Epidemiology of cutaneous sebaceous carcinoma.

There has been uncertainty about the demographics and anatomical distribution of cutaneous sebaceous carcinoma. This study aims to investigate these uncertainties by analysing data from various countries. Data were obtained from cancer registries of the United States, England, Norway and Taiwan, and incidence rates were calculated with uniform age-adjustment. sebaceous carcinoma was more commonly reported in males than females in white populations, whereas the inverse was true in Taiwan. Ocular sebaceous carcinoma was more commonly reported in females than males in all populations, despite male predominance in white populations. The majority (approx. 70-90%) occurred on head and neck in Asians and whites. Age-adjusted incidence rate (to the 2000-2025 WHO World Standard Population) ranged from 0.07 to 0.18 per 100 000 person-years and was not higher in Taiwanese than in white populations.

Cutaneous sebaceous carcinoma.

Cutaneous sebaceous carcinoma occurs almost exclusively on the head and neck and has a significant propensity for recurrence and metastasis. It is easily mistaken for benign conditions, resulting in inappropriate management. Thus, it is important to maintain a high index of suspicion. Despite previous reports, sebaceous carcinoma may occur with similar frequency in Asians and whites. Recent genetic data suggest there are multiple mutational groups of sebaceous carcinoma, paving the way for targeted treatment. After a diagnosis, investigations for staging and for Muir-Torre syndrome should be considered. The available evidence on the treatment options for sebaceous carcinoma is discussed, and specific recommendations for management are made.

An integrated multidisciplinary team approach to the management of vascular anomalies: challenges and benefits.

BACKGROUND: Vascular anomalies comprise highly variable pathophysiology and commonly pose diagnostic and management dilemmas. Consequently, patients often benefit from input from multiple specialists. This study describes the inception of a multidisciplinary team (MDT) Vascular Anomaly Clinic (VAC) in a tertiary paediatric centre, and the subsequent experience managing this complex patient group. METHODS: This was a retrospective study of paediatric patients (< 18 years old) attending an MDT VAC from its inception in October 2012 until November 2019. Patient demographics, presentation, diagnosis and management were reviewed. RESULTS: One hundred and thirty-three paediatric patients were seen over 7 years with a median age of 9.8 years. Vascular malformations were the most common diagnosis (88%), with venous malformations predominating (27%). The most common symptoms were pain (46%) and swelling (34%). Patients often required ≥ 2 investigations, with Doppler ultrasound (86%) and magnetic-resonance imaging (61%) being most common. Management included surgery (27%), sclerotherapy (26%), compression garments (23%), analgesia (12%), laser (15%), embolisation (5%) and sirolimus (3%). CONCLUSIONS: The complex nature of vascular anomalies and high proportion of patients requiring multi-specialty management justified the establishment of an MDT VAC in our centre. Our experience demonstrates the success of an efficient one-stop MDT environment in the management of these challenging conditions. LEVEL OF EVIDENCE: IV.

Dermatology teaching in Australian Medical Schools.

BACKGROUND/OBJECTIVES: Although skin disease and skin cancers cause significant morbidity and mortality in Australia, limited time is dedicated to dermatology teaching in most medical courses. The aim of this study was to define the current state of dermatology teaching in Australian medical schools with a view to developing a national core curriculum for dermatology. METHODS: An electronic questionnaire was circulated to the dermatology teaching leads and relevant medical program coordinators of the 18 medical schools in Australia. RESULTS: Replies were received from 17 medical schools. Dermatology was included as part of the core curriculum in 15 schools. Time set aside for dermatology teaching varied, as reflected by the number of lectures delivered (0-21, mean 5, median 3) and minimum clinics attended (0-10, mean 1.2, median 0). Only four medical schools had a compulsory clinical attachment in dermatology. Furthermore, satisfying requirements in dermatology was mentioned in the university examination regulations in only six schools. Certain core learning outcomes were addressed in most schools, including the structure and function of the skin, common conditions such as atopic dermatitis and psoriasis and cutaneous malignancies. However, there were important omissions, ranging from common problems like dermatophyte infections and drug reactions to the recognition of dermatological emergencies. CONCLUSIONS: These results are a compelling impetus to improve current standards of dermatology teaching, learning and assessment. The introduction of a national core curriculum would provide guidelines for dermatology teaching in medical schools, enabling the more effective utilisation of available time for key learning outcomes.

Perineural Invasion of the Orbit by Neurotropic Nondesmoplastic Melanoma.

