Management of Severe Hidradenitis Suppurativa.

Hidradenitis suppurativa (HS) is a devastating and disfiguring disease of the skin involving the terminal follicular epithelium within the apocrine-gland-bearing skin. We present an interesting case of a 58-year-old female who presented with a 10-year history of refractory HS of the gluteal, perineal, perianal, and groin region. She had been chronically treated with multiple antibiotics in the past with no improvement. The patient subsequently underwent surgical local excision with complex closure. Medical management alone may not be optimal, especially in refractory disease. Early and aggressive surgical intervention and interdisciplinary approach are needed for patients with chronic and advanced stage of HS.

An inducible intestinal epithelial cell-specific NHE3 knockout mouse model mimicking congenital sodium diarrhea.

The sodium-hydrogen exchanger isoform 3 (NHE3, SLC9A3) is abundantly expressed in the gastrointestinal tract and is proposed to play essential roles in Na+ and fluid absorption as well as acid-base homeostasis. Mutations in the SLC9A3 gene can cause congenital sodium diarrhea (CSD). However, understanding the precise role of intestinal NHE3 has been severely hampered due to the lack of a suitable animal model. To navigate this problem and better understand the role of intestinal NHE3, we generated a tamoxifen-inducible intestinal epithelial cell-specific NHE3 knockout mouse model (NHE3IEC-KO). Before tamoxifen administration, the phenotype and blood parameters of NHE3IEC-KO were unremarkable compared with control mice. After tamoxifen administration, NHE3IEC-KO mice have undetectable levels of NHE3 in the intestine. NHE3IEC-KO mice develop watery, alkaline diarrhea in combination with a swollen small intestine, cecum and colon. The persistent diarrhea results in higher fluid intake. After 3 weeks, NHE3IEC-KO mice show a ∼25% mortality rate. The contribution of intestinal NHE3 to acid-base and Na+ homeostasis under normal conditions becomes evident in NHE3IEC-KO mice that have metabolic acidosis, lower blood bicarbonate levels, hyponatremia and hyperkalemia associated with drastically elevated plasma aldosterone levels. These results demonstrate that intestinal NHE3 has a significant contribution to acid-base, Na+ and volume homeostasis, and lack of intestinal NHE3 has consequences on intestinal structural integrity. This mouse model mimics and explains the phenotype of individuals with CSD carrying SLC9A3 mutations.

Invasive Breast Carcinoma Tumor Size on Core Needle Biopsy: Analysis of Practice Patterns and Effect on Final Pathologic Tumor Stage.

INTRODUCTION: In the absence of nodal metastasis, pathologic tumor (pT) size remains one of the most important factors in adjuvant treatment decisions and patient prognosis in breast cancer. The aim of this study was to evaluate the effect of core needle biopsy (CNB) tumor size on final pT stage. MATERIALS AND METHODS: Our information system was searched to identify all patients who underwent excisional procedures for invasive breast carcinoma from January 1, 2014 to December 31, 2015. The tumor size on CNB and final excision, the number of cases in which the CNB size was larger, and the percentage of cases in which using the CNB tumor size changed the final pT stage were recorded. RESULTS: From 1380 primary breast excisions/mastectomies, a total of 870 cases were included. In 82 (9.4%) the CNB tumor size was larger (63 of 82 cases) or no residual tumor was identified on excision (19 of 82 cases). From these 82 cases, 40 (48.7%) were properly staged on the basis of CNB tumor size, 16 (19.5%) were not staged, and 26 (31.7%) were staged using the final excision tumor size. Change in stage occurred in 7 of these 26 patients. CONCLUSION: Our study revealed that in most cases, the largest tumor size is found in the excision/mastectomy specimen. However, in 9.4% (82 of 870), the CNB contains the most accurate tumor size for pT staging. On the basis of our results, including the largest linear tumor extent on the CNB report is recommended.

CDX2 protein expression compared to alcian blue staining in the evaluation of esophageal intestinal metaplasia.

