- Email: [email protected]
Abrikossoff’s tumor: an atypical presentation
NONMELANOMA SKIN CANCER 2408 A 13-year retrospective study of basal cell carcinoma in a Canadian population: A comparison between anatomical location and histopathologic subtypes Jason Rivers, MD, Department of Dermatology & Skin Science, University of British Columbia & Pacific Dermaesthetics, Vancouver, British Columbia, Canada; Bhavik Mistry, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Tawny Hung, MD, DynalifeDx, University of Alberta, Departments of Pathology and Dermatology, University of Alberta, Edmonton, Alberta, Canada; Kateryna Vostretsova, MD, Faculty of Medicine, University of British Columbia, Vancouver, British Columbia, Canada; Nisha Mistry, MD, Department of Medicine (Dermatology), University of Toronto, Mississauga, Ontario, Canada Background: It is unknown whether the histologic subtypes of basal cell carcinoma (BCC) arise from a common progenitor cell or whether other factors play a role in their pathogenesis. Although BCC is the most common type of skin cancer, there are no Canadian studies to document the histologic variants of this tumor and their relationship with age, gender and body site. Objective: To investigate the relationship between the different BCC histopathologic subtypes and anatomic distribution of the BCC in a Canadian population. Methods: Between 1993 and 2005, the charts of all patients diagnosed with BCC in a single private dermatology practice in Vancouver, Canada, were retrospectively reviewed. Only the first two histologically confirmed tumors were collected from each patient. Results: From a total of 418 patients with 1005 tumors, 557 qualifying BCCs were identified; 418 were a first BCC diagnosis. Overall, nodular BCCs accounted for 58% of all tumors and 66% were situated on the head/neck (OR ¼ 3.0, 95% CI ¼ 2.1-4.3, P \.0001) and in older people (OR ¼ 1.6, 95% CI ¼ 1.1-2.1, P ¼ .02). Superficial BCCs were often localized to the trunk (OR ¼ 3.2, 95% CI ¼ 2.1-4.9, P \.0001) and almost half were evident in those between the ages of 40-59 years (OR ¼ 1.8, 95% CI ¼ 1.2-2.7, P ¼ .004). Infiltrative and mixed BCCs appeared predominantly on the head and neck (OR ¼ 2.9, 95% CI ¼ 1.6-5.2, P \.0001). Nodular (OR ¼ 3.1, 95% CI ¼ 2.1-4.5, P \.0001) and mixed BCCs (OR ¼ 6.3, 95% CI ¼ 1.5-26.9, P ¼ .004) were more common in men. Infiltrative (OR ¼ 2.4, 95% CI ¼ 1.5-4.1, P ¼.0003) and superficial BCCs were more common in women (OR ¼ 3.7, 95% CI ¼ 2.5-5.7, P \ .0001). Conclusion: Our results show a preference of at least some BCC subtypes for certain anatomical locations and gender. This suggests possible differences in the pathogenesis of this malignancy. Commercial support: None identified.
3087 A dose finding trial with a novel ingenol derivative (LEO 43204) for field treatment of actinic keratosis on the scalp Jonathan Weiss, Gwinnett Clinical Research Center, Inc, Snellville, GA, United States; Martina Ulrich, Collegium Medicum Berlin, Berlin, Germany; Michael Bukhalo, Altman Dermatology Associates, Arlington Heights, IL, United States; Marie Louise Østerdal, LEO Pharma, Ballerup, Denmark; Astrid H Petersen, LEO Pharma, Ballerup, Denmark; C. William Hanke, Laser & Skin Surgery Center of Indiana, Carmel, IN, United States Introduction: LEO 43204 is a novel ingenol derivative developed for improved chemical and biologic properties compared to ingenol mebutate. This phase 1/2 trial investigated the efficacy and safety of LEO 43204 gel in patients with actinic keratosis (AK) on the scalp. Methods: This was a randomized, double-blind, parallel-group, vehicle-controlled 8week dose-finding trial investigating efficacy and safety of 0.037% and 0.05% LEO 43204 gel as field treatment once daily for 2 consecutive days on the balding scalp (25-250 cm2) with 5-20 clinically typical AKs in the treatment area. The 0.05% dose was selected based on the outcome of the dose escalation part of the trial. Six individual components of local skin responses (LSRs) were assessed on a scale from 0 to 4, yielding a max composite score of 24. Efficacy was assessed by AK count on days 29 and 57; LSRs and adverse events were assessed on days 1, 3, 8, 15, 29, and 57. Patients completed a Treatment Satisfaction Questionnaire (TSQM) on day 57. Results: A total of 163 patients were randomized and received treatment. In each treatment group at least 96% of patients completed all treatments. The median age was 72 years; all were men, white, and had Fitzpatrick skin type I-III. The median history of AK was 9 years. At baseline the median number of AKs in the