Fibrin associated large B-cell lymphoma accidentally identified in a breast implant capsule: a molecular report of a rare entity.

Breast implant-associated (BIA) lymphoma is a rare malignancy, typically originating from T-cells; however, few cases of diffuse large B-cell lymphoma (LBCL) have been recently described. These cases share major features: Epstein-Barr virus positivity and a favorable prognosis with surgical intervention alone, hinting at a potential link to fibrin-associated LBCL (FA-LBCL). This study presents the first case of BIA-FA-LBCL in Italy and one of the few assessed from a molecular standpoint so far. We identified two pathogenic mutations in DNMT3A and a variant of uncertain significance (VUS) in JAK2. These findings suggest that dysfunctional epigenetic mechanisms and constitutive activation of the JAK-STAT pathway may underpin BIA-FA-LBCL lymphomagenesis. Finally, we summarized all the previously reported cases in alignment with the updated WHO-HAEM5 classification, shedding further light on the nature of this new entity. This report highlights the rarity of BIA-FA-LBCL and underscores the importance of comprehensive capsule sampling and reporting to national databases for accurate characterization and management of these lymphomas. The study supports the classification of BIA-FA-LBCL within the spectrum of FA-LBCL, emphasizing the need for further research to elucidate its molecular underpinnings and improve clinical outcomes.

Cell-free biomimetic polyurethane-based scaffold for breast reconstruction following non-malignant lesion resection. A first-in-human study.

BACKGROUND: Based on the volume of tissue removed, conservative surgery (BCS) cannot always guarantee satisfactory cosmetic results, unless resorting to more complex oncoplastic approaches. Investigating an alternative to optimize aesthetic outcomes minimizing surgical complexity, was the purpose of this study. We assessed an innovative surgical procedure based on the use of a biomimetic polyurethane-based scaffold intended for regenerating soft-tissue resembling fat, in patients undergoing BCS for non-malignant breast lesions. Safety and performance of the scaffold, and safety and feasibility of the entire implant procedure were evaluated. METHODS: A volunteer sample of 15 female patients underwent lumpectomy with immediate device positioning, performing seven study visits with six-month follow-up. We evaluated incidence of adverse events (AEs), changes in breast appearance (using photographs and anthropomorphic measurements), interference with ultrasound and MRI (assessed by two independent investigators), investigator's satisfaction (through a VAS scale), patient's pain (through a VAS scale) and quality of life (QoL) (using the BREAST-Q© questionnaire). Data reported are the results of the interim analysis on the first 5 patients. RESULTS: No AEs were device related nor serious. Breast appearance was unaltered and the device did not interference with imaging. High investigator's satisfaction, minimal post-operative pain and positive impact on QoL were also detected. CONCLUSIONS: Albeit on a limited number of patients, data showed positive outcomes both in terms of safety and performance, paving the way to an innovative breast reconstructive approach with a potential remarkable impact on clinical application of tissue engineering. TRIAL REGISTRATION: ClinicalTrials.gov (NCT04131972, October 18, 2019).

Malignancies within rhinophyma: report of three new cases and review of the literature.

BACKGROUND: Rhinophyma, which represents the end stage of rosacea, is characterized by sebaceous hyperplasia, fibrosis follicular plugging, and telangiectasia. Although it is commonly labeled as an aesthetic problem, it may also determine airway obstruction and because of its nature to hide the growth of tumors. Due to the increasing number of reports of nonmelanoma skin types of cancer within rhinophyma, further concern about a higher incidence of malignancies in rhinophyma than in the skin of normal noses is reasonable. METHODS: We describe three male patients who developed malignancies (2 basal and 1 squamous cell carcinoma) associated with rhinophyma disease. The tumors developed over a mean of 23.3 years after primary diagnosis of rhinophyma. One case had a previous history of facial skin tumor. Surgical excision with clear margins allowed resolution in all three patients with a mean follow-up of 34.8 months. CONCLUSIONS: These three new cases and the review of 43 cases reported in the literature call attention to the clinical features of carcinomas arising in the context of rhinophyma, raising further concerns about the possible association between these two entities. The need for histologic examination of all surgically removed tissue in patients with rhinophyma is highlighted. Several macroscopic changes, including ulceration, drainage, and a rapid growth pattern, should alarm the physician and should be considered as suspicious of a malignant degeneration. Unexpected clinical modifications of a preexisting long-lasting silent rhinophyma could indicate the possibility of hidden malignancy rather than a rhinophyma itself. Although evidence of an association between the two entities remains inconclusive, half of the malignancies reported in our review were incidental findings associated with rhinophyma. Thus, since rhinophyma should not be considered solely a cosmetic problem, we recommend that all specimens be reviewed by a pathologist and if malignancy is diagnosed, re-excision with clear margins should be achieved when necessary with periodic follow-up.

Malignant melanoma of the nasal septum.

Primary malignant melanoma involving the nasal and paranasal sinus mucosa is a rare neoplasm, accounting for less than 1% of all melanomas. Being more aggressive than its cutaneous counterpart, it carries a poor prognosis. We report a case arising from the nasal septum mucosae in a 78-year-old man. We describe clinical features, diagnosis, and treatment of this rare disease along with a review of the pertinent literature. Endoscopic resection of the neoplasm was performed, enabling diagnosis of sinonasal mucosal melanoma involving the frontal sinus floor. Because the patient was too compromised to achieve radical surgical approach (craniofacial resection), he underwent radiation therapy. Through this article, we wish to emphasize that early diagnosis with a high index of suspicion is critical because this type of tumor is extremely aggressive. Its location and relatively nonspecific features frequently delay diagnosis, and its rarity avoids an optimal treatment guideline setting.

Merkel cell carcinoma of the lower extremity: report of four cases and new considerations.

Merkel cell carcinoma (MCC) is a rare neuroendocrine cutaneous malignancy that predominantly arises in the head and neck region. We describe clinical features, diagnosis, and treatment in 4 cases of MCC, presenting an uncommon female predominant occurrence and an unusual primary site: the lower limb. In all cases diagnosis was established by histopathologic examination. Primary MCC and locally recurrence disease were treated in all patients with a wide surgical excision (3-cm margin) including fascia. Lymphadenectomy was reserved for a patient with clinical evidence of nodal involvement. Both chemotherapy administered in 2 cases and radiotherapy in 1 case produced limited responses. Early diagnosis is critical because this tumor is aggressive and has a high rate of local recurrence and metastatic spread. However, its nondistinctive appearance frequently delays diagnosis and its rarity avoids an optimal treatment guideline setting.

Skin-reducing mastectomy: new refinements.

Skin-reducing mastectomy is a single-stage technique that helps us to overcome the cosmetic inadequacy of a Type IV Wise pattern skin-sparing mastectomy (final T-inverted scar) in heavy and pendulous breasts by filling the lower-medial quadrant with adequate volume. It also conceals scars as an aesthetic operation and at the same time provides satisfactory and safe coverage of the implant. We report our experience with 22 skin-reducing mastectomies done for 18 women. We modified part of the original description of raising the dermal flap to refine the anatomical results. This flap was mobilised better by detachment of the lateral part of its insertion along the inframammary fold, and this allowed us to close the dermomuscular pouch inferiorly and laterally without raising the serratus anterior or limiting its rise. The total or partial preservation of the serratus muscle together with the creation of a force directed medially, as indicated by the dermal flap, reduced the risks of lateral dislocation of the implant and improved the lateral breast contour to give a more natural shape. Skin-reducing mastectomy is an oncologically safe skin-sparing mastectomy that solves all cosmetic problems and reduces complications of the original Type IV Wise pattern in medium to large breasts. Doing the mastectomy and reconstruction in a single stage aids the favourable psychological approach of the patient. We emphasise the use of our small modification to refine the contour of the breast and improve the aesthetic outcomes by giving a natural curvilinear profile.

Periorbital necrotising fasciitis.

Necrotising fasciitis involving the periorbita is a devastating infection. Potential outcomes range from severe disfigurement, loss of the eye and even to death. Early recognition is critical, although its initially non-distinctive appearance frequently delays diagnosis and treatment. Herein, the authors have performed a systematic review of previously published cases including clinical features, diagnoses and differential diagnoses, pathological characteristics and management. Periorbital necrotising fasciitis is seen mainly in adults with a female predominance (54%); about one-half (47%) of the patients were previously healthy. The infection can follow local blunt trauma (17%), penetrating injuries (22%) and face surgery (11%), but in about one-third of cases (28%) no cause was identified. Non-specific erythema and localised painful swelling of the eyelids characterise the earliest manifestation of the disease, followed by formation of blisters and necrosis of the periorbital skin and subcutaneous tissues. The causative organism in periorbital infection was mainly ß-haemolytic Streptococcus alone (50%), occasionally in combination with Staphylococcus aureus (18%). The overall mortality rate was 14.42%. The main risk factor for mortality was the type of causative organism, since all reported cases of death were caused by ß-haemolytic Streptococcus alone or associated with other organisms. Unlike necrotising fasciitis affecting other body sites, there was not a strong correlation with age >50 years or the presence of associated chronic illness. Management of periorbital necrotising fasciitis is then based on early distinction of symptoms and signs and aggressive multidisciplinary treatment. Thus, the delay between initial debridement and initiating parenteral broad-spectrum antibiotic therapy should be considered the most critical factor influencing morbidity and mortality.