Cases reported "Adrenocortical Carcinoma"

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1/27. 11-Deoxycorticosterone-producing adrenocortical carcinoma.

    A woman presented with a history of weight loss and muscle weakness. A laboratory test revealed hypokalemia and elevation of plasma 11-deoxycorticosterone (DOC). CT showed a left adrenal mass. A left adrenalectomy was performed. The histological and immunohistochemical diagnosis showed a DOC-producing adrenocortical carcinoma. This cancer is very rare; only 10 cases including the present case have appeared in the literature.
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2/27. Two cases of osteosarcoma occurring as second malignancy of childhood cancer.

    We report on two patients in whom osteosarcoma occurred as second malignancy of childhood cancer. One patient had a malignant teratoma and the other adrenocortical carcinoma as the primary cancer. The emergence of cancer in cured cases and long-term survival cases of childhood cancer may result in an increase in the number of osteosarcomas seen in adolescence occurring as second malignancy. Anti-cancer drugs in large does were used for the treatment of a malignant teratoma in the former. These anti-cancer drugs may be involved in the occurrence of the second malignancy. In the latter, the patient has the germ-line mutation of p53 tumor suppressor gene, so genetic factors are presumably related to the occurrence of the second malignancy.
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3/27. adrenocortical carcinoma with cerebral metastasis in a child: case report and review of the literature.

    OBJECTIVE AND IMPORTANCE: adrenocortical carcinoma (ACC) is rare in the pediatric population, and brain metastasis seldom occurs. CLINICAL PRESENTATION: The authors report a case of metastatic ACC to the brain in a 9-year-old patient who had an adrenal cortex neoplasm removed at 4 years of age, and was free of symptoms for 5 years. Two weeks before admission she complained of blurred vision in both eyes. INTERVENTION: Examination revealed bilateral papilledema, and a magnetic resonance imaging (MRI) of the brain revealed a mass in the left lateral ventricle with extensive vasogenic edema and hydrocephalus. The tumor was removed, and histopathologic examination demonstrated metastatic ACC. CONCLUSION: Although ACC is a rare neoplasm it must be considered in the differential diagnosis of cerebral lesions in patients with a history of this tumor. Periodic long-term brain imaging is suggested as part of the follow up in patients with adrenocortical neoplasms.
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keywords = neoplasm
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4/27. Fine needle aspiration cytology of metastatic hepatic adrenocortical carcinoma mimicking hepatocellular carcinoma: a case report.

    BACKGROUND: adrenocortical carcinoma (AC) is a rare neoplasm, usually considered one of the most morbid and lethal human tumors. It occurs primarily in children and young adults and often presents with advanced and/or metastatic disease. CASE: A 9-year-old boy with a previous diagnosis of adrenocortical carcinoma underwent computed tomography (CT)-guided fine needle aspiration (FNA) for preoperative investigation of a hepatic mass. All smears revealed abundant groups of cells surrounding an interconnective, thin-walled, central vascular core. These cells showed finely vacuolated, large cytoplasm with eccentrically placed nuclei. Occasionally, cells underwent a sudden, marked increase in size, with prominent atypia. Multinucleated, atypical giant cells and high mitotic rate were also evident. The cytologic findings resembled the previous histologic adrenocortical carcinoma features. CONCLUSION: The cytologic features of metastatic hepatic adrenocortical carcinoma may mimic those of hepatocellular carcinoma. However, the presence of atypical multinucleated and pleomorphic cells with microvacuolated cytoplasm and eccentric nuclei as well as the absence of naked nuclei and endothelial linings yield the diagnosis of adrenocortical carcinoma. Nevertheless, other space-occupying liver lesions in children must also be considered. This case demonstrates the usefulness of CT-localized FNA biopsy in hepatic masses in children, and discusses the possible cytologic differential diagnosis.
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ranking = 0.56239638498756
keywords = neoplasm
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5/27. Coincident choroid plexus carcinoma and adrenocortical carcinoma with elevated p53 expression: a case report of an 18-month-old boy with no family history of cancer.

    We describe a young patient with no known family history of cancer who presented at 18 months with 2 advanced primary tumors, choroid plexus carcinoma and adrenal cortical carcinoma. Immunohistochemical studies demonstrated high levels of nuclear p53 protein expression in both tumors, as well as in the adjacent normal-appearing adrenal cortical cell nuclei of the adrenal gland. The immunohistologic distribution of elevated p53 expression suggests that this individual has a de novo germline mutation affecting p53 gene expression.
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keywords = cancer
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6/27. Oncocytic adrenocortical carcinoma: a morphologic, immunohistochemical and ultrastructural study of four cases.

    We present the clinical, histologic, immunohistochemical, and ultrastructural findings of four cases of non-functioning oncocytic adrenocortical carcinomas. The patients' ages ranged from 39 to 71 years. There was no sex predilection. Large yellow-tan tumors (8.5 to 17.0 cm), well demarcated from the adjacent kidney, were seen with a thin rim of normal adrenal gland along one edge. One tumor invaded the inferior vena cava and extended up to the level of the right atrium, and another metastasized to bone. The other two tumors had similar morphologic features and therefore were considered carcinomas. Histologic sections of all four cases showed a diffuse proliferation of polygonal neoplastic cells with large nuclei containing prominent nucleoli and abundant granular and eosinophilic cytoplasm. Occasional mononuclear and binucleated giant cells were noted in one case. There were rare mitotic figures (less than one per 10 high power fields). All tumors were immunoreactive for cytokeratins (AE1/AE3 and CAM5.2). Inhibin was focally expressed by one tumor and its bone metastasis. Ultrastructurally, the cytoplasm of the neoplastic cells was packed with innumerable mitochondria. Cytologic atypia or mitotic rate cannot reliably predict the biologic behavior of oncocytic adrenocortical neoplasms. Large tumor size (4/4), extracapsular extension (3/4), blood vessel invasion (2/4), necrosis (4/4), and metastasis (1/4) are features of malignancy for oncocytic adrenocortical carcinomas. The treatment of these tumors is complete surgical excision.
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ranking = 0.56239638498756
keywords = neoplasm
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7/27. Non-functioning adrenal cortical carcinoma presenting with metastasis to the tongue.

    We report a case of non-functioning adrenal cortical carcinoma (ACC) presenting with metastatic disease to the tongue, which is an extremely uncommon onset for this neoplasm. Histologically, the lesion had the appearance of an anaplastic neoplasm, and a panel of immunohistochemical markers including vimentin, MART-1, S100 protein, HMB-45, smooth muscle actin, common muscle actin, desmin, CD31, CD34, CD68, EMA and cytokeratins, was helpful in excluding melanoma, as well as other mesenchymal and epithelial neoplasms.
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ranking = 1.6871891549627
keywords = neoplasm
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8/27. Successful long-term disease-free survival following multimodal treatments in a patient with a repeatedly recurrent refractory adrenal cortical carcinoma.

    A 47-year-old male patient underwent surgery for a 10-cm adrenal cortical carcinoma. A large invasive adrenal mass was surgically removed en bloc with the right kidney and the lower lobe of the liver. Two months postoperatively, a 7-cm recurrent mass developed in the right psoas muscle. After a partial response was achieved by irradiation (40 Gy) and high-dose chemotherapy (carboplatin and etoposide) with peripheral blood stem cell transplantation, the patient underwent surgery with a wide excision of the psoas muscle. Twelve months after the initial surgery, an 8-cm rib metastasis developed and the patient again underwent surgery after a combination of irradiation (50 Gy) and chemotherapy (cisplatin and etoposide). The patient has been doing well without any evidence of recurrence for 5 years. Refractory or metastatic adrenal cortical carcinomas have been thought to be lethal, therefore, the present case provides support for multimodal treatments of refractory adrenocortical cancers.
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keywords = cancer
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9/27. Chance diagnosis of low stage non-metastasized adrenal cortical carcinoma in a young woman with retroperitoneal hemorrhage.

    Adrenal cortical carcinoma (ACC), a rare and highly malignant neoplasm of the cortical tract of the adrenal gland, is usually diagnosed at an advanced stage of development and often when metastatic spread already has begun. We report a very rare case of low-stage, non-metastasized ACC, the first clinical appearance of which was a large retroperitoneal hemorrhage caused by the rupture of the neoplastic mass. To the best of our knowledge, this is the third reported case of ACC rupture.
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ranking = 0.56239638498756
keywords = neoplasm
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10/27. Unusual double primary neoplasia: adrenocortical and ureteral carcinomas in werner syndrome.

    Adrenocortical and ureteral carcinomas were observed in a 50-year-old Japanese woman with werner syndrome (MIM No. 27770). The syndrome is an autosomal recessive disorder characterized by premature aging and an increased risk of rare cancers, which are often multiple. This is the first reported association of adrenocortical carcinoma in werner syndrome.
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