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INTRODUCTION: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with aggressive behavior. Most of our knowledge about this rare tumor is based on retrospective case series. This study aimed at analyzing the clinicopathological features and outcomes of patients treated at a tertiary cancer center in India. PATIENTS AND METHODS: We retrospectively reviewed the data of patients with ACC registered from January 2006 to December 2015. RESULTS: Thirty-seven patients were included in the study, 20 males and 17 females. Median age was 49 (18-78) years. Hormonal overproduction was noticed in 27% of patients. Median tumor size was 10 cm (2-22). Seventeen patients had metastatic disease and 20 patients were localised at diagnosis. Median follow-up was 22 months and median overall survival (OS) was 23.46 months. OS at 2 years and 5 years was 46.1% and 21%, respectively. The median disease-free survival (DFS) was 20 months. DFS at 2 years and 5 years was 45% and 24%, respectively. Age, sex, tumor size, hormonal overproduction, tumor laterality, and stage of the disease did not influence survival. However, advanced stage was associated with higher risk for recurrence. (P = 0.03). CONCLUSION: ACC is a rare endocrine malignancy with very poor survival rates. Rate of recurrence is high even after complete surgery. Systemic treatment options are limited. Newer agents are needed to improve outcome.
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Malignant peripheral nerve sheath tumour (MPNST) is a rare variety of sarcoma occurring in the uterine cervix. Only 16 cases have been reported in literature till date. There is no clear consensus on the treatment due to the rarity of the disease. This is primarily managed by surgery. Here, we present a case of MPNST of the cervix who was treated with preoperative radiotherapy followed by radical hysterectomy and bilateral salpingo-oophorectomy.
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Germ cell tumour of the testis is the most common cancer in young men in the western world. India has the lowest incidence globally, and hence Indian data are sparse. We report the outcomes of patients with nonseminomatous germ cell tumours of testis treated at a tertiary cancer centre in South India over a period of 10 years. Patients with a histopathological diagnosis of nonseminomatous germ cell tumours of the testis from 1 January 2006 to 31 December 2016 were included in the study. Patient demographics, tumour characteristics and treatment details were retrieved from case records. Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival (OS). Cox regression model was used to analyse the prognostic factors. One hundred and nineteen patients with nonseminomatous germ cell tumours of the testis were included in the study. The median follow-up was 81 months. The estimated 4-year OS and progression-free survival were 87.1% and 84.5%, respectively. The four-year OS for good, intermediate and poor-risk groups was 93.6%, 87.5% and 52.6%, respectively. The PFS at 4 years was 91.4%, 87.8% and 47.4% for good, intermediate and poor-risk groups, respectively. The presence of nonpulmonary visceral metastasis and biochemical response after chemotherapy were significant predictors for OS and PFS in multivariate cox proportional hazards regression. The survival figures are comparable to the rest of the world except in the poor prognostic risk group. The inferior survival noticed in this group of patients may be due to the lack of good salvage procedures. High-dose chemotherapy with stem-cell support may be considered more often for this group of patients.