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Cutaneous malignant peripheral nerve sheath tumor: A clinical and histopathologic study of 17 cases
1178
991
Clinical and dermatoscopic evaluation and degree of dysplasia of actinic keratoses: Differences among skin cancer experts Maria Longo, MD, PhD, Department of Dermatology, Hospital Central de la Defensa G omez Ulla, Madrid, Spain; Salvador Arias-Santiago, MD, PhD, Department of Dermatology, Hospital Virgen de las Nieves, Granada, Spain; Elena de las Heras, MD, PhD, Department of Dermatology, Hospital Ram on y Cajal, Madrid, Spain; Montserrat Fernandez-Guarino, MD, PhD, Department of Dermatology, Hospital Central de la Cruz Roja, Madrid, Spain; Jose Luis L opezEstebaranz, MD, PhD, Department of Dermatology, Hospital Fundaci on Alcorc on, Madrid, Spain; Agustı Toll, MD, PhD, Department of Dermatology, Hospital del Mar, Barcelona, Spain; Santiago Vidal-Asensi, MD, Department of Dermatology, Hospital Central de la Defensa, Madrid, Spain; Carlos Serra-Guillen, MD, PhD, Department of Dermatology, Instituto Valenciano de Oncologıa, Valencia, Spain Objective: To test the capability of several skilled observers to predict the degree of dysplasia based on the assessment of clinical and dermatoscopic images of patients with histologically confirmed actinic keratoses (AK). Methods: A first set of 32 images of 12 patients and 16 AK lesions were evaluated by 7 dermatologists experienced on skin cancer diagnosis with the intention to be classified according to clinical features (macule, papule or plaque), grade (I, II or III) and suspected degree of dysplasia (mild, moderate or severe). Then, the 16 pairs of images (clinical image [CI] + dermatoscopic image [DI]) were also randomly presented. The agreement between each dermatologist’s evaluation of a CI and the histologic diagnosis (HD) was assessed through the Cohen’s kappa coefficient (which ranges between 0 to 1, where 0 means ‘‘by chance’’ and 1 a total agreement while negative values imply that agreement occur less often than predicted by chance alone). This statistic was also used to determine the interevaluator agreement. All comparisons were also made with the nonparametric Spearman test.
Clinical characteristics of mucosal basal cell carcinomas of the lip: A 7-year, single institution retrospective review Tiffany Loh, University of California, San Diego, San Diego, CA, United States; Ashley Rubin, MD, University of California, San Diego, San Diego, CA, United States; Shang Jiang, MD, University of California, San Diego, San Diego, CA, United States
Results: More than a third of lesions were pigmented AK and were classified as macules. Clinically, 75% of AKs were graded as type I. More than 60% of all AK were histologically confirmed to have moderate dysplasia. In all cases, no agreement between the expert evaluation of CI and the confirmed pathologic findings was observed. Agreement between dermatologists evaluation of CI and HD has shown the worst results (mean kappa: -0.226; range -0.422 to -0.43) with an improved reciprocity with the conjunct evaluation with CI+D (0.144; -0.289 to 0.022). Evaluation of DI alone has shown that some of the specialists have adequate levels of concordance with HD (0.086; -0.275 to 0.448). Spearman test results have shown the same trend with evaluation through dermatoscopy as the best correlated measure with HD. Comparison among evaluators demonstrated a high correlation between them. In general, the average coefficients were positive and tended to reach high scores but with important disparities in case of CI’s evaluation for which negative correlations of up to -0.423 were observed.
