Oncocytic Adrenal Cortical Carcinosarcoma With Pleomorphic Rhabdomyosarcomatous Metastases

Thway, Khin; Olmos, David; Shah, Chirag; Flora, Rashpal; Shipley, Janet; Fisher, Cyril

doi:10.1097/pas.0b013e31824517d9

Cited by 25 publications

(21 citation statements)

References 40 publications

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“…Malignant adrenal tumors, which include neuroblastoma, adrenocortical carcinoma [11] with its oncocytic variant [12] and a minority of adrenal pheochromocytomas, are extremely rare, with an incidence of 1-2 per million population annually.…”

Section: Incidencementioning

confidence: 99%

“…On the other side, adrenocortical carcinoma and its oncocytic variant is an aggressive cancer, and often invaded nearby tissues or metastasized [95] to distant organs at the time of diagnosis, with a poor 5-year survival rate (20-35%) [96] . The 5-year survival rate after successful surgery is 50-60%, but unfortunately, a large percentage of patients are not surgical candidates, and will present local relapse [97] .…”

Section: Prognosismentioning

confidence: 99%

See 1 more Smart Citation

Adrenal Oncocytic Neoplasm: A Systematic Review

Mearini¹,

Sordo

Costantini³

et al. 2012

Urol Int

102

151

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Introduction: Oncocytic neoplasms as tumors arising in the adrenal glands are rare, usually considered as nonfunctional and benign. In the current literature, there are extremely limited reports of adrenal oncocytic neoplasms; as to date, only 147 cases have been described. The rarity of the event prompted this study which reviews and presents the incidence, histology, diagnosis and therapy of adrenal oncocytic neoplasms. Materials and Methods: A review by systematic literature search was done using the MEDLINE®/Cochrane libraries from 1950 to date using the medical subject headings ‘oncocytoma', ‘adrenal gland', ‘adrenal oncocytoma', ‘adrenal oncocytic neoplasm' and ‘adrenal oncocytic carcinoma'. Results: Adrenal oncocytic neoplasm is a rare disease, usually incidentally detected because only 17% are functional adrenal masses. The typical oncocyte displays abundant granular eosinophilic cytoplasm, due to the accumulation of mitochondria. Computed tomography and magnetic resonance imaging are not able to identify or differentiate benign and malignant oncocytic neoplasms. The mainstay of therapy is adrenalectomy, recently performed by laparoscopy. The prognosis is good for benign tumors, while adrenocortical oncocytic carcinoma has a poor survival rate of only 5 years. Conclusions: Adrenal oncocytic neoplasm, a rare and mostly benign tumor, usually presents as an incidental, large adrenal mass; surgery is the mainstay of therapy, by means of laparoscopy which is now the most diffuse approach to adrenalectomy.

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Section: Incidencementioning

confidence: 99%

Section: Prognosismentioning

confidence: 99%

Adrenal Oncocytic Neoplasm: A Systematic Review

Mearini¹,

Sordo

Costantini³

et al. 2012

Urol Int

102

151

View full text Add to dashboard Cite

show abstract

“…Of these, approximately 36 cases of OACs have been documented in the literature 1. OANs consist predominantly of oncocytes (epithelial cells with abundant granular eosinophilic cytoplasm), with tumours showing diffuse, nested, or trabecular patterns of large, neoplastic polygonal oncocytic cells with round nuclei, prominent nucleoli and abundant granular eosinophilic cytoplasm 1 2…”

Section: Discussionmentioning

confidence: 99%

“…Oncocytic adrenocortical carcinoma (OAC) represents a rare subgroup of oncocytic adrenocortical neoplasms (OANs) with approximately 36 cases documented in the literature 1. With progressive research, new insights have emerged in the molecular profiling of these tumours.…”

Section: Introductionmentioning

confidence: 99%

Oncocytic adrenocortical carcinoma—a rare pathological variant

2015

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Oncocytic adrenocortical carcinoma is a rare histopathological variant of adrenocortical carcinoma with very few instances reported in the literature to date. With progressive research, new insights have emerged in the molecular profiling of these tumours. This advancement has led to more clarity in reporting of this tumour. We report a case of oncocytic adrenocortical carcinoma with its attending clinical presentation, immunohistochemical profiling and characteristic electron microscopy findings.

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“…(18). Only 13 cases of sarcomatoid ACC, including adrenocortical carcinosarcoma, have been reported to date (18192021222324252627282930). …”

Section: Introductionmentioning

confidence: 99%

Myxoid and Sarcomatoid Variants of Adrenocortical Carcinoma: Analysis of Rare Variants in Single Tertiary Care Center

et al. 2017

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The aim of this study is to describe rare variants of adrenocortical carcinoma (ACC) and to compare the prognosis with that of conventional ACC. We retrospectively reviewed 8 cases of myxoid variant, 1 sarcomatoid variant, and 14 cases of conventional ACC, who underwent surgical resection at the Asan Medical Center between 1996 and 2014. An analysis of the clinicopathological characteristics, including the Weiss score, Ki-67 labeling index, and reticulin framework assessment is presented. The mean age of patients with myxoid/sarcomatoid ACC was 45 years; 4 out of 9 patients were women. Mean primary tumor size was 12.9 cm and the mean weight was 702.4 g. Seven patients presented in an advanced stage (stage III/IV); 8 of these eventually developed distant metastasis. The mean Weiss score was 5.0 points and the Ki-67 labeling index was 15.6%. The extent of myxoid or sarcomatoid change on histological examination ranged from 10% to 75% of the examined tumor areas; reticulin framework alteration was observed in all cases. Four patients showed venous tumor thrombus. Most of the clinicopathological parameters were not significantly different from those of conventional ACC. However, myxoid or sarcomatoid variant (hazard ratios [HR], 3.59; 95% confidence intervals [CI], 1.13–11.38; P = 0.030) and Ki-67 labeling index (HR, 3.97; 95% CI, 1.18–13.41; P = 0.030) were independent predictors of overall survival after adjusting for age and sex. Myxoid or sarcomatoid histological features or an increased Ki-67 labeling index may be associated with poor overall survival in patients with ACC.

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Oncocytic Adrenal Cortical Carcinosarcoma With Pleomorphic Rhabdomyosarcomatous Metastases

Cited by 25 publications

References 40 publications

Adrenal Oncocytic Neoplasm: A Systematic Review

Adrenal Oncocytic Neoplasm: A Systematic Review

Oncocytic adrenocortical carcinoma—a rare pathological variant

Myxoid and Sarcomatoid Variants of Adrenocortical Carcinoma: Analysis of Rare Variants in Single Tertiary Care Center

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