Indolent T-lymphoblastic proliferation involving hepatocellular carcinoma-presentation in novel settings and comprehensive review of literature.

Indolent T-lymphoblastic proliferation (iT-LBP) is a rare, non-clonal, extrathymic lymphoid proliferation with an immature T cell phenotype, indolent clinical course, and excellent prognosis. Although their pathogenesis is unclear, they are reported to be associated with Castleman disease, follicular dendritic cell tumors/sarcomas, angioimmunoblastic T cell lymphoma, hepatocellular carcinoma (HCC), myasthenia gravis, and acinic cell carcinoma. There are around 51 reported cases of iT-LBP in the literature. Recognition and accurate diagnosis of this entity is critical as it shares morphologic and immunophenotypic features with an aggressive malignancy-acute T cell leukemia/lymphoma (T-ALL). IT-LBP in HCC post-liver transplant and in metastatic sites has not been reported in the literature. Two case reports of patients presenting with recurrent and metastatic HCC in post-liver transplant settings are described. A 50-year-old man with an end-stage liver disease with HCC underwent liver transplant. A year later, he developed pulmonary metastasis with associated iT-LBP. A 69-year-old man underwent liver transplant for end-stage liver disease and HCC. Eighteen months later, he developed recurrent HCC in the transplanted liver and omental metastasis; both sites showed HCC with iT-LBP. iT-LBP in both patients expressed TdT, CD3, and CD4 and lacked CD34 and clonal T cell receptor gene rearrangements. On retrospective review, the pre-transplant HCC specimens lacked iT-LBP. We present two cases of iT-LBP associated with HCC in novel settings-in post-liver transplant patients and in recurrent/metastatic sites of HCC. In addition, a comprehensive literature review of clinical, histological, and immunophenotypic characteristics of reported cases of iT-LBP is presented.

Distortions but not Definitive Peaks by Capillary Serum Protein Electrophoresis Indicate Monoclonal Gammopathy of Undetermined Significance rather than Multiple Myeloma.

OBJECTIVE: Capillary electrophoresis of serum proteins demonstrates occasional distortions. Distortions or peaks in the gamma, beta, and alpha-2 zones may represent monoclonal gammopathy. In this study, we investigated if such distortions are associated with monoclonal gammopathy of undetermined significance (MGUS) or multiple myeloma. METHODS: Consecutive serum protein electrophoresis results were reviewed and immunofixation studies were recommended on specimens exhibiting distortions or distinct peaks in the gamma, beta or alpha-2 zones. RESULTS AND DISCUSSION: Of the 471 cases, we observed distortions in 101 cases. In the immunofixation studies, 17.8% of cases had a diagnosis of MGUS, but none contained multiple myeloma. CONCLUSIONS: We conclude that distortions in serum capillary electrophoresis may be associated with MGUS, but not multiple myeloma.

The risk for malignancy using the Milan salivary gland classification categories: A 5-year retrospective review.

AIMS: Since the six-tier Milan salivary gland classification has been introduced, there are very limited studies in literature reporting the risk stratification of the Milan classification. METHODS: We retrospectively classified a total of 285 salivary gland cytology cases into Milan reporting categories; there were 23 (8.1%) nondiagnostic, 48 (16.8%) nonneoplastic, 19 (6.7%) atypia of undetermined significance (AUS), 138 (48.4%) benign neoplasm, 13 (4.6%) neoplasm of uncertain malignant potential (NUMP), 8 (2.8%) suspicious for malignancy, and 36 (12.6%) malignant. Almost 110 cases (38.6%) had surgical follow-up resections. RESULTS: The overall risk for malignancy (ROM) was 12.5% for AUS, 3.2% for benign neoplasm, 72.7% for NUMP, and 100% for the suspicious for malignancy and malignant. The ROM for nondiagnostic and nonneoplastic categories was not representative due to limited follow-up resections. The salivary cytology had sensitivity, specificity, positive predictive value, and negative predictive value of 93.0%, 100%, 100%, and 46.2% for neoplasm and 82.3%, 95.8%, 90.3%, and 92.0% for malignant. CONCLUSION: Our study supports the adaptation of the six-tier Milan classification for reporting salivary gland cytology, as well as emphasizing the utility of the NUMP category.

Proteomics Analysis of FLT3-ITD Mutation in Acute Myeloid Leukemia Using Deep Learning Neural Network.

Deep Learning can significantly benefit cancer proteomics and genomics. In this study, we attempted to determine a set of critical proteins that were associated with the FLT3-ITD mutation in newly-diagnosed acute myeloid leukemia patients. A Deep Learning network consisting of autoencoders formed a hierarchical model from which high-level features were extracted without labeled training data. Dimensional reduction reduced the number of critical proteins from 231 to 20. Deep Learning found an excellent correlation between FLT3-ITD mutation with the levels of these 20 critical proteins (accuracy 97%, sensitivity 90%, and specificity 100%). Our Deep Learning network could hone in on 20 proteins with the strongest association with FLT3-ITD. The results of this study allow for a novel approach to determine critical protein pathways in the FLT3-ITD mutation, and provide proof-of-concept for an accurate approach to model big data in cancer proteomics and genomics.

