Non-functioning Adrenocortical Carcinoma Research Articles

Influence of sex and functional status on the value of serum steroid profiling in discriminating adrenocortical carcinoma from adrenocortical adenoma.

It is challenging for clinicians to distinguish adrenocortical carcinoma (ACC) from benign adrenocortical adenomas (ACA) in their early stages. This study explored the value of serum steroid profiling as a complementary biomarker for malignancy diagnosis of ACC other than diameter and explored the influence of sex and functional status. In this retrospective study, a matched cohort of patients diagnosed with either ACC or ACA based on histopathology was meticulously paired in a 1:1 ratio according to sex, age, and functional status. Eight serum steroids including 11-deoxycortisol, 11-deoxycorticosterone, progesterone, androstenedione, dehydroepiandrosterone (DHEA), dehydroepiandrosterone sulfate (DHEAS), 17-hydroxyprogesterone, and estradiol, were quantified by liquid chromatography tandem mass spectrometry. We conducted a comparative analysis of the clinical characteristics and serum steroid profiles of patients with ACC and ACA, with further subgroup analysis. The study included 31 patients with ACC and 31 matched patients with ACA. Patients with ACC exhibited significantly larger tumor diameters, lower body mass index (BMI), and higher levels of 11-deoxycortisol, progesterone, and androstenedione than those with ACA. 11-deoxycortisol was the only valuable index for discriminating ACC from ACA, regardless of functional status and sex. Progesterone, DHEA, and DHEAS levels were higher in the functional ACC group than in the non-functional ACC group. Female ACC patients, especially in postmenopausal female exhibited higher levels of androstenedione than male patients. The area under the curve of tumor diameter, 11-deoxycortisol, and BMI was 0.947 (95% CI 0.889-1.000), with a sensitivity of 96.8% and specificity of 90.3%. Serum steroid profiling serves as a helpful discriminative marker for ACC and ACA, with 11-deoxycortisol being the most valuable marker. For other steroid hormones, consideration of sex differences and functional status is crucial.

FRI222 Large Non-functional Adrenocortical Carcinoma In A Patient With History Of Occupational Radiation Exposure

Abstract Disclosure: A. Makawi: None. G. Elshimy: None. N. Martinez de Andino: None. A. Bolduc: None. Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.1-2.0 cases/per million per year. Up to 70% of tumors present with biochemically apparent adrenocortical hormone production. Diagnosis requires investigation of clinical, biological, and imaging features with measurement of steroid hormones key for evaluation. Anderson’s syndrome is commonly seen. Research has shown ACC is driven by several molecular changes that include the inactivation of tumor suppressor genes and activation of different oncogenes, DNA mutations, and epigenetic changes. The widely used staging of ACC is by the European Network of Study of Adrenal Tumors (ENSAT) as it is correlated with clinical outcomes. Here we present a case of a non-secretory ACC noted for 10 years with growth up to 7.5 cm in a patient with history of occupational radiation exposure. Case Presentation: Patient is a 57-year-old white male with history of colon polyps, basal and squamous cell carcinoma, and hypertension diagnosed 10 years prior, on amlodipine 5 mg and lisinopril 20 mg daily who presented to adrenal clinic for evaluation of a 7cm left adrenal mass that had grown from 1.8cm 10 years prior, to 5.7cm 7 years later to 7.5cm at time of surgery. He had symptoms of fatigue and occasional lightheadedness with no other signs or symptoms of Cushing's or pheochromocytoma. Family history noncontributory. He has a history of occupational exposure to radiation working at a nuclear reactor. Workup for adrenal incidentaloma included assays of serum normetanephrine which was increased at 1.2 nmol/L (reference <0.9 nmol/L) with normal metanephrine less than 0.20 nmol/L, normal DHEA-S of 52 mcg/dL, normal ACTH of 16 pg/mL and negative 1mg dexamethasone suppression test with serum cortisol suppressed to 0.78 mcg/dL. CT scan adrenal protocol showed a heterogeneous 7.7x6.6 cm left adrenal mass with 30 HU, with an absolute adrenal washout of 39% and relative washout of 31.4%. He then underwent left adrenalectomy on 12/16/2022. The tumor measured 7.5cm with pathology of low-grade ACC with staging of pT3. Margins were negative for carcinoma but microscopic angioinvasion and capsular invasion were noted. Postoperatively, the patient had resolution of his symptoms with marked improvement in BP. We continue to follow him in clinic for postoperative management. He has been referred for genetic testing with results pending. Discussion: The prognosis of ACC has been believed to be poor due to the low prevalence and sample size of ACC patients, but recent studies have shown that ACC presentation, survival, and prognosis are heterogeneous. Only 20-30% of ACC are nonfunctional. It is important to note that only 30% of ACC cases are curable at early stages, further impacting the poor prognosis. The goal of this report is to bring further attention to the timely assessment and personalized management of ACC given the evolving knowledge base. Presentation: Friday, June 16, 2023

