Dermoscopic Clues of Histopathologically Aggressive Basal Cell Carcinoma Subtypes.

Background: The group of histopathologically aggressive BCC subtypes includes morpheaform, micronodular, infiltrative and metatypical BCC. Since these tumors are at increased risk of recurring, micrographically controlled surgery is considered the best therapeutic option. Although dermoscopy significantly improves the clinical recognition of BCC, scarce evidence exists on their dermoscopic criteria. Aim: To investigate the dermoscopic characteristics of histopathologically aggressive BCC subtypes. Materials and Methods: Dermoscopic images of morpheaform, micronodular, infiltrative and metatypical BCC were analyzed for the presence of predefined variables. Descriptive and analytical statistics were performed. Results: Most histopathologically aggressive BCCs were located on the head and neck. Infiltrative was the most common subtype. All subtypes, except micronodular BCC, rarely displayed dermoscopic pigmentation. The most frequent dermoscopic features of infiltrative BCC were arborizing vessels (67.1%), shiny white structures (48.6%) and ulceration (52.9%). The features prevailing in morpheaform BCC were arborizing vessels (68.4%), ulceration (n = 12, 63.2%) and white porcelain areas (47.4%). Micronodular BCC was typified by milky red structureless areas (53.8%), arborizing vessels (53.8%), short fine telangiectasias (50%), ulceration (46.2%) and blue structures (57.7%). The most common findings in metatypical BCC were arborizing vessels (77.8%), shiny white structures (66.7%), ulceration (62.9%) and keratin mass (29.6%). Limitations: Study population of only white skin and relatively small sample size in some groups. Conclusions: Our study provided data on the clinical, dermoscopic and epidemiological characteristics of histopathologically aggressive BCCs.

Histologically Agressive Basal Cell Carcinoma With Particular Emphasis On Galeal Infiltration Of The Scalp. / Carcinoma basocelular histológicamente agresivo con especial atención a la infiltración galeal del cuero cabelludo.

Familiarity with predictors of more aggressive behavior is crucial to the management of basal cell carcinoma (BCC). Risk factors for aggressive BCC are essentially divided into clinical and histopathologic factors. In this review we examine histopathologic features predictive of aggressiveness in BCC. The morpheaform, infiltrative, micronodular, metatypical, and basosquamous subtypes and BCC with sarcomatoid differentiation are classically considered predictive of aggressive behavior. However, 2 other features associated with aggressive BCC are perineural invasion (invasion of nerves below the dermis or nerves larger than 0.1mm in caliber) and subgaleal extension. While the former is well known and widely described in the literature, the latter is not generally recognized as a risk factor, even though it is predictive of highly aggressive behavior. In this review, we draw attention to its importance.

Dermoscopic features of basal cell carcinoma and its subtypes: A systematic review.

BACKGROUND: Multiple studies have reported on dermoscopic structures in basal cell carcinoma (BCC) and its subtypes, with varying results. OBJECTIVE: To systematically review the prevalence of dermoscopic structures in BCC and its subtypes. METHODS: Databases and reference lists were searched for relevant trials according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were assessed for the relative proportion of BCC dermoscopic features. Random-effects models were used to estimate summary effect sizes. RESULTS: Included were 31 studies consisting of 5950 BCCs. The most common dermoscopic features seen in BCC were arborizing vessels (59%), shiny white structures (49%), and large blue-grey ovoid nests (34%). Arborizing vessels, ulceration, and blue-grey ovoid nests and globules were most common in nodular BCC; short-fine telangiectasia, multiple small erosions, and leaf-like, spoke wheel and concentric structures in superficial BCC; porcelain white areas and arborizing vessels in morpheaform BCC; and arborizing vessels and ulceration in infiltrative BCC. LIMITATIONS: Studies had significant heterogeneity. Studies reporting BCC histopathologic subtypes did not provide clinical data on pigmentation of lesions. CONCLUSION: In addition to arborizing vessels, shiny white structures are a common feature of BCC. A constellation of dermoscopic features may aid in differentiating between BCC histopathologic subtypes.

Diagnosis and Management of Basal Cell Carcinoma.

