Gardner syndrome is an autosomal dominant disease. It is characterized by a combination of familial adenomatous polyposis (FAP) of the intestine with extraintestinal changes as multiple osteomas and fibromas. Odontogenic Myxoma is a benign, aggressive intraosseous neoplasm. We report a rare case of a 14-year-old male patient with Gardner\'s syndrome and odontogenic myxoma, which involved the entire left half of the mandible, resulting in a gross facial deformity, within a span of one year.

OBJECTIVE: To determine the tolerability, effectiveness and outcomes of percutaneous image-guided cryoablation on inoperable extra-abdominal desmoid tumors.
METHODS: Between 2011 and 2012, 13 patients (mean age 39.3 years, range 15-74) with inoperable extra-abdominal desmoid tumors were consecutively treated with cryoablation (17 tumors treated in 17 procedures), including two patients with Gardner syndrome and nine recurrences after surgery. Disease-free survival (DFS) and local control based on RECIST criteria were calculated on prospective clinical and imaging follow-up until 2013.
RESULTS: Cryoablation was performed under ultrasound (n = 8), computed tomography (n = 1), or combined (n = 8) guidance, and under general (n = 15) or local (n = 2) anesthesia. A major complication was observed in one session (5.8%). Mean follow-up was 11.3 months (6-27 months). Among all the patients treated, eight patients (47%) presented residual tumors on the first magnetic resonance follow-up, which were asymptomatic. The DFS rate was stable at 82.3% (95% confidence interval 0.55-0.94) at 6, 12, and 24 months. The local tumor progression rate was 0% at 6, 12, and 24 months. Two patients (12%) presented an in situ recurrence.
CONCLUSIONS: Despite high rates of partial ablation, percutaneous image-guided cryoablation appears to be safe and effective for local control for patients with inoperable extra-abdominal desmoid tumors.

OBJECTIVE: Gardner syndrome (GS) is an autosomal dominant genetic disorder with almost complete penetrance (80%) and variable expression. GS is a variant of familial adenomatous polyposis and characterized by extracolonic manifestations including osteomas and soft tissue tumors (desmoid tumors, epidermoid cysts). We describe clinical and surgical approaches in a family in which the genetic disorder was diagnosed in 3 generations.
METHODS: The studied family underwent clinical history and instrumental and genomic studies. Two members of this family, affected with GS, underwent surgery for skeletal osteomas.
RESULTS: The patients that we treated with clinical-instrumental monitoring for a period of 5 years had no major disturbances of the stomatognathic system and no clinical signs of pathology of the gastrointestinal tract, eyes, or endocrine systems.
CONCLUSIONS: The orofacial complex disorders are exclusively functional and esthetic, concerning primarily the stomatognathic system. We had no cases of malignant transformation of osteomatosis lesions. Clinical sequelae are manly facial eumorphy and occlusion problems of the temporomandibular joint.

OBJECTIVE: Screening of Gardner syndrome (GS) patients is tailored towards prevention of colorectal cancer (CRC). However, many patients suffer from desmoid tumors, which are challenging to treat due to invasive growth and local recurrence. The aims of our study were to determine the effectiveness of screening in GS and analyze outcome of desmoid tumors by treatment modality.
METHODS: This was a cohort study of a family of 105 descendants with GS. All family members who agreed were screened by endoscopy, and colorectal resection was performed upon pending malignancy. Resectable desmoids were excised, whereas large tumors were treated by a combination of brachytherapy (BT) and radiotherapy (RT). Main outcome measures were the incidence of CRC and overall and disease-specific mortality (ClinicalTrial.gov ID NCT01286662).
RESULTS: Thirty-seven of 105 family members have GS. Preventive colorectal resections were performed in 16 patients (15 %), with one death due to gastric cancer. In four patients who denied screening endoscopy, invasive tumors of the colon (three patients) and stomach developed. Of 33 desmoid tumors, 10 (30 %) were located in the mesentery, 17 (52 %) in the abdominal wall, and 6 (18 %) in extra-abdominal sites. Excision of 12 desmoids was performed in eight patients. Four desmoids were treated by BT and RT and showed full or partial remission.
CONCLUSIONS: Provided adequate screening, good long-term control of colorectal tumors is achievable. However, desmoid tumors determine survival and quality of life in many patients. Our data suggest good local control using a combination of brachytherapy/radiotherapy in large desmoids unsuitable for surgical resection.

OBJECTIVE: The purpose of the article was to present 14 new cases of peripheral osteoma and to evaluate the diagnosis and management of peripheral osteoma of the maxillofacial region with an analysis of the literature.
METHODS: The records of 14 consecutive patients (6 males and 8 females) referred for the management of peripheral osteoma the maxillofacial region were reviewed. Demographic data, location, presenting symptoms, radiographic findings, bone scan, colonoscopy results and surgical management were analyzed. The criteria used to diagnose peripheral osteoma included radiographic and histologic features.
RESULTS: The 14 patients, ranged in age from 13 to 79 years with a mean age of 40.5 years. The lesions were located in the mandible (64%), maxilla (7%), temporal bone (22%) and maxillary sinus (7%). Colonoscopy results performed in 10 subjects were negative. Surgical excision was the treatment of choice with good results. There were no complications or recurrences.
CONCLUSIONS: Peripheral osteoma of the jaw bones is uncommon. The post surgical follow-up should include periodic clinical and radiographic studies. Patients with osteoma associated with impacted or supernumerary teeth, should be evaluated for the possible Gardner\'s syndrome.

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OBJECTIVE: The purpose of this article was to present 35 new cases of peripheral osteoma of the oral and maxillofacial region with an analysis of the literature.
METHODS: We performed a search of our files for the past 5 years for peripheral osteoma of the oral and maxillofacial region. The criteria used to diagnose osteoma included the radiographic and histologic features.
RESULTS: The 35 patients, which included 23 males (65%) and 12 females (35%), ranged in age from 14 to 58 years, with a mean age of 29.4 years. Most of the osteomas were located in frontal bone (28.57%), mandible (22.85%), and maxilla (14.28%).
CONCLUSIONS: Peripheral osteomas of the jaw bones are uncommon, and accordingly, patients with osteoma should be evaluated for Gardner\'s syndrome. In addition, it is appropriate to provide both clinical and radiographic follow-up after surgical excision of a peripheral osteoma.

BACKGROUND: Nuchal-type fibroma (NTF) usually arises in the posterior aspect of the neck. Previously published reports describe only 11 cases and provide limited clinicopathologic information.
METHODS: Fifty-two examples of NTF from 50 patients were analyzed from the files of the Soft Tissue Registries of the Armed Forces Institute of Pathology in Washington, DC, and the Faculty Hospital in Pilsen, Czech Republic.
RESULTS: The age of the patients ranged from 3 to 74 years (mean, 40 years). There were 41 males and 9 females. Thirty-six NTFs were located in the posterior neck region and 16 were from extranuchal sites. Two patients had had lesions excised from both a nuchal and an extranuchal location. The mean greatest tumor dimension was 3.2 cm. Microscopically, all examples had a superficial (subcutaneous and sometimes dermal) component and consisted of paucicellular, thick bundles of lobulated collagen fibers with inconspicuous fibroblasts. Entrapped adipose tissue and traumatic neuromalike nerve proliferations were typically present. Skeletal muscle infiltration was also seen in a minority of cases. Eleven of 25 patients (44%) for whom clinical information was available reportedly had diabetes. Gardner\'s syndrome was documented in one patient and was possibly present in two additional individuals. During follow-up, five patients had local recurrences, but none of the recurrences were destructive and all were ultimately controlled by local reexcision.
CONCLUSIONS: NTF is a rare, tumorlike accumulation of collagen that often affects the posterior neck region but can also occur in a number of other sites. The process has a strong association with diabetes and also appears to be linked to Gardner\'s syndrome. Local recurrence probably reflects the persistence of local or systemic factors related to its pathogenesis.

Assays of in vitro biological properties associated with cellular transformation revealed differences in cultured skin cells within the heritable colon cancer syndromes with and without polyposis coli. Such evidence suggested that an in vitro panel of biological assays could be used to recognize genotypes which increase the risk of colon cancer prior to clinical expression and supported the concept, long assumed on clinical differences, of genetic heterogeneity within the heritable colon cancer syndromes.