerated. Cervical ACC is thought to be a radiosensitive tumor, and early-stage patients treated with adjuvant radiation have shown improved outcomes than those seen in cases where surgery has been performed alone. This case is important because there are few case reports in the world, and it is the first case to be reported from Ethiopia.

Case presentation

Here we present a 65-year-old female patient presented with vaginal bleeding, and histomorphologic examination confirms the diagnosis of adenoid cystic carcinoma of the cervix.

Clinical discussion

Histologically, these tumors are composed of fairly uniform, small basaloid cells with scanty cytoplasm and rounded or angulated hyperchromatic nuclei. The cells are arranged in a cribriform pattern with central hyaline or mucinous material. Less frequent patterns including trabecular, tubular, solid, or undifferentiated patterns may be observed. Lymph vascular invasion is frequent. This case was confirmed to be primary adenoid cystic carcinoma of the cervix with all features.

Conclusion

ACC of the cervix is a rare, particularly aggressive neoplasm. It requires enhancement of postoperative treatment regimens and careful follow-up and thus needs to be distinguished from other tumors with similar histologic aspects. So histopathologic examination with IHC correlation is strongly advised to diagnose this aggressive tumor.

Highlights

• •Adenoid cystic carcinoma (ACC) is a rare malignant tumor that is most commonly seen in salivary glands but can involve many sites that have a secretory gland component
• •Cervical ACC is thought to be a radiosensitive tumor, and early-stage patients treated with adjuvant radiation have shown improved outcomes than those seen in cases where surgery has been performed alone.
• •Histopathologic examination with IHC correlation is strongly advised to diagnose this aggressive tumor.

1. Introduction

Adenoid cystic carcinoma (ACC) is a rare malignant tumor that is most commonly seen in salivary glands but can involve many sites that have a secretory gland component [1]. The first case of ACC of the cervix was reported in 1949 as a cylindroma. It makes up less than 1 % of all cervical carcinomas, making it an extremely uncommon malignancy [2]. It has mostly been reported in small case series and single case reports [[3], [4]]. Cervical ACC is typically observed in postmenopausal women, and very few occurrences in women under 40 have been documented [[5], [6]]. ACC is generally a locally aggressive tumor and has a high propensity for local recurrence and distant metastasis [7]. Therefore, it must be distinguished from other tumors that are less aggressive. Immunohistochemically, ACC cells stain positively with broad-spectrum cytokeratin, CAM 5.2, CK7, CD 117, and EMA. CEA may also be expressed. Nevertheless, cervical ACC contains few or no myoepithelial cells, which could distinguish this tumor from ACC at other sites [8]. In fact, S-100 protein reactivity occurs in some cases while others are unreactive or just focally and weakly reactive [9]. Additionally, ACC has infrequently been linked to sarcoma, invasive SCC, adenocarcinoma, and intraepithelial squamous neoplasia in the uterine cervix. [10]

This case report is written following the SCARE criteria [11].

2. Case presentation

Our case is a 65-year-old female patient from the Sidama region who has presented with vaginal bleeding of 2 months duration; initially it was intermittent and minimal in amount; later it became continuous and significant in amount associated with this. She has foul-smelling vaginal discharge, pelvic pain, pain during urination, easy fatigability, and loss of appetite. Otherwise, the patient didn't have any history of trauma to the site. She doesn't have any history of diabetes mellitus, hypertension, or immunocompromization. She doesn't have other sites bleeding.

2.1. Physical findings

On general appearance, the patient looked chronically sick and emaciated.

Vital signs were all within the normal limits.

Genitourinary examination: There is hard mass arising from the cervix that involves the upper 1/3rd of the vagina.

A bilateral inguinal area evaluation revealed no significant lymphadenopathy.

With clinical impressions of advanced cervical cancer, investigations were done on investigation.

Full blood count (WBC-8.8 × 10 9/L, Hgb-13.3 g/dl, platelet- 276 × 10 9/L), urine analysis: blood +3, RBC: 10–15, liver and renal function tests were within the normal range.

2.1.1. Abdominopelvic ultrasound

There is a heterogeneously hypoechoic cervical region mass measuring 8.6 × 6.7 cm extending to the lower uterine segment and upper 2/3 of the vaginal outlines. The mass has an obvious, clear fat plane with rectum and posteroinferior bladder wall. There is hypoechoic endometrial collection with anechoic content. Conclusion: Cervical region large mass with mild hydrometria secondary to likely cervical carcinoma.

2.1.2. Abdominopelvic CT scan

There is an ill-well defined soft tissue attenuating mass lesion seen in the cervix with internal foci of calcifications. The lesion measures 6.6 × 6.2 × 7.2 cm in size (Fig. 1). The lesion has resulted in an endometrial collection. There is no grossly visualized extension of the mass into parametrium and pelvic sidewall seen. The mass has extended into the upper portions of the vaginal canal but has not extended into the lower third of the vagina. Conclusion: Large ill-defined cervical mass lesion involving the upper third of the vagina resulting in endometrial collection secondary to cervical cancer. Histopathologic correlation was advised, and biopsy was done.

Fig. 1.

Large well-defined cervical soft tissue mass lesion (Blue arrow) involving the lower uterine segment and upper third of the vagina. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

A pap smear was done, and the finding was atypical glandular cells (favor neoplastic), not otherwise specified (NOS).

