Tumor-to-tumor metastasis of an adenocarcinoma to a glioblastoma with review of the literature on tumor-to-tumor metastasis to gliomas.

Tumor-to-tumor metastasis (TTM) is a process where one tumor metastasizes to another tumor. It is an exceedingly rare phenomenon, particularly in the central nervous system, where it most commonly occurs with meningiomas as the recipient. Herein, we present a case of tumor-to-tumor metastasis of an adenocarcinoma to a glioblastoma in a 75-year-old female. The patient had a history of high-grade ductal carcinoma in situ of the breast 8 years prior, treated with lumpectomy and radiation. She presented with a left fronto-parietal mass. Histologically, the lesion showed a glioblastoma, IDH-wildtype, WHO grade 4, associated with a metastatic adenocarcinoma (positive for estrogen receptor, progesterone receptor, and mammaglobin), suggesting a breast primary. The patient passed away 5 months after surgery. Involvement of glioblastoma by TTM is especially rare; only 1 case of TTM to glioblastoma is thus far reported in the English literature. The mechanism by which TTM occurs is poorly understood. TTM may be the first presentation of an occult malignancy and warrants thorough clinical, laboratory, and imaging investigation.

Keratin-positive giant cell-rich tumors of soft tissue with HMGA2::NCOR2 fusions.

BACKGROUND: Giant cell tumor of soft tissue (GCT-ST) is a rare soft tissue neoplasm that is morphologically similar to but genetically distinct from giant cell tumor of bone. A novel keratin-positive GCT-ST (KPGCT-ST) harboring HMGA2::NCOR2 fusions was recently discovered. Fewer than 30 cases have been described; herein is reported an additional seven. METHODS: Cases diagnosed as GCT-ST were retrieved from institutional archives and consultation files. The histopathologic characteristics were assessed, and the electronic medical record was reviewed. RESULTS: Seven tumors were identified in six women and one man with a median age of 23 years. All patients underwent excision; no recurrences or metastases were noted during a median follow-up period of 7 months. Histopathologically, the tumors were characterized by a multinodular proliferation of keratin-positive mononuclear cells with evenly admixed osteoclast-like giant cells and absent neoplastic bone. A fibrous capsule with lymphoid cuffing was frequently seen. Foamy macrophages, inflammation, hemorrhage, and hemosiderin were variably present. The HMGA2::NCOR2 fusion was detected in all cases. CONCLUSIONS: Our findings support previously reported hypotheses that KPGCT-ST is a spectrum of the same entity as the recently described xanthogranulomatous epithelial tumor. Although follow-up data are limited, to date, KPGCT-ST appears to follow an indolent course.

Nipple Adenoma: Clinicopathologic Characterization of 50 Cases.

Nipple adenoma (NA) is a rare, benign proliferation of the nipple ducts. It may be clinically mistaken for Paget disease or squamous cell carcinoma; thus, microscopic evaluation is paramount. A large case series of NA has not been undertaken since the 1980s. Therefore, we undertook this study to evaluate the clinicopathologic characteristics of NA, emphasizing differential diagnoses and follow-up data. We retrieved 50 cases from our in-house archives or consultation files between 2003 and 2022. Available slides were reviewed, and clinical data and follow-up information were obtained. Cases must have exhibited a dense ductal proliferation in the breast tissue with proximity to the nipple epidermis. All patients were women; median age was 56 years. In all, 68% of patients were symptomatic; 53% demonstrated a skin growth. Overall, 67% were excised completely, either primarily (33%) or via re-excision after biopsy (33%). Four histologic patterns were noted: adenosis (dense proliferation of small-to-medium ducts); large duct (medium-to-large caliber ducts); papillary-like (frond-like architecture with branching, slit-like lumens); and pseudoinfiltrative (ducts squished and distorted by dense stromal fibrosis). Follow-up in 44 patients (88%) with a median time of 66 months showed no evidence of recurrence. NA demonstrates a wide spectrum of histopathologic variation. Subtyping of this entity is unlikely to be clinically relevant. Differentiation from invasive carcinoma or other histologic mimics (syringocystadenoma papilliferum, syringomatous adenoma) may be difficult. Simple excision is curative, and recurrence is rare. A definitive link to invasive carcinoma has not been established.

Pseudoxanthoma-elasticum-like changes on the soft palate.

