Merkel cell carcinoma: Clinical and anatomopathological features, management and survival in our centre.

OBJECTIVE: To summarize the features of the Merkel cell carcinoma (MCC) and to sistematyze its diagnosis and therapeutic management. METHOD: We performed a literature review in PubMed, obtaining a total of 3,308 articles, selecting 10 for its complete reading and 22 for the reading of the summary according to the content. RESULTS: In none of our patients, the MCC was the first suspected diagnosis. The treatment consisted in surgical excision with tumor free margins and lymphadenectomy. We offered ad-juvant RT which they rejected. They remain disease-free at the present time. CONCLUSIONS: MCC is a rare and aggressive disease which presents as a fast-growing solitary asymptomatic erythematous nodule in those areas of skin which are exposed to sunlight in elderly patients. The main risk factors include radiative ultraviolet, immunosuppression and merkel cell polyomavirus. Surgery is the main loco-regional treatment. Lymph node metastases in the course of the disease is one of the main prognostic factors. If there are no adenopaties, sentinel lymph node biopsy must be done; if there are adenopaties or a positive biopsy, lymphadenectomy is indicated. Radiotherapy is indicated in all stages of disease since it has shown to improve loco-regional control. In distant metastatic disease, immunotherapy and participating in clinical trials are the first choice.

OBJETIVO: Resumir las características del carcinoma de células de Merkel (CCM) y sistematizar su manejo diagnóstico-terapéutico. MÉTODO: Realizamos una búsqueda bibliográfica en PubMed y aparecieron 3,308 artículos, de los que seleccionamos 10 para lectura completa y 22 para lectura del resumen acorde con su contenido. RESULTADOS: En ninguno de nuestros pacientes el CCM fue la primera sospecha diagnóstica. El tratamiento consistió en la extirpación quirúrgica con márgenes libres y linfadenectomía. Se les ofreció radioterapia adyuvante, que rechazaron. Se encuentran libres de enfermedad tras 1 año del tratamiento. CONCLUSIONES: El CCM es una condición rara y agresiva que se presenta como un nódulo eritematoso de rápido crecimiento y asintomático en zonas fotoexpuestas de pacientes añosos. Los principales factores de riesgo son la exposición ultravioleta, la inmunosupresión y el poliomavirus asociado al carcinoma de Merkel (MCPyV, Merkel cell polyomavirus). La cirugía es el pilar fundamental del tratamiento locorregional. La afectación ganglionar en el transcurso de la enfermedad es uno de los principales factores pronósticos. Si no existen adenopatías reconocibles, debe realizarse biopsia selectiva de ganglio centinela; si existen adenopatías o la biopsia es positiva, está indicada la linfadenectomía. La radioterapia adyuvante está indicada en todos los estadios y ha demostrado un mejor control locorregional. En la enfermedad a distancia es de primera elección la inmunoterapia y participar en ensayos clínicos.

The Management and Prognosis of Facial and Scalp Angiosarcoma: A Retrospective Analysis of 15 Patients.

