Virilizing Adrenocortical Carcinoma Resected With Negative Margins Treated With Surgery Alone

2021 ◽  
pp. 000313482110508
Author(s):  
Tyler Fields ◽  
Kelsee Felux ◽  
Alejandro Chavarriaga ◽  
Philip Ramsay ◽  
Evan Weitman
Keyword(s):  
Menstrual Cycle ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Rare Case ◽  
Physical Exam ◽  
Rare Malignancy ◽  
Uncommon Tumor

Adrenocortical carcinoma is a rare malignancy. A virilizing adrenocortical carcinoma is even more unique of a diagnosis. In this report, we present a rare case of this uncommon tumor with an interesting presentation, clearly documented physical exam changes over a span of at least 8 years, and a technically challenging case. We also briefly review the management of adrenocortical carcinoma. The tumor was successfully resected with no planned adjuvant treatment at this time. The patient had recurrence of menstrual cycle post-operatively and required no steroid supplementation.

scholarly journals Paediatric Nonfunctioning Adrenocortical Carcinoma with Extension up to Right-Side Heart: Cardiac Surgery Approach

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Federica Iezzi ◽  
Andrea Quarti ◽  
Chiara Surace ◽  
Marco Pozzi
Keyword(s):  
Cardiac Surgery ◽  
Adrenal Gland ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Rare Case ◽  
Cardiac Involvement ◽  
Adrenal Carcinoma ◽  
Rare Malignancy ◽  
The Right ◽  
Treatment Prognosis

Adrenocortical carcinoma is a rare malignancy. Due to late diagnosis and no adequate effective adjuvant treatment, prognosis remains poor. Only approximately 30% of these malignancies are confined to the adrenal gland when they are diagnosed, as these tumors tend to be found years after their genesis. Cardiac involvement of adrenal carcinoma is very rare. We report a rare case of a 7-year-old female with right adrenal cortical carcinoma, involving the right-side heart.

scholarly journals Pathobiology and genetics of adrenocortical carcinoma

2019 ◽  
Vol 62 (2) ◽  
pp. R105-R119 ◽  
Author(s):  
James F H Pittaway ◽  
Leonardo Guasti
Keyword(s):  
Adrenal Cortex ◽  
Adjuvant Treatment ◽  
Adrenocortical Carcinoma ◽  
Genetic Alterations ◽  
High Recurrence Rate ◽  
Self Renewal ◽  
Steroidogenic Cells ◽  
Rare Malignancy ◽  
Definition Of ◽  
Stem Cell Hierarchy

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence worldwide of 0.7–2.0 cases/million/year. Initial staging is the most important factor in determining prognosis. If diagnosed early, complete surgical resection +/− adjuvant treatment can lead to 5-year survival of up to 80%. However, often it is diagnosed late and in advanced disease, 5-year survival is <15% with a high recurrence rate even after radical surgery. The mainstay of adjuvant treatment is with the drug mitotane. Mitotane has a specific cytotoxic effect on steroidogenic cells of the adrenal cortex, but despite this, progression through treatment is common. Developments in genetic analysis in the form of next-generation sequencing, aided by bioinformatics, have enabled high-throughput molecular characterisation of these tumours. This, in addition to a better appreciation of the processes of physiological, homeostatic self-renewal of the adrenal cortex, has furthered our understanding of the pathogenesis of this malignancy. In this review, we have detailed the pathobiology and genetic alterations in adrenocortical carcinoma by integrating current understanding of homeostasis and self-renewal in the normal adrenal cortex with molecular profiling of tumours from recent genetic analyses. Improved understanding of the mechanisms involved in self-renewal and stem cell hierarchy in normal human adrenal cortices, together with the identification of cell populations likely to be co-opted by oncogenic mutations, will enable further progress in the definition of the molecular pathways involved in the pathogenesis of ACC. The combination of these advances eventually will lead to the development of novel, effective and personalised strategies to eradicate molecularly annotated ACCs.

A rare case of high-grade chondroblastic osteosarcoma post-radiotherapy for a right frontal oligodendroglioma

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Sinead Horan ◽  
◽  
Oscar Breathnach ◽  
Liam Grogan ◽  
David Fitzpatrick ◽  
...  
Keyword(s):  
Radiation Exposure ◽  
Brain Tumour ◽  
Radiation Field ◽  
Rare Case ◽  
Previous History ◽  
High Grade ◽  
History Of ◽  
Rare Malignancy ◽  
Chondroblastic Osteosarcoma

