Abstract：
:A case of pancreatic acinar cell tumor (ACC) is presented in a 10-year-old boy. The tumor manifested clinically with Cushing's syndrome, high serum adrenocorticotropic hormone (ACTH) and cortisol concentrations. In addition, excessive serum levels of alpha-fetoprotein (AFP) were detected. Surgical resection was not possible due to retroperitoneal invasion. Biopsy of the mass showed a solid, poorly differentiated ACC of the pancreas. Periodic acid Schiff positive cytoplasmic granules, trypsinogen, keratins, alpha-1-antitrypsin, and AFP were identified in the tumor cells. Electron microscopy demonstrated zymogen granules as well as isolated dense core granules. Using immunochemiluminometric assay, a high quantity of ACTH was found in the fresh frozen tumor extract. ACTH, chromogranin A, and corticotropin-releasing factor were identified only in a few cells by immunohistochemistry. Combined radiochemotherapy was temporarily effective in reducing the tumor mass and serum AFP. Serum ACTH and cortisol levels dropped progressively and definitively to normal values after chemotherapy, and the Cushing's syndrome subsided. Two years later, the patient died with metastatic disease. The presented case of ACC is interesting due to high serum AFP values and ectopic ACTH secretion resulting in Cushing's syndrome.
journal_name
Endocr Patholjournal_title
Endocrine pathologyauthors
Illyés G,Luczay A,Benyó G,Kálmán A,Borka K,Köves K,Rácz K,Tulassay T,Schaff Zdoi
10.1007/s12022-007-0018-4subject
Has Abstractpub_date
2007-07-01 00:00:00pages
95-102issue
2eissn
1046-3976issn
1559-0097pii
EP:18:2:95journal_volume
18pub_type
杂志文章abstract：:A cell fine of human medullary carcinoma of the thyroid, abundantly producing calcitonin (Ct) and related hormones, has proved remarkably useful as an endocrine tumor model for the study of the secretion mechanism. This cell line (TT cell) was used in studies to elucidate the dynamics of the release of Ct and chromogr...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02915322
更新日期：1991-03-01 00:00:00
abstract：:The recent increase in the detection of papillary thyroid carcinoma (PTC) has been influenced by the finding of incidental tumours. To this group, carcinomas measuring less than 1 cm (the so-called microcarcinomas) as well as those above 1 cm belong. Analyzing a case series from our own experience, this paper focuses ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-014-9323-x
更新日期：2014-09-01 00:00:00
abstract：:Gastrointestinal and pancreatic neuroendocrine tumors originate from the cells of the diffuse endocrine system. Their molecular genetic mechanism of development and progression is complex and remains largely unknown, and they are different in genetic composition from the gastrointestinal epithelial tumors. The current...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1007/s12022-007-9007-x
更新日期：2007-10-01 00:00:00
abstract：:Papillary carcinomas (PCs) of thyroid are among the most common but least aggressive human malignancies. The factors explaining the indolence of these tumors are unknown but host-tumor immune interactions may play a role. This study was designed to determine if there is morphologic evidence of these. Frozen tissues co...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739883
更新日期：1995-10-01 00:00:00
abstract：:Activating mutations of GNAq protein in a hotspot at codon 209 have been recently described in uveal melanomas. Since these neoplasms share with thyroid carcinomas a high frequency of MAP kinase pathway-activating mutations, we hypothesized whether GNAq mutations could also play a role in the development of thyroid ca...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-010-9129-4
更新日期：2010-12-01 00:00:00
abstract：:The morphologic distinction between parathyroid carcinoma and adenoma can be a difficult diagnostic problem. We analyzed nuclear immunoreactivity for the cell cycle-associated antigen Ki-67 with monoclonal antibody (MAb) MIB-1 and for retinoblastoma (RB) protein with two polyclonal antisera in 24 parathyroid carcinoma...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02738728
更新日期：1995-01-01 00:00:00
abstract：:A 59-yr old female presented with Cushing's syndrome due to ectopic ACTH production. At the time of initial diagnosis an obvious source for the Cushing's syndrome was not found and the patient was treated with bilateral adrenalectomy. Three years later she presented with hyperpigmentation and evidence of ACTH overprod...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:16:2:145
更新日期：2005-07-01 00:00:00
abstract：:Small cell carcinomas may occur in the thyroid gland. Infrequently, they are primary tumors, and have been interpreted as variants of medullary thyroid carcinoma. However, the vast majority of small cell carcinomas involving the thyroid gland are metastatic tumors. In some cases, demonstration of the primary tumor is ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-008-9024-4
