Adrenocortical cancer

Author(s):  
Steve Ball ◽  
Sajid Kalathil

Adrenocortical cancer (ACC) is rare and associated with poor prognosis. The incidence is estimated at 0.7–2 cases per one million. Overall survival rate at five years for ACC is 37–47%. While the pathogenesis of ACC is incompletely understood, inherited predisposition syndromes are common in childhood ACC. Clinical presentation can be with symptoms and signs of hormone excess (functional tumours), mass effects, or as an incidental radiological finding. A multidisciplinary approach combining radiology, biochemistry, and tissue-based pathology is needed to establish a diagnosis to guide a surgical approach aimed at complete resection of the tumour where possible. At present, recommended first-line therapies for advanced disease are mitotane monotherapy or etopiside, doxorubicin, and cisplatin plus mitotane. Metronomic capecitabine and gemcitabine have been used as alternatives. Adjuvant radiotherapy to the tumour bed should be considered for patients considered to be at high risk of recurrence.

2021 ◽  
Author(s):  
Subhash Thakur ◽  
Birendra Yadav ◽  
Karthik Nagamuthu ◽  
Saloni Chawla ◽  
Amit Dubey

Abstract Purpose: To present first case of refractory craniopharyngioma treated successfully with SRS in Nepal. Background: Craniopharyngioma is a benign tumor, which progresses slowly and compresses the pituitary gland and nearby structures. First line of treatment is surgery followed by adjuvant radiotherapy as complete resection is usually not feasible. Here, we are reporting a case of recurrent craniopharyngioma treated with LINAC based SRS. Case Presentation: A 43 years old man diagnosed case of craniopharyngioma in May 2019. He underwent left pterional craniotomy and subtotal resection of tumor and kept on observation. He developed symptomatic as well as radiological recurrence in July 2020. Second debulking was not possible, so we did SRS on 23rd August 2020; 14Gy Dose was delivered to gross tumor volume. Six months after SRS, Patient is doing well.Conclusions: LINAC based SRS is a frameless, non-invasive and safe procedure with excellent clinical outcomes for recurrent or residual craniopharyngioma.


2021 ◽  
Vol 6 (4) ◽  
pp. 529-531
Author(s):  
Subhash Thakur ◽  
Saloni Chawla

Purpose: To present first case of refractory craniopharyngioma treated successfully with SRS in Nepal. Bckground: Craniopharyngioma is a benign tumour, which progresses slowly and compresses the pituitary gland and nearby structures. First line of treatment is surgery followed by adjuvant radiotherapy as complete resection is usually not feasible. Here, we are reporting a case of recurrent craniopharyngioma treated with LINAC based SRS. Case Presentation: A 43 years old man diagnosed case of craniopharyngioma in May 2019. He underwent left pterional craniotomy and subtotal resection of tumour and kept on observation. He developed symptomatic as well as radiological recurrence in July 2020. Second debulking was not possible, so we did SRS on 23rd August 2020; 14Gy Dose was delivered to gross tumour volume. Six months after SRS, Patient is doing well. Conclusions: LINAC based SRS is a frameless, non-invasive and safe procedure with excellent clinical outcomes for recurrent or residual craniopharyngioma.


2021 ◽  
Author(s):  
Subhash Thakur ◽  
Saloni Chawla

Abstract Purpose: To present first case of refractory craniopharyngioma treated successfully with SRS in Nepal.Background: Craniopharyngioma is a benign tumour, which progresses slowly and compresses the pituitary gland and nearby structures. First line of treatment is surgery followed by adjuvant radiotherapy as complete resection is usually not feasible. Here, we are reporting a case of recurrent craniopharyngioma treated with LINAC based SRS.Case Presentation: A 43 years old man diagnosed case of craniopharyngioma in May 2019. He underwent left pterional craniotomy and subtotal resection of tumour and kept on observation. He developed symptomatic as well as radiological recurrence in July 2020. Second debulking was not possible, so we did SRS on 23rd August 2020; 14Gy Dose was delivered to gross tumour volume. Six months after SRS, Patient is doing well.Conclusions: LINAC based SRS is a frameless, non-invasive and safe procedure with excellent clinical outcomes for recurrent or residual craniopharyngioma.


Biomedicines ◽  
2020 ◽  
Vol 8 (12) ◽  
pp. 551
Author(s):  
Rosa Maria Paragliola ◽  
Andrea Corsello ◽  
Pietro Locantore ◽  
Giampaolo Papi ◽  
Alfredo Pontecorvi ◽  
...  