BACKGROUND: Angiosarcomas are extremely aggressive malignant tumors that arise from vascular endothelial cells. The risk factors, etiology, prognostic factors, and optimal management strategies for angiosarcomas are as yet unknown. METHODS: We retrospectively analyzed data from 15 patients who were treated in Asan Medical Center, Seoul, Republic of Korea, in the past 12 years, to assess the effect of different treatment modalities and reconstructive methods on the locoregional recurrence, metastasis, and overall survival. RESULTS: A total of 15 patients were identified (median age at diagnosis, 72 years; range, 61-82 years). Median tumor size was 6 cm. Median follow-up was 287 days. The median overall survival was 14.96 months; a total of 13 (87%) patients had died by the end of the study.The median locoregional recurrence, metastasis, and overall survival were 7.3, 6.5, and 16.7 months, respectively. On univariate analysis, the use of adjuvant therapy after surgery (vs surgery without adjuvant therapy) was associated with delayed median time to detection of recurrence (7.9 months vs 3.1 months, respectively; P = 0.825), delayed median time to metastasis (8.7 months vs 3.1 months, respectively; P = 0.191), and better median overall survival (7.3 months vs 3.1 months, respectively; P = 0.078).The use of flap versus skin graft as a reconstructive method was associated with delayed median recurrence (8.75 vs 7.32 months, respectively; P = 0.274) and earlier median metastasis (3.75 vs 6.53 months, respectively; P = 0.365), but the same median overall survival of 16.7 months (P value: 0.945) and tumor smaller or bigger than 5 cm show earlier median time to detection of recurrence (4.17-7.32 months; P = 0.41), earlier median time to metastasis (3.75-6.53 months; P = 0.651), but better median overall survival of 18.21 versus 16.7 months, respectively (P = 0.111). CONCLUSIONS: Multimodal treatment that combines surgery with adjuvant therapy is the best management strategy that influences survival positively in patients with angiosarcoma. The study shows that the reconstructive method does not affect the prognosis in these patients. So it is better to choose the simplest suitable resection and reconstructive method with the least complications and to avoid unnecessary procedures.

Cheek Cutaneous Melanomas: A Review of 98 Cases.

BACKGROUND: Most head and neck melanomas occur on the face, with the cheek being the most frequently affected site. So far, small numbers of researches on cheek melanomas have yielded insufficient and controversial results. In this trial, we aimed to define the histopathological and clinical features specific to cheek melanomas and to compare them with other head and neck melanomas by using a large group of patients from a single tertiary center. PATIENTS AND METHODS: A total of 98 cheek melanomas and 183 other (noncheek) head and neck melanoma cases were analyzed retrospectively. RESULTS: The median age was 62 years and malar/zygomatic was the mostly affected site (78.6%). The cheek melanomas developed more frequently in females (61.2%) and most of them were associated with lentigo maligna histopathological subtype (49.2%) and early-stage disease (stage 0-II) (77.6%). The cheek melanomas were found more significantly associated with older patients (P = 0.05), females (P = 0.0001), lentigo maligna subtype (P = 0.0001), lower vertical growth phase (P = 0.03), and early-stage of disease (P = 0.0001) compared with other lesions that developed other sites. Furthermore, they were associated with lower relapse rates (18.6% vs 39.5%, P = 0.0001) and the 5-year recurrence-free survival rate of cheek melanomas was 80% (P = 0.002). Moreover, the 5-year overall survival rate of cheek melanomas was 62%, and they were found to be associated with a favorable overall survival (P = 0.004). CONCLUSIONS: Cheek melanomas are associated with lower relapse rates and favorable outcomes compared with other (noncheek) head and neck melanomas.

Pediatric Ewing sarcoma of the head and neck: A retrospective survival analysis.

OBJECTIVE: To determine the demographics, treatment modalities, and overall survival of pediatric Ewing sarcoma of the head and neck. METHODS: The National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify cases of pediatric Ewing sarcoma of the head and neck between 1973 and 2015. Additional variables collected included age, gender, ethnicity, tumor grade, staging, treatment modality, and follow-up time. Kaplan-Meier survival curves were generated and overall survival was calculated. RESULTS: One hundred and twenty-seven cases of pediatric Ewing sarcoma were identified. The majority of patients were male (52.8%), white (88.2%), and non-Hispanic (75.6%), and the mean age at diagnosis was 10.5 years. The most common tumor sites were bones and joints of the skull and face (45.5%), followed by soft tissue of the head, face, and neck (25.2%), followed by the mandible (13.4%). Most patients (69.2%) had some type of surgery, 70.0% received radiation, and 92.1% received chemotherapy as part of their treatment. Overall 1-, 5-, and 10-year survival was 91.1%, 72.9%, and 68.2%, respectively. There was no significant difference in survival based on patient gender, age, tumor location, radiation, or chemotherapy treatment. CONCLUSION: This study demonstrates that pediatric patients with Ewing sarcoma of the head and neck most commonly have stage II disease, no distant metastasis, and are treated with chemotherapy, with or without radiation. One, five, and ten-year survival are 91.1%, 72.9%, and 68.2%, respectively, and age, gender, and radiation do not seem to affect prognosis.

