mart-1-antigen and Adrenocortical-Carcinoma

The authors report a case of myxoid adrenal cortical carcinoma (ACC) clinically manifesting as primary hyperaldosteronism. The 82-year-old female patient had a history of hypertension and was sent to the emergency room because of change in consciousness. Ventricular fibrillation occurred, and severe hypokalemia was found. Increased renal loss of potassium, high serum aldosterone level, low renin activity, and a huge tumor in the left suprarenal area were revealed when tests were conducted to determine the cause of her hypokalemia. Left adrenalectomy was performed. The tumor measured 13 cm in diameter and showed a heterogeneous cut surface with gelatinous material. Microscopically, the lesion was composed of polygonal cells with eosinophilic cytoplasm and arranged in arborizing cords in a myxoid background. Capsular and vascular invasion were observed. The tumor stained positive for synaptophysin, melan-A, vimentin, and α-inhibin but negative for cytokeratin. A primary myxoid ACC was diagnosed, which is a rare histological variant. The authors review 13 other reported cases. Most of these were functional tumors causing Cushing syndrome, and only 2 cases presented as primary hyperaldosteronism. All cases had similar microscopic and immunohistochemical features. Distal metastases and local recurrence were not uncommon. Close clinical follow-up is imperative.

Topics: Adrenal Cortex Neoplasms; Adrenalectomy; Adrenocortical Carcinoma; Aged, 80 and over; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Hyperaldosteronism; Inhibins; MART-1 Antigen; Mucins; Synaptophysin; Treatment Outcome; Vimentin

Adrenocortical carcinoma (ACC) is an uncommon primary cancer in the adrenal gland. Its incidence of showing metastasis in the head and neck region is very rare. Herein, we present a case of a 46-year-old man who presented with complaints of pain and numbness on the left side of the lower face for 4 months. Radiographic examination revealed an osteolytic lesion with an ill-defined border in the left body region of the mandible. Histopathologic examination revealed a tumor composed of sheets of oval to polygon-shaped tumor cells predominantly displaying abundant eosinophilic granular cytoplasm. These tumor cells showed features of a high degree of anaplasia. On immunohistochemical examination, tumor cells were focally positive for synaptophysin, inhibin, vimentin, pancytokeratin (pan-CK), cytokeratin (CK)5/6, CD68, and CK8/18 and immunonegative for CK7, chromogranin, melan-A, S100, SMA, and SATB2. The Ki-67 proliferation index was approximately 20%. To the best of our knowledge, this is the first case of metastatic oncocytic ACC to the oral cavity region.

Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Biomarkers, Tumor; Chromogranins; Humans; Inhibins; Keratins; Ki-67 Antigen; Male; Mandible; MART-1 Antigen; Middle Aged; Synaptophysin; Vimentin

We report two cases of patients presenting myxoid variant of adrenocortical carcinoma (ACC). This very rare variant is characterized by a tumoral proliferation organized in trabeculae, cords or even pseudo-glands within an acellular myxoid materiel stained by Alcian Blue and negative for PAS. Tumor cells have a small to medium size and have little atypia. Their immunohistochemical profil (positivity of Synaptophysin, SF1, Melan A, Vimentin and Inhibin, with a weak or negative pancytokeratin expression) eliminate the main differential diagnoses (metastasis of a myxoid adenocarcinoma and soft tissue myxoid tumor). Many scoring systems have been proposed in order to evaluate the risk of malignancy of these lesions: the Weiss score seems less efficient to evaluate malignancy in this variant than the reticulinic algorithm or the Helsinki score. Prognosis of myxoid variant of ACC seems worse than classical ACC.

Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Biomarkers, Tumor; Humans; MART-1 Antigen; Prognosis; Vimentin

Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Adult; Aged; Antibodies, Monoclonal, Murine-Derived; Biomarkers, Tumor; Female; Humans; Inhibins; Ki-67 Antigen; Male; MART-1 Antigen; Matrix Metalloproteinase 9; Middle Aged; Prognosis; Retrospective Studies; Survival Rate; Synaptophysin; Young Adult

Adrenocortical carcinomas (ACCs) with myxoid features are rare neoplasms. We identified 7 cases of myxoid ACC and studied the clinicopathologic and immunohistochemical features of these neoplasms. The patients were 5 men and 2 women with a mean age of 45 years. Histologically, the tumors contained alcian blue-positive myxoid areas ranging from 10% to 50% of the tissue examined. One case showed lipomatous metaplasia. Areas of conventional ACC were present in all cases. Immunohistochemically, the tumors were positive for steroid receptor cofactor 1, inhibin, melan A, calretinin, and synaptophysin but negative for high-molecular-weight cytokeratin, CAM5.2, and Pax8. Clinical follow-up information for 4 patients demonstrated that all patients had died of their disease 11 to 69 months after diagnosis. Myxoid ACCs are rare tumors that expand the differential diagnosis of myxoid neoplasms involving the retroperitoneum. Contrary to previous reports proposing that the biologic behavior is similar to conventional ACC, our series seems to indicate that myxoid morphology is associated with more aggressive behavior.

