Multiple Gastrointestinal Stromal Tumors, Malignant Peripheral Nerve Sheath Tumor and Atypical Neurofibromatous Neoplasm With Uncertain Biologic Potential Developing in A Single Patient With Neurofibromatosis Type 1 Syndrome.

Neurofibromatosis type 1 (NF1) is the most common human genetic disease. In these patients, the incidence of malignant peripheral nerve sheath tumors (MPNST) and gastrointestinal stromal tumors (GIST) is increased. A male patient in his forties with neurofibromatosis 1, presented with the coexistence of multiple GISTs located at intestinal and colonic mesentery, MPNST located at his leg and atypical neurofibromatous neoplasm with uncertain biologic potential located at colonic mesentery. By FISH, the MPNST harbored CDKN2A loss and recurred 1 year later. After reresection and radiotherapy, the patient is now disease-free without evidence of disease. Atypical neurofibromatous neoplasm with uncertain biologic potential is a newly defined entity, and it is important to discriminate it from low-grade MPNST, which requires more aggressive treatment methods. To the best of our knowledge, this is the first report describing synchronous GISTs, MPNST, and atypical neurofibromatous neoplasm with uncertain biologic potential developing in a single NF1 patient.

Endometrial Staining of CD56 (Uterine Natural Killer), BCL-6, and CD138 (Plasma Cells) Improve Diagnosis and Clinical Pregnancy Outcomes in Unexplained Infertility and Recurrent IVF Failures: Standardization of Diagnosis with Digital Pathology.

In women with unexplained infertility (UI) and recurrent in vitro fertilization (IVF) failures, the etiology is often unclear. Endometrial immune perturbations and the use of immune markers associated with these dysregulations are of great interest in the diagnosis and treatment of UI. However, reliable biomarkers and standardized quantification methods are lacking. Here, to address endometrial immune dysregulation in UI patients with recurrent IVF failures, we performed endometrial tissue sampling and immunostaining of CD56 (uNK), CD138, and BCL-6. Of these cases, 57.9% had positive CD56 in the endometrial stroma, while 46.1% had positive BCL-6 in the glandular epithelium, and 14.5% of the cases were found to be positive for CD138. Combined staining rates were 60.5%, 68.4%, and 71.05% for (CD56 or BCL-6), (CD56 or CD138), and (CD56, BCL-6, or CD138), respectively. There was a significant correlation between CD56 and BCL-6 positivity, while CD138 positivity was an independent parameter. After the recommended targeted therapy, pregnancy rates were found to increase from 58.5% to 61.6% and 73.8% in CD56-positive, (CD56- or BCL-6-positive), and (CD56-, BCL-6-, or CD138-positive) cases, respectively. Notably, a retrospective evaluation of digital pathology and light microscopy results showed a significant correlation. This study suggests that the examination of CD56, BCL-6, and CD138 in the same endometrial sample may be an effective method in determining the etiology of UI and reaching an early diagnosis and treatment options. Moreover, digital pathology can be used in the evaluation of CD56 and BCL-6 to provide objective, rapid, and reliable results.

Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation: A Case Report.

INTRODUCTION: A tumor with EWSR1/FLI fusion displaying extensive well differentiated neuroblastomatous differentiation is presented. CASE REPORT: A nine-year-old female patient had a thoracic vertebra 8 paraspinal mass. The lesion was resected incompletely. Histopathologically, a small round cell tumor with gangliomatous differentiation was seen. This was initially diagnosed as an intermixed ganglioneuroblastoma. In the completion surgery biopsy material, the small round cell component was more prominent. Immunohistochemistry for both samples showed membrane positivity for CD99 and nuclear positivity for NKX2.2 in the small round cell component of the tumor. Molecular analysis revealed EWSR1/FLI fusion. The diagnosis then considered a "Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation". CONCLUSION: Tumors with the EWSR1/FLI fusion may show neuroblastomatous differentiation. We chose to treat this as an Ewing Sarcoma (ES). Recognition of this phenomenon in ES cases may prevent a possible misinterpretation and a failure in oncologic treatment.

Dilated Minute Chambers in Laryngeal Vocal Fold Polyps: Histopathological and Ultrastructural Features.

