INTRODUCTION:Adrenocortical cancer is a rare tumor with a poor prognosis. Patients who present with distant metastatic disease generally are treated with the adrenocorticolytic drug, mitotane. Localized metastases may be palliated by surgical removal or radiation therapy. We present a case of a woman with metastatic adrenocortical cancer, on mitotane, who presented with dyspnea and atelectasis due to endobronchial obstruction, with prolonged improvement following endobronchial excision, YAG laser therapy, and sequential brachytherapy.
CASE PRESENTATION:A 56-year old female non smoker with a past medical history significant for adrenocortical cancer, s/p left nephrectomy and adrenolectomy ten years ago, presented with a 6 month history of nonproductive cough and weight loss. Physical examination was unremarkable. Chest radiograph revealed bilateral pulmonary nodules and a widened mediastinum. CT scan of the chest confirmed the bilateral nodules ranging in size from subcentimeter to 4.5 cm, plus a 3.5 cm left hilar mass compressing the left upper lobe; there was also a 4 cm right adrenal mass. A CT guided biopsy of one of the lung nodules revealed pathology consistent with metastatic adrenocortical cancer. The patient was started on the chemotherapeutic agent mitotane. Despite treatment, her cough persisted. Three months later, the patient developed shortness of breath. Repeat chest radiograph and CT scan revealed interim collapse of the entire left lung. The patient underwent flexible bronchoscopy which revealed a mass in the left mainstem bronchus (figure 1). Biopsy of the lesion was attempted however immediate bleeding was encountered. The patient then underwent immediate rigid bronchoscopy with excision of the mass and YAG laser treatment. A follow-up CT of the chest revealed re-expansion of the left lower lobe; left upper lobe atelectasis was without change. A narrowed left upper lobe orifice with a patent left lower lobe was seen on repeat bronchoscopy. A brachytherapy catheter was then placed in the left lower lobe segment (figure 2). After a total of three courses of endoluminal brachytherapy, the patient reported considerable improvement of her symptoms. She was last seen 8 months s/p brachytherapy and denied dyspnea and only reported a minimal non-productive cough. CT scan of the chest at this time revealed continued complete expansion of the left lower lobe. She remains on mitotane.
DISCUSSIONS:Adrenocortical cancer (ACC) is a rare tumor with an overall incidence of approximately one per million population per year. Median age at diagnosis is 44 years. Most patients present with disease outside the adrenal gland, and survival for such patients is poor. Common sites of metastasis include liver, lungs, lymph nodes and bone. Treatment for patients with metastatic disease generally includes the adrenocorticolytic drug mitotane, with or without the addition of cytotoxic drugs. Localized metastases may be surgically resected. The efficacy of adjuvant radiotherapy is controversial and data from retrospective series are conflicting. There is limited available data regarding brachytherapy. Several studies have shown symptom improvement in the treatment of obstructing primary lung tumors with a combination of endobronchial treatment modalities. This is the first case showing improvement following a combination of endobronchial procedures for adrenocortical cancer with pulmonary metastasis. After successful endobronchial excision of the tumor and YAG laser therapy, followed by sequential endoluminal brachytherapy, our patient demonstrated prolonged re-expansion of the left lower lobe and relief of dyspnea.
CONCLUSION:We report a rare case of metastatic adrenocortical cancer to the lung causing lung collapse and dyspnea, with improvement following a variety of endobronchial procedures. We recommend consideration of a combination approach using interventional pulmonary techniques in the management of such patients.
DISCLOSURE:Naricha Chirakalwasan, No Financial Disclosure Information; No Product/Research Disclosure Information