1/33. indium-111 pentetreotide lung uptake in infectious lung disease.Bilateral diffuse lung uptake of In-111 pentetreotide (OCT) was observed during a whole-body scan performed in a 68-year-old woman with Cushing's syndrome and suspected ectopic adrenocorticotropic hormone secretion. A few days later, she was found to have bilateral bacterial pneumonia (of mixed anaerobic origin). Cushing's syndrome was finally proved to be of pituitary origin. The OCT lung uptake in pneumonia probably resulted from tracer binding by somatostatin receptors on the inflammatory leukocytes. Although the rapid wash-out from experimentally induced abscesses does not make OCT a suitable tracer for detecting acute infections, the images and data here reported suggest that infectious lung disease should be excluded before diagnosing lung involvement by neuroendocrine tumors.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
2/33. Cushing's syndrome in prostate cancer. An aggressive course of prostatic malignancy.We report a case with an initial diagnosis of adenocarcinoma of the prostate in whom Cushing's syndrome developed. The disease did not respond to estrogen treatment and the patient died of severe septicemia. Histopathologic examination of the autopsy specimens revealed a small cell carcinoma intermingled with a moderately differentiated adenocarcinoma in the prostate and widespread metastases of small cell carcinoma. Immunoreactivity for neuroendocrine differentiation was found only in the small cell carcinoma. Determination of different tumor markers in plasma samples showed markedly elevated levels of prostate-specific antigen as well as carcinoembryonic antigen prior to treatment, with no significant changes after treatment. The concentration of the neuroendocrine marker chromogranin a was initially within the normal range, but increased during estrogen treatment, whilst neuron-specific enolase was moderately elevated throughout the observation period. copyright copyright 1999 S. Karger AG, Basel- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
3/33. An exception to the rule: ectopic ACTH production from functional neuroendocrine tissue in an ovarian dermoid cyst.Ectopic ACTH production accounts for 15% of Cushing's syndrome presentations and is characterized by the presence of an excess of ACTH precursors. However in the case presented here ectopic ACTH production was from functional pituitary tissue within an ovarian dermoid cyst. Endocrine investigations showed that the problem behaved more like pituitary-dependent Cushing's disease and this is discussed. Furthermore, this case is one of familial dermoid cysts, another unusual phenomenon.- - - - - - - - - - ranking = 4keywords = endocrine (Clic here for more details about this article) |
4/33. Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome.Neuroendocrine carcinoma (carcinoid) of the thymus associated with Cushing's syndrome is a rare disease. Recent evidence suggests that these tumors form part of a continuous spectrum ranging from well-differentiated carcinomas to small cell carcinomas. We report two new cases and review the 23 cases reported in the literature since 1972. The different diagnostic modalities are discussed, and an algorithm for the diagnosis of ectopic secretion of adrenocorticotropin (ACTH) is presented. In the future, the advent of radiologic and nuclear imaging as well as more accurate workup should help to diagnose these tumors at an earlier stage and improve the long-term outcome.- - - - - - - - - - ranking = 5keywords = endocrine (Clic here for more details about this article) |
5/33. Pancreatic exocrine carcinoma producing adrenocorticotropic hormone.A rare case of pancreatic exocrine carcinoma with an endocrine component secreting adrenocorticotropic hormone (ACTH) is reported and discussed in relation to other cases previously published. The patient initially presented with a severe form of diabetes, which was treated accordingly. Persistence of hyperglycemia, along with other metabolic alterations and marked hypokalemia, led to the suggestion of abnormal ACTH secretion. In this patient, however, a florid Cushing's syndrome was not observed. The patient also developed hematological alterations, mainly leukopenia and thrombocytopenia, whose origins were unclear. At autopsy, a poorly-defined mass was discovered between the body and tail of the pancreas. Standard histology showed a moderately-differentiated adenocarcinoma. Immunohistochemical analysis of the tumor specimen demonstrated the presence of some neoplastic cells immunoreactive for chromogranin a, neuron-specific enolase and ACTH. These findings are consistent with the existence of an endocrine component within the exocrine carcinoma with ACTH differentiation.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
6/33. Small cell carcinoma with two paraendocrine syndromes.Simultaneous elevated levels of ectopic arginine vasopressin (AVP) and ectopic adrenocorticotropin (ACTH) were found in a patient with small cell carcinoma (SCC). The finding of one of these paraendocrine syndromes at the time of diagnosis is common; however, the simultaneous presence of both syndromes has been reported in the literature only on four occasions in the past 25 years. This is the only report in which elevated plasma levels of both hormones are documented in a patient who simultaneously fulfills the criteria for the syndrome associated with each ectopically produced peptide. In the English-language literature, this is the first case that demonstrates by immunohistochemical staining the presence of both of these hormones in the patient's neoplasm. In addition to the use of radiographs, the presence of paraendocrine disorders can provide a method of monitoring the patient's response to therapy. The levels of ACTH and AVP were assayed during this patient's course and correlated with disease refractory to therapy, resulting in poor survival.- - - - - - - - - - ranking = 6keywords = endocrine (Clic here for more details about this article) |
7/33. Cushing's syndrome associated with adenocarcinoma of the lung.Paraneoplastic Cushing's syndrome caused by ectopic adrenocorticotropin production has been reported in association with a variety of malignant tumors. However, most cases of this syndrome are associated with neuroendocrine tumors such as small-cell lung cancer (SCLC), carcinoid tumors, and medullary carcinoma of the thyroid. We present a 49-year-old man who developed Cushing's syndrome associated with adenocarcinoma of the lung. He had a wide variety of clinical symptoms and comorbidity related to this syndrome during the course, and died 4 months after the diagnosis. This is a very rare case report of Cushing's syndrome associated with adenocarcinoma of the lung.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
8/33. pro-opiomelanocortin expression in a metastatic breast carcinoma with ectopic ACTH secretion.Cushing's syndrome secondary to ectopic adrenocorticotropic hormone (ACTH) secretion is rarely observed in breast carcinoma and only four cases have been previously published. We report here the case of a 50-year-old woman who presented with a history of diffuse bone pain associated with multiple hepatic, pulmonary, and bone metastases. A core needle biopsy specimen revealed an invasive ductal carcinoma in the right breast. The patient subsequently developed an ACTH-dependent paraneoplastic Cushing's syndrome and she died of arrhythmia and heart failure, despite treatment. At autopsy, immunohistochemical staining showed chromogranin a and ACTH positivity in the breast tumor and a lung metastasis. The mRNA expression of the pro-opiomelanocortin (POMC) gene was detected in tumoral cells by reverse transcriptase polymerase chain reaction (RT-PCR). This is the first case of Cushing's syndrome secondary to ectopic ACTH secretion where the presence of ACTH by immunohistochemistry and the expression of the POMC gene by RT-PCR have both been demonstrated in a breast carcinoma with metastases. The clinical history and the pathologic findings are presented with the methods and results of the molecular analysis. This case illustrates an example of ectopic ACTH syndrome in a breast carcinoma with neuroendocrine (NE) differentiation. This NE phenotype is directly related to the synthesis of ACTH by the tumoral cells. It should be kept in mind that an ectopic ACTH syndrome may be produced not only by small cell carcinoma or endocrine tumors but also by breast cancer. No relationship has been established between NE features and prognostic factors or patient outcome for this peculiar type of breast carcinoma. The demonstration of mRNA POMC in breast carcinoma with NE features suggests a depression and/or an activation of the POMC gene linked to the NE differentiation.- - - - - - - - - - ranking = 2keywords = endocrine (Clic here for more details about this article) |
9/33. Pancreatic neuroendocrine tumor with ectopic adrenocorticotropin production upon second recurrence.We present a 54-yr-old woman with ectopic corticotropin syndrome caused by a neuroendocrine tumor of the pancreas. At initial presentation, the patient suffered from diarrhea, heartburn, and nonspecific abdominal pain. There was no evidence of Cushing's syndrome. A neuroendocrine tumor in the head of the pancreas with metastases into peripancreatic lymph nodes was diagnosed and completely resected. Fourteen months later, abdominal computed tomography and scintigraphy with (111)In-labeled octreotide suggested relapse of the tumor. The patient again had no evidence of Cushing's syndrome. A second in toto tumor resection was performed. Another 8 months later, the patient developed forgetfulness, depressive episodes, muscle weakness, new-onset hypertension, hypokalemia, plethora, diabetes mellitus, polyuria, and weight loss. Endocrine testing suggested a source of ectopic ACTH production. An octreotide scan showed an intense uptake ventromedial of the left kidney, an area that showed a mass lateral of the superior mesenteric artery on abdominal magnetic resonance imaging. A complete pancreatectomy with splenectomy and left-sided adrenalectomy were performed. At this second relapse, this neuroendocrine tumor clinically had changed its hormonal profile. Immunohistochemically, in contrast to primary tumor and first relapse, we found strong immunostaining for ACTH in tumor cells of the second relapse and a MIB-1 index greater than 20%. To our knowledge, this is the first report describing a pancreatic neuroendocrine tumor that started to secrete ACTH de novo at the time of the second relapse after two former complete tumor resections. This case underscores the pluripotency of neuroendocrine tumor cells and the importance of keeping in mind a possible shift in hormone production during tumor evolution and progression.- - - - - - - - - - ranking = 9keywords = endocrine (Clic here for more details about this article) |
10/33. Use of radioguided surgery with [111In]-pentetreotide in the management of an ACTH-secreting bronchial carcinoid causing ectopic Cushing's syndrome.Intraoperative [111In]-pentetreotide scintigraphy with a hand-held gamma detector probe has recently been proposed to increase the intraoperative detection rate of small neuroendocrine tumors and their metastases. We report a case of a 28-yr-old woman with ectopic Cushing's syndrome due to an ACTH-secreting bronchial carcinoid, in whom the use of radioguided surgery improved disease management. At presentation, radiolabeled pentetreotide scintigraphy was the only procedure able to detect the ectopic source of ACTH. After radiologic confirmation, the patient underwent removal of a bronchial carcinoid, with disease persistence. After surgery, pentetreotide scintigraphy showed pathologic uptake in the mediastinum not previously detected at surgery and only subsequently confirmed by radiologic studies. Despite a second thoracic exploration, hormonal, scintigraphic, and radiological evidence of residual disease persisted. Radioguided surgery was then performed using a hand-held gamma probe 48 h after iv administration of a tracer dose of radiolabeled [111In-DTPA-D-Phe1]-pentetreotide, which permitted detection and removal of multiple residual mediastinal lymph node metastases. Clinical and radiologic cure, with no evidence of tracer uptake at pentetreotide scintigraphy, was subsequently observed. The use of an intraoperative gamma counter appears a promising procedure in the management of metastatic ACTH-secreting bronchial carcinoids.- - - - - - - - - - ranking = 1keywords = endocrine (Clic here for more details about this article) |
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