In Reinke's space of human vocal fold, type III collagen forms a three dimensional network and this contains numerous minute chambers in between these fibers. These compartments are occupied by glycosaminoglycans and glycoproteins. In laryngeal fold lesions, such as Reinke's edema and vocal fold polyps, proteoglycan (PG)/hyaluronic acid (HA) components of extracellular matrix increased. We investigated the size and quantity of the minute chambers within Reinke's space, filled with PG/HA with the aid of transmission electron microscopy. Eight vocal fold polyps and 10 mucosal biopsies (as control group) were all evaluated by light microscopy and electron microscopy. We detected that PG/HA in extracellular matrix had been increased in vocal fold lesions when compared with control group, by Alcian Blue-pH 2.5 stain. The mean volume of the chambers in Reinke's space of normal larynx was measured as 0.040233 µm2 whereas the mean volume of these chambers in vocal fold polyps was measured as 6.420221 µm2. The difference between the volumes of these chambers in vocal fold polyps and in control group was statistically significant (P = 0.001). Within these chambers PG/HA were found and PG/HA filling these chambers were increased in vocal fold polyps. We think proteoglycan and glycosaminoglycans, especially HA, play an important role in determining biochemical properties of vocal fold lesions.

Mucosal Malignant Melanoma of Nasal Cavity Recurring a Year After Radiotherapy.

OBJECTIVE: Sinonasal mucosal malignant melanoma is a rare entity. In this report we present a nasal mucosal malignant melanoma case with its histopathological and clinical features. CASE REPORT: An 88-year-old female patient presented with epistaxis a month ago. Examination revealed a polypoid mass lesion of right nasal cavity originating from the middle concha. Her medical history revealed that she had been found to have a mass lesion in the right nasal cavity 15 months ago. She then underwent a punch biopsy from that lesion. A definitive histopathological diagnosis was not made but it was declared that the lesion had been a malignant epithelial tumor. The patient then had radiotherapy and the lesion showed complete regression. One year after completion of radiotherapy, the lesion recurred. Her last PET-CT showed multiple metastatic foci. Endoscopic excisional biopsy was performed for her recurrent lesion. Fragmented tumoral tissues were measured as 3,6x3x0,5 cm. Macroscopically the tumor was brownish in color. Histopathologically the tumor consisted of spindled and epitheloid cells. Immunohistochemically the tumor cells displayed positivity for S-100, HMB-45 and Melan-A. Findings were consistent with malignant melanoma. DISCUSSION: Mucosal malignant melanomas have a poor prognosis despite chemotherapy and radiotherapy. Five-year survival for sinonasal melanoma is reported to be lower than 35%. Sinonasal melanomas show a high recurrence rate. The immunohistochemical markers showing high specificity for malignant melanoma such as S-100, HMB-45 and Melan-A are used in order to reach a correct diagnosis. In our case the tumor showed recurrence and multiple metastases 1 year after completion of radiotherapy. For this recurrent tumor, chemotherapy and radiotherapy have been planned.

Hidroa vacciniforme.

Hidroa vacciniforme is a rare chronic photodermatosis with unknown cause in which lesions appear with exposure to sun. Early diagnosis and treatment of this disease which is characterized with recurrent vesicles and bullae on areas which are exposed to sun including the face and distal parts of the extremities is important, since it recovers by leaving scar. Here, a six-year old girl who presented to our clinic with eruptions on the face for three years and who was diagnosed with hidroa vacciniforme was presented in accompaniment of current information because of the rarity of the disease.

Rosai-Dorfman disease presenting as mediastinal lymphadenopathy: case report and review of the literature.

A 6-year-old boy presented with recurrent pneumonia and diarrhoea for 3 years. He had extensive mediastinal lymphadenopathy and atelectasis with low serum levels of IgA, IgG and IgG3. An inguinal lymph node biopsy demonstrated histological and histochemical features of Rosai-Dorfman disease. There was emperipolesis and histiocytes were immune-positive for S100 proteins. He responded to corticosteroids and regular infusions of immunoglobulins.

Endobronchial lipoma.

Endobronchial lipomas are rare benign tumours of the lung. The reported case was a 56-year-old man who visited the public hospital with complaints of chest pain and persistent cough. On bronchoscopy, a smooth-surfaced polypoid tumour obstructing the main bronchus in the left lobe was detected. The case was evaluated and surgical resection was performed. Histopathological investigation revealed that the tumour was an endobronchial lipoma; the tumour composed of mature fat tissue and was covered with bronchial epithelium.

Pyrrolidine dithiocarbamate attenuates the development of monocrotaline-induced pulmonary arterial hypertension.

