Paget\'s disease of the bone (PDB) is a benign osteodystrophy of the elderly characterized by excessive remodeling of bone tissue, mainly in the pelvis, femur, and skull. Its neurological manifestations are numerous and affect both the central and peripheral nervous systems. As headaches are often reported, epileptic seizures remain exceptional. We report the case of a 75-year-old female patient with a history of chronic worsening headache who was admitted to the emergency department for the first episode of a seizure. Brain imaging revealed heterogeneous bone thickening and circumscribed skull osteoporosis. Bone scintigraphy showed pagetoid lesions restricted to the skull and face. Alkaline phosphatases increased. The rest of the biological work-up and the cerebrospinal fluid study ruled out other metabolic causes or central nervous system infections. The patient was treated with bisphosphonates and anti-convulsive treatment. The evolution was satisfactory, with progressive improvement in headache and seizure control, even several months after discontinuation of anti-seizure medication. Our case report highlights the importance of exploring chronic headaches in the elderly, not only in search of lesions of the cerebral parenchyma but also of the structures containing them, in this case, the skull.

Introduction Primary extramammary Paget\'s disease (EMPD) is a rare neoplasm that manifests as well-defined erythematous plaques, often misdiagnosed due to its similarity with different dermatoses. It may exhibit invasive features, involving adnexal invasions. The study aims to assess and compare the clinicopathological correlation of primary EMPD with adnexal features. Materials and methodology The monocentric observational retrospective study observed 43 confirmed primary EMPD cases in patients aged 45-95, excluding those with infectious dermatoses, pseudo-tumors, secondary lesions, or survived less than a month. Demographical, clinical and pathological observations were recorded. Expert dermatopathologists, blinded to the initial diagnosis, conducted a comprehensive histopathological evaluation yielding differential pathological diagnosis. Statistical analysis involved Pearson\'s Chi-square, Mann-Whitney U, and Spearman\'s Correlations for clinicopathological concordance and adnexal features. Recurrence was evaluated using Kaplan-Meier and log-rank tests, while multivariate recurrence analyses include Cox regression. A p-value < 0.05 was deemed significant. Results There was a significant association between adnexal involvement and the site of lesion (p < 0.05). There was a significant association (p < 0.05) between involved adnexal depth and primary EMPD subtypes. Adnexal involvement has a significant association with the concordance rates derived from clinicopathological correlations (p < 0.05). Smaller lesions and non-invasive EMPD significantly predict longer recurrence onset (p < 0.01). The primary EMPD subtype was the only independent predictor for recurrence time using the Cox regression model. Conclusion Adnexal proliferation in primary EMPD is considered vital on clinicopathological correlations and recurrence predictions, suggestive of its utility on both diagnosis and prognosis.

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OBJECTIVE: The aims of the current study were to describe clinical and biochemical features of patients with Paget disease of bone (PDB) followed at our medical center, and to examine the long-term effectiveness of zoledronate.
METHODS: Retrospective cohort study included consecutive patients≥18 years with a diagnosis of PDB, followed in the Rabin Medical Center (RMC) Institute of Endocrinology from 1973 to 2023. The cohort comprised two groups: patients treated/not treated with zoledronic acid (ZOL/NZOL). The primary outcome was the percentage of patients who achieved a biochemical therapeutic response.
RESULTS: Overall, 101 patients with PDB were included, 68 in the ZOL group and 33 in the NZOL group. The mean age was 65.2 ± 10.0 years, and 47% were female. Notably, 77% exhibited monostotic involvement, and only 3% had experienced fractures attributed to PDB. Mean ALP level at diagnosis was 160 ± 70.6 U/L. The median follow-up duration was 17 years since PDB diagnosis, comparable between the groups. Primary outcome was more prevalent in the ZOL compared to the NZOL group [42 patients (88%) VS 11 patients (52%) respectively, P = 0.004]. At the end of follow-up, mean ALP levels in the NZOL group were significantly higher than the levels in the ZOL group irrespective of the number of infusions received.
CONCLUSIONS: The majority of patients with PDB experience a mild disease course, marked by monostotic involvement and a low prevalence of fractures. Zoledronic acid effectively manages PDB, providing sustained biochemical response. The necessity for multiple zoledronic acid injections remains questionable, often implemented due to osteoporosis.

