NONMELANOMA SKIN CANCER

P8197A 10-year retrospective review of vulval squamous cell carcinomaassociated with lichen sclerosus

Shaheen Haque Hussain, MBBS, Addenbrooke’s Hospital, Cambridge, UnitedKingdom; Aslam Shiraz, MBBS, Addenbrooke’s Hospital, Cambridge, UnitedKingdom; George Meligonis, MBBS, Addenbrooke’s Hospital, Cambridge, UnitedKingdom; Jane Sterling, MBBCh, PhD, Addenbrooke’s Hospital, Cambridge,United Kingdom; Moumita Chattopadhyay, MBBS, Addenbrooke’s Hospital,Cambridge, United Kingdom; Peter Baldwin, MBBS, MD, Addenbrooke’’sHospital, Cambridge, United Kingdom

Vulval squamous cell carcinomas (vSCCs) are uncommon, and are diagnosed in 1-2/100,000 women annually. HPV-related VIN or chronic dermatoses, eg, lichensclerosus (LS) or lichen planus (LP) are recognized aetiologic factors. Retrospectivestudies published upto 15 years ago have shown histologic evidence of LS inapproximately 60% of vSCCs. Our aim was to identify whether the introduction ofsuperpotent topical steroid treatment as standard practice has effectively controlledinflammation such that LS-associated vSCCs occur less frequently. 224 vSCCs in 197patients (mean age, 70.3 years [range, 34-94]) were diagnosed from January 2003 toJanuary 2013. Review of histopathology reports identified 71 (31.7%) cases whereLS was found in adjacent skin. 83 (37.1%) cases had adjacent VIN, 3 (1.3%) cases hadadjacent LP. No adjacent skin abnormality was described in 67 (29.9%) cases. Clinicalnote review of patients with histologic evidence of LS showed that 19 (27%) patientswere known to have longstanding LS and 9 patients had longstanding vulvalsoreness or itching, not otherwise specified. 42 (59%) patients were not known tohave LS despite histologic evidence. Our results show that LS is associated with ?\comment[This should probably not be a question mark.\/comment[ of vSCCshistologically. This reduction from previous reports may suggest that currentmanagement of LS decreases cancer risk, or that the incidence of VIN-associatedcancer is rising. It is interesting, however, that a significant proportion of patientswith histologic LS who have developed a vSCC, have had asymptomatic disease ornot been clinically identified.

AB132

cial support: None identified.

Commer

P8009An unusual presentation of angiosarcoma: Solid facial edema in an 82-year-old man

Kathryn Dempsey, MD, Medical University of South Carolina, Charleston, SC,United States; Brian Leach, MD, Medical University of South Carolina Departmentof Dermatology and Dermatologic Surgery, Charleston, SC, United States; SarahJacks, Medical University of South Carolina College of Medicine, Charleston, SC,United States

Background: Angiosarcoma is an aggressive, malignant neoplasm of endothelialorigin that comprises\2% of all soft tissue sarcomas. Early lesions often appear asexpanding erythematous or bruise-like patches and can be quite deceptive, leadingto delayed presentation and diagnosis.

Case report: An 82-year-old white male was referred for evaluation of generalizedfacial edema. He had previously been evaluated by 9 physicians for this same issue.His symptoms began as left lower eyelid swelling that progressively spread toencompass his entire face, scalp, and neck over 6 to 7 months. Upon presentation toour clinic, the edema was so severe that he was having difficulty opening his eyes.Previous diagnoses had included keratoconjunctivitis sicca, contact dermatitis, andcellulitis. Work-up had included a normal facial radiograph, extensive normallaboratory assessments, and CT imaging showing mild skin thickening and subcu-taneous edema. Physical examination revealed edematous, indurated, red, weepyplaques encompassing the entire facial region extending onto his neck, proximalchest, and scalp. Significant periorbital edema was present. An excisional biopsyperformed from the right mandible was consistent with angiosarcoma, confirmedwith CD34+ staining in the atypical cells. CT scan of the head and chestdemonstrated no evidence of metastatic disease. Given the locally advanced,unresectable extent of his disease at diagnosis, paclitaxel was the recommendedinitial treatment. After 4 rounds of paclitaxel, dramatic improvement was noted inhis appearance with significant reduction in the edema and erythema of his face andscalp. He subsequently developed a nonpruritic, erythematous, papular rash on hisanterior chest and neck shortly after completion of the paclitaxel. Biopsy wasconsistent with angiosarcoma.

Discussion: Diagnosis of angiosarcoma is often delayed because of its variable andfrequently innocuous clinical presentation. Our patient presented with progressivesolid facial edema that was misdiagnosed for months as contact dermatitis andinfection. During the course of treatment, he developed amorbilliform eruption thatproved to be progression of his disease. Angiosarcoma should be considered in thedifferential diagnosis of a patient with the presentation of airborne contactdermatitis or cellulitis refractory to conventional therapy. Prompt recognition anddiagnosis of this highly aggressivemalignancy can significantly impact morbidity andmortality.

cial support: None identified.

