Definition, Association with Malignancy, Biologic Behavior, and Treatment of Ectopic Extramammary Paget's Disease: A Review of the Literature.

Background: Extramammary Paget's disease (EMPD) is a rare neoplasm that affects apocrine gland-bearing skin. Patients usually present with a long-standing, unilateral, ill-defined, erythematous, crusted patch or plaque. In certain cases, this disease can be associated with internal malignancy. EMPD arising in anatomic areas devoid of apocrine glands is exceedingly rare and is termed ectopic extramammary Paget's disease (E-EMPD). Objective: We discuss the classification, origin, and management of E-EMPD. Methods: We reviewed the literature and herein discuss the reported cases of true E-EMPD. Discussion: Forty-five cases of E-EMPD disease were identified in the literature. The etiology and pathophysiology are largely unknown. Based on there only being one reported case of lymph node metastasis and associated underlying malignancy identified, compared to EMPD, ectopic lesions appear to be less aggressive. Various treatment options have been reported, with surgical removal being the preferred method of treatment. Conclusion: E-EMPD is a rare neoplasm that manifests in areas without apocrine glands. With adequate formal data unavailable, the authors recommend that a diagnosis of E-EMPD warrants a focused malignancy screening. Surgical excision is the most common first-line treatment. Further research is needed regarding the etiology, biologic behavior, and treatment of E-EMPD.

Polypoid amelanotic melanoma: a diagnostic challenge.

Melanoma is a highly aggressive cutaneous malignancy with considerable risk for metastasis. These malignant tumors are typically pigmented given that they arise from melanocytes capable of producing melanin. Amelanotic melanomas are a rare variant and there is often a delay in diagnosis owing to lack of pigmentation. Although there are various presentations of amelanotic melanoma, a solitary polypoid nodule is unusual and warrants further reporting. Herein, we present a patient with a 3-year history of a tender firm, skin-to-pink colored polypoid nodule. Excisional biopsy and work up showed an aggressive amelanotic melanoma with depth of 20mm and nodal metastasis consistent with stage IIIC disease. This case highlights the necessity of recognition and prompt management of this rare subtype of melanoma.

A Review of the Global Guidelines on Surgical Margins for Nonmelanoma Skin Cancers.

Background: Adequate treatment and follow-up of a patient with nonmelanoma skin cancer is of utmost concern for a clinician. However, there is a lack of international consensus on recommendations for surgical excision margins. Furthermore, lack of familiarity of the published guidelines leads to a variety in practice styles. Objective: To compare the consistency in global recommendations for surgical excision margins for basal cell carcinoma, cutaneous squamous cell carcinoma, dermatofibrosarcoma protuberans, and Merkel cell carcinoma. Methods: A review of the current literature and global guidelines for surgical excision margins for basal cell carcinoma, cutaneous squamous cell carcinoma, dermatofibrosarcoma protuberans, and Merkel cell carcinoma. Results: Upon review of international guidelines, variations do exist among guidelines for peripheral and deep surgical margins. Guideline recommendations were found to be more globally consistent in margin selection for low-risk basal cell carcinoma and low-risk cutaneous squamous cell carcinoma, however, least consistent when concerning margin selection for dermatofibrosarcoma protuberans and Merkel cell carcinoma. Conclusion: Although guidelines exist, there is a need for international collaboration and consensus to determine a more unified and evidence-based approach to surgical excision as a treatment for nonmelanoma skin cancer.

Reed syndrome: an atypical presentation of a rare disease.

Reed syndrome, also known as Multiple Uterine and Cutaneous Leiomyomas (MCUL), is an autosomal dominant defect in the fumurate hydrase gene, leading to a predisposition of leiomyomas of the skin and uterus. Patients with Reed syndrome may present with cutaneous leiomyomas, uterine leiomyomas and/or leiomyosarcomas. A 37-year-old woman presented to the dermatology clinic with several subcutaneous nodules. Punch biopsy was performed and the diagnosis of angioleiomyosarcoma with supervening degenerative changes was made. Medical history was positive for uterine leiomyomas. These concomitant findings led to the diagnosis of Reed syndrome. At the present time, genetic counseling is a suggested screening parameter for patients with multiple cutaneous leiomyomas. The superficial nature of these lesions and the low staging made surgical excision the preferred and actual treatment method. Adjunct radiation and chemotherapy have not been shown to provide clear benefit of survival. Owing to an association with renal cell carcinoma, a referral for nephrology consultation is also recommended.