treatment area was 13. For both doses the reduction in AK count at week 8 compared to baseline was significantly greater than with vehicle (73% (0.037%); 79% (0.05%) vs 13% (vehicle); P \.001) and most (91% to 97%) of this effect was observed by week 4. For both active doses the composite LSR score peaked at day 3, rapidly declined thereafter and were almost back to baseline at 2 weeks. The peak composite LSR score for the active treatments was higher than for vehicle (8.6; 8.7 vs 1.5). Both active treatments were well-tolerated with the most common adverse drug reactions being application-site pain (including burning) (48%; 57% vs 6%) and applicationsite pruritus (25%; 27% vs 3%). There were no treatment-related serious adverse events. Global treatment satisfaction score was high for both active treatments and higher than vehicle (75%; 76% vs 36%; P \.001). Conclusion: Both doses of LEO 43204 gel were effective as field treatment of AK on the balding scalp and statistically significantly superior to vehicle, were welltolerated based on the adverse event profile and LSRs, and were associated with high global treatment satisfaction. Supported by LEO Pharma A/S.
3909
2650
A case of metastatic Merkel cell carcinoma with lymphoepithelioma like features negative for cytokeratin 20 Stefanie Grewe, University of South Florida Morsani College of Medicine, Tampa, FL, United States; Brooke Baldwin, MD, James A. Haley VA Hospital, Tampa, FL, United States Merkel cell carcinoma (MCC), also known as neuroendocrine carcinoma of the skin, is an aggressive cutaneous neoplasm that has a poor prognosis with a high metastasis and recurrence rate. Merkel cell carcinoma arises most often on sun-exposed areas in fairskinned individuals over age 50. It usually appears as a firm, painless, violaceous lesion on the head and neck region. Histopathologically, MCC usually appears as a dermal tumor nodule, which frequently extends into the subcutaneous fatty tissue. The tumor cells are small blue cells with basophilic nuclei and minimal cytoplasm. Mitoses are frequent and the apoptosis index is high. The ‘‘small round blue cell’’ histologic pattern of MCC must be differentiated from several other tumors, such as small-cell lung carcinoma, carcinoid tumor, malignant lymphoma, and small-cell melanoma. Therefore immunohistochemical staining is required. One of the hallmarks of Merkel cell carcinoma is positivity for cytokeratin 20 in a perinuclear dot like pattern. Here we present a rare case of an invasive poorly differentiated carcinoma with neuroendocrine differentiation with two rare histopathologic features—lymphoepithelioma like differentiation and cytokeratin 20 negativity. Weedon calls the lymphoepithelioma like differentiation pattern of MCC ‘‘exceedingly rare.’’ While poorly differentiated carcinomas can lose CK 20 positivity, 95% of Merkel cell carcinomas express positivity for CK20. In our case a 91-year-old white male, with an extensive history of radiation treatments, presented in clinic for a rapidly enlarging post auricular mass measuring 6 x 4 cm that he states tripled in size over two months after originally presenting as a nonhealing ulcer. A punch biopsy revealed the presence of a carcinoma infiltrating the dermis with trabeculae of neoplastic cells also forming larger nests and syncytia. A strong lymphoid response and basaloid appearing areas were noted on dermatopathology. The carcinoma expressed the neuroendocrine markers synaptophysin and chromogranin. Notably pathology was negative for Ber-Ep4, CK20, and TTF-1. A PET scan and CT head/neck revealed metastatic involvement of the parotid lymph nodes. No lesions were noted in the lungs thus ruling out metastatic small cell neuroendocrine carcinoma of the lung. Wide local excision was performed along with a superficial parotidectomy with facial nerve preservation. One out of two intraparotid lymph nodes excised was positive for poorly differentiated carcinoma. A wide cervical facial advancement flap repaired the defect after frozen section pathologic examination revealed clear margins. Merkel cell carcinoma is a rare and deadly tumor and this case is unique given its two unusual histopathologic findings. We present this case with corresponding clinical and histopathology images to remind clinicians and pathologists that Merkel cell carcinoma can have unusual presentations under the microscope. We highlight the importance of thorough histopathologic investigation as well as thorough clinical workup with PET/CT to rule out metastasis of other neuroendocrine tumors such as small cell lung cancer.