Surgery with curative intent is associated with prolonged survival in patients with cutaneous angiosarcoma of the scalp and face -a retrospective study of 38 untreated cases in the Japanese population.

BACKGROUND: In patients with cutaneous angiosarcoma of the scalp and face, the validity of surgery remains controversial, because of the potentially diffuse nature of involvement and difficulty in obtaining negative margins. OBJECTIVE: To evaluate the survival benefit of surgery as a primary treatment. PATIENTS AND METHODS: Fifty-one patients with primary cutaneous angiosarcoma of the scalp and face presenting with locoregional involvement were referred to National Cancer Center Hospital, Tokyo, Japan, between May 1982 and March 2013. Data of those patients in whom the diagnosis had been confirmed histologically and the primary treatments had been initiated at our center were analysed retrospectively. Only untreated cases were selected with aim to evaluate actual survival benefit of surgery as a primary treatment. RESULTS: Of the 51 patients, 38 were found to be eligible for inclusion in this analysis; of these 38 patients, 29 (29/38 = 76.3%) patients had tumour diameter > 5 cm, and 29 underwent surgery with curative intent (curative-intent surgery) as the initial treatment. Histologically positive margins were found in 10 patients. Multivariate analysis identified curative-intent surgery as being significantly associated with improved overall survival (OS; HR = 0.26; 95% CI, 0.10-0.63). In the Surgery group, neither negative margins nor combined-modality treatment had any significant influence on the OS. CONCLUSION: Removal of primary tumour with curative-intent surgery may be a valid treatment option even for patients with primary cutaneous angiosarcoma of the scalp and face larger than 5 cm in size, regardless of the histological surgical margin status.

Melanoma of the Face and Mohs Micrographic Surgery: Nationwide Mortality Data Analysis.

BACKGROUND: Although Mohs micrographic surgery (MMS), narrow margin excision (NME), and wide margin excision (WME) are commonly used to treat melanoma of the face, there is a paucity of data comparing mortality outcomes for each method. OBJECTIVE: To determine the association between surgical method used to treat cutaneous melanoma of the face and patient survival. MATERIALS AND METHODS: A retrospective review of Surveillance, Epidemiology, and End Results registries for patients diagnosed with melanoma of the face between 2003 and 2012 was conducted. RESULTS: The authors query resulted in 43,443 records. Patients with melanoma were more likely to undergo NME (57.79%) than WME (27.86%) or MMS (14.36%). Overall 5-year risk of death was higher with WME (hazard ratio [HR], 1.11; 95% confidence interval [CI], 1.00-1.23; p = .043) and NME (HR, 1.10; 95% CI, 1.00-1.20; p = .046) relative to MMS after adjusting for patient demographics, residence socioeconomic factors, and tumor characteristics. No statistically significant difference in melanoma-specific mortality was found between different surgical methods on multivariate analysis. CONCLUSION: Patients with melanoma of the face treated with MMS had similar melanoma-specific mortality or overall survival outcome as patients treated by other surgical modalities.

Treatment of cutaneous angiosarcoma of the scalp and face in Chinese patients: local experience at a regional hospital in Hong Kong.

