Efficacy of Adjuvant Radiotherapy of the Tumor Bed on Local Recurrence of Adrenocortical Carcinoma

Fassnacht, Martin; Hahner, Stefanie; Polat, Bülent; Koschker, Ann-Cathrin; Kenn, W.; Flentje, Michael; Allolio, Bruno

doi:10.1210/jc.2006-1007

Cited by 232 publications

(146 citation statements)

References 16 publications

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“…Radiotherapy of the tumour bed is recommended after R1/Rx resection in ENSAT stage I-III tumours, should be individualized for completely resection (R0) in high-risk patients specially if there is microscopic blood vessels invasion and Ki-67>10%, and is not recommended after R2 resection and ENSAT stage IV tumours [31,32].…”

Section: Discussionmentioning

confidence: 99%

Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Nunes¹

2018

EMIJ

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We performed an informatics search of the medical registers of our centre (filtered by age -over 18-years-old) for the diagnosis codes 'adrenal neoplasia' and 'adrenocortical carcinoma', and Review ArticleEndocrinol Metab Int J 2018, 6(1): 00148 AbstractPurpose: Adrenocortical carcinoma (ACC) is an uncommon and aggressive malignancy. We aimed to investigate the clinical and pathological findings of ACC diagnosed/treated in our hospital from January/1988 to January/2013.Methods: Analysis of presentation, imagiology, histopathology review, staging and survival data. Results:We reviewed 31 patients. The average age at diagnosis was 54.6±12.6years and 51.6% were females. At diagnosis, 11.8% were asymptomatic, 23.5% presented back pain, 17.6% general malaise, 9.4% hypercortisolism and 17.6% hirsutism/virilization. Tumours had 11.7±6.6cm, 53.3% located on the right side, 72.7% with local invasion, 25% inferior vena cava invasion and 63.7% distant metastasis. Ten were submitted to adrenalectomy: 80% presented atypical mitosis, 20% diffuse architecture, 60% ≤25% clear cells, 90% necrosis, 70% venous invasion, 90% sinusoidal invasion, 60% capsule invasion, mitotic index 41.2/50HPF (±36.8). One patient was treated with adjuvant radiotherapy, three with cytotoxic therapy and five with adrenolytic treatment (the remaining didn't start mitotane due to death or difficulties in obtaining the drug). Two patients, on stage II ENSAT, maintain follow up with mitotane and no evidence of recurrence. The mean overall survival was 59.2±52.4 months for stages I+II and 6.2±6.8 months for stages III+IV (ENSAT) (p=0.004). Conclusion:Our series reinforces the importance of early diagnosis by demonstrating a worse prognosis in more advanced stages and the need for a multidisciplinary approach from a team of experts in the management of such a rare malignancy.

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Section: Discussionmentioning

confidence: 99%

Adrenocortical Carcinoma - A 25 Years Tertiary Centre Experience and Short Review of the Literature

Nunes¹

2018

EMIJ

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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]. There are a few findings that suggest the potential benefit of palliative treatment of brain metastases or vena cava obstruction [54].…”

Section: Radiotherapymentioning

confidence: 99%

“…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]. 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%

“…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%

“…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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