Abrikossoff’s tumor: an atypical presentation Estela Garcıa Peris, MD, Hospital Universitario de Canarias, San Crist obal de La lvarez, MD, Hospital Universitario de Canarias, Laguna, Spain; Irene Latour A Spain; Cristina Vazquez Rodrıguez, MD, Hospital Universitario de Canarias, Spain; Marıa Arteaga Henrıquez, MD, Hospital Universitario de Canarias, Spain; Rosa Rodrıguez Rodrıguez, MD, PhD, Hospital Universitario de Canarias, Spain; Marta Garcıa Bustınduy, MD, PhD, Hospital Universitario de Canarias, Spain; Rosalba Sanchez Gonzalez, MD, PhD, Hospital Universitario de Canarias, Spain; Francisco Guimera Martın-Neda, MD, PhD, Hospital Universitario de Canarias, Spain Introduction: Abrikossoff’s tumor or granular cell tumor (GCT) is usually benign. It might involve multiple anatomical sites, most frequently the head and neck. In the skin it has an unspecific clinical appearance, leading to a wide range of differential diagnoses.
Commercial support: None identified.
Commercial support: None identified.
AB192
J AM ACAD DERMATOL
Case report: A 55-year-old woman was referred to us with a hyperesthesic lesion, not painful, that appeared one year before in the lower abdomen after a strong trauma in this area. It was a brownish nummular tumor lesion, infiltrating but not adhered to deep planes, well defined, measuring 3 x 2 cm. With the clinical suspicion of atypical dermatofibroma, we made surgical excision of the lesion. Histopathology revealed a nonencapsulated tumor in the dermis and subcutaneous tissue disposed in sheets, nests and trabeculae organized irregularly. It lied between collagen bundles and surrounded skin appendages. Tumor cells were large and polyhedral with abundant eosinophilic granular cytoplasm surrounding a hyperchromatic nucleus. Cellular atypia, mitosis, vascular invasion and necrosis were not observed. Inmunohistochemical (IH) study showed positive S-100 and CD68. Hence, a histologic and IH diagnosis of a GCT was made. Discussion: Abrikossoff’s tumor is a rare benign neoplasm that might affect any part of the body. It usually appears from the second to the sixth decade of life and it is twice as common in women as in men. It’s relatively more common among black people. This tumor usually presents as a slow growing, solitary and painless nodular mass, located mainly in head and neck areas, the tongue being the most frequent location. It histogenesis is disputed, but the neural origin is the most defended. Histopathology of the tumor shows polygonal cells arranged in sheets with granular eosinophilic cytoplasm and small nuclei, that infiltrates the dermis and subcutaneous tissue. ‘‘Pustulo-ovoid bodies of Milian’’ have been described as a constant histological finding. IH studies have shown the cells to express S-100 protein and CD-68, among others. GCT metastasis are rare (1-3%). Tumor size [5 cm, rapid growth, vascular invasion, necrosis and cell spindling are important indicators of malignancy. The treatment of choice is local surgical excision with a wide margin. Conclusion: We present a case of cutaneous GCT, benign, localized in lower abdomen (an infrequent location), presenting as a histiocytoma-like lesion that appeared after a strong trauma in this anatomic area.