GIANT NON-FUNCTIONING ADRENOCORTICAL CARCINOMA

Objective: This study aims to report a case of a 51 year old male who was referred to our Department of Urology and was diagnosed with a nonfunctioning giant ACC. Case(s) Presentation: The case report describes a 51-year-old Indonesian man who presented to our department with complaints of persistent upper left abdomen discomfort, previously misdiagnosed as dyspepsia. He was diagnosed as having non-functioning adrenocortical carcinoma and underwent open adrenalectomy. Discussion: Although the patient is 51 years old and the tumor has grown to a signify cant size, no lymph node involvement or metastases was found and resection margins were found to be negative. In our patient, functional adrenal work-up results were normal. This has led us to suspect a non-functioning adrenal tumor. Metastatic work-up with CT cranial, chest, and bone survey were performed, with negative result. The biopsy result after resection confirmed the diagnosis of ACC. Conclusion: ACC is a rare aggressive tumor, malignant with poor prognosis. In our case, the non-secretory mass was diagnosed late by manifestation of mass effect symptoms in the upper left quadrant of the abdomen and was previously misdiagnosed as symptoms of dyspepsia. The functional work-up of adrenal masses helped in the determination of its non-functional status. Early diagnosis of ACC and early surgical excision helps in improving the overall survival rate of the patient.
 Keywords: Non-functioning, adrenocortical carcinoma (ACC), adrenalectomy, case report.

Challenges in diagnosis and management of a giant non-functioning adrenocortical carcinoma: A case report

Adrenocortical carcinoma (ACC) despite being a rare endocrine malignancy arising from adrenal cortex carries a poor prognosis. It is also challenging to diagnose these tumours preoperatively especially if it is a non-functioning tumour. As the rarity of the disease pose a great challenge in taking up statistically significant studies that would be needed to improve the management strategies and outcomes of ACC, all the recommendations are based on retrospective case series or expert opinions. Hence Multidisciplinary approach is warranted to improve the outcomes in the management of ACC. Despite the enormous progress achieved in the biological knowledge of this tumor, the ACC remains an oncological disease burdened by a high mortality. Surgery is still the first therapeutic option and the only potentially curative treatment.

ODP024 Adrenocortical Carcinoma Identified in a Patient With Familial Adenomatous Polyposis Syndrome

