Primary dermatofibrosarcoma protuberans invading the orbit.

A 38-year-old man presented with a slow-growing, firm cutaneous mass beneath his left eyebrow. Histopathology and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans. The mass infiltrated the medial canthal tendon and anterior orbital fat and could not be completely excised with Mohs micrographic surgery. The patient underwent exenteration and dacryocystectomy with margin-controlled excision and remained free of disease 9 months after surgery. To our knowledge, no prior case of primary dermatofibrosarcoma protuberans involving the orbit has been reported.

Disease Progression in Cutaneous Squamous Cell Carcinoma Patients With Satellitosis and In-transit Metastasis.

BACKGROUND/AIM: Satellitosis/in-transit metastasis (S-ITM) has prognostic value in melanoma and Merkel cell carcinoma, but is not incorporated into cutaneous squamous cell carcinoma (cSCC) staging. PATIENTS AND METHODS: From our IRB-approved registry, patients with high-risk cSCC, including patients with S-ITM, were identified. Univariate (UVA) and multivariate (MVA) analyses were performed to compare disease progression (DP) and overall survival (OS). Cumulative incidence of DP and OS analyses were performed using Fine-Gray and Kaplan-Meier methods, respectively. RESULTS: A total of 18 S-ITM subjects were compared to 247 high risk subjects including T3N0 (n=143), N1-N3 without extranodal extension (ENE) (n=56), N1-N3 with ENE (n=26) and M1 disease (n=22). Median follow up was 16.5 months. Three-year rates of DP were 22% for T3N0, 42% for S-ITM, 48% for T4 bone invasion, 50% for N1-N3 without extranodal extension (ENE), 53% for N1-N3 with ENE, and 66% for M1. Patients with S-ITM did not experience significantly worse DP compared to those with T3N0 (HR=1.96, 95%CI=0.8-4.9; p=0.14). CONCLUSION: Cutaneous SCC patients with S-ITM experienced outcomes similar to locally advanced non-metastatic cSCC patients. Larger studies are needed to guide incorporation into staging systems.

Flaps and grafts reconstruction.

Reconstruction of Mohs surgical defects is a challenging venture. A thorough understanding of skin physiology and anatomy (cosmetic subunits, relaxed skin tension lines, underlying neurovascular structures at risk, potential functional compromise, character of adjacent skin, and so forth), careful wound analysis, and meticulous operative techniques is key to a successful reconstruction. This article discusses in detail the use of local skin flaps and graft reconstruction.

Klippel-Trenaunay syndrome: a multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth.

Klippel-Trenaunay syndrome is characterized by a triad of varicose veins, cutaneous capillary malformation, and hypertrophy of bone and soft tissue. Appropriate evaluation and treatment of children displaying features of the disease may minimize morbidity. The clinical appearance, etiology, genetics, diagnostics, and treatment of Klippel-Trenaunay syndrome are herein explored.

Primary melanoma of the umbilicus: report of a case and review of the relevant anatomy.

BACKGROUND: Malignant melanoma presenting on the umbilicus is rare. Treatment of melanoma on the umbilicus is difficult because of the unusual anatomy of the umbilicus. OBJECTIVE: To present the case of a patient with a primary malignant melanoma of the umbilicus and to discuss treatment concerns that are relevant to the umbilical anatomy. METHODS: This is a case report as well as a review of the pertinent anatomy. RESULTS: The initial excision was read as having clear deep and lateral margins on histopathologic examination. Postoperative complications led to a re-excision down to and including the umbilical attachment to the peritoneum. The re-excision revealed residual primary cutaneous malignant melanoma in a remnant umbilical cyst. CONCLUSION: When a malignant melanoma is located on the vertical sidewall or bottom of the umbilicus, one should consider surgical excision down to peritoneum, including the umbilical attachment to the peritoneum. Because this is beyond the scope of training for most dermatologic surgeons, it would be appropriate to refer these patients to a surgical oncologist or a plastic surgeon.