OPINION STATEMENT: Basal cell carcinomas (BCCs) are common skin cancers that tend to appear on sun-exposed skin. Pathobiologically, activation of the Hedgehog signaling pathway characterizes the majority of cases. In general, BCCs are slow-growing and rarely metastasize. Nevertheless, they are locally invasive and can be destructive. While typical cases are diagnosed based on clinical findings, the clinicopathological manifestations are varied. Consequently, skin biopsy is essential to confirm the diagnosis and evaluate the risk of recurrence. In the treatment of primary lesions, the initial goal is to complete tumor removal, whether by conventional surgical excision, Mohs micrographic surgery, cryosurgery, electrodesiccation and curettage, topical application of imiquimod or fluorouracil, photodynamic therapy, or radiation therapy. Of these treatments, surgical excision and Mohs surgery are the most commonly used because of their association with a low recurrence rate and the ability to confirm residual tumor pathologically. However, other treatment options may be preferred according to patient condition, tumor location, and risk of recurrence. In the treatment of metastatic or locally advanced lesions, smoothened inhibitors, which inhibit Hedgehog signaling pathway activation, were recently approved and impressive tumor shrinkage effects have been described. Although the exact prognosis of metastatic BCC has not been analyzed, it is probably poor due to the rarity of such condition. However, emerging molecular targeting agents hold therapeutic promise.

Comparative analysis of cytokeratin 15, TDAG51, cytokeratin 20 and androgen receptor in sclerosing adnexal neoplasms and variants of basal cell carcinoma.

BACKGROUND: Desmoplastic trichoepithelioma (DTE), morpheaform basal cell carcinoma (BCC) and microcystic adnexal carcinoma (MAC) are sclerosing adnexal neoplasms with overlapping histopathologic features. We compared cytokeratin 15, (CK15), T-cell death-associated gene 51 (TDAG51), cytokeratin 20 (CK20) and androgen receptor (AR) in differentiating these tumors and assessed their expression in BCC subtypes. METHODS: Fifteen DTE, 15 infundibulocystic BCC, 18 micronodular BCC, 18 morpheaform BCC and 6 MAC were assessed for CK15, TDAG51, CK20 and AR expression. RESULTS: Quantitative CK15 staining was higher in DTE compared with BCC (p < 0.0001) and MAC (p = 0.02). Quantitative TDAG51 staining was higher in DTE than BCC (p < 0.0001). The CK20+AR- immunophenotype was 100% sensitive and specific in diagnosing DTE. The CK20-AR+ immunophenotype was 95.24% specific and 83.33% sensitive for BCC. The CK20-AR- immunophenotype was 83.33% sensitive and 90.91% specific for MAC. CK15, CK20 and AR were positive in 87, 53 and 67% of infundibulocystic BCC cases, respectively. CONCLUSION: Combination of CK20 and AR best differentiated these sclerosing adnexal neoplasms. Greater positivity for CK15 and TDAG51 generally favors benign lesions. Infundibulocystic BCC has higher CK20 and lower AR immunopositivity than other BCC variants and a high degree of CK15 and TDAG51 positivity.

Giant Morpheaform Basal Cell Carcinoma Mimicking Scarring Alopecia: Exception Prone to Neglect.

A 74-year-old woman in good general health presented with a 5-year history of progressive hair loss over several years, interpreted as female androgenetic alopecia (AGA), and was treated with topical 5% Minoxidil without improvement. The patient's relevant medical history revealed infiltrating, triple-negative apocrine carcinoma of the right breast four years before, treated by quadrantectomy, radiation, lymphadenectomy and chemotherapy, with no recurrence at the last follow-up. On examination, there was an asymptomatic 15 × 15 cm firm and whitish area of scarring alopecia on the central scalp. Dermoscopy revealed multiple arborizing vessels and many telangiectasia. The clinical considerations included mainly cutaneous metastasis of breast carcinoma (alopecia neoplastica), pseudopelade of Broque and morpheaform basal cell carcinoma (BCC). A histopathologic examination revealed characteristic changes of morpheaform BCC with basaloid islands and cords of atypical basaloid cells diffusely infiltrating the dermis, embedded in a sclerotic and hypervascularized stroma. Secondary alopecia neoplastica due to morpheaform BCC on the scalp is an exceedingly rare entity, possessing subtle clinical features that may mimic both scarring and non-scarring alopecia. Delayed recognition may contribute to aggressive behavior and extensive local destruction. Treatment with hedgehog inhibitors in locally advanced BCC of the scalp, both in adjuvant and neoadjuvant modalities, is promising.

