δ-Aminolevulinic Acid and Blue Light Photodynamic Therapy for Treatment of Multiple Basal Cell Carcinomas in Two Patients with Nevoid Basal Cell Carcinoma Syndrome

BACKGROUND: Patients with nevoid basal cell carcinoma syndrome suffer from multiple basal cell carcinomas, requiring numerous surgical procedures that over time leave them with multiple disfiguring scars. Photodynamic therapy with delta-aminolevulinic acid using red light (approximately 630 nm) sources has been reported as effective in treatment of superficial and small nodular basal cell carcinomas. To our knowledge, the blue light source (417 nm peak irradiance) approved by the FDA for treatment of actinic keratoses has not been used for photodynamic therapy with delta-aminolevulinic acid of basal cell carcinoma. OBJECTIVE: We report treatment of two nevoid basal cell carcinoma syndrome patients, women aged 21 and 47, with 20%delta-aminolevulinic acid solution and 417-nm blue light source (irradiance 10 mW/cm(2)). METHODS: delta-Aminolevulinic acid was applied topically on lesions 1 to 5 hr before light treatment. Lesions were illuminated with 417+/-5-nm blue light for 1000 sec (10 J/cm(2)). Two consecutive treatments 1 week apart were administered as a therapeutic course. Each patient underwent two courses of photodynamic therapy with delta-aminolevulinic acid 2 to 4 months apart. The reported assessment was made 8 months after initial treatment. In most sessions the entire face, rather than visible basal cell carcinomas only, was treated. The treated basal cell carcinomas were clinically subdivided to superficial or nodular type guided by their morphologic features. A total of 9 superficial and 16 nodular basal cell carcinomas on the face and 27 superficial basal cell carcinomas on the lower extremities were treated. RESULTS: Complete clinical response was observed in 8 of 9 (89%) superficial basal cell carcinomas and 5 of 16 (31%) nodular basal cell carcinomas on the face and in 18 of 27 (67%) of superficial basal cell carcinomas on the lower extremities. The remaining 21 lesions showed partial clinical resolution. No new basal cell carcinomas were observed during the 8-month follow-up period in areas treated with a broad application technique. Resolution of the lesions was accompanied by an excellent cosmetic outcome and decreased prominence of old surgical scars in the more severely affected patient. Treatments were well tolerated, but associated with moderate to severe stinging during illumination. CONCLUSION: To our knowledge this is the first use of photodynamic therapy with delta-aminolevulinic acid with 417-nm blue light for treatment of multiple basal cell carcinomas in patients with nevoid basal cell carcinoma syndrome. Our clinical results demonstrate that the blue light reduces cutaneous tumor burden in such patients. Further studies are needed to confirm that broad-area photodynamic therapy with delta-aminolevulinic acid may eradicate subclinical tumors in nevoid basal cell carcinoma syndrome sufferers, as suggested by a strikingly decreased incidence of new basal cell carcinomas in our patients.     

Multiple Subclinical Syringomatous Proliferations Encountered during Mohs Surgery for Basal Cell Carcinoma

BACKGROUND: Examination of Mohs surgery frozen sections may lead to discovery of incidental benign neoplasms such as syringomas. OBJECTIVE: An unusual occurrence of numerous subclinical syringomatous proliferations discovered during Mohs surgery for a basal cell carcinoma that posed a diagnostic and management dilemma is reported. METHODS: Clinical records and histologic sections are examined. RESULT: Multiple syringomatous proliferations were noted around a basal cell carcinoma during Mohs surgery. A few foci were atypical appearing and focally extending into the deep dermis causing a diagnostic dilemma. All atypical syringomatous proliferations were excised. Evaluation of final Mohs margins by permanent sections and biopsies of normal-appearing facial skin showed multiple benign syringomas. CONCLUSION: Although solitary subclinical syringomas have been described in association with basal cell carcinomas, the occurrence of multiple syringomas and syringomatous proliferations has not been previously reported. Dermatologists should be aware of the existence of this phenomenon and consider permanent section evaluation to better determine nature of unusual incidental tumors identified in frozen sections during Mohs surgery.     