Background: Basal cell carcinoma (BCC) is the most common type of malignancy in the United States (US), comprising about 80% of nonmelanoma skin cancer (NMSC) cases and affecting approximately 2.8 million people annually. Most BCCs occur on cutaneous surfaces, especially in the head and neck areas, but rare instances of BCCs on the mucosal lip have also been documented. As BCCs are believed to originate from pilar structures, the appearance of these tumors on mucosal surfaces is surprising. Only a small number of mucosal BCC cases have been reported to date, and data on the specific clinical characteristics of these tumors is still very limited. Methods: We conducted a 7-year retrospective review of all Mohs micrographic surgery (MMS) cases presenting between May 2007 and May 2014 at UCSD Dermatologic and MMS Center to evaluate the incidence, risk factors, and clinical features of BCCs occurring on the mucosal lip. Results: Of 6654 cases presenting at UCSD MMS center between May 2007 and May 2014, 8 were BCCs of the mucosal lip. The average age at presentation was 76.9 years, and the male to female ratio was 5:3. 7 of the 8 patients had a prior history of skin cancer. All of the cases occurred in patients with Fitzpatrick skin types 2 or 3 (6 and 2 patients, respectively), and all patients were Caucasian. Out of all cases, 6 occurred on the upper lip, and 2 were on the lower lip. Tumor clearance was achieved after an average of 2.0 stages, and the mean final surgical margins were 7.8 3 13.4 mm. After MMS treatment, all but 2 cases showed involvement of the cutaneous skin surrounding the vermilion border. No recurrence was noted after a mean follow-up period of 22.1 months. Conclusions: Mucosal BCCs are rare, with an incidence of about 0.12% at UCSD Dermatologic and MMS Center, and appear to occur more frequently on the upper lip. Although tumors may initially be restricted to the mucosal lip, post-MMS involvement of the cutaneous skin surrounding the vermilion zone suggests epithelial migration and implantation as a possible mechanism for mucosal BCC development. In our patient population, mucosal BCCs of the lip occur more commonly in men, particularly those of Caucasian descent, and in individuals with a prior history of skin cancer. MMS was shown to be an effective treatment method with no recurrence after a mean follow-up period of 22.1 months. Commercial support: None identified.
Conclusions: Only dermatoscopy alone achieves a reasonable agreement with histologically confirmed diagnosis in skilled observers. Nor CI alone or combined with dermatoscopy seem to match histologic diagnosis. Data analysis Sponsored by LEO Pharma.
1750 994 Clinical characteristics of basal cell carcinomas of the dorsal hand: A 7-year, single institution retrospective review Tiffany Loh, University of California, San Diego, San Diego, CA, United States; Ashley Rubin, MD, University of California, San Diego, San Diego, CA, United States; Shang Jiang, MD, University of California, San Diego, San Diego, CA, United States Background: Basal cell carcinoma (BCC) is the most common type of malignancy in the United States (US), accounting for approximately 2.8 million new cancer diagnoses each year. It is well-established that excessive exposure to ultraviolet radiation (UVR) is the primary predisposing factor for BCC development, and the frequent occurrence of these tumors in the sun-exposed head and neck supports this model. However, surprisingly, BCCs occur very rarely on the dorsal hand, which is subject to intense sun exposure, and their infrequent presentation here suggests that other factors besides UVR may play a role in BCC pathogenesis. As there have only been a few documented cases of BCC on the dorsal hand, knowledge of the characteristics of these tumors is limited, and more data is needed in order to describe the risk factors, clinical presentation, and management of these malignancies. Methods: We conducted a 7-year retrospective review of all Mohs micrographic surgery (MMS) cases presenting between May 2007 and May 2014 at UCSD Dermatologic and MMS Center to evaluate the incidence, risk factors, and clinical features of BCCs occurring on the dorsal hand. Results: Of 6654 cases presenting within a 7-year period, 14 were BCCs that occurred on the dorsal hand. The average age at presentation was 65.1 years, and the male to female ratio was 6:1. All subjects were Caucasian, and 12 had a prior history of skin cancer. All cases with the exception of one occurred on the main dorsum of the hand (excluding fingers). The most common BCC subtype was nodular (4 cases, 28.6%). An average of 2.29 stages was required to achieve tumor clearance, and the mean final surgical margins were 6.85 3 8.86 mm. All subjects were treated with MMS, and there were no recurrences after a mean follow-up period of 31.7 months. Conclusions: BCCs of the dorsal hand occur very rarely, with an incidence of about 0.2% at UCSD Dermatologic and MMS Center. Most cases do not involve the fingers. Risk factors for BCC occurrence on the dorsal hand include being a male of Caucasian descent, as well as having a history of skin cancer. MMS was shown to be an effective treatment option with no recurrence after a mean follow-up period of 31.7 months. Commercial support: None identified.
