R are and common tumors in unusual presentations or settings are best managed when diagnosed early and accurately. As dermatologists, we provide a critical diagnostic and therapeutic role in the management of these rare or difficult tumors. The following cases are from patients seen in routine dermatology visits in Houston and from the consultation dermatology service at the MD Anderson Cancer Center. This quiz represents cases that may present to any dermatologist and asks secondary questions that would be critical in the proper management of a rare tumor or an aggressive form of a common tumor. Case #1 Diagnosis:______________________________________ 1. This patient is at risk for metastases from her tumor. T___ F___ 2. Surgical excision of this type of tumor without histologic margin control should aim to include a 4-mm clear margin around the visible tumor. T___ F___ 3. Indications for Mohs surgery include basal cell carcinomas (BCCs) with aggressive histologic patterns such as morpheaform or micronodular. T___ F___ 4. Imiquimod 5% cream (Aldara) has demonstrated efficacy in treating certain small BCCs. T___ F___ 5. Zinc chloride paste is an acceptable form of treatment for certain small, superficial forms of non-melanoma skin cancers. T___ F___ Case #2 Diagnosis:___________________________________________________ 1. Appropriate imaging studies for this patient do not include CT scans or Panorex films since these modalities would expose him to X-Ray radiation, which could exacerbate his condition. T___ F___ 2. This condition is transmitted in an X-linked pattern. T___ F___ 3. This patient is at risk for central nervous system tumors and jaw cysts. T___ F___ 4. Gene defects in this condition commonly involve the patched tumor suppressor gene or other activating mutations of the hedgehog pathway. T___ F___ 5. Hypertelorism is distinctly uncommon in these patients. T___ F___ Case #3 Diagnosis:__________________________________________________ 1. The rate of metastatic disease for patients with this tumor in this location is less than 10%. T___ F___ 2. Early circumcision significantly reduces the rate of the genital form of this malignancy in men. T___ F___ 3. Verrucous forms of this malignancy haven’t been associated with the human papillomavirus and are limited to the oral and genital mucosa. T___ F___ 4. BCCs typically outnumber squamous cell carcinomas (SCCs) in organ transplant patients with chronic immunosuppression. T___ F___ 5. Benign inflammatory dermatoses may predispose a patient to developing this tumor. T___ F___ Case #4 Diagnosis:__________________________________________________ 1. Histopathology of these lesions frequently shows cytologic atypia and resembles actinic keratoses (AKs). T___ F___ 2. A history of childhood atopic dermatitis treated with oral medicines is pertinent in this case. T___ F___ 3. A carpenter using only pressure-treated lumber is at risk for this condition. T___ F___ 4. This patient may be predisposed to bronchial and genitourinary carcinomas. T___ F___ 5. In patients with this condition, skin examination frequently reveals hyperpigmentation with discrete hypopigmented ovals (“raindrops”) in the inguinal folds and areolae. T___ F___ Case #5 Diagnosis:___________________________________________________ 1. Assuming there is no nodal or other metastatic disease, this patient has a stage III cancer. T___ F___ 2. Histologically, Breslow’s depth is more important than ulceration in determining prognosis. T___ F___ 3. Satellite metastases (within 2 cm of the primary lesion) in a patient with this disease ensure that the patient will be classified with at least stage III cancer. T___ F___ 4. Sentinel lymph node biopsies are indicated for patients with in situ forms of this malignancy. T___ F___ 5. Elective lymph node dissections are indicated for patients with in situ forms of this malignancy. T___ F___ Case #6 Diagnosis:_________________________________________________ 1. This condition has an in situ form that is not associated with an underlying adenocarcinoma. T___ F___ 2. This condition most frequently arises in association with either underlying adnexal carcinoma or an underlying carcinoma of the gastrointestinal or genitourinary tracts. T___ F___ 3. Young people are less likely to develop this condition. T___ F___ 4. This condition is most commonly found in the genital, anal and axillary regions (areas with a high density of apocrine