Nevoid Basal Cell Carcinoma Syndrome Research Articles

Prognostic relevance of clinicopathological factors in sporadic and syndromic odontogenic keratocysts: A comparative study.

Current evidence suggests that nevoid basal cell carcinoma syndrome (NBCCS)-associated odontogenic keratocysts (OKCs) exhibit more aggressive clinical behavior and a higher tendency to relapse. The prognostic efficacy of various markers in sporadic and syndromic OKCs is unclear, and so are the results of studies on the usefulness of immunohistochemistry in distinguishing syndromic from sporadic OKCs. This retrospective study aimed to compare the prognostic relevance of various clinicoradiological and histopathological features, as well as the immunoexpression of COX-2, Bcl-2, proliferating cell nuclear antigen (PCNA), p53, Ki-67, osteoprotegerin (OPG), receptor activator of nuclear factor κ B (RANK) and receptor activator of nuclear factor κ B ligand (RANKL), as well as RANKL/OPG balance between sporadic and syndromic OKCs, and to test their utility in distinguishing the 2 types of OKC. We compared the immunoexpression of the aforementioned markers between 31 sporadic and 12 syndromic OKCs, and tested clinicopathological findings and levels of immunostaining against recurrence. We found a significant association between NBCCS and OKC recurrence. There were significant differences in PCNA, p53 and OPG immunoexpression between sporadic and syndromic OKCs. We also found that recurrent sporadic OKCs were significantly larger and markedly more often associated with cortical perforation. Recurrent sporadic OKCs exhibited COX-2 upregulation, but we failed to demonstrate its prognostic relevance. Recurrent syndromic OKCs showed a markedly higher RANKL > OPG ratio. The NBCCS-associated OKCs are significantly more prone to recur than their sporadic counterparts. Larger size and radiological signs of cortical perforation in sporadic OKCs may indicate a higher risk of recurrence. The COX-2 is upregulated in recurrent sporadic OKCs, whereas recurrent syndromic OKCs exhibit higher RANKL and lower OPG expression; however, these findings have no prognostic relevance. The immunoexpression of p53, PCNA and OPG may help to distinguish syndromic from sporadic OKCs.

Gorlin Goltz Syndrome- A Rare Disease Reported In Bangladesh

Gorlin-Goltz syndrome is an infrequent multisystemic disease with an autosomal dominant trait with complete penetrance and various expressivity. Gorlin Goltz Syndrome is a rare autosomal characterized by an increased predisposition to basal cell carcinoma and associated with multiorgan anomalies having a high level of penetrance. We report here a 60-year-old patient with positive findings of Gorlin-Goltz Syndrome.The following report emphasizes the identification of all the essential clinical diagnostic criteria ,radiological manifestations, and possible genetic tests to be performed for setting up an adequate treatment plan. Update Dent. Coll. j: 2022; 12(2): 32-36

Molecular Mechanisms and Targeted Therapies of Advanced Basal Cell Carcinoma.

Among human cutaneous malignancies, basal cell carcinoma is the most common. Solid advances in unveiling the molecular mechanisms of basal cell carcinoma have emerged in recent years. In Gorlin syndrome, which shows basal cell carcinoma predisposition, identification of the patched 1 gene (PTCH1) mutation was a dramatic breakthrough in understanding the carcinogenesis of basal cell carcinoma. PTCH1 plays a role in the hedgehog pathway, and dysregulations of this pathway are known to be crucial for the carcinogenesis of many types of cancers including sporadic as well as hereditary basal cell carcinoma. In this review, we summarize the clinical features, pathological features and hedgehog pathway as applied in basal cell carcinoma. Other crucial molecules, such as p53 and melanocortin-1 receptor are also discussed. Due to recent advances, therapeutic strategies based on the precise molecular mechanisms of basal cell carcinoma are emerging. Target therapies and biomarkers are also discussed.