Abstract Background Numerous extracolonic manifestations are associated with familial adenomatous polyposis (FAP), including benign thyroid and adrenal nodules, less commonly thyroid and adrenal cancers. We describe a case of a patient with history of FAP who was found to have malignant lesions both in the thyroid and adrenal glands. Clinical Case A 54 year-old female with familial adenomatous polyposis (FAP) underwent partial colectomy at age 16. Known extracolonic manifestation at initial presentation was limited to a dermoid tumor of the right breast. Upon evaluation by endocrinology, she was found to have a dominant thyroid nodule in the right lobe that eventually doubled in size and was positive for papillary thyroid cancer with a BRAF V600E mutation. Successful total thyroidectomy with central neck lymph node dissection was completed, and she is now undergoing routine surveillance for ATA low risk PTC. She maintained annual endoscopic surveillance with gastroenterology and underwent serial abdominal imaging studies for complaints of abdominal discomfort. Right adrenal adenoma was first identified in August 2017 and screening for functionality, to include pheochromocytoma, was negative. Repeat abdominal imaging in 2020 showed the lesion increased in size by 30%. CT imaging identified the nodule as indeterminate based on the washout characteristics (HU 27 pre-contrast, absolute washout = 13% and relative washout = 8%). A 2cm left adrenal mass was also noted with radiographic features consistent with a lipid poor, benign, adenoma (HU 33 pre-contrast, absolute washout = 58%, relative washout = 39%). PET-CT was performed to further characterize the right sided lesion, which was vividly FDG avid at 8.7 SUV (high probability of malignancy). The left adrenal nodule was mildly FDG avid with an SUV of 2.4. CT-guided biopsy of the right adrenal gland was performed and immunohistochemistry did not stain for chromogranin, inhibin, pankeratin or calretinin. Positive staining for MART-1 was seen, which is a typically associated with adrenal cortical cells. There was a high index of suspicion for malignancy given her hereditary cancer syndrome, and the decision was made to proceed with right adrenalectomy. Final surgical pathology confirmed a low grade adrenocortical carcinoma with two nodular lesions, both staining CD10 positive with the mitotic marker (Ki-67) 2-3% positive. The patient's left adrenal gland remains functionally intact, and there were no indications to pursue postoperative treatment or adjuvant therapy. Conclusion The patient presented here is representative of incidentally discovered nonfunctional adrenocortical carcinoma in the setting of a hereditary cancer syndrome. The available literature suggests that adrenocortical tumors should be considered FAP tumors, and sporadic mutations leading to adrenocortical carcinoma are less likely related to germline APC gene mutations Presentation: No date and time listed

Giant non-functioning adrenocortical carcinoma - the elephant in the room

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Giant non-functioning adrenocortical carcinoma: an unusual tumour presentation

Adrenocortical carcinoma is a rare tumour with incidence of 1 per million all over the world. Age distribution is bimodal with peaks occurring at 5-20 years and 40-50 years. Tumours greater than 9 cm commonly involve inferior vena cava (IVC) or right side of heart. 80 percent of the carcinomas are functional. We describe a case report of a rare tumour of a non-functioning adrenocortical carcinoma in a 40 year old female having incidence of 0.5/million world over with no extra-adrenal spread at presentation without IVC or right heart involvement in spite of having a large size of 12 cm. Patient underwent successful surgical extirpation with adjuvant radiotherapy with regular follow ups and disease free survival since the surgery. It is imperative to determine hormone levels in symptomatic and asymptomatic patients with adrenal masses. The possibility of adrenocortical carcinoma should not be ruled out in a functioning/non-functioning tumour. All solid incidentalomas on computed tomography (CT)/magnetic resonance imaging (MRI) greater than 5 cm in size should be removed surgically with adjuvant therapy consideration after histopathological reporting.

Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report

BackgroundAdrenocortical carcinoma is a rare malignancy, with 43% being non-functional. These may arise from adrenal rest anywhere in the embryonic pathway of the adrenal glands. In the context of extra-adrenal and retroperitoneal tumors, the exact pathologic diagnosis is challenging. The case reported here, to the best of our knowledge, is the seventh reported case of extra-adrenal non-functional adrenocortical carcinoma.Case presentationWe report a case of extra-adrenal non-functional adrenocortical carcinoma in a 15-year-old Persian boy who presented with an acute abdomen. He underwent surgical resection. Pathologic findings based on immunohistochemistry and cellular morphology confirmed adrenocortical carcinoma. He was treated with mitotane for 24 months. During a follow-up period of 30 months, no recurrence or metastases were found.ConclusionDespite the rarity of extra-adrenal adrenocortical carcinoma, presentation with an acute abdomen may occur, and the tumor may be found anywhere in the adrenal embryonic pathway. On the other hand, tumor behavior and prognosis in children may be different from what we expect in adults.

