Noonan syndrome associated with central giant cell granuloma

We report a case of Noonan syndrome associated with central giant cell granuloma. The patient was a 101/2-year-old boy with the chief complaint of proptosis of the right eye. He also had various malformations such as short stature, webbed neck, pectus excavatum, cubitus valgus, pulmonary valve stenosis and patent foramen ovale, a characteristic face appearance and cryptorchidism and so on. Chromosome analysis showed a 46, XY karyotype. A computed tomographic scan and magnetic resonance imaging showed a mass originated from the lateral wall of the right maxillary sinus. The patient underwent Caldwell-Luc operation. Histological examination of the mass showed the characteristics of central giant cell granuloma. This case report describes a patient with the features of the recently described Noonan-like/multiple giant cell lesion syndrome.     

Four synchronous female genital malignancies: the ovary,cervix, endometrium and fallopian tube

Objective To present a unique case of a 63 year-old woman with coexistent adenocarcinoma of the ovary, endometrium, cervix and fallopian tube. Materials and methods A case report from a tertiary health center. Results A woman presenting with postmenopausal bleeding and abdominal distantion was assessed by endometrial biopsy and explorative surgery. The frozen section of the mass on the right adnex revealed malign mucinous carcinoma of the ovary. As usual, optimal debulking was performed as initial surgical staging procedure of ovarian cancer. The microscopic examination of the right ovary revealed a typical mucinous cystadenocarcinoma. Furthermore, the focal endometrial irregularity at the left uterine cornus turned out to be a well differentiated endometrial carcinoma of the endometrioid type with <1/3 myometrial invasion. The pale infiltrative lesion in the cervix also turned out to be an adenocarcinoma of the endocervical type with deep stromal invasion and areas of diffuse glandular dysplasia and in-situ glandular neoplasia at the periphery. Besides, several sections from the left fallopian tube uncovered diffuse dysplasia in the lining epithelium and a focus of adenocarcinoma with papillary and cribriform pattern. Discussion When compared with patients having metastatic lesions, most synchronous female malignancies are accompanied with early stage and low-grade with a more favorable prognosis. However, there is paucity of data for the exact criterion to distinguish primary tumors from metastatic lesions. In such cases, the validity of immunohistochemical and cloning studies are not clear.     

Gut Microbiota in Psoriasis

Psoriasis is a chronic inflammatory skin disease with autoimmune pathogenic characteristics and is caused by chronic inflammation, which results in uncontrolled keratinocyte growth and defective differentiation. The link between the gut microbiota and immune system regulation opened a novel angle to understand the pathogenesis of many chronic multifactorial diseases, including psoriasis. Current evidence suggests that modulation of the gut microbiota, both through dietary approaches and through supplementation with probiotics and prebiotics, could represent a novel therapeutic approach. The present work aims to highlight the latest scientific evidence regarding the microbiome alterations of psoriatic patients, as well as state of the art insights in terms of microbiome-targeted therapies as promising preventive and therapeutic tools for psoriasis.     

Spindle cell melanoma coexisting with chronic lymphocytic leukemia/small lymphocytic lymphoma: a rare collision tumor in multiple sites

A strong association has been reported between chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL) and malignant melanoma (MM). In rare cases of MM, lymphoid malignancies may be detected incidentally during sentinel lymph node biopsies. In this case, we found a unique collision of MM and CLL infiltration in the skin. An 88-year-old male patient presented with a mass on the nasal root. Histopathological examination of the skin biopsy specimen revealed a deeply infiltrative, atypical spindle cell proliferation in the background of a collagenous stroma. Accompanying this lesion, there were foci of monotonous lymphoid cell populations involving skin appendages. In the immunohistochemical studies, the spindle cells were diffusely positive for S100, and focally positive for Melan-A and HMB45; the lymphoid cells were positive for CD20, CD5, and Bcl-2 and negative for CD3, Bcl-6, CD10, and Cyclin D1. Histopathological and immunohistochemical findings were consistent with diagnoses of spindle cell melanoma and CLL. Interestingly, these two tumors together in their same morphological appearance were confirmed in a subsequent liver biopsy. Active skin surveillance of patients with CLL may be important to detect MM at an early stage that correlates with a better prognosis.     

Laparoscopic low anterior resection with total mesorectal excision for rectal cancer

Rectal resections are the only treatment with curative intent currently accepted world wide. When performed in elective circumstances, laparoscopic rectal excision is technically feasible in surgical approach of mid-rectal cancer in a considerable number of patients. There are many benefits of the laparoscopic approach to rectal resection such as short hospitalization, less pain, less postoperative complications and improved quality of life. However, one mandatory condition in laparoscopic resection of rectum includes complete excision of the rectum and mesorectum, generally ensuring a minimal distal margin of 2cm and circumferential radial clearance before performing a coloanal anastomosis. Here, we present a laparoscopic approach for rectal cancer treatment consisting in a wide resection of the rectum, including the entire fascia with the enclosed mesentery of the rectum.     

