Background and objectives: Leiomyosarcoma of skin (LMS) can be sub-classified on pathology appearances as Dermal or Subcutaneous. The aim of this study was to provide treatment recommendations for these uncommon tumours. Methods: A retrospective review of all patients with dermal and subcutaneous leiomyosarcoma managed at the Peter MacCallum Cancer Centre, Australia from January 2003 to December 2018 was performed. Eighty-three patients were identified (64 dermal leiomyosarcoma, 19 subcutaneous leiomyosarcoma). Results: Subcutaneous leiomyosarcoma were larger (median size 14 mm dermal, 49 mm subcutaneous, P = 0.01). No patient with a dermal leiomyosarcoma developed metastatic disease compared to 4 of the 19 subcutaneous leiomyosarcoma (5-year overall survivals, 98% and 88%, respectively, P = 0.03). The most common site of metastasis was to the lung. No difference in risk of local recurrence was apparent (5-year recurrence-free survivals were 85% and 78%, respectively, P = 0.17). Adjuvant radiotherapy was used in 16 (25%) dermal leiomyosarcoma patients and 13 (68%) subcutaneous leiomyosarcoma patients (P < 0.001). Local recurrence was uncommon in both tumour subtypes when patients received definitive surgical excision (minimum histological margins of 10 mm as per institutional protocol) regardless of whether radiotherapy was used. The 5-year local recurrence-free survival for dermal leiomyosarcoma treated with radiotherapy was 93% versus 83% without radiotherapy (P = 0.7) and for subcutaneous leiomyosarcoma was 69% and 100%, respectively (P = 0.9). Conclusions: Dermal leiomyosarcoma have an excellent prognosis, particularly after definitive surgical excision with margins of at least 10 mm. Subcutaneous leiomyosarcoma has poorer outcomes and should be managed by wider excision and considered for adjuvant radiotherapy.

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PEODDN is a congenital hamartoma, adult-onset cases have been described. 1 Histopathological examination shows an epidermal invagination with a parakeratotic column placed over a hyperplastic eccrine duct with a dilated acrosyringium and loss of the granular layer. 1 The exact pathogenesis of PEODDN is unclear. Positive immunohistochemical staining for carcinoembryonic antigen along the eccrine duct lumina and acrosyringium through the parakeratotic column of the cornoid lamellae leads to a suggestion that PEODDN result from circumscribed keratinisation abnormality relating to the hypertrophic and abnormal acrosyringia. 2 Moreover, mosaic mutation in GJB2 in these patients was indicated and introduced PEODDN as a possible mosaic form of KID syndrome. 4 Our case presented with late-onset asymptomatic linear keratotic papules, with sparing the face, palms and soles, but with characteristic histopathological features of PEODDN, which progressed despite various local and systemic therapies. 3 Various therapeutic modalities including cryotherapy, topical steroids, retinoids, 5-fluorouracil, anthralin, phototherapy 2 and oral retinoids 5 have been tried with limited efficacy. Although PEODDN is a benign disease, long-term follow-up are recommended because of possible progression to SCC or Bowen disease. 2

A Caucasian man aged 60 years presented with a six-month history of an annular, red, scaly plaque on his posterior thigh (Figure 1a). The plaque had started as a small papule, which then slowly expanded to >10 cm in diameter. He was systemically well but had a past history of alcohol dependence and liver cirrhosis. 1 Question 1 What conditions commonly present as annular scaly plaques? Question 2 What additional history would be useful for refining the differential diagnosis? Question 3 What initial investigations would be appropriate?