A case report of cytoreductive surgery of metastatic adrenal cancer in a patient with tumor progression

Introduction

Background

The incidence of adrenal cancer is 0.7–2 cases per 100,000 population. The disease is 2–4 times more common in women than in men (1,2). According to National Comprehensive Cancer Network (NCCN) and European Society of Endocrinology (ESE), there are no definitive guidelines for the treatment of advanced and metastatic adrenal cancer (3,4).

The management of adrenal cancer continues to present considerable challenges within the medical community. Adrenal cancer is characterized by unfavorable prognoses, with minimal advancements in survival rates observed over several decades. Approximately 30% of patients diagnosed with adrenal cancer exhibit metastatic disease at the time of presentation. The efficacy of systemic therapy for them remains limited, consequently positioning cytoreductive surgery as the primary curative intervention (5-7). Available studies indicate sufficient effectiveness and safety of cytoreductive interventions (6,7), the possibility of increasing patient survival time (5), so far, the number of reports of successful cases of cytoreductive surgery is low.

Rationale and knowledge gap

In the case of progression and recurrence of adrenal gland cancer, surgical removal of metastatic lesions and recurrent neoplasms is recommended if it is possible to remove >90% of the tumor mass, and/or systemic adjuvant therapy. In cases where it is possible to remove <90% of the tumor mass, surgical treatment is recommended if there is a response to systemic adjuvant therapy. however, there is little experience of successful treatment of large adrenal tumors growing into neighboring organs (8,9). As a rule, palliative treatment is offered to these patients, cytoreductive surgical interventions remain rare cases (10-12). However, cytoreduction could improve the quality of life and survival time.

Objective

Describe a case of successful treatment of metastatic adrenal gland cancer in a patient with tumor progression. We present this case in accordance with the CARE reporting checklist (13) (available at https://acr.amegroups.com/article/view/10.21037/acr-24-198/rc).

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal. Only necessary personal information is disclosed in this case description.

Male patient O., born in 1967, has been suffering from the cancer of right adrenal gland since 2020. The patient underwent surgical treatment in April 2020: right-sided epinephrectomy. According to positronic emission tomography (PET)/CT data from February 2024, tumor progression with lung metastatic lesions on both sides and recurrence in the area of the bed of the right adrenal gland was diagnosed. Since removal of >90% of the tumor mass was impossible under such circumstances, the patient was referred for systemic chemotherapy. He got since April 2022 to July 2022 5 polychemotherapy (PCT) courses. According to computed tomography (CT) control data, negative dynamic was determined and since August 2022 the patient received mitotane 500 mg per day, under the control of the level of mitotane in the blood in the range of 14–20 µg/mL. In September 2023 CT oncoscreening, there was determined invasion of the adrenal neoplasm in the liver (Figure 1).

Figure 1 Relapse of adrenocortical cancer (red circle—tumor invasion into liver).

The patient underwent surgery September 2023—laparoscopic biopsy of a recurrent tumor of the bed of the right adrenal gland. According to immunochemistry data [evidence of liver metastasis, +++ excision repair cross-complementation group 1 (ERCC1) expression, + phosphatase and tensin homolog deleted on chromosome 10 (PTEN) expression; + resistance protein M1 (RRM1) expression, negative anaplastic lymphoma receptor tyrosine kinase (ALK) and programmed death-ligand 1 (PD-L1) expression], 5% Ki-67 positive cells, no mutations in the endothelial growth factor receptor (EGFR) gene. The patient had 3 courses of everolimus and 2 courses of PCT (gecispal + capecitabine) and then 1 course of monochemotherapy (temozolomide 350 mg 5 days).

Because of the progression of the tumor process in the area of the right adrenal gland and the mixed response to systemic therapy the patient underwent laparoscopic cytoreductive assisted excision of the recurrent neoplasm of the right adrenal gland on 04 April 2024. During the revision, a pronounced adhesion process in the epigastrium is determined, mobilization of the hepatic angle and colon is performed. The retroperitoneal space was opened, during the revision, 10 cm × 12 cm tumor was identified between the liver and the right kidney, which was intimately connected to the right lobe of the liver and the upper pole of the right kidney, with the spread of the tumor process into the liver behind (Figure 2). Using the Harmonic ultrasonic scalpel, the tumor was excised with coagulation and ligation of bleeding vessels. Due to the large size of excised tissues surgical intervention was converted conversion into the laparotomy. After desufflation and removing trocars, the abdominal cavity was opened by a lateral incision by Sergei Fedorov. A macro-preparation was removed through the wound (Figure 3). The tumor bed and liver parenchyma are coagulated with bipolar, large vessels are ligated with nylon 3.0. Hemostatic sponge Surgicel installed in the tumor bed. Hemostasis control. The abdominal cavity is drained through the counter-aperture. Total blood loss 1,000 mL. Postoperative bed-day: 11 days.

