Adrenocortical Carcinoma Presenting Research Articles

A Rare Case of Adrenocortical Carcinoma Presenting as Cushing’s Syndrome

A Rare Case of Adrenocortical Carcinoma Presenting as Cushing’s Syndrome

Primary infertility: a rare presentation of adrenocortical carcinoma

Primary infertility: a rare presentation of adrenocortical carcinoma

S2932 Adrenocortical Carcinoma Presenting as Abdominal Pain

S2932 Adrenocortical Carcinoma Presenting as Abdominal Pain

Adrenocortical Carcinoma Presenting with flank pain: A Case Report

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

Adrenocortical Carcinoma presenting as florid Cushing's Syndrome

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

ODP021 A Feminizing Adrenocortical Carcinoma Presenting with Marie-Antoinette Syndrome

Abstract Introduction Adrenocortical carcinomas (ACCs) are rare and aggressive malignant tumors often with an unfavorable prognosis accounting for 0. 05-0.2% of all cancers. ACCs can be separated into 2 groups: Functioning (hormone-producing) and non-functioning tumors. About 60% of ACCs synthesize hormones. Hormone-secreting ACCs most commonly cause virilization or Cushing's syndrome. On very rare occasions, ACCs can lead to feminization in male subjects. Clinical manifestations are gynecomastia and/or hypogonadism. Here, we present a case of an estradiol-secreting adrenal cortical carcinoma. Case presentation A 58-year-old male presented with patchy hair loss, change in hair colour to white, loss of body hair and eyebrows, and breast tenderness. Hormone testing revealed significantly elevated estradiol came 582 pmol/L [NR<190] with a very low testosterone of 1.6 nmol/L [NR10-30], low FSH 0.1 mIU/mL [NR 0.95-11.5] and LH of 0.6 mIU/mL [NR1.14-8.75]. A testicular ultrasound was unremarkable. An adrenal MRI revealed an 11 cm heterogeneous right adrenal mass invading the inferior vena cava. FDG PET-CT revealed an intensely metabolically active tumor without any evidence of metastatic disease. The tumor was successfully removed via an open procedure. Histology confirmed an adrenocortical carcinoma with a Weiss score of 8 with a mitotic account of 10/50 HPF and Ki-67 labelling index of 20-25%. There was vascular invasion with large areas of necrosis, ENSAT (European Network for the Study of Adrenal Tumours) stage II. Postoperatively, he made a good clinical recovery and was offered adjuvant Mitotane and EDP (Doxorubicin, Etoposide and Cisplatin) chemotherapy. Due to a significant rise in GGT and platelets, Mitotane had to be stopped temporarily. During the course of his treatment, he developed a hoarse voice and CT neck revealed abnormal thickening of the lateral aspects of the aortic arch suggesting possible tumor recurrence or aortitis. A biopsy was not diagnostic, but did not show any features of malignancy. He completed his five cycles of EDP chemotherapy. Estradiol has remained normal and his hoarse voice improved. Conclusion We describe a rare case of an estradiol-secreting ACC presenting with feminization and Marie-Antoinette syndrome as well as possible paraneoplastic aortitis. Targeted molecular tumor analysis may guide further treatment options and enrolment in clinical trials should be considered. Presentation: No date and time listed

ODP050 Oncocytic Adrenocortical Carcinoma Presenting with Hematuria

Abstract Introduction Adrenocortical carcinoma (ACC) is rare and aggressive endocrine tumor. It is commonly noticed due to symptoms of hormone excess, mass-effect, or incidentalomas. Different histological variants are noted, which include: oncocytic, myxoid, sarcomatoid, and the conventional subtype. The oncocytic variant is usually seen on the left side, and usually presents with symptoms of mass-effect. Only around 17% were noted to be functional. We present an interesting case of ACC (oncocytic type) which presented with hematuria. Case Presentation 70-year-old female with a history of morbid obesity, hypertension, anxiety, GERD, tubular adenoma, hyperlipidemia, and lumbar radiculopathy came in to see her primary care for hematuria and back pain. She has a long-standing history of back pain, for which she has had an EMG, which showed mild axonal polyneuropathy and bilateral L5 radiculopathy. Apart from hematuria, she denies any other urinary complaints. Additionally, she denied any fever, chills, nausea, abdominal pain, sweating, tremor, headache, palpitations, easy bruising, fractures, muscle weakness, hirsutism, or changes in her weight. She has a family history of pancreatic cancer and is currently retired, and denies alcohol, tobacco, or recreational drug use. A KUB X-ray was ordered which showed calcification in the left upper quadrant, concerning for a splenic artery aneurysm; this led to a CTA abdomen, which showed a large 7.2 cm heterogenous enhancing solid left adrenal mass, concerning for primary adrenal carcinoma, which was new compared to imaging 2 years ago. Also seen was a 0.7 cm enhancing right hepatic lobe lesion, along with a 1.5 cm splenic artery aneurysm. Following this, an abdomen MRI with contrast showed a 7×7×7 cm solid heterogeneously enhancing left upper quadrant mass beside the left adrenal gland and pancreas. Subsequently, a CT guided needle biopsy was done, which revealed scant adrenal tissue. PET scan showed a hypermetabolic mass, arising from the left adrenal, consistent with adrenal carcinoma. At this point, an endocrinology referral was placed, and other lab work including 24-hour urine free cortisol, DHEA-S, 24-hour urine metanephrine, and aldosterone/renin ratio was done. DHEA-S level was elevated at 264 mcg/dl (12-133). She was then transferred to a tertiary care facility, where she underwent a left adrenalectomy, para-aortic lymphadenectomy, nephrectomy, splenectomy, and distal pancreatectomy. Pathology confirmed adrenal cortical carcinoma (oncocytic type), with negative margins, Ki-67 labelling index of 25%, and no lymphovascular invasion, pT2pNO. She was then discharged home with visiting nurses. Conclusion Oncocytic ACC is an infrequent tumor, which presents with symptoms of mass effect, and definitive diagnosis requires histopathological assessment. The Lin–Weiss–Bisceglia (LWB) system and Helsinki score help establish malignant potential. Fortunately, it has a decreased tendency to metastasize and a better prognosis than other ACC subtypes. Further studies to understand oncocytic ACC are needed. Presentation: No date and time listed

Massive adrenocortical carcinoma presenting as peripheral edema: a case report

BackgroundAdrenocortical carcinoma is a rare, but potentially lethal, malignancy that is usually detected as an incidental finding on abdominal imaging studies or owing to hormonal complications. This report recounts an unusual presentation with leg edema due to compression of the inferior vena cava. The dearth of proven effective treatment is also addressed.Case presentationA 65-year-old White male physician presented with severe, bilateral pitting edema that extended from the toes to the thighs. It progressed over several months. He also experienced paroxysmal dyspnea. Evaluation of cardiac, hepatic, and renal function failed to determine a cause. Computed tomography revealed a tumor above the right kidney, with compression of the intrahepatic inferior vena cava and upstream distension. Serum cortisol and dehydroepiandrosterone sulfate concentrations were elevated, 24-hour urinary cortisol level was elevated, and serum adrenocorticotropic hormone and testosterone concentrations were suppressed. A 27-cm tumor, the right lobe of the liver, the right kidney, and 26 lymph nodes were resected. Histological study confirmed the diagnosis of adrenocortical carcinoma. Ki67 proliferation index was 26.7% (worse prognosis associated with index > 10%). Lymph nodes were negative for malignancy, but a separate 2.7-cm tumor was found near the renal hilum. Adjuvant mitotane chemotherapy was prescribed. Serum testosterone concentration returned to normal. High-dose hydrocortisone administration was needed because of adrenal suppression and CYP 3A4 induction by mitotane.ConclusionImaging of the abdomen and pelvis should be conducted in cases of unexplained leg edema. In this case, a large adrenal cancer compressed the vena cava. Iron deficiency followed resection of the large tumor. Advanced stages of adrenocortical carcinoma are associated with poor prognosis. Mitotane chemotherapy is a standard but unproven adjuvant treatment that is associated with many complications, and its induction of hepatic CYP 3A4 enzymes necessitates adjustment of other medications.

