An Unusual Presentation of Adrenocortical Carcinoma (ACC): Panic Attacks and Psychosis.

BACKGROUND Adrenocortical carcinoma (ACC) is a very rare disease, with an incidence of 1.02 per million population per year. The most commonly secreted hormone in ACC is cortisol, often presenting as a rapidly progressive Cushing syndrome (CS). We describe a case of ACC with an unusual presentation, mainly with psychiatric manifestations, including panic attacks and hallucinations. CASE REPORT A 52-year-old woman presented with episodes of acute anxiety, hallucinations, palpitations, hot flashes, gastrointestinal upset associated with paroxysmal hypertension, tachycardia, and flushing for 1 week. The initial workup was aimed at ruling out causes of acute psychosis and/or anxiety such as substance use, and organic diseases such as pheochromocytoma (PCC). Our initial suspicion of PCC was ruled out based on the negative serum and urinary metanephrines (MN) and normetanephrines (NMN). Recurrent metabolic alkalosis and hypokalemia despite fluid and potassium supplementation prompted us to work up for hyperaldosteronism. Her renin level was elevated and the aldosterone level was appropriately suppressed. Elevated cortisol, positive dexamethasone (DXM) suppression test, low adrenocorticotropic hormone (ACTH), imaging revealing an adrenal mass, and postoperative histology confirmed the diagnosis of cortisol-producing ACC. CONCLUSIONS It is essential to recognize psychiatric presentations of CS to achieve early diagnosis and prevent mortality and morbidity. Panic attacks, a common presentation of CS, can present with features mimicking pheochromocytoma (PCC), including palpitations, sweating, tachycardia, and paroxysmal hypertension. A comprehensive workup is warranted to reach a diagnosis, with a combination of hormonal levels, imaging, and histology.

Adrenocortical tumours in children: a review of surgical management at a tertiary care centre.

BACKGROUND: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management. METHODS: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively. RESULTS: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months. CONCLUSIONS: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases.

Patterns of Lymph Node Recurrence in Adrenocortical Carcinoma: Possible Implications for Primary Surgical Treatment.

BACKGROUND: In the surgical treatment of adrenocortical carcinoma (ACC), lymphadenectomy may improve oncologic outcome. However, patterns of metastatic lymphatic spread in ACC are unknown. METHODS: Clinical data of patients included in the European Network for the Study of Adrenal Tumors (ENSAT) registry were retrospectively reviewed. Inclusion criteria were: nonmetastatic ACC, complete resection of the primary tumor, a disease-free time of > 3 months, and lymph node metastases as the first disease relapse. The retroperitoneal lymphatic drainage area was evaluated by using follow-up imaging. RESULTS: Of 971 patients from the ENSAT registry, 56 patients were included. In left-sided ACC (n = 36), lymphatic recurrence was detected in the left renal hilum (50%), in the perirenal fat tissue cranial to the renal hilum (ventral, 47%; dorsal, 55%), para-aortic (47%), interaorto-caval (22%), and/or in the perirenal fat tissue caudal to the renal hilum (ventral, 20%; dorsal, 17%). In right-sided ACC (n = 20), lymph node metastases were detected in the perirenal fat tissue cranial to the renal hilum (dorsal, 55%; ventral, 45%), interaorto-caval (35%), in the area of the right renal artery (10%), and/or paracaval (15%). Patients with right-sided ACC showed left-paraaortic lymph node recurrences in 10% of cases. CONCLUSION: Metastatic lymphatic spread appears to be more extensive than previously thought. The distribution pattern of lymph node metastases described in our study could be used as a guide for a more extended lymph node dissection.

Adjuvant radiotherapy for the primary treatment of adrenocortical carcinoma: are we offering the best?

ABSTRACT Purpose: To evaluate the role of ARDT after surgical resection of ACC. Materials and Methods: Records of patients from our institutional ACC database were retrospectively assessed. A paired comparison analysis was used to evaluate the oncological outcomes between patients treated with surgery followed by ARDT or surgery only (control). The endpoints were LRFS, RFS, and OS. A systematic review of the literature and meta-analysis was also performed to evaluate local recurrence of ACC when ARDT was used. Results: Ten patients were included in each Group. The median follow-up times were 32 months and 35 months for the ARDT and control Groups, respectively. The results for LRFS (p=0.11), RFS (p=0.92), and OS (p=0.47) were similar among subsets. The mean time to present with local recurrence was significantly longer in the ARDT group compared with the control Group (419±206 days vs. 181±86 days, respectively; p=0.03). ARDT was well tolerated by the patients; there were no reports of late toxicity. The meta-analysis, which included four retrospective series, revealed that ARDT had a protective effect on LRFS (HR=0.4; CI=0.17-0.94). Conclusions: ARDT may reduce the chance and prolong the time to ACC local recurrence. However, there were no benefits for disease recurrence control or overall survival for patients who underwent this complementary therapy.

Adjuvant radiotherapy for the primary treatment of adrenocortical carcinoma: Are we offering the best?

