Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma.

Hermsen, I G C; Gelderblom, Hans; Kievit, J.; Romijn, Johannes A.; Haak, Harm R.

doi:10.1530/eje-07-0723

Cited by 35 publications

(26 citation statements)

References 45 publications

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“…Agents with anecdotal activity are mitotane (o,p-DDD) and cisplatin. Radiation therapy is generally reserved for palliation of refractory disease (15). According to the ARAR0332 protocol (Treatment of Adrenocortical Tumors with Surgery plus Lymph Node Dissection and Multiagent Chemotherapy) (16), a collaboration between Children’s Oncology Group (COG) and Brazilian institutions, recommended management for stage II patients is surgery with complete resection of tumor, retroperitoneal lymph node dissection, without chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Pubertal outcome in a female with virilizing adrenocortical carcinoma

Breidbart

Cameo

Garvin

et al. 2016

Journal of Pediatric Endocrinology and Metabolism

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Adrenocortical tumors are neoplasms that rarely occur in pediatric patients. Adrenocortical carcinoma (ACC) is even more uncommon, and is an aggressive malignancy with 5-year survival of 55% in a registry series. There is a lack of information on long-term endocrine outcome in survivors. We describe a 10-year follow-up in a patient who presented at 3 years 5 months with a 1-year history of axillary odor and 6 months’ history of pubic hair development with an increased clitoral size. Androgen levels were increased and a pelvic sonogram revealed a suprarenal mass of the left kidney. The tumor was successfully removed. At 6 years 11 months, androgen levels increased again. Workup for tumor recurrence was negative and the findings likely represented early adrenarche. The patient had menarche at an appropriate time and attained a height appropriate for her family.

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Section: Discussionmentioning

confidence: 99%

Pubertal outcome in a female with virilizing adrenocortical carcinoma

Breidbart

Cameo

Garvin

et al. 2016

Journal of Pediatric Endocrinology and Metabolism

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“…Even in patients with stage 4 disease, survival ranges from a few months to several years. Exceptional cases of long-term survival with the diagnosis of ACC have been reported (242). In the Michigan Endocrine Oncology Repository, roughly 5% of all patients diagnosed with ACC will have a disease course of Ͼ10 years (T.E., unpublished results).…”

Section: Prognostic Factorsmentioning

confidence: 99%

Adrenocortical Carcinoma

et al. 2013

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Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Here we summarize the knowledge about diagnosis, epidemiology, pathophysiology, and therapy of ACC. Over recent years, multidisciplinary clinics have formed and the first international treatment trials have been conducted. This review focuses on evidence gained from recent basic science and clinical research and provides perspectives from the experience of a large multidisciplinary clinic dedicated to the care of patients with ACC.

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“…As a result, survival is only a few weeks or months (Kerkhofs et al 2013a). In other patients, the disease follows a more indolent course and survival of up to several years has been observed (Hermsen et al 2008). The search for clinical predictors of survival in these patients is still ongoing.…”

Section: Advanced Diseasementioning

confidence: 99%

Developing treatment for adrenocortical carcinoma

Kerkhofs¹,

Ettaieb²,

Hermsen³

et al. 2015

Endocrine-Related Cancer

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Cancer of the adrenal cortex (ACC) is a rare endocrine malignancy with limited treatment options. Patients typically present with autonomous hormonal overproduction and/or a large abdominal mass. Hormonal assays and medical imaging can be diagnostic, but urinary steroid profiling might be a more sensitive technique to assess malignancy in adrenal tumours. The stage of the disease at diagnosis is the most important prognostic factor. The current staging system needs refinement, especially to separate aggressive from indolent disease in stage IV patients and to select patients who need adjuvant treatment after complete surgical resection. Regarding the latter, assessing the proliferation index Ki-67 seems the best tool currently available. Genomic profiling is expected to become of clinical relevance in the future. Medical therapy is centred on the adrenolytic drug mitotane, which carries considerable toxicity and is not easy to manage. Its tolerability and long plasma level build-up phase may be improved by therapeutic drug monitoring based on pharmacokinetic modelling and intensive counselling of patients. Current chemotherapy regimens can offer disease stabilization in about 50% of patients, but an objective response should be expected in !25%. Research on targeted therapy and immunotherapy is difficult in this rare disease with often heavily pre-treated patients and has not yet been successful. Quality of care should be ensured by treating patients in centres with established experience in multidisciplinary oncologic care, who adhere to prevailing guidelines and state-of-the-art in diagnostic and treatment concepts. International collaboration in fundamental research and clinical trials is the key to further elucidate the pathogenesis and to improve patient care.

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Extremely long survival in six patients despite recurrent and metastatic adrenal carcinoma.

Cited by 35 publications

References 45 publications

Pubertal outcome in a female with virilizing adrenocortical carcinoma

Pubertal outcome in a female with virilizing adrenocortical carcinoma

Adrenocortical Carcinoma

Developing treatment for adrenocortical carcinoma

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