Pseudoxanthoma elasticum (PXE) is an autosomal recessive genetic disorder characterized by aberrant fragmentation and calcification of elastic fibers, leading to characteristic cutaneous, ophthalmic, and cardiovascular manifestations. PXE demonstrates significant phenotypic variability; involvement of the oral mucosa may be the only clue to the diagnosis. Reports on mucous membrane involvement in PXE are scarce. Here, we present a case of PXE-like changes in the oral cavity. A 70-year-old male patient presented with a painless leukoplakic lesion on the soft palate. Biopsy revealed numerous degenerated fibers in the lamina propria. Verhoeff-van Gieson and von Kossa staining confirmed their identity as calcified elastic fibers. A histopathological diagnosis of PXE-like changes was made; the patient was referred to ophthalmology where angioid streaks were visualized fundoscopically. PXE-like changes in the absence of the characteristic genetic mutation have also been reported with or without systemic manifestations. Furthermore, PXE-like changes have been reported in up to 10% of oral biopsy specimens undertaken without clinical suspicion for PXE. Therefore, the significance of such changes in isolation is unclear. Clinicians and pathologists should be aware of the potential oral manifestations of PXE to facilitate prompt diagnosis and subspecialist referral.

Primary cutaneous extraskeletal osteosarcoma: a series of 16 cases.

Extraskeletal osteosarcoma (EOS) is a high grade soft tissue tumour characterised by the production of malignant osteoid, without attachment/involvement of underlying bone/periosteum. Rarely, EOS presents as a cutaneous tumour. The clinical behaviour of primary cutaneous EOS (PC-EOS) remains incompletely characterised. Herein we present the largest case series of PC-EOS reported to date. Sixteen PC-EOS cases from the archives/consultation files were retrieved (male:female 1:1; age 31-96 years, mean age 66 years). The tumours measured 1-10 cm (mean 3.2 cm) and were located on the lower extremity (7), head (6), upper extremity (2), and trunk (1). They consisted of pleomorphic, spindled-to-epithelioid cells, with fascicular, nodular, or sheet-like growth patterns and foci of malignant osteoid. Immunohistochemistry did not reveal specific lines of differentiation, and there was no evidence of other tumour types. A literature review was conducted to identify all well characterised cases of PC-EOS. A combined analysis of present and past cases was performed to determine overall trends in clinical characteristics and outcomes. The mean follow-up period was 23.9 months, during which 67.5% of patients experienced progression-free survival and 18% of patients died of disease. Rates of local recurrence and metastasis were 10% and 25%, respectively, approximately double past estimates. These data suggest that the prognosis of PC-EOS is less favourable than previously thought. The differential diagnosis includes benign entities (e.g., ossifying pyogenic granuloma) and malignant neoplasms with heterologous osteosarcomatous differentiation (e.g., carcinosarcoma, transdifferentiated melanoma). Wide excision remains the standard of care, and the role of chemotherapy and radiation remains inconclusive. Recognition of this rare entity can facilitate prompt diagnosis and appropriate treatment.

Extramammary myofibroblastoma of the oral cavity.

Myofibroblastoma is a rare, benign mesenchymal tumor first described as a neoplasm of the breast. Extramammary myofibroblastoma is a histopathologically and genetically identical lesion occurring outside the breast. Herein is presented a case of extramammary myofibroblastoma arising in the oral cavity. A 59-year-old woman presented with a 1.5 cm nodule on the buccal surface of the lower lip. Wide local excision was performed. Histopathologic examination revealed haphazard fascicles of monomorphic spindle cells with hyalinized collagen bundles without fat. The spindled cells were diffusely positive for CD34, and focally for progesterone receptor. Desmin, smooth muscle actin, estrogen receptor, androgen receptor, S100, and STAT6 were negative. Rb1 expression was lost in tumor cells. Thus, the diagnosis of extramammary myofibroblastoma was made. Differential diagnoses include spindle-cell lipoma and angiofibroma. All three tumors are members of the 13q14 deletion/RB1 loss family. Indolent but locally aggressive (solitary fibrous tumor, desmoid fibromatosis) and frankly malignant (low-grade peripheral nerve sheath tumor, dermatofibrosarcoma protuberans) entities can be excluded by immunohistochemistry and careful microscopic examination. Extensive sampling extramammary myofibroblastoma is important to exclude the possibility of malignancy. Clinicians and pathologists alike should be aware of this entity and its potential to arise rarely in unusual locations.

Advanced Wound Diagnostics: Toward Transforming Wound Care into Precision Medicine.