This is the fourteenth case of an intracranial and dural-based osteosarcoma in the literature to date. The case involves a forty-year old woman with a previous history of a brain tumour, which was treated with surgery, chemotherapy and radiotherapy six years previously. The hypothesis is that this rare malignancy is secondary to the radiation exposure given the growth of the lesion in the previous radiation field

scholarly journals Case Report: Ectopic Adrenocortical Carcinoma in the Ovary

2021 ◽  
Vol 12 ◽  
Author(s):  
Wen-Hsuan Tsai ◽  
Tze-Chien Chen ◽  
Shuen-Han Dai ◽  
Yi-Hong Zeng
Keyword(s):  
Adrenocortical Carcinoma ◽  
Performance Status ◽  
Poor Performance ◽  
Abdominal Circumference ◽  
Poor Performance Status ◽  
Test Results ◽  
Ki 67 ◽  
Complete Survey ◽  
Rare Malignancy ◽  
Captopril Test

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. ACCs are rare and usually endocrinologically functional. We present the case of a 59-year-old woman who experienced abdominal fullness for 6 months and increased abdominal circumference. A large pelvic tumor was observed. She underwent cytoreductive surgery and the pathological test results revealed local tumor necrosis and prominent lympho-vascular invasion. Neuroendocrine carcinoma was the first impression, but positivity for synaptophysin, alpha-inhibin, transcription factor enhancer 3 (TFE-3), calretinin (focal), and CD56 (focal) and high Ki-67-labeling proliferating index (&gt;80%) confirmed the diagnosis of ectopic ACC. Ectopic primary aldosteronism could not be excluded. However, we did not perform saline infusion test or captopril test due to poor performance status. When pathological test reports reveal neuroendocrine features not typically found in the organ being examined, IHC staining should be performed to rule out ectopic ACC. Whether the ectopic ACC is functional or not requires complete survey.

A Rare Case of Stage IV Adrenocortical Carcinoma with Acute Adrenocortical Dysfunction After Trans-Catheter Arterial Chemo-Embolization (TACE)

2020 ◽  
Author(s):  
Zhilong Wang ◽  
Xin Sun ◽  
Fengli Zhang ◽  
Ting Wang ◽  
Ping Li
Keyword(s):  
Liver Metastasis ◽  
Adrenocortical Carcinoma ◽  
Poor Prognosis ◽  
Rare Case ◽  
Stage Iv ◽  
Postoperative Recurrence ◽  
Tumor Burden ◽  
Case Presentation ◽  
Embolization Therapy ◽  
New Treatment

Abstract Background: Adrenocortical carcinoma(ACC) is a rare and highly invasive endocrine malignant tumor with poor prognosis and insensitivity tochemotherapy, which don't have effective treatment. Although Surgical resection is considered to be the main treatment for ACC, postoperative recurrence and metastasis have become the most important factors of death. Therefore, local treatments such as trans-catheter arterial chemo-embolization, radiofrequency ablation become be new treatment for ACC. Trans-catheter arterial chemo-embolization therapy for ACC patients with liver metastasis has good efficacy and can effectively reduce the tumor burden of patients, which is considered to be safe and easy for patients. Case presentation: We report a 47-year-old female patient diagnosed with stage Ⅳ ACC with liver metastases, who developed symptoms of acute adrenocortical dysfunctions after hepatic arterial catheter chemo-embolization.Conclusion: Trans-catheter arterial chemo-embolization therapy for ACC patients with liver metastasis is not completely safe, and there is a certain probability that it will lead to secondary adrenal dysfunction. Hydrocortisone supplementation can effectively alleviate the symptoms.

scholarly journals Adjuvant Radiation Improves Recurrence-Free Survival and Overall Survival in Adrenocortical Carcinoma

2019 ◽  
Vol 104 (9) ◽  
pp. 3743-3750 ◽  
Author(s):  
Laila A Gharzai ◽  
Michael D Green ◽  
Kent A Griffith ◽  
Tobias Else ◽  
Charles S Mayo ◽  
...  
Keyword(s):  
Overall Survival ◽  
Adrenocortical Carcinoma ◽  
Adjuvant Radiation ◽  
Recurrence Free Survival ◽  
University Of Michigan ◽  
Free Survival ◽  
Tumor Bed ◽  
Distant Failure ◽  
Rare Malignancy ◽  
The University

Abstract Context Adrenocortical carcinoma (ACC) is a rare malignancy with high rates of recurrence and poor prognosis. The role of radiotherapy (RT) in localized ACC has been controversial, and RT is not routinely offered. Objective To evaluate the benefit of adjuvant RT on outcomes in ACC. Design This is a retrospective propensity-matched analysis. Setting All patients were seen through the University of Michigan’s Endocrine Oncology program, and all those who underwent RT were treated at the University of Michigan. Participants Of 424 patients with ACC, 78 were selected; 39 patients underwent adjuvant radiation. Intervention Adjuvant RT to the tumor bed and adjacent lymph nodes. Main Outcomes Measures Time to local failure, distant failure, or death. Results Median follow-up time was 4.21 years (95% CI, 2.79 to 4.94). The median radiation dose was 55 Gy (range, 45 to 60). The 3-year overall survival estimate for patients improved from 48.6% for patients without RT (95% CI, 29.7 to 65.2) to 77.7% (95% CI, 56.3 to 89.5) with RT, with a hazard ratio (HR) of 3.59 (95% CI, 1.60 to 8.09; P = 0.002). RT improved local recurrence-free survival (RFS) from 34.2% (95% CI, 18.8 to 50.3) to 59.5% (95% CI, 39.0 to 75.0), with an HR of 2.67 (95% CI, 1.38 to 5.19; P = 0.0035). RT improved all RFS from 18.3% (95% CI, 6.7 to 34.3) to 46.7% (95% CI, 26.9 to 64.3), with an HR 2.59 (95% CI, 1.40 to 4.79; P = 0.0024). Conclusions In the largest single institution study to date, adjuvant RT after gross resection of ACC improved local RFS, all RFS, and overall survival in this propensity-matched analysis. Adjuvant RT should be considered a part of multidisciplinary management for patients with ACC.

