Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen

Leung, Ling; Leung, W. Y.; Chan, Kwong-Shun; Fan, TW; Chung, K. W.; Chan, C. H.

doi:10.1007/s00383-002-0782-7

Cited by 13 publications

(3 citation statements)

References 6 publications

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“…Because of tumor friability, rupture of the capsule with resultant tumor spillage is frequent (approximately 20% of initial resections and 43% of resections after recurrence) 51,53. There have been anecdotal reports of ruptured ACT as a cause of acute abdomen in children 57. When the diagnosis of ACT is suspected, laparotomy and a curative procedure are recommended rather than fine‐needle aspiration, to avoid the risk of tumor rupture 58.…”

Section: Treatment Of Adrenocortical Tumorsmentioning

confidence: 99%

Biology, clinical characteristics, and management of adrenocortical tumors in children

Rodríguez‐Galindo

Figueiredo

Zambetti

et al. 2005

Pediatric Blood & Cancer

129

104

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Childhood adrenocortical tumors (ACT) are very aggressive endocrine neoplasms whose incidence is quite low. Little is known about their pathogenesis, clinical presentation, and optimal treatment. In recent years, however, new information has been derived from the International Pediatric Adrenocortical Tumor Registry (IPACTR), and new clues to its pathogenesis have emerged. To provide an overview of the available data that may apply to pediatric ACT, we reviewed the epidemiology, pathogenesis, and treatment of ACT in adults and in children. Germline TP53 mutation is almost always the predisposing factor in childhood ACT. A unique germline mutation (TP53-R337H) has been described in Southern Brazil, where the incidence of ACT is 10-15 times the general incidence. Childhood ACT typically present during the first 5 years of life and has female predominance. Hormone hyperproduction is almost universal, and most patients present with virilization. Two-thirds of patients have resectable tumors. Surgery is the definitive treatment for ACT, and a curative complete resection should always be attempted. Cisplatin-based chemotherapy with mitotane is indicated for unresectable or metastatic disease, although its impact on overall outcome is slight. In childhood ACT, age, tumor size, and tumor resectability are the most important prognostic indicators. Outcome is stage-dependent; patients with small, resectable tumors have survival rates in excess of 80%, whereas the outcome for patients with unresectable disease is dismal. Patients with large, resectable tumors have an intermediate outcome. Childhood ACT are rare, but their unique epidemiology appear to implicate novel oncogenic pathways that are unique to the pediatric population. Multi-institutional and prospective studies are necessary to further our understanding of the pathogenesis and to improve outcomes.

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Section: Treatment Of Adrenocortical Tumorsmentioning

confidence: 99%

Biology, clinical characteristics, and management of adrenocortical tumors in children

Rodríguez‐Galindo

Figueiredo

Zambetti

et al. 2005

Pediatric Blood & Cancer

129

104

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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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“…Spontaneous rupture or infarction of a large adrenal mass is the logical prerequisite for such phenomenon. A limited number of cases, less than ten to our knowledge, exhaust the available literature on the subject [4–10]. In the present study we present the rare case of a young female that underwent emergency laparotomy due to signs of acute abdomen and concomitant cardiovascular collapse caused by a spontaneously ruptured large adrenocortical carcinoma.…”

Section: Introductionmentioning

confidence: 94%

Adrenocortical Carcinoma Presenting with Signs of Acute Abdomen

Symeonidis

Chatzinikolaou

Koukoulis

et al. 2013

Case Reports in Surgery

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Background. Adrenocortical carcinomas represent rare malignancies. In cases of hormone-secreting tumors, the hormone in excess determines the nearly diagnostic clinical presentation. Biologically inert tumors are diagnosed either due to the mass effect or incidentally. The purpose of the present study was to present an extremely rare presentation pattern of adrenocortical carcinoma. Case Presentation. We present the case of a 35-year-old female patient that underwent emergency laparotomy due to signs of acute abdomen and concomitant cardiovascular collapse caused by a spontaneously ruptured large adrenocortical carcinoma. Conclusion. Spontaneous rupture of an adrenocortical carcinoma presenting with signs of acute abdomen is an extremely rare clinical scenario. Increased level of suspicion is essential in order to diagnose and treat timely this life-threatening complication.

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Ruptured adrenocortical carcinoma as a cause of paediatric acute abdomen

Cited by 13 publications

References 6 publications

Biology, clinical characteristics, and management of adrenocortical tumors in children

Biology, clinical characteristics, and management of adrenocortical tumors in children

Spontaneously metachronous ruptures of adrenocortical carcinoma and its contralateral adrenal metastasis

Adrenocortical Carcinoma Presenting with Signs of Acute Abdomen

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