Paraneoplastic opsoclonus myoclonus syndrome associated with inflammatory myofibroblastic tumor in a pediatric patient

Opsoclonus myoclonus syndrome (OMS) is a rare neurological syndrome caused by a paraneoplastic autoimmune process that affects children with neuroblastic tumors. Treatment includes corticosteroids, intravenous gamma globulin (IVIG), rituximab, and other immunosuppressive therapies. Here, we describe a patient diagnosed with OMS associated with a localized inflammatory myofibroblastic tumor. The patient has no evidence of tumor recurrence following surgical resection with 8‐month follow‐up. The neurologic symptoms resolved with corticosteroids and IVIG. This case demonstrates that in children, neoplasms other than neuroblastoma may be associated with this paraneoplastic syndrome, and highlights the importance of evaluating patients with OMS for underlying malignancies.     

What is the Significance of the Circumferential Margin in Locally Advanced Rectal Cancer After Neoadjuvant Chemoradiotherapy?

Background

The circumferential resection margin (CRM) is highly prognostic for local recurrence in rectal cancer surgery without neoadjuvant treatment. However, its significance in the setting of long-course neoadjuvant chemoradiotherapy (nCRT) is not well defined.

Methods

Review of a single institution’s prospectively maintained database from 1998 to 2007 identified 563 patients with locally advanced rectal cancer (T3/T4 and/or N1) receiving nCRT, followed after 6 weeks by total mesorectal excision (TME). Kaplan-Meier, Cox regression, and competing risk analysis were performed.

Results

The authors noted that 75 % of all patients had stage III disease as determined by endorectal ultrasound (ERUS) and/or magnetic resonance imaging (MRI). With median follow-up of 39 months after resection, local and distant relapse were noted in 12 (2.1 %) and 98 (17.4 %) patients, respectively. On competing risk analysis, the optimal cutoff point of CRM was 1 mm for local recurrence and 2 mm for distant metastasis. Factors independently associated with local recurrence included CRM ≤1 mm, and high-grade tumor (p = 0.012 and 0.007, respectively). CRM ≤2 mm, as well as pathological, nodal, and overall tumor stage are also significant independent risk factors for distant metastasis (p = 0.025, 0.010, and <0.001, respectively).

Conclusion

In this dataset of locally advanced rectal cancer treated with nCRT followed by TME, CRM ≤1 mm is an independent risk factor for local recurrence and is considered a positive margin. CRM ≤2 mm was associated with distant recurrence, independent of pathological tumor and nodal stage.     

Common hair loss disorders

Hair loss (alopecia) affects men and women of all ages and often significantly affects social and psychologic well-being. Although alopecia has several causes, a careful history, dose attention to the appearance of the hair loss, and a few simple studies can quickly narrow the potential diagnoses. Androgenetic alopecia, one of the most common forms of hair loss, usually has a specific pattern of temporal-frontal loss in men and central thinning in women. The U.S. Food and Drug Administration has approved topical minoxidil to treat men and women, with the addition of finasteride for men. Telogen effluvium is characterized by the loss of "handfuls" of hair, often following emotional or physical stressors. Alopecia areata, trichotillomania, traction alopecia, and tinea capitis have unique features on examination that aid in diagnosis. Treatment for these disorders and telogen effluvium focuses on resolution of the underlying cause.     

Biaxial mechanical properties of porcine ascending aortic wall tissue

BACKGROUND AND AIM OF THE STUDY: Biaxial mechanical properties have been reported for porcine aortic valve leaflets, but not for the aortic root wall. These data are important for understanding the relationship between tissue material properties and function, providing a baseline for diseased tissue, and for providing a basis for numerical models of aortic mechanics. The study aim was to determine the biaxial material properties of porcine aortic root wall tissue. METHODS: Tissue samples (20 mm x 20 mm) were obtained from the aortic root walls of 18 pigs (anterior and posterior samples from each pig) and tested with a custom-built biaxial tensile testing apparatus. The data were fitted to the strain energy formulation: W = ?[a(long)E11(2) + a(circ)Ecc(2) + 2a(int)E11Ecc], where W is the strain energy, E11 = longitudinal strain, Ecc = circumferential strain, along, a(circ), and aint are the constants that were determined, and represent the longitudinal and circumferential elastic moduli, and interaction between the two axes, respectively. RESULTS: The root wall tissue was less stiff in the longitudinal direction (along = 115.8 +/- 8.4 kPa) than the circumferential direction (a(circ) = 169.9.3 +/- 7.4 kPa). As expected, there was mechanical interaction between the two axes (a(int) = 45.7 +/- 3.4 kPa). Additionally, anterior tissue samples were less stiff than posterior samples. All tissue samples exhibited a linear stress-strain relationship up to 40% strain, in contrast to aortic leaflet tissue, which was highly non-linear. CONCLUSION: These results demonstrated that the porcine aortic root wall tissue is an anisotropic material with linear elastic properties, in contrast to leaflet tissue. Additionally, the data suggest that a finite element model using an isotropic material as a basis for the aorta is insufficient for a physiologically accurate representation.     