Comparison between functional and non-functional adrenocortical carcinoma

BackgroundWhile roughly half of adrenocortical carcinomas are functional, whether functional status impacts outcomes remains controversial. We compared presentation and survival for functional and nonfunctional neoplasms. MethodsAdult patients presented with adrenocortical carcinomas at the Mayo Clinic were included. Tumor characteristics and outcomes were analyzed. ResultsThe 266 identified patients presented with stage I (6%), II (33%), III (26%), and IV disease (32%); stage was unknown in 3%. Fifty-three percent of tumors were functional; patients with functional adrenocortical carcinomas were younger, more likely to be female, and more likely to present with metastatic disease. Surgical resection was undertaken in 84% of patients with 69% having R0 resection. While 30-day morbidity was similar between functional and nonfunctional adrenocortical carcinomas, median overall survival was better for nonfunctional adrenocortical carcinomas (median 66 vs 22 months, P = .01). Functional adrenocortical carcinomas was independently associated with shorter survival after adjusting for age, sex, grade, stage, and resection attempt: hazard ratio = 1.5 (95% confidence interval, 1.04–2.14, P = .03). ConclusionIn our cohort, long term survival was worse for all patients with functional tumors. However, when analyzing patients with R0 resection, there was no survival difference between functional and nonfunctional adrenocortical carcinomas, signaling need for better understanding of adrenocortical carcinomas behavior to individualize and optimize treatment strategies.

MON-344 A Large Adrenal Mass with Neuroendocrine Features Presenting as Pulmonary Embolism: A Case Report

Background: Adrenocortical Carcinoma (ACC) is a rare disease that has a bimodal distribution. 60% of ACC presents with symptoms and signs of adrenal steroid hormone excess [1] while non-functional ACCs present less conspicuously with abdominal mass or other nonspecific symptoms making diagnosis challenging. The usual sites of metastasis are the lung (71%), lymph node (68%), liver (42%), and bone (26%) [1]. According to literature, there has been few reported cases of ACC extending to the inferior vena cava and right atrium presenting with pulmonary embolism. Clinical Case: Patient is a 38 year old male admitted due to dyspnea, BP elevation and weight loss. Initial CXR was done showing canon ball lesions. On work up, Chest CT scan revealed pulmonary metastasis and pulmonary embolism. Abdominal CT scan showed a large right suprarenal mass with tumor infiltrating the inferior vena cava, hepatic and portal veins. 2 d echo revealed a large pedunculated mass attached to the free wall of the right atrium. The patient was started on anticoagulation. Diagnostic work up for adrenal function was done. Plasma renin and aldosterone in supine position were 3.30ng/mL/hr (reference range 0.3-1.9), and 23.28 ng/dL (reference range: 3.78-23.3) respectively with an ARR 7. 8 am cortisol was 12.30 ug/dL (reference range: 3.7-19.4ug/dL), 24 hour urine metanephrine 0.43 (reference range: 0-1) and plasma free metanephrine 36.75 pg/mL (reference range <65pg/mL) were all normal. Chromogranin A was normal at 90.71 ng/mL (reference range: <100ng/mL) however, DHEAS was 3x elevated at 1,406.00 ug/dL (Reference Range: 139.70–484.40 ug/dL). The patient underwent FNAB of the lung nodules revealing a malignant neoplasm with neuroendocrine features. Since the etiology of the adrenal mass was still uncertain, a functional imaging with 68Ga-DOTATATE PET-CT scan was done. However, the large right suprarenal mass was mostly necrotic with a small 68Ga-DOTATATE-avid focus. During admission, patient had sudden onset of chest pain, diaphoresis, hypotension and desaturation. He deteriorated and eventually expired. Post mortem examination revealed adrenocortical carcinoma with neuroendocrine features; hemorrhagic infarction secondary to massive pulmonary tumor embolism. Conclusion: Adrenocortical carcinoma is a rare cancer with varied initial presentation. A rare presentation is pulmonary embolism. In advanced stage diseases, with distant metastases, ACC has a poor prognosis. Multimodality approach with radiotherapy, aggressive surgical resection and mitotane can be offered as treatment options. Reference: 1. Kim, KH, et al. "A case of non-functioning huge adrenocortical carcinoma extending into inferior vena cava and right atrium."

