Morphological and immunohistochemical features of neuroendocrine neoplasms in gastroenteropancreatic tract.

BACKGROUND AND AIMS: Neuroendocrine neoplasms (NENs) result from neuroendocrine cell neoplastic proliferation, with both characteristics of endocrine and nerve cells. NENs arise in different localizations but the most common site is the gastrointestinal tract, including the pancreas. The morphology and frequency of NENs in our hospital were evaluated, as well as the correlation between lymph node status and Ki67 proliferative index. MATERIALS AND METHODS: Retrospectively, we analyzed NEN pathohistological reports of patients who underwent surgical excision of gastroenteropancreatic tumors in the Clinical Center of Vojvodina in Novi Sad, from 2011 to 2018. The descriptive and correlation statistics were done. Chromogranin A, synaptophysin, CD56, and Ki67 were usually used as immunohistochemical markers. RESULTS: The study registered 78 surgical specimens. The average age of patients was 58.88 (±17) years, whereby 53.8% were males. The most frequent site was the pancreas (29.5%), followed by the large intestine (26.9%). The mean tumor size was 3.71 (±3.13) cm. No significance was found in tumor differentiation degree (G1 = 32.1%, G2 = 26.9%, and G3 = 29.5%). Lymph node metastases were found in 47.44%. Positive correlation was found between the Ki67 index and the percentage of positive lymph nodes (r = 0.343, p = 0.046, p < 0.05, respectively). The correlation between tumor size and lymph node metastases revealed no significance in our study (r = 0.015, p = 0.480, p > 0.05, respectively). CONCLUSION: Most NENs lymph node metastases originated from tumors with a high Ki67 index. The results confirm the use of proliferative index Ki67 as a prognostic marker for the presence of metastases, disease progression, and neuroendocrine tumor prognosis.

Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.

Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood. Laboratory analyses showed loss of circadian rhythm of cortisol, undetectable adrenocorticotropic hormone (ACTH) level, impaired fasting glucose, polycythemia and elevated white blood count (WBC). Radiology investigation revealed a slightly enlarged medial branch of the left adrenal gland and a normal right one, so a unilateral adrenalectomy was performed. Pathohistology described multiple dark brownish pigmented nodules of various sizes confined to the cortex. Contralateral adrenalectomy was done 3 months later. Follow-up of 3 years was uneventful, except for one adrenal crisis during an intercurrent respiratory illness.

An enigma of eosinophilic esophagitis.

Introduction: Eosinophilic esophagitis is a chronic immunogenic-antigen mediated disease of the esophagus, characterized by symptoms related to esophagus dysfunction, histologically defined by over 15 eosinophil counts seen in high-power microscopic field, without gastroesophageal reflux disease. In adults, the most common clinical manifestations are dysphagia, reflux, chest pain, regurgitation and bolus impaction. Case report: We presented the case of a female patient, hospitalized for a serious form of pancreatitis with complications, which required artificial ventilation and enteral feeding, after the initial esophagoscopy verified reflux esophagitis. Further treatment cured the primary illness, and peroral feeding was reintroduced. However, dysphagia with regurgitation occurred, and endoscopic and radiological tests verified esophagus stenosis, which histopathologically corresponded to erosive esophagitis. Two months of treatment by a double dosage of proton pump inhibitors led to no regression of disorders, and the repeated biopsies from the stenotic segments resulted in over 30 eosinophil counts in the high-power microscopic field, which histologically corresponds to eosinophilic esophagitis. Subsequent therapy included fluticasone 880 µg/day orally for a period of eight weeks, which led to complete regression of disorders, and endoscopic and histopathologic remission. Conclusion: In case of irresponsiveness to the conventional therapy by proton pump inhibitors, repeated esophagoscopy and histopathological analyses of esophagus mucosa biopsy can point to the diagnosis of eosinophilic esophagitis, and a good therapeutic response to topical corticosteroids can be regarded as the clinical confirmation of the diagnosis.

The expression and localization of estrogen receptor beta in hyperplastic and neoplastic prostate lesions.

