Ectopic adrenocortical carcinoma located in the ovary

Chentli, F.; Terki, Nadia; Azzoug, S.

doi:10.1530/eje-16-0224

Cited by 15 publications

(18 citation statements)

References 30 publications

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“…All of the above is consistent with the malignant nature of this tumor. Immunohistochemical studies confirmed the tumor cells' steroidogenic capability as they were immunopositive for calretinin, inhibin, vimentin, melanoma antigen recognized by T cells-1, and synaptophysin (5,7). Immunohistochemical studies were also useful to differentiate the tumor from renal cell carcinoma as the tumor was immunonegative for cytokeratin and CD 10, markers that are found in renal cell carcinoma (7).…”

Section: Discussionmentioning

confidence: 79%

“…Tumors arising from adrenal remnants are usually scarce. They are often benign and non-functioning (5). Primary ACC is also rare with an incidence of 0.5 to 2 per million annually (3).…”

Section: Discussionmentioning

confidence: 99%

“…Primary ACC is also rare with an incidence of 0.5 to 2 per million annually (3). Malignant transformation in adrenal rest tissue is extremely rare and those secreting cortisol or androgens are even rarer (5). The incidence and follow-up are totally unknown.…”

Section: Discussionmentioning

confidence: 99%

“…Searching PubMed and Google Scholar, we found only 15 ectopic ACC cases (1,4,5,(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19). The demographic, clinical, pathologic, and follow-up data of these cases as well as our index case are summarized in Table 2.…”

Section: Discussionmentioning

confidence: 99%

“…The treatment of ectopic ACC does not seem to differ from that of eutopic tumors (5). Surgery is the cornerstone.…”

Section: Discussionmentioning

confidence: 99%

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Ectopic Cortisol and Androgen-Producing Adrenocortical Carcinoma Arising from Adrenal Rest Tissue in a Kidney: Case Report and Literature Review

Kim

Anaedo

Nozad

et al. 2018

AACE Clinical Case Reports

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Objective: To present a case of an aggressive adrenocortical carcinoma producing both ectopic cortisol and androgens and to provide an extensive literature review of similar cases. Methods: A case presentation is provided that illustrates clinical, biochemical, radiologic, and pathologic findings. We also conducted a PubMed search for similar cases and summarize 15 other cases of malignant adrenal rest tissue. Results: A 50-year-old woman presented with symptoms of Cushing syndrome and virilization. Urine free cortisol and serum adrenal androgens were significantly elevated. Computed tomography of the abdomen revealed a 12-cm mass within the right kidney. Subsequently, the patient underwent right radical nephro-adrenalectomy. The tumor showed a high-grade neoplasm centered in the kidney with polymorphism, highly atypical nuclei, abundant mitotic activity, and necrosis. The Ki-67 proliferation index was 25 to 30%. Immunohistochemical staining of the neoplastic cells favored an adrenal origin. The native adrenal gland was atrophic and free of the tumor. Postoperatively, cortisol and adrenal androgen levels became undetectable. However, her disease unfortunately recurred 5 months later with innumerable peritoneal, mesenteric, omental, and serosal implants within the abdomen and pelvis. She was started on mitotane and mifepristone but unfortunately expired within a few days from a septic shock secondary to Pseudomonas aeruginosa pneumonia. Conclusion: We present an unusual case of a functional adrenocortical carcinoma arising in adrenal rest tissue in a kidney resulting in Cushing syndrome and virilization with very aggressive biology. To the best of our knowledge, no similar case has been reported thus far.