Giant nonfunctioning adrenal tumors: two case reports and review of the literature

BackgroundThere are an estimated 1–2 cases per million per year of adrenocortical carcinoma in the USA. It represents a rare and aggressive malignancy; it is the second most aggressive endocrine malignant disease after anaplastic thyroid carcinoma. Non-secretory adrenal masses are diagnosed late due to a mass effect or metastatic disease or found incidentally (adrenal incidentalomas).Case presentationThe first case report describes a 39-year-old Greek woman who presented to our department with complaints of repeated symptoms of flatulence and epigastric discomfort over a few months. The second case report is about a 67-year-old Greek woman who presented to our department after being evaluated for fatigue, mass effect, and epigastric discomfort. Both of them were diagnosed as having a nonfunctioning adrenocortical carcinoma and underwent open adrenalectomy.ConclusionsApproximately 60% of patients with adrenocortical carcinoma present with symptoms and signs of hormonal secretion. Our cases’ adrenocortical carcinomas were not functional. Hormone secretion is not a discriminating feature between benign and malignant adrenocortical masses. The silent clinical nature of nonfunctioning adrenocortical carcinoma results in late diagnosis, while the majority of patients present with locally advanced and/or metastatic disease.Adrenocortical carcinoma is a rare endocrine tumor with a poor prognosis that can be diagnostically challenging and demands high clinical suspicion. The work-up for adrenal masses must include determination of whether the mass is functioning or nonfunctioning and whether it is benign or malignant.

Nationwide analysis of adrenocortical carcinoma reveals higher perioperative morbidity in functional tumors

BackgroundCurrent adrenalectomy outcomes for functional adrenocortical carcinoma (ACC) remain unclear. This study examines nationwide in-hospital post-adrenalectomy outcomes for ACC. MethodsA retrospective analysis of the Nationwide Inpatient Sample database (2006–2011) to identify unilateral adrenalectomy patients for functional or nonfunctional ACC was performed. Patient demographics, comorbidities and postoperative outcomes were evaluated by t-test, Chi-square and multivariate regression. ResultsOf 2199 patients who underwent adrenalectomy, 87% had nonfunctional and 13% had functional ACC (86% hypercortisolism, 16% hyperaldosteronism, 4% hyperandrogenism). Functional ACC patients had significantly more comorbidities, and experienced certain postoperative complications more frequently including wound issues, adrenocortical insufficiency and acute kidney injury with longer hospital stay compared to nonfunctional ACC (P < 0.01). On multivariate analysis, functional ACC was an independent prognosticator for wound complications (28.1, 95%CI 4.59–176.6). ConclusionPatients with functional ACC manifest significant comorbidities with certain in-hospital complications. Such high-risk patients require appropriate preoperative medical optimization prior to adrenalectomy.