INTRODUCTION: Angiosarcoma is a rare aggressive sarcoma that occurs mostly in the skin of the head and neck in the elderly population. The optimal management is dubious and most studies are from Caucasian populations. We aimed to examine the treatment and outcome of this disease in Chinese patients. METHODS: Data of patients with histopathologically verified cutaneous angiosarcoma of the head and neck during December 1997 to September 2016 were retrieved from our hospital cancer registry. The demographic data, clinicopathological information, modality of treatment, and outcomes were reviewed. RESULTS: During the study period, 17 Chinese patients were treated. Their median age was 81 years. The tumours were present in the scalp only (n=11), face only (n=4), or both scalp and face (n=2). Only two patients had distant metastases. The modalities of treatment were surgery (n=6), surgery and adjuvant radiotherapy (n=1), palliative radiotherapy (n=5), or palliative chemotherapy (n=3). The remaining two patients refused any treatment initially. Of the seven patients treated surgically, there were four local and two regional recurrences. The median time to relapse was 7.5 months. Overall, 16 patients had died; causes of death were disease-related in 12 whereas four other patients died of inter-current illnesses. One patient was still living with the disease. The median overall survival was 11.1 months and the longest overall survival was 42 months. CONCLUSION: The outcome of angiosarcoma in our series is poor. A high index of suspicion is mandatory for prompt diagnosis. Adjuvant radiotherapy is recommended following surgery. The benefit and role of systemic treatment in various combinations with surgery or radiotherapy require further study.

Survival outcomes for cutaneous angiosarcoma of the scalp versus face.

BACKGROUND: The primary purpose of this study was to examine whether angiosarcoma outcomes differ for the scalp and face. METHODS: We conducted a retrospective outcomes analysis of 50 patients with cutaneous angiosarcoma treated by curative intent identified from the Princess Margaret Cancer Centre Registry (from 1958 to 2014). RESULTS: Median survival was 26 months (95% confidence interval [CI], 17.6-34.6) and median follow-up 29 months. For the scalp and face, respectively, the 5-year locoregional control rate was 9% and 53% (p = .04); the recurrence-free survival (RFS) rate was 5% and 27% (p = .017); and the overall survival (OS) rate was 9% and 26% (p = .017). Scalp lesions were larger, more likely to be multifocal, and presented more rapidly once noticed. In multivariate Cox proportional hazards analysis, scalp location was independently prognostic for mortality (hazard ratio [HR], 2.10; 95% CI, 1.03-4.28; p = .04). CONCLUSION: Scalp angiosarcoma has worse survival than angiosarcoma of the face. Scalp angiosarcoma tends to be larger at presentation, which may be because it is not noticed until more advanced. © 2017 Wiley Periodicals, Inc. Head Neck 39: 1205-1211, 2017.

Impact of scalp location on survival in head and neck melanoma: A retrospective cohort study.

BACKGROUND: Scalp melanomas have more aggressive clinicopathological features than other melanomas and mortality rates more than twice that of melanoma located elsewhere. OBJECTIVE: We sought to describe the survival of patients with scalp melanoma versus other cutaneous head and neck melanoma (CHNM), and explore a possible independent negative impact of scalp location on CHNM survival. METHODS: A retrospective cohort study was performed of all invasive primary CHNM cases seen at a tertiary referral center over a 20-year period. Melanoma-specific survival (MSS) was compared between scalp melanoma and other invasive CHNM. Multivariable Cox proportional hazards regression was performed to determine associations with survival. RESULTS: On univariate analysis, patients with scalp melanoma had worse MSS than other CHNM (hazard ratio 2.22, 95% confidence interval 1.59-3.11). Scalp location was not associated with MSS in CHNM on multivariable analysis (hazard ratio 1.11, 95% confidence interval 0.77-1.61) for all tumors together, but remained independently associated with MSS for the 0.76- to 1.50-mm thickness stratum (hazard ratio 5.51, 95% confidence interval 1.55-19.59). LIMITATIONS: Disease recurrence was not assessed because of unavailable data. CONCLUSION: The poorer survival of scalp melanoma is largely explained by greater Breslow thickness and a higher proportion of male patients.

Orbitofacial Metastatic Basal Cell Carcinoma: Report of 10 Cases.

PURPOSE: To explore the clinical features, management, and prognosis of metastatic basal cell carcinoma originating in the orbitofacial region. METHODS: Ten cases of orbitofacial metastatic basal cell carcinoma were identified by searching databases at 2 institutions from 1995 to 2015. A retrospective chart review was performed. Main outcome measures included patient demographics, lesion size, location of metastases, histologic subtype, recurrence rate, time between primary tumor diagnosis and metastasis, perineural invasion, treatment modalities, and survival from time of metastasis. RESULTS: The median tumor size at largest dimension was 3.3 cm (range, 1.9-11.5 cm), and 6 of 10 patients had at least 1 local recurrence before metastasis (range, 0-2 recurrences). The most common sites of metastasis included the ipsilateral parotid gland (n = 6) and cervical lymph nodes (n = 5). Histologic subtypes included infiltrative (n = 5), basosquamous (n = 2), nodular (n = 1), and mixed (n = 1). The median time from primary tumor diagnosis to metastasis was 7.5 years (range, 0-13). The median survival time from diagnosis of metastasis to last documented encounter or death was 5.3 years (range, 7 months-22.8 years). Treatment regimens included surgical excision, radiotherapy, and hedgehog inhibitors. CONCLUSIONS: Based on our findings, the following features may be markers of high risk orbitofacial basal cell carcinoma: 1) increasing tumor size, 2) local recurrence of the primary tumor, 3) aggressive histologic subtype, and 4) perineural invasion. Screening should include close observation of the primary site and tissues in the distribution of regional lymphatics, particularly the parotid gland and cervical lymph nodes.

Immunology of a Transmissible Cancer Spreading among Tasmanian Devils.

Devil facial tumor disease (DFTD) is a transmissible cancer that has killed most of the Tasmanian devil (Sarcophilus harrissii) population. Since the first case appeared in the mid-1990s, it has spread relentlessly across the Tasmanian devil's geographic range. As Tasmanian devils only exist in Tasmania, Australia, DFTD has the potential to cause extinction of this species. The origin of DFTD was a Schwann cell from a female devil. The disease is transmitted when devils bite each other around the facial areas, a behavior synonymous with this species. Every devil that is 'infected' with DFTD dies from the cancer. Once the DFTD cells have been transmitted, they appear to develop into a cancer without inducing an immune response. The DFTD cancer cells avoid allogeneic recognition because they do not express MHC class I molecules on the cell surface. A reduced genetic diversity and the production of immunosuppressive cytokines may also contribute.

Angiosarcoma of the scalp and face: the Mayo Clinic experience.

IMPORTANCE: The etiology and optimal treatment are unknown for angiosarcoma, an aggressive malignant tumor that affects vascular endothelial cells and can be mistaken for benign lesions such as hemangioma. OBJECTIVE: To determine the treatment outcomes of patients with angiosarcoma of the face or scalp treated with a combination of surgery, radiation therapy, and/or chemotherapy. DESIGN, SETTING, AND PARTICIPANTS: Retrospective study of 55 patients with angiosarcoma of the face or scalp treated between January 1, 1973, and December 31, 2012, at a tertiary-care academic medical institution. INTERVENTIONS: Surgery, radiation therapy, and/or chemotherapy. MAIN OUTCOMES AND MEASURES: Locoregional control (LRC), recurrence-free survival (RFS), and overall survival (OS). RESULTS: Fifty-five patients had angiosarcoma localized to the face or scalp. Forty of these patients (73%) received a combination of surgery, radiation therapy, and/or chemotherapy. Eight patients (15%) were treated with surgery alone, 1 (2%) with radiation alone, 5 (9%) with chemotherapy alone, and 1 (2%) with observation alone. Median (range) follow-up for surviving patients was 25.2 (4.7-227.1) months. Five-year LRC, RFS, and OS (95% CI) were 18% (7%-32%), 16% (6%-31%), and 38% (21%-54%), respectively. Of 36 patients with failed treatment, 34 had failure in a local and/or regional site. On univariate analysis, the use of multimodality therapy (vs no multimodality therapy) was associated with higher 5-year LRC (95% CI) (20% [3%-37%] vs 11% [0%-29%]; P = .04), higher RFS (19% [2%-36%] vs 10% [0%-27%]; P = .02), and higher OS (46% [26%-66%] vs 16% [0%-43%]; P = .04). Age 70 years or older (vs <70 years) was associated with lower 5-year LRC (95% CI) (5% [0%-14%] vs 48% [23%-74%]; P = .02) and lower RFS (5% [0%-13%] vs 49% [24%-75%]; P = .04). Radiation therapy (vs no radiation therapy) was associated with higher 5-year LRC (95% CI) (20% [3%-36%] vs 12% [0%-32%]; P = .02) and higher RFS (19% [2%-35%] vs 12% [0%-31%]; P = .004). On multivariable analysis, age younger than 70 years (vs ≥70 years) was associated with improved 5-year LRC (95% CI) (48% [23%-74%] vs 5% [0%-14%]; P = .03) and RFS (49% [24%-75%] vs 49% [24%-75%]; P = .04). CONCLUSIONS AND RELEVANCE: Multimodality therapy for angiosarcoma is associated with improved LRC, RFS, and OS. Younger patients with resectable disease undergoing multimodality therapy for angiosarcoma had the best clinical outcomes.

Surgical excision versus Mohs' micrographic surgery for basal cell carcinoma of the face: A randomised clinical trial with 10 year follow-up.

BACKGROUND: Basal cell carcinoma (BCC) is the most common form of cancer among Caucasians and its incidence continues to rise. Surgical excision (SE) is considered standard treatment, though randomised trials with long-term follow-up are rare. We now report the long-term results of a randomised trial comparing surgical excision with Mohs' micrographic surgery (MMS) for facial BCC. METHODS: 408 facial, high risk (diameter at least 1cm, H-zone location or aggressive histological subtype) primary BCCs (pBCCs) and 204 facial recurrent BCCs (rBCCs) were randomly allocated to treatment with either SE or MMS between 5th October 1999 and 27th February 2002. The primary outcome was recurrence of carcinoma. A modified intention to treat analysis was performed. FINDINGS: For primary BCC, the 10-year cumulative probabilities of recurrence were 4.4% after MMS and 12.2% after SE (Log-rank test χ(2) 2.704, p=0.100). For recurrent BCC, cumulative 10-year recurrence probabilities were 3.9% and 13.5% for MMS and SE, respectively (Log-rank χ(2) 5.166, p=0.023). A substantial proportion of recurrences occurred after more than 5years post-treatment: 56% for pBCC and 14% for rBCC. INTERPRETATION: Fewer recurrences occurred after treatment of high risk facial BCC with MMS compared to treatment with SE. The proportion of recurrences occurring more than 5years post-treatment was especially high for pBCC, stressing the need for long-term follow-up in patients with high risk facial pBCC.

The role of radiotherapy and intrathecal CNS prophylaxis in extralymphatic craniofacial aggressive B-cell lymphomas.

To define the role of radiotherapy and intrathecal prophylaxis in extralymphatic craniofacial involvement (ECFI) of aggressive B-cell lymphoma, we analyzed 11 consecutive German High-Grade Non-Hodgkin Lymphoma Study Group trials. ECFI occurred in 290/4155 (7.0%) patients (orbita, 31; paranasal sinuses, 93; main nasal cavity, 38; tongue, 27; remaining oral cavity, 99; salivary glands, 54). In a multivariable analysis adjusted for International Prognostic Index rituximab improved event-free and overall survival both in patients with and without ECFI. Three-year event-free (79% vs 79%; P = .842) and overall survival (86% vs 88%; P = .351) rates were similar in 145 patients receiving and 57 not receiving radiotherapy. Without rituximab, the 2-year cumulative rate of central nervous system (CNS) disease was increased in 205 ECFI patients compared with 2586 non-ECFI patients (4.2% vs 2.8%; P = .038), whereas this was not observed with rituximab (1.6% in 83 ECFI vs 3.4% in 1252 non-ECFI patients; P = .682). In 88 ECFI patients who received intrathecal prophylaxis with methotrexate, the 2-year rate of CNS disease was 4.2% compared with 2.3% in 191 patients who did not (P = .981). In conclusion, rituximab eliminates the increased risk for CNS disease in patients with ECFI. This retrospective analysis does not support intrathecal prophylaxis or radiotherapy to ECFI patients in complete remission/unconfirmed complete remission. These findings should be confirmed in a prospective study.

Incidence and survival for Merkel cell carcinoma in Queensland, Australia, 1993-2010.

IMPORTANCE: Merkel cell carcinoma (MCC) is an uncommon but highly invasive form of skin cancer. The mechanisms that cause MCC are yet to be fully determined. OBJECTIVES: To compare the incidence and survival rates of MCC in Queensland, Australia, known to be a high-risk area, with MCC incidence and survival elsewhere in the world. We also analyzed incidence trends and differences in survival by key demographic and clinical characteristics. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study of population-based administrative data for MCC collected by the Queensland Cancer Registry and supplemented with detailed histopathologic data. Deidentified records were obtained of all Queensland residents diagnosed as having MCC during the period from 1993 to 2010. A subsample of histopathologic records were reviewed by a senior dermatopathologist to determine the potential for misclassification. A total of 879 eligible cases of MCC were included in the study. MAIN OUTCOMES AND MEASURES: Incidence rates were directly age standardized to the 2000 United States Standard Population. Trends were examined using Joinpoint software with results expressed in terms of the annual percentage change. The period method was used to calculate 5-year relative survival, and adjusted hazard ratios were obtained from multivariate Poisson models. RESULTS: There were 340 cases of MCC diagnosed in Queensland between 2006 and 2010, corresponding to an incidence rate of 1.6 per 100,000 population. Men (2.5 per 100,000) had higher incidence than women (0.9 per 100,000), and rates peaked at 20.7 per 100,000 for persons 80 years or older. The overall incidence of MCC increased by an average of 2.6% per year from 1993 onwards. Relative survival was 41% after 5 years, with significantly better survival found for those younger than 70 years at diagnosis (56%-60%), those with tumors on the face or ears (51%), and those with stage I lesions (49%). CONCLUSIONS AND RELEVANCE: Incidence rates for MCC in Queensland are at least double those of any that have been previously published elsewhere in the world. It is likely that Queensland's combination of a predominantly white population, outdoor lifestyle, and exposure to sunlight has played a role in this unwanted result. Interventions are required to increase awareness of MCC among clinicians and the public.

Does buccal cancer have worse prognosis than other oral cavity cancers?

OBJECTIVES/HYPOTHESIS: To determine whether buccal squamous cell carcinoma has worse overall survival (OS) and disease-specific survival (DSS) than cancers in the rest of the oral cavity. STUDY DESIGN: Retrospective analysis of a large population database. METHODS: We began with a Kaplan-Meier analysis of OS and DSS for buccal versus nonbuccal tumors with unmatched data, followed by an analysis of cases matched for race, age at diagnosis, stage at diagnosis, and treatment modality. This was supported by a univariate Cox regression comparing buccal cancer to nonbuccal cancer, followed by a multivariate Cox regression that included all significant variables studied. RESULTS: With unmatched data, buccal cancer had significantly lesser OS and DSS values than cancers in the rest of the oral cavity (P < .001). After case matching, the differences between OS and DSS for buccal cancer versus nonbuccal oral cancer were no longer significant. Univariate Cox regression models with respect to OS and DSS showed a significant difference between buccal cancer and nonbuccal cancer. However, with multivariate analysis, buccal hazard ratios for OS and DSS were not significant. CONCLUSIONS: With the largest series of buccal carcinoma to date, our study concludes that the OS and DSS of buccal cancer are similar to those of cancers in other oral cavity sites once age at diagnosis, tumor stage, treatment, and race are taken into consideration. The previously perceived poor prognosis of buccal carcinoma may be due to variations in tumor presentation, such as later stage and older patient age. LEVEL OF EVIDENCE: 2b.

