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Purpose: To describe the histopathologic findings in a series of eyelid basal cell carcinomas removed from patients with basal cell nevus syndrome., Methods: Retrospective case series of 5 patients with basal cell nevus syndrome identified from our oculoplastics service. The systemic and ophthalmic features were reviewed, and a retrospective histopathologic analysis of all available previously excised eyelid lesions was performed. The pertinent published literature on basal cell nevus syndrome and eyelid basal cell carcinoma was reviewed., Results: A total of 26 eyelid lesions were examined histopathologically. Twenty-three of these lesions were basal cell carcinomas. The infundibulocystic variant of basal cell carcinoma was identified most commonly (57%)., Conclusions: Eyelid basal cell carcinomas in patients with basal cell nevus syndrome were commonly of the infundibulocystic variety in our series. Infundibulocystic basal cell carcinomas, which can be clinically indistinguishable from the more common forms, are thought to be less aggressive than other types of basal cell carcinoma and are a reassuring histopathologic diagnosis. It is important for the ophthalmologist and pathologist to be aware of infundibulocystic basal cell carcinomas, as they are more common in patients with basal cell nevus syndrome and may be a clue to the diagnosis of this autosomal dominant cancer-predisposition syndrome or other associated syndromes. To our knowledge, this variant of basal cell carcinoma has not been previously discussed in the ophthalmic literature.

Gorlin-Goltz (GG) syndrome is an inherited autosomal dominant condition. Its diagnosis may be clinically confirmed by checking either major or minor signs that define the diagnostic criteria. It may occur that, although GG syndrome is a well-known condition, only the specific symptom could be observed by different specialists. Therefore, the patient cannot be placed into an always complex clinical panel. We introduce an example in this report. Throughout a 20-year clinical history characterized by the lack of proper diagnosis and missed follow-up operations, a patient with GG syndrome underwent partial amputation of the jaw after severe complications. A 52-year-old man required an implant-prosthetic rehabilitation since becoming edentulous after a partial resection of the jaw due to a keratocyst, which was later reconstructed through a free fibula flap. The observation of a typical phenotype and various symptoms that succeeded for longer than 20 years, with anamnestic evaluation and clinical examination, led us to suspect a complex pathologic condition such as GG syndrome, which was not previously considered, although the patient had undergone several polyspecialistic evaluations. Diagnosis has been eventually confirmed by a genetic study, which was always mandatory. The simultaneous presence of muscular and skeletal malformations, basocellular nevi, and multiple cysts of the jaw can represent signs linking to a condition such as GG syndrome. There are many syndromes involving the head and neck region, and specialists are supposed to be alerted when faced with similar typical expressions associated with a characteristic soma so as to avoid delays in diagnosing the syndrome.

We report a case of testicular thecoma in an 11-year-old Japanese boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome). He presented with left testicular swelling and underwent a radical orchiectomy on suspicion of a malignant paratesticular tumor. The tumor arose from the testis exophytically and was diagnosed as a thecoma histopathologically. Ovarian thecoma-fibroma group tumors are closely associated with Gorlin syndrome or with abnormalities in PTCH, a candidate gene for the syndrome. The occurrence of an extremely rare testicular thecoma in this case (the second in the literature) suggests that such an etiological association may also exist in the pathogenesis of testicular tumors.

Gorlin-Goltz syndrome or Nevoid basal cell carcinoma syndrome is an autosomal dominant disorder with a predisposition to cancer. Features like basal cell carcinoma, odontogenic keratocysts, calcification of falx cerebri, bifid ribs, pits on palms and soles and hypertelorism are evident. A case of this rare disease seen on a 13 year old female patient is presented here, where multiple odontogenic keratocysts were causing disfigurement of the lower jaw as well as displacement and malocclusion of the lower teeth.