We aimed to demonstrate the potential protective effects of pyrrolidine dithiocarbamate (PDTC) on monocrotaline (MCT)-induced pulmonary arterial hypertension (PAH). Adult male rats were randomly assigned to 4 groups: control group, MCT-treated rats only, MCT-injected rats treated with PDTC, and PDTC-treated rats only. Blood and tissue samples were collected after the sacrifice. Levels of malondialdehyde (MDA) were measured by using the thiobarbituric acid method. Total antioxidant status (TAS) was determined using a commercially available ImAnOx kit. A histopathological evaluation was accomplished by scoring the degree of severity. Endothelial damage of the main pulmonary artery was evaluated by immunohistochemical labeling of endothelial cells using anti-rat endothelial cell antigen 1 (RECA-1) antibody. MCT-induced right ventricular hypertrophy (RVH) was reduced significantly in the MCT+PDTC-treated group. MDA levels were significantly lowered in the MCT+PDTC-treated group. TAS was significantly higher in the MCT+PDTC-treated group when compared with the rats with PAH. Histopathological examination demonstrated that PDTC treatment reduced the development of inflammation, hemorrhage and congestion, and collagen deposition. In conclusion, PDTC attenuated PAH and protected pulmonary endothelium in rats administered MCT. These findings suggest that PDTC treatment may provide a new effective therapeutic approach in the treatment of PAH.

Utility of semiquantitative parameters to differentiate benign and malignant focal hepatic lesions.

OBJECTIVE: Any distinction and the usefulness of semiquantitative parameters derived from dynamic-contrast-enhanced (DCE) MRI obtained with extracellular gadolinium contrast agent in hemangiomas, hepatocellular carcinomas (HCC) and metastases of the liver was studied prospectively. METHODS: Seventy-four focal liver lesions (consisted of 34 hemangiomas, 23 HCC, and 17 metastases) of the 37 patients underwent DCE-MRI (six phases). Functional coloured maps and subsequently semiquantitative parameters were obtained using the FuncTool. Maximum and average (avg) values of mean time to enhancement (MTE), positive enhancement integral (PEI), time to peak (TP), maximum slope of increase (MSI), maximum slope of decrease (MSD) values were measured by placing the region of interest. The diagnosis of HCC and metastases were proven histopathologically and/or clinically. RESULTS: The mean values of the paremeters were: In hemangiomas, avgMTE: 271.2 ± 4.7; avgPEI: 840.3 ± 77.3; avgTP: 146.6 ± 13.1; avgMSI: 999.1 ± 108.4; avgMSD: 254.1 ± 30.4. In HCC, avgMTE: 246 ± 3.6; avgPEI: 424.9 ± 31.6; avgTP: 132.8 ± 9.5; avgMSI: 484.1 ± 36.5; avgMSD: 109.1 ± 13.3. In metastases, avgMTE: 248.1 ± 8.2, avgPEI: 453.9 ± 39.6; avgTP: 142.8 ± 13.9; avgMSI: 472.6 ± 50.4 and avg MSD: 200.1 ± 38.2. Both maximum and avg values of MTE, PEI, MSI, and MSD were significantly higher in hemangiomas (P<.05). The most significant difference was found in avgPEI with 82.1% sensitivity and 67.6% specificity when 570 cutoff value was considered. The values however were not significantly different among HCC and metastases (P>.05). CONCLUSIONS: Semiquantitative DCE-MRI parameters provide useful, complementary, and quantitative information. This technique increases diagnostic value of extracellular gadolinium contrast agent to characterize focal liver lesions and may be useful for follow-up after local-regional therapies.

Association of speculum lubrication with pain and Papanicolaou test accuracy.

PURPOSE: To determine the effects of lubrication of the vaginal speculum before insertion during a Papanicolaou test on perceived pain and quality of the cytology specimen. METHODS: Four hundred eligible women participating in cervical cancer screening and an early detection program were randomized with respect to lubricant gel use before speculum insertion. Perceived pain during speculum insertion and cytology results were assessed in study groups. RESULTS: The mean age of the patients was 45.7 years (range, 23-77 years). Pain score of the NO GEL group was significantly higher than that of the GEL group (2.3 and 1.6, respectively; P < .05). For premenopausal women, lubricant gel use significantly reduced the pain scores of the participants (P < .05). Cytological interpretations of the Papanicolaou test specimens were comparable among all groups. CONCLUSIONS: During the collection of Papanicolaou test specimens, lubrication of the vaginal speculum with a small amount of K-Y Jelly (a water-soluble lubricant gel) decreases the pain associated with insertion of the vaginal speculum among postmenopausal women without obscuring the cytological interpretation of conventional or liquid-based cytology. In women of reproductive age, lubrication of the speculum with K-Y Jelly does not cause a meaningful effect with respect to perceived pain.

