Clustered yellow papules in the posterior axilla of a middle-aged woman.

Naevus lipomatosus cutaneous superficialis (NLCS) of Hoffman-Zurhelle is a rare hamartomatous benign condition first described in 1921. Two clinical variants have been described: a classical form of multiple yellow papules that coalesce to form larger plaques with segmental distribution, and a solitary form also known as pedunculated lipofibroma. We present a case of early-stage NLCS with characteristic histopathological and dermoscopic features.

Melanocytic lesions with peripheral globules: results of an observational prospective study in 154 high-risk melanoma patients under digital dermoscopy follow-up evaluated with reflectance confocal microscopy.

BACKGROUND: The management of melanocytic lesions with peripheral globules (MLPGs) is usually age-dependent and can be challenging in high-risk melanoma patients. OBJECTIVES: To evaluate clinical, dermoscopic and reflectance confocal microscopy (RCM) features of MLPG in patients under digital dermoscopic surveillance. To know whether dermoscopic or RCM findings correlate with histologic diagnosis and the accuracy of the dermoscopy-RCM compared with histopathology. METHODS: During 24 months, we prospectively enrolled MLPG in patients under digital dermoscopy follow-up. All were evaluated by dermoscopy and RCM and excised for histologic examination. RESULTS: We enrolled 154 patients, mean age 42.45 years (18.78-73.19). Three melanomas and 19 dysplastic naevi (DNs) were diagnosed. There were no significant differences in the age of the patients (P = 0.662). MLPGs with diameter of 6 mm or more and asymmetry in two axes were associated with melanoma (P = 0.01, P = 0.003). Patients with more than one MLPG were less likely to have melanoma. Blue-grey and red colours were more frequent in melanoma (P = 0.013 and P = 0.000). Different sizes and shapes of PG were associated with DN and melanoma (P = 0.000 and P = 0.001). In a new lesion, PG in <25% of the circumference was related to malignancy (P = 0.010). RCM signs of malignancy were related to melanoma: pagetoid cells (P = 0.000), non-edged papillae (P = 0.001), atypical junctional thickenings (P = 0.000) and atypical cells at the dermal-epidermal junction (P = 0.000). Dense irregular nests were associated to melanoma (P = 0.019). Dermoscopy and confocal evaluation were able to diagnose 100% of melanomas and 84.21% of DNs. The kappa coefficient between dermoscopy-RCM vs. histology was 0.76. CONCLUSIONS: We recommend to excise a MLPG when it presents asymmetry in two axes, 6 or more mm, new lesion with PG in less than the 25% of the circumference, irregular size and shape PGs and irregular dense nests on RCM, regardless of the patient's age.

Merkel Cell Carcinoma: A Description of 11 Cases. / Carcinoma de células de Merkel: estudio descriptivo de 11 casos.

Merkel cell carcinoma (MCC) is a malignant neuroendocrine tumor. Metastasis or lymph node spread is often detected at diagnosis. We performed a descriptive, retrospective study of patients diagnosed with MMC at Hospital Universitario Fundación Alcorcón in the Community of Madrid, Spain between January 1998 and December 2018. Eleven patients (7 men [63%] and 4 women [36%]; mean age, 77.6 years) were diagnosed with MCC during this 21-year period; 45% of patients had stage IIIB disease (pTNM) at diagnosis. All patients but one underwent local surgery, and lymphovascular invasion was detected in 7 cases. Eight patients received adjuvant therapy after surgery (radiation therapy in 5 cases and chemotherapy in 3). Six patients (54%) died of MCC (mean survival, 14.5 months). MCC is an uncommon malignant tumor with an annual incidence of around 0.18 to 0.41 cases per 100 000 inhabitants; this is similar to the rate of 0.29 to 0.32 cases per 100 000 inhabitants a year detected in our series. Results with avelumab, a drug recently approved for the treatment of metastatic MCC; have been promising.

Early outcome of a 31-gene expression profile test in 86 AJCC stage IB-II melanoma patients. A prospective multicentre cohort study.

