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Metastatic hidradenocarcinoma on the scalp
P2917
P2919
Metastatic hidradenocarcinoma on the scalp Manuel Moragon, MD, MPH, Department of Dermatology, Hospital de San Juan, Alicante, Spain; Amparo Sevila, Hospital de San Juan, Alicante, Spain; Jose Onrubia, Hospital de San Juan, Alicante, Spain; Julia Miralles, Hospital de San Juan, Alicante, Spain; Remedios Alfonso, Hospital de San Juan, Alicante, Spain
Koebnerizing keratoacanthoma Jason Hadley, DDSc, CAN, CAN, University of UT, Salt Lake City, UT, United States; Michael Hadley, MD, University of UT, Salt Lake City, UT, United States; Payam Tristani-Firouzi, MD, University of UT, Salt Lake City, UT, United States Keratoacanthomas (KAs) are rapidly proliferating epidermal tumors felt to represent a subtype of squamous cell carcinoma (SCC) with a tendency to spontaneously resolve. Current management of KA varies, but surgical removal is generally felt to be the preferred treatment modality. The Ko¨ebner or isomorphic response refers to the appearance of dermatoses at sites of trauma. Koebnerization is a process not typically ascribed to KA; however, we herein report three cases of KA that repeatedly worsened from trauma caused by appropriate surgical measures to remove these lesions. After repeated surgical procedures created more burden of KA disease, we abandoned further surgical intervention and tried varying medical treatment options. Intralesional methotrexate, intralesional 5-fluorouracil, soriatane, and external beam radiation were all used. In two cases, the medical interventions cleared all KAs. However, all the interventions attempted in our third case failed, and the burden of KA progressed in spite of treatment. Eventually, our third case was diagnosed as having probable lung cancer by computed tomographic scan. The patient refused both lung biopsy and any medical intervention. She was referred to hospice, and died shortly thereafter. Autopsy was not performed, and no histopathologic diagnosis of the lung disease was ever obtained. We feel practitioners should be aware of this Koebnerizing phenomenon of KA so they may consider medical treatment options early that would avoid worsening of these lesions through surgical trauma.
Case report: A 42-year-old male was referred to our hospital for the evaluation of two painless nodules on his scalp. One of them was located in occipital and had been present for many years and recently had increased in size, reaching 2 cm. The other was a lymph node near the neck which had appeared several weeks before. The main lesions were excised and a biopsy was done in lymph node. Histologically, the lesion was a nodular proliferation composed of lobulate masses invading the dermis. Overall, the tumor was well circumscribed, although small nets infiltrated the surrounding collagen at the periphery. Some lobules showed a comedo pattern because of central necrosis. Cellular pleomorphism, irregular cell arrangement, mitotic figures, increased nuclear to cytoplasmic ratios, ductal proliferation, and areas with decapitation secretion were seen. A high proportion of tumor cells showed a vacuolated cytoplasm related to the presence of glycogen (clear cell). There were not areas with typical clear cell hidradenoma. The histologic figures in lymph node were similar to primary lesion. Immunohistochemical staining revealed immunoreactivity for CK 7, CAM 5.2, carcinoembryonic antigen, and epithelial membrane antigens Ki-67 and Her-2/neu, which showed a diffuse membranous staining. The diagnostic was apocrine hidradenocarcinoma, and the patient was treated with wide local excision of primary lesion and lymph node dissection, radiotherapy, and cisplatin. The patient is presently free of recurrence. Discussion: Malignant nodular hidradenoma is an infrequent, aggressive tumor. Most of them arise de novo, but in rare cases they may arise in preexisting nodular hidradenomas. Metastases have been reported in more than 60% of patients, and they appear first in regional lymph nodes. There is also a high rate of local recurrence, probably related with narrow excision margin. Some authors recommend wide surgical margins ([2 cm.) Other options of treatment are hormonal therapy and trastuzumab. The patient of this report was treated with wide local excision and regional lymph node dissection, followed of radiotherapy and cisplatin but we did not exclude the possibility of adding trastuzumab if it were necessary.
Commercial support: None identified.
Commercial support: None identified.
P2918 Isolated limb infusion with melphalan for the treatment of advanced nonmelanoma cutaneous malignancies Nicole McMahon, MD, Duke University Medical Center, Durham, NC, United States; Douglas Tyler, MD, Duke University Medical Center, Durham, NC, United States; Georgia Beasley, MD, Duke University Medical Center, Durham, NC, United States
P2920
Conclusions: In this small subset of patients with nonmelanoma cutaneous skin cancers that recurred in a multifocal pattern in the extremity, ILI was a safe and tolerable procedure. ILI with melphalan and D’actinomycin appeared most effective in the patient with advanced Merkel cell carcinoma.
