a case of dermatofibrosarcoma protuberans

1
P6226 The effect of full body skin examinations on the prognosis of melanoma Michelle Cheng, University of Pittsburgh School of Medicine, PIttsburgh, PA, United States; Jacqueline Moreau, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States; Laura Ferris, MD, PhD, University of Pittsburgh, Department of Dermatology, Pittsburgh, PA, United States; Sean McGuire, MD, University of Pittsburgh School of Medicine, Pittsburgh, PA, United States Background: The greatest impact of melanoma mortality is likely to occur through improved early detection of thinner melanomas. Evidence suggests that access to a dermatologist and having had a recent skin examination is associated with lower melanoma mortality and thinner invasive melanomas. However, the optimal frequency of dermatologic evaluation to impact melanoma depth is not known. This can be estimated by determining the impact of time from last dermatologic examination on melanoma depth. Methods: We reviewed the charts of 406 patients age 18 years and older diagnosed with melanoma. Patient demographics, melanoma depth, method of melanoma detection (dermatologist vs other) and days between last dermatology office visit and date of melanoma diagnosis were collected. Results: Of the 406 melanomas reviewed, depths were available for 401, of which 226 (56.4%) were in situ and 175 (43.4%) were invasive. 236 (58.3%) of patients had been seen for at least one dermatology visit in our department before their melanoma diagnosis, and 169 (41.9%) had not been seen by a dermatologist in our system before their melanoma diagnosis. In comparison to patients without a prior visit, patients who had been seen by a dermatologist before their melanoma diagnosis were more likely to be diagnosed with a melanoma in situ (63% vs 46%, P ¼ .001) and to have a thinner invasive melanoma (mean Breslow depth 0.62 mm vs 1.29 mm, P \.001). There was no significant difference in the depth of melanomas between patients seen less than a year before diagnosis and those seen at any time before diagnosis, and the difference in thickness was seen in both dermatologist- detected melanomas and melanomas that were part of the patient’s chief complaint. Conclusion: A single visit to a dermatologist may reduce the risk of being diagnosed with an invasive melanoma and may improve the likelihood of being diagnosed with a thinner invasive melanoma. More investigation is needed to determine if this effect is due to increased patient education and awareness or a direct screening benefit. A one-time screening for melanoma in the general population of adults over age 50 has been shown to be cost effective. Our data support that patients who have had even a single dermatology visit are more likely to be diagnosed with a thinner melanoma and have a better prognosis than patients without a history of a past dermatologic examination. Commercial support: None identified. P6738 Tumor with 2 years’ development in the heel of a 71-year-old woman: A difficult diagnosis Mar ıa Salazar Nievas, San Cecilio Hospital, Granada, Spain; Jose Abad Romero Balmas, San Cecilio Hospital, Granada, Spain; Mercedes Caba Molina, San Cecilio Hospital, Granada, Spain Background: Amelanotic melanoma represents between 1.8% and 8.1% of melano- mas. Due to the lack of pigment, diagnostic errors tend to occur delaying the treatment and reducing the survival of these patients. Case report: A 71-year-old woman with no personal or family history, consulted because of the appearance of a pink-colored lesion on the left heel, an asymptomatic lesion with progressive growth over last 2 years. Examination revealed a skin lesion of 2 to 3 cm in diameter, with sharp borders, strong consistency, nodular and pink colored. Other satellite bluish lesion was also observed next to the previous one. With the suspicion of basal cell carcinoma versus Kaposi sarcoma, surgery is decided. The anatomicepathologic study concludes that it is an amelanotic melanoma in vertical growth phase, Breslow 3, 7 mm and Clark level IV. Following this finding, the patient is referred to the Melanoma Unit for reexcision with 2 cm safety margins and to perform the sentinel node technique. Conclusion: We report a case of amelanotic melanoma, a clinical rare variant of melanoma. The definition of amelanotic melanoma is clinicopathologic, defined as an injury where melanin is absent or very weak, implying that clinically the tumor lacks pigment. Its diagnosis presents difficulties that often lead to delays that affect treatment and prognosis, so that both dermatologists and primary care physicians should have a high index of suspicion for early diagnosis or timely referral. Commercial support: None identified. NONMELANOMA SKIN CANCER P6455 A case of dermatofibrosarcoma protuberans I. Lym Chan, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; Mariana Campos Souza Menezes, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; Stella Velloso Ramos e Silva, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; Sueli Coelho da Silva Carneiro, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil; Tullia Cuzzi, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil Dermatofibrosarcoma protuberans (DFSP) consists of a rare fibrohistiocytic neopla- sia with intermediate to low grade malignancy and has an annual incidence of 0.8- 4.5/million. The standard treatment is wide local excision. Some therapeutic alternatives are described, such as the use of imatinib mesylate. Herein, we report a case of an exuberant DFSP. A 41-year-old man presented with a mass that had been growing for over 2 years on the back. He underwent surgery to remove a similar lesion from the same site 2 months before the emergence of the current disease. At that time, histopathology was not performed. Clinical examination revealed the tumor to be exophytic, lobulated, brown at the periphery and lighter in the center, with telangiectasia, as well as pain to the touch in right lower paravertebral region. Histopathology was consistent with DFSP. Metastases were excluded and he was referred for surgical resection and adjuvant radiotherapy. DFSP is a cutaneous sarcoma that accounts for 0.1% of all malignancies and 1% of all soft tissue sarcomas. It has a slow growth, low metastatic potential and high recurrence rate due to its ability to infiltrate subcutaneous tissue, fascia and underlying muscle. Clinically, it usually presents as an asymptomatic multinodular reddish-blue or brown plaque, more common on the trunk and extremities. DFSP is most commonly seen among those in their second to fifth decade, with slight male predominance. Histopathology shows uniform spindle cells containing elongated nuclei arranged in a predominantly storiform pattern. The general immunostaining pattern is CD34 positive and factor XIIIa negative. It is also negative for other markers such as S-100, HMB45, desmin and actin. Over 90% of cases present rearrangement of chromo- somes 17 and 22, resulting in continuous activation of tyrosine kinase, promoting DFSP cell growth. Imatinib mesylate is a potent tyrosine kinase inhibitor approved to treat adults with unresectable, recurrent, and/or metastatic disease, with 65% response. The standard treatment to local disease is radical, local excision with 2-3 cm wide margins and three-dimensional resection that includes skin, subcutaneous tissue and the underlying fascia. Late local recurrence of the tumor is up to 24% to 90%. Mohs micrographic surgery emerges as treatment of choice. Radiotherapy is an adjuvant therapy after wide surgical excision or in inoperable macroscopic disease, reducing the risk of local recurrence. Commercial support: None identified. P6486 A case of multiple actinic keratosis, Bowen disease, squamous cell carci- noma, basal cell carcinoma after PUVA therapy in a psoriasis patient Kyu Uang Whang, MD, PhD, Soonchunhyang University Hospital, Seoul, South Korea; Han Eul Lee, MD, Soonchunhyang University Hospital, Cheonan, Cheonan, Chungcheongnam-do, South Korea; Ji Hoon Sim, MD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea; Sung Yul Lee, MD, PhD, Soonchunhyang University Hospital, Cheonan, Cheonan, Chungcheongnam-do, South Korea; Ye Seul Kim, MD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea; You In Bae, MD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea; Young Lip Park, MD, PhD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea Systemic photochemotherapy (PUVA) has been widely used as effective treatment of psoriasis, vitiligo, cutaneous T-cell lymphoma, and other skin diseases. However, the use of PUVA has been restricted when it comes to a long-term treatment as it may cause premalignant lesion such as actinic keratosis and skin cancers including squamous cell carcinoma, basal cell carcinoma, and malignant melanoma. A recent report showed that squamous cell carcinoma is proportionate to the cumulative dose, especially when treated with PUVA more than 150 times it shows a significant increase of various skin disorders , and was not decreased over 15 years’ follow-up since the treatment. Mutations of the p53 gene in malignant tumors after the PUVA treatment have been reported, and a study on its mechanism of carcinogenesis is underway. A 64 - year - old female who had been treated with PUVA for chronic plaque psoriasis, and admitted with exudative erythema of right upper arm 17 years past termination of treatment. The patient received 332 times of PUVA therapy for 40 months about 20 years ago and her past history showed that she had frequent sun exposure. Skin biopsies at the patient’s right upper arm, trunk, abdomen, and bilateral thigh were performed, and DNA sequence analysis for exon 5, 6, 7, 8 on some of these samples was went through to examine p53 gene mutation. Actinic keratosis, squamous cell carcinoma, basal cell carcinoma and Bowen disease were diagnosed from various biopsied sites. p53 sequence analysis on the abdominal lesions, diagnosed as actinic keratosis, confirmed TAT / GAT mutation of exon 6 codon 205. All lesions were completely resected and have been followed up regularly. Here we report the case of multiple actinic keratosis, Bowen disease, squamous cell carcinoma, basal cell carcinoma after PUVA therapy in a psoriasis patient as it is very rare in East Asian population. Commercial support: None identified. APRIL 2013 JAM ACAD DERMATOL AB155