MAY 2016
3087 A dose finding trial with a novel ingenol derivative (LEO 43204) for field treatment of actinic keratosis on the scalp Jonathan Weiss, Gwinnett Clinical Research Center, Inc, Snellville, GA, United States; Martina Ulrich, Collegium Medicum Berlin, Berlin, Germany; Michael Bukhalo, Altman Dermatology Associates, Arlington Heights, IL, United States; Marie Louise Østerdal, LEO Pharma, Ballerup, Denmark; Astrid H Petersen, LEO Pharma, Ballerup, Denmark; C. William Hanke, Laser & Skin Surgery Center of Indiana, Carmel, IN, United States Introduction: LEO 43204 is a novel ingenol derivative developed for improved chemical and biologic properties compared to ingenol mebutate. This phase 1/2 trial investigated the efficacy and safety of LEO 43204 gel in patients with actinic keratosis (AK) on the scalp. Methods: This was a randomized, double-blind, parallel-group, vehicle-controlled 8week dose-finding trial investigating efficacy and safety of 0.037% and 0.05% LEO 43204 gel as field treatment once daily for 2 consecutive days on the balding scalp (25-250 cm2) with 5-20 clinically typical AKs in the treatment area. The 0.05% dose was selected based on the outcome of the dose escalation part of the trial. Six individual components of local skin responses (LSRs) were assessed on a scale from 0 to 4, yielding a max composite score of 24. Efficacy was assessed by AK count on days 29 and 57; LSRs and adverse events were assessed on days 1, 3, 8, 15, 29, and 57. Patients completed a Treatment Satisfaction Questionnaire (TSQM) on day 57. Results: A total of 163 patients were randomized and received treatment. In each treatment group at least 96% of patients completed all treatments. The median age was 72 years; all were men, white, and had Fitzpatrick skin type I-III. The median history of AK was 9 years. At baseline the median number of AKs in the treatment area was 13. For both doses the reduction in AK count at week 8 compared to baseline was significantly greater than with vehicle (73% (0.037%); 79% (0.05%) vs 13% (vehicle); P \.001) and most (91% to 97%) of this effect was observed by week 4. For both active doses the composite LSR score peaked at day 3, rapidly declined thereafter and were almost back to baseline at 2 weeks. The peak composite LSR score for the active treatments was higher than for vehicle (8.6; 8.7 vs 1.5). Both active treatments were well-tolerated with the most common adverse drug reactions being application-site pain (including burning) (48%; 57% vs 6%) and applicationsite pruritus (25%; 27% vs 3%). There were no treatment-related serious adverse events. Global treatment satisfaction score was high for both active treatments and higher than vehicle (75%; 76% vs 36%; P \.001). Conclusion: Both doses of LEO 43204 gel were effective as field treatment of AK on the balding scalp and statistically significantly superior to vehicle, were welltolerated based on the adverse event profile and LSRs, and were associated with high global treatment satisfaction. Supported by LEO Pharma A/S.
3909
2650
A case of metastatic Merkel cell carcinoma with lymphoepithelioma like features negative for cytokeratin 20 Stefanie Grewe, University of South Florida Morsani College of Medicine, Tampa, FL, United States; Brooke Baldwin, MD, James A. Haley VA Hospital, Tampa, FL, United States Merkel cell carcinoma (MCC), also known as neuroendocrine carcinoma of the skin, is an aggressive cutaneous neoplasm that has a poor prognosis with a high metastasis and recurrence rate. Merkel cell carcinoma arises most often on sun-exposed areas in fairskinned individuals over age 50. It usually appears as a firm, painless, violaceous lesion on the head and neck region. Histopathologically, MCC usually appears as a dermal tumor nodule, which frequently extends into the subcutaneous fatty tissue. The tumor cells are small blue cells with basophilic nuclei and minimal cytoplasm. Mitoses are frequent and the apoptosis index is high. The ‘‘small round blue cell’’ histologic pattern of MCC must be differentiated from several other tumors, such as small-cell lung carcinoma, carcinoid tumor, malignant lymphoma, and small-cell melanoma. Therefore immunohistochemical staining is required. One of the hallmarks of Merkel cell carcinoma is positivity for cytokeratin 20 in a perinuclear dot like pattern. Here we present a rare case of an invasive poorly differentiated carcinoma with neuroendocrine differentiation with two rare histopathologic features—lymphoepithelioma like differentiation and cytokeratin 20 negativity. Weedon calls the lymphoepithelioma like differentiation pattern of MCC ‘‘exceedingly rare.’’ While poorly differentiated carcinomas can lose CK 20 positivity, 95% of Merkel cell carcinomas express positivity for CK20. In our case a 91-year-old white male, with an extensive history of radiation treatments, presented in clinic for a rapidly enlarging post auricular mass measuring 6 x 4 cm that he states tripled in size over two months after originally presenting as a nonhealing ulcer. A punch biopsy revealed the presence of a carcinoma infiltrating the dermis with trabeculae of neoplastic cells also forming larger nests and syncytia. A strong lymphoid response and basaloid appearing areas were noted on dermatopathology. The carcinoma expressed the neuroendocrine markers synaptophysin and chromogranin. Notably pathology was negative for Ber-Ep4, CK20, and TTF-1. A PET scan and CT head/neck revealed metastatic involvement of the parotid lymph nodes. No lesions were noted in the lungs thus ruling out metastatic small cell neuroendocrine carcinoma of the lung. Wide local excision was performed along with a superficial parotidectomy with facial nerve preservation. One out of two intraparotid lymph nodes excised was positive for poorly differentiated carcinoma. A wide cervical facial advancement flap repaired the defect after frozen section pathologic examination revealed clear margins. Merkel cell carcinoma is a rare and deadly tumor and this case is unique given its two unusual histopathologic findings. We present this case with corresponding clinical and histopathology images to remind clinicians and pathologists that Merkel cell carcinoma can have unusual presentations under the microscope. We highlight the importance of thorough histopathologic investigation as well as thorough clinical workup with PET/CT to rule out metastasis of other neuroendocrine tumors such as small cell lung cancer.
