Adrenocorticol Cancer - a Single Center Case Series
Presentation Number: SUN 416
Date of Presentation: April 2nd, 2017
Sehrish Faud1, Umal Azmat1 and Waqas Shafiq*2
1Shaukat khanam Memorial Cancer Hospital and Research Centre, Lahore, 2Shaukat Khanam Memorial Cancer Hospital and Research Centre, Lahore, PAKISTAN
Adrenocorticol carcinoma (ACC) is a rare but highly aggressive malignancy with incidence of 0.72 per million persons per year. In approximately 60% of these patients, symptoms related to excessive hormone secretion are the main reason for seeking medical attention. Others present with vague abdominal symptoms mostly due to mass effect of the tumor itself. A small percentage of ACCs is incidentally discovered on imaging studies. Due to its rarity, single institution experience is limited and optimum treatment modalities are less well established especially in developing Countries.
Clinical case Series:
This is a retrospective review of patients with ACC presenting to Shaukat Khanum Memorial Cancer Hospital and Research center from 2011 to 2016. In this case series we illustrate the clinical presentation, staging, treatment and survival of patients with ACC at a tertiary care Center in Pakistan.A total of fourteen patients were seen with median age of 35 years at time of diagnosis.
Out of which eleven patients were females and three were males.
Three tumors secreted aldosterone, two presented with hypokalemia and hypertension and one with abdominal pain. Two tumors secreted DHEA and both presented with virilization and abdominal pain. One secreted cortisol and preseneted with abdominal pain. One tumor co-secreted DHEA and cortisol and patient presented with virilization, diabetes mellitus and hypertension.
Amongst non-secretory tumors, five presented with abdominal pain and two were found incidentally.
At time of initial presentation two patients were stage I, four were stage II, two were stage III and three were stage IV. Three patients presented after surgery and therefore initial stage could not be determined.
Thirteen patients underwent surgery as initial treatment. One patient had irresectable disease and therefore received chemotherapy as initial therapy.
Median follow up was 17.5 months. During this follow up four patients had disease relapse.
Two out of fourteen patients died, one is on palliative follow up and one patient was lost to follow up. Ten out of fourteen patients were without any evidence of relapse.
Radical open surgical excision is the treatment of choice for patients with localized ACC and remains the only method by which long term disease free survival may be achieved. However in patients who are not cured by surgery alone, their treatment options are limited due to partial response and adverse effects leading to poor tolerability and hence deterioration of quality of life.
In our single center experience of 14 patients over 17.5 months mortality was 14%, which is not consistent with other studies, however our median follow-up at this point is not long enough to deduce that mortality at our center is lower as compared to other centers.
Nothing to Disclose: SF, UA, WS