Multiple metastatic renal cell carcinoma isolated to pancreas.

Renal cell carcinoma (RCC) metastases to the pancreas are reported to be rare. Isolated multiple pancreatic metastases are even rarer. We report a 68-year-old asymptomatic male patient who presented with multiple metastatic nodular lesions in the pancreas demonstrated by computerized tomography 3.5 years after radical nephrectomy performed for clear cell RCC. Spleen-preserving total pancreatectomy was performed. Gross examination revealed five well-demarcated tumoral nodules in the head, body and tail of the pancreas. Histopathological examination revealed clusters of epithelial clear cells, immunohistochemically positive for CD10 and vimentin, and negative for CK19 and chromogranin, supporting a diagnosis of metastatic RCC. The patient has remained well at 29 months post-resection, in agreement with recent experience that radical resection for multiple isolated metastatic nodular lesions can achieve improved survival and better quality of life.

Female genital system tuberculosis: a retrospective clinicopathological study of 1,548 cases in Turkish women.

PURPOSE: The incidence of tuberculosis (TB) has increased worldwide in the past decade and it still remains an important global public health problem. METHOD: A retrospective clinicopathological study of 1,548 cases of female genital tuberculosis between 1940 and 2011 was conducted. RESULTS: The mean age of the cases was 29.49 years. Involvement of the endometrium was noted in 1,073, fallopian tubes in 164, cervix in 157, and 154 had multiple organ involvement. Clinically, 115 cases (7.4%) were diagnosed as having primary infertility and 12 cases (0.8%) as having secondary infertility. There was a coexistent carcinoma in 1.5% of the cases. Peritoneal tuberculosis in 21 cases and tuberculous lymphadenitis in 7 cases were seen as well. CONCLUSION: The clinicopathological criteria of female genital tuberculosis in the different organs are described, and special attention is paid to infertility associated with tuberculous lesion, and awareness of the fact that the disease is still with us is thus particularly important.

Xanthogranulomatous sialadenitis clinically mimicking a malignancy: case report and review of the literature.

BACKGROUND: Xanthogranulomatous tissue reaction is a well-documented process that is most common in kidney. There are other uncommon sites being documented as case reports in the literature. We would like to describe the clinicopathologic findings in a case of xanthogranulomatous sialadenitis that involved the parotid gland, which was clinically thought to be a tumoral mass, and compare it with the 4 previously reported cases. CASE REPORT: A 52-year-old man presented with a left parotid mass. Fine needle aspiration biopsy was consistent with Warthin's tumor. The mass lesion was excised. DISCUSSION: The lesion measured 2.5 × 1.5 × 1.5 cm. Microscopic examination revealed sheets of foamy macrophages centrally admixed with neutrophils, eosinophils, lymphocytes, plasma cells and scattered giant cells indicating a xanthogranulomatous reaction. As a conclusion xanthogranulomatous tissue reaction can mimic neoplasms.

Could Riedel's thyroiditis be subacute thyroiditis? A case report.

Our purpose in this paper is to make a case for the need to question whether Riedel's thyroiditis is in fact a rare variety of subacute thyroiditis. Total thyroidectomy was performed on a patient with a huge thyroid mass. The histopathological diagnosis was Riedel's thyroiditis with classical lymphoplasmacytic infiltration destroying thyroid tissue and invading perithyroidal soft tissue. However, in addition, histopathology showed epithelioid granulomas containing multinuclear giant cells. The existence of these giant cells led us to place this case somewhere between Hashimoto's thyroiditis and subacute thyroiditis (de Quervain's). Since giant cells are seen in the histopathology of subacute thyroiditis, we propose that the possibility of Riedel's thyroiditis being a form of subacute thyroiditis be observed and studied.

Occult papillary thyroid carcinoma: prevalence at autopsy in Turkish people.

The epidemiological features of occult papillary thyroid carcinoma (OPTC) at autopsy have not previously been reported in Turkish people. This study provides data on the prevalence of OPTC in people from the Marmara region in Turkey. The study includes thyroid glands from 93 male and 15 female cadavers between 18 and 80 years of age. The thyroid glands were removed, fixed and sectioned at 2-mm intervals. The sections were stained with hematoxylin and eosin, and were examined by a single pathologist. A total of 108 thyroid glands were examined. Macroscopic lesions were found in 36 (33.3%) glands (95% confidence interval=24-42). OPTC was found in four (3.7%) cases (95% confidence interval=1.0-7.5). It was concluded that in the Turkish people from the Marmara region, the prevalence of OPTC found at autopsy was relatively low. No age or sex predilection was detected.