更新日期：2008-10-01 00:00:00
abstract：:Crohn's disease is one of the causes of secondary amyloidosis, which can lead to amyloid infiltration of the thyroid gland. It is essential to follow strict controls to prevent the appearance of a large amyloid goiter. Two patients with amyloid goiter secondary to Crohn's disease, with a large adipose tissue component...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-012-9220-0
更新日期：2012-12-01 00:00:00
abstract：:We report two different cases of IgG4-related hypophysitis. In the first case, a pituitary lesion was accompanied by lymphocytic meningitis possibly mimicking tuberculous meningitis. The second case was unassociated with involvement of other organs. No histologic differences were noted between the two cases indicating...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9464-1
更新日期：2017-12-01 00:00:00
abstract：:Hurthle cell carcinoma of the thyroid is generally considered to be a subtype of follicular carcinoma. We report a case of a small solitary usual-type papillary carcinoma of the thyroid, with metastatic tumor in cervical lymph nodes. The lymph node tumor consisted of both tall-cell papillary carcinoma and Hurthle cell...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-002-0002-y
更新日期：2002-10-01 00:00:00
abstract：:Oncocytic phaeochromocytomas are exceedingly rare tumours. To date, there are three reported cases in the literature. This report describes a case of adrenal oncocytic phaeochromocytoma with unique features and malignant potential in a 68-year-old man. The patient presented with an incidental non-functional mass disco...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1007/s12022-014-9333-8
更新日期：2014-12-01 00:00:00
abstract：:A 31-year-old woman was incidentally found to have a large right adrenal mass by computed tomography imaging and underwent a workup that included endocrinological evaluation and positron emission tomography imaging. Laboratory results revealed the mass to be non-functioning. Imaging studies revealed a 9-cm heterogeneo...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-008-9013-7
更新日期：2007-01-01 00:00:00
abstract：:High-grade neuroendocrine carcinomas (HGNECs) of the urinary bladder encompass small cell (SCNEC) and large cell neuroendocrine carcinomas (LCNEC). Currently, recommended initial management is with systemic chemotherapy, followed by consolidative therapy with either radical cystectomy or radiotherapy in patients with ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-018-9516-9
更新日期：2018-09-01 00:00:00
abstract：:Nonradioisotopic electron microscopic (EM) in situ hybridization (ISH) (EM-SH) with biotinylated oligonucleotide probes is utilized for the ultrastructural visualization of pituitary hormone mRNA in rat pituitary cells. EMISH is an important tool for clarifying the intracellular localization of mRNA and the exact site...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02738881
更新日期：1999-10-01 00:00:00
abstract：:A 41-yr-old patient with a history of von Hippel-Lindau (VHL) disease with previously removed bilateral pheochromocytomas and renal cell carcinoma presented with progressive obstructive jaundice due to multiple lesions in the pancreas. The pancreatectomy specimen showed a range of endocrine lesions including islet hyp...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:15:2:141
更新日期：2004-07-01 00:00:00
abstract：:This study is aimed to investigate the BRAF (V600E) and TERT promoter mutation profile of 50 pediatric papillary thyroid carcinomas (PTCs) to refine their clinicopathological correlates. The median age at the time of surgery was 16 years (range, 6-18). No TERT promoter mutations were identified in this series. The BRA...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-016-9420-0
更新日期：2016-06-01 00:00:00
abstract：:Granular cell tumours of the thyroid gland are rare, with only six previously reported cases in the English literature. Current histological, immunohistochemical and electron microscopic evidence favours a neural/Schwannian relationship. A case of a granular cell tumour of the thyroid gland in a healthy 36-year-old wo...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1007/s12022-011-9146-y
更新日期：2011-03-01 00:00:00
abstract：:Neuroendocrine neoplasms comprise a heterogeneous group of tumors, categorized into neuroendocrine tumors (NETs) and neuroendocrine carcinomas (NECs) depending on tumor differentiation. NECs and high-grade NETs (G3) confer a poor prognosis, demanding novel treatment strategies such as immune checkpoint inhibition in t...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-020-09612-7
更新日期：2020-06-01 00:00:00
abstract：:Pheochromocytomas and paragangliomas are neuroendocrine neoplasias of neural crest origin. Genetic mutations that are characterized in other human neoplasms are rarely seen in these tumors. About 10% of the patients with pheochromocytomas and paragangliomas present with a family history of von Hippel-Lindau disease (V...
