Congenital Complete Tracheal Ring in a Neonate: A Case Report.

Abnormalities of the upper airway tract lead to congenital high airway obstruction and may complicate neonatal airway management in the delivery room. Congenital complete tracheal rings are a rare and unusual tracheal anomaly, usually presenting in the neonate or infant as respiratory distress. The clinical presentation can vary from almost asymptomatic patients to near-fatal airway obstruction. It may exist as an isolated entity, or in association with other congenital malformations, in particular, cardiac anomalies along with vascular rings and pulmonary slings. Other associated anomalies have also been reported, for example, chromosomal anomalies, malformation of other parts of the respiratory tract, esophagus and skeletal systems. Here, we report an extreme case of VACTERL/TACRD association presented with congenital complete tracheal ring, encephalocele, bilateral radial agenesis with absent thumbs, equinovalgus deformity on right foot, low-set ears and micrognathia.

Natural Killer-Like T-Cell Lymphoma Localized to the Terminal Ileum: Case Report.

Intestinal intraepithelial lymphocytes are non-organized lymphoid populations that are composed of heterogeneous subsets with diverse ontogeny and phenotypes, and the differential diagnosis is crucial. A 43-year-old male patient underwent an emergency laparotomy due to a perforated mass of the terminal ileum. A right hemicolectomy plus small bowel resection was performed. Histopathological examination showed medium to large cells with vesicular nuclei, including marked nucleoli with large, colorless cytoplasm. No signs of celiac disease were found in the adjacent mucosa. The tumor cells were immunohistochemically CD45+, CD3+, CD4+, CD8+, CD56+, Pan-Cytokeratin-, CD20-, CD79a-, CD5- and CD30-. Endomysial antibody and antigliadin antibody, IgM and IgG tests; and anti-Ebstein Barr virus latent membrane protein all proved negative. Finally, the histopathological diagnosis of tumor mass was natural killer-like T-cell lymphoma. Primary intestinal cytotoxic natural killer-like T-cell lymphoma is a rare entity, which is difficult to distinguish from other T-cell lymphomas. In addition to microscopic evaluation, immunohistochemical analysis and serological tests are essential to reach a definitive diagnosis.

Müllerian cyst of the vagina masquerading as a cystocele.

Müllerian cysts are usually small, ranging from 0.1 to 2 cm in diameter. Rarely, they may be enlarged and mistaken for other structures, such as a cystocele or urethral diverticulum. We report on a female with symptomatic vaginal wall prolapse, diagnosed as a vaginal Müllerian cyst, which was originally misdiagnosed as a cystocele. The mass was soft and could be compressed manually without difficulty. Perineal ultrasonography and cystoscopy revealed no relationship between the cyst and the lower urinary tract, suggesting independence of the lesion. We performed surgical treatment with complete excision of the mass via a vaginal approach under spinal anaesthesia. The pathology result confirmed a benign Müllerian cyst lined with mucinous and squamous epithelium. When evaluating an anterior vaginal cyst, assessment of the lesion via history taking and pelvic examination is important to confirm both lesion size and location. Perineal ultrasonography performed with an empty bladder is useful to differentiate such vaginal cysts and to define their communication, if any, with adjacent organs.

Importance of P53, Ki-67 expression in the differential diagnosis of benign/malignant phyllodes tumors of the breast.

BACKGROUND: Conventionally growth pattern, stromal overgrowth, stromal cellularity and stromal mitotic activity are the main parameters in the grading of phyllodes tumors (PTs). Recent studies revealed that both p53 and Ki-67 expressions are correlated with grade of PTs of the breast. Expression of hormone receptors and overexpression/amplification of HER2 has been studied in PTs to discover the roles of these markers as new treatment modalities. MATERIALS AND METHOD: We studied 26 PT cases. Seventeen benign and nine malignant PTs were re-evaluated as regards stromal cellularity mitotic activity, p53/Ki-67 expression rates and the relation between these parameters. Estrogen receptor and progesterone receptor (ER, PR) positivity were determined by counting nuclear staining in five high-power fields. Also, the presence of any HER2 staining and staining patterns were documanted. RESULTS: Stromal cellularity, mitotic rate, p53 and Ki-67 expression rates were all correlated with benign and malignant histologic subgroups (P = 0.000-0.001). Ki-67 and p53 expressions were statistically significantly correlated with histologic subgroups, stromal cellularity and mitotic rate (P < 0.005). ER and PR expressions in the epithelial component were not statistically significant between the two groups. HER2 showed different staining patterns in the epithelial component, and there was no staining in the stromal component. CONCLUSION: Ki-67 and p53 expression rates were statistically significantly correlated with grade of mammary PTs; therefore, they can be used in the determination of tumor grade, especially for the differential diagnosis of benign and malignant tumors. Malignant and benign tumors did not differ significantly in terms of hormone receptor and HER2 expression. HER2 expression showed different patterns in the epithelial component of the PTs.

An unusual case of trisomy 18 associated with paucity of bile ducts.

A case of neonatal cholestasis associated with Trisomy 18 (Edward's syndrome) is presented. A 3-day-old boy was referred to our clinic due to respiratory distress, elevated serum direct bilirubin levels, a systolic heart murmur, growth restriction and micrognathia. Liver biopsy and chromosomal analysis revealed paucity of intrahepatic bile ducts and Trisomy 18. Extrahepatic biliary atresia was reported in only a few patients with Trisomy 18. To our knowledge, we described for the first time a patient with Trisomy 18 and neonatal cholestasis associated with paucity of interlobular bile ducts.

[Immunohistochemical characteristics of triple negative/basal-like breast cancer]. / Triple-Negatif/Bazal Benzeri Meme Kanserinin Immünohistokimyasal Özellikleri.

OBJECTIVE: Triple-negative-breast-cancer that accounts for 10-20% of all breast carcinomas is defined by the lack of estrogen receptor, progesterone receptor, HER2 expression, and agressive clinical behavior. Triple-negative-breast-cancer is categorized into basal like and other types. The basal-like subtype is characterized by the expression of myoepithelial/basal markers. MATERIAL AND METHOD: We studied 41 immunohistochemically triplenegative- breast-cancer patients to determine EGFR, Cytokeratine 5/6, p53, Ki67, GCDFP-15 expression profiles, HER2 and Chromosome 17 centromere gene status by fluorescence-in-situ-hybridization method. RESULTS: Histological type was invasive ductal carcinoma in 90.2% of the tumors. p53, Ki67, GCDFP-15 mean positivity rates were 55.6%, 51.7%, and 3.2%, respectively. GCDFP-15 positivity was noted in 8 cases of which 6 were Cytokeratine 5/6 negative. The cut-off value for Cytokeratine 5/6 positivity was 5%. EGFR immunoreactivity was grouped into 0, 1+ as negative; 2+, 3+ as positive categories. Cytokeratine 5/6 was positive in 56,1%, EGFR was positive in 51.2% of the patients. The relation between Cytokeratine 5/6 and EGFR expression was statistically significant (p < 0.01). None of the cases showed HER2 amplification by fluorescence-in-situ-hybridization method. CONCLUSION: GCDFP-15 alone is not a useful marker to detect the metastasis of basaloid type breast cancers. Cytokeratine 5/6 and EGFR expressions showed correlation so these markers are reliable to diagnose basaloid type tumors with a 5% cut-off value.

Giant cell angiofibroma in unusual localization: a case report.

Giant cell angiofibroma (GCA) was initially described as a potentially recurrent tumor in the orbit of adults. However, it is now recognized that it can also present in other locations. The morphological hallmark is a richly vascularized patternless spindle cell proliferation containing pseudovascular spaces and floret like multinucleate giant cells. Our case was a 32-years-old female complaining of painless solitary nodule arising on the occipital region of the scalp, which was diagnosed as giant cell angiofibroma. We report the case because of its extremely rare localization.

Cytology of a giant adult-type rhabdomyoma of the tongue.

Adult rhabdomyoma is a rare primary benign tumour of striated muscle origin that almost exclusively presents in the head and neck region with predilection for male. We herein report a case of an adult rhabdomyoma in a 67-year-old male with cytologic features. The lesions was located in the tongue extended to the left parapharyngeal region. We discuss the cytological findings with a brief review of the literature on this entity.

Cholesteatoma of the concha bullosa: a case report.

INTRODUCTION: Cholesteatoma is a relatively common disease within the middle ear cavity, but rarely it manifests in the paranasal sinuses. There is, to the best of our knowledge, only one other published case of cholesteatoma inside the concha bullosa in the English language literature. CASE PRESENTATION: An 81-year-old Caucasian woman was admitted to our hospital complaining of nasal obstruction, headache and diplopia. After endoscopic and radiological evaluation a transnasal endoscopic approach was chosen. The diagnosis of cholesteatoma was established by histopathological evaluation of the mass inside the concha bullosa. CONCLUSION: Although it is rarely seen, cholesteatoma should be considered in the differential diagnosis of slow-growing and destructive paranasal masses.

Sarcomatoid carcinoma of the upper aerodigestive tract: an immunohistochemical analysis demonstrating latent Epstein-Barr virus in a subset of eight cases.

BACKGROUND: Sarcomatoid or spindle cell carcinomas are rare malignancies which are considered as a poorly differentiated variant of squamous cell carcinoma. Epstein-Barr virus (EBV) is associated with a wide variety of malignancies. MATERIALS AND METHODS: We examined the expression of EBV latent membrane protein-1 (LMP-1) and EBV EBNA-2 as well as the immunohistochemical profile of AE-1/AE-3, vimentin, desmin, CK 5-6, smooth muscle actin (SMA), p63, S-100, p53 and CD-117 with the clinicopathological correlation of eight patients of sarcomatoid carcinoma (SC) in the upper aerodigestive tract. RESULTS: Four cases showed EBV LMP-1 positivity (50%) and there was no EBV EBNA-2 positivity. However, the EBV LMP-1 results of our series could be considered only as a coincidental finding in the SCs of the upper aerodigestive tract. CONCLUSIONS: This finding supports the idea that further studies based on larger series might be helpful enlighting the role played by EBV in carcinogenesis of SC.