Dental Screening Including Panoramic Radiograph for Gorlin-Goltz Syndrome in Patients With Multiple Basal Cell Carcinomas.

To answer the following clinical research question: "Among patients with multiple basal cell carcinomas (mBCCs), can panoramic radiograph (PaR) facilitate the diagnosis of Gorlin-Goltz syndrome (GGS)?" This retrospective study enrolled mBCCs subjects who presented to a German tertiary care center between 1 January 2015 and 31 December 2021. The primary predictor was presence of syndromic mBCCs, and the main outcomes were jaw cysts and odontogenic keratocysts (OKCs). Descriptive, bi- and multivariate statistics, diagnostic test evaluation, and number needed to screen (NNS) were computed at α = 95%. The sample comprised 527 mBCCs patients (36.1% females; 6.8% GGS; 5.5% OKCs; mean age, 74.5 ± 15.8 years [range, 15-102]). There was a significant association between syndromic mBCCs and jaw cysts (P < .0001; NNS = 2 [95% CI, CI, 1.1 to 1.4]). In the adjusted logistic model, PaR identified GGS via radiographic diagnosis of jaw cysts in case of 1) age ≤ 35 years, 2) ≥ 5 BCCs, and 3) ≥ 1 high-risk BCCs. Nearly every jaw cyst identified by PaR was OKCs (P = .01; 95% CI, 3.1 to 3,101.4; NNS = 1.3 [95% CI, .9 to 2]). The post hoc power was 100%. Dental screening with the use of PaR for mBCCs patients, especially those aged ≤35 years, or with ≥5 BCCs, or ≥1 high-risk BCCs, may be helpful in detection and identification of GGS through recognition of OKCs.

Gorlin-Goltz Syndrome

Gorlin-Goltz syndrome (GGS) is a rare autosomal dominant condition. Diverse incidence rates of this syndrome have been reported all over the world. It is estimated to occur 1 in 50,000–150,000. GGS is of particular interest to dental surgeons and maxillofacial radiologists because of their association with odontogenic keratocysts (OKC). GGS associated with OKCs tend was known to occur in individuals who were at a much younger age. In our case, cysts that were radiographically similar to OKCs were present in our male patient. OKCs associated with GGS are reported to be more frequent in the maxilla than in the mandible. GGS may manifest with the triad of basal cell nevi, keratocysts of the jaws, and deformities in the skeletal system. Recognition of the condition is crucial for establishment for early diagnosis as it can minimize the severity of the condition. The aim of this article was to report a case of GGS who was diagnosed primarily based on craniofacial manifestations.

Nervous system (NS) Tumors in Cancer Predisposition Syndromes.

Genetic syndromes which develop one or more nervous system (NS) tumors as one of the manifestations can be grouped under the umbrella term of NS tumor predisposition syndromes. Understanding the underlying pathological pathways at the molecular level has led us to many radical discoveries, in understanding the mechanisms of tumorigenesis, tumor progression, interactions with the tumor microenvironment, and development of targeted therapies. Currently, at least 7-10% of all pediatric cancers are now recognized to occur in the setting of genetic predisposition to cancer or cancer predisposition syndromes. Specifically, the cancer predisposition rate in pediatric patients with NS tumors has been reported to be as high as 15%, though it can approach 50% in certain tumor types (i.e., choroid plexus carcinoma associated with Li Fraumeni Syndrome). Cancer predisposition syndromes are caused by pathogenic variation in genes that primarily function as tumor suppressors and proto-oncogenes. These variants are found in the germline or constitutional DNA. Mosaicism, however, can affect only certain tissues, resulting in varied manifestations. Increased understanding of the genetic underpinnings of cancer predisposition syndromes and the ability of clinical laboratories to offer molecular genetic testing allows for improvement in the identification of these patients. The identification of a cancer predisposition syndrome in a CNS tumor patient allows for changes to medical management to be made, including the initiation of cancer surveillance protocols. Finally, the identification of at-risk biologic relatives becomes feasible through cascade (genetic) testing. These fundamental discoveries have also broadened the horizon of novel therapeutic possibilities and have helped to be better predictors of prognosis and survival. The treatment paradigm of specific NS tumors may also vary based on the patient's cancer predisposition syndrome and may be used to guide therapy (i.e., immune checkpoint inhibitors in constitutional mismatch repair deficiency [CMMRD] predisposition syndrome) [8]. Early diagnosis of these cancer predisposition syndromes is therefore critical, in both unaffected and affected patients. Genetic counselors are uniquely trained master's level healthcare providers with a focus on the identification of hereditary disorders, including hereditary cancer, or cancer predisposition syndromes. Genetic counseling, defined as "the process of helping people understand and adapt to the medical, psychological and familial implications of genetic contributions to disease" plays a vital role in the adaptation to a genetic diagnosis and the overall management of these diseases. Cancer predisposition syndromes that increase risks for NS tumor development in childhood include classic neurocutaneous disorders like neurofibromatosis type 1 and type 2 (NF1, NF2) and tuberous sclerosis complex (TSC) type 1 and 2 (TSC1, TSC2). Li Fraumeni Syndrome, Constitutional Mismatch Repair Deficiency, Gorlin syndrome (Nevoid Basal Cell Carcinoma), Rhabdoid Tumor Predisposition syndrome, and Von Hippel-Lindau disease. Ataxia Telangiectasia will also be discussed given the profound neurological manifestations of this syndrome. In addition, there are other cancer predisposition syndromes like Cowden/PTEN Hamartoma Tumor Syndrome, DICER1 syndrome, among many others which also increase the risk of NS neoplasia and are briefly described. Herein, we discuss the NS tumor spectrum seen in the abovementioned cancer predisposition syndromes as with their respective germline genetic abnormalities and recommended surveillance guidelines when applicable. We conclude with a discussion of the importance and rationale for genetic counseling in these patients and their families.

Retroauricular dermoid cyst associated with Gorlin syndrome. A case report

Gorlin syndrome is an autosomal dominant genodermatosis characterized by the early appearance of multiple basal cell carcinomas, odontogenic keratocysts and skeletal abnormalities. It is caused by mutations in the hedgehog signalling pathway, primarily in the tumour suppressor gene PTCH1 (9q22.1-q31). We present a 14-year-old girl who consulted for asymmetrical ears, in the context of multiple disorders such as mental retardation, snoring, non-specific coagulation abnormalities, retrognathia, pectus excavatum and scaphoid duplication. During the intervention, a retroauricular cystic tumour was found incidentally, reported by Pathology as a dermoid cyst. The syndrome is confirmed by a genetic study with the result of a new pathological variant in PTCH1. We describe the coexistence of this entity with a dermoid cyst. Furthermore, it is exceptional in its retroauricular location and the pathological point mutation in the PTCH1 gene, consisting of the pCys56Gly variant.

Gorlin Syndrome Associated With a Solitary Circumscribed Retinal Astrocytic Proliferation in a Pediatric Patient.

Gorlin syndrome is a rare autosomal dominant disorder with near complete penetrance. The underlying genetic mechanism is a mutation in a tumor suppressor gene. Thus far, mutations in patched homolog 1 and 2 genes (PTCH1 and PTCH2) and the suppressor of fused gene (SUFU) have been identified. The syndrome is characterized by neoplasms arising early in childhood as well as developmental abnormalities, including ophthalmic anomalies. We present the first case associating Gorlin syndrome with a rare retinal lesion known as solitary circumscribed retinal astrocytic proliferation (SCRAP). SCRAP is a benign, stable retinal tumor. For this reason, it is essential to differentiate it from similar retinal lesions that are associated with poor prognosis. [Ophthalmic Surg Lasers Imaging Retina 2022;53:514-516.].

Clinical Impact of Cardiac Fibromas

Cardiac fibromas are rare primary tumors that can cause significant morbidity and mortality. There has not been a large clinical case review since 1994. This study provides an updated analysis of clinical impact, thereby enhancing understanding, increasing awareness, and revealing important factors in the diagnosis and management of cardiac fibromas. A retrospective case series was conducted at a tertiary care institution by reviewing radiology, surgical and pathology archives (1964 to 2020). Cases were included if cardiac fibroma was diagnosed through imaging or pathology. Demographics, symptomatology, electrophysiologic data, radiographic findings, pathology, interventions, and outcomes were examined. A total of 26 patients with cardiac fibromas were identified, including 12 women. The median age was 20.5 years (0 days to 72 years). Symptoms included palpitations (commonly due to ventricular tachycardia, 31%), syncope (15%), angina (15%), heart failure (12%), emboli (4%), and murmur (27%). One patient had Gorlin syndrome. A total of 22 patients were diagnosed through imaging, 15 of whom were biopsy-confirmed. A total of 9 patients were initially observed. A total of 2 eventually had surgery, 1 was lost to follow-up, 3 were asymptomatic, 1 had heart failure and atrial fibrillation, and 1 had atrial fibrillation and tachy-brady syndrome, requiring ablation and pacemaker placement. A total of 19 underwent resection. A total of 4 required complex operations, 1 required a second resection, and 1 operative death occurred. In conclusion, cardiac fibromas primarily affect the pediatric population; however, this study demonstrates a significant prevalence in adults. Ventricular tachycardia is common, and multimodality imaging is diagnostically sensitive. Resection is largely successful in symptomatic patients. Surveillance may be appropriate for asymptomatic patients.

"Family History of Mandibular Keratocysts from Early Age: Gorlin Goltz Syndrome"

"Family History of Mandibular Keratocysts from Early Age: Gorlin Goltz Syndrome"

Multiple, non-syndromic BCCs involving a hypopigmented patch

Basal cell carcinoma (BCC) is the most common human malignant neoplasm. It has a slowly progressive nature and a locally invasive behavior. Both genetic predisposition and exposure to environmental risks are involved in its pathogenesis. An ever-increasing annual incidence is observed. Multiple BCCs may be seen in some syndromes such as nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin–Goltz syndrome. The occurrence of multiple, concurrent, non-syndromic BCCs is rare. Herein, we present a concise report of an observation of multiple, non-syndromic BCCs involving a hypopigmented patch on the forehead in an adult Afghani patient.

565 Gorlin Syndrome: A Case of Recurrence of Odontogenic Keratocysts

Abstract Gorlin Syndrome (GS) is a rare condition with multisystemic manifestations. Known features include basal cell carcinomas, odontogenic keratocysts (OKCs), and skeletal abnormalities. Multidisciplinary involvement is paramount with surgical intervention as a common treatment modality. Multiple OKCs with recurrence is common in GS. However, limited up-to-date guidelines are available on the appropriate radiographic monitoring of such lesions, in the form of orthopantomogram, in these patients. This is a case of a 12-year-old girl with GS and multiple episodes of OKCs. She is monitored regularly with the Oral and Maxillofacial team since the age of 3, after her dentist referred her due to her condition and its association with OKCs. A multidisciplinary approach with Dermatology, Pediatrics, Ophthalmology, Ear Nose and Throat, and Orthodontics and Restorative Dentistry within the dental specialty is undertaken to manage her condition. Yearly clinical reviews of her mouth and radiographs biannually were done, with her first episode of OKCs presenting at 10 years old. Orthopantomogram and magnetic resonance imaging revealed 6 OKCs – 2 in the maxilla, 4 in the mandible. These were surgically enucleated with liquid nitrogen cryotherapy adjunct, alongside extractions of associated deciduous teeth. Eighteen months following this, her OPG showed 2 new OKCs – 1 in the maxilla, 1 in the mandible. These were removed successfully in a similar fashion and active monitoring is ongoing. This case underscores the importance of regular clinical and radiographic follow-ups due to the frequency of multiple OKCs with recurrence in GS. Long-term multidisciplinary approach in this multisystemic syndrome is also paramount.

SELLA TURCICA BRIDGING IN PATIENTS WITH GORLIN-GOLTZ SYNDROME AND ITS IMPLICATIONS

Patients with Gorlin-Goltz syndrome, also called nevoid basal cell carcinoma syndrome (NBCCS), can present sella turcica bridging (STB), which has been associated with the development of dental and cervical vertebral anomalies. In our clinical cases series, we analyzed skull and cervical spine x-rays of 7 patients with NBCCS and STB. Three patients had STB associated with dental (agenesis, tooth impaction, hyperdontia, diastemata, microdontia, gyroversion, and supernumerary teeth) and cervical (rudimentary cervical discs and ossification of the nuchal ligament) anomalies, 3 had STB associated with dental alterations (tooth impaction, gyroversion, and agenesis), and 1 had STB associated with cervical abnormality (partial fusion of the vertebrae). The pathogenic mutation of genes (PTCH1, PTHC2, or SUFU) involved in the main pathway related to embryonic development, sonic hedgehog, promotes accumulate changes in the neural crest cells of patients with NBCCS, which phenotypically express as STB and dental and cervical vertebrae anomalies.

TREATMENT OF KERATOCYSTS IN CARRIER OF GORLIN-GOLTZ SYNDROME: CASE REPORT

Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome is a rare autosomal dominant disorder. Although there are cases of spontaneous mutation, it is mostly hereditary. This study reports the treatment of multiple keratocysts in a 13-year-old Afro-White male patient with facial asymmetry caused by the increased volume seen in the jaw and lower jaw. Pigmented macules on the face and skeletal anomalies were also observed. Image exams exhibited multiple unilocular radiolucent lesions in the maxilla and mandible. The anatomopathologic diagnosis, based on incisional biopsies, was keratocyst. To begin, marsupialization of cysts with a decompression device installation was the chosen procedure. After the lesions reduced, cryotherapy with liquid nitrogen was performed. The histopathologic diagnosis of keratocyst-associated Gorlin-Goltz syndrome was confirmed. The plan is to perform orthodontic traction of teeth involved with the injury. The patient is in follow-up without recurrence.

GORLIN-GOLTZ SYNDROME: A REPORT OF TWO CLINICAL CASES WITH DIAGNOSIS AND TREATMENT AT HOSPITAL LEVEL

Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare hereditary autosomal dominant condition, with high penetrance and variable expressivity. The aim of this study is to report 2 cases diagnosed at emergency hospital units with the specialty of maxillofacial surgery and traumatology. The first refers to a 10-year-old patient who sought consultation due to an expressive increase in the maxilla volume with panoramic radiography evidencing multiple unilocular radiolucent images in the gnathic bones. The second is a 20-year-old woman presenting extraoral increased volume in the mandibular region with signs of infection; imaging exams showed 2 unilocular radiolucent images in the mandible. After clinical, radiological, and histopathologic evaluation, multiple keratocysts, skeletal changes, and other criteria that characterize the syndrome were identified. In both cases, surgical treatment of the odontogenic cysts was performed, and the patients remain in multiprofessional follow-up.

