Risk factors for local atypical fibroxanthoma recurrence and progression to pleomorphic dermal sarcoma: A meta-analysis of individualized participant data.

BACKGROUND: Risk factors for local atypical fibroxanthoma (AFX) recurrence and progression to pleomorphic dermal sarcoma (PDS) have not previously been identified. OBJECTIVE: To identify risk factors and provide follow-up suggestions for local AFX recurrence and progression to PDS. METHODS AND MATERIALS: A literature search was performed in the PubMed, EMBASE, and Cochrane databases. The PRISMA and MOOSE guidelines were followed. The risks of local AFX recurrence and progression to PDS were presented as Kaplan-Meier plots and risk factors were presented as hazard ratios (HRs) calculated with univariate and multivariate Cox regression. RESULTS: Five hundred and ninety-eight patients with AFX from 14 studies were included. Age >74 years and male sex significantly increased the risk of local recurrence (HR: 7.31 [95% confidence interval [CI]: 1.78-30.0], p < 0.01 and HR: 2.89 [95% CI: 1.04-8.01], p < 0.05, respectively). There was no difference when comparing wide local excision and Mohs' micrographic surgery (p = 0.89). The risks of local AFX recurrence and progression to PDS after 2 years were <1%. CONCLUSION: A more intensive follow-up regimen could be considered in patients >74 years old and males due to the higher risk of local AFX recurrence.

Soft tissue myoepithelial carcinoma-a case report with molecular and electron microscopic analysis.

Myoepithelial tumors are rare neoplasms that primarily occur in the salivary gland but over the last decade have also been described in other locations, including soft tissue. Myoepithelial carcinoma of soft tissue has shown to have a recurrence rate of 39-42% and distant metastases in 32-53% of patients. The only known predictor of malignancy in soft tissue myoepithelial tumors described is cytologic atypia. There are no other validated criteria for differentiating benign and malignant tumors, neither for grading nor for standard of care for these tumor types. Herein, we present a case of myoepithelial carcinoma of inguinal soft tissue in a 37-year male. We also describe the molecular and previously sparingly described detailed electron microscopic features of this case.

[Primary cutaneous sarcomas].

Primary cutaneous sarcomas are a heterogenous group of malignant soft tissue tumours arising in the dermal and subcutaneous layers of the skin. These tumours are rare and have a variety of clinical manifestations. They differ from deep soft tissue sarcomas in terms of prognosis, which for most types of cutaneous sarcomas is favourable. The tumours are primarily managed by complete surgical excision with margin control. The purpose of this review is to present some of these rare tumors and describe their management.

[Breast reconstruction with a breast-sharing technique].

Many techniques have been described for breast reconstruction after mastectomy, and in many cases a significant reduction of the contralateral breast is needed for symmetry. This is a case report of a single-stage secondary breast reconstruction after mastectomy using the excided tissue from the contralateral reduced breast based on the perforant vessels of the internal thoracic artery. We used this technique in a 62-year-old woman with a hypertrophic contralateral breast, who had undergone mastectomy due to breast cancer. It is the first time, this technique has been described in Denmark.

[Atypical presentation of penile blue naevus].

Blue naevus is a rare lesion on genital mucosa and may cause confusion in differential diagnosis with other pigmented lesions. In this case report, a 39-year-old man presented with a sudden onset in adulthood of blue naevus on the glans penis. A biopsy confirmed the diagnosis. Due to the unusual presentation, the onset and the risk of turning invasive, a careful examination was performed in order to minimise any risk of misclassification with melanoma. Afterwards, the patient was followed in a dermatologic department every six months. To our knowledge, only few similar cases have been described in literature.

[Atypical presentation of lentigo maligna melanoma].

Lentigo maligna melanoma (LMM) is the most common subtype of melanoma in the face. In this case report, a 95-year-old woman had a patch of dark hair growing out of her white hair on her scalp. A punch biopsi confirmed the diagnosis of LMM. The unusual in this case is the finding of melanocytic hair growth with no obliteration of follicles in the affected area. As described, obliteration of follicles is usually seen in histology when lentigo maligna progresses. To our knowledge, no similar cases have been described in literature.

Management Recommendations for Merkel Cell Carcinoma-A Danish Perspective.