glands). T___ F___ 5. Because of the histologic resemblance to melanoma, Mohs surgery is an unacceptable treatment modality. T___ F___ Case #7 Diagnosis:___________________________________________________ 1. These growths most commonly occur on the head and neck. T___ F___ 2. Metastatic disease is usually present by the time of diagnosis. T___ F___ 3. Metastatic disease is usually hematogenous rather than nodal. T___ F___ 4. Immunohistochemical staining of biopsy specimens will be factor XIIIa positive and CD34 negative. T___ F___ 5. Intralesional interferon is the treatment of choice for these lesions. T___ F___ Suggested Reading 1. Moy RL, Taheri DP, Ostad A. Practical Management of Skin Cancer, 1st ed. Lippincott, Williams, and Wilkins, 1999. 2. Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol 1987;123:340-4. 3. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, Bale AE, Bale SJ. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299-308. 4. Gailani MR, Bale AE. Acquired and inherited basal cell carcinomas and the patched gene. Adv Dermatol 1999;38:363. 5. Berg D, Otley CC. Skin cancer in organ transplant recipients: Epidemiology, pathogenesis, and management. J Am Acad Dermatol 2002;47:1-17. 6. Maloney ME. Arsenic in Dermatology. Dermatol Surg 1996;22:301-4. Pilla, L. Demystifying the new melanoma staging guidelines. Skin and Aging 2002; 10:7:38-42. 7. Kurzl RG. Paget’s disease. Semin Dermatol 1996;15:60-6.
Rare and Aggressive Skin Cancer TumorsA Self-Assessment Quiz
R are and common tumors in unusual presentations or settings are best managed when diagnosed early and accurately. As dermatologists, we provide a critical diagnostic and therapeutic role in the management of these rare or difficult tumors. The following cases are from patients seen in routine dermatology visits in Houston and from the consultation dermatology service at the MD Anderson Cancer Center. This quiz represents cases that may present to any dermatologist and asks secondary questions that would be critical in the proper management of a rare tumor or an aggressive form of a common tumor. Case #1 Diagnosis:______________________________________ 1. This patient is at risk for metastases from her tumor. T___ F___ 2. Surgical excision of this type of tumor without histologic margin control should aim to include a 4-mm clear margin around the visible tumor. T___ F___ 3. Indications for Mohs surgery include basal cell carcinomas (BCCs) with aggressive histologic patterns such as morpheaform or micronodular. T___ F___ 4. Imiquimod 5% cream (Aldara) has demonstrated efficacy in treating certain small BCCs. T___ F___ 5. Zinc chloride paste is an acceptable form of treatment for certain small, superficial forms of non-melanoma skin cancers. T___ F___ Case #2 Diagnosis:___________________________________________________ 1. Appropriate imaging studies for this patient do not include CT scans or Panorex films since these modalities would expose him to X-Ray radiation, which could exacerbate his condition. T___ F___ 2. This condition is transmitted in an X-linked pattern. T___ F___ 3. This patient is at risk for central nervous system tumors and jaw cysts. T___ F___ 4. Gene defects in this condition commonly involve the patched tumor suppressor gene or other activating mutations of the hedgehog pathway. T___ F___ 5. Hypertelorism is distinctly uncommon in these patients. T___ F___ Case #3 Diagnosis:__________________________________________________ 1. The rate of metastatic disease for patients with this tumor in this location is less than 10%. T___ F___ 2. Early circumcision significantly reduces the rate of the genital form of this malignancy in men. T___ F___ 3. Verrucous forms of this malignancy haven’t been associated with the human papillomavirus and are limited to the oral and genital mucosa. T___ F___ 4. BCCs typically outnumber squamous cell carcinomas (SCCs) in organ transplant patients with chronic immunosuppression. T___ F___ 5. Benign inflammatory dermatoses may predispose a patient to developing this tumor. T___ F___ Case #4 Diagnosis:__________________________________________________ 1. Histopathology of these lesions frequently shows cytologic atypia and resembles actinic keratoses (AKs). T___ F___ 2. A history of childhood atopic dermatitis treated with oral medicines is pertinent in this case. T___ F___ 3. A carpenter using only pressure-treated lumber is at risk for this