Adrenocortical carcinoma (ACC) represents one of the most aggressive endocrine tumors. In spite of a correct therapeutic strategy based on a multidisciplinary approach between endocrinologist, surgeon and oncologist, the prognosis is often poor. Surgery is the mainstay treatment in ACC. Mitotane, a dichloro-diphenyl-trichloro-ethane derivate, represents the main medical treatment of ACC in consideration of its adrenocytolitic activity and it is mainly employed as adjuvant treatment after complete surgical resection and for the treatment of advanced ACC. However, the use of mitotane as adjuvant therapy is still controversial, also in consideration of the retrospective nature of several studies. The recurrence of disease is frequent, especially in advanced disease at the diagnosis. Therefore, in these contexts, conventional chemotherapy must be considered in association with mitotane, being the combination etoposide, doxorubicin and cisplatin (EDP) the standard of care in this setting. A more modern therapeutic approach, based on the need of a salvage therapy for advanced ACC that progresses through first-line EDP, is focused on molecular-targeted therapies. However, robust clinical trials are necessary to assess the real efficacy of these treatments.


2020 ◽  
Vol 2 (4) ◽  
pp. 1-3
Author(s):  
Azeez T A ◽  

Hyperprolactinaemia is one of the commonest non-diabetes endocrine disorder seen by Endocrinologists. Prolactin is a polypeptide hormone secreted by the anterior pituitary gland. Its main physiological role is in milk production. Hyperprolactinaemia is more common among females and there is paucity of literature on hyperprolactinaemia among men. The causes of hyperprolactinaemia include physiological causes (such as sleep), drugs, sellar masses, including prolactinoma, other endocrine disorders such as hypothyroidism. The clinical presentation in males results from the disruption of the hypothalamus-pituitary-testicular axis and mass effect. It is an important and often overlooked cause of hypogonadism in males. Men usually present with macroprolactinomas with accompanying symptoms of mass effect such as visual impairment. Diagnosis of hyperprolactinaemia is made from relevant symptoms and signs and confirmed by immunoassay of the serum prolactin. Other relevant investigations need to be done to identify the potential causes of the disorder. Magnetic resonance imaging is the radiological investigation of choice to visualize the pituitary. Treatment of hyperprolactinaemia in males is dependent on aetiology and symptomatology. Some medications may need to be stopped or changed. Pharmacotherapy with dopamine agonists is the first line treatment of prolactinomas. Symptomatic cases of prolactinomas with drug resistance or drug intolerance are referred for surgery or radiotherapy


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Tsiachristas ◽  
H West ◽  
E.K Oikonomou ◽  
B Mihaylova ◽  
N Sabharwall ◽  
...  

Abstract Background The National Institute for Health and Care Excellence (NICE) updated their guidance for the management of patients with stable chest pain and recommended that all patients undergo computed tomography coronary angiography (CTCA). This update has sparked a great deal of debate, and was followed by upgrade of CTCA into a Class I indication in the recent ESC guidelines. The cost-effectiveness of using CTCA as first line investigation is still unclear. Purpose To describe the current clinical pathway of patients with stable chest pain presented to outpatient clinics, assess the compliance with the updated NICE guideline, and explore the costs and health outcomes of different non-invasive diagnostic tests in real-world clinical setting. Methods We used data of 4,297 patients who attended chest pain clinics in Oxford between 1 January 2014 and 31 July 2018. Data included clinical presentation (e.g. age and previous cardiovascular conditions), diagnostic tests, outpatient visits, hospitalization, and hospital mortality and was compared between 6 alternative first-line diagnostic tests. Multinomial regressions were performed to estimate the probability of receiving each alternative and the associated cost after adjusting for clinical presentation. A decision tree was developed to describe the clinical pathway for each alternative first-line diagnostic in terms of subsequent diagnostic tests and treatments and to estimate the associated costs and life days. Results The proportion of patients who received CTCA as first line diagnostic test increased from 1% in 2014 to 17% in 2018, while the publication of the updated NICE guidelines in 2016 led to a threefold increase in this proportion. CTCA is less likely to be provided as a first-line diagnostic to patients who are younger age, males, smokers, and have angina, PVD, or diabetes. The standardised rate of hospital admission was the lowest in the exercise ECG cohort (0.35 admissions per 1,000 life-days) followed by the CTCA cohort (0.40 admissions per 1,000 life-days) while the latter cohort had the lowest standardised rate of cardiovascular treatment (2.74% per 1,000 life days). Stress echocardiography and MPS were associated with higher costs compared with CTCA, other ECG, and exercise ECG after adjusting for clinical presentation and days of follow-up. CTCA is the pathway most likely to be cost-effective, even compared to exercise ECG, while the other diagnostic alternatives are dominated (i.e. they cost more for less life-days). Conclusions Currently, the updated NICE guidelines for stable chest pain are implemented only to a fifth of the cases in England. Our findings support existing evidence that CTCA is the most-cost effective first-line diagnostic test for this population. Hopefully, this will inform the debate around the implementation of the guidelines and help commissioning and clinical decision processes worldwide. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Institute of Health Research Oxford Biomedical Research Centre


Author(s):  
Kevin C. Miller ◽  
John P. Marinelli ◽  
Jeffrey R. Janus ◽  
Ashish V. Chintakuntlawar ◽  
Robert L. Foote ◽  
...  