Merkel cell carcinoma (MCC) is a rare malignant neuroendocrine carcinoma of the skin with a poor prognosis and an apparent increase in incidence. Due to its rarity, evidence-based guidelines are limited, and there is a lack of awareness among clinicians. This review constitutes the consensus management recommendations developed by the Danish MCC expert group and is based on a systematic literature search. Patients with localized disease are recommended surgical excision and adjuvant radiotherapy to the primary site; however, this may be omitted in patients with MCC with low risk features. Patients with regional lymph node involvement are recommended complete lymph node removal and adjuvant radiotherapy in case of extracapsular disease. Metastatic disease was traditionally treated with chemotherapy, however, recent clinical trials with immune therapy have been promising. Immune checkpoint inhibitors targeting the programmed cell death protein 1(PD-1)/programmed death-ligand 1(PD-L1) axis should therefore be strongly considered as first-line treatment for fit patients. A 5-year follow-up period is recommended involving clinical exam every 3 months for 2 years and every 6 months for the following 3 years and PET-CT one to two times a year or if clinically indicated. These national recommendations are intended to offer uniform patient treatment and hopefully improve prognosis.

Novel SUFU Frameshift Variant Leading to Meningioma in Three Generations in a Family with Gorlin Syndrome.

Gorlin syndrome is mainly caused by pathogenic germline variants in the tumour suppressor genes PTCH1 and SUFU, both regulatory genes in the hedgehog pathway. However, the phenotypes of patients with PTCH1 and SUFU pathogenic variants seem to differ. We present a family with a frameshift variant in the SUFU gene c.954del, p.Asn319Thrfs∗42 leading to meningiomas and multiple basal cell-carcinomas.

[Muir-Torre syndrome].

Muir-Torre syndrome (MTS) is an autosomal dominant disease with approximately 200 reported cases worldwide, which is characterised by multiple neoplasms of the skin and internal organs. This is a case report of a 57-year-old woman with MTS, who was referred to a plastic surgery department from a gastroenterology department due to a neoplasm of the skin. After treatment, she was referred to a dermatology department for follow-up. MTS is a rare disease, which is now reported in Denmark. Doctors treating any of the composing elements should have basic knowledge of the condition in order to refer patients appropriately.

[Melanonychia].

Melanonychia is seen, when melanin is incorporated in the nail, which gives a band of dark discolouration of the nail. Nail discolouration is a common cause for patients to seek medical attention, and it is often benign. Melanonychia can be seen due to melanocytic proliferation (ungual naevi), benign hyperplasia (lentigo) and hypermelanosis (infections, traumas). However, subungual melanoma also commonly presents with melanonychia and is often overseen, leading to a worse prognosis. It is therefore important systematically to examine all nail discolourations to find the cause and rule out malignancy.

[Entrapped nerve causing severe post-operative pain from scar tissue].

Entrapment of a cutaneous nerve in a surgical scar may cause chronic post-operative pain. The condition presents with similar symptoms as a traumatic neuroma or as an anterior cutaneous nerve entrapment syndrome, which, however, is often idiopathic. We present a case, where entrapment of a cutaneous nerve in a laparotomy scar caused chronic pain. The symptoms were immediately relieved after selective neurectomy.

[Pyogenic granuloma].

Pyogenic granuloma is a common benign, rapidly growing vascular papule or polyp. The tumour is very vulnerable and bleeds easily after minor trauma. It can occur in the skin or mucosa and is typically localized within the gingiva, the lips, the mucosa of the nose and the face. It can occur at any age but is often seen on children and young adults. The diagnosis pyogenic granuloma is often straightforward, but several differential diagnoses should be considered, including malignant tumour.

[Accidential consumption of wart remover].

Not seldom do people buy medicine abroad while on business or holiday, where international labelling is less than optimal. Once home, the medication is often kept alongside every-day products, sometimes resulting in home accidents due to a confusion of products. In this case a six-month-old girl was administered five drops of monochloroacetic acid orally as a result of mistaking a bottle of D-vitamin with a bottle of acid for the removal of warts. She suffered a mild poisoning, chemical burns and required intubation due to oedema of the upper airways, but no long-term effects.

[Keystone flap for reconstruction of skin defects]. / Keystone ø-lap til rekonstruktion af huddefekter.

INTRODUCTION: The keystone design perforator island flap was first described in 2003. It is a local flap based on axial perforators from the underlying structures. It is designed as a curvilinear shaped trapezoidal design flap which is essentially two V-Y flaps end to end. Most reports are of the lower limbs, but it has been used successfully almost all over the body. MATERIAL AND METHODS: We report our experience in 21 patients, who underwent excision of skin malignancies and reconstruction of the defect with a keystone flap (24 flaps in total). RESULTS: Only few complications were seen. Two patients had signs of infection at the border of the flap and were treated with local and/or oral antibiotics. In two cases a small necrosis developed at the border of the flap. In both cases, it healed conservatively. CONCLUSION: The keystone flap has proven to be very reliable with only few reported flap losses. Furthermore, it is technically easy to perform and thus may be performed by trainee surgeons as well as senior surgeons. The cosmetic result is excellent and far superior to the alternative skin grafting with its color differences and contour defect. In addition, many patients may be treated as day-only surgery or discharged from the hospital after only one day.