BACKGROUND/AIM: Benign acini in benign prostatic hyperplasia (BPH) are lined with pseudostratified cylindrical epithelium with a continuous basal cell layer. Adenocarcinoma of the prostate is the most common cancer in men. High gradus-prostatic intraepithelial neoplasia (HGPIN) lesions precede invasive cancer. Prostate adenocarcinoma (PCa) implies a complete absence of basal cells and stromal invasion by malignant acini. Estrogen receptor (ER) is located in nuclei of acinar basal and secretory cells and partially in stromal cells. The aim of this research was to demonstrate and localize ER in BPH and in PCa of different Gleason scores. Considering literature data for ER-beta. expression in different morphologic prostate lesions, it is assumed that there is expression of ER-beta in most moderately differentiated PCa, and that the observed receptor expression is lost with increasing of the Gleason score. METHODS: Four groups of patients were formed: the control with BPH and three experimental groups with PCa of different grades and scores, according to the Gleason grading system. The patients were male of various ages suspected of PCa, based on clinical and laboratory parameters. The study was conducted in a period 2010-2012. None of the patients received prior hormonal therapy. Sextant byopsies with BPH and PCa were treated for ER-beta (Novacastra). Localization and intensity of ER-beta expression is reported through the score: 0 = zero; 1 = < 1%; 2 = 1-10%; 3=11-33%; 4= 34-66%; 5- > 66%. Positive fibroblasts and endothelial cells are used for comparison. RESULTS: ER-beta expression in acinar epithelial cells was the weakest in well-differentiated adenocarcinoma. A decline of ER-beta expression was noticed in malignant lesions of the prostate vs. benign ones. Less differentiated adenocarcinomas showed a decrease of ER-beta expression in basal and in the secretory cells. ER-beta expression in basal cells was stronger than in secretory ones in BPH and well-differentiated adenocarcinoma. CONCLUSION: ER-beta expression was most pronounced in BHP samples and declined in malignant prostate lesions. This finding supports statement on anticiproliferative role of ER-beta in prostatic tissue.

PENTALOGY OF CANTRELL IN TWINS: A CASE REPORT.

UNLABELLED: INTRODUCTION. Pentalogy of Cantrell is a very rare congenital multiple malformation with the poor outcome. Syndrome included the following pentad: abdominal wall defect, a defect of the lower sternum, anterior diaphragm defect, ectopia cordis, as well as a variety of intracardiac anomalies. CASE REPORT: In the fifteenth week of the twin pregnancy, ultrasound examination showed multiple anomalies in one, as well as the absence of the nose apex in the other twin. The autopsy of the fetuses was performed after the abortion had been approved by the Ethics Committee. The external examination of the fetus number one showed ectopic heart and omphalocele. The internal examination revealed the existence of sternum cleft, ectopic heart and part of the left lung outside the thoracic cavity, the presence of diaphragmatic hernia with protrusion of abdominal organs, heart with only one large vessel and the horseshoe kidney. The full expression of the Pentalogy of Cantrell was confirmed. The external and internal examination of the other twin showed just agenesis of the nose apex. CONCLUSION: Recognition and diagnosis of the syndrome is of the exceptional importance. Proper and timely diagnosis should contribute to good outcome of the pregnancy and delivery.

Rare solitary fibrous tumor of the stomach: A case report.

INTRODUCTION: Solitary fibrous tumors are rare soft tissue tumors of submesothelial origin and variable malignant potential. The most common localization is pleural, whereas only 0.6% are of extrapleural localization. Solitary fibrous tumor of the peritoneum, especially of gastric serosa is an extremely rare form of this tumor. CASE REPORT: We presented a 65-year-old female patient with solitary fibrous tumor of the stomach. Histopathological analysis of removed tissue showed the presence of tumor tissue built of spindle cells, elongated nuclei with moderately abundant cytoplasm. Cells were in a noncohesive arrangement, in smaller areas distributed in the form of palisade. There were amounts of hipocellular connective tissue, hyalinised, with small foci of dystrophic calcification. Mitoses were rare (less than 3/10 HPF). Blood vessels surrounded the connective tissue. Reviewed material did not contain elements of the parent organ. Immunohistochemically there were positivity on CD34 and vimentin, and negativity to S100, SMA, CD117, dezmin, and Ki-67 is < 2%. The change was diagnosed as a solitary fibrous tumor. CONCLUSION: Considering that benign solitary fibrous tumors of extrathoracic localizations are extremely rare neoplasms with unpredictable biological behavior and the possibility of recurrence, a long-term clinical and endoscopic follow-up on yearly basis of patients with this disease is recommended.

Mesenchymal hamartoma of the liver in adults--case report.

