Familial atypical multiple mole melanoma (FAMMM) syndrome is a rare autosomal dominant disorder, in which patients present with a large number of melanocytic naevi and a strong history of malignant melanoma, usually at a young age. The most common genetic alteration, implicated in 40 per cent of FAMMM syndrome families, is a mutation of cyclin-dependent kinase inhibitor 2A (CDKN2A).1 CDKN2A encodes the tumour suppressor gene p16INK4a, a critical cell cycle inhibitor.2 The diagnosis and management of patients with FAMMM syndrome is relevant to the plastic surgeon who manages melanoma. However, clear guidelines on its diagnostic criteria and its relationship to associated but distinct syndromes, such as hereditary melanoma and B-K mole syndrome, are lacking in the extant literature. The aim of this review is to clarify the diagnostic criteria and management principles for FAMMM syndrome. We propose a new system of classifying FAMMM syndrome patients as a subset of all patients with hereditary melanoma. We also present a management algorithm for these distinct patient groups (FAMMM syndrome, hereditary melanoma and germline CDKN2A mutations).

The desire to rapidly integrate 3D printing technology by the surgical community, using both commercially available 3D printed custom devices and the emerging trend toward benchtop solutions. Sterilization guidelines for commercially available medical devices, is defined by manufacturers following industrial testing of approved sterilization protocols, whereas benchtop or bedside manufacture of devices by surgical units wishing to adopt and use 3D printed materials represents a grey area where regulation, standards or guidelines do not currently exist. To address this question, we have reviewed the literature. A search of Pubmed for search terms relating to sterilization and 3D printing was subjected to abstract and bibliographic review to identify relevant articles discussing sterilization of 3D printed devices or materials. Only a handful of authors in the peer reviewed literature have addressed the issue of sterilization of 3D printed materials. Commercial “white papers” provide the remainder of available information on the subject. In the quest to adapt and evolve our surgical practice toward the use of “benchtop” 3D-printing, the issue of sterilization was readily identified. The anecdotal experience reported here, with failed attempts using heat dependent sterilization together with evaluation of the literature and review of the available options has directed us toward low-temperature sterilization techniques as standard for sterilization of “in house” 3D printed Models. VHP as a widely institutionally available and employed technology would seem the logical choice as it is readily available, commonly used technique in hospital sterilization departments and quick , simple process compatible with a variety of materials.

BackgroundExisting prognostic systems for metastatic cutaneous squamous cell carcinoma of the head and neck (cSCCHN) do not discriminate between the number of involved nodes beyond single versus multiple. This study aimed to determine if the number of metastatic lymph nodes is an independent prognostic factor in metastatic cSCCHN and whether it provides additional prognostic information to the American Joint Committee on Cancer (AJCC) staging.MethodsWe retrospectively analysed 101 patients undergoing curative intent treatment for metastatic cSCCHN to parotid and/or neck nodes by surgery +/− radiotherapy at Liverpool Hospital, Sydney, Australia. The impact of number of nodal metastases on disease‐free survival (DFS) and risk of distant metastases was assessed using multivariate Cox regression.ResultsThe mean number of nodal metastases was 2.5 (range 1–12). On multivariate analysis, increasing number of nodal metastases significantly predicted reduced DFS (hazard ratio 1.17; 95% confidence interval 1.05–1.30; P = 0.004), with a 17% increased risk of recurrence or death for each additional node. This remained significant in multivariate models adjusted for AJCC 8th edition nodal and TNM stages. Number of nodal metastases was also associated with risk of distant metastatic failure (hazard ratio 1.21; 95% confidence interval 1.05–1.39; P = 0.009).ConclusionIncreasing number of nodal metastases is associated with decreased DFS and increased risk of distant metastases in metastatic cSCCHN, with a cumulative risk increase with each additional node. It provides additional prognostic information to the AJCC staging, which may be improved by incorporating information on the number of nodal metastases beyond the current single versus multiple distinction.

OBJECTIVESto describe the clinicopathologic features of oral squamous cell carcinoma in patients who develop locoregional recurrence of disease, to identify factors that predict prognosis in the subset of patients treated with salvage surgery, and to determine the adjusted effect of time to recurrence.DESIGNcohort study.SETTINGa head and neck cancer institute in Sydney, New South Wales, Australia.PATIENTSa total of 77 patients who underwent salvage surgery for oral squamous cell carcinoma that had been treated initially by surgery, radiotherapy, or surgery with postoperative radiotherapy.MAIN OUTCOME MEASURESunivariable and multivariable analysis of clinical and pathologic risk factors.RESULTSmedian time to recurrence from initial treatment was 7.5 months (range, 0.9-143.9 mo), with 86% of recurrences occurring within the first 24 months. Surgical salvage was attempted in 77 patients who had experienced recurrence at the primary site (n = 39), ipsilateral neck (n = 27), and contralateral neck (n = 11). Time to recurrence, initial treatment modality, and site of failure were independent prognostic variables.CONCLUSIONSthe relationship of these prognostic variables displays a dynamic interaction. Initial combined-modality treatment and shorter time to recurrence were associated with worse outcome, while the effect of site of recurrence (local vs regional) was dependent on an interaction with the time to recurrence. The result of this interaction was that local recurrence was worse for those who experienced it early (eg, <6 mo after the initial treatment) and nodal recurrence was worse for those who experienced it late (eg, ≥ 6 mo after the intial treatment).

INTRODUCTION Locally advanced oral cavity malignancies involving the tongue or floor of mouth often require detachment from the mandible and en bloc resection of the floor of mouth musculature. In subtotal glossectomy and floor of mouth defects, the reconstructive surgeon must balance the volume of soft-tissue replacement against residual tongue mobility to optimize restoration of speech and swallowing and ensure reliable separation of the oral cavity from the neck to prevent postoperative salivary fistulas. The mylohyoid, digastric, and geniohyoid muscles provide dynamic structural support for the tongue to combat the combined forces of gravity, mastication, articulation, and swallowing. When the floor of mouth ‘‘diaphragm’’ is resected or detached, the reconstruction may leave patients with a poorly positioned neotongue that provides inadequate flap-palatal valving for speech and swallowing. Furthermore, without adequate support, prolapse of the oral floor can lead to pooling of food and saliva. Traditional methods of subtotal and total glossectomy reconstruction using muscle flaps such as the rectus abdominus muscle (VRAM) are far from ideal because the surgeon needs to guess the appropriate volume of tissue to compensate for muscle atrophy. This provides a situation where the initial volume is overcompensated, interfering with oral function; the laryngeal apparatus cannot be resuspended, promoting aspiration; and the final volume is frequently insufficient. Since its introduction by Song et al. in 1984, the anterolateral thigh (ALT) flap has gained widespread use in reconstruction of soft-tissue defects in the head and neck after ablative surgery. It is considered a highly versatile and reliable flap that allows a two-team approach and is associated with minimal donor site morbidity. The fasciocutaneous ALT flap is particularly suitable for oral cavity reconstruction because it provides a large surface area and volume of soft tissue that is pliable. Furthermore, the volume is more predictable when compared with muscle flaps, which tend to atrophy significantly over time. Here, we describe a technique using the fascia lata component of the fasciocutaneous ALT flap to re-create the mylohyoid sling and provide structural support designed to improve postoperative speech and swallowing function.