Background: Nevoid basal cell carcinoma syndrome (NBCCS) is characterized by the development of multiple basal cell carcinomas (BCCs). A major problem for these patients is the enormous amount of BCCs which can invade in the deep underlying structures, especially in the face. Different treatment modalities are used in these patients; surgical excision, Mohs micrographic surgery, cryotherapy, photodynamic therapy, ablative laser therapy and topical 5% imiquimod. There is no evidence based advice how to treat a NBCCS patient., Objective: To give a review of the literature about the possible treatment modalities for the multiple BCCs in NBCCS patients., Results: Literature consists mainly of case reports; no evidence based advice how to treat a NBCCS patient exists. Multiple treatments are available (surgical and non-surgical), and a lot of them can be combined. Treatment in a megasession is an option to diminish the medical and social inconvenience for the patient.

Basal cell carcinoma (BCC) is a rare disease in the pediatric population that usually presents in children with predisposing genetic conditions. It is often diagnosed on final pathology of an excisional biopsy, and the treatment may be delayed in younger populations because of a physician's low index of suspicion. Increased knowledge of BCC by pediatric caregivers would expedite definitive therapy for childhood BCC as well as any necessary evaluation by subspecialists for predisposing syndromes. We report 3 cases of BCC in pediatric patients and review the literature concerning BCC in children.

Gorlin-Goltz syndrome is a rare autosomal dominant disorder that involves multiple organ systems, including the skin, skeleton and jaws. We report the case of a mild mentally retarded 7-year-old boy who was referred with a swelling of his left mandible. Imaging studies showed a unilocular well-defined lytic mandibular lesion, calcifications of the falx, bifid ribs and fusion anomalies of the ribs. The mandibular lesion was treated with surgical decompression and proved to represent a keratocyst on histological examination. Further clinical examination revealed cutaneous lesions, Sprengel deformity, pectus excavatum and facial dysmorphism. Based on the combination of imaging and clinical findings the diagnosis of Gorlin-Goltz syndrome was made. This was confirmed by genetic tests. During three-year follow-up the boy presented with recurrent and multiple odontogenic keratocysts. The occurrence of multiple and recurrent keratocysts at young age, should alert the radiologist to the potential diagnosis of an underlying Gorlin-Goltz syndrome. This paper reviews the imaging findings in Gorlin-Goltz syndrome, with emphasis on maxillofacial imaging.

Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinomas, maxillary keratocysts, and musculoskeletal malformations. Occasionally, it is associated with aggressive basal cell carcinomas and internal malignancies. Early diagnosis and treatment are essential, as well as genetic counseling. A patient with characteristic symptoms of nevoid basal cell carcinomas and a review of the literature are presented.

A retrospective analysis was conducted on patients diagnosed with and treated for keratocystic odontogenic tumor (KCOT) at Mashhad School of Dentistry between 1996 and 2006. The patients comprised 44 men and 30 women with a mean age of 27.08 years. Among the total of 83 lesions, 56 (67.5%) occurred in the mandible and 27 (32.5%) in the maxilla. Swelling tended to be the most common complaint (45.8%), while 24.1% of the lesions were diagnosed incidentally. Six patients (8.1%) with a total of 15 lesions had nevoid basal cell carcinoma syndrome; 28 lesions (33.7%) were associated with an impacted tooth, and 12 (14.5%) presented daughter cysts. Sixty-six KCOTs were treated by enucleation [5 recurrences (7.6%)], 6 by marsupialization [2 recurrences (33.3%)] and 11 by marsupialization followed by enucleation (no recurrences). KCOTs in the mandible showed a higher recurrence rate than those in the maxilla (10.7% vs 3.7%). Although the demographics of Iranian patients are closely similar to those of other nationalities, in this series KCOTs tended to develop in younger patients with a peak in teenagers. The posterior region of the mandible showed the highest likelihood of KCOT occurrence and recurrence. Marsupialization followed by enucleation resulted in the lowest recurrence rate.