Abrikossoff’s tumor: an atypical presentation Estela Garcıa Peris, MD, Hospital Universitario de Canarias, San Crist obal de La lvarez, MD, Hospital Universitario de Canarias, Laguna, Spain; Irene Latour A Spain; Cristina Vazquez Rodrıguez, MD, Hospital Universitario de Canarias, Spain; Marıa Arteaga Henrıquez, MD, Hospital Universitario de Canarias, Spain; Rosa Rodrıguez Rodrıguez, MD, PhD, Hospital Universitario de Canarias, Spain; Marta Garcıa Bustınduy, MD, PhD, Hospital Universitario de Canarias, Spain; Rosalba Sanchez Gonzalez, MD, PhD, Hospital Universitario de Canarias, Spain; Francisco Guimera Martın-Neda, MD, PhD, Hospital Universitario de Canarias, Spain Introduction: Abrikossoff’s tumor or granular cell tumor (GCT) is usually benign. It might involve multiple anatomical sites, most frequently the head and neck. In the skin it has an unspecific clinical appearance, leading to a wide range of differential diagnoses.
Commercial support: None identified.
Commercial support: None identified.
AB192
J AM ACAD DERMATOL
Case report: A 55-year-old woman was referred to us with a hyperesthesic lesion, not painful, that appeared one year before in the lower abdomen after a strong trauma in this area. It was a brownish nummular tumor lesion, infiltrating but not adhered to deep planes, well defined, measuring 3 x 2 cm. With the clinical suspicion of atypical dermatofibroma, we made surgical excision of the lesion. Histopathology revealed a nonencapsulated tumor in the dermis and subcutaneous tissue disposed in sheets, nests and trabeculae organized irregularly. It lied between collagen bundles and surrounded skin appendages. Tumor cells were large and polyhedral with abundant eosinophilic granular cytoplasm surrounding a hyperchromatic nucleus. Cellular atypia, mitosis, vascular invasion and necrosis were not observed. Inmunohistochemical (IH) study showed positive S-100 and CD68. Hence, a histologic and IH diagnosis of a GCT was made. Discussion: Abrikossoff’s tumor is a rare benign neoplasm that might affect any part of the body. It usually appears from the second to the sixth decade of life and it is twice as common in women as in men. It’s relatively more common among black people. This tumor usually presents as a slow growing, solitary and painless nodular mass, located mainly in head and neck areas, the tongue being the most frequent location. It histogenesis is disputed, but the neural origin is the most defended. Histopathology of the tumor shows polygonal cells arranged in sheets with granular eosinophilic cytoplasm and small nuclei, that infiltrates the dermis and subcutaneous tissue. ‘‘Pustulo-ovoid bodies of Milian’’ have been described as a constant histological finding. IH studies have shown the cells to express S-100 protein and CD-68, among others. GCT metastasis are rare (1-3%). Tumor size [5 cm, rapid growth, vascular invasion, necrosis and cell spindling are important indicators of malignancy. The treatment of choice is local surgical excision with a wide margin. Conclusion: We present a case of cutaneous GCT, benign, localized in lower abdomen (an infrequent location), presenting as a histiocytoma-like lesion that appeared after a strong trauma in this anatomic area.
MAY 2016