Giant papillary hemangioma-A rare tumor with an exceptional size.

Papillary hemangioma (PH) is a recently described vascular tumor with a predilection for the skin of the head and neck. Histopathologically, it is characterized by a bland endothelial proliferation arranged in a papillary configuration, bearing resemblance to glomeruloid hemangioma seen in the context of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes syndrome. The largest cutaneous PH reported to date measured 1.5 cm in greatest dimension. Here, we report a case of PH with an alarming size of 10 cm. We present this case to add to the limited literature on this rare tumor, highlight the histopathologic differences between PH and its mimics, and emphasize the variable nature of PH tumor size.

Minocycline-Induced Hyperpigmentation of Nails.

Minocycline is an antibiotic used for several dermatologic conditions, including rosacea. The development of skin, scleral, and nail hyperpigmentation may occur with long-term use of minocycline, and this is associated with no adverse effect on function. We present a case of a 66-year-old male who developed blue-gray hyperpigmentation of his nail beds after treating rosacea with systemic minocycline for over 20 years. The remainder of the physical exam was unremarkable for hyperpigmentation elsewhere. The patient was informed that this was likely an adverse effect of his chronic minocycline use. He insisted upon the continuation of minocycline, so he was counseled on the adverse effects of the medication and scheduled for follow-up.

Comparing Travel Distance to Care and Staging of Parotid Gland Malignancies in South Dakota and Surrounding States.

BACKGROUND: Parotid gland malignancies are difficult to characterize in terms of incidence and risk factors due to their relatively rare occurrence. Common cancers often present more aggressively in rural areas, albeit occurring less frequently. Some previous studies have found increased distance to care has been linked to more advanced malignancies. This study hypothesized decreased access to parotid gland malignancy specialists (otolaryngologists or dermatologists), evaluated as longer travel distances, would be associated with more advanced staging of parotid gland malignancies. METHODS: A retrospective chart review of the Sanford Health system electronic medical record in South Dakota and surrounding states from 2008-2018 was conducted to obtain data of parotid gland malignancies, respective staging, and patient home addresses to assess distance, both driving and straight-line, to the nearest parotid gland malignancy specialist, including outreach clinics. Travel distance was categorized (0-20 miles, 20-40 miles, and 40+ miles) and compared to categorized tumor stage (early 0/I, late II/III/IV) using a Fisher's Exact test. RESULTS: Overall, chart review yielded 134 patients with parotid gland malignancies from 2008-2018 in the Sanford Health system and associated data was gathered. The malignancies were categorically organized as 52.3 percent in early stage (0/I) vs. 47.7 percent in late (II/III/IV). When comparing parotid malignancy stage to driving distance, no significant association was found when outreach clinics were excluded (p=0.938) or included (p=0.327). When comparing parotid malignancy stage to straight-line distance, no significant association was found when outreach clinics were excluded (p=0.801) or included (p=0.874). CONCLUSIONS: Although no association was established between travel distance and parotid gland malignancy staging, further studies are needed to evaluate at population levels the incidence of parotid gland malignancies in rural communities and if there are specific risk factors in these areas for these malignancies which is currently unknown.

Acrometastasis of Occult Renal Cell Carcinoma: A Case Report.

Renal cell carcinoma is a common malignancy with 30,000 new cases reported annually in the U.S. While bone is one of the most common sites of metastases of renal cell carcinoma, acrometastases are rare with an estimated incidence of 0.1 percent among patients with malignant disease. We present an 89-year-old white male who presented with a painful mass of the left thenar eminence. A preoperative medical evaluation revealed metastatic renal cell carcinoma with lytic infiltration of the diaphysis of the left thumb metacarpal with soft tissue involvement. The patient was treated with two intralesional currettage procedures and later radiation therapy. This approach allowed the patient to maintain functional use of the thumb for activities of daily living.

The Matter of the Missing Melanoma: A Case Series.

Melanoma of the skin is the fifth most common malignancy in the U.S. In 2020, an estimated 6,850 people will die from melanoma of the skin. Further, given the broad distribution in ages affected, melanoma has a tremendous burden on health care costs and years of productive life lost. Every year, melanoma cases may go undetected, or have a significant delay in diagnosis, which may lead to poor outcomes. Here, three cases of melanoma encountered in the author's first clinical year of medical school, each of which had been overlooked, misdiagnosed, or lost to follow-up are reported. Implementing an integrated skin exam into every physical examination for high-risk patients and introducing preventative healthcare education into communities could help alleviate the burden of delay in diagnoses.

Aggressive Langerhans cell histiocytosis following T-cell acute lymphoblastic leukemia.

A 4-year-old female child developed cutaneous Langerhans cell histiocytosis 6 months following a diagnosis of T-cell acute lymphoblastic leukemia. Imaging revealed no evidence of systemic disease. Seven months later, the first systemic lesion was discovered on laryngoscopy. Restaging Positron Emission Tomography - Computed Tomography at that time revealed new 18-fluorodeoxyglucose-positive lesions in the left apical pleural margin, right lower peri-esophageal region, left ventricular myocardium, pancreas, upper pole of the left kidney, and inguinal and gluteal regions consistent with progressive systemic disease. Genomic testing revealed a low tumor mutational burden as well as mutations in KRAS G12A, ARID1A Q524, CDKN2A/B loss, and an alteration in NOTCH1.