Pseudo-bullous lymphangiomaelike Kaposi sarcoma: Case report Celia Posada, MD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Aranzazu Garcı´a-Cruz, MD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a., Pontevedra, Spain; Ignacio Garcı´a-Doval, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Jose´ Carlos De La Torre, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Manuel Cruces, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. Controversy persists whether the origin is of vascular or lymphatic endothelial cell. It is characterized by clinical and histologic polymorphism. The ‘‘lymphangiomalike’’ variant is very uncommon, accounting for \5% of all cases. We report the case of a 76-year-old HIV-negative female with a 4-year history of classic KS treated with cryotherapy who developed new bullous erythematosus asymptomatic lesions on her lower extremities. Biopsy revealed an angiomatous proliferation composed of dilated vascular channels lined by banal-appearing endothelia in the reticular dermis coexisting with focal areas of classic KS. Human herpes virus-8 (HHV-8) staining was positive. Diagnosis of lymphangioma-like KS was made and the patient was proposed for radiotherapy. The lymphangioma-like KS is a rare morphologic expression of KS. It can present as a typical-appearing KS but also as ‘‘bulla-like’’ lesions, which have been considered as a hallmark of this variant. The histologic pattern is characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. This pattern may resemble other vascular tumors, making diagnosis a challenge. It also suggests a lymphatic origin of KS. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.
Commercial support: None identified.
Commercial support: None identified.
Background: Isolated limb infusion (ILI) is a recently described treatment for in-transit recurrent melanoma of the extremity that is associated with complete response rates of 23% to 41% using the alkylating agent melphalan. The purpose of this study was to determine how effective regional infusion using melphalan together with D’actinomycin may be for other malignancies that appear in a multifocal cutaneous pattern on the extremities. Methods: ILI was performed circulating melphalan and D’actinomycin together for 30 minutes after limb temperatures reached 378C in patients with sarcoma (n ¼ 4), squamous cell carcinoma (SCC; n ¼ 1), and Merkel cell carcinoma (n ¼ 1). Of the sarcoma patients, two had malignant fibrohistiocytomas, one had epithelial cell sarcoma, and one had synovial cell sarcoma. Regional and systemic toxicity information was gathered prospectively and response was defined at 3 months according to RECIST criteria modified for cutaneous lesions. Results: The patient with Merkel cell carcinoma, who had a high burden of disease before treatment, had a complete response to therapy. Of the sarcoma patients, two experienced partial responses, one had stable disease, and one patient had progressive disease. The SCC patient tolerated the ILI well, but had progressive disease very shortly after the procedure. All six patients experienced regional toxicity typical to that seen in other limb infusion patients with erythema, swelling, pain, and nausea being among the most common adverse events experienced. In addition, elevation in creatinine phosphokinase was also observed in these patients including (n ¼ 4) grade IV elevations as defined by CTCAE version 3.0.
MARCH 2009
J AM ACAD DERMATOL
AB139
P2919
Metastatic hidradenocarcinoma on the scalp Manuel Moragon, MD, MPH, Department of Dermatology, Hospital de San Juan, Alicante, Spain; Amparo Sevila, Hospital de San Juan, Alicante, Spain; Jose Onrubia, Hospital de San Juan, Alicante, Spain; Julia Miralles, Hospital de San Juan, Alicante, Spain; Remedios Alfonso, Hospital de San Juan, Alicante, Spain
Koebnerizing keratoacanthoma Jason Hadley, DDSc, CAN, CAN, University of UT, Salt Lake City, UT, United States; Michael Hadley, MD, University of UT, Salt Lake City, UT, United States; Payam Tristani-Firouzi, MD, University of UT, Salt Lake City, UT, United States Keratoacanthomas (KAs) are rapidly proliferating epidermal tumors felt to represent a subtype of squamous cell carcinoma (SCC) with a tendency to spontaneously resolve. Current management of KA varies, but surgical removal is generally felt to be the preferred treatment modality. The Ko¨ebner or isomorphic response refers to the appearance of dermatoses at sites of trauma. Koebnerization is a process not typically ascribed to KA; however, we herein report three cases of KA that repeatedly worsened from trauma caused by appropriate surgical measures to remove these lesions. After repeated surgical procedures created more burden of KA disease, we abandoned further surgical intervention and tried varying medical treatment options. Intralesional methotrexate, intralesional 5-fluorouracil, soriatane, and external beam radiation were all used. In two cases, the medical interventions cleared all KAs. However, all the interventions attempted in our third case failed, and the burden of KA progressed in spite of treatment. Eventually, our third case was diagnosed as having probable lung cancer by computed tomographic scan. The patient refused both lung biopsy and any medical intervention. She was referred to hospice, and died shortly thereafter. Autopsy was not performed, and no histopathologic diagnosis of the lung disease was ever obtained. We feel practitioners should be aware of this Koebnerizing phenomenon of KA so they may consider medical treatment options early that would avoid worsening of these lesions through surgical trauma.
Case report: A 42-year-old male was referred to our hospital for the evaluation of two painless nodules on his scalp. One of them was located in occipital and had been present for many years and recently had increased in size, reaching 2 cm. The other was a lymph node near the neck which had appeared several weeks before. The main lesions were excised and a biopsy was done in lymph node. Histologically, the lesion was a nodular proliferation composed of lobulate masses invading the dermis. Overall, the tumor was well circumscribed, although small nets infiltrated the surrounding collagen at the periphery. Some lobules showed a comedo pattern because of central necrosis. Cellular pleomorphism, irregular cell arrangement, mitotic figures, increased nuclear to cytoplasmic ratios, ductal proliferation, and areas with decapitation secretion were seen. A high proportion of tumor cells showed a vacuolated cytoplasm related to the presence of glycogen (clear cell). There were not areas with typical clear cell hidradenoma. The histologic figures in lymph node were similar to primary lesion. Immunohistochemical staining revealed immunoreactivity for CK 7, CAM 5.2, carcinoembryonic antigen, and epithelial membrane antigens Ki-67 and Her-2/neu, which showed a diffuse membranous staining. The diagnostic was apocrine hidradenocarcinoma, and the patient was treated with wide local excision of primary lesion and lymph node dissection, radiotherapy, and cisplatin. The patient is presently free of recurrence. Discussion: Malignant nodular hidradenoma is an infrequent, aggressive tumor. Most of them arise de novo, but in rare cases they may arise in preexisting nodular hidradenomas. Metastases have been reported in more than 60% of patients, and they appear first in regional lymph nodes. There is also a high rate of local recurrence, probably related with narrow excision margin. Some authors recommend wide surgical margins ([2 cm.) Other options of treatment are hormonal therapy and trastuzumab. The patient of this report was treated with wide local excision and regional lymph node dissection, followed of radiotherapy and cisplatin but we did not exclude the possibility of adding trastuzumab if it were necessary.
Commercial support: None identified.
Commercial support: None identified.
P2918 Isolated limb infusion with melphalan for the treatment of advanced nonmelanoma cutaneous malignancies Nicole McMahon, MD, Duke University Medical Center, Durham, NC, United States; Douglas Tyler, MD, Duke University Medical Center, Durham, NC, United States; Georgia Beasley, MD, Duke University Medical Center, Durham, NC, United States
P2920
Conclusions: In this small subset of patients with nonmelanoma cutaneous skin cancers that recurred in a multifocal pattern in the extremity, ILI was a safe and tolerable procedure. ILI with melphalan and D’actinomycin appeared most effective in the patient with advanced Merkel cell carcinoma.
Pseudo-bullous lymphangiomaelike Kaposi sarcoma: Case report Celia Posada, MD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Aranzazu Garcı´a-Cruz, MD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a., Pontevedra, Spain; Ignacio Garcı´a-Doval, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Jose´ Carlos De La Torre, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain; Manuel Cruces, PhD, Complexo Hospitalario de Pontevedra. Servicio de Dermatologı´a, Pontevedra, Spain Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. Controversy persists whether the origin is of vascular or lymphatic endothelial cell. It is characterized by clinical and histologic polymorphism. The ‘‘lymphangiomalike’’ variant is very uncommon, accounting for \5% of all cases. We report the case of a 76-year-old HIV-negative female with a 4-year history of classic KS treated with cryotherapy who developed new bullous erythematosus asymptomatic lesions on her lower extremities. Biopsy revealed an angiomatous proliferation composed of dilated vascular channels lined by banal-appearing endothelia in the reticular dermis coexisting with focal areas of classic KS. Human herpes virus-8 (HHV-8) staining was positive. Diagnosis of lymphangioma-like KS was made and the patient was proposed for radiotherapy. The lymphangioma-like KS is a rare morphologic expression of KS. It can present as a typical-appearing KS but also as ‘‘bulla-like’’ lesions, which have been considered as a hallmark of this variant. The histologic pattern is characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. This pattern may resemble other vascular tumors, making diagnosis a challenge. It also suggests a lymphatic origin of KS. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.
Commercial support: None identified.
Commercial support: None identified.
Background: Isolated limb infusion (ILI) is a recently described treatment for in-transit recurrent melanoma of the extremity that is associated with complete response rates of 23% to 41% using the alkylating agent melphalan. The purpose of this study was to determine how effective regional infusion using melphalan together with D’actinomycin may be for other malignancies that appear in a multifocal cutaneous pattern on the extremities. Methods: ILI was performed circulating melphalan and D’actinomycin together for 30 minutes after limb temperatures reached 378C in patients with sarcoma (n ¼ 4), squamous cell carcinoma (SCC; n ¼ 1), and Merkel cell carcinoma (n ¼ 1). Of the sarcoma patients, two had malignant fibrohistiocytomas, one had epithelial cell sarcoma, and one had synovial cell sarcoma. Regional and systemic toxicity information was gathered prospectively and response was defined at 3 months according to RECIST criteria modified for cutaneous lesions. Results: The patient with Merkel cell carcinoma, who had a high burden of disease before treatment, had a complete response to therapy. Of the sarcoma patients, two experienced partial responses, one had stable disease, and one patient had progressive disease. The SCC patient tolerated the ILI well, but had progressive disease very shortly after the procedure. All six patients experienced regional toxicity typical to that seen in other limb infusion patients with erythema, swelling, pain, and nausea being among the most common adverse events experienced. In addition, elevation in creatinine phosphokinase was also observed in these patients including (n ¼ 4) grade IV elevations as defined by CTCAE version 3.0.
MARCH 2009
J AM ACAD DERMATOL
AB139