AB182
J AM ACAD DERMATOL
Cutaneous malignant peripheral nerve sheath tumor: A clinical and histopathologic study of 17 cases Garrett Lowe, MD, Mayo Clinic, Rochester, MN, United States; Daniel Winchester, MD, Mayo Clinic, Rochester, MN, United States; Julia Lehman, MD, Mayo Clinic, Rochester, MN, United States; Clark Otley, MD, Mayo Clinic, Rochester, MN, United States Background: Superficial malignant peripheral nerve sheath tumor (MPNST) is the cutaneous variant of a rare biologically aggressive spindle cell sarcoma of neural origin. While high recurrence rates and metastases are well documented in the deep seated, subfascial counterpart of MPNST, less clinical and histopathologic information is available for the superficial form. The 28 documented cases of superficial MPNST suggest a more indolent course. Methods: After institutional review board approval, 17 patients diagnosed with cutaneous MPNST between 1980 and 2013 were reviewed using diagnostic criteria for superficial MPNST from Enzinger and Weiss’s Soft Tissue Tumors, 5th edition. Clinical parameters of patient age, sex, tumor location, size, recurrence, and metastasis, cause of death, associated tumors, and timelines for these factors were reviewed. Results: The mean age at diagnosis was 58.2 years with a male predominance (58.8%). Four of the patients had confirmed diagnosis of neurofibromatosis type 1. Seven cases occurred on the head/neck, 2 on the trunk and 4 on both the upper and lower extremities. Two tumors arose in fields of prior radiation. With regards to clinical outcomes, only one case resulted in metastasis (scalp). No deaths attributed to MPNST observed. Nine of the 17 cases recurred at a mean time of 2.3 years. When [3 years of follow-up data available, this recurrence rate rose to 69.3%. The mean and median diameters of tumor size were 5 cm. Where objective data for tumor size was noted, recurrence rates were 60% in tumors [5 cm and 20% for those \5 cm. When simple excision without attempted surgical margins was used (7 cases), 85.7% recurred. When this modality was coupled with radiation (6 cases), 50% recurred. When documentation of free margins was observed, no recurrences seen (2 cases with wide local excision, 1 case with Mohs micrographic surgery, and 1 case of amputation), but follow-up was limited to 3 years. Histopathologic grade (I-IV) was determined in 15 cases. Those classified as grade II or higher resulted in all but one case of recurrence. Conclusion: Malignant peripheral nerve sheath tumors frequently recur in both the superficial and deep forms. The low rate of metastasis and lack of disease-specific mortality in our case series, which is the largest to date on cutaneous MPNST, suggests superficial MPNST may carry a better prognosis than its deep seated counterpart. Commercial support: None identified.
MAY 2015
991
Clinical and dermatoscopic evaluation and degree of dysplasia of actinic keratoses: Differences among skin cancer experts Maria Longo, MD, PhD, Department of Dermatology, Hospital Central de la Defensa G omez Ulla, Madrid, Spain; Salvador Arias-Santiago, MD, PhD, Department of Dermatology, Hospital Virgen de las Nieves, Granada, Spain; Elena de las Heras, MD, PhD, Department of Dermatology, Hospital Ram on y Cajal, Madrid, Spain; Montserrat Fernandez-Guarino, MD, PhD, Department of Dermatology, Hospital Central de la Cruz Roja, Madrid, Spain; Jose Luis L opezEstebaranz, MD, PhD, Department of Dermatology, Hospital Fundaci on Alcorc on, Madrid, Spain; Agustı Toll, MD, PhD, Department of Dermatology, Hospital del Mar, Barcelona, Spain; Santiago Vidal-Asensi, MD, Department of Dermatology, Hospital Central de la Defensa, Madrid, Spain; Carlos Serra-Guillen, MD, PhD, Department of Dermatology, Instituto Valenciano de Oncologıa, Valencia, Spain Objective: To test the capability of several skilled observers to predict the degree of dysplasia based on the assessment of clinical and dermatoscopic images of patients with histologically confirmed actinic keratoses (AK). Methods: A first set of 32 images of 12 patients and 16 AK lesions were evaluated by 7 dermatologists experienced on skin cancer diagnosis with the intention to be classified according to clinical features (macule, papule or plaque), grade (I, II or III) and suspected degree of dysplasia (mild, moderate or severe). Then, the 16 pairs of images (clinical image [CI] + dermatoscopic image [DI]) were also randomly presented. The agreement between each dermatologist’s evaluation of a CI and the histologic diagnosis (HD) was assessed through the Cohen’s kappa coefficient (which ranges between 0 to 1, where 0 means ‘‘by chance’’ and 1 a total agreement while negative values imply that agreement occur less often than predicted by chance alone). This statistic was also used to determine the interevaluator agreement. All comparisons were also made with the nonparametric Spearman test.