A gross evaluation of the biopsy specimen revealed multiple gray-white to gray-brown cervical tissue fragments measuring 2 cm in aggregate, all are embedded. The lesion's microscopic examination revealed a tissue with bland stratified squamous epithelium with focal ulceration and sub-epithelial lobules, solid sheets, and cribriform architectures of basaloid round to oval exhibiting moderate pleomorphism, coarse chromatin, conspicuous nucleoli, and a moderate amount of eosinophilic cytoplasm with scattered myoepithelial cells with central hyalinized globules. There is an area of necrosis and abnormal mitotic figures. There is lymphovascular but no perineural invasion noted. The conclusion of the biopsy was a malignant cervical neoplasm histomorphologically consistent with adenoid cystic carcinoma of uterine cervix (Fig. 2, Fig. 3, Fig. 4). and IHC examination with CD 117 turned out to be positive (Fig. 5) and negative for p16.

Fig. 2.

Low power view(4×) shows malignant cells with cribriform (blue arrow) and solid sheet pattern of growth with central hyaline globules (black arrow) invading in to desmoplastic stroma. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 3.

Medium power view (20×) shows basaloid cells in solid sheets (blue arrow) and cribriform pattern of growth with central a cellular hyalin globules and invading in to desmoplastic stroma with stromal retraction (black arrow showing empty spaces). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 4.

Medium power view(40×) shows hyperchromatic basaloid cells surrounding pseudoglandular spaces with central acellular hyaline globules (black arrow) and focus of necrosis noted (blue arrow). (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)

Fig. 5.

IHC examination with CD 117 turned out to be positive.

She was treated with radiotherapy and concurrent chemotherapy (CDDP given at dose 100 mg/m2). The patient has been well, without evidence of recurrent disease for 3 months after the initial treatment. Currently she is on strict follow up.

3. Discussion

This cause of ACC is still a mystery [9]. There are disagreements on this tumor's epidemiological characteristics [12,13]. Some authors suggested the association between ACC, high parity and black race [14]. Our patient was a multiparous, African and postmenopausal woman.

Clinical and radiological characteristics of primary ACC of the cervix were similar to those of a squamous cell cancer [15]. In the analysis that Dix et al. reported, the most majority of patients were diagnosed at early stages, while only 10 cases were diagnosed at stage IIIB and 4 at stage IV [15].

Histologically, these tumors are composed of fairly uniform, small basaloid cells with scanty cytoplasm and rounded or angulated hyperchromatic nuclei [5]. The cells are arranged in cribriform pattern with central hyaline or mucinous material. Less frequent patterns including trabecular, tubular, solid, or undifferentiated patterns may be observed. Lymph vascular invasion is frequent [8].

Adenoid basal carcinoma (ABC) and basaloid squamous cell carcinoma (SCC) are included in the differential diagnosis. ABC is believed to have a much less aggressive course than ACC [9]. The differential diagnosis is based on morphology; Morphologic features like cellular pleomorphism, necrosis, mitosis, and stromal hyalinization are observed in ACC; in addition, Intraluminal hyaline material, which is often present in ACC, is absent in ABC. The tumor cells in ACC tend to have less pleomorphic nuclei and show less mitotic activity. Additionally, ABC can be distinguished by the absence of CD117 immunostaining [16].

As of yet, no conventional treatment has been offered due to the disease's rarity and the lack of prospective trials. The majority of patients were incorrectly treated for squamous cell carcinoma. When combined with adjuvant radiotherapy and/or chemotherapy, surgery appears to be the preferred course of treatment depending on the clinical stage and existence of metastases. Dixit recommended surgery with adjuvant CRT for early stage, and CRT in advanced stage [15]. For the purpose of managing disease, numerous chemotherapy regimens have been employed. The most used chemotherapeutic drug is CDDP given at dose 100 mg/m2. Investigations have also been conducted on other regimens, including bleomycin, Adriamycin, and 5-fluoro-uracil [15].

The cribriform subtype has shown greater local aggressiveness and a poorer survival rate as compared with the tubular subtype [6]. Tumor features like large tumor diameter, deep stromal invasion, and the presence of tumor in the capillary lymphatic spaces are associated with high risk of recurrence and mortality in stage Ib cervical cancers patients [12]. It had been claimed that the prognosis for advanced disease (stages III and IV) was always poor [12].

ACC of the cervix seems to be more aggressive than squamous cell carcinoma of the cervix, with higher tendency to local and metastatic recurrence even if diagnosed in their earliest stages [17]. The prognosis for the solid subtype is worse in terms of distant metastases and overall survival [17].

4. Conclusions

ACC of the cervix is a rare, particularly aggressive neoplasm. It requires enhancement of postoperative treatment regimens and careful follow-up and thus needs to be distinguished from other tumors with similar histologic aspects. So histopathologic examination with IHC correlation is strongly advised to diagnose this aggressive tumor.

Abbreviations

IHC

Immunohistochemistry

ACC

Adenoid cystic carcinoma

SCC

Squamous cell carcinoma

CRT

Chemotheraphy

Author contribution

Abraham Kassahun Tadele, MD - Study concept and design, writing the paper, literature review and editing and critical review of the paper.

Fekade Yerakly Lucas, MD -Involved in acquisition of data, literature review of the paper, writing and drafting the paper, editing and critical review of the paper.

Teketel Tadesse Geremew,MD: literature review of the paper, writing and drafting the paper, editing and critical review of the paper.

Patient consent

The report was done with patient's consent.

Ethics approval

No ethics approval was needed as the case was encountered incidentally and it doesn't involve any human or animal experiment.

Guarantor

Teketel Tadesse Geremew.

Funding

No funding was provided for this case report.

Declaration of competing interest

The authors declare that there are no conflicts of interest on this case report.