Background: The nevoid basal cell carcinoma syndrome (NBCC) is a rare and autosomal dominant inherited disease with well-defined characteristics, summarized by Gorlin and Goltz in 1960. In the head and neck region, cerebral calcifications, basal cell carcinoma (BCC) and multiple keratocysts of the jaws are the predominant findings. The aim of this study was to determine the diagnostic findings and the therapy for patients with NBCC., Patients and Methods: The medical files of 17 patients with NBCC, treated in a single institution (females: 9, males: 8) were evaluated. Thirteen patients were also physically investigated, including X-ray diagnosis., Results: The age at the time of first surgical treatment related to the syndrome was 3 to 57 years (mean: 21.3 years). A family history for NBCC was evident for 4 patients. The number of patients with characteristic head and neck findings in the spectrum of NBCC varied: basal cell carcinoma (n = 15), keratocysts of the jaws (n = 13), cerebral calcification visible on plain radiographs (n = 15), palmar pits (n = 9). Facial dysmorphism (hypertelorism) was evident in 4, and skeletal anomalies outside the skull occurred in 10 patients. The medical histories revealed a cleft lip and palate in 2, and unilateral kidney agenesis in further 2 patients, emphasizing the variability of the syndrome. Treatment was exclusively surgical in all but 2 patients. One of these 2 patients underwent external irradiation for a BCC of the frontotemporal region. Nine years later a frontal BCC had to be treated. The other developed several other BCC inside and outside the irradiation field. Up to 50 BCC per patient had to be resected. The number of keratocysts of all patients was 66, with a predeliction for the mandibular angle in 44%. On cranial computed tomograms (CCT, n = 9) a number of calcifications became evident: falx (8/9), tentorium (9/9), petrosellar ligament (2/9) and carotid siphon (1/9). Cerebral cysts occurred in one third of these patients (3/9). One patient underwent surgery for a medulloblastoma during childhood. In this series of CCT of 9 patients, no cortical atrophy was found., Conclusion: The NBCC is a well-known syndrome with a variety of findings inside and outside the head and neck region. Interdisciplinary cooperation is mandatory in the diagnosis and follow-up control of patients with NBCC.

Basal cell nevus syndrome is an autosomal dominant disorder characterized by multiple basal cell carcinomas, along with numerous other documented clinical features. Acrochordons (or skin tags) are common benign neoplasms that are appropriately left untreated in most patients. We describe two patients with known BCNS who were found to have multiple BCCs that clinically resembled acrochordons. Our findings support the biopsy of acrochordon-like growths in patients with basal cell nevus syndrome to rule out basal cell carcinoma.

The authors present the case of a 2.5-year-old African-American boy with desmoplastic medulloblastoma (MB) and nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, an autosomal dominant disorder resulting from mutations in the patched (PTCH) gene that predisposes to neoplasias (including basal cell carcinomas [BCCs] and MB) and to widespread congenital malformations. The diagnosis of NBCCS was suspected based on the clinical examination, patient and family medical histories, and histopathological characteristics of the tumor. Radiotherapy was withheld. The diagnosis of NBCCS was confirmed by DNA testing, which revealed a novel mutation in the PTCH gene. This is the first report of an African-American child with MB diagnosed with NBCCS prior to radiotherapy. Although only a small number of patients with MB have NBCCS, the diagnosis must be considered because radiotherapy in such patients can lead to the formation of BCCs and other intracranial neoplasms within the irradiated field. This case emphasizes the importance of obtaining thorough family and patient medical histories and of carefully examining the patient and close relatives for signs of NBCCS to avoid the potentially devastating consequences of missing this diagnosis.

Gorlin's syndrome is a genetic disorder of autosomal dominant inheritance with characterized primarily by five major findings: multiple basal cell carsinoma (BCC), jaw cysts, pits on the palms and soles, ectopic calcification of the falx cerebri and skeletal anomalies. BCC is the most frequently accompanied tumor with this syndrome. The risk of recurrent BCC with Gorlin's syndrome is higher than non-syndromic BCC. The authors present a 25-year-old man affected by recurrent basal cell carcinoma on the scalp. The patient was treated by excising the tumor and reconstructing latissimus dorsi musculocutaneous flap. The fascial component of the scalp forms an additional layer between the skin and the cranium. This structure creates an extra distance before the invasion into the cranium which needs to be penetrated by the skin tumor. Muscle tissue transformed in scalp reconstruction imitates the fascia layer in forming an additional layer against the invasion of skin tumors such as recurrent BCC into the cranium. Free flap reconstruction for recurrent scalp BCC can be best therapy model at Gorlin's syndrome.