Clinical characteristics of mucosal basal cell carcinomas of the lip: A 7-year, single institution retrospective review Tiffany Loh, University of California, San Diego, San Diego, CA, United States; Ashley Rubin, MD, University of California, San Diego, San Diego, CA, United States; Shang Jiang, MD, University of California, San Diego, San Diego, CA, United States
Results: More than a third of lesions were pigmented AK and were classified as macules. Clinically, 75% of AKs were graded as type I. More than 60% of all AK were histologically confirmed to have moderate dysplasia. In all cases, no agreement between the expert evaluation of CI and the confirmed pathologic findings was observed. Agreement between dermatologists evaluation of CI and HD has shown the worst results (mean kappa: -0.226; range -0.422 to -0.43) with an improved reciprocity with the conjunct evaluation with CI+D (0.144; -0.289 to 0.022). Evaluation of DI alone has shown that some of the specialists have adequate levels of concordance with HD (0.086; -0.275 to 0.448). Spearman test results have shown the same trend with evaluation through dermatoscopy as the best correlated measure with HD. Comparison among evaluators demonstrated a high correlation between them. In general, the average coefficients were positive and tended to reach high scores but with important disparities in case of CI’s evaluation for which negative correlations of up to -0.423 were observed.
Background: Basal cell carcinoma (BCC) is the most common type of malignancy in the United States (US), comprising about 80% of nonmelanoma skin cancer (NMSC) cases and affecting approximately 2.8 million people annually. Most BCCs occur on cutaneous surfaces, especially in the head and neck areas, but rare instances of BCCs on the mucosal lip have also been documented. As BCCs are believed to originate from pilar structures, the appearance of these tumors on mucosal surfaces is surprising. Only a small number of mucosal BCC cases have been reported to date, and data on the specific clinical characteristics of these tumors is still very limited. Methods: We conducted a 7-year retrospective review of all Mohs micrographic surgery (MMS) cases presenting between May 2007 and May 2014 at UCSD Dermatologic and MMS Center to evaluate the incidence, risk factors, and clinical features of BCCs occurring on the mucosal lip. Results: Of 6654 cases presenting at UCSD MMS center between May 2007 and May 2014, 8 were BCCs of the mucosal lip. The average age at presentation was 76.9 years, and the male to female ratio was 5:3. 7 of the 8 patients had a prior history of skin cancer. All of the cases occurred in patients with Fitzpatrick skin types 2 or 3 (6 and 2 patients, respectively), and all patients were Caucasian. Out of all cases, 6 occurred on the upper lip, and 2 were on the lower lip. Tumor clearance was achieved after an average of 2.0 stages, and the mean final surgical margins were 7.8 3 13.4 mm. After MMS treatment, all but 2 cases showed involvement of the cutaneous skin surrounding the vermilion border. No recurrence was noted after a mean follow-up period of 22.1 months. Conclusions: Mucosal BCCs are rare, with an incidence of about 0.12% at UCSD Dermatologic and MMS Center, and appear to occur more frequently on the upper lip. Although tumors may initially be restricted to the mucosal lip, post-MMS involvement of the cutaneous skin surrounding the vermilion zone suggests epithelial migration and implantation as a possible mechanism for mucosal BCC development. In our patient population, mucosal BCCs of the lip occur more commonly in men, particularly those of Caucasian descent, and in individuals with a prior history of skin cancer. MMS was shown to be an effective treatment method with no recurrence after a mean follow-up period of 22.1 months. Commercial support: None identified.
Conclusions: Only dermatoscopy alone achieves a reasonable agreement with histologically confirmed diagnosis in skilled observers. Nor CI alone or combined with dermatoscopy seem to match histologic diagnosis. Data analysis Sponsored by LEO Pharma.