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P6226The effect of full body skin examinations on the prognosis of melanoma

Michelle Cheng, University of Pittsburgh School of Medicine, PIttsburgh, PA,United States; Jacqueline Moreau, University of Pittsburgh School of Medicine,Pittsburgh, PA, United States; Laura Ferris, MD, PhD, University of Pittsburgh,Department of Dermatology, Pittsburgh, PA, United States; Sean McGuire, MD,University of Pittsburgh School of Medicine, Pittsburgh, PA, United States

Background: The greatest impact of melanoma mortality is likely to occur throughimproved early detection of thinner melanomas. Evidence suggests that access to adermatologist and having had a recent skin examination is associated with lowermelanoma mortality and thinner invasive melanomas. However, the optimalfrequency of dermatologic evaluation to impact melanoma depth is not known.This can be estimated by determining the impact of time from last dermatologicexamination on melanoma depth.

Methods: We reviewed the charts of 406 patients age 18 years and older diagnosedwith melanoma. Patient demographics, melanoma depth, method of melanomadetection (dermatologist vs other) and days between last dermatology office visitand date of melanoma diagnosis were collected.

Results: Of the 406 melanomas reviewed, depths were available for 401, of which226 (56.4%) were in situ and 175 (43.4%) were invasive. 236 (58.3%) of patients hadbeen seen for at least one dermatology visit in our department before theirmelanoma diagnosis, and 169 (41.9%) had not been seen by a dermatologist in oursystem before their melanoma diagnosis. In comparison to patients without a priorvisit, patients who had been seen by a dermatologist before their melanomadiagnosisweremore likely to be diagnosedwith amelanoma in situ (63% vs 46%, P¼.001) and to have a thinner invasive melanoma (mean Breslow depth 0.62 mm vs1.29 mm, P\.001). There was no significant difference in the depth of melanomasbetween patients seen less than a year before diagnosis and those seen at any timebefore diagnosis, and the difference in thickness was seen in both dermatologist-detected melanomas and melanomas that were part of the patient’s chief complaint.

Conclusion: A single visit to a dermatologist may reduce the risk of being diagnosedwith an invasive melanoma and may improve the likelihood of being diagnosed witha thinner invasive melanoma. More investigation is needed to determine if this effectis due to increased patient education and awareness or a direct screening benefit. Aone-time screening for melanoma in the general population of adults over age 50 hasbeen shown to be cost effective. Our data support that patients who have had even asingle dermatology visit are more likely to be diagnosed with a thinner melanomaand have a better prognosis than patients without a history of a past dermatologicexamination.

APRIL 20

cial support: None identified.

Commer

P6738Tumor with 2 years’ development in the heel of a 71-year-old woman: Adifficult diagnosis

Mar�ıa Salazar Nievas, San Cecilio Hospital, Granada, Spain; Jose Abad RomeroBalmas, San Cecilio Hospital, Granada, Spain; Mercedes Caba Molina, San CecilioHospital, Granada, Spain

Background: Amelanotic melanoma represents between 1.8% and 8.1% of melano-mas. Due to the lack of pigment, diagnostic errors tend to occur delaying thetreatment and reducing the survival of these patients.

Case report: A 71-year-old woman with no personal or family history, consultedbecause of the appearance of a pink-colored lesion on the left heel, an asymptomaticlesion with progressive growth over last 2 years. Examination revealed a skin lesionof 2 to 3 cm in diameter, with sharp borders, strong consistency, nodular and pinkcolored. Other satellite bluish lesion was also observed next to the previous one.With the suspicion of basal cell carcinoma versus Kaposi sarcoma, surgery isdecided. The anatomicepathologic study concludes that it is an amelanoticmelanoma in vertical growth phase, Breslow 3, 7 mm and Clark level IV.Following this finding, the patient is referred to the Melanoma Unit for reexcisionwith 2 cm safety margins and to perform the sentinel node technique.

Conclusion: We report a case of amelanotic melanoma, a clinical rare variant ofmelanoma. The definition of amelanotic melanoma is clinicopathologic, defined asan injury where melanin is absent or very weak, implying that clinically the tumorlacks pigment. Its diagnosis presents difficulties that often lead to delays that affecttreatment and prognosis, so that both dermatologists and primary care physiciansshould have a high index of suspicion for early diagnosis or timely referral.

cial support: None identified.

Commer

13

NONMELANOMA SKIN CANCER

P6455A case of dermatofibrosarcoma protuberans

I. Lym Chan, MD, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil;Mariana Campos Souza Menezes, MD, Federal University of Rio de Janeiro, Rio deJaneiro, Brazil; Stella Velloso Ramos e Silva, MD, Federal University of Rio deJaneiro, Rio de Janeiro, Brazil; Sueli Coelho da Silva Carneiro, MD, FederalUniversity of Rio de Janeiro, Rio de Janeiro, Brazil; Tullia Cuzzi, MD, FederalUniversity of Rio de Janeiro, Rio de Janeiro, Brazil