Morpheaform basal cell carcinoma of the nasal ala associated with multiple familial trichoepithelioma reconstructed by anterolateral thigh flap: a case report.

The right nasal ala had been destroyed and was diagnosed as morpheaform basal cell carcinoma associated with multiple familial trichoepithelioma (Brooke-Spiegler syndrome). After extended resection, the right buccal and nasal ala defect was reconstructed with a flow-through type anterolateral thigh (ALT) flap, which achieved good functional and cosmetic results.

Clinical and Molecular Features of Morpheaform Basal Cell Carcinoma: A Systematic Review.

Basal cell carcinoma (BCC) is the most common skin cancer, with a lifetime risk currently approaching up to 40% in Caucasians. Among these, some clinical and pathological BCC variants pose a higher risk due to their more aggressive biological behavior. Morpheaform BCC (morBCC), also known as sclerosing, fibrosing, or morpheic BCC, represents up to 5-10% of all BCC. Overall, morBCC carries a poorer prognosis due to late presentation, local tissue destruction, tumor recurrence, and higher frequency of metastasis. In this systematic review, we review the epidemiological, clinical, morphological, dermatoscopical, and molecular features of morBCC. After the title and abstract screening of 222 studies and the full-text review of 84 studies, a total of 54 studies met the inclusion criteria and were thus included in this review.

Giant morphea-form basal cell carcinoma of the umbilicus: Successful debulking with vismodegib.

Basal cell carcinoma of the umbilicus is very rare. The nodular subtype is the main representative. Giant basal cell carcinomas represent around 1% of all basal cell carcinomas. The hedgehog pathway inhibitor vismodegib is indicated for advanced basal cell carcinoma and CD56-negative immunostaining seems indicative for successful treatment. A 54-year-old man presented a 10 cm × 14 cm large and 4.5 cm deep morphea-form basal cell carcinoma with faint immunohistochemical CD56 expression arising from the umbilicus. A sequential treatment was initiated with debulking using vismodegib 150 mg per day for 4 months, followed by reconstructive surgery. To the best of our knowledge, this is the first report of a giant basal cell carcinoma of the morphea-form type of the umbilicus. The sequential treatment plan reduces the duration of vismodegib inherent adverse effects and significantly reduces the tumor mass prior to surgery. Besides increasing adherence to vismodegib treatment, this approach facilitates the surgical technique and improves cosmetic outcome.

Carcinoma basocelular histológicamente agresivo con especial atención a la infiltración galeal del cuero cabelludo / Histologically Agressive Basal Cell Carcinoma With Particular Emphasis On Galeal Infiltration Of The Scalp

En el manejo del carcinoma basocelular (CBC) es fundamental conocer los factores de riesgo que predicen un comportamiento más agresivo. Estos factores de riesgo se dividen esencialmente en factores clínicos y en factores histopatológicos. En este trabajo revisamos e ilustramos los hallazgos histopatológicos predictivos de agresividad en el CBC. Dichos factores histopatológicos predictivos de agresividad incluyen las variedades histológicas clásicas de CBC «agresivas»: la morfeiforme, la infiltrativa, la micronodular, la metatípica, la basoescamosa y el CBC con diferenciación sarcomatoide. Pero, aparte de las variedades referidas, existen también 2hallazgos histopatológicos asociados a CBC agresivos, uno bien conocido y reflejado en la literatura, que es la infiltración perineural de filetes nerviosos de más de 0,1mm de diámetro o subdérmicos, y el otro es la extensión subgaleal. La infiltración galeal no está bien reconocida como tal en literatura, pero es un factor predictivo de agresividad importante en el CBC y queremos llamar la atención sobre él (AU)

Familiarity with predictors of more aggressive behavior is crucial to the management of basal cell carcinoma (BCC). Risk factors for aggressive BCC are essentially divided into clinical and histopathologic factors. In this review we examine histopathologic features predictive of aggressiveness in BCC. The morpheaform, infiltrative, micronodular, metatypical, and basosquamous subtypes and BCC with sarcomatoid differentiation are classically considered predictive of aggressive behavior. However, 2 other features associated with aggressive BCC are perineural invasion (invasion of nerves below the dermis or nerves larger than 0.1mm in caliber) and subgaleal extension. While the former is well known and widely described in the literature, the latter is not generally recognized as a risk factor, even though it is predictive of highly aggressive behavior. In this review, we draw attention to its importance (AU)