AIM: To compare the sensitivity and specificity of CDX2 and alcian blue (AB) pH 2.5 staining in identifying esophageal intestinal metaplasia. METHODS: One hundred and ninty-nine biopsies from 186 patients were retrospectively reviewed and categorized as Barrett's esophagus (BE) (n = 108); non-Barrett's esophagus (NBE) (n = 48); columnar blue cells (CB) and esophageal glands (EG) (n = 43). The biopsies were stained with AB and immunostained for CDX2 using a mouse monoclonal antibody from Biogenex (clone CDX2-88) and the Ventana Discovery X automated immunostainer. The positive and negative predictive value of each group was used to determine the predictive power of CDX2 and AB in diagnosing intestinal metaplasia. RESULTS: All of the 108 BE biopsies (100%) were positive for AB and 102 of them (94.4%) were positive for CDX2. The six BE patients (5.6%) who failed to stain with CDX2 were found to have lost the focus of intestinal metaplasia upon deeper sectioning for immunostaining. Both AB and CDX2 were negative in 43 out of 48 (89.6%) NBE cases. Five NBE patients (10.4%) were falsely positive for AB due to the presence of EG and CB in these biopsies. These cases were all CDX2 negative. In addition, 5 AB negative NBE were found to be CDX2 positive. Based on these results the CDX2 immunostain had similar sensitivity but higher specificity (100% vs about 91%) than AB in detecting intestinal type metaplasia in these samples. Our data shows that CDX2 has a better PPV in detecting intestinal metaplasia as compared to AB (95.6% vs 71.5%, respectively). CONCLUSION: CDX2 has a better positive predictive value than AB in detecting intestinal metaplasia. CDX2 may be useful when challenged by gastro-esophageal biopsies containing mimikers of BE.

Gangliocytic paraganglioma of the appendix with features suggestive of malignancy, a rare case report and review of the literature.

We report a case of appendicial paraganglioma in a 40 year old female who presented with acute appendicitis and underwent laparoscopic appendectomy. To the best of our knowledge this is the first reported case of appendicial gangliocytic paraganglioma with features suggestive of malignancy in the modern literature. Van Eeden S. et al. reported the first case of appendicial paraganglioma in a 47 year old man who also presented with acute appendicitis. The appendectomy specimen showed a distended appendix with thickened wall, and a 1.3 cm mucosal based yellow lesion. Microscopically this lesion was centered in the submucosa and consisted of three different cell types: (a) epithelioid cells with pale eosinophilic finely granular cytoplasm containing bland oval nucleus with stippled chromatin, that form solid nests lying in a trabecular pattern and in formations reminiscent of 'Zellballen' as seen in paragangliomas (b) second type cells have large vesicular nuclei with prominent nucleoli and abundant cytoplasm that are scattered singly, (c) third type cells with bland elongated nuclei form broad fascicle and envelop the epithelioid and ganglion cells. Immunohistochemical analysis showed the epithelioid cell nests immunoreactive for synaptophysin and the ganglion-like cells and spindle Schwann cells to be immunoreactive for S100 protein, whereas all three cells populations were negative for CAM5.2 and Pancytokeratin. We do believe that an accurate diagnosis of Gangliocytic paraganglioma (GP) of the appendix was rendered, detailed microscopic examination of doubled hematoxylin and eosinophil stained sections as well as the immunohistochemical phenotype of the three components have been undertaken to confirm the diagnosis of GP.

Primary uterine cervix schwannoma: a case report and review of the literature.

Schwannoma (neurilemmoma) is a benign peripheral nerve sheath tumor that occurs in a wide variety of locations; however, its finding in the uterine cervix is extremely rare. We report a case of an incidental primary benign cervical schwannoma in a 48-year-old woman. In the English literature, a few cases of primary schwannoma of the cervix have been reported, which include seven cases of primary malignant cervical schwannoma and only two that are benign. These cases are reviewed in the following discussion.

The Persian, last 7-day, long form of the International Physical Activity Questionnaire: translation and validation study.

PURPOSE: To translate long form, interview-administered International Physical Activity Questionnaire (IPAQ) from English to Persian and evaluate its validity, reliability and reproducibility. METHODS: A forward-backward translation procedure was followed to develop the Persian version of the IPAQ. A total of 218 respondents (53.7% women, aged 22-76 yr) completed the Persian version in Tehran, Iran. To examine the test-retest reliability, 48 healthy volunteers completed the IPAQ twice during a 7-day period.. The PA indicators derived from the IPAQ were assessed for reliability and were compared with aerobic fitness and body mass index (BMI) for construct validity. RESULTS: In general, the questionnaire was received well and all domains met the minimum reliability standards (intra-class correlation [ICC]>0.7), except for Leisure-time physical activity (PA). Aerobic fitness showed a weak positive correlation with all of the PA results derived from the IPAQ. A significant correlation was observed between the IPAQ data for total PA and both aerobic fitness (r=0.33, P<0.001) and BMI (r=0.26, P<0.001). Performing a known group comparison analysis, the results indicated that the questionnaire was discriminated well between the subgroups of the study samples expected to be different in their physical activity. CONCLUSIONS: The Persian version of the long form, interview-administered IPAQ had an acceptable reliability and validity for assessing total PA in our Iranian sample of individuals. It may be a useful instrument for generating internationally comparable data on PA.