Full-Face Carbon Dioxide Laser Resurfacing in the Management of a Patient With the Nevoid Basal Cell Carcinoma Syndrome

BACKGROUND: Multiple surgical procedures are often a source of discomfort, pain, and disfigurement for patients with the nevoid basal cell carcinoma syndrome (NBCCS). OBJECTIVE: The patient with NBCCS had approximately 45 identifiable basal cell carcinomas on her face. Multiple Mohs surgeries were performed to remove the largest tumors. A tumor-free plane was not achieved in several lesions despite removal of multiple tissue layers. The treatment of the remaining tumors presented a significant challenge. METHODS: Full-face CO2 laser resurfacing was performed after curettage and local laser ablation of all visible tumors. RESULTS: The patient healed well postoperatively. She developed six basal cell carcinomas on her face during the 10-month follow-up period. These were easily treated with Mohs surgery and imiquimod cream. CONCLUSION: CO2 laser resurfacing is a useful surgical modality for treatment of multiple facial basal cell carcinomas in the patients with NBCCS. It allows treatment of all the facial lesions in a single session and minimizes suffering resulting from repeated surgical procedures. Additionally, the layer of skin damaged by ultraviolet radiation is removed, allowing for easier management after resurfacing.     

5% 5-Fluorouracil Cream for the Treatment of Small Superficial Basal Cell Carcinoma: Efficacy, Tolerability, Cosmetic Outcome, and Patient Satisfaction

BACKGROUND: Five percent 5-fluorouracil (5-FU) cream is approved by the FDA for the treatment of superficial basal cell carcinomas but has been underutilized. OBJECTIVE: The objective was to evaluate the efficacy, tolerability, cosmetic outcome, and patient satisfaction of 5% 5-FU in the treatment of superficial basal cell carcinomas. MATERIALS AND METHODS: A total of 29 patients with 31 biopsy-proven superficial basal cell carcinoma lesions on the trunk or limbs were treated with 5% 5-FU cream twice daily for up to 12 weeks. Treatment could be stopped sooner if the lesion was clinically resolved. The lesional site was surgically excised 3 weeks after the end of treatment for histologic evaluation of cure. RESULTS The histologic cure rate was 90% (28/31 lesions cured) and the mean time to clinical cure was 10.5 weeks. 5-FU was generally well tolerated with a good cosmetic outcome-the majority of patients had no pain or scarring and only mild erythema. Patients were generally very satisfied with their treatment. CONCLUSION: Five percent 5-FU is a highly effective and well-tolerated treatment option for superficial basal cell carcinomas offering a generally good cosmetic outcome and high levels of patient satisfaction.     

Multiple Hypersonographic Spots in Basal Cell Carcinoma

BACKGROUND: High-frequency ultrasound is a useful method to obtain preoperative information regarding extension of basal cell carcinoma. Its contribution to diagnosis is generally limited, however. Recently, we observed hypersonographic spots in some cases of basal cell carcinoma. OBJECTIVE: The present study was performed to determine the diagnostic value of this finding in this type of tumor. MATERIALS AND METHODS: We conducted a retrospective study of archived sonographic images with a 30- or 15-MHz scanner and histology specimens of a total of 85 lesions, consisting of 29 basal cell carcinomas and 56 melanomas. RESULTS: The findings were classified into four patterns as follows: Type A, multiple (more than five spots/lesion) hypersonographic spots (14 cases, 48%); Type B, sparse (3-5 spots/lesion) hypersonographic spots (7 cases, 24%); Type C, multiple moderate sonographic spots (3 cases, 10%); and Type D, sparse moderate sonographic spots (5 cases, 17%). Three of 56 melanoma lesions examined as controls showed the Type D pattern, but none showed Type A, B, or C patterns. Histopathologically, these hypersonographic spots in BCCs seemed to correspond to calcification, horn cysts, or clusters of apoptotic cells in the centers of nests of basal cell carcinoma. CONCLUSION: Multiple hypersonographic spots might become a useful finding for differential diagnosis between basal cell carcinoma and melanoma.     

Superficial Basal Cell Carcinoma of the Face Successfully Treated with Ingenol Mebutate 0.05% Gel: Case Report and a Review of the Literature

A 64 year-old Caucasian male patient with a long history of ultraviolet light exposure and multiple actinic keratoses presented with a large, erythematous, and scaly plaque on his forehead. Biopsies revealed superficial basal cell carcinoma (sBCC). Because the patient wanted the shortest possible topical regimen, his sBCC was treated with two overnight ingenol mebutate (IM) 0.05% gel applications. He tolerated the local skin reaction (LSR) well, and at approximately six weeks post-treatment, biopsies showed no evidence of sBCC. The patient was happy with the cosmetic outcome and has remained free of clinical recurrence for 18 months. Although IM gel is only FDA approved for the treatment of actinic keratosis, it has also been used off-label to treat other epithelial lesions, including basal cell carcinoma (BCC), anogenital warts, and Bowen’s disease. One clinical trial, multiple case series and case reports, and now this report, have demonstrated IM’s utility in treating BCC. IM treatment is therefore a promising alternative to surgery for select BCC, with major advantages, including a short treatment duration and generally favorable cosmetic outcome.     

NAEVOID BASAL CELL CARCINOMA (GORLIN'S) SYNDROME

The multiple naevoid basal cell carcinoma syndrome (Gorlin's syndrome) is an inherited multisystem disorder which affects not only the skin, but also the skeletal system and a variety of other tissues and organs. This is a report of the case of a 29 year old woman who presented with multiple advanced basal cell carcinomas. On the basis of a literature review, guidelines for management are suggested and the importance of early diagnosis and regular follow-up is emphasized.     

Incidence of invasive versus non-invasive carcinoma in comparing palpable and non-palpable solid breast lesions

A retrospective study was done of all patients with a suspicious mammographic breast lesion surgically biopsied in our institution within the last 5 years. Incidence of invasive versus non-invasive carcinoma and stage at presentation (according to TNM classification system) of palpable and non-palpable lesions were compared. We found a significant difference of non-invasive carcinoma in non-palpable and palpable cancers: 42.2% versus 4.3% (p < 0.001). Patients with a non-palpable invasive carcinoma presenting at stage I (i.e. pT1 with no axillary metastasis) rated significantly higher compared to those with palpable lesions 51.8% versus 9.4% (p < 0.001). The true positive biopsy rate is 30%. As low as 10% has been considered reasonable. We have a total of 56% carcinomas detected on all biopsies: 30% for non-palpable lesions and 66.8% for palpable lesions. A more aggressive approach towards screening and biopsy of breast lesions might increase early detection of carcinoma and so improve survival.     

Inverted papillomas and associated squamous cell carcinomas

Inverted papillomas of the nose and paranasal sinuses are found to be associated with squamous cell carcinomas in a small percentage of cases. The records of forty-six patients treated for inverted papillomas in these areas over a 10-year period were reviewed. Eleven patients (24%) were found to have squamous cell carcinoma, and one carcinoma in situ, in relationship to the inverted papillomas. This is a much higher percentage than generally reported. Four of these patients were found to have inverted papillomas coincidentally in surgical specimens resected for squamous cell carcinoma. Patients with squamous cell carcinoma were found to be in older age groups, were more likely to manifest epistaxis than the more common unilateral nasal obstructive symptoms, and had less time between the onset of symptoms and presentation than those with inverted papillomas alone. The clinical presentation, treatment, and outcome of inverted papillomas and squamous cell carcinomas in association with inverted papillomas are discussed.     

Surgical treatment of malignant lip tumors. Personal experience

The malignant tumours of the lip account for nearly 1-2% of the cervicofacial neoplasms. These lesions are frequently spinous cell carcinomas and basal cell carcinomas (25% of all oral cancers). The spinous cell carcinoma is mainly located in the lower lip, the basal cell carcinoma is more common in the upper lip. The incidence of lip cancer in males is much high than in females. The etiopathogenesis of these lesions is connected with exposure to sun, smoking, genetics predisposition (mutation of the p53 suppressor factor) and with the evolution of precancerous lesions (radiodermatitis, chronic cheilitis, xeroderma pigmentosum). Some Authors emphasized the viral etiopathogenesis: HPV16, HPV24, HSV1, HSV2. The treatment of lip carcinoma is surgical: excision and reconstruction. The numerous reconstructive techniques are mostly the cutaneous local sliding flaps and the rotation flaps. The lip reconstruction require a remarkable diligence for preserve, as much possible, the shape and functions of lip. The Authors report their experience about the surgical treatment of 19 patients with lip carcinoma (16 spinous cell carcinomas, 3 basal cell carcinomas) and describe the main surgical reconstructive techniques to preserve the feeding, phonation and mimic expression.     

Basal cell carcinoma of the dorsum of the hand

Although extremely frequent elsewhere on the body, basal cell carcinomas are infrequent on the hands (< 1% of all basal cell carcinomas). Chronic sun exposure is considered as the main etiologic factor for its development but it does not explain low frequency of hand involvement. Presented here is a series of seven patients with basal cell carcinoma of the hand. All were treated by surgery alone. Clinical presentation was classical: a slowly growing chronic ulceration. None of the patients was chronically exposed to chemical agents but two of them developed their tumour in previously irradiated areas, focusing on this specific etiologic factor for basal cell carcinomas of the hands.     

Basal Cell Carcinoma of the Dorsum of the Hand

Although extremely frequent elsewhere on the body, basal cell carcinomas are infrequent on the hands (< 1% of all basal cell carcinomas). Chronic sun exposure is considered as the main etiologic factor for its development but it does not explain low frequency of hand involvement. Presented here is a series of seven patients with basal cell carcinoma of the hand. All were treated by surgery alone. Clinical presentation was classical: a slowly growing chronic ulceration. None of the patients was chronically exposed to chemical agents but two of them developed their tumour in previously irradiated areas, focusing on this specific etiologic factor for basal cell carcinomas of the hands.     

Examination of Lesions (Including Dermoscopy) without Contact with the Patient Is Associated with Improper Management in about 30% of Equivocal Melanomas

BACKGROUND: In clinical practice, decisions regarding management of a pigmented skin lesion are based on morphologic examination, as well as on anamnestic, emotional, and medicolegal aspects. In some cases, the "ugly duckling" sign may be an indication for excision of a morphologically featureless melanoma. Therefore, examination of pigmented skin lesions based on clinical and dermoscopic images, without contact with the patient, may be associated with a not negligible risk of incorrect lesion management. OBJECTIVE: In this study, we tried to assess to what extent lesion management based on purely morphologic examination diverges from optimal management based on in vivo examination with direct contact with the patient, lesion history, and clinical and dermoscopic evaluation. METHODS: The study included clinical and dermoscopic images of 100 diagnostically equivocal pigmented lesions, including 20 early melanomas and 5 pigmented basal cell carcinomas consecutively referred for surgery; the images were reviewed by six dermatologists who specialize in melanoma screening and were previously trained in dermoscopy. RESULTS: The percentage of melanomas correctly classified was less than 50% both for naked eye and combined examination. Regarding lesion management, only about 70% of malignancies (melanomas and basal cell carcinomas) are correctly referred for surgery by observers. Similar results have been obtained focusing on melanoma (72.5%). CONCLUSION: Facing difficulties in diagnosing pigmented skin tumors, lesion management based on the morphology of the lesion, even including dermoscopic images, but without direct contact with the patient, diverges greatly from the gold standard management established by face-to-face examination and comports a not negligible risk of leaving a melanoma unexcised.     

Multiple Pigmented Basal Cell Carcinomas Arising in the Normal-Appearing Skin After Radiotherapy for Carcinoma of the Cervix

BACKGROUND: The most frequent radiation-induced skin cancers are basal cell carcinomas (BCCs), and in many cases, they may occur subsequent to tinea capitis, hypertrophic tonsillitis, acne vulgaris, atopic dermatitis, and hyperthyroidosis. BCCs occurring after radiotherapy for carcinoma of the cervix are extremely rare. OBJECTIVE: To describe a case of multiple pigmented BCCs caused by radiotherapy for carcinoma of the cervix. RESULTS: A 76-year-old female presented with four tumoral lesions within a previously irradiated area. The patient had undergone radiation therapy for cervical cancer 38 years before. All of the lesions were totally excised and histopathologically diagnosed as BCCs. There was no apparent gross chronic radiodermatitis around the lesions, but characteristic histopathologic alterations of radiodermatitis were observed. No new tumors were observed to date. CONCLUSION: The latent period for radiation-induced skin cancer is generally very long; secondary skin cancers can develop very late in life. Patients previously treated with radiation therapy require careful follow-up for life.     

A case of small cell carcinoma of the kidney

A 47-year-old man had a retroperitoneal tumor measuring 18 cm in maximum diameter of the left kidney that was diagnosed with computed tomography (CT)-guided needle biopsy as small cell carcinoma. Microscopically, the tumor cells showed immunohistochemical reaction for neural cell adhesion molecule antibodies. This patient with advanced renal small cell carcinoma and multiple metastatic lesions was treated with the first-line combination chemotherapy of cisplatin, etoposide and ifosphamide, which showed a partial response of primary renal tumor and a complete response of liver metastasis, and with the second-line chemotherapy of cisplatin and irinotecan, which showed a complete response of Virchow's nodal metastasis. Thereafter, in spite of salvage chemotherapy of amurubicin hydrochloride for persistent and refractory renal small cell carcinoma, he died 32 months after the first presentation due to local progression. However, combination chemotherapy of these anticancer agents made his prognosis more favorable than we expected before treatment. The extrapulmonary small cell carcinomas are generally known to be more aggressive and malignant than the lung small cell carcinomas, and small cell carcinoma arising from the kidney is an extremely rare malignant neoplasm, with only 34 cases reported in the English or Japanese literature. The prognosis of renal small cell carcinomas is currently limited as compared with the lung small cell carcinomas, and therefore a cumulative investigation of a larger number of cases treated with multidisciplinary modalities including combination chemotherapy is necessary.     

Skin Tumors in Patients Aged 90 Years and Older

BACKGROUND: The aging of the population in the developed world is an accepted fact. Consequently, the number of patients aged 90 years or over with cutaneous cancer will increase in coming years. OBJECTIVE: The objective was to determine the nature of skin lesions amenable to dermatologic surgery in patients aged 90 years or more. METHODS: We studied the clinical histories of 52 outpatients in their 90 s, who had had histopathologic studies made of their skin tumors when attended in the Hospital General de Alicante (Spain) between January 1999 and July 2002. We studied sex, age, type of tumor, site, associated disorders, regular medications, type of anesthesia, and type of treatment given. RESULTS: The average age of the patients was 92.4 years. Thirty-six patients were women and 16 were men. Altogether the 52 patients had a total of 72 lesions. The most frequent diagnosis was basal cell carcinoma with 36 lesions, followed by 20 squamous cell carcinomas. The overall ratio of basal cell carcinoma to squamous cell carcinoma was 1.8. Patients had an average of 1.5 comorbid medical conditions and were taking an average of 2.3 regular medications. CONCLUSION: Dermatologists often attend patients aged 90 years or over with nonmelanoma skin cancer. The most important decision is as to what is the best management of these patients.     

Actinic Keratoses and the Incidence of Occult Squamous Cell Carcinoma: A Clinical–Histopathologic Correlation

BACKGROUND: The ability to clinically diagnose actinic keratoses (AKs) lesions has been taken for granted for some time. The importance of the malignant potential of these lesions is well known. However, a recent Phase IV, multicenter study assessing the long-term benefit of aminolevulinic acid-based photodynamic therapy provided a unique opportunity to prospectively examine the clinical histopathologic correlation of AKs. OBJECTIVE: The objective was to characterize the histopathology of clinically diagnosed AK lesions in the study population. METHODS: Punch biopsies of 220 clinically diagnosed untreated AKs were performed at baseline plus 51 lesions unresponsive to treatment (total, 271). RESULTS: Clinical diagnosis and histopathologic findings agreed in 91% (246/271) of the lesions biopsied. The balance of the biopsied lesions were: (1) benign changes 4% (11/271) and (2) occult cutaneous malignancy in 5% (14/271) of the cases, 12 squamous cell carcinomas and 2 basal cell carcinomas. CONCLUSIONS: In this study, about 1 in 25 clinically diagnosed AK lesions identified by board-certified dermatologist investigator(s) were occult early-stage squamous cell carcinomas on histologic assessment, a fact surmised by the medical community that until now had not been well quantified. These findings should be considered when clinicians decide how to treat and manage AK patients.     

Pulmonary metastasis in nevoid basal cell carcinoma syndrome

Nevoid basal cell carcinoma syndrome is a relatively uncommon clinical entity. It is essentially characterized by the early onset of multiple basal cell carcinomas of the skin generally above wrist-line level, by the presence of jaw cysts, musculoskeletal and other anomalies. Metastatic disease secondary to ordinary basal cell carcinoma is indeed rare. What is even more rare is pulmonary metastases from basal cell carcinoma. Very few cases have been reported in which pulmonary resection was performed for metastatic basal cell carcinoma. We were unable to find a reported case in which pulmonary resection was performed for a pulmonary nodule metastatic from the nevoid basal cell carcinoma syndrome. Herein is reported what we believe to be the first case of pulmonary resection performed for a metastatic nodule in this syndrome.