[Translated article] Histologically agressive basal cell carcinoma with particular emphasis on galeal infiltration of the scalp / Carcinoma basocelular histológicamente agresivo con especial atención a la infiltración galeal del cuero cabelludo

Familiarity with predictors of more aggressive behavior is crucial to the management of basal cell carcinoma (BCC). Risk factors for aggressive BCC are essentially divided into clinical and histopathologic factors. In this review we examine histopathologic features predictive of aggressiveness in BCC. The morpheaform, infiltrative, micronodular, metatypical, and basosquamous subtypes and BCC with sarcomatoid differentiation are classically considered predictive of aggressive behavior. However, 2 other features associated with aggressive BCC are perineural invasion (invasion of nerves below the dermis or nerves larger than 0.1mm in caliber) and subgaleal extension. While the former is well known and widely described in the literature, the latter is not generally recognized as a risk factor, even though it is predictive of highly aggressive behavior. In this review, we draw attention to its importance (AU)

En el manejo del carcinoma basocelular (CBC) es fundamental conocer los factores de riesgo que predicen un comportamiento más agresivo. Estos factores de riesgo se dividen esencialmente en factores clínicos y en factores histopatológicos. En este trabajo revisamos e ilustramos los hallazgos histopatológicos predictivos de agresividad en el CBC. Dichos factores histopatológicos predictivos de agresividad incluyen las variedades histológicas clásicas de CBC «agresivas»: la morfeiforme, la infiltrativa, la micronodular, la metatípica, la basoescamosa y el CBC con diferenciación sarcomatoide. Pero, aparte de las variedades referidas, existen también 2hallazgos histopatológicos asociados a CBC agresivos, uno bien conocido y reflejado en la literatura, que es la infiltración perineural de filetes nerviosos de más de 0,1mm de diámetro o subdérmicos, y el otro es la extensión subgaleal. La infiltración galeal no está bien reconocida como tal en literatura, pero es un factor predictivo de agresividad importante en el CBC y queremos llamar la atención sobre él (AU)

Desmoplastic trichoepithelioma: A clinicopathological study of three cases and a review of the literature.

Desmoplastic trichoepithelioma (DTE) is a rare benign adnexal tumor with the characteristic features of asymptomatic, solitary, annular, indurated and centrally depressed papules or plaques, most commonly occurring in younger individuals on the face. Microscopically and clinically, DTE may be difficult to distinguish from other cutaneous adnexal neoplasms, particularly syringoma, cutaneous metastatic breast cancer, morpheaform basal cell carcinoma and microcystic adnexal carcinoma. The present study reports three cases of DTE. The first case was of a 45-year-old male with an asymptomatic flesh-colored plaque below the right edge of the outer canthus that had been present for seven years. The second case was of a 23-year-old female with an asymptomatic skin lesion on the right cheek that had slowly and progressively increased in size. The third case was of a 26-year-old female who presented with a hard yellowish-white plaque, which gradually grew and formed a rectangular, 3×4-cm patch, on the tip of the left brow. This plaque was present for three years without evident cause or subjective symptoms. In all three cases, the routine systemic examinations and laboratory findings were normal. Histopathological and immunohistochemical findings from incisional biopsies of the lesions were consistent with a diagnosis of DTE. DTE treatment methods and immunohistochemical markers were analyzed by reviewing clinical pathological aspects in order to avoid a misdiagnosis and to provide the best available treatment approach for DTE.

A case of morpheaform sarcoidosis.

Sarcoidosis is an idiopathic multisystem disease with various cutaneous presentations, and it is characterized by the presence of non-caseating granulomas in the affected organs. The specific manifestations are papules, plaques, nodules, ulcers and scar. We report here on a variant of sarcoidosis on a 71-year-old woman who showed an indurated plaque on the forearm. Her lesion's appearance was clinically similar to that of a morphea and the appearance of the lesion was unlike the commonly observed manifestations of sarcoidosis.

A Case of Morpheaform Sarcoidosis

Sarcoidosis is an idiopathic multisystem disease with various cutaneous presentations, and it is characterized by the presence of non-caseating granulomas in the affected organs. The specific manifestations are papules, plaques, nodules, ulcers and scar. We report here on a variant of sarcoidosis on a 71-year-old woman who showed an indurated plaque on the forearm. Her lesion's appearance was clinically similar to that of a morphea and the appearance of the lesion was unlike the commonly observed manifestations of sarcoidosis.