Treatment Patterns, Outcomes, and Patient Satisfaction of Primary Epidermally Limited Nonmelanoma Skin Cancer.

BACKGROUND: Epidermally limited nonmelanoma skin cancer (ELNMSC) (superficial basal cell carcinoma [SBCC] and squamous cell carcinoma in situ [SCCIS]) is common. Data on outcomes and patient satisfaction are lacking. OBJECTIVE: To examine treatment efficacy and satisfaction in ELNMSC patients. PATIENTS AND METHODS: Retrospective cohort study of adults with primary SBCC or SCCIS. A 25% random subset completed a satisfaction questionnaire. RESULTS: Five hundred and fifty patients with 227 SBCC and 451 SCCIS were included; 329 tumors (49%) were treated with Mohs micrographic surgery (MMS) and 349 (51%) with non-MMS (imiquimod [n = 26], 5% 5-fluorouracil [n = 234], ingenol mebutate [n = 32], or cryotherapy [n = 57]). Five-year recurrence-free survival was high in both groups, with MMS having a small but statistically significant advantage (99% vs 95%, p = .004). More MMS patients were willing to undergo treatment again (97% vs 86%, p = .024). Dissatisfaction was mostly due to prolonged treatment course and pain associated with non-MMS treatments. CONCLUSION: Surgical and nonsurgical treatments for primary ELNMSC have low recurrence rates, though cure rate and patient satisfaction are higher with MMS. Treatment choice for epidermal NMSC may be guided through patient preferences regarding ability to comply with topical treatment, out-of-pocket costs, desire to treat surrounding field disease, and desire to avoid a surgical scar.

Ablative Fractional Laser-Assisted Topical Fluorouracil for the Treatment of Superficial Basal Cell Carcinoma and Squamous Cell Carcinoma In Situ: A Follow-Up Study.

BACKGROUND: The authors previously reported the safety and short-term efficacy of ablative fractional laser (AFXL)-assisted delivery of topical fluorouracil in the treatment of superficial basal cell carcinoma (sBCC) and squamous cell carcinoma in situ (SCCis). OBJECTIVE: This follow-up study was conducted to assess whether tumor clearance was sustained in this cohort of patients at >9 months post-treatment. METHODS: Thirty primary sBCC or SCCis <2 cm on the trunk or extremities were treated with AFXL and a single application of topical 5-fluorouracil 5% under occlusion for 7 days. Among the 26 patients who achieved tumor clearance at 4 to 8 weeks post-treatment, 20 patients presented for this follow-up study and underwent shave biopsy to confirm histologic clearance. Mean follow-up time was 15 months. RESULTS: Considering those who had persistent tumor at 4 to 8 weeks post-treatment and those who presented for follow-up at >9 months post-treatment, overall treatment success was 79% (95% confidence interval: 67%-96%), with 92% (11/12) for SCCis and 67% (8/12) for sBCC. Neither the tumor location nor size significantly impacted treatment outcome (p = .96 and 0.87, respectively). CONCLUSION: Ablative fractional laser-assisted topical fluorouracil is a reasonable noninvasive treatment option for primary SCCis and sBCC, especially for lesions located in areas where self-application is not possible, or when clinician-administered therapy is preferred.

A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.

BACKGROUND: To our knowledge, no systematic review of dermatofibrosarcoma protuberans (DFSP) outcomes based on the presence or absence of fibrosarcomatous (FS) change has been performed. OBJECTIVE: We sought to compare available outcome data for DFSP versus DFSP-FS. METHODS: The literature was searched for DFSP and DFSP-FS reports with outcome data (local recurrence, metastasis, or death from disease). Chi-square tests were calculated to determine whether DFSP and DFSP-FS significantly differed in risk of local recurrence, metastasis, and death from disease. RESULTS: In all, 24 reports containing 1422 patients with DFSP and 225 with DFSP-FS are summarized. Risk of local recurrence, metastasis, and death from disease in DFSP-FS was significantly higher as compared with DFSP (local recurrence 29.8% vs 13.7%, risk ratio 2.2 [95% confidence interval 1.7-2.9]; metastasis 14.4% vs 1.1%, risk ratio 5.5 [95% confidence interval 4.3-7.0]; and death from disease 14.7% vs 0.8%, risk ratio 6.2 [95% confidence interval 5.0-7.8]). There was no significant difference in DFSP-FS outcomes based on proportion of FS change within tumors. LIMITATIONS: This study is based on previously reported data from different hospitals with no uniform process for reporting FS change. The impact of confounders (age, immune status, tumor location, treatment) could not be evaluated because of limited data. CONCLUSION: Based on available retrospective data, risk of metastasis and death is elevated in DFSP-FS as compared with DFSP. Even a low degree of FS involvement portends worse outcomes.