Topics: Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Adult; Aged; Calbindin 2; Female; Humans; Immunohistochemistry; Inhibins; Male; MART-1 Antigen; Metaplasia; Middle Aged; S100 Calcium Binding Protein G; Synaptophysin

Tumors of the adrenal glands are among the most frequent tumors in cattle; however, few studies have been conducted to describe their characteristics. The aim of this study was to classify 41 bovine adrenal neoplasms from 40 animals based on macroscopic and histologic examination, including electron microscopy and immunohistochemistry for melan A, synaptophysin, chromogranin A, vimentin, pan-cytokeratin, 2',3'-cyclic nucleotide-3'-phosphohydrolase (CNPase), and Ki-67. The tumors were classified as 23 adrenocortical adenomas, 12 adrenocortical carcinomas, 2 schwannomas, 2 pheochromocytomas (1 malignant), and 1 ganglioneuroma. Five histologic features were characteristic of metastasizing adrenocortical tumors: invasion of the capsule, vascular invasion, diffuse growth pattern, spindle-cell morphology, and nuclear pleomorphism. Adrenocortical tumors with at least 3 of these features were classified as malignant. Immunohistochemically, adrenocortical tumors expressed melan A (16/19), vimentin (14/26), cytokeratin (11/26), and chromogranin A (9/27), whereas pheochromocytomas expressed chromogranin A (2/2), synaptophysin (2/2), and vimentin (1/2). Both schwannomas expressed CNPase. An immunohistochemistry panel consisting of antibodies against melan A, synaptophysin, and CNPase was considered most useful to classify bovine adrenal tumors. However, the distinction between benign and malignant adrenocortical tumors was based on histologic features as in human medicine.

Topics: 2',3'-Cyclic-Nucleotide Phosphodiesterases; Abattoirs; Adrenal Cortex Neoplasms; Adrenal Gland Neoplasms; Adrenal Glands; Adrenocortical Adenoma; Adrenocortical Carcinoma; Animals; Biomarkers, Tumor; Cattle; Cattle Diseases; Chromogranin A; Denmark; Humans; Immunohistochemistry; Keratins; MART-1 Antigen; Microscopy, Electron; Synaptophysin; Vimentin

Myxoid variant of adrenocortical carcinomas (ACC) are rare, there being only 11 cases in the literature to date. Reported herein are the findings of a case, which in contrast to all previously reported myxoid ACC, was devoid of typical non-myxoid areas. The patient was a 61-year-old man in whom a left adrenal mass was detected during investigation of Cushing's syndrome. The adrenal was replaced by malignant cells and expanses of myxoid material. The cells were positive for melan-A, synaptophysin, vimentin and alpha-inhibin. The ultrastructural features of the cells were typical of adrenal cortical differentiation. The differential diagnosis of myxoid ACC includes extraskeletal myxoid chondrosarcoma, chordoma, myxoid adenocarcinoma, myxoma, lipomatous tumors, nerve sheath tumors, smooth muscle tumors, gastrointestinal stromal tumor and other sarcomas. The presence of myxoid material in a retroperitoneal lesion raises a broad differential diagnosis in which myxoid adrenocortical neoplasms should be included. Clinicoradiological correlation may be helpful, but special stains, immunohistochemistry and ultrastructural examination may be necessary to establish the diagnosis.

Topics: Adenocarcinoma; Adrenal Cortex Neoplasms; Adrenocortical Carcinoma; Antigens, Neoplasm; Chordoma; Diagnosis, Differential; Humans; Immunohistochemistry; Male; MART-1 Antigen; Microscopy, Electron; Middle Aged; Myxoma; Neoplasm Proteins; Nerve Sheath Neoplasms; Retroperitoneal Neoplasms; Synaptophysin; Vimentin

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.

Topics: Adrenal Gland Neoplasms; Adrenalectomy; Adrenocortical Carcinoma; Anaplasia; Antigens, Neoplasm; Fatal Outcome; Humans; MART-1 Antigen; Middle Aged; Neoplasm Proteins; Tongue Neoplasms; Vimentin

The Melan-A (MART-1) gene encodes an antigen recognized by cytotoxic T cells. It has been said to be restricted in its expression to melanocytes. However, here we report the presence of immunoreactivity for A103, an antibody to Melan-A, in five adrenocortical adenomas, 16 primary and 13 metastatic adrenocortical carcinomas, four Leydig cell tumors of the testis, and three Sertoli-Leydig cell tumors of the ovary. To evaluate the potential diagnostic role of this antibody, we studied immunoreactivity for A103 in 111 carcinomas, 40 germ cell tumors, and 33 miscellaneous nonmelanocytic epithelioid tumors. All of them were negative for A103. Our findings suggest that once melanoma is excluded, A103 can aid in the recognition of steroid hormone-producing tumors and may be particularly useful in the diagnosis of adrenocortical carcinoma. The presence of immunoreactivity for A103 practically excludes any other carcinoma that may enter into the differential diagnosis of adrenocortical tumors.

Topics: Adrenal Cortex Neoplasms; Adrenocortical Adenoma; Adrenocortical Carcinoma; Animals; Antibodies, Monoclonal; Antibodies, Neoplasm; Antigens, Neoplasm; Cross Reactions; Female; Humans; Immunoenzyme Techniques; Leydig Cell Tumor; Male; MART-1 Antigen; Mice; Neoplasm Proteins; Sertoli-Leydig Cell Tumor; Urogenital Neoplasms