LONG-TERM RESULTS OF SURGICAL TREATMENT FOR ADRENOCORTICAL CANCER

Background: Adrenocortical cancer (ACC) is a rare adrenal neoplasm with aggressive clinical course and poor prognosis, and with many unresolved problems of diagnostics, treatment and predictive factors. Aim: To identify the most significant clinical and morphological predictors of the outcome based on assessment of long-term results of surgical treatment of ACC patients. Materials and methods: This retrospective study included data on diagnosis and results of treatment of 73 ACC patients from 1999 to 2015. Results: Radical surgery was performed in 59 patients, and long-term results assessed in 52 of them (maximal duration of the follow-up was 12 years). The most favorable treatment results were seen in stages I and II of the disease, with a 5-year survival rate of 87%. The 5-year overall survival in patients with ACC stage III was significantly (p=0.042, multiple comparisons) lower (48%). Two patients who had been operated with ACC stage IV (adrenalectomy with excision of a solid liver metastasis and atypical lung resection) died of progressive tumor within 13 months after surgery. The survival rates in patients with stage III of the tumor were evaluated depending on the main clinical characteristics of the tumor. It was shown that the 5-year overall survival in non-functioning adrenocortical carcinomas was 59%, being significantly (p&lt;0.05) better than that in patients with functionally active malignancies (22%). In the group of patients without metastases in the regional lymphatic nodes, the 5-year overall survival was 56%, whereas all patients with regional lymphatic metastases by the time of the surgery died from progression within 4 years after the intervention. There was an association between 5-year overall survival and the size of primary tumor, with significantly (p&lt;0.05) lower numbers of 5-year overall survivors (29%) among those with tumors of more than 10 cm in diameter, compared with 65% survival rate in those with tumors of less than 10 cm. Conclusion: The results obtained confirm that the stage of ACC by the time of diagnosis is the major prognostic factor. Functional activity of the tumor, its size and regional lymphatic node metastases also have some prognostic value. At present, surgical intervention remains the single radical treatment approach to ACC; however, its results are far from being satisfactory. Patients with ACC stage III and IV require an individual approach to assessment of prognosis and to the choice of the treatment strategy after potentially curative surgery, which is possible based on immunohistochemical molecular biologic tests of the tumor.

A Case of Recurrent Adrenocortical Carcinoma with Stomach Metastasis

Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. We experienced a case of recurrent ACC with stomach metastasis which had been completely cured a long while ago. A 52-year-old man who presented with right thigh pain was hospitalized. We found a lumbar spine mass on magnetic resonance imaging scan. And this lesion was identified as metastatic adrenocortical carcinoma. The patient had been treated as stage II non-functioning ACC 19 years ago. At that time radical resection and adjuvant chemotherapy were successfully done. And the follow-up evaluation was discontinued, since it had been checked as no evidence of disease (NED) state for 5 years. But this time, there were multiple metastatic sites revealed in positron emission tomography– computed tomography scan including stomach. Therefore, we report a case of ACC herewith that it could be recurred even though long-term NED state was passed after treatment and stomach could be a metastatic site of ACC.

Non-Functioning Adrenocortical Carcinoma (incidentaloma) - A Case Report and Review of Literature

Adrenocortical carcinoma is a rare endocrine tumour with poor prognosis. Herein we report a case of adrenocortical carcinoma which was non-functioning, and non-metastasizing. A female patient presented with vague abdominal pain and palpable lump on examination. A large sized adrenal mass on Ultrasonography (USG) and computed tomography was revealed. USG guided fine needle aspiration cytology was performed, the smears were highly suspicious of malignancy. Tumour was surgically excised and histopathology confirmed the final diagnosis of stage II adrenocortical carcinoma. No metastasis was seen. Rarity of non-functioning adrenocortical carcinoma prompted us to report this case. Volume 2 | Issue 5

A giant non-functional adrenocortical carcinoma presenting with acute kidney injury

Hormonally inactive adrenocortical carcinoma (ACC) is a rare disease where abdominal discomfort and back pain are common presenting symptoms due to mass effect from a large tumor. Acute kidney injury (AKI) from retroperitoneal tumors has rarely been reported. The most common etiologies include venous thrombosis, ureteral compression, or both. Here, we described a man who presented with AKI from a large retroperitoneal tumor, which was finally diagnosed as a non-functional ACC. The inferior vena cava (IVC) was nearly completely compressed by the large retroperitoneal tumor leading to venous outflow obstruction and AKI. After surgical resection, his urine output increased and renal function recovered. Unfortunately, AKI recurred 2 months later due to recurrence of the tumor. Treatment with a tyrosine kinase inhibitor stabilized his tumor size, and hemodialysis was started. IVC-compression-associated AKI can be the presenting scenario for ACC, a rare but prognostically important aggressive neoplasm.