condition. T___ F___ 4. This patient may be predisposed to bronchial and genitourinary carcinomas. T___ F___ 5. In patients with this condition, skin examination frequently reveals hyperpigmentation with discrete hypopigmented ovals (“raindrops”) in the inguinal folds and areolae. T___ F___ Case #5 Diagnosis:___________________________________________________ 1. Assuming there is no nodal or other metastatic disease, this patient has a stage III cancer. T___ F___ 2. Histologically, Breslow’s depth is more important than ulceration in determining prognosis. T___ F___ 3. Satellite metastases (within 2 cm of the primary lesion) in a patient with this disease ensure that the patient will be classified with at least stage III cancer. T___ F___ 4. Sentinel lymph node biopsies are indicated for patients with in situ forms of this malignancy. T___ F___ 5. Elective lymph node dissections are indicated for patients with in situ forms of this malignancy. T___ F___ Case #6 Diagnosis:_________________________________________________ 1. This condition has an in situ form that is not associated with an underlying adenocarcinoma. T___ F___ 2. This condition most frequently arises in association with either underlying adnexal carcinoma or an underlying carcinoma of the gastrointestinal or genitourinary tracts. T___ F___ 3. Young people are less likely to develop this condition. T___ F___ 4. This condition is most commonly found in the genital, anal and axillary regions (areas with a high density of apocrine glands). T___ F___ 5. Because of the histologic resemblance to melanoma, Mohs surgery is an unacceptable treatment modality. T___ F___ Case #7 Diagnosis:___________________________________________________ 1. These growths most commonly occur on the head and neck. T___ F___ 2. Metastatic disease is usually present by the time of diagnosis. T___ F___ 3. Metastatic disease is usually hematogenous rather than nodal. T___ F___ 4. Immunohistochemical staining of biopsy specimens will be factor XIIIa positive and CD34 negative. T___ F___ 5. Intralesional interferon is the treatment of choice for these lesions. T___ F___ Suggested Reading 1. Moy RL, Taheri DP, Ostad A. Practical Management of Skin Cancer, 1st ed. Lippincott, Williams, and Wilkins, 1999. 2. Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol 1987;123:340-4. 3. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, Bale AE, Bale SJ. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299-308. 4. Gailani MR, Bale AE. Acquired and inherited basal cell carcinomas and the patched gene. Adv Dermatol 1999;38:363. 5. Berg D, Otley CC. Skin cancer in organ transplant recipients: Epidemiology, pathogenesis, and management. J Am Acad Dermatol 2002;47:1-17. 6. Maloney ME. Arsenic in Dermatology. Dermatol Surg 1996;22:301-4. Pilla, L. Demystifying the new melanoma staging guidelines. Skin and Aging 2002; 10:7:38-42. 7. Kurzl RG. Paget’s disease. Semin Dermatol 1996;15:60-6.
R are and common tumors in unusual presentations or settings are best managed when diagnosed early and accurately. As dermatologists, we provide a critical diagnostic and therapeutic role in the management of these rare or difficult tumors. The following cases are from patients seen in routine dermatology visits in Houston and from the consultation dermatology service at the MD Anderson Cancer Center. This quiz represents cases that may present to any dermatologist and asks secondary questions that would be critical in the proper management of a rare tumor or an aggressive form of a common tumor. Case #1 Diagnosis:______________________________________ 1. This patient is at risk for metastases from her tumor. T___ F___ 2. Surgical excision of this type of tumor without histologic margin control should aim to include a 4-mm clear margin around the visible tumor. T___ F___ 3. Indications for Mohs surgery include basal cell carcinomas (BCCs) with aggressive histologic patterns such as morpheaform or micronodular. T___ F___ 4. Imiquimod 5% cream (Aldara) has demonstrated efficacy in treating certain small BCCs. T___ F___ 5. Zinc chloride paste is an acceptable form of treatment for certain small, superficial forms of non-melanoma skin cancers. T___ F___ Case #2 Diagnosis:___________________________________________________ 1. Appropriate imaging studies for this patient do not include CT scans or Panorex films since these modalities would expose him to X-Ray radiation, which