Significance: Nonhealing wounds are an ever-growing global pandemic, with mortality rates and management costs exceeding many common cancers. Although our understanding of the molecular and cellular factors driving wound healing continues to grow, standards for diagnosing and evaluating wounds remain largely subjective and experiential, whereas therapeutic strategies fail to consistently achieve closure and clinicians are challenged to deliver individualized care protocols. There is a need to apply precision medicine practices to wound care by developing evidence-based approaches, which are predictive, prescriptive, and personalized. Recent Advances: Recent developments in "advanced" wound diagnostics, namely biomarkers (proteases, acute phase reactants, volatile emissions, and more) and imaging systems (ultrasound, autofluorescence, spectral imaging, and optical coherence tomography), have begun to revolutionize our understanding of the molecular wound landscape and usher in a modern age of therapeutic strategies. Herein, biomarkers and imaging systems with the greatest evidence to support their potential clinical utility are reviewed. Critical Issues: Although many potential biomarkers have been identified and several imaging systems have been or are being developed, more high-quality randomized controlled trials are necessary to elucidate the currently questionable role that these tools are playing in altering healing dynamics or predicting wound closure within the clinical setting. Future Directions: The literature supports the need for the development of effective point-of-care wound assessment tools, such as a platform diagnostic array that is capable of measuring multiple biomarkers at once. These, along with advances in telemedicine, synthetic biology, and "smart" wearables, will pave the way for the transformation of wound care into a precision medicine. Clinical Trial Registration number: NCT03148977.

Psychosocial impact of hidradenitis suppurativa: a practical guide for clinicians.

INTRODUCTION: Hidradenitis suppurativa (HS) is a debilitating skin disease with significant and often underappreciated effects on quality of life; available treatments fail to achieve consistent rates of remission. Targeting the psychosocial impact of HS has great potential to improve care for these patients. Although the literature on this topic is broad, there is a lack of specific tools that guide clinicians in this domain. METHODS: The authors surveyed the literature to find the aspects of psychosocial functioning that most significantly impact Health-Related Quality of Life (HRQOL) for HS patients, and which may be assessed in a simple and efficient manner. RESULTS AND DISCUSSION: Depression and anxiety, sexuality and body image, and financial strain were identified as the most significant drivers of poor HRQOL with the greatest potential to be screened for and addressed succinctly and effectively. A practical psychosocial management guide for clinicians is presented. The guide includes a list of preexisting validated screening questions, clear guidelines for interpretation, and a suggested management algorithm all geared toward a 'real-life' medical practice. CONCLUSION: Such an approach holds a great potential for improving the care of patients with HS. Validation of this approach via controlled trials is a logical next step.

Peristomal Pyoderma Gangrenosum Responding to Risankizumab.

ABSTRACT: Evidence to support available therapies for pyoderma gangrenosum (PG) is limited. Many patients do not respond to topical therapies such as tacrolimus or topical steroids. Currently favored oral systemic treatments (eg, cyclosporine and steroids) achieve complete remission in only 50% of patients and have unfavorable adverse effect profiles. There is a growing body of evidence to support biologic agents for the treatment of PG, but their exact role remains unclear. Here the authors present a patient with peristomal PG, the first reported case of PG responding to treatment with risankizumab, an anti-interleukin 23 monoclonal antibody. Risankizumab may represent an effective and relatively safe treatment for PG that merits additional exploration in prospective, controlled studies.

Sonographic Evaluation of Hidradenitis Suppurativa with Smartphone-Linked Portable Ultrasound.

BACKGROUND: Clinical staging systems for hidradenitis suppurativa (HS) have poor interrater reliability and may underestimate disease activity. Sonographic staging systems may overcome these challenges, but conventional ultrasound (US) machines are expensive and bulky. Portable (p)US may facilitate the integration of sonography into routine practice. OBJECTIVES: To assess the ability of a novel smartphone-linked pUS device to identify key sonographic lesions of HS. METHODS: The charts of 16 patients with HS who were assessed with pUS at the outpatient Dermatology and Wound Care Clinics of a university hospital center were retrospectively reviewed. Clinical and sonographic images of the affected areas were examined. The main outcome measures were the number of patients with identifiable sonographic lesions and the number of patients with subclinical lesions detected by pUS. RESULTS: All 3 key sonographic lesions of HS were identifiable with pUS. Sonographic lesions were identified in 10 patients (62.5%). Subclinical lesions were identified in 2 patients (12.5%); in both cases, this affected management decisions. CONCLUSIONS: We demonstrate the ability of pUS to identify the key sonographic lesions of HS. pUS is a simple and affordable way to integrate HSUS into clinical and research settings, with clear potential benefits to patients.

Evidence-Based Review of Antibiofilm Agents for Wound Care.

Significance: Biofilms in vivo are small densely packed aggregations of microbes that are highly resistant to host immune responses and treatment. They attach to each other and to nearby surfaces. Biofilms are difficult to study and identify in a clinical setting as their quantification necessitates the use of advanced microscopy techniques such as confocal laser scanning microscopy. Nonetheless, it is likely that biofilms contribute to the pathophysiology of chronic skin wounds. Reducing, removing, or preventing biofilms is thus a logical approach to help clinicians heal chronic wounds. Recent Advances: Wound care products have demonstrated varying degrees of efficacy in destroying biofilms in in vitro and preclinical models, as well as in some clinical studies. Critical Issues: Controlled studies exploring the beneficial role of biofilm eradication and its relationship to healing in patients with chronic wounds are limited. This review aims to discuss the mode of action and clinical significance of currently available antibiofilm products, including surfactants, dressings, and others, with a focus on levels of evidence for efficacy in disrupting biofilms and ability to improve wound healing outcomes. Future Directions: Few available products have good evidence to support antibiofilm activity and wound healing benefits. Novel therapeutic strategies are on the horizon. More high-quality clinical studies are needed. The development of noninvasive techniques to quantify biofilms will facilitate increased ease of research about biofilms in wounds and how to combat them.