INTRODUCTION: Mesenchymal hamartoma of the liver is a benign lesion presenting as an enlarging abdominal mass in children less than 2 years of age. Fewer than 5% cases are present in individuals over 5 years of age, and this lesion is extremely rare in adults. It may affect the left or the right lobe of liver as a cystic or solid mass or both components may be present. The pathogenesis remains incompletely understood, but these lesions have generally been considered to represent a development abnormality in the bile duct plate formation. CASE REPORT: In this report, we present a case of a 44-year-old man who was surgically treated at the Department of Abdominal, Endocrine and Transplantation Surgery of the Clinical Center of Vojvodina due to cystic lesion in the liver segment IV that had been verified by computed tomography imaging diagnostics. The patient was sent from a smaller health center with the diagnosis of echinococcosis. After the adequate preparation of the patient, surgical excision ofthe liver cystic lesion was done. Once a thorough histological examination had been performed, the diagnosis of mesenchymal hamartoma was made. CONCLUSION: Mesenchymal hamartoma of the liver is a benign tumor resulting from abnormal, intra-uterine development of bile ducts and has a delayed clinical manifestation, thus this lesion appears to be related to the processes of maturation. It is potentially pre-malignant lesion presenting as a solid and/or cystic neoplasm. Symptoms, laboratory results and radiographic imaging are nonspecific and inconclusive, so surgical excision of the whole lesion is the imperative for the definitive diagnosis.

[Mucinous cystadenofiobroma of the ovary].

INTRODUCTION: Cystadenofibromas are tumors of the ovary which originate from the surface coelomic epithelium. Benign mucinous cystadenofibroma is a very rare form of these tumors, which consists of dominant stromal component of the connective tissue and one or more cysts. CASE REPORT: The case of a 62-year-old female with tumor of right ovary is reported in this paper. Histologically, tumor of the ovary had multilocular cystic formation, lined by a single-layer of mucoproductive cylindrical epithelium - endocervical type. In one area of tumor, the stromal component was abundant and made from partially hyalinised dense connective tissue. Mucinous cystadenofibroma was diagnosed on the basis of histological examination. Since the mucinous type of cystadenofibroma or adenofibroma is rare, this case has been chosen to be presented. CONCLUSION: Mucinous cystadenofibromas are differentially-diagnostically very similar to different malignant tumors and it is extremely important to make correct diagnosis of these neoplasms.

[Thoracoabdominal teratoma--rare location].

INTRODUCTION: Teratomas are tumours derived from pluripotent germ cells. They appear most frequently on ovaries. However, other locations are also possible: testicles, retroperitoneum, stomach, neck, and bottom of the mouth cavity. It is not very common to find teratomas in two different body cavities. CASE REPORT: A 51-year-old patient visited her doctor complaining of an intensive pain which suddenly appeared in her low back. The computed tomography scan of the abdomen was performed, which revealed cystic formation, 12.5 x 18 cm in size, with remarkable capsule visualization. It had paraaortic, subdiaphragmatic, retropancreatic and suprarenal left localisation with signs of compression and pancreatic dislocation. The cystic formation had a septated appearance filled with clear fluid in all its parts except at the bottom, where it was dense. The intra-operative finding was left retroperitoneal mass placed above the left kidney, dislocating surrounding structures and their infiltration could not be excluded for sure. A tumour mass passed through the diaphragmatic hiatus and continued in the left thoracic space. The cyst was only drained and the fenestration was performed. Three months later, the second operation was performed--splenectomy, left suprarenaladenectomy, left nephrectomy, aortic deliberalisation, the left diaphragmatic crus was opened and tumour mass was completely removed from the thoracic space. The histological examination showed mature teratoma with morphological characterisations of the skin, so the definite diagnosis was dermoid cyst. CONCLUSION: The patient presented with thoracoabdominal teratoma. Although without malignant transformation, the teratoma required splenectomy, supraadrenalectomy and nefrectomy due to its position and size.

[Morphological parameters of prostatic adenocarcinoma].

INTRODUCTION: Prostatic carcinoma is one of the most common malignancies in men and the second most common cause of cancer-related deaths, after lung cancer. However, many of these tumors are latent, of no biologic significance, and are recognized only as an incidental findig at autopsy. PROSTATIC CARCINOMA: HISTOLOGIC FEATURES: Prostatic carcinomas have multiple histologic patterns, which can easily be confused with benign lesions. The following histologic changes are associated with prostatic carcinomas. prostatic acini are close to one another and present with linear infiltrates in the fibromuscular tissue; cells lining the acini often consist of a single layer, and the basal cell layer is absent; prominent large eosinophilic nucleoli are usually present in malignant cells; nuclear hyperchromatism is rare and it depends on the quality of the tissue fixation; perineural invasion is often observed. Today, the Gleason grading system is the most commonly used (Gleason score). Immunohistochemistry is widely used in pathology and clinical diagnosis of prostatic carcinoma, metastases of prostatic origin in staging malignant tumors and in the prognosis.