Aim: Angioid streaks (ASs) are a rare retinal condition and compromise visual acuity when complicated with choroidal neovascularization (CNV). They represent crack-like dehiscences at the level of the Bruch\'s membrane. This objective narrative review aims to provide an overview of pathophysiology, current treatment modalities, and future perspectives on this condition. Materials and Methods: A literature search was performed using \"PubMed\", \"Web of Science\", \"Scopus\", \"ScienceDirect\", \"Google Scholar\", \"medRxiv\", and \"bioRxiv.\" Results: ASs may be idiopathic, but they are also associated with systemic conditions, such as pseudoxanthoma elasticum, hereditary hemoglobinopathies, or Paget\'s disease. Currently, the main treatment is the use of anti-vascular endothelial growth factors (anti-VEGF) to treat secondary CNV, which is the major complication observed in this condition. If CNV is detected and treated promptly, patients with ASs have a good chance of maintaining functional vision. Other treatment modalities have been tried but have shown limited benefit and, therefore, have not managed to be more widely accepted. Conclusion: In summary, although there is no definitive cure yet, the use of anti-VEGF treatment for secondary CNV has provided the opportunity to maintain functional vision in individuals with AS, provided that CNV is detected and treated early.

BACKGROUND: Nipple adenoma is a relatively rare benign disease. Clinically, it often presents with nipple erosions, and it should be differentiated from Paget\'s disease.
METHODS: The patient was a 63-year-old woman who complained of a lump in her left nipple for more than 30 years. Computed tomography performed for screening congestive heart failure suggested a left nipple mass of 40 mm in size. Needle biopsy revealed nipple adenoma, and skin biopsy was also performed to confirm the diagnosis. Nipple tumor resection was performed under local anesthesia, and we confirmed that the final diagnosis was nipple adenoma with negative margins. The patient has been free from recurrence for 2 years since the surgery.
CONCLUSIONS: We have reported our experience of a case of giant nipple adenoma.

This review directs the focus on the imaging features of various fibro-osseous lesions and other bone lesions that can be of similar presentation. Broad diagnosis of \"fibrous osseous lesion\" may culminate in improper treatment and management. Radiographic discriminating factors between these entities are highlighted and summarized to improve the diagnostic process when encountering these lesions.

Paget\'s disease (PD) of the breast is a rare underlying malignant tumor. Approximately 50% to 60% of patients with mammary PD are concurrently diagnosed with invasive ductal carcinoma (PD-IDC), a condition associated with a worse prognosis than IDC without PD. Thus far, there has been a lack of an accurate and efficient prognostic model for PD-IDC, and the factors influencing the effectiveness of chemotherapy and radiotherapy for these patients remain unknown. In this study, we developed a web-based nomogram based on the data from the Surveillance Epidemiology and End Results (SEER) database. We subjected the model to a series of validation methods, including area under the curve (AUC) values, receiver operating characteristic curve (ROC) analysis, calibration curves, and decision curve analysis (DCA). Our results demonstrated that our model exhibited high discrimination, accuracy, and clinical applicability in predicting the overall survival (OS) of patients with PD-IDC (testing set: three- and five-year AUCs, 0.831 and 0.841, respectively). To further validate our nomogram, we used external data from both our institution and sister hospitals (external data: three- and five-year AUCs, 0.892 and 0.914, respectively). Multivariable Cox regression analysis identified several independent unfavorable prognostic factors for the OS of patients with PD-IDC, including increasing age, high grade, widowed status, higher T stages, and the presence of bone metastases. Furthermore, propensity score matching (PSM)-adjusted analysis was conducted, revealing that chemotherapy did not significantly improve the survival of patients with PD-IDC across molecular subtypes, except for those in the grade III/IV group, where it improved both OS and breast cancer-specific survival (BCSS). Additionally, our findings indicated that only patients with PD-IDC with T4 and N3 stages benefited from radiotherapy, leading to improvements in both OS and BCSS. In conclusion, we have comprehensively analyzed the clinical characteristics and prognosis of patients with PD-IDC, culminating in the development of a user-friendly web-based nomogram for predicting their survival. Our predictive model is not only highly accurate but also offers simplicity, making it accessible for healthcare providers and patients. Furthermore, our stratified analysis highlights that the pathological grade, rather than the molecular subtype, plays a pivotal role in determining the efficacy of chemotherapy in improving the prognosis for patients with PD-IDC, while radiotherapy confers survival benefits to patients with PD-IDC in T4 and N3 stages.