The authors report a case of neurotropic nondesmoplastic melanoma involving the ophthalmic division of the trigeminal nerve and the cavernous sinus in a patient with recurrent scalp melanoma. This case highlights the importance of earlier diagnosis of local recurrence of melanoma and the rare association of neurotropic melanoma and orbital metastasis.

Eyebrow reconstruction.

This is a systematic review of eyebrow reconstruction options, using the PubMed database, as well as dermatology and plastic surgery texts. Eyebrow reconstruction options in various clinical scenarios (small, large, medial, lateral, and total defects) are presented. The goals of eyebrow reconstruction are to provide structural, functional, and aesthetic restoration. A good understanding of various eyebrow reconstruction techniques is essential for plastic, dermatologic, and oculoplastic surgeons.

Scalp Basal Cell Carcinoma: Review of 2,202 Cases.

BACKGROUND: Increases in the incidence of basal cell carcinoma (BCC) in women, younger age groups and in aggressive scalp subtypes in younger women have been reported. OBJECTIVE: To describe lesion and patient characteristics in scalp BCC. MATERIALS AND METHODS: Retrospective audit of scalp BCCs from 3 pathology laboratories in Adelaide, South Australia, January 2009-December 2013. RESULTS: Scalp BCC was 2.6% of all BCC. Of 2,202 patients with scalp BCC, 62% were male and 78% were >60 years. Histologic subtypes included nodular (55%), mixed (30%), and superficial (8%). The concordance between biopsy and excision was 83% for division into nonaggressive and aggressive subtypes. The incomplete excision rate was 16%. Aggressive subtypes were larger and had perineural invasion (PNI) in 8.5% and incomplete excision in 26%. CONCLUSION: Basal cell carcinoma on the scalp was less common. Men and the elderly had the majority of cases, with no predilection for women, including aggressive histologic subtypes in younger women. Aggressive subtypes were associated with increased size, incomplete excision, and PNI. A preliminary biopsy assisted division into aggressive and nonaggressive histologic subtypes. Incomplete excision rates were higher and increased in aggressive histologic subtypes and PNI. Mohs surgery or wider margins are suggested in these cases.

In-Transit Metastasis From Squamous Cell Carcinoma.

BACKGROUND: In-transit metastasis from cutaneous squamous cell carcinoma (SCC) is an uncommon form of metastasis through lymphatics and occurs more commonly in immunosuppressed patients. OBJECTIVE: To identify cases of in-transit SCC and determine patient characteristics, tumor features, management, and prognosis. METHODS AND MATERIALS: A multicenter case series treated by Australian and New Zealand clinicians. RESULTS: In 31 patients, median age was 72 years (range 52-99) and 68% were immunocompetent. Tumors occurred on the head and neck in 94% of cases, with 71% of all tumors occurring on the scalp, forehead, or temple. The median time to presentation with in-transit SCC from treatment of the initial tumor was 5 months. Management included surgery (94%), radiotherapy (77%), chemotherapy (10%), and reduction of immunosuppression (3%). Median follow-up was 12 months. Overall survival at 3 and 5 years were 27% and 13%, respectively. CONCLUSION: In-transit metastases are described in 31 patients, of whom the majority was immunocompetent. The scalp, forehead, and temple were the most common sites. New clinical and histological diagnostic criteria are proposed. Prognosis was poor with 5-year survival of 13%. Recommended management is a combination of surgery and adjuvant radiotherapy. Reduction of any iatrogenic immunosuppression should be considered.

Management of squamous cell and basal cell carcinomas of the head and neck with perineural invasion.

Perineural invasion (PNI) occurring in non-melanoma skin cancers (NMSC) is associated with an increased risk of locoregional recurrence and reduced disease-free survival. This necessitates early and accurate diagnosis, appropriate risk-stratification and a clear management strategy. The diagnosis of PNI is based on careful clinical assessment, imaging and histopathology. Surgery, preferably with margin control, and definitive or adjuvant radiotherapy (ART) are established treatment strategies for PNI. Clinical uncertainty remains over the role of ART in incidental PNI. This review synthesises current literature to ascertain which clinicopathological features impart a higher risk to individuals with PNI in NMSC, in order to provide treatment algorithms, including the identification of patient subsets that are most likely to benefit from ART. This includes those with extratumoural PNI, involvement of larger-calibre nerves, tumour invasion beyond dermis, recurrent tumour or diffuse intratumoural spread. Patients with clinical PNI may be optimally managed by a multidisciplinary head and neck cancer service that is best placed to offer skull base surgery and intensity-modulated radiation therapy (IMRT). The management options presented are stratified by histological subtype and a new classification of PNI into low-risk, medium-risk and high-risk groups.

