Adrenocortical Carcinoma Presenting with Signs of Acute Abdomen

Symeonidis, Dimitrios; Chatzinikolaou, Ioannis; Koukoulis, Georgios; Mamaloudis, Ioannis; Tepetes, Konstantinos

doi:10.1155/2013/132726

Cited by 8 publications

(7 citation statements)

References 12 publications

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“…Paraneoplastic manifestations of having ACC such as hypoglycaemia (due to insulin growth factor 2 [IGF2] production) [ 23 ] and clinical manifestations related to adrenocorticotropic hormone ACTH production have been reported [ 24 ]. Rare manifestations of patients with ACC include cancer-related thrombotic microangiopathy [ 25 ] and tumour rupture with retroperitoneal haemorrhage [ 26 , 27 , 28 ].…”

Section: Conventional Adrenocortical Carcinomamentioning

confidence: 99%

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

Lam

2021

Biomedicines

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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.

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Section: Conventional Adrenocortical Carcinomamentioning

confidence: 99%

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

Lam

2021

Biomedicines

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“…Abdominal pain due to tumor growth may happen, but acute abdomen is a very rare presentation in ACC, although there are a few cases that reported acute abdomen as the presentation of pheochromocytoma [ 12 – 14 ]. ACC cases reported with acute abdomen seem much less frequent [ 15 , 16 ]. As far as we are aware, there are no reported cases of extra-adrenal ACC, which presented with an acute abdomen.…”

Section: Discussionmentioning

confidence: 99%

Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report

et al. 2020

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Background Adrenocortical carcinoma is a rare malignancy, with 43% being non-functional. These may arise from adrenal rest anywhere in the embryonic pathway of the adrenal glands. In the context of extra-adrenal and retroperitoneal tumors, the exact pathologic diagnosis is challenging. The case reported here, to the best of our knowledge, is the seventh reported case of extra-adrenal non-functional adrenocortical carcinoma. Case presentation We report a case of extra-adrenal non-functional adrenocortical carcinoma in a 15-year-old Persian boy who presented with an acute abdomen. He underwent surgical resection. Pathologic findings based on immunohistochemistry and cellular morphology confirmed adrenocortical carcinoma. He was treated with mitotane for 24 months. During a follow-up period of 30 months, no recurrence or metastases were found. Conclusion Despite the rarity of extra-adrenal adrenocortical carcinoma, presentation with an acute abdomen may occur, and the tumor may be found anywhere in the adrenal embryonic pathway. On the other hand, tumor behavior and prognosis in children may be different from what we expect in adults.

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“…Spontaneous rupture of an ACC is considered anecdotal since, in an extensive review of the literature, we have been able to retrieve only 15 previously described cases [6][7][8][9][10][11][12][13][14][15][16][17] ( Table 2). The present case being the 16th and, to our knowledge, the very first report of a spontaneous rupture of the primary cancer and a short-term rupture of the contralateral metachronous metastasis.…”

Section: Discussionmentioning

confidence: 99%

Spontaneously metachronous ruptures of adrenocortical carcinoma and its contralateral adrenal metastasis

Polistina

Farruggio²,

Gasparin³

et al. 2015

Int Canc Conf J

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Adrenocortical carcinomas (ACCs) are rare neoplasms. In spite of its rarity, ACCs are the second most lethal endocrine cancer after anaplastic thyroid carcinomas. Currently, the only chance for a cure is an early diagnosis and a radical surgical resection. We present the case of a previously unreported bilateral adrenal hemorrhage occurring in a 59-year-old Caucasian male who was admitted to our surgical division with the diagnosis of a right retroperitoneal spontaneous hemorrhage. Imaging revealed a 10-cm ruptured right adrenal mass with no other abdominal lesions, endocrine screening results were normal, and a right adrenalectomy was performed. Pathology revealed a ruptured ACC. The postoperative period was uneventful and the patient was discharged. While recovering, 3 weeks after the operation, the patient showed the same symptoms on the contralateral side. Imaging once again revealed a retroperitoneal hemorrhage due to a 5-cm ruptured left adrenal mass. Endocrine screening showed a frank peripheral hypercortisolism and imaging showed a huge metastatic dissemination to the liver, lungs, and retroperitoneal space. An urgent left adrenalectomy was performed and pathology showed a metastatic ruptured ACC. The patient was placed in substitutive therapy but never recovered and died of penta lobar pneumonia on postoperative day 31. An extensive review of the current literature on the issue was performed. ACC is confirmed to be a lethal cancer. Rupture is the rarest clinical presentation and appears to be caused by the tumor's growth rate more than the tumor dimensions itself. The use of endocrine screening on such hemodynamically unstable patients is questionable.

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Adrenocortical Carcinoma Presenting with Signs of Acute Abdomen

Cited by 8 publications

References 12 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

Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report

Spontaneously metachronous ruptures of adrenocortical carcinoma and its contralateral adrenal metastasis

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