Human adipose-derived stromal vascular fraction: characterization, safety and therapeutic potential in an experimental mouse model of articular injury.

Due to their capacity to self-renew, proliferate and generate multi-lineage cells, adult-derived stem cells offer great potential in regenerative therapies to treat maladies such as diabetes, cardiac disease, neurological disorders and orthopedic injuries. Commonly derived from adipose tissue, the stromal vascular fraction (SVF), a heterogeneous cell population enriched with mesenchymal stem cells (MSCs), has garnered interest as a cellular therapy due to ease of accessibility as an autologous, point-of-care application. However, the heterogeneous cell population within SVF is not historically taken into consideration when injecting into patients. Here, we characterized SVF, determined its safety and verify its therapeutic effects in a NOD/scid mouse model of articular injury. SVF were isolated from lipoaspirates utilizing a commercially available system (InGeneron Inc.), while MSCs were isolated from SVF via cell culture. Flow cytometry showed that neither age nor BMI affects the frequency of progenitor cells-like (CD31+CD34+), immune cells-like (CD4+) T cells, (CD14+) monocytes and total number of cells obtained. However, there was a negative correlation between donor BMI and MSC frequency within the SVF. ELISAs showed that following LPS activation in SVF, there were low levels of TNF-α and high levels of IL-10 secreted. However, T cell activation with anti-CD3 or anti-CD3+ anti-CD28, while leading to expected high levels of IFN-γ, did not lead to significant levels of TGF-ß. PCR analysis showed no significant numbers of cells outside the joint 1-hour post injection, moreover, no engraftment or abnormal growth in other organs 60-days post injection. Finally, both cell populations were able to ameliorate disease progression, as confirmed by the increase in movement of treated groups compared to injured groups. Noteworthy, the histological analysis indicated that there was no cartilage growth, suggesting an alternative therapeutic mechanism to cartilage regeneration.

Genetic ataxia telangiectasia porcine model phenocopies the multisystemic features of the human disease.

Ataxia telangiectasia (AT) is a progressive multisystem autosomal recessive disorder caused by mutations in the AT-mutated (ATM) gene. Early onset AT in children is characterized by cerebellar degeneration, leading to motor impairment. Lung disease and cancer are the two most common causes of death in AT patients. Accelerated thymic involution may contribute to the cancer, and recurrent and/or chronic respiratory infections may be a contributing factor to lung disease in AT. AT patients have fertility issues, are highly sensitive to ionizing radiation and they present oculocutaneous telangiectasia. Current treatments only slightly ameliorate disease symptoms; therapy that alters or reverses the course of the disease has not yet been discovered. Previously, we have shown that ATM-/- pigs, a novel model of AT, present with a loss of Purkinje cells, altered cerebellar cytoarchitecture and motor coordination deficits. ATM-/- porcine model not only recapitulates the neurological phenotype, but also other multifaceted clinical features of the human disease. Our current study shows that ATM-/- female pigs are infertile, with anatomical and functional signs of an immature reproductive system. Both male and female ATM-/- pigs show abnormal thymus structure with decreased cell cycle and apoptosis markers in the gland. Moreover, ATM-/- pigs have an altered immune system with decreased CD8+ and increased natural killer and CD4+CD8+ double-positive cells. Nevertheless, ATM-/- pigs manifest a deficient IgG response after a viral infection. Based on the neurological and peripheral phenotypes, the ATM-/- pig is a novel genetic model that may be used for therapeutic assessments and to identify pathomechanisms of this disease.

Concise Review: Fat and Furious: Harnessing the Full Potential of Adipose-Derived Stromal Vascular Fraction.

Due to their capacity to self-renew, proliferate and generate multi-lineage cells, adult-derived stem cells offer great potential for use in regenerative therapies to stop and/or reverse degenerative diseases such as diabetes, heart failure, Alzheimer's disease and others. However, these subsets of cells can be isolated from different niches, each with differing potential for therapeutic applications. The stromal vascular fraction (SVF), a stem cell enriched and adipose-derived cell population, has garnered interest as a therapeutic in regenerative medicine due to its ability to secrete paracrine factors that accelerate endogenous repair, ease of accessibility and lack of identified major adverse effects. Thus, one can easily understand the rush to employ adipose-derived SVF to treat human disease. Perhaps faster than any other cell preparation, SVF is making its way to clinics worldwide, while critical preclinical research needed to establish SVF safety, efficacy and optimal, standardized clinical procedures are underway. Here, we will provide an overview of the current knowledge driving this phenomenon, its regulatory issues and existing studies, and propose potential unmapped applications. Stem Cells Translational Medicine 2017;6:1096-1108.