Metastatic Adrenocortical Carcinoma Presenting as Intracavitary Mass in Right Atrium: A Case Report

1.1. Background: Adrenocortical carcinomas grow rapidly and tend to metastasize to liver and lungs and invade kidney, renal vein and Inferior vena cava (IVC). Cardiac metastases of the tumor is rare, less than 20 cases have been described and most of them occurred via direct extension through inferior vena cava into right atrium [1]. Endocardial Metastases from adrenocortical carcinoma presenting as an intracavitary growth in right atrium of heart without associated IVC involvement is very rare. 1.2. Case Presentation: We describe a case of 20 years old young male who presented to emergency with gradual worsening of dyspnea and facial swelling for last 6 months. Echocardiography showed a large Right atrial mass filling the whole chamber, attached to IAS (interatrial septum) via thin stalk suggestive of right atrial myxoma. Patient underwent resection of myxoma and histopathology proved it to be metastatic adrenocortical neoplasm. Whole body contrast enhanced CT scan showed primary large soft tissue mass lesion in right suprarenal location with metastatic bilateral lung metastases. On biochemical investigation associated hypercortisolemia was found. 1.3. Conclusion: Usually renal cell carcinomas spread intraluminally via inferior vena cava into the right atrium (reported in 1 % of tumors). Intracavitary presentation of metastatic adrenocortical carcinoma is very rare, and index case report was a similar presentation of a young male that underwent resection of intracavitary growth of right atrium (suspected as Myxoma), that came out to be metastatic adrenocortical carcinoma. In patients with advanced local or metastatic adrenocortical carcinomas, not amenable to surgical resection, cytotoxic chemotherapy may be attempted.

Disease-free remission of metastatic adrenocortical carcinoma following en bloc tumor resection and neoadjuvant chemotherapy for suspected adrenohepatic infiltration

A 3 year old boy with a family history of Li-fraumeni Syndrome presented with a right-sided adrenocortical carcinoma, hepatic, and pulmonary metastases. He underwent eight cycles of chemotherapy with Cisplatin, Etoposide, Doxorubicin and Mitotane. Follow-up CT Thorax/Abdomen/Pelvis with contrast demonstrated a reduction in size of the right suprarenal mass, decreased attenuation of liver metastases and one remaining pulmonary nodule. Discussion at multidisciplinary meeting ensued and open adrenalectomy and resection of hepatic metastases was recommended. Intra-operatively, gross appearance of adrenohepatic infiltration into the right hepatic lobe was identified and the decision was made to perform complete, en bloc resection of mass. The remaining pulmonary nodule resolved radiologically. The patient remains well and continues on maintenance adrenolytic therapy with mitotane. Thirteen months post-operatively, no locoregional disease recurrence has been identified on imaging. Disease-free remission is a rare occurrence in metastatic adrenocortical carcinoma. To the author's knowledge this is the first case report demonstrating the detailed surgical management of a child with an initial presentation of metastatic adrenocortical carcinoma.

Recurrence-Free Survival Following Aggressive Multimodal Treatment of an Isolated Recurrence of Adrenocortical Carcinoma

A 43-year old woman demonstrated an intra-abdominal drop metastasis 6 years after initial presentation of stage-III adrenocortical carcinoma (ACC), previously treated with resection, adjuvant radiation and mitotane therapy. This recurrence was managed aggressively with complete excision, adjuvant radiation and mitotane. Imaging at 3.25-year follow-up showed no evidence of recurrence, a remarkable response given ACC’s poor prognosis and high recurrence rates. Management of oligometastatic ACC remains unclear and intra-abdominal drop metastases are particularly rare in ACC; both require further documentation. Aggressive management with adjuvant radiation and mitotane therapy may represent an effective and well-tolerated approach for improving local control for recurrent ACC, including drop metastases. Further research is required to codify potential benefits.

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.

SUN-169 A Rare Case of Adrenocortical Carcinoma Presenting as Hyperaldosteronism Combined with Cushing’s Syndrome

Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Most ACC cases are hormonally functional. Commonly produced hormones are cortisol, followed by adrenal androgens. Aldosterone secretion is quite rare (< 2%) (1). Co-secretion of multiple hormones is further rare. Case Presentation: A 59 year old female presented with progressive worsening hypertension. Initial biochemical evaluation confirmed primary hyperaldosteronism. Her potassium was low at 2.9 mEq/L (N: 3.5-5.3 meq/L). Aldosterone level was elevated at 23 ng/dL (N: < or = 28 ng/mL), renin value was 0.90 ng/mL/h (N: 0.25-5.82 ng/mL/h). Aldosterone Renin Ratio was 25. CT abdomen with and without contrast showed 2 lesions within the right adrenal gland. The larger nodule was 3.7 x 2.7 x 4.9 cm with an absolute washout of 61%. A smaller nodule was 2.3x1.8 cm with an absolute washout of approximately 64%. Left adrenal gland was unremarkable.Further biochemical workup showed plasma catecholamines and metanephrines were normal. A random cortisol value was 22.8 mcg/dL (N: 3.0-16.0 mcg/dL) with a low ACTH level of 3.3 pg/mL (N: 7.2 - 63.3 pg/mL). Given suspicion for adrenal Cushing’s syndrome, we further obtained a 24 hour free urinary cortisol which was elevated at 84.1 mcg/24 h (N: 4-50 mcg/24 h). 8 am Cortisol after an overnight 1 mg Dexamethasone failed to suppress at 17.5 mcg/dL (n<1.8 mcg/dL). DHEAS level was low at 14.1 ug/dL (N: 29.4-220.5 ug/dL). The patient eventually underwent a right adrenalectomy. Post-operatively, her cortisol was suppressed at 1.9 mcg/dL (8 am ref range: 4-22 mcg/dL), and Hydrocortisone replacement dose was initiated. Surgical pathology report was consistent with adrenocortical carcinoma. The patient continues to follow-up with the endocrinology and oncology department for treatment. Conclusion: This case is particularly interesting given the co-secretion of both aldosterone and cortisol by an adrenocortical carcinoma which has been reported in only a few cases in literature. The case highlights the importance of completing a comprehensive biochemical workup pre-operatively in patients with suspicious adrenal mass. There should especially be a low threshold for initiating workup for cortisol hypersecretion as early intervention can help avoid an adrenal crisis in the post-operative period for such patients. A low DHEAS level should raise suspicion for cortisol hypersecretion in a patient with adrenal lesions. As ACTH is the primary stimulant of DHEA, the suppression of ACTH secretion in the setting of adrenal Cushing’s syndrome can contribute to a low DHEAS level. Reference 1.Else, T et al. Adrenocortical Carcinoma. Endocr Rev. 2014; 35(2):282-326.

The diagnosis and treatment of adrenocortical carcinoma in pregnancy: a case report

BackgroundPregnancy complicated with adrenocortical carcinoma (ACC) is a sporadic syndrome that is characterized by hypertension, uncontrolled hypokalemia, severe heart failure, premature delivery and other adverse effects. The clinical presentation of adrenocortical carcinoma is vague and nonspecific, it is challenging to identify complications of pregnancy with adrenocortical carcinoma. Here we present a case of adrenocortical carcinoma during pregnancy. We describe how to distinguish secondary hypertension from other conditions and the importance of timely detection and treatment of such patients.Case presentationA 22-year-old woman 30 weeks pregnant was hospitalized with uncontrolled hypertension and hypokalemia. An ultrasound examination of the right adrenal gland revealed a large mass. She underwent transabdominal adrenalectomy, and histopathology from the sample removed revealed an adrenocortical carcinoma. Five days after surgery, the patient had a premature rupture of the fetal membranes and gave birth to a newborn girl via vaginal delivery at 32 weeks of gestation. The newborn was transferred to the neonatal pediatrics ward, and the woman started receiving chemotherapy.ConclusionsPregnancy with adrenocortical carcinoma is a rare condition. This case alerts the obstetricians that analysis of hypertension, hypokalemia, the plasma level and circadian rhythm of plasma cortisol provides a strategy to diagnose adrenocortical carcinoma during pregnancy.

A Adrenocortical carcinoma (ACC) presenting with episodes of intermittent hypertension: Sarcomatoid type adrenocortical carcinoma

A Adrenocortical carcinoma (ACC) presenting with episodes of intermittent hypertension: Sarcomatoid type adrenocortical carcinoma

MON-323 Metastatic Adrenocortical Carcinoma Presenting with Mixed Cushing's and Virilization Syndrome along with Bilateral Pulmonary Emboli