Comparison of the systemic and intratumoral effects of tamoxifen and the aromatase inhibitor vorozole in postmenopausal patients with primary breast cancer.

PURPOSE: To determine biologic differences, if any, between presurgical endocrine treatment with an aromatase inhibitor (vorozole) and tamoxifen in patients with postmenopausal primary breast cancer. PATIENTS AND METHODS: Randomization was to 12 weeks of 2.5 mg of vorozole per day or 20 mg of tamoxifen per day, both orally. Clinical response was assessed monthly together with serum sex hormone binding globulin (SHBG), luteinizing hormone (LH), follicle-stimulating hormone (FSH), estrogens (E1, E2, and E1S), lipids, insulin-like growth factor-1 (IGF-1), and bone metabolites (CrossLaps CTx). Tissue samples for Ki67, apoptotic index (AI), estrogen receptor, and progesterone receptor were collected at 0, 2, and 12 weeks. RESULTS: Ki67 fell by 58% and 43% (means) at 2 weeks in the vorozole and tamoxifen patients, respectively (P =.13). In the vorozole group, the correlations of proportional changes in Ki67 at 2 weeks with tumor volume changes and clinical response at 12 weeks were not significant (P =.09) and marginally significant (P =.04), respectively. Serum lipids did not differ between groups. Serum levels of EI, E2, and E1S were suppressed markedly by vorozole, whereas levels of SHBG increased and LH and FSH fell significantly with tamoxifen. IGF-1 levels fell significantly with tamoxifen (P =.001) compared with the nonsignificant rise with vorozole. Twelve-week CTx values fell by 19% with tamoxifen (P =.006) and rose by 11% with vorozole (P =.15). CONCLUSION: The correlation with vorozole of Ki67 with volume and clinical response supports this as an intermediate marker. The nonsignificant effects on bone and lipid metabolism by the aromatase inhibitor may be important to consider for adjuvant and potential prevention strategies.     

Early growth response gene 1 (EGR1) is deleted in estrogen receptor-negative human breast carcinoma

BACKGROUND: Patients with estrogen receptor (ER)-positive and ER-negative breast carcinomas differ in terms of disease progression, treatment regimens, and prognosis. The mechanism underlying the biologic differences of the two groups is understood poorly. Array comparative genome hybridization (CGH) on breast carcinoma subtypes demonstrated a consistent association between loss in regions of chromosome 5q and ER-negative tumors. It was shown previously that early growth response gene 1 (EGR1) on 5q acts like a tumor-suppressor gene, with its expression repressed in breast carcinomas. METHODS: To test the hypothesis that EGR1 is deleted differentially in ER-negative versus ER-positive breast carcinomas, fluorescence in situ hybridization was employed in this study to determine the EGR1 deletion status in 50 breast carcinoma specimens. Deletion status was measured by the signal ratio of EGR1/chromosome 5. Linear regression was used to assess the results. RESULTS: The mean EGR1/chromosome 5 ratio for the ER-negative group was significantly lower compared with the same ratio for the ER-positive group (P < 0.001). Although grade alone was not significant for predicting the ratio, the interaction between ER status and grade was significant (P < 0.01): For ER-negative specimens, the higher the grade, the lower the EGR1/chromosome 5 ratio. CONCLUSIONS: The EGR1 gene appeared to be deleted in ER-negative human breast carcinomas. Egr-1 may contribute to the pathogenesis of ER-negative breast carcinomas versus ER-positive breast carcinomas.     

Use of methadone for the treatment of diabetic neuropathy

Editor's comment: This is the second Commentary of those that will appear from time to time describing treatments that may not have been validated by appropriate clinical trials but seem to be effective in diabetic patients based on small studies and/or extensive clinical experience. This one describes effective opioid treatment for those diabetic patients failing nonopioid therapies for painful neuropathy.     

Pulpotomy of deciduous teeth

Rather easy to perform, pulpotomy of the deciduous teeth is the most frequent endodontic treatment performed on children, but also the most controversial. Based on the amputation of the pulp chamber and the conservation of the inflammation-free root canals, the clinical results can be good, depending on the materials used. In this, calcium hydroxide (Ca(OH)2) as well as zinc-oxide-eugenol (ZOE) have been proven very inflammatory whereas Formocresol (FC) remains the reference even if its clinical toxicity is still reported in literature on a very controversial way. Nevertheless, this was sufficient to trigger and stimulate a search for alternatives, and led to the proposition to use ferric sulfate and even more recently MTA as new bases for the treatment of the pulp stumps after pulp chamber amputation.