Brachytherapy in children with rhabdomyosarcomas of the nasolabial fold.

BACKGROUND: Rhabdomyosarcomas (RMS) of the nasolabial fold can be difficult to manage surgically due to functional and cosmetic limitations. Therefore, brachytherapy (BT) has been proposed to improve local control while limiting the volume of irradiation as well as the extent of the surgical excision. MATERIALS AND METHODS: Sixteen pediatric cases with RMS of the nasolabial fold treated from 1971 to 2005 were retrospectively reviewed. RESULTS: Median follow-up was 4.4 years (1.7-33). Half of the patients were male and their age at diagnosis ranged from 4 months to 13.5 years. Histological subtypes included 10 embryonal and 6 alveolar RMS. Initial treatment consisted of induction multi-agent chemotherapy in all cases. In 12 patients, BT was combined with local excision (4 complete resections, 1 with macroscopic residual disease, and 7 with microscopic disease). Low dose-rate brachytherapy was performed in all cases according to the Paris system, using plastic catheters implanted per-operatively. The doses delivered ranged from 50 to 70 Gy, depending on chemotherapy response, and surgical margin status. 10 patients relapsed: 4 local, 6 regional, and 2 metastatic failures were reported. The median time to relapse was 6.5 months. At the time of analysis eight patients were alive and four had died. Four cases, under palliative care at last check-up, were lost to follow-up. CONCLUSION: BT provided an acceptable local control rate, but the poor regional control of these cases may suggest a need for more aggressive management of cervical regional lymph node regions in RMS of the nasolabial fold.

Comparison of outcomes for malignant melanoma of the face treated using Mohs micrographic surgery and wide local excision.

BACKGROUND: Mohs micrographic surgery (MMS) is an accepted treatment for nonmelanoma skin cancer and has an evolving role in melanoma. OBJECTIVE: To review oncologic outcomes of MMS and wide local excision (WLE) treatments for facial melanoma. METHODS AND MATERIALS: A retrospective review of patients with invasive melanoma of the face between 1997 and 2007 identified from the Alberta Cancer Registry (Canada) was performed. Outcome measures were local recurrence (recurrence <2 cm from excision scar), distant recurrence (regional or systemic), and disease-specific survival. RESULTS: One hundred fifty-one patients were available for analysis (60 MMS, 91 WLE). Median follow-up time was 48 months. The groups differed in tumor location and mitotic rate. Overall, there was no significant difference in 5-year local recurrence (7.9% WLE vs 6.2% MMS, p = .58), regional or systemic recurrence (18.8% vs 8.8%, p = 0.37) or disease-specific survival (82.8% vs 92.4%, p = .59). Breslow thickness was the only consistent predictor of local recurrence or other recurrence and disease-specific survival on multivariate analysis. Subset analysis of tumors with Breslow thickness less than 2 mm did not reveal any difference in outcomes. CONCLUSION: Mohs micrographic surgery has oncologic outcomes of local recurrence, distant recurrence and overall survival similar to those of WLE for invasive facial melanoma.

[Clinical course of cutaneous melanoma of the head and neck, and the factors affecting patient survival].

It was found that melanomas of the skin of the head and neck, compared to the trunk and limbs, were characterized by a greater proportion of men, an older average age of the patients, high frequency spindle cell tumors, more frequent synchronous distant metastasis and worse survival. Melanomas, which are localized on the skin of the neck and scalp, have similar aggressive clinical course and are characterized by worse overall and disease-free survival than skin melanomas on the trunk and limbs. However, melanomas of the skin on the ear and face proceed more favorably as compared to skin melanomas of the scalp and neck. Multivariate regression analysis using Cox models showed that the melanoma of the head and neck Breslow tumor thickness was the strongest predictor of overall survival. For skin melanomas of the neck and scalp Breslow tumor thickness was the only significant independent factor for overall survival. In melanoma, skin and ear predictors of survival are: sex, age and tumor thickness. In skin melanomas of the face and ear predictors of survival were sex, age and tumor thickness.