Commer

J AM ACAD DERMATOL

P7701Carbozantanib may be an effective treatment of TMEP with D816V muta-tion in KIT

Kabeer Shah, MS, Rocky Vista University, Parker, CO, United States; Aaron Dunn,DO, MPH, University of Maryland, Baltimore, MD, United States; Carol Amato,MS, University of Colorado, Aurora, CO, United States; David Norris, MD,University of Colorado, Aurora, CO, United States; Joshua Wisell, MD,University of Colorado, Aurora, CO, United States; William Robinson, MD, PhD,University of Colorado, Aurora, CO, United States

Background: Telangectasia macularis eruptiva perstans (TMEP) is an uncommonsubtype of mastocytosis that shares the same somatic activating mutation in KIT(D816V). No current therapy has been shown to be of consistent long-term benefitin TMEP. We examined a number of signal transduction inhibitors in vitro for theirability to block the growth of cells containing a D816V construct. The most effectiveof these that is currently available for clinical use was cabozantinib. Based on our invitro data, we initiated a trial of this agent in a patient with TMEP and systemicmastocytosis.

Case report: A 35-year-old female presented with a telangectatic rash on the anteriorthighs which progressed over the next 4 years to encompass her arms, legs, andtrunk, sparing only the hands and face. A skin biopsy showed TMEP with a D816Vmutation in KIT. Despite treatment with diphenhydramine, cetirizine, cromolyn,narrowband UV, electron beam radiation, and cytoreductive therapies (dasatinib andinterferon-alfa, cladribine) her disease burden progressed with biopsy-proveninvolvement of the small bowel and bone marrow. A trial of oral cabozantanib at140 mg/day was begun and the dose reduced to 100 mg/day after 4 weeks when thetelangectasia had largely cleared from her arms and lower legs. She continues ontreatment with minimal side effects and continued clinical improvement. Clinicalphotographs and skin biopsies correlate pre- and posttreatment results.

Conclusions: This single case study suggests that cabozantanib may be an effectivetreatment for patients with TMEP and other forms of mastocytosis with D816Vmutations in KIT.

cial support: None identified.

Commer

P7872Clincopathologic predictive factors in breast angiosarcoma

Erica Ghareeb, MD, University of Pittsburgh Cancer Institute, Magee Women’sHospital of UPMC, Pittsburgh, PA, United States; Anca Florea, MD, University ofPittsburgh Cancer Institute, Magee Women’s Hospital of UPMC, Pittsburgh, PA,United States; John Vargo, MD, University of Pittsburgh Cancer Institute, MageeWomen’s Hospital of UPMC, Pittsburgh, PA, United States; Rohit Bhargava, MD,University of Pittsburgh Cancer Institute, Magee Women’s Hospital of UPMC,Pittsburgh, PA, United States; Sushil Beriwal, MD, University of Pittsburgh CancerInstitute, Magee Women’s Hospital of UPMC, Pittsburgh, PA, United States

Purpose: Angiosarcoma of the breast is an aggressive malignancy with highrecurrence rates and poor overall survival. Because of its rarity, limited data existto guide treatment. Thus, we sought to identify clinicopathologic features associatedwith tumor recurrence and survival to better guide management.

Methods: After IRB approval, we identified 35 patients with histopathologicallydiagnosed breast angiosarcoma at UPMC Magee Women’s Hospital from June 1994to March 2011. All slides were rereviewed by an expert breast pathologist beforeinclusion. At time to pathology rereview, pathologic descriptors of morphology:anastomosing vascular channels, nuclear hyperchromasia, infiltrative growth,endothelial tufting, solid/spindle cell foci, blood lakes, necrosis, apoptosis, absolutemitoses per 10 high-power fields, nuclear size, vesicular nuclei, prominent nucleoli,and percentage of epithelioid tumor cell component were recorded and comparedusing the KaplaneMeier method to tumor recurrence and survival.

Results: Complete pathology was available for 22 patients, with a median clinicalfollow-up of 9months (range, 3-133). The presence of necrosis and absolute numberof mitotic figures (continuous and¼ 10 per 10 high-powered fields) were significantpredictors of local recurrence, which drove disease-free and overall survival. Whileendothelial tufting and solid/spindle cell foci did not significantly impact local failureor survival the presence of these morphologic characteristics correlated withsignificantly higher rates of distant failure. In a modified combined morphologicscoring system (using the parameters mentioned inmethods), combined scores of¼15 correlated with significantly decreased rates of local control (2-year local control14% vs. 80%, respectively; P ¼ .004).

Conclusions: While limited by small patient numbers and retrospective design, thedata presented herein suggest potential pathologic correlates for recurrencepatterns that, with further validation, may help guide local and systemic manage-ment in angiosarcoma.

cial support: None identified.

Commer

MAY 2014