BACKGROUND: The clinical and pathological features of primary melanoma are not sufficiently sensitive to accurately predict which patients are at a greater risk of relapse. Recently, a 31-gene expression profile (DecisionDx-Melanoma) test has shown promising results. OBJECTIVES: To evaluate the early prognostic performance of a genetic signature in a multicentre prospectively evaluated cohort. METHODS: Inclusion of patients with AJCC stages IB and II conducted between April 2015 and December 2016. All patients were followed up prospectively to assess their risk of relapse. Prognostic performance of this test was evaluated individually and later combined with the AJCC staging system. Prognostic accuracy of disease-free survival was determined using Kaplan-Meier curves and Cox regression analysis. Results of the gene expression profile test were designated as Class 1 (low risk) and Class 2 (high risk). RESULTS: Median follow-up time was 26 months (IQR 22-30). The gene expression profile test was performed with 86 patients; seven had developed metastasis (8.1%) and all of them were in the Class 2 group, representing 21.2% of this group. Gene expression profile was an independent prognostic factor for relapse as indicated by multivariate Cox regression analysis, adjusted for AJCC stages and age. CONCLUSIONS: This prospective multicentre cohort study, performed in a Spanish Caucasian cohort, shows that this 31-gene expression profile test could correctly identify patients at early AJCC stages who are at greater risk of relapse. We believe that gene expression profile in combination with the AJCC staging system could well improve the detection of patients who need intensive surveillance and optimize follow-up strategies.

Reflectance Confocal Microscopy: A Promising Tool to Identify Malignancy in Melanocytic Lesions Exhibiting a Dermoscopic Island. / Microscopia confocal de reflectancia: una herramienta útil para diagnosticar malignidad en lesiones melanocíticas que presenten el signo de la isla.

The dermoscopic island is described as a well-defined area in a melanocytic lesion, with a different dermoscopic pattern from the rest of the lesion. It is predictive of melanoma, particularly when the pattern of the island is atypical. We present the reflectance confocal microscopy (RCM) findings in 3 lesions with dermoscopic islands: nevus-associated melanoma, melanocytic nevus, and in situ melanoma. The nevus-associated melanoma and in situ melanoma presented cellular atypia (atypical cells in isolation or forming nests) and architectural distortion on RCM. The nevus presented a dermoscopic island with a typical globular pattern with dense nests and no atypia on RCM. Dermoscopic island is mainly associated with in situ and nevus-associated melanomas. RCM offers good cellular resolution to the depth of the reticular dermis and is useful for diagnosing of melanomas presenting a dermoscopic island.

High-risk cutaneous squamous cell carcinoma.

Cutaneous squamous cell carcinoma (cSCC) is the second most common form of skin cancer and its incidence has increased in recent decades. Most cSCCs are successfully treated by surgery, but local and distant metastases develop in approximately 5% of cases; this proportion is higher in certain forms of cSCC with high-risk factors, namely: tumor size >2cm, depth >2mm, Clark level ≥IV, perineural invasion, lymphovascular invasion, poor differentiation, certain histologic subtypes (desmoplastic or adenosquamous carcinoma, invasive Bowen disease, or a cSCC arising in areas of chronic inflammation), immunosuppression, human papillomavirus infection, high-risk anatomic location (pinna of the ear, labial mucosa), expression of certain tumor genes, and inadequate tumor resection. The latest TNM (tumor, lymph node, metastasis) classification of cSCC published by the American Joint Committee on Cancer (AJCC) in the seventh edition of its Cancer Staging Manual now incorporates several of these risk factors to improve disease staging. We review all the factors currently considered to be markers of poor prognosis in cSCC and analyze the new AJCC classification and the different treatment options for high-risk cSCC.

Squamous cell carcinoma of the lip. A retrospective study of 146 patients.