Dermatofibrosarcoma protuberans (DFSP) consists of a rare fibrohistiocytic neopla-sia with intermediate to low grade malignancy and has an annual incidence of 0.8-4.5/million. The standard treatment is wide local excision. Some therapeuticalternatives are described, such as the use of imatinib mesylate. Herein, we reporta case of an exuberant DFSP. A 41-year-old man presented with a mass that had beengrowing for over 2 years on the back. He underwent surgery to remove a similarlesion from the same site 2 months before the emergence of the current disease. Atthat time, histopathology was not performed. Clinical examination revealed thetumor to be exophytic, lobulated, brown at the periphery and lighter in the center,with telangiectasia, as well as pain to the touch in right lower paravertebral region.Histopathology was consistent with DFSP. Metastases were excluded and he wasreferred for surgical resection and adjuvant radiotherapy. DFSP is a cutaneoussarcoma that accounts for 0.1% of all malignancies and 1% of all soft tissue sarcomas.It has a slow growth, low metastatic potential and high recurrence rate due to itsability to infiltrate subcutaneous tissue, fascia and underlying muscle. Clinically, itusually presents as an asymptomatic multinodular reddish-blue or brown plaque,more common on the trunk and extremities. DFSP is most commonly seen amongthose in their second to fifth decade, with slight male predominance.Histopathology shows uniform spindle cells containing elongated nuclei arrangedin a predominantly storiform pattern. The general immunostaining pattern is CD34positive and factor XIIIa negative. It is also negative for other markers such as S-100,HMB45, desmin and actin. Over 90% of cases present rearrangement of chromo-somes 17 and 22, resulting in continuous activation of tyrosine kinase, promotingDFSP cell growth. Imatinib mesylate is a potent tyrosine kinase inhibitor approved totreat adults with unresectable, recurrent, and/or metastatic disease, with 65%response. The standard treatment to local disease is radical, local excision with 2-3cm wide margins and three-dimensional resection that includes skin, subcutaneoustissue and the underlying fascia. Late local recurrence of the tumor is up to 24% to90%. Mohs micrographic surgery emerges as treatment of choice. Radiotherapy is anadjuvant therapy after wide surgical excision or in inoperable macroscopic disease,reducing the risk of local recurrence.

cial support: None identified.

Commer

P6486A case of multiple actinic keratosis, Bowen disease, squamous cell carci-noma, basal cell carcinoma after PUVA therapy in a psoriasis patient

Kyu Uang Whang, MD, PhD, Soonchunhyang University Hospital, Seoul, SouthKorea; Han Eul Lee, MD, Soonchunhyang University Hospital, Cheonan,Cheonan, Chungcheongnam-do, South Korea; Ji Hoon Sim, MD,Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, SouthKorea; Sung Yul Lee, MD, PhD, Soonchunhyang University Hospital, Cheonan,Cheonan, Chungcheongnam-do, South Korea; Ye Seul Kim, MD, SoonchunhyangUniversity Hospital, Bucheon, Bucheon, Gyenggi-do, South Korea; You In Bae,MD, Soonchunhyang University Hospital, Bucheon, Bucheon, Gyenggi-do, SouthKorea; Young Lip Park, MD, PhD, Soonchunhyang University Hospital, Bucheon,Bucheon, Gyenggi-do, South Korea

Systemic photochemotherapy (PUVA) has beenwidely used as effective treatment ofpsoriasis, vitiligo, cutaneous T-cell lymphoma, and other skin diseases. However, theuse of PUVA has been restricted when it comes to a long-term treatment as it maycause premalignant lesion such as actinic keratosis and skin cancers includingsquamous cell carcinoma, basal cell carcinoma, and malignant melanoma. A recentreport showed that squamous cell carcinoma is proportionate to the cumulativedose, especially when treated with PUVA more than 150 times it shows a significantincrease of various skin disorders , and was not decreased over 15 years’ follow-upsince the treatment. Mutations of the p53 gene in malignant tumors after the PUVAtreatment have been reported, and a study on its mechanism of carcinogenesis isunderway. A 64 - year - old female who had been treated with PUVA for chronicplaque psoriasis, and admitted with exudative erythema of right upper arm 17 yearspast termination of treatment. The patient received 332 times of PUVA therapy for40months about 20 years ago and her past history showed that she had frequent sunexposure. Skin biopsies at the patient’s right upper arm, trunk, abdomen, andbilateral thigh were performed, and DNA sequence analysis for exon 5, 6, 7, 8 onsome of these samples was went through to examine p53 gene mutation. Actinickeratosis, squamous cell carcinoma, basal cell carcinoma and Bowen disease werediagnosed from various biopsied sites. p53 sequence analysis on the abdominallesions, diagnosed as actinic keratosis, confirmed TAT / GAT mutation of exon 6codon 205. All lesions were completely resected and have been followed upregularly. Here we report the case of multiple actinic keratosis, Bowen disease,squamous cell carcinoma, basal cell carcinoma after PUVA therapy in a psoriasispatient as it is very rare in East Asian population.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB155