Clinicopathologic Features of Hydroa Vacciniforme-Like Lymphoma: A Series of 9 Patients.

Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14.5 years; median age, 13.3 years; age range, 4-27 years), expanding on previous observations of this elusive disease. The most common clinical aspects were persistent facial edema with necroses and pitted scars. Histopathological analyses revealed variably dense lymphoid infiltrates with common angiodestructive features. Neoplastic cells expressed CD3 and cytotoxic markers in all cases and were constantly positive for Epstein-Barr virus (EBER-1). Expression of other markers was variable. Follow-up data revealed that all patients died within 6 months or less, thus showing a very aggressive course with poor prognosis.

[Role of viruses with oncogenic potential and their associations in oncogenesis in maxillofacial area].

The potential role of viruses with oncogenic potential such as human papilloma virus, cytomegalovirus, Epstein-Barr virus, herpes simplex virus type 1 and 2, herpes virus type 6, in the development of benign and pre-cancerous tumors of maxillofacial region was assessed in the study. We examined 26 patients with tumors in maxillofacial region (skin and mucosa) using molecular-genetic and histological studies of surgically removed neoplasms removed. In 53.8% of the examined samples DNA of Epstein-Barr virus, herpes virus type 6, herpes simplex virus type 1, or cytomegalovirus and in 35.7% of them the association of the above mentioned viruses were detected. It may confirm their relation with the development of benign, precancerous and malignant neoplasms in maxillofacial region.

Epstein-Barr virus-positive diffuse large B-cell lymphoma in an elderly patient.

Epstein-Barr virus-positive (EBV) diffuse large B-cell lymphoma (DLCBL) of the elderly is a newly described lymphoproliferative disorder that arises in elderly patients without a predisposing immunodeficiency. Clinical features at presentation may include lymphadenopathy, B-symptoms and extranodal involvement. The main sites of extranodal involvement are the skin, lung, tonsil and stomach. Histopathological findings include atypical large lymphoid cells with variable amounts of reactive cells, such as small lymphocytes, plasma cells and histiocytes. The neoplastic cells are positive for CD20, and in situ hybridization for EBV-encoded RNA is positive in the majority of neoplastic cells. We present a new case of EBV-positive DLBCL in an 85-year-old man, who presented to our clinic with a 2-month history of asymptomatic cutaneous lesions involving his face and scalp.

Metachronous Merkel cell carcinoma on both cheeks.

Merkel cell carcinoma (MCC), an aggressive skin cancer with neuroendocrine features, has been found to be associated with a new type of human polyomavirus called Merkel cell polyomavirus (MCV). Patients diagnosed with MCC have a significantly increased risk of a second primary cancer. We report here the first case of two primary MCCs arising on the face at different times, associated with MCV infection. The tumour on the patient's right cheek was surgically removed, followed by chemoradiation. After a 10-year tumour-free period, a new tumour developed on the patient's left cheek. Histological and immunohistochemical findings were consistent with MCC. The tumours had high MCV copy numbers and expressed large T antigen, which may play a major role in MCV-mediated carcinogenesis. This case highlights the close links between MCC and MCV.

Disseminated Kaposi's sarcoma and HCV infection: only a casual relationship? A case report.

The authors present a case of disseminated Kaposi's sarcoma in a male patient, HIV negative and Hepatitis C virus (HCV) positive. Although it is well-known that in HCV positive patients the onset of cutaneous diseases such as porphyria cutanea tarda, mixed essential cryoglobulinemia, lichen planus, polyarteritis nodosa, itch/prurigo, is possible, papers on its association with disseminated Kaposi's sarcoma in HIV negative patients are rare in the literature. Such an association is probably not a matter of chance: in fact, the changes to the immune system induced by the HCV virus, in synergy with those induced by the Human Herpetic virus-8, could likewise play a role in the development of Kaposi's sarcoma as happens in patients with immunodeficiency .

'Bowenoid wart': wart with bowenoid histological changes? Report of 9 cases.

INTRODUCTION: Cases of lesions that simulate Bowen's disease have been previously described in the literature. CASE REPORT: Nine exophytic verruca-like lesions with histological findings of Bowen's disease (BD) are described. All cases had a rapid growth, and were located on the face and neck of elderly patients with chronic solar skin damage. We carried out p16 immunohistochemical staining using the immunoperoxidase technique, which was negative in all cases. DISCUSSION: We think that these 9 lesions are only histologically mimicking BD, and could be a subtype of verruca ('bowenoid wart'). These lesions could be provoked by nononcogenic human papillomavirus (HPV), as in other cases previously described. The p16 staining was negative in all cases, in contrast with most BD cases. It would be interesting to study whether positive p16 staining is related to oncogenic HPV, whereas negative p16 staining could be associated with low or nononcogenic HPV; thus, more studies are needed.

Absence of Epstein-Barr virus, human papillomavirus, and simian virus 40 in patients of central european origin with lymphoepithelioma-like carcinoma of the skin.

The authors report 10 cases of lymphoepithelioma-like carcinoma (LELC) of the skin and the results of a molecular biological study for HPV, EBV, and SV40 in lesional tissues. All patients originated from Central Europe. There were seven men and three women, ranging in age from 57 to 86 years. Locations included the face (n = 4), scalp (n = 2), penis (n = 2), and retroauricular area (n = 1); location was unknown for one subject. All but two patients presented with a tumor confined to the skin; in both patients with the penile carcinoma, the tumors had metastasized to an inguinal lymph node. Six patients with available follow-up included four individuals with no evidence of tumor metastasis or recurrences at 2, 3, 4, and 5 years, one patient who died with metastatic disease 7 years after diagnosis, and one patient who died of an unrelated course. Microscopically, all cases showed distinctive features of LELC characterized by variably sized and shaped nodules or syncytial sheets of epithelial cells that contained vesicular chromatic and prominent nucleoli and that were permeated and surrounded by small, well-differentiated lymphocytes and plasma cells. Because all 10 cases studied proved negative for EBV, HPV, and SV40, these viruses seem to play no causal role in LELC of the skin in patients from Central Europe.