AbstractEsthesioneuroblastoma (ENB) is a rare olfactory malignancy that can present with locally advanced disease. At our institution, patients with ENB in whom the treating surgeon believes that a margin-negative resection is initially not achievable are selected to undergo induction with chemotherapy with or without radiotherapy prior to surgery. In a retrospective review of 61 patient records, we identified six patients (10%) treated with this approach. Five of six patients (83%) went on to definitive surgery. Prior to surgery, three of five patients (60%) had a partial response after induction therapy, whereas two of five (40%) had stable disease. Microscopically margin-negative resection was achieved in four of five (80%) of the patients who went on to surgery, while one patient had negative margins on frozen section but microscopically positive margins on permanent section. Three of five patients (60%) recurred after surgery; two of these patients died with recurrent/metastatic ENB. In summary, induction therapy may facilitate margin-negative resection in locally advanced ENB. Given the apparent sensitivity of ENB to chemotherapy and radiotherapy, future prospective studies should investigate the optimal multidisciplinary approach to improve long-term survival in this rare disease.


2021 ◽  
Vol 15 ◽  
pp. 117955492098710
Author(s):  
Hyehyun Jeong ◽  
Yong Sang Hong ◽  
Young-Hoon Kim ◽  
Chan Wook Kim ◽  
Si Yeol Song ◽  
...  

Background: A multimodal approach is the standard treatment for desmoplastic small round cell tumor (DSRCT); however, many patients are diagnosed with inoperable disease, which leaves chemotherapy as the only treatment option. There are limited data on the effectiveness of palliative chemotherapy, especially when used after first-line treatment. Here, we evaluated the clinical outcomes of patients with DSRCT treated with multiple lines of chemotherapy. Methods: We reviewed medical records of 14 patients with pathologically confirmed DSRCT at Asan Medical Center between 2004 and 2018. Results: The median age at diagnosis was 25, with males comprising 92.9% of patients. All patients had inoperable disease at presentation and received chemotherapy as the initial treatment. Four patients (28.6%) were treated with surgery, and complete resection was achieved in 1 patient. Median overall survival (OS) was 23.9 months, and 1-, 2-, and 3-year survival rates were 92.9%, 48.6%, and 19.5%, respectively. In patients receiving first- (N = 14), second- (N = 10), and third-line (N = 8) chemotherapy, median time-to-progression was 9.9, 3.5, and 2.5 months, respectively, and the disease control rates were 100%, 88.9%, and 75.0%, respectively. Factors associated with longer OS in the univariable analysis were ⩽2 metastatic sites at presentation (27.0 vs 14.7 months; P = .024) and surgery with intended complete resection (43.5 vs 20.1 months; P = .027). Conclusions: Although advanced DSRCT may initially respond to chemotherapy after first-line treatment, the response becomes less durable as the disease progresses. Individualized treatment decisions focused on palliation should be made.


2021 ◽  
Vol 10 (15) ◽  
pp. 3230
Author(s):  
Jun Nishio ◽  
Shizuhide Nakayama ◽  
Kazuki Nabeshima ◽  
Takuaki Yamamoto

Dedifferentiated liposarcoma (DDL) is defined as the transition from well-differentiated liposarcoma (WDL)/atypical lipomatous tumor (ALT) to non-lipogenic sarcoma, which arises mostly in the retroperitoneum and deep soft tissue of proximal extremities. It is characterized by a supernumerary ring and giant marker chromosomes, both of which contain amplified sequences of 12q13-15 including murinedouble minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4) cell cycle oncogenes. Detection of MDM2 (and/or CDK4) amplification serves to distinguish DDL from other undifferentiated sarcomas. Recently, CTDSP1/2-DNM3OS fusion genes have been identified in a subset of DDL. However, the genetic events associated with dedifferentiation of WDL/ALT remain to be clarified. The standard treatment for localized DDL is surgery, with or without radiotherapy. In advanced disease, the standard first-line therapy is an anthracycline-based regimen, with either single-agent anthracycline or anthracycline in combination with the alkylating agent ifosfamide. Unfortunately, this regimen has not necessarily led to a satisfactory clinical outcome. Recent advances in the understanding of the pathogenesis of DDL may allow for the development of more-effective innovative therapeutic strategies. This review provides an overview of the current knowledge on the clinical presentation, pathogenesis, histopathology and treatment of DDL.


2021 ◽  
pp. 004947552110365
Author(s):  
Abhijeet Kumar ◽  
Nirmal Prasad Shah ◽  
Narendra Pandit ◽  
Suresh Prasad Sah ◽  
Rakesh Kumar Gupta ◽  
...  

Gallbladder perforation still continues to perplex surgeons; 25 such patients diagnosed either pre- or intra-operatively and managed at our institute over the last 10 years period were analysed. Only eight were diagnosed pre-operatively, while a large majority (17) had a wrong initial working diagnosis. Symptoms and signs were variable. No blood investigation was specific. A computed tomography scan was generally better than ultrasound in detecting the perforation. All our cases were managed operatively with no mortality and a mean duration of hospital stay of 6.8 days. Most perforations were extra-hepatic (84%) and those of Niemeier’s type I (52.2%). Because of its varied clinical presentation, gallbladder perforation is often an intra-operative diagnosis, but early intervention carries a good outcome.


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