Background: Adrenocortical carcinoma is a rare, often aggressive tumor that is more common in women than men. The tumors are more often than not biochemically active in adults, and the majority secrete glucocorticoids alone (1,2).Clinical case: A 57yo woman w/ longstanding hypertension and history of total abdominal hysterectomy presented with shortness of breath. She reported worsening of her hypertension over the past six months associated with male pattern balding, body hair growth, anxiety, facial and abdominal swelling for the past four months. Computed tomography PE protocol showed bilateral pulmonary emboli and multiple pulmonary nodules. Subsequent computed tomography chest/abdomen/pelvis revealed pulmonary nodules in both lungs, the largest being 3.7cm x 2.5cm in the right upper lobe, and a large right retroperitoneal mass centered at the expected position of the right adrenal gland measures 12.4cm x 10.5cm causing mass effect on the inferior surface of the liver along with bulky mediastinal, hilar, paratracheal, gastrohepatic, portocaval, and retroperitoneal lymphadenopathy. Exam was significant for cushingoid habitus with central obesity, rounded facies, supraclavicular and dorsocervical fat pads, proximal muscle weakness, frontotemporal balding, severe hirsutism consistent with modified ferriman-gallwey score of 13, and an annular, scaly rash along gluteal cleft. 8am serum cortisol was 65mcg/dL. 24 hour urinary cortisol was 450mcg/24 hour. ACTH was undetectable <2.0pg/mL. Total Testosterone was 835ng/dL. DHEAS was 1,000mcg/dL. Renin, aldosterone, plasma fractionated metanephrines were normal. Excisional supraclavicular lymph node biopsy was positive for metastatic adrenocortical carcinoma. Subsequent FDG PET/CT also showed metastatic disease in the liver, right humeral head, sacrum, and bilateral iliac bones. Punch biopsy of the rash was consistent with tinea cruris. The patient was moved to the oncology service and started on therapy with mitotane, cisplatin, doxorubicin and etoposide.Conclusion: Rapid virilization is concerning for an underlying malignancy and should always include androgen evaluation with a high index of suspicion for ovarian or adrenal tumors.

Abstract #113 Acute Psychosis as an Initial Presentation of Adrenocortical Carcinoma

Abstract #113 Acute Psychosis as an Initial Presentation of Adrenocortical Carcinoma

Large functional adrenocortical carcinoma presenting with hyperandrogenism and hypercortisolism

Large functional adrenocortical carcinoma presenting with hyperandrogenism and hypercortisolism

Late recurrent adrenocortical carcinoma presenting radiologically as a gastrointestinal stromal tumour: A case report

IntroductionAdrenocortical carcinoma (ACC) is a rare malignancy with an estimated incidence of 1–2 per million people. It may recur, after complete surgical removal by local or distant metastasis. ObservationWe report a case of late metastatic ACC presented as a mesenteric mass, 10 years post left adrenalectomy. Our case was initially misdiagnosed radiologically as gastrointestinal stromal tumour (GIST), and then the decision for exploration was made. The mass could be safely excised and confirmed pathologically to be an adrenocortical tumour.

A Rare Case of Virilizing Adrenocorical Carcinoma in a Child Presented with Peripheral Precocious Puberty

Objective: To report a rare case of virilizing adrenocortical carcinoma presenting with peripheral precocious puberty. Methods: we present the initial clinical findings, laboratory tests and medical imaging findings with surgical procedure, tumor staging and histopathological study. Post-operative follows up schedule and management. In addition a brief review literature Results: A 8.5 year old boy presented with pubarche and other virilizing features that dated back to one year. Physical examination revealed; height 121 cm, weight 30 kg, Tanner Stages for pubic hair, penile length and testes volume were IV, IV, ІI respectively. Laboratory findings gonadotropins were low, testosterone high so the precocious puberty was gonadotropins independent ( peripheral) either adrenal or testicular, so abdominal ultrasonography performed that showed well defined mass at right suprarenal region (49×48) mm., and abdominal magnetic resonance demonstrated a mass at right adrenal gland region measures (5×5) cm then dynamic computed tomography of adrenal glands revealed: right adrenal mass (5×4x4) cm well defined. Scrotal ultrasonography revealed both testes slightly enlarged in size with normal echogenicity. Based on the above data the interpretation was adrenal androgen-secreting tumor either adenoma or carcinoma. Therefore, the patient underwent right open adrenalectomy: grossly; encapsulated mass, no invasion to surroundings, size (5×4x4). Histopathology study was consistent with adrenocortical carcinoma confirmed by Immunohistochemistry. Postoperatively: glucocorticoid replacement therapy was initiated, and he was scheduled for follow –up examination. Conclusion: This case illustrates the rare occurrence of adrenocortical carcinoma in this age group and to our knowledge is the first case report in Iraq.