could exacerbate his condition. T___ F___ 2. This condition is transmitted in an X-linked pattern. T___ F___ 3. This patient is at risk for central nervous system tumors and jaw cysts. T___ F___ 4. Gene defects in this condition commonly involve the patched tumor suppressor gene or other activating mutations of the hedgehog pathway. T___ F___ 5. Hypertelorism is distinctly uncommon in these patients. T___ F___ Case #3 Diagnosis:__________________________________________________ 1. The rate of metastatic disease for patients with this tumor in this location is less than 10%. T___ F___ 2. Early circumcision significantly reduces the rate of the genital form of this malignancy in men. T___ F___ 3. Verrucous forms of this malignancy haven’t been associated with the human papillomavirus and are limited to the oral and genital mucosa. T___ F___ 4. BCCs typically outnumber squamous cell carcinomas (SCCs) in organ transplant patients with chronic immunosuppression. T___ F___ 5. Benign inflammatory dermatoses may predispose a patient to developing this tumor. T___ F___ Case #4 Diagnosis:__________________________________________________ 1. Histopathology of these lesions frequently shows cytologic atypia and resembles actinic keratoses (AKs). T___ F___ 2. A history of childhood atopic dermatitis treated with oral medicines is pertinent in this case. T___ F___ 3. A carpenter using only pressure-treated lumber is at risk for this condition. T___ F___ 4. This patient may be predisposed to bronchial and genitourinary carcinomas. T___ F___ 5. In patients with this condition, skin examination frequently reveals hyperpigmentation with discrete hypopigmented ovals (“raindrops”) in the inguinal folds and areolae. T___ F___ Case #5 Diagnosis:___________________________________________________ 1. Assuming there is no nodal or other metastatic disease, this patient has a stage III cancer. T___ F___ 2. Histologically, Breslow’s depth is more important than ulceration in determining prognosis. T___ F___ 3. Satellite metastases (within 2 cm of the primary lesion) in a patient with this disease ensure that the patient will be classified with at least stage III cancer. T___ F___ 4. Sentinel lymph node biopsies are indicated for patients with in situ forms of this malignancy. T___ F___ 5. Elective lymph node dissections are indicated for patients with in situ forms of this malignancy. T___ F___ Case #6 Diagnosis:_________________________________________________ 1. This condition has an in situ form that is not associated with an underlying adenocarcinoma. T___ F___ 2. This condition most frequently arises in association with either underlying adnexal carcinoma or an underlying carcinoma of the gastrointestinal or genitourinary tracts. T___ F___ 3. Young people are less likely to develop this condition. T___ F___ 4. This condition is most commonly found in the genital, anal and axillary regions (areas with a high density of apocrine glands). T___ F___ 5. Because of the histologic resemblance to melanoma, Mohs surgery is an unacceptable treatment modality. T___ F___ Case #7 Diagnosis:___________________________________________________ 1. These growths most commonly occur on the head and neck. T___ F___ 2. Metastatic disease is usually present by the time of diagnosis. T___ F___ 3. Metastatic disease is usually hematogenous rather than nodal. T___ F___ 4. Immunohistochemical staining of biopsy specimens will be factor XIIIa positive and CD34 negative. T___ F___ 5. Intralesional interferon is the treatment of choice for these lesions. T___ F___ Suggested Reading 1. Moy RL, Taheri DP, Ostad A. Practical Management of Skin Cancer, 1st ed. Lippincott, Williams, and Wilkins, 1999. 2. Wolf DJ, Zitelli JA. Surgical margins for basal cell carcinoma. Arch Dermatol 1987;123:340-4. 3. Kimonis VE, Goldstein AM, Pastakia B, Yang ML, Kase R, DiGiovanna JJ, Bale AE, Bale SJ. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet 1997;69:299-308. 4. Gailani MR, Bale AE. Acquired and inherited basal cell carcinomas and the patched gene. Adv Dermatol 1999;38:363. 5. Berg D, Otley CC. Skin cancer in organ transplant recipients: Epidemiology, pathogenesis, and management. J Am Acad Dermatol 2002;47:1-17. 6. Maloney ME. Arsenic in Dermatology. Dermatol Surg 1996;22:301-4. Pilla, L. Demystifying the new melanoma staging guidelines. Skin and Aging 2002; 10:7:38-42. 7. Kurzl RG. Paget’s disease. Semin Dermatol 1996;15:60-6.