The Z-advancement flap for reconstruction of lateral nasal tip and medial alar defects.

BACKGROUND: Reconstruction of lateral nasal tip and medial alar defects is challenging. Contour, symmetry, and skin texture of the nose, along with adequate nasal airway patency, should be preserved. The Z-advancement flap is a novel reconstruction technique designed for optimal cosmesis and function. OBJECTIVE: To evaluate the aesthetic and functional outcomes of Z-advancement flap nasal reconstruction. MATERIALS AND METHODS: Twenty-nine consecutive patients with defects 1 cm or less in diameter on the lateral nasal tip or medial ala underwent Z-advancement flap repair. Patients completed a survey assessing cosmesis and airway patency. Three physicians evaluated standardized photographs on visibility of scar lines, erythema and telangiectasia, and contour and symmetry of the ala and nostril opening. RESULTS: Twenty-eight (96%) patients completed survey questionnaires. All patients were satisfied with the look and feel of their reconstructed nose. Twenty-four (86%) saw no visible scar or abnormality. Postoperative photographs were available for review in 19 (66%) patients. In 95% to 96% of physician ratings, scars were invisible or visible only on close inspection, and alar symmetry was unchanged or only slightly altered. In 88%, nostril opening symmetry was unchanged or slightly altered. CONCLUSIONS: The Z-advancement flap preserves aesthetic subunits of the nose to produce excellent cosmesis and patient satisfaction for defects of the lateral nasal tip or medial ala 1 cm or less in diameter.

Perioperative antibiotic use of dermatologic surgeons in 2012.

BACKGROUND: Recommendations for antibiotic prophylaxis in dermatologic surgery have been established, but there is variability in perioperative antibiotic use of dermatologists. Authoritative guidelines have shifted away from routine use of antibiotic prophylaxis because there is no conclusive evidence that antibiotic use reduces risk of infective endocarditis or prosthetic joint infection. OBJECTIVE: To determine current practices of perioperative antibiotic use of Mohs-trained surgeons and to compare patterns of use with updated administration guidelines. METHODS AND MATERIALS: A survey was sent to American College of Mohs Surgery members in 2012. The main outcome measures were survey responses relating to demographic characteristics, experience with postoperative infection, familiarity with antibiotic guidelines, perioperative antibiotic practices, attitudes regarding antibiotic use, and antibiotic selection. RESULTS: Most survey respondents are familiar with the Antibiotic Prophylaxis in Dermatologic Surgery Advisory Statement 2008, but respondents give antibiotics for more indications than are recommended. Although not recommended, a high percentage reported giving antibiotics to patients at high risk of infective endocarditis or joint infection even when surgery does not breach oral mucosa or involve infected skin. CONCLUSION: Dermatologic surgeons overuse perioperative antibiotics for prevention of surgical site infection, infective endocarditis, and prosthetic joint infection based on current recommendations.

The appearance of pili annulati following alopecia areata.

Pili annulati is a rare autosomal-dominant hair shaft abnormality. It is characterized by alternating light and dark bands along the shaft due to air-filled cavities within the cortex of the hair shaft. Alopecia areata has been previously described as a common association with pili annulati, with improvement in alopecia areata coinciding with resolution of pili annulati. We report the case of a patient with a history of alopecia areata and alopecia universalis who developed the characteristic banded hair of pili annulati upon resolution of her alopecia areata. We provide direct microscopic examination of postregrowth hairs compared to normal and cross-polarized light microscopy.

Musculoskeletal disorders and ergonomics in dermatologic surgery: a survey of Mohs surgeons in 2010.

BACKGROUND: Dermatologic surgeons perform numerous procedures that put them at risk of developing work-related musculoskeletal disorders. OBJECTIVE: To study the prevalence of work-related musculoskeletal disorders and role of ergonomics in dermatologic surgery. METHODS AND MATERIALS: A survey study was sent to members of the American College of Mohs Surgery in 2010. The main outcome measures were survey responses relating to surgeon demographics, musculoskeletal symptoms, workstyle habits and attitudes, and ergonomic practices. RESULTS: Ninety percent of respondents reported some type of musculoskeletal symptoms or injuries. The most common complaints were neck, lower back, shoulder, and upper back pain. Most respondents were not using ergonomic modifications in their practice. CONCLUSION: Mohs surgeons have a high prevalence of musculoskeletal disorders. Symptoms present early and persist throughout the careers of Mohs surgeons. The nature of the occupation leaves the surgeon vulnerable to injury. Ergonomic modifications in behavior and workplace are recommended to reduce pain and injury to surgeons.