Adrenocortical Carcinoma

Introduction

Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy originating from the adrenal cortex. It is characterized by a poor prognosis and a complex pathogenesis that is not yet fully understood. The disease often presents with hormone excess or as a large abdominal mass, and its treatment remains challenging due to limited effective therapeutic options.

Key Insights

Pathogenesis and Molecular Mechanisms:
- Overexpression of insulin-like growth factor 2 (IGF-2) and activation of the Wnt/β-catenin pathway are frequently observed in ACC, but these factors alone are insufficient for malignant transformation .
- Recent studies have focused on onco-developmental genes and local adrenal paracrine and autocrine effects of cellular peptides.
Clinical Presentation and Diagnosis:
- ACC often presents with symptoms of hormone excess (e.g., Cushing's syndrome, virilization) or as a large abdominal mass .
- Diagnostic workup includes comprehensive hormonal assessment and detailed imaging, such as computerized tomography (CT) and magnetic resonance imaging (MRI), which help differentiate ACC from benign adenomas .
Prognosis and Survival:
- The overall 5-year survival rate for ACC remains poor, ranging between 20% and 45%.
- Factors associated with poor prognosis include age over 40 years and the presence of metastases at diagnosis.
Treatment Strategies:
- Surgery is the mainstay of treatment for localized ACC, with complete resection including lymphadenectomy recommended.
- Mitotane is the preferred adjuvant treatment and is also used in metastatic disease, often combined with cytotoxic drugs like etoposide, doxorubicin, and cisplatin .
- New targeted therapies, such as IGF-1 receptor inhibitors, are under investigation and may improve future treatment options.
Challenges and Future Directions:
- Despite advances in diagnostic techniques, significant progress in treatment and outcomes has been limited due to the rarity and heterogeneity of ACC .
- Collaborative efforts, including international conferences and networks like the European Network for the Study of Adrenal Tumors (ENSAT), are crucial for advancing research and developing new therapeutic strategies.

Conclusion

Adrenocortical carcinoma is a rare and aggressive cancer with a poor prognosis. Key molecular pathways involved in its pathogenesis include IGF-2 overexpression and Wnt/β-catenin activation. The disease often presents with hormone excess or as a large abdominal mass, and comprehensive hormonal and imaging assessments are essential for diagnosis. Surgery remains the primary treatment for localized disease, while mitotane is the cornerstone of adjuvant and metastatic treatment. Despite these strategies, the overall survival rate remains low, highlighting the need for continued research and the development of new targeted therapies. Collaborative international efforts are essential to improve understanding and treatment of this challenging malignancy.

Adrenocortical Carcinoma

Summary

Introduction

Key Insights

Conclusion

Sources and full results

Adrenocortical Carcinoma: Clinical Update

Adrenocortical carcinoma (ACC) is a rare and heterogeneous malignancy with poor prognosis, and current diagnostic and therapeutic strategies require further refinement.

Adrenocortical carcinoma

Medical advances have improved diagnostic techniques and supportive measures, potentially impacting the natural history of adrenocortical carcinoma.

Adrenocortical carcinoma

Adrenocortical carcinoma has a high incidence of nonfunctioning tumors and a low incidence of childhood adrenal carcinoma, with underemphasized incidence rates and natural history.

Adrenocortical carcinoma: a clinician's update

Adrenocortical carcinoma is a rare neoplasm with poor prognosis, and current treatment options include mitotane and cytotoxic drugs, with new targeted therapies like IGF-1 receptor inhibitors under investigation.

Adrenocortical carcinoma in the United States

Adrenocortical carcinoma (ACC) treatment utilization and factors associated with long-term survival after resection in the United States are discussed.

Adrenocortical carcinoma

Adrenocortical carcinoma has a poor 5-year survival rate, with surgery being the mainstay treatment and mitotane showing modest effects in unresectable or metastatic cases.

Adrenocortical carcinoma: Past, present, and future

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with limited understanding of its pathogenesis and treatment progress, but understanding and developing targeted therapies may improve outcomes.

Update in adrenocortical carcinoma.

Adrenocortical carcinoma (ACC) is an orphan malignancy with a complex pathogenesis and requires new treatment concepts to improve outcomes.

Adrenocortical Carcinoma: Review and Update

Adrenocortical carcinoma is a rare, aggressive tumor with poor prognosis, and gene expression profiling can help distinguish it from adenomas and identify treatment targets.

Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy.

Adrenocortical carcinoma has a poor prognosis, but mitotane therapy may offer temporary benefits in controlling endocrine symptoms without significantly affecting survival.

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