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Section: Discussionmentioning

confidence: 79%

“…Tumors arising from adrenal remnants are usually scarce. They are often benign and non-functioning (5). Primary ACC is also rare with an incidence of 0.5 to 2 per million annually (3).…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…The treatment of ectopic ACC does not seem to differ from that of eutopic tumors (5). Surgery is the cornerstone.…”

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Ectopic Cortisol and Androgen-Producing Adrenocortical Carcinoma Arising from Adrenal Rest Tissue in a Kidney: Case Report and Literature Review

Kim

Anaedo

Nozad

et al. 2018

AACE Clinical Case Reports

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Adrenal Rests in the Uro-genital Tract of an Adult Population

et al. 2021

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Ectopic adrenal rests are a rare condition which can be found in various sites, generally in the retroperitoneum or pelvis along the path of gonadal descent. Their real prevalence is unknown. Males are more commonly affected, at least in the pediatric age. Adrenal rests are usually clinically silent and incidentally found in surgical samples, mostly in the pediatric population, and rarely in adults. With the aim of increasing knowledge and estimating the prevalence of ectopic adrenocortical tissue in the adult population, 44 adrenal rests in the urogenital tract of 40 adults are described. These represent approximately 0.07% of the total number of urogenital and gynecological surgeries performed in the 22 considered years. Adrenal rests were identified in the spermatic cord (10 males) and in paraovarian, parasalpingeal, or infundibulopelvic ligament locations (30 females). All but one was incidental findings. One case regarded an adrenocortical carcinoma arisen in adrenal rests. A literature review of adrenal ectopia in the urogenital tract of adults identified 57 reported cases from 53 patients, with similar clinicopathological features as those of our series, with the exception of a lower incidence of parasalpingeal locations. Despite their limited clinical implications, awareness of ectopic adrenal rests is essential also in adults for at least two reasons: (a) to correctly identify sources of adrenocortical hormone production in case of adrenal insufficiency or hormonal imbalance and (b) to avoid misinterpretations in the diagnostic workup of renal cell carcinoma, adrenocortical tumors, and rare gonadal neoplasms, including Sertoli/Leydig cell tumors.

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Multiple hormone secretion may indicate worse prognosis in patients with ectopic Cushing’s syndrome

et al. 2020

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Purpose Ectopic Cushing’s syndrome (ECS) caused by an ACTH secreting neuroendocrine neoplasm (NEN) is a rare and challenging condition. We aimed to detect predictive and prognostic parameters for ECS patients identified from a retrospective, comprehensive cohort of NENs treated at a tertiary referral center. Methods Medical records of 886 patients with NENs were reviewed. We identified 51 patients with ECS (33 females/18 men); mean age 52 ± 15 years (SD). Clinical parameters including symptoms, biochemical markers, and survival were extracted and further analyzed. Results The primary tumor was located in the thorax ( n = 28) or pancreas ( n = 15) or was of unknown primary origin ( n = 8). In 30 patients, tumor and ECS were diagnosed simultaneously. In 12 patients, the NEN diagnosis preceded ECS development, with a median time of 43.5 months (range: 9–96), and 10 of these showed radiological tumor progression at ECS diagnosis. Twenty-one patients had multiple hormone secretion, which correlated with shorter overall survival (OS), p = 0.012 (HR 2.4 (95% CI 1.2–4.9)), as did high morning cortisol, p = 0.037 (HR 2.3 (1.0–5.2)), higher tumor grade, p = 0.044 (HR 2.3 (1.0–5.1)), and diabetes, p = 0.050 (HR 2.4 (1.0–6.0)). Conclusions Multiple hormone secretion, high morning cortisol, higher tumor grade, and diabetes were correlated with shorter OS. Development of ECS in patients with a non-functioning NEN may indicate tumor progression. Multiple hormone secretion should be considered as a bad prognostic sign in ECS patients and should lead to intensified clinical management.

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Ectopic adrenocortical carcinoma located in the ovary

Cited by 15 publications

References 30 publications

Ectopic Cortisol and Androgen-Producing Adrenocortical Carcinoma Arising from Adrenal Rest Tissue in a Kidney: Case Report and Literature Review

Ectopic Cortisol and Androgen-Producing Adrenocortical Carcinoma Arising from Adrenal Rest Tissue in a Kidney: Case Report and Literature Review

Adrenal Rests in the Uro-genital Tract of an Adult Population

Multiple hormone secretion may indicate worse prognosis in patients with ectopic Cushing’s syndrome

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