Coincidental consort clear cell cutaneous carcinoma: facial squamous cell carcinoma in situ containing human papillomavirus and cancer cells with clear cytoplasm in an octogenarian couple.

Clear cell squamous cell carcinoma in situ, also referred to as pagetoid or clear cell Bowen disease, is a rare pathologic variant of this neoplasm. It is characterized by neoplastic cells with clear or pale cytoplasm. An octogenarian husband and wife concurrently developed new facial skin lesions which demonstrated squamous cell carcinoma in situ consisting of cancer cells with clear cytoplasm. Cutaneous human papillomavirus (HPV) typing detected HPV Type 5 and HPV Type 21 in the tumors of the husband and wife, respectively. HPV is a potential etiologic factor in the oncogenesis of nonmelanoma skin cancer, and HPV DNA has been demonstrated in extragenital squamous cell carcinoma in situ. The detection of DNA from different HPV types in the tumors of our patients suggests that the concurrent occurrence of their skin cancers may have been coincidental. However, the presence of HPV DNA in their tumors introduces the possibility of a viral-associated oncogenesis for clear cell squamous cell carcinoma in situ.

Extranodal NK/T-cell lymphoma mimicking cellulitis.

NK/T-cell lymphoma is difficult to diagnose because there is no characteristic cytology to help the diagnosis in tissue sections, particularly when there is polymorphic cellular infiltration in the early stage of the disease. However, the nasal type of extranodal NK/T-cell lymphoma has a characteristic histologic pattern, which is angiocentric, angioinvasive and angiodestructive. Therefore, many cases of this tumor may show extensive necrosis that mimics infectious process. Furthermore, because the immunosuppressive status of these patients, they may, in fact, have superimposed infections. We are reporting a case that presented as cellulitis and only after careful examination with immunohistochemistry that a correct diagnosis of extranodal NK/T-cell lymphoma, nasal type, was established. Since this lymphoma is incurable and immunophenotyping is instrumental for the diagnosis and prediction of the prognosis, a high index of suspicion for this tumor is needed when an angiocentric lesion is found in the midline of the head and neck region, and a thorough immunohistological study should always be conducted in these cases.

Photodynamic therapy of virus-associated epithelial tumours of the face in organ transplant recipients.

PURPOSE: The benefit for organ recipients is still counteracted by the side effects of immunosuppression. Among other effects, there is a 50-250 times increased risk of developing malignant skin tumours. Because these malignomas are known to develop particularly aggressivly, there is a special need for an efficient therapy. Here we demonstrate the treatment response to aminolaevulinic acid (ALA)-based photodynamic therapy (PDT) in these patients. METHODS: Five organ recipients with multiple tumours of the face were multifocally treated with ALA-PDT (32 tumours in all). After topical application of ALA using a thermogel, irradiation was done with a 635 nm diode laser (Ceralas 635, Biolitec, Jena, Germany). After intervals of 2 weeks, 4 weeks, and 12 weeks, therapeutic efficacy was assessed. RESULTS: There was complete remission in 24 tumours (75%). In six tumours (18.8%) a second or third PDT session was necessary for complete clinical remission. In two tumours (5.6%, invasive squamous cell carcinomas) the lesions were refractory to PDT. CONCLUSIONS: ALA-PDT is a valuable therapeutic alternative for the treatment of multifocal skin tumours in organ-transplanted patients. Furthermore, we see a growing role of ALA-PDT also for patients with frequently relapsing tumours of the skin with known genetically determined tumourigenesis (Gorlin-Goltz syndrome).

The therapy of virus-associated epithelial tumors of the face and the lips in organ transplant recipients.

The risk of developing malignant cutaneous neoplasms is increased after organ transplantation. We report three patients with malignant tumors of the epithelium of the facial skin and the lips after kidney and heart transplantation, respectively. They showed an aggressive course of the disease with more than five synchronous or metachronous basal cell and squamous cell carcinomas. Tissue samples were Epstein-Barr virus (EBV) positive by PCR. Using an in situ hybridization technique EBV-encoded RNA (EBER) was detected in tumor-infiltrating lymphocytes. The aggressive course was not alone controllable by surgical or radiological therapy. The systemic and topical application of cidofovir (Vistide) led to remarkable remissions, to a better confinement and operability of the tumors, and to a cessation of tumor pain. The photodynamic therapy represents another opportunity for managing superficial local recurrences and multiple tumors. In conclusion, the results of these case reports demonstrate that combined antiviral, photodynamic and surgical therapy may be used successfully to treat aggressive cutaneous malignancies in patients after organ transplantation.

Nine-year follow-up of a case of Grzybowski type multiple keratoacanthomas and failure to demonstrate human papillomavirus.

We describe a patient with a 9-year history of generalized eruptive keratoacanthoma (KA) of the Grzybowski type whose multiple skin lesions showed steady progression, resulting in a sclerotic, mask-like facial expression and ectropion. Eleven tumour biopsies representing lesions of different stages and localizations (erupting and regressing KAs, biopsies from non-involved light-protected and light-exposed skin, dermatosclerosis and squamous cell carcinomas) were analysed for human papillomavirus (HPV) sequences using a polymerase chain reaction approach capable of detecting the majority of all presently known HPV genotypes. None of the biopsy specimens proved to be HPV-positive, although HPV was detected in weakly and heavily affected control specimens by the method applied. These findings suggest an HPV-independent aetiology of this rare type of multiple KA.