We report the case of an 8-month-old male infant presenting with life-threatening ventricular tachycardia. Echocardiography revealed a left ventricular tumor. The tumor was resected through a left ventriculotomy, and the left ventricle was reconstructed after a partial ventriculectomy Histologic investigation showed a completely resected benign fibroma. The 30-year-old mother was known to have nevoid basal-cell carcinoma syndrome, which can be associated with cardiac fibromas. We believe that this is an interesting addition to the medical literature.

Nevoid basal cell carcinoma syndrome (NBCCS) is characterised by skeletal anomalies, cutaneous basal cell carcinomas and multiple keratocysts. NBCCS is an autosomal dominant disorder, but can have a variable phenotypic penetration. NBCCS can also arise spontaneously. The prevalence is 1:60.000 and 50-65% of patients with NBCCS have affected family members. A recently diagnosed patient is presented and the manifestations of the syndrome are discussed.

Pediatric, orthopedic, plastic, and hand surgeons perform the surgical correction of syndactylism. It is sometimes forgotten, however, that syndactylism can be part of a syndrome. The components of such a syndrome can each be life threatening. A 7-month-old boy was hospitalized for correction of cutaneous syndactylism. The mother claimed that, with the exception of the syndactylism, her son was healthy. However, review of the admission documents found that the family physician suspected Gorlin Goltz syndrome. A preoperative echocardiography showed 3.9-cm intramural tumor in the wall of the left ventricle. Electrocardiography documented ventricular tachycardia. Because of the danger of life-threatening malignant ventricular tachycardia, the tumor was resected. Before the resection, cardiac transplantation was considered because of the size of the tumor. Histologic examination found a fibroma. When observing syndactylism, one must always be aware of the possibility of a syndromic disease. It is particularly important that screening investigations, including electrocardiography and echocardiography, are performed before surgical treatment.

The nevoid basal cell carcinoma syndrome (NBCCS) or Gorlin-Goltz Syndrome is an autosomal dominant disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. The present report reviews current knowledge of this disorder that has profound relevance to specialists in Oral and Maxillo-Facial Surgery, Oral Medicine and Radiology.

Background: Numerous possibilities are available for the reconstruction of facial bone defects. The materials used to fill such defects must satisfy various requirements. One of the most important is that they must undergo transformation into autologous bone tissue in the process of remodeling., Aim: A report is given of the long-term results of augmentations of large bone defects performed with different bone-substitute materials in two patients., Patients and Methods: In one case, augmentation was carried out with beta-tricalcium phosphate after the removal of a fibromyxoma. In the second case, three large cystic lesions in the mandible of a patient with Gorlin-Goltz syndrome were filled with beta-tricalcium phosphate, with a mixture of beta-tricalcium phosphate and platelet concentrate, or with hydroxyapatite of algal origin. The process of ossification was checked at 6-month intervals by means of clinical, radiologic (orthopantomograms and two-dimensional and three-dimensional computer tomograms), and histologic methods., Results: At 1 year after the intervention, the site of the augmentation was in all cases occupied by hard tissue of good quality. With the given imaging procedures, it was difficult to distinguish between the original bone and the region filled with bone-substitute material. The three-dimensional computer tomogram images indicated that the contours and quality of the new bone corresponded with the physiologic and anatomical conditions. The histologic examinations show the remodeling of the bone-substitute materials., Discussion: The bone-substitute materials applied in these cases fully satisfied the demands of transformation into bone (remodeling). The speed of remodeling seemed to be the fastest when the mixture of beta-tricalcium phosphate and platelet concentrate was used.

Background: Multiple surgical procedures are often a source of discomfort, pain, and disfigurement for patients with the nevoid basal cell carcinoma syndrome (NBCCS)., Objective: The patient with NBCCS had approximately 45 identifiable basal cell carcinomas on her face. Multiple Mohs surgeries were performed to remove the largest tumors. A tumor-free plane was not achieved in several lesions despite removal of multiple tissue layers. The treatment of the remaining tumors presented a significant challenge., Methods: Full-face CO2 laser resurfacing was performed after curettage and local laser ablation of all visible tumors., Results: The patient healed well postoperatively. She developed six basal cell carcinomas on her face during the 10-month follow-up period. These were easily treated with Mohs surgery and imiquimod cream., Conclusion: CO2 laser resurfacing is a useful surgical modality for treatment of multiple facial basal cell carcinomas in the patients with NBCCS. It allows treatment of all the facial lesions in a single session and minimizes suffering resulting from repeated surgical procedures. Additionally, the layer of skin damaged by ultraviolet radiation is removed, allowing for easier management after resurfacing.

Basal cell nevus syndrome is an autosomal dominant condition with complete penetrance and variable expressivity. It is characterized by five major components, including multiple nevoid basal cell carcinomas, jaw cysts, congenital skeletal abnormalities, ectopic calcifications, and plantar or palmar pits. Other features include a host of benign tumors, ocular defects, and cleft lip and palate. Guidelines for diagnosis include a family history, careful oral and skin examinations, chest and skull radiographs, panoramic radiographs of the jaw, magnetic resonance imaging of the brain, and pelvic ultrasonography in women.

Background: Multiple basal cell carcinomas (BCCs) are infrequently seen in patients under 30 years of age. Their occurrence at a young age is often linked to some genodermatosis, including Nevoid Basal Cell Carcinoma Syndrome (NBCCS). The exposure to ionizing radiation is also considered to be a predisposing factor in the development of BCCs., Methods: We report the case of a 35-year-old patient who presented with seven synchronous, nodular, brownish-pigmented BCCs, confined within the radiation-treated cutaneous areas, 15 years after receiving Cobalt-60 (60Co) irradiation for Hodgkin's disease., Results: On the basis of clinical, radiological, and anamnestic data we excluded a NBCCS, thus proposing irradiation as the cause of the multiple synchronous pigmented BCCs., Conclusions: Previous therapeutic ionizing radiation leads to an increased risk of BCCs confined to the region of the body to which radiotherapy was delivered. We consider our patient's BCCs represents a late adverse effect of the treatment with Cobalt-60.

Purpose: To describe the ophthalmic and systemic features in a series of patients initially seen with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome., Design: Retrospective noncomparative case series., Participants: Of 105 consecutive patients with eyelid basal cell carcinoma managed at an Ocular Oncology Center between January 1973 and December 1999, four patients with Gorlin-Goltz syndrome were identified., Methods: The ophthalmic and systemic features, management, and outcome of patients with eyelid basal cell carcinoma associated with Gorlin-Goltz syndrome were analyzed. The published literature on Gorlin-Goltz syndrome, specifically related to genetics, systemic features, ophthalmic associations, and prophylactic management strategies, was reviewed., Main Outcome Measures: Response of the eyelid basal cell carcinoma to treatment and the final systemic condition were the main outcome measures., Results: All four patients had a family history of Gorlin-Goltz syndrome. The systemic manifestations included multiple basal cell carcinomas in all the patients, frontal bossing or increased occipitofrontal circumference in three patients, palmar pits in two patients, odontogenic keratocyst in one patient, ectopic calcification in one patient, and bifid rib in one patient. The mean age at the detection of the first basal cell carcinoma was 30 years (range, 16-38 years). All four patients had multiple basal cell carcinomas on the face and elsewhere. The eyelid basal cell carcinoma was advanced with orbital infiltration in three patients, one of whom opted for palliative radiotherapy, whereas the other two underwent orbital exenteration. The fourth patient, who had localized recurrent basal cell carcinoma in the upper eyelid, was treated with excision and eyelid reconstruction. At the final follow-up (mean, 41 months), eyelid basal cell carcinoma was cured in three patients and stable in one patient. No patient had life-threatening sequelae of Gorlin-Goltz syndrome., Conclusions: Gorlin-Goltz syndrome is a rare autosomal dominant cancer predisposition syndrome that may be associated with eyelid basal cell carcinoma. The associated systemic findings may be a clue to the diagnosis of this condition. It is important to recognize Gorlin-Goltz syndrome when a patient has multiple basal cell carcinomas or when a young patient with eyelid basal cell carcinoma is seen by an ophthalmologist, because lifelong monitoring is essential for patient management.

Nevoid basal cell carcinoma syndrome is an autosomal dominant condition characterized by multiple basal cell carcinomas, skeletal abnormalities and sometimes mental retardation. The large number of tumors, which are often disfiguring, presents extreme difficulties in the treatment of these patients. Microscopically controlled excision, compared to other modalities (radiation therapy, photodynamic therapy, intralesional interferon alpha-2b) offers the highest cure rate. However, because of the large size and involvement of wide areas of the skin, this approach is sometimes impractical. The ultrapulse CO2 laser with high energy and short pulses achieves char-free ablation of the tumors, bloodless surgical field, minimal nonspecific thermal damage, rapid healing and diminished postoperative pain. Also, a number of lesions can be removed in a single session. We present a 48-year-old man with a 6.5 x 4.5 cm large basal cell carcinoma involving the anterior abdomen and navel area. The central thick portion of the tumor was resected by microscopically controlled excision with 3 stages, and wide thinner peripheral crescentic plaque vaporized with ultrapulse CO2 laser. The laser settings were 300 mJ energy/pulse and 100 W average power, which corresponds to the fluence of 7.5 J/cm2. Computerized pattern generator (ultrascan handpiece) was adjusted to patterns of 3 (circle) and 1 (square) with sizes varying from 5 to 7, and density of 9 (60% overlapping). The tumor was vaporized with 6 passes, all the way to deep reticular dermis. A fifteen month-follow up disclosed no recurrent disease. Subsequent biopsies revealed only a scar with postinflammatory hyperpigmentation. Our experience indicates that combined treatment with microscopically controlled excision and ultrapulse CO2 laser ablation is a suitable modality for the large tumor plaques involving concave and convex areas of the skin respectively. Microscopically controlled excision of thicker, concave portions of basal cell carcinoma plaques, where CO2 laser surgery is less feasible, presents an effective addition that renders this combined modality a successful method for the treatment of nevoid basal cell carcinoma syndrome.

The treatment of multiple basal cell carcinomas in patients with Gorlin's syndrome presents a therapeutic challenge. The carbon dioxide laser presents a unique treatment option due to increased surgical speed, a bloodless operating field, reduced postoperative pain and discomfort, and acceptable scars. Six patients with Gorlin's syndrome have been treated with the carbon dioxide laser. Between 14 and 110 lesions were treated in one session under local anesthesia. Pre- and postlaser biopsies of the lesions confirm complete eradication of the tumors. Mean follow-up is 20 months. No local recurrence has been observed.

The major features of the nevoid basal-cell carcinoma syndrome are epidermal multiple cell carcinomas, cysts of the jaws and skeletal abnormalities, mainly in the ribs. The authors have reviewed the literature concerning multiple anomalies which are associated to this syndrome, and report a clinical case, emphasizing the advantages of early diagnosis, mainly for dermal and maxillary lesions.

A 28-year-old man presented to the authors' hospital with multiple intracranial tumors. At 2 years of age, he had undergone resection of a medulloblastoma and received adjunctive craniospinal irradiation. Subsequently, he was diagnosed with nevoid basal cell carcinoma syndrome, Gorlin's syndrome. Since his first presentation, he has required surgery for multiple basal cell carcinomas, an osteochondroma of the rib, two meningiomas, a trigeminal schwannoma, and a pleomorphic liposarcoma, all of which arose within the radiation field. Despite this impressive list of benign and malignant neoplasms, the patient is relatively well and leads a normal life. The authors examine the relationships between Gorlin's syndrome and radiation therapy and the subsequent development of tumors.

The Nevoid Basal-Cell Carcinoma Syndrome (NBCC), or as it is also referred to, basal-cell nevus syndrome or Gorlin-Goltz syndrome, is characterized by multiple early-appearing basal cell carcinomas, keratocytosis of the mandible, and anomalies of the ocular, skeletal reproductive system. We describe four patients in the same family, all of them possessing a large number of skin tumors associated with other typical clinical and X-Ray anomalies of NBCC. The definitive treatment of NBCC has yet to be established, however, early diagnosis is very important as well as the periodical follow-up examination of ten patients, mainly due to the transformations in the skin lesions that may occur.

The authors review clinical and X-ray examinations of 30 patients with keratocysts and show difficulties of differential diagnosis with other cysts. Keratocysts are aggressive and prone to recurrences. The authors disclose some clinico-radiological, macroscopic and histological features of keratocysts which may be helpful in valid diagnosis making.

Two cases of patients with Gorlin-Goltz syndrome are described. The Gorlin-Goltz syndrome is a polydistrectual, hereditary disease characterized by multiple keratocysts and basocellular nevomatosis. It is pointed out that the inconstancy of the main symptoms makes early diagnosis difficult. The histologic characteristics of odontogenic keratocysts and the possible etiopathogenetic relations between cystic lesions and hamartia of adjacent oral mucosa, as considered by Stoelinga, are also discussed. In view of the course of the cases presented, a surgical approach considering such potential findings, is supposed to favorably affect the clinical evolution in respect to a high relapsing drift.

Background: Even though 5-year cure rates are as high as 99% for Mohs micrographic surgery (MMS) of cutaneous carcinomas, theoretically 100% cure rates of unifocal carcinomas should be possible., Objective: To determine why cutaneous malignancies recur locally (persist) after MMS., Methods: A retrospective study of all cutaneous malignancies treated with MMS during a 4-year period at Washington University School of Medicine was made. All lesions that were locally recurrent after previous MMS were analyzed for any possible cause of the recurrence., Results: Of 2,414 cutaneous malignancies treated, 33 (1.4%) were locally recurrent after previous MMS by four different Mohs surgeons and five separate technicians. Technical or interpretation problems that could have accounted for the local recurrence were identified in 77% of the lesions. The mean time to recurrence was 38.5 months, with 20% developing more than 5 years after the initial MMS., Conclusion: MMS local recurrences are rare with procedural errors representing a majority of them. Five-year cure rates for basal cell carcinoma may somewhat overestimate the ultimate cure rate.

More than 100 anomalies are associated with this syndrome. In this case, a cyst removed from a 15-year-old male was diagnosed as an odontogenic keratocyst. The long history of this syndrome with its associated problems is described.

Gorlin's syndrome is a complex hamartomatous/neoplastic syndrome with multisystemic manifestations involving the skin, central nervous system, and bony skeleton. The purpose of this article is to describe how a patient with Gorlin's syndrome was managed with a multipronged approach that included exposure osteotomies for keratocyst removal combined with orthognathic surgery. This case demonstrates the advantages of standard maxillofacial osteotomies to gain access for the removal of odontogenic cysts and benign tumors.

The unerupted maxillary canine is the tooth that most commonly requires surgical exposure and guided eruption. The orthodontic specialist must be aware that delayed eruption is caused, sometimes, by serious pathologic conditions such as cystic or neoplastic malformation. A case of a 12-year-old girl who suffered from Gorlin's syndrome (nevoid basal cell carcinoma syndrome) is presented. Her symptoms were multiple jaw keratocysts, skeletal anomalies, and cleft lip and palate. Some authors advocate an aggressive surgical approach to jaw keratocysts because of their tendency to recur, thus sacrificing those teeth associated with them. Our view is that in young patients with Gorlin's syndrome, an attempt should be made to preserve permanent teeth, especially those vital for a normal growth pattern of the jaws. Therefore we recommend a conservative surgical approach, enabling guided eruption of unerupted teeth associated with cystic lesions in patients with Gorlin's syndrome.

A young girl (16 years old) came to our attention because she needed dental surgery for multiple cysts; she was affected by Gorlin-Goltz syndrome and by a partial deficit of clotting factor V. This syndrome is characterized by many alterations, some of which can interfere with a normal conduct of anesthesia, especially at intubation (prognathism, kyphoscoliosis, incomplete segmentation of cervical-thoracic vertebrae). Moreover this patient was heterozygote for factor V (it was reduced to 48%), so that she had some clotting alteration. We present therefore this case and the treatment undertaken.