scholarly journals Functioning Adrenocortical Carcinoma with Extension upto the Right Atrium Producing Cushing's Syndrome

2013 ◽  
Vol 3 ◽  
pp. 32 ◽  
Author(s):  
Santosh Kumar ◽  
Gautam R. Choudhary ◽  
Arawat Pushkarna
Keyword(s):  
Inferior Vena Cava ◽  
Cushing’S Syndrome ◽  
Adrenocortical Carcinoma ◽  
Right Atrium ◽  
Inferior Vena ◽  
Vena Cava ◽  
Cushing's Syndrome ◽  
Curative Therapy ◽  
Rare Malignancy ◽  
The Right

Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Surgery is the only curative therapy available and overall 5-year survival for patients who undergo a complete resection is 32% to 48%. They are known to produce intravascular invasion and into the inferior vena cava (IVC) and in rare cases they may reach the right atrium. We report a case of functioning ACC extending into the inferior vena cava and right atrium in a female with Cushing's syndrome.

scholarly journals A rare case of oncocytic adrenocortical carcinoma clinically presented as an incidentaloma

2020 ◽  
Vol 67 (8) ◽  
pp. 883-888
Author(s):  
Ko Harada ◽  
Miho Yasuda ◽  
Yasuhiro Nakano ◽  
Kazuhiro Yoshida ◽  
Yuzo Umeda ◽  
...  
Keyword(s):  
Adrenocortical Carcinoma ◽  
Rare Case

scholarly journals Adrenocortical Carcinoma With Renal Vein Thrombus Extended to Inferior Vena Cava: A Case Report

2015 ◽  
Vol 100 (7-8) ◽  
pp. 1190-1193 ◽  
Author(s):  
Mohsen Ayati ◽  
Jafar Shahbazi ◽  
Ali Tehranchi ◽  
Elnaz Ayati ◽  
Yousef Rezaei
Keyword(s):  
Inferior Vena Cava ◽  
Adrenocortical Carcinoma ◽  
Renal Vein ◽  
Inferior Vena ◽  
Vena Cava ◽  
Venous Invasion ◽  
Survival Duration ◽  
Comprehensive Therapy ◽  
Advanced Stages ◽  
Rare Malignancy

Adrenocortical carcinoma (ACC) is a rare aggressive tumor. Renal vein and inferior vena cava (IVC) thrombi have been found as uncommon presentations of ACC; however, the implementation of comprehensive therapy has remained controversial in such cases. We report a case of a 46-year-old woman with a large ACC associated with the invasion of tumor to IVC confirmed by imaging and immunohistochemistry examinations. The patient was treated successfully using aggressive surgery, including adrenalectomy and thrombectomy adjunct to an adrenocorticolytic agent. However, she died of metastasis complications at 3-month follow-up period. ACC is a rare malignancy, mostly presenting in advanced stages with poor prognosis. Implementing aggressive surgical therapy might be effective for the management of such cases; however, the short survival duration in our case underscores the need for defining the precise therapy of metastatic ACC associated with venous invasion.

scholarly journals A Rare Case Report: A Malignant Histiocytic Tumor in the Form of Ovarian Mass

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Emsal Pinar Topdagi Yilmaz ◽  
Yakup Kumtepe ◽  
Yunus Emre Topdagi ◽  
Seray Kaya Topdagi
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Medical Oncology ◽  
Virgin Female ◽  
Reproductive Age ◽  
Ovarian Malignancy ◽  
Ovarian Mass ◽  
Histiocytic Cell ◽  
Rare Case Report ◽  
Rare Malignancy

Histiocytic cell malignancies are very rare. Hence, the information about this disease in hematology is limited. In this case report, we present a case of malignant histiocytic tumor affecting the ovary of a 40-year-old virgin female. Primary ovarian malignancy was not considered for the patient who was approached as if she had ovarian malignancy, since there was an indication of a mass in the ovary. Therefore, an aggressive surgery was not performed. Since our patient was in the reproductive age, fertility-preserving surgery was performed. Our patient was then treated systemically by medical oncology. In conclusion, the rare malignancy group was investigated in the present study along with an evaluation of the current literature.

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