MULTIPLES ODONTOGENIC KERATOCYST IN TWO CHILDREN (BROTHER AND SISTER): CASE REPORT

The aim of these study was to describe a clinical case of a brother, 13 years old, and sister, 11 years old, who reported purulent drainage in the posterior region of the mandible. Imaging exams showed multiples radiolucent lesions, bilateral, with well-defined limits, located at the anterior region, body, angle of the mandible, and posterior region of the maxilla. Many included teeth were seen in the imaging exams. The diagnostic hypotheses were odontogenic keratocyst related to Gorlin-Goltz syndrome. Surgical decompression was performed, and the histopathologic analysis confirmed the diagnosis of odontogenic keratocyst. The father reported that he had previously undergone surgery to remove a mandibular lesion but did not remember the diagnosis. The investigation of Gorlin-Goltz syndrome is still being performed. The children are in follow-up and lesions will be enucleated after reduction in the lesions’ size. The aim of these study was to describe a clinical case of a brother, 13 years old, and sister, 11 years old, who reported purulent drainage in the posterior region of the mandible. Imaging exams showed multiples radiolucent lesions, bilateral, with well-defined limits, located at the anterior region, body, angle of the mandible, and posterior region of the maxilla. Many included teeth were seen in the imaging exams. The diagnostic hypotheses were odontogenic keratocyst related to Gorlin-Goltz syndrome. Surgical decompression was performed, and the histopathologic analysis confirmed the diagnosis of odontogenic keratocyst. The father reported that he had previously undergone surgery to remove a mandibular lesion but did not remember the diagnosis. The investigation of Gorlin-Goltz syndrome is still being performed. The children are in follow-up and lesions will be enucleated after reduction in the lesions’ size.

IMMUNOHISTOCHEMICAL ANALYSIS OF CXCL12 AND CXCR4 IN SYNDROMIC AND NONSYNDROMIC ODONTOGENIC KERATOCYSTS

Objectives To evaluate the immunoexpression of chemokine CXCL12 and C-X-C chemokine receptor type 4 (CXCR4) in odontogenic keratocysts associated with Gorlin-Goltz syndrome (SOKCs) and nonsyndromic odontogenic keratocysts (NSOKCs). Study Design The percentages of cytoplasmic (CXCL12 and CXCR4) and nuclear (CXCR4) staining in epithelial and fibrous capsule cells of 22 SOKCs and 22 NSOKCs were determined. The results were analyzed statistically using the nonparametric Mann-Whitney test and Spearman correlation test. Results Higher cytoplasmic expression of CXCL12 was observed in the epithelial lining and fibrous capsule of NSOKCs compared to SOKCs (P < .001). No statistically significant differences in the cytoplasmic expression of CXCR4 were observed between SOKCs and NSOKCs (P > .05). Compared to SOKCs, NSOKCs exhibited higher nuclear expression of CXCR4 in the epithelial lining and lower immunoexpression in the fibrous capsule (P < .05). In the epithelial lining of SOKCs, a positive correlation was observed between cytoplasmic and nuclear expression of CXCR4 (P = .003). In the fibrous capsule of SOKCs and NSOKCs, cytoplasmic and nuclear expressions of CXCR4 were positively correlated (P < .001). Conclusions The results suggest a potential involvement of CXCL12 and CXCR4 in the development of OKCs. The heterogenous expression of these proteins in SOKCs and NSOKCs may reflect differences in their pathogenesis and biological behavior. To evaluate the immunoexpression of chemokine CXCL12 and C-X-C chemokine receptor type 4 (CXCR4) in odontogenic keratocysts associated with Gorlin-Goltz syndrome (SOKCs) and nonsyndromic odontogenic keratocysts (NSOKCs). The percentages of cytoplasmic (CXCL12 and CXCR4) and nuclear (CXCR4) staining in epithelial and fibrous capsule cells of 22 SOKCs and 22 NSOKCs were determined. The results were analyzed statistically using the nonparametric Mann-Whitney test and Spearman correlation test. Higher cytoplasmic expression of CXCL12 was observed in the epithelial lining and fibrous capsule of NSOKCs compared to SOKCs (P < .001). No statistically significant differences in the cytoplasmic expression of CXCR4 were observed between SOKCs and NSOKCs (P > .05). Compared to SOKCs, NSOKCs exhibited higher nuclear expression of CXCR4 in the epithelial lining and lower immunoexpression in the fibrous capsule (P < .05). In the epithelial lining of SOKCs, a positive correlation was observed between cytoplasmic and nuclear expression of CXCR4 (P = .003). In the fibrous capsule of SOKCs and NSOKCs, cytoplasmic and nuclear expressions of CXCR4 were positively correlated (P < .001). The results suggest a potential involvement of CXCL12 and CXCR4 in the development of OKCs. The heterogenous expression of these proteins in SOKCs and NSOKCs may reflect differences in their pathogenesis and biological behavior.

