Treatment with docetaxel and cisplatin in advanced adrenocortical carcinoma, a phase II study

Urup, Thomas; Pawlak, Wojciech; Petersen, Peter Meidahl; Pappot, Helle; Rørth, Mikael; Daugaard, Gedske

doi:10.1038/bjc.2013.229

Cited by 23 publications

(19 citation statements)

References 15 publications

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“…Other studies including cytotoxic chemotherapy only produced disappointing results. Among the tested agents were capecitabine (combined with bevacizumab) (Wortmann et al 2010), paclitaxel (Berruti et al 2012b), irinotecan (Baudin et al 2002) and docetaxel (combined with cisplatin) (Urup et al 2013). Again, mitotane is a hindrance in investigating new drugs, since virtually all patients in advanced stages are pre-treated.…”

Section: Chemotherapymentioning

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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Section: Chemotherapymentioning

confidence: 99%

Developing treatment for adrenocortical carcinoma

Kerkhofs¹,

Ettaieb²,

Hermsen³

et al. 2015

Endocrine-Related Cancer

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“…Meta-analysis suggests that radiotherapy should be performed to the tumour bed in patients with a high risk of local recurrence [53]. Until better evidence is available, scientists recommend a total dose > 40 Gy (grays) with single fractions of 1.8-2 Gy, even 4 Gy in some cases (including a boost volume of 50-60 Gy) [43,54].…”

Section: Radiotherapymentioning

confidence: 99%

“…There are a few findings that suggest the potential benefit of palliative treatment of brain metastases or vena cava obstruction [54]. However, the role of radiotherapy remains controversial and is still discussed, especially in recurrent disease [52,53,54].…”

Section: Radiotherapymentioning

confidence: 99%

“…Przeprowadzone metaanalizy sugerują, że radioterapia loży pooperacyjnej powinna być stosowana u pacjentów z wysokim ryzykiem miejscowego nawrotu [53]. W świetle aktualnej wiedzy, naukowcy zalecają zastosowanie terapii o całkowitej aktywności > 40 Gy (włącznie z pierwszą, bolusową dawką o wartości nawet do 50-60 Gy), stosując frakcje 1,8-2 Gy, do 4 Gy podczas pojedynczego podania [45,56].…”

Section: Patogeneza I Aspekty Molekularneunclassified

“…Istnieje kilka doniesień sugerujących potencjalną korzyść z paliatywnej radioterapii przerzutów do mózgu lub naciekania żyły głównej dolnej [56]. Jakkolwiek wartość radioterapii nadal pozostaje nie w pełni określo-na i ciągle trwają dyskusje szczególnie dotyczące jej zastosowania w przypadku nawrotu choroby [52][53][54].…”

Section: Patogeneza I Aspekty Molekularneunclassified

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Rak kory nadnercza (ACC) — przegląd piśmiennictwa i doświadczenia własne

Dworakowska

Drabarek

Wenzel

et al. 2015

Endokrynologia Polska

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Adrenocortical carcinoma (ACC) is a malignant endocrine tumour. The rarity of the disease has stymied therapeutic development. Age distribution shows two peaks: the first and fifth decades of life, with children and women more frequently affected. Although 60-70% of ACCs are biochemically found to overproduce hormones, it is not clinically apparent in many cases. If present, endocrine symptoms include signs of hypercortisolaemia, virilisation or gynaecomastia. ACC carries a poor prognosis, and a cure can be achieved only by complete surgical resection. Mitotane is used both as an adjuvant treatment and also in non-operative patients. The role of radio-and chemotherapy is still controversial. The post-operative disease free survival is low and oscillates around 30% due to high tumour recurrence rate. The diagnosis is based on tumour histological assessment with the use of the Weiss score, however urinary steroid profiling (if available) can serve to differentiate between ACC and other adrenal tumours. Conventional prognostic markers in ACC include stage and grade of disease, and, as currently reported, the presence of hypercortisolaemia. Molecular analysis has had a significant impact on the understanding of the pathogenetic mechanism of ACC development and the evaluation of prognostic and predictive markers, among which alterations of the IGF system, the Wnt pathway, p53 and molecules involved in cancer cell invasion properties and angiogenesis seem to be very promising. We here summarise our own experience related to the management of ACC and present a literature overview. We have not aimed to include a detailed summary of the molecular alterations biology described in ACC, as this has already been addressed in other papers. StreszczenieRak kory nadnercza (ACC) jest złośliwym guzem endokrynnym. Rzadkość jego występowania przyczyniła się do spowolnienia rozwoju nowych opcji terapeutycznych. Zachorowalność występuje dwuszczytowo: w pierwszej oraz piątej dekadzie życia, przy czym zachorowalność dotyczy częściej kobiet i dzieci. Mimo że 60-70% ACC jest zwykle czynna hormonalnie, to klinicznie nie zawsze jest to oczywiste. W przypadku guzów aktywnych hormonalnie mamy do czynienia z objawami hiperkortyzolemii, wirylizacji czy ginekomastii. ACC jest nowotworem o złym rokowaniu, a jego wyleczenie może być osiągnięte poprzez całkowitą resekcję zmiany. Mitotan znajduje zastosowanie zarówno w leczeniu adjuwantowym jak i w przypadkach guzów nieresekcyjnych. Natomiast rola radio-oraz chemioterapii pozostaje nadal kontrowersyjna. Przeżywalność po zabiegu operacyjnym oscyluje w granicach 30% i wiąże się z wysokim odsetkiem nawrotów. Rozpoznanie stawiane jest na podstawie analizy histopatologicznej przy użyciu Weiss score, jednakże analiza wydalania steroidów w moczu (o ile jest dostępna) może pomóc w rozróżnieniu pomiędzy ACC a gruczolakiem nadnercza, a także innymi guzami nadnercza. Głównymi czynnikami prognostycznymi są: stopień zróżnicowania i zaawansowania nowotworu i, według ostatnich doniesień, obecność hiperko...

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Adrenal Neoplasms

Stewart

Story

2017

Textbook of Uncommon Cancer

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Treatment with docetaxel and cisplatin in advanced adrenocortical carcinoma, a phase II study

Cited by 23 publications

References 15 publications

Developing treatment for adrenocortical carcinoma

Developing treatment for adrenocortical carcinoma

Rak kory nadnercza (ACC) — przegląd piśmiennictwa i doświadczenia własne

Adrenal Neoplasms

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