Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome

Wright, Jesse P.; Montgomery, Kathleen W.; Tierney, Joshua; Gilbert, Jill; Solórzano, Carmen C.; Idrees, Kamran

doi:10.1007/s10689-017-0042-6

Cited by 13 publications

(15 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Ectopic ACCs, presumably arise from ectopic adrenal rests, have been reported in the retroperitoneum [42], ovary [43,44], spinal region [45], liver [46] and abdominal wall [47]. A case of retroperitoneal ACC was noted in 68-year-old man with Lynch syndrome [48].…”

Section: Pathologymentioning

confidence: 99%

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

Lam

2021

Biomedicines

View full text Add to dashboard Cite

Adrenocortical carcinoma (ACC) is a heterogenous group of diseases with different clinical behaviour between adult and paediatric patients. In addition, three histological variants, oncocytic, myxoid and sarcomatoid are noted on the recent World Health Organisation (WHO) classification of ACC. A review of recent literature showed that the different types of ACC have distinctive demographic data, clinical presentation, pathology, biological behaviour, genomic and patients’ prognosis. In addition, recent updates of pathology staging for ACC allow refinement of prognostic grouping for planning treatment of the patients with ACC. These advances in genomic, pathology and staging have driven the development of standardisation of pathology reporting. International standardisation of pathological reporting of adrenocortical carcinoma and adaption to local pathology communities provide universal platforms for clinicians and researchers involved in the management of patients with ACC. To conclude, all these advances in the field of pathology will improve development of management strategies including improvement of clinical care, development of prognostic markers and testing of novel therapeutic approaches for patients with adrenocortical carcinoma.

show abstract

Section: Pathologymentioning

confidence: 99%

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

Lam

2021

Biomedicines

View full text Add to dashboard Cite

show abstract

“…In this study, five of 114 patients with ACC and LS had MSH2 (in three patients), MSH6 (one patient), and MLH1 (one patient) germline mutations, with four patients demonstrating microsatellite stability. More recently, two more case reports published in 2016 and 2018 [13, 14] again showed association between LS and ACC with MSH2 germline mutation.…”

Section: Discussionmentioning

confidence: 97%

Adrenal Cortical Carcinoma Associated With Lynch Syndrome: A Case Report and Review of Literature

Kaur

Pichurin

Hines

et al. 2019

Journal of the Endocrine Society

View full text Add to dashboard Cite

Objective Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. ACC was reported in 3.2% patients with Lynch syndrome (LS), however no particular case-detection strategies have been recommended. Participants We report a case of a 65-year-old woman who was incidentally discovered with a large adrenal mass during work-up of postmenopausal uterine bleeding. She was recently diagnosed with MSH6 germline mutation after her sister presented with uterine carcinoma in the setting of LS. Results Whereas the patient was asymptomatic for overt hormonal excess, biochemical work-up confirmed glucocorticoid autonomy and androgen and estrogen excess. Urine steroid profiling was suggestive of ACC. Adrenalectomy confirmed an oncocytic ACC with focal extracapsular extension into the periadrenal adipose tissue with a Ki-67 of 15% and a peak mitotic count of 40/50 high-power fields. Conclusion ACC can be the only manifestation of LS. A best case-detection approach for ACC in the asymptomatic patient with LS is unclear, however urine steroid profiling could be considered.

show abstract

“…Approximately 40% cases of ACC are nonfunctional ( 3 ), with nonfunctional ectopic ACC being even rarer. Ectopic ACC is usually found in the genitourinary system and abdominal cavity ( 5 , 6 , 8 – 10 , 12 , 18 – 20 ) and occasionally found in the nervous system ( 8 ). However, to the best of our knowledge, ectopic ACC has not been reported in the lungs.…”

Section: Discussionmentioning

confidence: 99%

“…Nonfunctional ACC generally occurs in older adults and exhibits a rapidly worsening course ( 6 ). Ectopic ACC is a condition in which ACC appears in locations other than the adrenal glands, usually in the genitourinary system [ovary ( 10 )] and abdominal cavity [abdominal wall ( 6 ), liver ( 4 ) and retroperitoneum ( 11 , 12 )] and occasionally in the nervous system ( 8 ). However, to the best of our knowledge, ectopic ACC has not been reported in the lungs.…”

Section: Introductionmentioning

confidence: 99%

Nonfunctional ectopic adrenocortical carcinoma in the lung: A case report and literature review

et al. 2023

View full text Add to dashboard Cite

BackgroundEctopic adrenocortical tissues and neoplasms are rare and usually found in the genitourinary system and abdominal cavity. The thorax is an extremely rare ectopic site. Here, we report the first case of nonfunctional ectopic adrenocortical carcinoma (ACC) in the lung.Case presentationA 71-year-old Chinese man presented with vague left-sided chest pain and irritable cough for 1 month. Thoracic computed tomography revealed a heterogeneously enhancing 5.3 × 5.8 × 6.0-cm solitary mass in the left lung. Radiological findings suggested a benign tumor. The tumor was surgically excised upon detection. Histopathological examination using hematoxylin and eosin staining showed that the cytoplasm of the tumor cells was rich and eosinophilic. Immunohistochemical profiles (inhibin-a+, melan-A+, Syn+) indicated that the tumor had an adrenocortical origin. The patient showed no symptoms of hormonal hypersecretion. The final pathological diagnosis was non-functional ectopic ACC. The patient was disease-free for 22 months and is still under follow-up.ConclusionsNonfunctional ectopic ACC in the lung is an extremely rare neoplasm that can be easily misdiagnosed as primary lung cancer or lung metastasis, both preoperatively and on postoperative pathological examination. This report may provide clues to clinicians and pathologists regarding the diagnosis and treatment of nonfunctional ectopic ACC.

show abstract

Ectopic, retroperitoneal adrenocortical carcinoma in the setting of Lynch syndrome

Cited by 13 publications

References 14 publications

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

Adrenal Cortical Carcinoma Associated With Lynch Syndrome: A Case Report and Review of Literature

Nonfunctional ectopic adrenocortical carcinoma in the lung: A case report and literature review

Contact Info

Product

Resources

About