OBJECTIVE: Extramammary Paget\'s disease (EMPD) is a rare, slow growing intra-epidermal malignant neoplasm that arises in areas rich in apocrine glands. Several common sites of occurrence have been reported, including the vulva, perianal region, perineum, and scrotum. Most relevant studies rely on small data bases. Our objective was to evaluate prognostic factors of EMPD patients at a single medical center.
METHODS: We retrospectively analyzed 19 patients (8 males, 11 females) diagnosed with genital EMPD who were treated at the Taichung Veterans General Hospital between 2006/04 and 2022/08. Collected information included tumor location, margin condition in the case of surgical resection, recurrence rate, recurrence management, accompanied gastrointestinal malignancy, treatment details and survival data.
RESULTS: Among 19 cases, 4 with initial margin being positive, and 3 received second surgery (one refused surgery and another expired within a year). Tumor recurrence was found in 7 cases, with 6 of them later receiving second surgery, and the remaining one received radiation therapy. Median DFS was 7.57 years. During the 15-year follow-up, 2 patients expired. Overall survival rate was 87.5%. Among all factors we had analyzed, only those accompanied with GI tract malignancy had significantly worse survival rate (p=0.018). Frozen sections taken at surgical margin during surgery significantly reduced cancer recurrence rate (p=0.45). Permanent pathology margins appeared to affect the recurrence rate, but that was not significant when comparing with intraoperative frozen sections.
CONCLUSIONS: Local wide excision with skin flap reconstruction remains the major treatment option for genital EMPD. Following the standard-of-care procedure, the overall patient outcome was excellent. Among factors potentially associated with recurrence rate, intraoperative frozen biopsy was the most significant one. Performing intraoperative frozen biopsy is essential for recurrence-free rate elevation.

UNASSIGNED: Paget\'s disease of the breast (PDB) is a rare nipple entity associated with multifocality. Due to its location, resection of the entire nipple-areolar complex is necessary. Historically central quadrantectomy and mastectomy have the surgical treatments of choice. The feasibility of oncoplastic breast surgery (OBS) for PDB is unknown.
UNASSIGNED: This was a retrospective study performed in a Brazilian oncological hospital. We evaluated the factors related to the performance of OBS in PDB. In addition, the impact of OBS on local recurrence and survival was analysed. Comparisons were made between groups using the chi-square test, Mann-Whitney U test, and Kaplan-Meier method. To assess the impact factor of the variables on the performance of OBS, logistic regression was performed.
UNASSIGNED: Eighty-five patients were evaluated. OBS was performed in 69.4% (n=59), and of these, 16 (27.2%) were symmetrized with contralateral surgery. Mastectomy without reconstruction was performed in 28.3% of the patients. The primary procedure performed was mastectomy with reconstruction (n=38; 44.7%), and the preferential technique for immediate reconstruction was skin-sparing mastectomy with prosthesis; for late reconstruction, the preferred technique was using the latissimus dorsi. Breast conserving-surgery was performed in 27.0% (n=23), primarily using the plug-flap technique (OBS). Age was associated with the use of OBS; as patients aged 40-49 exhibited a higher rate of OBS (p = 0.002; odds ratio 3.22). OBS did not influence local recurrence (p=1.000), overall survival (p=0.185), or cancer-specific survival (p=0.418).
UNASSIGNED: OBS improves options related to surgical treatment in PDB without affecting local recurrence or survival rates.