Cases reported "Adrenal Gland Neoplasms"

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1/30. somatostatin receptor scintigraphy for early detection of regional and distant metastases of medullary carcinoma of the thyroid.

    Three patients are described who had regional and distant metastases of medullary thyroid cancer detected by somatostatin receptor scintigraphy but not by CT; two had minimal disease that was amenable to surgery. The first patient had been followed for 2 years before having a repeated scan and positive CT, with subsequent surgical removal of metastatic paratracheal nodes. The plasma calcitonin level, however, did not approach normal values after surgery, and a third scan showed persistence of focal uptake in the left paratracheal area of the lower neck, whereas CT was negative. At repeated exploration, a tumor mass of medullary carcinoma, embedded in lymphatic tissue, was removed. Nine months after the last surgical procedure, calcitonin and carcinoembryonic antigen levels were normal. The second patient underwent microdissection of the mediastinum and removal of two metastatic nodes that were demonstrable only by the scintigraphic technique. The plasma calcitonin level subsequently became normal. The third patient, with multiple endocrine neoplasia IIB and associated pheochromocytoma, had bony metastatic involvement of the left shoulder, demonstrable initially on somatostatin receptor scintigraphy and subsequently with radioiodinated metaiodobenzylguanidine but not on CT.
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2/30. Primary epithelioid angiosarcoma of the adrenal gland case report and review of the literature.

    Primary angiosarcoma of the adrenal gland is extremely rare. Here, we report on a 70-year-old man with an angiosarcoma of the right adrenal gland who died 3 weeks after tumor resection due to intestinal infarction and acute renal failure. No metastases were found at autopsy. Histologically, the tumor showed a predominantly epithelioid differentiation. Immunohistochemical examination revealed positive reactivity for cytokeratin, epithelial membrane antigen, vimentin, factor viii-related antigen, CD31, CD34 and ulex europaeus agglutinin-I. Features of endothelial origin were also demonstrable by electron microscopy. The differential diagnosis of this uncommon neoplasm is discussed. The present case emphasizes problems in differential diagnosis that arise from its epithelioid differentiation. A review of the literature underlines the poor clinical outcome of adrenal angiosarcoma.
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3/30. Functioning adrenal black adenoma with pulmonary and cutaneous cryptococcosis: a case report and review of English literature.

    A 53-year-old woman experienced progressive general weakness and lumbago in the 2 years prior to a physical examination which disclosed cushingoid manifestations and a skin ulcer on the back of her right knee joint. Her plasma cortisol concentration ranged from 24.7 to 31.1 microg/dl, with an ACTH level <5 pg/ml. Urinary excretions of 17-hydroxycorticosteroid (17-OHCS) and 17-ketosteroid (17-KS) were 20.5 mg/day and 5.1 mg/day, respectively, and urinary cortisol was also increased (421 microg/day). Cortisol was not suppressed after the administration of 8 mg dexamethasone. Abdominal ultrasound sonography, computed tomography (CT) scan, and magnetic resonance imaging (MRI) studies demonstrated a left adrenal tumor and further, a chest X-ray examination showed a cavitary lesion containing a fungus ball-like mass in the left lower lung field. The serum cryptococcal antigen titer was positive at 1:128 and a bronchoalveolar lavage fluid culture yielded a growth of cryptococcus neoformans. A biopsy specimen of the skin ulcer also suggested cryptococcosis. As a result, a left adrenectomy was performed, and the excised specimen was shown to be an adenoma consisting of compact cells with abundant pigmentation (black adenoma). A diagnosis of functioning black adenoma of the adrenal gland, complicated with pulmonary and cutaneous cryptococcosis was made.
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4/30. Unusual clinical manifestation of pheochromocytoma in a MEN2A patient.

    A case of unusual clinical manifestation of pheochromocytoma in a type 2A multiple endocrine neoplasia (MEN2A) patient is presented. A 27-year-old man affected by MEN2A syndrome, complaining of anxiety and depression, was admitted in our Division. Past medical history included a total thyroidectomy for medullary carcinoma in 1985, and left adrenalectomy for pheochromocytoma in 1994. blood pressure was 130/ 85 mmHg without orthostatic hypotension and pulse rate was 72 beats/min. Laboratory data revealed thyroid hormones and carcinoembryonic antigen (CEA) in the normal range and high basal serum calcitonin levels (158 pg/ml). plasma catecholamines and vanillylmandelic acid resulted in normal levels but epinephrine/norepinephrine ratio was elevated (0.65). The glucagon stimulation test showed positive clinical and biochemical response. magnetic resonance imaging (MRI) and meta-iodobenzylguanidine (MIBG) scintiscan confirmed the presence of bilateral adrenal masses. Bilateral adrenalectomy by laparoscopic anterior approach was performed. histology was consistent with adrenal pheochromocytomas. After surgical approach, psychiatric findings disappeared and did not recur at follow-up in spite of no medication for two years. In conclusion, bilateral pheochromocytoma is more frequent in MEN2A syndrome and probably understimated if the follow-up is not prolonged. In these cases clinical features are often aspecific and basal hormonal data may be normal in a great number of patients. Therefore long-term observation is justified in these patients. pheochromocytoma was described as the "great mimic" for the numerous subjective manifestations. Differential diagnosis among typical features of neuropsychiatric disorders and pheochromocytoma must be considered.
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5/30. Pleomorphic leiomyosarcoma of the adrenal gland.

    Primary leiomyosarcomas arising in the adrenal gland are exceedingly rare, with only 3 cases reported in the literature. We present the clinical, morphologic, and immunohistochemical features of a pleomorphic leiomyosarcoma, a variant of leiomyosarcoma that has not been described in the adrenal gland. A 63-year-old man presented with a 1-year history of enlarging right upper quadrant mass and pulmonary nodule. A diagnosis of metastatic pulmonary carcinoma to the adrenal gland was rendered on a needle biopsy specimen. Preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge. Documented by computed tomography and confirmed at surgery, the tumor had completely replaced the right adrenal gland, invading into both the posterior aspect of the right liver and the superior pole of the right kidney. Histologic sections showed a diffuse proliferation of pleomorphic, large, and polygonal neoplastic cells with prominent nucleoli. Many bizarre mitotic figures were present. The neoplastic cells were strongly positive for desmin, calponin, and vimentin. Approximately 80% of the neoplastic cells were positive for the proliferation marker Ki-67. They were negative for smooth muscle actin, muscle-specific actin, myoglobin, myogenin, CD117, cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, chromogranin, CD34, CD31, S100 protein, and HMB-45.
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6/30. Adrenal metastasis from rectal cancer: report of a case.

    We report a case of heterochronic adrenal metastasis from colorectal carcinoma in a 51-year-old woman. A left adrenal metastasis was found by computed tomography and magnetic resonance imaging 8 months after an anterior resection for advanced rectal carcinoma, and a left hepatectomy for a solitary liver metastasis. The level of serum carcinoembryonic antigen was still within the normal range. A left adrenalectomy was performed, and histopathological examination revealed adenocarcinoma, compatible with the rectal carcinoma resected 8 months earlier. The patient died of lung metastases 6 months after the adrenalectomy. A review of autopsy series in the world literature revealed that adrenal metastasis from colorectal cancer is not rare. Therefore, the possibility of adrenal metastasis should be considered in the follow-up of patients after primary surgery for colorectal cancer, even though the liver and lung are the main metastatic sites.
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7/30. adenomatoid tumor of the adrenal gland: a clinicopathologic study of five cases and review of the literature.

    We report the clinicopathologic, immunophenotypic, dna ploidy, and MIB-1 proliferative findings of five adenomatoid tumors of the adrenal gland. All patients were male, and tumors were incidental radiologic, surgical, or autopsy findings. Mean patient age at diagnosis was 41 years (range 31-64 years). The tumors ranged from 1.2 to 3.5 cm (mean 2.8 cm; median 3.2 cm) in greatest dimension, and all originated within the adrenal gland. The tumors were composed of anastomosing variably sized tubules lined by epithelioid as well as flattened cells. Signet-ring-like cells were present in all cases. The previously described histologic patterns of adenomatoid tumor, adenoid, angiomatoid, cystic, and solid, were observed, and each tumor contained multiple histologic patterns. In three of five cases, there was extra-adrenal extension of tumor into periadrenal adipose tissue. All adenomatoid tumors infiltrated the adrenal cortex, and in four cases the adrenal medulla was involved. All tumors exhibited strong immunoreactivity for calretinin, cytokeratins AE1/AE3, and CAM 5.2, cytokeratin 7, and vimentin. Tumors showed weak and focal immunoreactivity for cytokeratin 5/cytokeratin 6 and were negative for CD15, CD31, CD34, cytokeratin 20, MOC31, and polyclonal carcinoembryonic antigen. Ploidy analysis using Feulgen-stained sections and image analysis showed that three tumors were diploid and two were tetraploid. Tumors exhibited low MIB-1 proliferative activity, ranging from 0.2% to 2.7% (mean 1.6%). In three cases with clinical follow-up, no recurrence or metastases occurred. Adrenal gland adenomatoid tumors are morphologically and immunophenotypically identical to adenomatoid tumors of the genital tract and appear benign.
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8/30. Epithelioid angiosarcoma of the adrenal gland with cytokeratin expression. Report of a case with accompanying mesenteric fibromatosis.

    A case report of epithelioid adrenal angiosarcoma is presented. Tumor cells showed expression of cytokeratin, factor viii-related antigen, ulex europaeus agglutinin-I, and vimentin. The patient also was found to have mesenteric fibromatosis (abdominal desmoid tumor) and an elevated serum level of estradiol. The authors discuss the unique appearance of these rare tumors, their relationship to hyperestrinism, and review the recent data in the literature showing cytokeratin expression by malignant epithelioid vascular tumors.
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9/30. A case of adenocarcinoma of the lung with a spindle cell component.

    We report a 60-year-old man, who was admitted to the hospital with complaints of cough and sputum. His chest x-ray showed an abnormal mass in the right upper lobe. After admission he noticed the painful gingival tumor. Right upper lobectomy and resection of the gingival tumor were performed. Their histological features showed that the tumor consisted of a papillary and tubular adenocarcinoma mixed with a component of spindle cells. Immunohistochemical study demonstrated a positive reaction in the epithelial component for keratin and epithelial membrane antigen, and not only these epithelial markers but also vimentin were expressed in some spindle tumor cells. Electron microscopic study confirmed the biphasic pattern, showing gland formation and undifferentiated cells. We diagnose this case as adenocarcinoma of the lung with a spindle cell component and the gingival tumor was metastatic. autopsy showed that metastatic lesions were found in the left adrenal gland and in the left kidney. The tumor cells in the left adrenal gland were composed of spindle cells and the tumor in the left kidney showed gland formation. Immunohistochemical and electron microscopic findings of surgical and autopsy specimen suggest that this tumor is of epithelial origin, and the spindle cells are derived from immature mesenchymal cell transformation of epithelial cells.
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10/30. Membranous glomerulonephritis in a patient with an adrenal ganglioneuroma.

    Membranous glomerulonephritis has been reported in association with several tumors. This report is the first case of membranous glomerulonephritis associated with an adrenal ganglioneuroma. The sera obtained from the patient at presentation stained the fibrillary component of the ganglioneuroma and the glomerular basement membrane of the kidney. The antibodies present in the patient's serum also reacted with the glomerular basement membrane of several normal and randomly selected abnormal kidneys, including 1 from a patient with Alport's syndrome. The patient's sera contained a circulating tumor antigen-specific antibody that cross-reacted with an antigen present on the podocyte membrane of the renal glomeruli. The resection of the ganglioneuroma resulted in a complete remission of the nephrotic syndrome within months of removal of the tumor. This was associated with loss of antibodies in the patient's serum. The patient was not treated with steroids or any immunosuppressive agents. The patient remains in remission with normal renal function after 9 years of follow-up.
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