Specificity and sensitivity of differentiation antigens in superficial soft tissue tumors: comparison of SMA, calponin, H-caldesmon, C-kit, PLAP and HPL.

We examined the expression pattern of smooth muscle actin (SMA), h-caldesmon (HCD), calponin (CALP), placental alkaline phosphatase (PLAP) and human placental lactogen (HPL) in benign and malignant spindle cell superficial soft tissue tumors in order to determine the role of these markers in differential diagnosis. Archival tissue from 38 patients with superficial smooth muscle cell and so-called fibrohistiocytic tumors (8 benign fibrous histiocytomas (BFHs), 6 dermatofibrosarcoma protuberans (DFPT), 9 malignant fibrous histiocytomas (MFHs), 9 leiomyomas (LMs) and 6 leiomyosarcomas (LMSs)) were immunostained with antibodies against SMA, HCD, CALP, PLAP and HPL. smooth muscle cell (SMC) tumors showed significantly high immunopositivity for HCD than that of so-called fibrohistiocytic tumors (p is less than or equal to 0.05) but 1/3 of DFPT and MFH cases and half of BFH cases also showed HCD immunopositivity; thus, this difference is debatable and not highly discriminative as expected. All tumor groups showed 100% immunopositivity for CALP. SMC tumors displayed significantly stronger and more widespread immunostaining pattern for PLAP than so-called fibrohistiocytic tumors (p < 0.05). Superficial soft tissue tumors did not express c-kit. In conclusion, HCD and PLAP can be used as ancillary immunomarkers in differential diagnosis of SMC tumors (Tab. 2, Fig. 7, Ref. 37).

Ovarian hemangioma occurring synchronously with contralateral mature cystic teratoma in an 81-year-old patient.

PURPOSE: Ovarian hemangiomas are seen rarely. We present a case of an ovarian hemangioma occurring synchronously with contralateral mature cystic teratoma. CASE HISTORY: An 81-year-old woman presented with hypertension and hyponatremia. In ultrasonographic evaluation a pelvic mass was found located at the left ovary. Histologically, a mature cystic teratoma measuring 9.5 × 9 × 8 cm was seen in left ovary. In the right ovary an incidental vascular lesion measuring 3.5 × 1.5 × 1 cm was observed. Final histopathological examination of this lesion demonstrated a hemangioma of cavernous type. CONCLUSION: To the best of our knowledge, this is the first ovarian hemangioma case occurring synchronously with contralateral mature cystic teratoma.

Central nervous system complications of diabetes in streptozotocin-induced diabetic rats: a histopathological and immunohistochemical examination.

Diabetes mellitus is a common, potentially serious metabolic disorder. Over the long term, diabetes leads to serious consequences in a number of tissues, especially those that are insulin insensitive (retina, neurons, kidneys). It also causes a variety of functional and structural disorders in the central and peripheral nervous systems. We investigated whether neurodegenerative changes were observable in the hippocampus, cortex, and cerebellum after 4 weeks of streptozotocin (STZ)-induced diabetes in rats and the effect(s) of melatonin. Male Wistar rats (n = 32) were divided into four groups (n = 8 each): untreated controls, melatonin-treated controls, untreated diabetics, and melatonin-treated diabetics. Experimental diabetes was induced by a single dose of STZ (60 mg/kg, intraperitoneal (ip)). For 3 days before the administration of STZ, melatonin (200 microg/kg/day, ip) was injected and continued for 4 weeks. Sections of hippocampus, cortex, and cerebellum were stained with hematoxylin and eosin and examined using light microscopy. In addition, brain tissues were examined immunohistochemically for the expression of glial and neuronal markers, including glial fibrillary acidic protein (GFAP), neuron-specific enolase (NSE), and heat shock protein-70 (HSP-70). No neurodegenerative changes were observed in the hippocampus, cortex, or cerebellum of the untreated diabetic group after 4 weeks compared with the other groups. We did not observe any change in GFAP, NSE, or HSP-70 immunostaining in the brain tissues of STZ-induced diabetic rats. In summary, after 4 weeks of STZ-induced diabetes in rats, no degenerative or immunohistochemical changes were detected in the hippocampus, cortex, or cerebellum.