The therapeutic potential of cannabinoids for integumentary wound management.

The increasing legalization of Cannabis for recreational and medicinal purposes in the United States has spurred renewed interest in the therapeutic potential of cannabinoids (CBs) for human disease. The skin has its own endocannabinoid system (eCS) which is a key regulator of various homeostatic processes, including those necessary for normal physiologic wound healing. Data on the use of CBs for wound healing are scarce. Compelling pre-clinical evidence supporting the therapeutic potential of CBs to improve wound healing by modulating key molecular pathways is herein reviewed. These findings merit further exploration in basic science, translational and clinical studies.

Thymol-Induced Chemical Leukoderma Successfully Treated with 308-Nanometer Excimer Laser.

INTRODUCTION: Chemical leukoderma (CL) is an acquired depigmenting disorder caused by repeated exposure to chemical compounds. Thymol is a phenol derivative used as a preservative or antiseptic in many commercially available products. Here, we present the second reported case of CL caused by a thymol-containing compound. CASE PRESENTATION: A 48-year-old woman presented with a 4-month history of depigmentation of the nail folds of all ten fingers. This occurred after 1 month of twice-daily application of a thymol-containing compound intended for the removal of gel nails. No improvement was noted after the product was discontinued. There was no family history of vitiligo or other autoimmune disorders. On physical exam, depigmentation of all ten proximal and lateral nail folds was seen, with accentuation on Wood's lamp exam. Partial re-pigmentation was achieved after 32 treatments with 308-nm excimer laser. DISCUSSION: A thorough history and physical exam are instrumental in differentiating CL from other causes of depigmentation. Avoidance of the offending agent is an essential part of management. It is important to note that many cosmetic products are not tightly regulated by the FDA. Excimer laser is an effective treatment for CL with a favorable side-effect profile.

Traditional and advanced therapeutic modalities for wounds in the paediatric population: an evidence-based review.

OBJECTIVE: Children can have non-healing wounds due to a wide range of pathologies, including epidermolysis bullosa (EB), pilonidal disease and Stevens-Johnson syndrome, with some causes being iatrogenic, including extravasation injuries and medical device-related hospital-acquired pressure ulcers. Furthermore, paediatric wounds are vastly different from adult wounds and therefore require a different treatment approach. While there are numerous types of dressings, topical remedies, and matrices with high-tier evidence to support their use in adults, evidence is scarce in the neonatal and paediatric age groups. The purpose of this review is to discuss the basic principles in paediatric wound management, as well as to present new treatment findings published in the literature to date. The benefits and risks of using different types of debridement are discussed in this review. Various topical formulations are also described, including the need to use antibiotics judiciously. METHOD: Databases were searched for relevant sources including Pubmed, Embase, Web of Science and DynaMed. Search terms used included 'wound care', 'wound management', 'paediatrics', 'children', 'skin substitutes', and 'grafts'. Additionally, each treatment and disease entity was searched for relevant sources, including, for example: 'Apligraf', 'dermagraft', 'Manuka honey', 'antibiotic', 'timolol', and 'negative pressure wound therapy' (NPWT). RESULTS: Amniotic membrane living skin equivalent is a cellular matrix that has been reportedly successful in treating paediatrics wounds and is currently under investigation in randomised clinical trials. Helicoll is an acellular matrix, which shows promise in children with recessive dystrophic EB. NPWT may be used as a tool to accelerate wound closure in children; however, caution must be taken due to limited evidence to support its safety and efficacy in the paediatric patient population. Integra has been reported as a useful adjunctive treatment to NPWT as both may act synergistically. Hospitalised children and neonates frequently have pressure ulcers, which is why prevention in this type of wound is paramount. CONCLUSION: Advancements in wound care are rapidly expanding. Various treatments for non-healing wounds in paediatric and neonatal patients have been reported, but high tier evidence in these populations is scarce. We hope to shed light on existing evidence regarding the different therapeutic modalities, from debridement techniques and dressing types to tissue substitutes and topical remedies. There have been promising results in many studies to date, but RCTs involving larger sample sizes are necessary, in order to determine the specific role these innovative agents play in paediatric wounds and to identify true safety and efficacy.