journal_title：Endocrine pathology
pub_type： 杂志文章,评审
doi：10.1385/ep:14:4:329
更新日期：2003-01-01 00:00:00
abstract：:Twenty-four acromegalic patients were treated with octreotide subcutaneously for periods of 3 to 6 weeks (group I, 12 cases) or 6 months (group II, 12 cases) before transsphenoidal surgery. Radiological studies performed in 19 patients before and at the end of this treatment period revealed no changes in 8 cases. In 8...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02915452
更新日期：1991-09-01 00:00:00
abstract：:Neoplastic plasma cell involvement of thyroid is an uncommon condition, and it may involve thyroid in multiple myeloma or solitary plasmacytoma. Its clinical and pathological features are not well understood. We present a rare case of synchronous thyroid involvement in plasma cell leukemia presenting as thyroid nodule...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-015-9392-5
更新日期：2015-12-01 00:00:00
abstract：:Folliculostellate (FS) cells are present in the anterior pituitary and have important regulatory functions including controlling hormone release from other anterior pituitary cells. FS cells do not usually express neuroendocrine genes such as chromogranin A (CgA). We analyzed transcriptional regulation and gene expres...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:16:3:173
更新日期：2005-10-01 00:00:00
abstract：:Objective. Information on angiogenesis in parathyroid pathology is scanty and in particular no data are available in parathyroid carcinomas. The aim of this study was to analyze angiogenesis as microvascular density (MVD) in parathyroid neoplastic progression from normal gland to adenoma and carcinoma. Methods. Sectio...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:17:2:175
更新日期：2006-07-01 00:00:00
abstract：:Somatostatin and its analogs have been included in experimental treatment protocols for advanced pancreatic adenocarcinoma based on their known antisecretory and antiproliferative properties. Somatostatin receptor type 2 (sstr2A) mediates antiproliferative actions of somatostatin and has the strongest affinity to the ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1385/ep:12:2:147
更新日期：2001-07-01 00:00:00
abstract：:The pituitary contains estrogen receptor (ER), progesterone receptor (PR), and androgen receptor (AR). In accordance with immunocytochemistry, it is agreed that sex hormone receptors reside into the nucleus. All three receptors are found predominantly in gonadotrophs and lactotrophs, and less frequently in other cell ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739938
更新日期：1997-07-01 00:00:00
abstract：:A case of ectopic thymic tissue of the thyroid in a 30-year-old woman with Graves' disease is reported. The thyroidectomy specimen, removed because of failure of medical therapy, showed a continuous track of thymic tissue extending from the center of the gland to the capsule along an interiobular septum. This finding ...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02915305
更新日期：1993-09-01 00:00:00
abstract：:Two unrelated families (CA and NA) in which an iodide organification defect (lOD) was present in two siblings of each family were studied. These patients had congenital goiters with hypothyroidism and a positive perchlorate discharge test. Examination of the thyroid tissue revealed no thyroid peroxidase (TPO) activity...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739706
更新日期：1997-04-01 00:00:00
abstract：:The histological pattern and the cell proliferative activity (as detected by Ki-67 immunostaining) of a series of 50 parathyroid hyperplasias (PTHs) secondary to renal failure were studied to assess their value in predicting recurrence of hyperparathyrodism (HPT). On account of their clinical evolution, these cases we...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/BF02739915
更新日期：1996-04-01 00:00:00
abstract：:We report here the case of a 43-year-old woman with hyperparathyroidism, parathyroid chief cell adenoma, and massive chronic parathyroiditis. The patient was diagnosed with breast cancer and received chemotherapy 3 years before parathyroidectomy. The question can be raised whether chemotherapeutic agents may damage pa...
journal_title：Endocrine pathology
pub_type： 杂志文章
doi：10.1007/s12022-007-0001-0
更新日期：2007-04-01 00:00:00