Periocular Squamous Cell Carcinoma: TNM Staging and Recurrence.

PURPOSE: To analyze the TNM stage, management, and recurrence rates of patients with histologically confirmed squamous cell carcinoma (SCC) of the eyelid. DESIGN: Retrospective case series from 3 Australian centers. PARTICIPANTS: A total of 254 cases of eyelid SCC from 254 patients (median age, 73 years; range, 28-102 years; 159 were male). METHODS: Tumors were staged according to The American Joint Committee on Cancer 7th edition TNM criteria for eyelid carcinoma. MAIN OUTCOME MEASURES: Outcomes and recurrence rates according to TNM stage at presentation. RESULTS: A total of 25 cases (9.8%) were recurrent tumors. TNM classifications were as follows: T1N0M0, 74 patients (29.1%); T2aN0M0, 92 patients (36.2%); T2bN0M0, 50 patients (19.7%); T3aN0M0, 31 patients (12.2%); T3bN0M0, 5 patients (2.0%); T2bN0M1, 1 patient (0.4%); and T3bN1M1, 1 patient (0.4%). Perineural invasion (PNI) was present histologically in 8.3% of cases. Treatment modalities included Mohs microsurgery (31.1%), wide local excision (WLE) with paraffin section control (21.7%), WLE with frozen-section control (19.3%), and excision without margin control (24.4%). Three cases did not receive treatment. Median follow-up was 40 months (range, <1-132 months). Local recurrence occurred in 17 treated patients (6.8%). The recurrence rate was 5.3% (12/226 patients) for primary tumors and 20% (5/25 patients) for recurrent tumors (P = 0.019). Four patients (1.6%) died of their disease during follow-up. Higher T stage was significantly associated with both PNI (P = 0.035) and local recurrence (P < 0.001). We could not identify a T-stage threshold below which there was no risk of recurrence, as evidenced by 3 T1 primary tumors that recurred. CONCLUSIONS: Higher T stage was significantly associated with local recurrence, and recurrent tumors had a 4-fold increased risk of further recurrence compared with primary tumors. Therefore, it may be reasonable to consider sentinel lymph node biopsy or close nodal surveillance and follow-up for patients with recurrent or high T-stage tumors. Of note, we could not identify a T-stage threshold below which there was no risk of recurrences; therefore, clinicians should be aware of the potential for low T-stage tumors to recur.

Erbium-doped yttrium aluminium garnet ablative laser treatment for endogenous ochronosis.

Ochronosis is a rare disease characterised clinically by bluish-grey skin discolouration and histologically by yellow-brown pigment deposits in the dermis. It occurs in endogenous and exogenous forms. Endogenous ochronosis, also known as alkaptonuria, is an autosomal recessive disease of tyrosine metabolism, resulting in the accumulation and deposition of homogentisic acid in connective tissue. We report a case of facial endogenous ochronosis and coexistent photodamage, which was successfully treated with erbium-doped yttrium aluminium garnet laser resurfacing and deep focal point treatment to remove areas of residual deep pigment.

Successful treatment of Dowling-Degos disease using intense pulsed light.

Dowling-Degos disease (DDD) is a rare inherited pigmentary disorder characterised by reticulate pigmentation at flexural sites. No treatment modality has yet been established as a definitive method for the effective elimination of lentigines in DDD. We present a case of a 24-year old woman with DDD treated successfully using intense pulsed light as a novel management strategy.

Accuracy of Biopsy in Subtyping Periocular Basal Cell Carcinoma.

PURPOSE: To determine the accuracy of initial biopsy in the diagnosis of basal cell carcinoma (BCC) histologic subtype. METHODS: Retrospective histopathologic review of patients with a diagnosis of primary periocular BCC from 2006 to 2013 inclusive. RESULTS: A total of 174 primary BCCs were identified. BCCs were classified as nodular, superficial, or aggressive (including mixed cases with an aggressive component). Punch biopsies were used in 41% of cases, while the remaining patients underwent shave or incision biopsies. The final histologic subtypes at excision were nodular (59%), superficial (7%), nodular and superficial (7%), and aggressive (51%). The overall concordance between the BCC subtype identified in the biopsy specimen and the subsequent excision specimen was 54%. In total, there were 51 cases (29%) of BCC, which included aggressive subtypes, of which 52% of initial biopsies failed to detect an aggressive component. There were 45 cases (26%) of mixed BCC, and an aggressive histologic subtype was present in 73% of these cases. CONCLUSIONS: The accuracy of initial biopsy for BCC histologic subtype at excision is highest for nodular BCC. For aggressive BCC, biopsy was able to detect the aggressive component in only 48% of cases. This may have implications for choice of treatment modality.

Endocrine mucin-producing sweat gland carcinoma of the eyelid.

An elderly woman was incidentally noted to have a nodular mass on the upper eyelid, whilst under investigation for cataracts. Punch biopsy of this presumed basal cell carcinoma revealed it to be endocrine mucin-producing sweat gland carcinoma (EMPSGC). The tumour extended to the deep dermal layer and comprised solid nests with foci of cystic and papillary change, and additional cytoplasmic and focal extracellular mucin deposits. Immunohistochemistry confirmed epithelial lineage and neuroendocrine differentiation, and adjacent tissue invasion. The tumour was excised completely with Mohs micrographic surgery with no recurrence after 8 months. EMPSGC is a low-grade sweat gland carcinoma with variable neuroendocrine differentiation, a solid, papillary, or cystic growth pattern, and a predilection for the eyelid of elderly women [Am J Surg Pathol 29:1330-1339, 2005]. There have been 54 previously documented cases of EMPSCG. We report an additional case and review the literature.

Double island pedicle or V-Y flap repair for partial-thickness combined defects of the cutaneous and mucosal lip.

BACKGROUND: Removal of skin cancer at or near the vermilion border may result in a partial-thickness combined cutaneous and mucosal lip defect for which repair has potential for poor cosmetic and functional outcomes. OBJECTIVE: We sought to describe the closure and results from repair of combined lip defects using 2 island pedicle or V-Y flaps, 1 for the cutaneous lip and 1 for the mucosa. METHODS: A retrospective review of all patients with combined defects of the lip who underwent double island pedicle or V-Y flap repair from June 2008 to December 2013 was performed. RESULTS: Ten patients (6 female, 4 male; ages 35-89 years, mean age 60 years) had defects on the upper lip in 8 cases and on the lower lip in 2 cases. Follow-up was for 3 months or longer with good or excellent outcomes in all cases. LIMITATIONS: This was a nonrandomized, unblinded clinical case series with a limited sample size. CONCLUSION: Double island pedicle or V-Y flap repair is an elegant closure with good to excellent results and may avoid potential problems inherent in other repair options.

Histological subtypes of periocular basal cell carcinoma.

BACKGROUND: To determine the proportion of different subtypes of periocular BCC in South Australia. DESIGN: Retrospective review. PARTICIPANTS: One thousand seven hundred thirteen consecutive periocular basal cell carcinoma (BCC) excision specimens. METHODS: Histological analysis of consecutive periocular BCC specimens. MAIN OUTCOME MEASURES: Date of resection, patient age at resection, gender, tumour location, histological subtype and perineural invasion. RESULTS: From 2006 to 2012, a total of 1713 consecutive periocular BCC excision specimens were analysed. The mean age at resection was 68.8 years (median: 71, range: 21-101). Most specimens (56.4%) were removed from male patients. 52.7% involved the lower eyelid, 29.0% the medial canthus, 10.9% the lateral canthus and 7.5% the upper eyelid. The main histological subtypes identified were nodular (65.7%), infiltrative (17.5%), superficial (12.6%) and micronodular (4.2%). Of the specimens, 25.6% had more than one subtype. The most common subtype combinations were nodular with infiltrative (49.7%), and nodular with superficial (26.0%). CONCLUSIONS: The majority of periocular BCC were located on the lower lid and classified histologically as nodular. Infiltrative BCC occurred more frequently than the superficial subtype. As the proportion of mixed BCC containing aggressive subtypes is high, surgical excision with margin control should be considered for periocular BCC.

Intense pulsed light therapy (IPL) induced iritis following treatment for a medial canthal capillary malformation.

The popularity of intense pulsed light (IPL) therapy continues to increase due to its relative safety, high skin coverage rate and ability to treat both vascular and pigmented lesions. An often-overlooked risk is the potential for IPL-induced ocular damage. The damage sustained can cause significant, persistent morbidity and can occur even with very limited IPL exposure to the eye.