Figure 2 Intraoperative screenshot.

Figure 3 Resected tissues.

There was evidence of recurrence of adrenocortical cancer of the adrenal gland with invasion of the fibrous capsule without signs of tumor growth at the edges of the resection. According to immunohistochemistry data, there was a decrease in PTEN expression, an increase in Ki-67 expression, and a lack of PD-L1 expression. According to multispiral computed tomography (MSCT) data from May 2024 they found an increase in cells in the lungs by 2 mm, an increase in cells in the liver by 18 and 6 mm, an increase in paracaval lymph nodes by 12 mm. The patient received everolimus 10 mg per day postoperatively. The patient feels well, notes a decrease in discomfort. The prognosis remains to be serious but multimodal treatment with cytoreduction could improve survival and quality of life.

Discussion

Key findings

The given case of relapse and progression of the disease is of interest, because the removal of part of the liver during cytoreductive surgery and preservation of the kidney, which improves the quality of life. The ability to conduct adequate targeted chemotherapy according to the results of an immunohistochemical study is of great importance.

Strengths and limitations

The given case of cytoreductive intervention with satisfactory intermediate results is important for clinical oncology. However, the lack of the possibility of using PET to monitor tumor progression led to the use of MSCT, which has a lower resolving power (14). In addition, in the conditions of a large-scale military invasion, Ukrainian medical institutions are limited in their resources, which affects the treatment possibilities and the fate of patients. Thus, the limitations of this study were related to resource constraints in the context of a military invasion, so advanced monitoring methods like PET were unavailable, relying instead on MSCT with lower resolution; and intraoperative chemotherapy such as hyperthermic intraperitoneal chemotherapy (HIPEC) was not used, potentially affecting outcomes compared to studies that did.

Comparison with the similar researches

In our case, intraoperative chemotherapy was not used. Instead, in their research Tiberio et al. [2020] demonstrated that HIPEC in adrenocortical cancer may affect clinical outcomes positively (15). Weng et al. [2023] described remarkable response to a combination of mitotane, etoposide, paraplatin, and sintilimab in a patient with metastatic adrenocortical cancer, however their case does not include cytoreductive surgery (16). The research (4) examined a cohort of 239 patients diagnosed with European Network for the Study of Adrenal Tumor (ENSAT) stage IV adrenocortical carcinoma from nine medical centers. The participants were stratified into two groups based on whether they received cytoreductive surgery (n=128) or did not undergo such intervention (n=111). After an average follow-up period of 67 months, it was observed that patients who did not receive cytoreduction exhibited a significantly higher mortality risk compared to those who did, with a hazard ratio calculated at 3.18 and a 95% confidence interval ranging from 2.34 to 4.32. The median overall survival time for patients who underwent cytoreduction was found to be 25 months, contrasted with 9 months for those who did not receive the procedure (5).

Explanations of findings

The described case of secondary cytoreduction in case of recurrent adenocarcinoma with invasion into the liver and preservation of the kidney is quite rare. Reducing the size of the tumor reduces its impact on the body—intoxication, load on the immune system. In addition, the findings revealed during the surgical intervention made it possible to clarify the patient management plan in the postoperative period and choose the optimal chemotherapy regimen.

Implications and actions needed

Additional observations are needed in the cohort of patients with relapsed adrenocortical cancer. The implication of cytoreductive surgery has the capacity to offer patients a disease-free interval, alleviate the burden of disease, and diminish the likelihood of the emergence of resistant mutations, thereby facilitating sustained disease control.

Conclusions

Adrenocortical cancer is a rare malignancy with a poor prognosis and limited treatment options for disease progression. Cytoreductive surgery is an important option for complex treatment in the advanced stages of adrenocortical cancer.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-198/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-198/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-198/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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