A rare coexistence of non-functional adrenocortical carcinoma and multicentric papillary thyroid microcarcinoma: a case report

IntroductionIn this report, we describe a rare case of papillary thyroid carcinoma with adrenocortical carcinoma without excess hormone production.Case presentationA 40-year-old Turkish man was admitted to our institution with a large left adrenal mass that was identified during the work-up for shortness of breath. The patient did not have specific signs and symptoms of hormone excess. The mass was removed surgically. The pathological findings were consistent with adrenocortical carcinoma. The patient was also found to have a multicentric papillary thyroid microcarcinoma.ConclusionMost adrenocortical carcinomas and papillary thyroid carcinomas are sporadic; however, the occurrence of two different endocrine neoplasms during the same period of time is a rare situation, but it is possible, as in our patient. When an endocrine tumor is diagnosed, endocrinologists must be consider the possibility of the existence of another endocrine tumor.

An unusual presentation of congenital adrenocortical carcinoma: a case report and review of the literature

We describe a case of congenital non-functional adrenocortical carcinoma in a male infant who presented with recurrent pneumonia, paraparesis and sclerotic skeletal metastasis. To the best of our knowledge such presentation has never been reported.

Adrenocortical carcinoma presenting as varicocele and renal vein thrombosis: a case report

IntroductionAdrenocortical carcinomas are rare aggressive tumors. Their annual incidence is approximately one to two per million among the population of the United States of America. Patients with active endocrine tumors often present with Cushing's syndrome accompanied by virilizing features. Conversely, patients with non-functioning tumors may present with symptoms related to a mass-occupying lesion, such as abdominal pain and flank pain. Although varicoceles and acute kidney injuries are common problems in medicine, they are uncommon presentations of these rare tumors and easy to miss. We report a case of a large adrenocortical carcinoma that presented as testicular pain, varicocele, and acute kidney injury secondary to renal vein thrombosis.Case presentationA 54-year-old Caucasian man with a left-sided varicocele presented to our emergency department with lower abdominal pain and a decrease in urination. Four months previously, he had noticed pain and swelling in his left groin and had been diagnosed with left-sided varicocele. For one week, he began developing left-sided abdominal pain and decreased urination frequency, so he came to our emergency department for evaluation. His physical examination revealed a hard mass occupying the entire left side of his abdomen, crossing the midline, and extending to the pelvic brim. His blood tests showed acute kidney injury and mild anemia. Computed tomography of his abdomen showed a large retroperitoneal mass on the left side, displacing the left kidney inferiorly and the spleen superiorly with thoracic epidural compression. Thrombus was also identified in his left renal vein and inferior vena cava. Computed tomography of his chest showed bilateral pulmonary nodules. A computed tomography-guided abdominal mass biopsy was performed, and the diagnosis of adrenocortical carcinoma was made on the basis of pathology and immunohistochemistry. His hormonal evaluations were normal. His kidney function improved with intravenous hydration and anti-coagulation treatment. Unfortunately, the adrenal mass was unresectable because of the extent of the tumor. Treatment with mitotane, an adrenocorticolytic drug, was started with concomitant with irradiation of a lesion at T5, followed by combination chemotherapy thereafter.ConclusionUnilateral right-sided varicoceles are rare and should alert the clinician to possible underlying pathology causing inferior vena caval obstruction. Left-sided varicoceles, in contrast, are common secondary to the venous anatomy of the left testis; however, the enlargement of the left testicle can be associated with blockage of the left testicular vein by tumor invasion of the left renal vein. Varicoceles could be an early presentation of a non-functioning adrenocortical carcinoma. Acute kidney injury can occur as a result of mass effect or thrombosis of renal vessels. Large tumors can cause abdominal pain as a late manifestation. Physicians should perform a complete abdominal examination in every patient with varicocele or testicular pain.

Spontaneous Rupture of a Functioning Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis, and it can be classified as either a functional or nonfunctional tumor. Affected patients usually present with abdominal pain or with symptoms related to the mass effect or hormonal activity of the tumor. Several cases of spontaneously ruptured nonfunctional adrenocortical carcinoma have been reported, but no case of a spontaneous rupture of functioning adrenocortical carcinoma has been described. We report a functioning adrenocortical carcinoma that spontaneously ruptured during a work-up.