Nevoid basal cell carcinoma syndrome is a relatively uncommon clinical entity. It is essentially characterized by the early onset of multiple basal cell carcinomas of the skin generally above wrist-line level, by the presence of jaw cysts, musculoskeletal and other anomalies. Metastatic disease secondary to ordinary basal cell carcinoma is indeed rare. What is even more rare is pulmonary metastases from basal cell carcinoma. Very few cases have been reported in which pulmonary resection was performed for metastatic basal cell carcinoma. We were unable to find a reported case in which pulmonary resection was performed for a pulmonary nodule metastatic from the nevoid basal cell carcinoma syndrome. Herein is reported what we believe to be the first case of pulmonary resection performed for a metastatic nodule in this syndrome.

A survey is given of patients with odontogenic keratocysts in childhood and adolescence which were treated at the Clinic for Maxillofacial Surgery Tübingen in the last 10 years. Odontogenic keratocysts occurring in the growth period are treated in most cases by enucleation or partial marsupialization. To avoid damaging the nerve or dental germs, fixation with Carnoy's solution is not performed. In a second part an experimental study dealing with the effects of Carnoy's solution on peripheral nerves is presented. After incubating the sciatic nerve of rats with Carnoy's solution lumbar SEP, M-response and Hoffmann's reflex were recorded. It was possible to demonstrate the detrimental effect of Carnoy's solution on peripheral nerves. After incubation periods of more than 2 minutes almost complete functional loss was observed. There was a strong correlation between electrophysiological and histological findings.

A pregnant woman with Gorlin's syndrome presented for the removal of malignant ovarian tumours. The main problem encountered by the anaesthetists was an unexpected increase in arterial blood pressure. This was most probably associated with surgical manipulation of the ovaries with an increase in prorenin/renin production.

A 36-year-old Latin American man with the basal cell nevus syndrome had multiple, soft, hairless, depressed scars of the face, scalp, neck, and back, ranging in size from 0.3 x 0.5 cm to 3.5 x 3.9 cm. These areas had never been treated and previously were sites of basal cell carcinomas that underwent complete spontaneous regression 4-5 years from the date of onset. Pathology of these spontaneously regressed sites was consistent with the criteria established by Curson and Weedon (1979). Type III transepithelial elimination was demonstrated for a basal cell carcinoma. Type III transepithelial elimination may play a role in the spontaneous regression of basal cell carcinomas.

Two patients developed new primary basal cell carcinomas (BCCs) in skin flaps used to reconstruct wounds that followed an earlier primary BCC and a recurrent BCC treated by Mohs micrographic surgery. Criteria for distinguishing a new primary BCC arising in a skin flap or full-thickness skin graft at a previous treatment site for BCC from a truly recurrent BCC are presented. The distinction between a new primary BCC and true tumor recurrence is important for accurate clinical assessment and may have a dramatic impact on the type of subsequent treatment. In addition, there may be less medicolegal liability in the case of a new primary BCC arising at the site of a previously treated BCC than for a BCC that is determined to be recurrent.

The main features of the nevoid basal cell syndrome, also called Gorlin-Goltz-syndrome, are basal cell nevi, jaw cysts, anomalies of the vertebras and ribs, and intracerebral calcifications. The diagnosis is often incidentally made by discovery of bilateral jaw cysts, as it was in the case of our patient and her family. The therapy is the complete enucleation of the cyst. The cavity is filled with homologous cancellous bone graft. Recurrence and little cysts are treated with the solution of Carnoy eliminating remaining microcysts.

The nevoid basal cell carcinoma syndrome is a rare and complex disease with multiple manifestations. As an example, we present the case of an eight-year-old girl with this syndrome whose initial complaint was acute abdominal pain secondary to torsion of an ovarian fibroma. Because of its multisystem effects, patients with this syndrome can present to any of a number of medical or surgical specialists. Close attention to the family and past medical history and physical examination will alert the clinician to its presence, allowing for appropriate genetic counseling and serial screening for the development of malignancies and other complications.