PURPOSE: To evaluate the role of ARDT after surgical resection of ACC. MATERIALS AND METHODS: Records of patients from our institutional ACC database were retrospectively assessed. A paired comparison analysis was used to evaluate the oncological outcomes between patients treated with surgery followed by ARDT or surgery only (control). The endpoints were LRFS, RFS, and OS. A systematic review of the literature and meta-analysis was also performed to evaluate local recurrence of ACC when ARDT was used. RESULTS: Ten patients were included in each Group. The median follow-up times were 32 months and 35 months for the ARDT and control Groups, respectively. The results for LRFS (p=0.11), RFS (p=0.92), and OS (p=0.47) were similar among subsets. The mean time to present with local recurrence was significantly longer in the ARDT group compared with the control Group (419±206 days vs. 181±86 days, respectively; p=0.03). ARDT was well tolerated by the patients; there were no reports of late toxicity. The meta-analysis, which included four retrospective series, revealed that ARDT had a protective effect on LRFS (HR=0.4; CI=0.17-0.94). CONCLUSIONS: ARDT may reduce the chance and prolong the time to ACC local recurrence. However, there were no benefits for disease recurrence control or overall survival for patients who underwent this complementary therapy.

Functioning unilateral adrenocortical carcinoma in a dog.

An 11-year-old, 24-kg, intact female Siberian husky dog in anestrus had a 2-month history of polyuria and polydipsia. The dog had signs of mineralocorticoid excess such as hypertension and hypokalemia refractory to potassium supplementation. Abdominal ultrasound revealed an irregular mass in the left adrenal gland. The ACTH stimulation test for aldosterone concentration did not reveal hyperaldosteronism. Unilateral adrenalectomy was performed and histopathology identified adrenal cortical carcinoma. All clinical signs of mineralocorticoid excess ceased after surgery.

Approach to the patient with adrenocortical carcinoma.

Adrenocortical cancer (ACC) is a rare and often aggressive malignancy that requires multidisciplinary expertise for optimal management. It can present with symptoms of rapidly appearing excess steroid secretion or an abdominal mass, or it can be discovered incidentally. Thorough imaging and endocrine evaluations can identify the majority of ACCs amongst adrenal tumors; however, some smaller ACCs are better identified using fluorodeoxyglucose-positron emission tomography/computed tomography scan. Complete resection by an expert surgeon is the only potentially curative treatment for ACC, and tumor spillage should be avoided. Histopathology is important for diagnosis, but immunohistochemistry markers and gene profiling of the resected tumor may become superior to current staging systems to stratify prognosis. Despite complete resection in stage I-III tumors, approximately 40% of patients develop metastasis within 2 yr. Some retrospective studies indicate that adjuvant mitotane therapy prolongs disease-free survival, leading several centers to recommend its administration; prospective studies are under way to provide future evidence-based recommendations. For locally invading ACC, extensive en bloc resection is attempted, followed by adjuvant mitotane and, in selected cases, adjuvant radiotherapy. When ACC is not surgically resectable, mitotane therapy is adjusted to reach serum levels of 14-20 µg/ml. Careful replacement of glucocorticoid and mineralocorticoid deficiency after surgery or mitotane therapy is important; steroid excess from remaining tumor burden should also be controlled to avoid its morbidities. For metastatic disease, combination chemotherapy should be administered, if possible, in the context of multicenter collaborative research protocols. New insights in the molecular pathogenesis of ACC should allow the development of improved targeted therapies.

Carcinoma corticosuprarrenal en niños. Experiencia de 16 años en el Hospital Infantil de México Federico Gómez / Corticosuprarenal carcinoma in childre. Sixteen years of experience in the \"Hospital Infantil de Mexico Federico Gomez\"

Introducción. Los tumores de la corteza suprarrenal son raros, se estima una incidencia de 0.5-2 casos/millón de habitantes /año, con muy escasos reportes pediátricos en la literatura. Material y métodos. Se presenta una revisión de los casos de carcinoma corticosuprarrenal vistos en un período de 16 años en el Hospital Infantil de México Federico Gómez. Resultados. En total fueron 8 pacientes, con edades de 5 meses a 7 años 11 meses; predominó el sexo femenino. Siete con manifestaciones clínicas de exceso hormonal, uno se consideró hormonalmente no funcionante. El síndrome más común fue virilización, con pubarca como dato más frecuente seguido por clitoromegalia. Un paciente presentó síndrome de Cushing con sobrepeso como dato inicial, acompañado de detención del crecimiento. Todos los casos mostraron alteración en la determinación de metabolitos urinarios y los estudios de gabinete revelaron la presencia de masa tumoral. Se realizó resección quirúrgica total a todos los pacientes. Hubo 2 pacientes con metástasis desde el momento del diagnóstico y uno más con metástasis tardías; los 3 fallecieron a pesar de quimioterapia múltiple. Conclusión. El diagnóstico precoz, resección quirúrgica total y peso bajo del tumor favorecieron el pronóstico