Factors associated with gastro-duodenal ulcer in compensated type 2 diabetic patients: a Romanian single-center study

IntroductionHelicobacter pylori infection is accepted as the leading cause of chronic gastritis, ulcer disease and gastric cancer, with an important impact on health care burden, especially in countries with a high prevalence of infection. The aim of the study was to investigate the influence of H. pylori infection, medication, associated medical conditions or social habits on endoscopic ulcer occurrence in the compensated type 2 diabetic population.Material and methodsTwo hundred and sixty type 2 diabetic patients investigated on endoscopy (57 patients with peptic ulcer and 203 controls) with a complete set of biopsies, demographic and medical data were enrolled.ResultsOn univariate regression analysis, H. pylori infection (42.1% vs. 35.5%, p = 0.359) or a history of peptic ulcer (61.4% vs. 61.6%, p = 0.981) was not a predictor for ulcer on endoscopy in the diabetic population, and heartburn was more frequent in diabetics without ulcer (21.2% vs. 8.8%, p = 0.033). Anemia was the best predictor for ulcer on endoscopy in both diabetics with (p < 0.001, OR = 4.77, 95% CI: 2.02–11.28) and without (p = 0.027, OR = 2.76, 95% CI: 1.10–6.91) chronic proton pump inhibitor (PPI) therapy. In diabetic patients on PPI more than 1 month anticoagulants – acenocoumarol or low-weight molecular heparin (p = 0.038, OR = 2.37, 95% CI: 1.04–5.40), low-dose aspirin 75–125 mg/day (p = 0.029, OR = 2.61, 95% CI: 1.08–6.28) and alcohol consumption (p = 0.015, OR = 2.70, 95% CI: 1.19–6.13) were predictors for ulcer on endoscopy.ConclusionsIn diabetic patients, anemia is the most important predictor for ulcer on endoscopy, but not H. pylori or digestive symptoms, while low-dose aspirin or anticoagulant therapy and alcohol consumption are the most important predictors for ulcer in diabetics on chronic proton pump inhibitor therapy.     

Microscopic nephrocalcinosis and hypercalciuria in nephrotic syndrome

Focal calcification is an occasional tubular abnormality seen in minimal-change nephrotic syndrome. Nephrocalcinosis was also reported in premature infants as a consequence of hypercalciuria resulting from long-term furosemide therapy. We describe 4 nephrotic children (3 minimal change, 1 diffuse proliferative glomerulonephritis) with transient hypercalciuria and intraluminal calcifications in renal histopathological specimens without radiologic evidence of renal calcification. These children were resistant to corticosteroid therapy and were receiving furosemide therapy along with albumin for management of oedema. Two of the children also had urinary infection. We were concerned that children with nephrotic syndrome are at risk for nephrocalcinosis, and urinary calcium and pH should be monitored carefully during prolonged furosemide use, especially in children with nephrotic syndrome with reduced initial responsiveness to corticosteroid therapy. HUM PATHOL 31:1363:1367.     

Endosalpingiosis of the axillary lymph nodes--primary or metastatic tumour?

The involvement of extra-abdominal sites by serous ovarian tumours of low malignant potential is extremely rare. In this paper we present the case of a 33 years old woman, diagnosed with atypical endosalpingiosis in the axillary lymph nodes before the diagnosis of a bilaterally ovarian serous tumour of low malignant potential. The occurrence of axillary lymph nodes involvement associated with serous tumours of the ovary could be explained by the presence of circulating serous cells that remained dormant for a period of time or by the development of an independent primary tumour from glandular inclusions in axillary lymph nodes. The recognition of the occurrence of axillary atypical endosalpingiosis in association with serous tumours of the ovary is important to avoid misdiagnosis. It is better to define these lesions as secondary serous papillary involvement.     

Changing incidence of anencephaly in the eastern Black Sea region of Turkey and Chernobyl

The incidence of neural tube defects (NTD) was 2.12 per 1000 births and that of anencephaly was 1.29 per 1000 births when we reviewed the 40,997 consecutive births between 1981 and 1986 in the major maternity hospital in the eastern Black Sea region of Turkey. There was a significant increase in the incidences of NTD and anencephaly after 1986 to 4.39 and 2.46 per 1000 births respectively (P less than 0.0001; P less than 0.005). Although this might be a coincidence, it may be due to the nuclear contamination of the region following the Chernobyl accident.