Cases reported "Endocrine Gland Neoplasms"

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1/4. Mammary endocrine ductal carcinoma in situ: a case report.

    Endocrine differentiation represents a pathway of neoplastic development available to a range of breast cancers. This pattern occurs in tumors with different morphological appearances as ductal carcinoma in situ (DCIS), mucinous carcinoma, a variant of lobular carcinoma, and low-grade invasive ductal carcinoma. Endocrine ductal carcinoma in situ is an uncommon entity. It occurs in older women with a mean age of 70 years. Histologically it shows expansile intraductal growth forming solid sheets and festoons transversed by delicate fibrovascular septa. Conventional microscopy permits the diagnosis in most cases. Specialized techniques such as immunohistochemistry and electron microscopy can serve as the basis of diagnosis in the absence of the appropriate morphological features. We present a 68-year-old female with a 1.5-cm firm mobile nodule of the left breast. mammography and ultrasounds showed a 15 x 15-mm circumscribed solid lobulated nodule. The mass was excised and pathology was positive for endocrine DCIS. Although endocrine DCIS has a biologic marker profile similar to that of well-differentiated or noncomedo DCIS it may constitute a different histogenetic pathway of carcinogenesis in the breast. The tumor may exhibit the invasive characteristics of a neuroendocrine neoplasm. Larger studies and longer follow-up are needed for the determination of the clinical behavior.
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2/4. Genetic analysis in a patient with recurrent cardiac myxoma and endocrinopathy.

    A 60 year-old male was referred for treatment of a cardiac myxoma in the right atrium. He had a past history of left atrial cardiac myxoma at age 49 and pituitary microadenoma related to acromegaly at age 55. He did not have a family history of cardiac neoplasm or endocrinopathy. The intracardiac tumor was resected and its pathology was compatible with myxoma. A diagnosis of carney complex (CNC) was made because the diagnostic criteria of this neoplastic syndrome were satisfied by the presence of recurrent cardiac myxoma, endocrine tumor and spotty skin pigmentation. In genetic analysis novel frame shift mutation was detected in exon 2 in a heterozygous fashion in the causative gene of CNC, protein kinase A regulatory subunit 1 alpha (PRKAR1A). This genetic mutation is thought to cause haplo-insufficiency of PRKAR1A resulting in tumorigenesis. Although it is the most common, usually benign, cardiac tumor, myxoma can cause a critical clinical situation and thus detecting the PRKAR1A mutation can assist with prognosis.
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3/4. Pancreatic endocrine neoplasm can mimic serous cystadenoma.

    A 57-yr-old female patient was referred to our hospital with a cystic lesion of the head of the pancreas that had been noted on abdominal computed tomography (CT). Endoscopic ultrasonography (EUS) showed a 3.0 cm rounded mass in the head of the pancreas. EUS images showed that the tumor had a solid component consisting of multiple microcysts separated by septae and a cystic component consisting of a macrocystic lesion. Thus, the tumor was suspected of being a serous cystadenoma (SCA). However, the histopathological diagnosis based on endoscopic ultrasound- guided fine-needle-aspiration biopsy (EUS-FNAB) was that of a pancreatic endocrine neoplasm (PEN). Surgical resection was performed. Despite having very similar macroscopic findings to SCA, microscopic examination revealed that the patient's tumor was definitely a PEN. This case suggests that it is very difficult to distinguish PENs from SCAs based solely on imaging methods. EUS-FNAB is essential for determining the appropriate therapeutic strategy, as it provides the histopathological diagnosis.
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4/4. Composite glandular and endocrine tumors of the stomach with pancreatic acinar differentiation.

    Composite tumors of the stomach consisting of mixed glandular and endocrine components are rare. We report 3 cases of composite glandular and endocrine tumors with pancreatic acinar differentiation in the stomach with their clinicopathologic findings. The patients' presenting symptoms were variable and included abdominal pain, gastrointestinal hemorrhage, and weight loss. One patient with abdominal pain also had an elevated serum lipase level, clinically mimicking acute pancreatitis. The histology of these tumors was similar. They showed admixture of well-differentiated endocrine components with acinar and glandular components. The glandular component consisted of columnar epithelial cells resembling gastric foveolar or intestinal goblet cells, consistent with a well-differentiated adenocarcinoma. A panel of histochemical and immunohistochemical stains was performed, which included PAS, alcian blue, Mib1, CEA, cytokeratin 7, cytokeratin 20, Muc2, Muc5AC, chromogranin, synaptophysin, trypsin, chymotrypsin, lipase, insulin, gastrin, serotonin, and pancreatic polypeptide. While the immunoreactivity for cytokeratin 7, cytokeratin 20, Muc2, Muc5AC, and CEA was largely restricted to the glandular component, the endocrine and pancreatic acinar markers showed marked variability and overlap. All cases showed immunoreactivity for at least one of the exocrine pancreatic enzymes, and all expressed endocrine differentiation. Some degree of amphicrine differentiation was suggested in all cases. Two cases showed metastases in perigastric lymph nodes, which histologically resembled the primary tumor. In summary, these tumors represent another distinct type of composite glandular and endocrine gastric neoplasm with pancreatic acinar differentiation.
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