BACKGROUND: Squamous cell carcinoma of the lip (SCCL) accounts for 90% of all tumours of the oral cavity. We present a series of 146 patients with SCCL studied in our hospital. OBJECTIVE: To evaluate and identify the primary tumour parameters related to local and lymph node recurrence. METHODS: We retrospectively analysed the clinical characteristics, surgical procedures and tumour recurrences of the 146 patients diagnosed with SCCL in Hospital Universitario Fundación Alcorcón (Spain). RESULTS: A total of 122 of the 146 patients (91.7%) showed tumour stage ≤ T1N0M0 at diagnosis, and 11 (8.3%) showed stage >T1N0M0. Local recurrences were observed in 11 of the 146 patients (7.5%), and five patients (3.4%) developed lymph node metastases during follow-up. Kaplan-Meier survival analysis showed an increased tumour size to imply a greater risk of local recurrence (P = 0.025). The probability of local recurrence over the 24 months of follow-up was 1% for tumour stages ≤ T1N0M0 and 20% for stages ≥ T1N0M0. There appears to be a greater tendency towards local relapse in male patients, in smokers, patients living in rural areas, in lower lip tumours and in those patients showing infiltration at physical exploration. Eleven patients (8%) died during follow-up, although in only two of them (18%), SCCL was the cause of death. CONCLUSION: The low tumour stage of our patients may explain the few local and lymph node disease recurrences seen in our study. We have shown tumour size to be directly related to the probability of local recurrence.

[Splenic marginal zone B-cell lymphoma with epidermotropic skin involvement]. / Linfoma B esplénico de la zona marginal con afectación cutánea epidermotropa.

Marginal zone B-cell lymphoma (MZL) is subclassified into extranodal MZL of mucosa-associated lymphoid tissue (including cutaneous lymphomas), splenic MZL, and nodal MZL. We report the case of a 68-year-old man with erythematous-violaceous plaques and nodules. Skin biopsy showed an epidermotropic lymphocytic infiltration and cytology and immunohistochemistry were consistent with MZL. The workup revealed disease in the peripheral blood and bone marrow and massive splenomegaly. Splenectomy confirmed the diagnosis of splenic MZL and led to resolution of the skin lesions. Cutaneous recurrence was treated successfully with chemotherapy and rituximab but caused fatal hepatitis due to hepatitis B virus reactivation. Skin involvement by splenic MZL is uncommon; this form of the disease can present epidermotropism, a very rare finding in primary cutaneous MZL. Treatment consists of splenectomy, which may be associated with chemotherapy and/or rituximab; this treatment may lead to reactivation of latent hepatitis B infection and screening for hepatitis should therefore be performed prior to starting therapy.

[Otalgia as presentation of primary nasopharynx tuberculosis]. / Otalgia como presentación de tuberculosis primaria de nasofaringe.

We report a case of a 63-year-old woman who presented with left otalgia during more than six months. On examination, otoscopy was normal, whereas flexible nasopharyngoscopy revealed an asymmetry in the nasopharynx. CT scans confirmed the presence of a left nasopharyngeal mass. A transnasal endoscopic removal of the nasopharyngeal lesion was performed. Necrotizing granulomatosis was found. Tuberculosis of the nasopharynx was considered the final diagnosis, but oral treatment was not started. After 36 months of follow-up, nasal endoscopy and CT images show no sign of recurrence. Discussion is focused on the inclusion of nasopharyngeal diseases in otalgia differential diagnosis, histopathological diagnosis of granulomas and the need of any further treatment for these limited cases of extrapulmonary tuberculosis.

Gastrointestinal autonomic nerve tumours: a case report with ultrastructural and immunohistochemical studies.

A case of gastrointestinal autonomic nerve tumour with light microscopic, immunohistochemical and ultrastructural examination is reported. The tumour was composed of spindle cells or large cells with clear cytoplasm and showed intense staining for vimentin and focal staining for neuron-specific enolase, chromogranin, synaptophysin, gastrin, P substance and S-100 protein. Ultrastructural examination showed long processes with dense core granules and the absence of features characteristic of other gastrointestinal stromal tumours. In addition we noted small traces of basal lamina and the absence of synaptic vesicles. It seems that the biological behaviour of gastrointestinal autonomic nerve tumours is aggressive but there are too few reports on which to conclude anything about their prognosis. Our findings suggest that tumour has a neuroectodermal differentiation.