Author: Dr Irina Robinson, Adult Endocrinologist Practicing in Boca Raton, Florida

SUMMARY

This case study focuses on a 45-year-old female patient diagnosed with metastatic hormonally active adrenal adenocarcinoma (adrenal cancer), presenting with symptoms of Cushing’s syndrome and other hormonal imbalances. Despite undergoing systemic chemotherapy and palliative care to manage her symptoms and disease progression, the patient’s condition deteriorated due to extensive metastatic spread to the lungs and brain.

The study highlights the complexity of managing aggressive adrenal malignancies and underscores the need for early detection and innovative therapeutic strategies in treating adrenal cancer.

INTRODUCTION

The incidental discovery of adrenal nodules, known as adrenal incidentalomas, has become more common due to the frequent use of advanced imaging techniques like CT scans and MRIs for unrelated medical issues. These nodules are found in up to 5% of all abdominal imaging studies. While most are harmless and pose minimal health risks, some can be hormonally active or malignant, requiring careful evaluation.

The challenge of managing adrenal cancer lies in identifying potentially life-threatening tumors, such as pheochromocytomas or adrenocortical carcinomas (ACCs), which are rare but aggressive. ACCs, with an incidence of 1-2 cases per million annually, are often diagnosed at advanced stages, complicating treatment and worsening prognosis. Effective management of ACCs requires a multidisciplinary approach, emphasizing early detection and intervention to improve patient outcomes.

PATIENT PROFILE

Age: 45 years

Gender: Female

Diagnosis: Metastatic hormonally active adrenal adenocarcinoma

Clinical Background: The patient had a history of adrenal gland resection in 2013 due to adrenal adenocarcinoma. She presented with symptoms including shortness of breath, weakness, unexplained weight loss, hirsutism, fatigue, and abdominal pain. Further medical tests indicated symptoms of Cushing’s syndrome, increased body hair, deepening of the voice, and Conn’s syndrome. Imaging studies revealed extensive metastatic lesions in the lungs and brain.

SIGNS & SYMPTOMS

Adrenal Cancer

Patients with hormonally active adrenal adenocarcinoma often present with:

  • Shortness of breath
  • Weakness
  • Unexplained weight loss
  • Hirsutism
  • Fatigue
  • Abdominal pain
  • Symptoms of Cushing’s syndrome (moon face, high blood pressure, fat hump on the neck, weak proximal muscles)
  • Hyperandrogenism (increased body hair, deepening of the voice)
  • Conn’s syndrome (high blood pressure, low potassium levels)

INITIAL ASSESSMENT

Primary Diagnosis: Poorly differentiated adenocarcinoma

Secondary Diagnoses: Cortisol, aldosterone, and androgen-secreting adenocarcinoma with metastases to the lungs and brain

Imaging Studies: CT scans, MRIs, and PET scans revealed extensive metastatic lesions and hypermetabolic activity in multiple organs, indicating aggressive disease progression.

TREATMENT MODALITIES

Systemic Chemotherapy: Multiple rounds of systemic chemotherapy were administered in an attempt to stabilize the disease. However, these attempts were unsuccessful.

Medications: The patient was treated with ketoconazole and metyrapone to manage the symptoms of Cushing’s syndrome.

Palliative Care: Due to the failure of chemotherapy to control disease progression, palliative care was initiated to manage pain and neurological symptoms caused by brain metastases.

CHALLENGES & CONSIDERATIONS

Aggressive Nature of Disease: The poor differentiation of the adenocarcinoma and extensive metastatic spread complicated treatment efforts.

Endocrine Dysfunction: Hormonal imbalances due to cortisol, aldosterone, and androgen secretion led to complex symptomatology, necessitating a comprehensive management approach.

Palliative Focus: As disease progression continued despite aggressive therapy, the primary focus shifted to palliative care for achieving symptom relief and improving quality of life.

PROGNOSIS AND FUTURE DIRECTIONS

Despite aggressive treatment efforts, the patient’s condition deteriorated due to the extensive spread of cancer and associated endocrine dysfunction, ultimately leading to multi-organ failure.

This case underscores the lethality of metastatic, hormonally active adrenal adenocarcinoma. Early detection through comprehensive evaluation of adrenal incidentalomas and close follow-up is crucial. Despite aggressive multidisciplinary treatment, extensive metastatic disease and endocrine dysfunction can lead to multi-organ failure.

The need for early detection, continuous monitoring, and the development of innovative treatment approaches is paramount. Collaborative, multidisciplinary care and research into targeted therapies hold promise for improving outcomes in patients with this aggressive form of cancer.

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