GORLIN-GOLTZ SYNDROME IN ADOLESCENTS—CASE REPORT

A 14-year-old dark-skinned female patient sought dental care for the presence of secretion in the right posterior maxilla. Panoramic x-ray showed well-defined radiolucent lesions at the posterior region of the 4 quadrants, with displacement of the respective included teeth and absence of expansion of the cortical bone. Tomography, incisional biopsy, and installation of a cannula for cystic decompression were requested. In the aspiration puncture, a whitish-colored material was found. Biopsies confirmed the diagnosis of odontogenic keratocyst. In the general clinical examination, palmoplantar depressions, hypertelorism and bifid ribs were observed on thorax x-ray. In view of the clinical, imaging, and histopathologic findings, the case was diagnosed as Gorlin-Goltz syndrome. The patient underwent surgery for keratocyst enucleation, extraction of related teeth, peripheral osteotomy, and application of Carnoy's solution. In the last evaluation, the patient presented with no signs of recurrence. A return visit was scheduled for June 2021.

DIAGNOSIS OF GORLIN-GOLTZ SYNDROME IN A PEDIATRIC PATIENT

Gorlin-Goltz syndrome (GGS) is a hereditary autosomal dominant disorder with high penetrance and variable expressivity. It is characterized by several basal cell carcinomas (BCC) of early onset, multiple odontogenic keratocysts (OKs), and skeletal abnormalities. Due to the broad clinical spectrum, treatment must be individualized and requires a multidisciplinary team. We presented a rare case of GGS in an 11-year-old female patient. Extraoral clinical examination showed discrete frontal bossing, hypertelorism, and palmoplantar pits. Imaging exams showed calcification of the falx cerebri and multiple osteolytic lesions in the mandible and maxilla. An incisional biopsy of the lesions was performed. The histopathologic diagnosis was OK. The OKs were treated through marsupialization and enucleation and curettage. The expression of several characteristics of GGS is rare in children and adolescents. Early diagnosis is essential to reduce the morbimortality associated with BCC.

Photodynamic therapy in pediatric age: Current applications and future trends.

Photodynamic therapy (PDT) is a photochemotherapy based on local application of a photosensitive compound and subsequent exposure to a light source of adequate wavelength. It is a non-invasive therapeutic procedure widely used in oncodermatology for treatment of numerous skin cancers, but in the last years its use has been gradually extended to an increasing list of skin diseases of both infectious and inflammatory nature. Although PDT is proven as a safe and effective therapeutic option in adults, its use is not well standardized in the pediatric population. In this review, we will focus on clinical applications, mechanisms of action, protocols, and adverse events in children and adolescents. Most of pediatric experiences concerned treatment of skin cancers in Gorlin syndrome and xeroderma pigmentosum, acne vulgaris, and viral warts, but other applications emerged, such as cutaneous lymphoma and pseudo-lymphomas, necrobiosis lipoidica, hidradenitis suppurativa, dissecting cellulitis, leishmaniasis, angiofibromas, verrucous epidermal nevus, and linear porokeratosis. In these pediatric diseases, PDT appeared as an effective therapeutic alternative. The results on vitiligo were limited and not fully encouraging. Although highly versatile, PDT is not a therapy for all skin diseases, and a deeper knowledge of its mechanisms of action is required to better define its spectrum of action and safety in pediatric patients.