1750 994 Clinical characteristics of basal cell carcinomas of the dorsal hand: A 7-year, single institution retrospective review Tiffany Loh, University of California, San Diego, San Diego, CA, United States; Ashley Rubin, MD, University of California, San Diego, San Diego, CA, United States; Shang Jiang, MD, University of California, San Diego, San Diego, CA, United States Background: Basal cell carcinoma (BCC) is the most common type of malignancy in the United States (US), accounting for approximately 2.8 million new cancer diagnoses each year. It is well-established that excessive exposure to ultraviolet radiation (UVR) is the primary predisposing factor for BCC development, and the frequent occurrence of these tumors in the sun-exposed head and neck supports this model. However, surprisingly, BCCs occur very rarely on the dorsal hand, which is subject to intense sun exposure, and their infrequent presentation here suggests that other factors besides UVR may play a role in BCC pathogenesis. As there have only been a few documented cases of BCC on the dorsal hand, knowledge of the characteristics of these tumors is limited, and more data is needed in order to describe the risk factors, clinical presentation, and management of these malignancies. Methods: We conducted a 7-year retrospective review of all Mohs micrographic surgery (MMS) cases presenting between May 2007 and May 2014 at UCSD Dermatologic and MMS Center to evaluate the incidence, risk factors, and clinical features of BCCs occurring on the dorsal hand. Results: Of 6654 cases presenting within a 7-year period, 14 were BCCs that occurred on the dorsal hand. The average age at presentation was 65.1 years, and the male to female ratio was 6:1. All subjects were Caucasian, and 12 had a prior history of skin cancer. All cases with the exception of one occurred on the main dorsum of the hand (excluding fingers). The most common BCC subtype was nodular (4 cases, 28.6%). An average of 2.29 stages was required to achieve tumor clearance, and the mean final surgical margins were 6.85 3 8.86 mm. All subjects were treated with MMS, and there were no recurrences after a mean follow-up period of 31.7 months. Conclusions: BCCs of the dorsal hand occur very rarely, with an incidence of about 0.2% at UCSD Dermatologic and MMS Center. Most cases do not involve the fingers. Risk factors for BCC occurrence on the dorsal hand include being a male of Caucasian descent, as well as having a history of skin cancer. MMS was shown to be an effective treatment option with no recurrence after a mean follow-up period of 31.7 months. Commercial support: None identified.
AB182
J AM ACAD DERMATOL
Cutaneous malignant peripheral nerve sheath tumor: A clinical and histopathologic study of 17 cases Garrett Lowe, MD, Mayo Clinic, Rochester, MN, United States; Daniel Winchester, MD, Mayo Clinic, Rochester, MN, United States; Julia Lehman, MD, Mayo Clinic, Rochester, MN, United States; Clark Otley, MD, Mayo Clinic, Rochester, MN, United States Background: Superficial malignant peripheral nerve sheath tumor (MPNST) is the cutaneous variant of a rare biologically aggressive spindle cell sarcoma of neural origin. While high recurrence rates and metastases are well documented in the deep seated, subfascial counterpart of MPNST, less clinical and histopathologic information is available for the superficial form. The 28 documented cases of superficial MPNST suggest a more indolent course. Methods: After institutional review board approval, 17 patients diagnosed with cutaneous MPNST between 1980 and 2013 were reviewed using diagnostic criteria for superficial MPNST from Enzinger and Weiss’s Soft Tissue Tumors, 5th edition. Clinical parameters of patient age, sex, tumor location, size, recurrence, and metastasis, cause of death, associated tumors, and timelines for these factors were reviewed. Results: The mean age at diagnosis was 58.2 years with a male predominance (58.8%). Four of the patients had confirmed diagnosis of neurofibromatosis type 1. Seven cases occurred on the head/neck, 2 on the trunk and 4 on both the upper and lower extremities. Two tumors arose in fields of prior radiation. With regards to clinical outcomes, only one case resulted in metastasis (scalp). No deaths attributed to MPNST observed. Nine of the 17 cases recurred at a mean time of 2.3 years. When [3 years of follow-up data available, this recurrence rate rose to 69.3%. The mean and median diameters of tumor size were 5 cm. Where objective data for tumor size was noted, recurrence rates were 60% in tumors [5 cm and 20% for those \5 cm. When simple excision without attempted surgical margins was used (7 cases), 85.7% recurred. When this modality was coupled with radiation (6 cases), 50% recurred. When documentation of free margins was observed, no recurrences seen (2 cases with wide local excision, 1 case with Mohs micrographic surgery, and 1 case of amputation), but follow-up was limited to 3 years. Histopathologic grade (I-IV) was determined in 15 cases. Those classified as grade II or higher resulted in all but one case of recurrence. Conclusion: Malignant peripheral nerve sheath tumors frequently recur in both the superficial and deep forms. The low rate of metastasis and lack of disease-specific mortality in our case series, which is the largest to date on cutaneous MPNST, suggests superficial MPNST may carry a better prognosis than its deep seated counterpart. Commercial support: None identified.
MAY 2015