Keratocystic odontogenic tumors as first clinical manifestation of nevoid basal cell carcinoma syndrome in pediatric age: our microinvasive surgical approach.

Keratocystic odontogenic tumors as first clinical manifestation of nevoid basal cell carcinoma syndrome in pediatric age: our microinvasive surgical approach. Aim. Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is a rare genetic condition involving multiple organs; Keratocystic Odontogenic Tumors (KCOTs) are often the first clinical manifestation in pediatric age. The aim of this study was to describe the clinical and histopathological features of KCOTs as first clinical sign of NBCCS in pediatric patients allowing an early diagnosis, and their treatment with conservative microinvasive piezosurgery. Methods. Twenty pediatric patients affected by NBCCS showing 60 KCOTs came to our attention at the Complex Operating Unit of Odontostomatology, Policlinic of Bari, from 1996 to 2014. After clinical examination, OPT and computed tomography analysis, all patients underwent conservative micro-invasive surgical treatment under general anesthesia, consisting in enucleation of KCOTs, cavity ostectomy and osteoplasty with conventional rotative instruments and piezoelectric tools in order to remove damaged bone, epithelial remnants and satellite cysts with maximal teeth preservation in consideration of the young age of the patients, and to possibly minimize the recurrence risk. A sterile gel formulation of sodium hyaluronate and amino acids (Gly-Pro-Leu-Lys) was put into the bone defect, allowing for faster bone regeneration and healing of the surgical site. All the surgical specimens were sent for histopathological examination. The patients underwent clinical and radiological (OPT) follow-up after 7, 15 and 30 days, 2, 3, 6 and 12 months, and, then, once a year. The followup time ranged from 18 months to 8 years. Results. There were 10 males and 10 females, with an average age of 10,6 years. At the initial evaluation, 13 patients showed swelling, teeth agenesis, and dental inclusions or dislocations as first clinical manifestation of their disease; in the remaining cases, KCOTs were asymptomatic. Among the 20 patients, 11 had been previously diagnosed with NBCCS because of familiarity or presence of characteristic features of the syndrome, while KCOTs were the first clinical sign of NBCCS for the remaining 9 patients without familial history of the syndrome; subsequently, the genetic analysis showing PTCH1 mutations confirmed the diagnosis. 60 KCOTs were totally identified: 39 lesions were located in the mandible and 21 lesions were located on the upper jaw. The histopathological analyses highlighted thin connective tissue walls covered by para- and orthokeratotic stratified squamous epithelium, usually about 5-8 cell layers thick, around cystic lumens filled with desquamated keratin, thus confirming the diagnosis of KCOT. The epithelial lining included a well-defined, palisading basal layer of cuboidal to small columnar cells and a superficial layer with corrugated appearance. Satellite cysts could also be seen due to tissue budding of the basal cell layers into the adjacent connective tissue. The clinico-radiological follow-up of 53 lesions showed progressively decreased radiolucent areas up to complete healing within 12 months from the surgical treatment. Differently, OPT disclosed lack of decreasing radiolucency of 7 lesions after 12 months suggesting KCOTs recurrence, that is more frequent in syndromic KCOTs than in sporadic ones due to their higher dimensions and to the involvement of nobile structures such as teeth which should be preserved in these young patients as much as possible. Conclusion. KCOTs in pediatric patients require conservative approaches for permanent teeth preservation. While conventional enucleation leads up to 60% recurrence rates, cavity ostectomy with piezoelectric tools significantly reduced the recurrence risk allowing the preservation of permanent teeth

Peripheral Giant Cell Granuloma of the Jaws as First Sign of Primary Hyperparathyroidism: A Case Series

Peripheral giant cell granulomas (PGCG) associated with hyperparathyroidism (HPT) are rare clinical entities. The aim of this study is to report on 21 PGCGs of the oral cavity as the first clinical sign of unknown primary HPT (PHPT) referred to the Complex Operating Unit of Odontostomatology of Aldo Moro University of Bari from 2009 to 2019. Surgical treatment consisted in conservative enucleation of the lesion, if possible, with contextual bone rim osteoplasty with piezosurgical tools and following histological examination. After histological diagnosis of PGCG, PHPT screening was performed dosing parathyroid hormone and serum calcium. In all the patients haematological investigation demonstrated elevated values of parathyroid hormone and serum calcium ruling out an unknown PHPT. Specifically, after endocrinological evaluation, patients showed PHPT related to: parathyroid adenoma (13), parathyroid hyperplasia (two, one of which occurred in a intra-thyroidal parathyroid), and parathyroid carcinoma (1) and were scheduled for surgical treatment. Considering that PGCGs could represent the first clinical sign of an undiagnosed PHPT and the screening of PHPT is a non-invasive and cheap exam, in case of histological diagnosis of a giant cell lesion, both central and peripheral, especially in patients with synchronous or history of methacronous giant cell lesions, parathyroidal screening should be mandatory

Spitz Nevus with Features of Clark Nevus, So-Called SPARK Nevus: Case Series Presentation with Emphasis on Cytological and Histological Features

Background: SPARK nevus represents a little-known and characterized entity, with few case series available in the literature. Methods and results: we present a case series of 12 patients (6 F and 6 M) between January 2005 and December 2020 and conduct a review of the current literature. Ten articles were selected on the basis of the adopted inclusion criteria and the PRISMA guidelines. Conclusions: The definition of histopathological and dermoscopic criteria are important to allow for an agreement to be reached among dermopathologists, and for the development of a consensus on higher case studies. To our knowledge, there are not many case series in the literature, and ours is part of the attempt to increase the knowledge of an entity that remains little-known and characterized

New insights in Diffuse Large B Cell Lymphoma Pathobiology

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma (NHL), accounting for about 40% of all cases of NHL. Analysis of the tumor microenvironment is an important aspect of the assessment of the progression of DLBCL. In this review article, we analyzed the role of different cellular components of the tumor microenvironment, including mast cells, macrophages, and lymphocytes, in the tumor progression of DLBCL. We examined several approaches to confront the available pieces of evidence, whereby three key points emerged. DLBCL is a disease of malignant B cells spreading and accumulating both at nodal and at extranodal sites. In patients with both nodal and extranodal lesions, the subsequent induction of a cancer-friendly environment appears pivotal. The DLBCL cell interaction with mature stromal cells and vessels confers tumor protection and inhibition of immune response while delivering nutrients and oxygen supply. Single cells may also reside and survive in protected niches in the nodal and extranodal sites as a source for residual disease and relapse. This review aims to molecularly and functionally recapitulate the DLBCL-milieu crosstalk, to relate niche and pathological angiogenic constitution and interaction factors to DLBCL progression

Fibrolipoma of the lip treated by diode laser surgery: A case report

<p>Abstract</p> <p>Introduction</p> <p>Several neoplasms of the adipose tissue can involve the soft tissues of the head and neck region. These neoplasms are mainly treated surgically and an accurate histological examination is mandatory for a precise diagnosis.</p> <p>Case presentation</p> <p>We report a case of fibrolipoma involving the lower lip of a 43-year-old man, which was successfully treated by diode laser surgery. This approach allowed adequate resection of the neoplasm with minimal damage to the adjacent tissues, thus reducing post-surgical scarring.</p> <p>Conclusion</p> <p>Diode laser surgery for the treatment of benign lesions of the oral mucosa appears to be a convenient alternative to conventional blade surgery and has proved to be effective for the excision of fibrolipoma of the lip. The possibility of avoiding direct suture after excision is surely helpful when aesthetic areas, such as the lip, are surgically treated. For these reasons, and also considering the lower histological alteration of the specimen obtained with diode laser surgery if adequately used, the diode laser is undoubtedly a good alternative to conventional surgery.</p

Primary Oro-Facial Manifestations of Langerhans Cell Histiocytosis in Pediatric Age: A Bi-Institutional Retrospective Study on 45 Cases

Aims: Langerhans Cell Histiocytosis is a rare hematologic disorder usually affecting children and most commonly involving the head and neck region. Primary oro-facial manifestations are rare, and their diagnosis is often challenging as they are numerous and often resemble common pathologies, refractory to conventional medical and/or instrumental treatments. For such reasons, the diagnosis is frequently delayed, as is the following staging and therapy onset. We retrospectively studied 45 pediatric patients affected by Langerhans Cell Histiocytosis with onset in the head and neck, to examine their clinical and radiological features at the early stage. Materials and Methods: The study was a retrospective bi-institutional analysis (Department of Pediatric Dentistry and Pediatric Oncology of “Sapienza” University of Rome, Department of Interdisciplinary Medicine of the University of Bari “Aldo Moro”), which enrolled 45 patients (age range 0–18 year-old) affected by Langerhans Cell Histiocytosis with oro-facial onset. Data regarding clinical appearance, number, site, synchronous or metachronous occurrence, involved tissues/organs, radiographic features and clinical outcomes were collected, listed and overall differentiated by two age ranges (0–10-year-olds and 10–18-year-olds). Results: Patients were 26 males and 19 females, with an average age at the time of diagnosis of 4.8 ± 3.8 years (median = 3.9 years). The most common findings were inflamed, hyperplastic, painful and often ulcerated gingival lesions (22 cases), associated with deciduous tooth mobility and/or dislocation with bone loss in 18 cases, followed by nine single eosinophilic granulomas of the mandible and two of the maxilla. Lesions of the palatal mucosa were observed in six patients; nine patients showed on radiograms the characteristic “floating teeth” appearance in the mandible with synchronous lesions of the maxilla in six. Paresthesia was relatively un-frequent (three cases) and the pathological fracture of the mandible occurred in six. Head/neck lymph nodes involvement was associated with oral lesions in 12 cases and skull lesions in 14. Otitis (media or externa) was detected in four instances, exophthalmia in two, cutaneous rush in nine, contextual presence or subsequent onset of insipidus diabetes in eight. As for therapy, single or multiple small jaw lesions were all surgically removed; chemotherapy with vinblastine alone or associated with corticosteroids was the principal treatment in almost the 80% of cases; more than 50% of patients received corticosteroids, while only three patients received adjunctive radiotherapy. The overall mortality account for less than 9% (four of 45 cases) and recurrence observed in eight patients after therapy. Conclusions: Langerhans Cell Histiocytosis may mimic several oro-facial inflammatory and neoplastic diseases. Considering the potential disabling sequela following head and neck localization of Langerhans Cell Histiocytosis in children, especially at the periodontal tissues with teeth and alveolar bone loss, lesion recognition along with the histological examination of suspicious tissues is mandatory to achieve an early diagnosis and to prevent further organ involvement

Covid-19 Symptomatic Patients with Oral Lesions: Clinical and Histopathological Study on 123 Cases of the University Hospital Policlinic of Bari with a Purpose of a New Classification

The aim of this study is to report on the oral lesions detected in 123 patients diagnosed at the University Hospital of Bari from October 2020 to December 2020, focusing on the correlation of clinical and pathological features in order to purpose a new classification. Methods. General and specialistic anamnesis were achieved and oral examination was performed. The following data were collected: age/gender, general symptoms and form of Covid-19, presence and features of taste disorders, day of appearance of the oral lesions, type and features of oral lesions and day of beginning of therapies. If ulcerative lesions did not heal, biopsy was performed. Results. Many types of oral lesions were found and classified into four groups considering the timing of appearance and the start of the therapies. Early lesions in the initial stages of Covid-19 before the start of therapies was observed in 65.9% of the patients. In the histopathological analysis of four early lesions, thrombosis of small and middle size vessels was always noticed with necrosis of superficial tissues. Conclusion. The presence of oral lesions in early stages of Covid-19 could represent an initial sign of peripheral thrombosis, a warning sign of possible evolution to severe illness. This suggests that anticoagulant therapies should start as soon as possible

Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patient

PReferentially Expressed Antigen in MElanoma (PRAME):preliminary communication on a translational tool able to early detect Oral Malignant Melanoma (OMM)

: Oral malignant melanoma (OMM) has a prevalence less than 1% of all melanomas and it commonly develops on the oral mucosa following a slow and unspecific transformation of unstable melanocytic lesions, often resulting in a diagnostic delay. The marker PReferentially Expressed Antigen in MElanoma (PRAME) seems to be a valid tool to investigate the biological and histological nature of cutaneous melanocytic lesions, but to date its use to characterize pigmented lesions in the oral cavity is largely unexplored. The aim of this study was to create preliminary knowledge on the PRAME expression in OMM, and to compare its expression respect to other dysplastic pigmented lesions of the oral cavity. Interestingly, PRAME has been demonstrated to be reliable in the clinical conditions investigated in our pilot study; in fact, it has clearly differentiated the cases of Melanoma, which showed diffuse and intense positivity (score 6+/7+) to PRAME, from the other melanocytic nevi, which resulted to be mainly negative to PRAME. This means a better differential diagnosis, a reliable early diagnosis and a proper clinical/surgical management of the oncological lesions. In conclusion, PRAME can be a valid qualitative marker for differential diagnosis, not only in cutaneous melanomas, but also in malignant melanoma of the entire head and neck area

New Dimensional Staging of Bisphosphonate-Related Osteonecrosis of the Jaw Allowing a Guided Surgical Treatment Protocol: Long-Term Follow-Up of 266 Lesions in Neoplastic and Osteoporotic Patients from the University of Bari

Bisphosphonate-related osteonecrosis of the jaw (BRONJ) is the most serious side effect in patients receiving bisphosphonates (BPs) for neoplastic disease and osteoporosis. The aim of this study is to propose a new dimensional stage classification, guiding the surgical treatment of BRONJ patients, and to evaluate the success rate of this new management. From 2004 to 2013, 203 neoplastic and osteoporotic patients with 266 BRONJ lesions were referred to the Odontostomatology Unit of the University of Bari. All patients underwent surgery after suspension of BPs therapy and antibiotic treatment. The surgical procedure was complemented by piezosurgery and followed by the application of hyaluronate and amino acids. The new dimensional staging suggests the choice of the surgical approach, and allows the prediction of postoperative complications and soft and hard tissues healing time, guiding the surgical treatment protocol. This protocol could be a successful management strategy for BRONJ, considering the low recurrences rate and the good stabilisation of the surgical sites observed after a long-term follow-up