Familial papular epidermal nevus with "skyline" basal cell layer and multiple pilomatricomas: A new association?

Papular epidermal nevus with "skyline" basal cell layer is a newly described keratinocytic nevus. Recently, papular epidermal nevus with "skyline" basal cell layer has been reported in association with extracutaneous involvement, and the term papular epidermal nevus with "skyline" basal cell layer syndrome is used to indicate a neurocutaneous syndrome characterized by the presence of papular epidermal nevus with "skyline" basal cell layer and different neurologic symptoms that seem to improve during infancy and adolescence. Multiple pilomatricomas have been reported in association with various syndromes. We report herein papular epidermal nevus with "skyline" basal cell layer associated with multiple pilomatricomas in two members of a family with the aim of drawing attention to this peculiar epidermal nevus to improve our knowledge of the syndrome.

Ossifying trichilemmal cyst.

Although a trichilemmal cyst is a relatively common benign cutaneous condition and ossification has been observed within many cutaneous lesions to our knowledge, the association between ossification and trichilemmal cyst has not been previously reported. We describe for the first time the case of a trichilemmal cyst with marked osseous metaplasia arising on the scalp of a 46 year-old woman. Microscopically the lesion showed the typical features of a trichilemmal cyst with the finding of intra-extraluminal foci of calcification and the exceptional feature of mature bone formation. No areas of mature cartilage were observed near the focus of ossification. The cutaneous bone may have been directly formed from osteogenic stromal elements without a cartilaginous precursor (membranous or mesenchymal ossification). The dystrophic calcification might also contribute to the bone-forming process.

Melanosis tubae: histogenesis and appropriate terminology.

We report 2 cases of abnormal localized deposition of lipofuscin-laden macrophages in the lamina propria of the fallopian tube. In both cases there was evidence of ovarian and tubal endometriosis. The authors propose the term "melanosis tubae" (MT) as the most appropriate terminology in the diagnosis of the disease, whose pathogenesis is similar to that occurring in gastrointestinal tract pigmented conditions. The lesion is not of an inflammatory nature so that the term "pseudoxanthomatous salpingitis" is inaccurate and should not be used. Other definitions such as "pseudoxanthomatous salpingiosis," "pigmentosis salpingiosis," "pigmentosis tubae" may be used, but these are descriptive terms that are not suitable for denoting histogenesis. None of the previously reported MT cases showed coexisting tubal endometriosis. Our cases are the first examples of MT associated with tubal endometriosis.

Clinical Features, Dermoscopic Patterns, and Histological Diagnostic Model for Melanocytic Tumors of Uncertain Malignant Potential (MELTUMP).

Cutaneous melanocytic lesions with atypical histological features can be difficult to categorize as benign or malignant. In the diagnosis of melanocytic lesions, the melanocytic tumor of uncertain malignant potential (MELTUMP) category has been widely used. Although one may favor a benign or malignant interpretation, a definitive diagnosis is not always possible, and long term clinical follow-up remains the only true evidence of biological behavior. We report 14 cases of MELTUMP with expert second opinion. Clinical pictures were available in 8 cases; dermoscopy was available in 5 cases. Accurate guidelines are delineated in the formulation of the diagnosis. We think that the histological diagnosis should be accompanied by a note in which the pathologist describes the histological pattern that has generated diagnostic uncertainty. Since the MELTUMP term does not exclude the malignant nature of the lesion, all microstaging attributes for melanoma should be added. Moreover, superficial atypical melanocytic proliferation of uncertain significance (SAMPUS) and MELTUMP categories should be included in the WHO classification of melanocytic tumors of the skin. The role of sentinel lymph node biopsy in MELTUMP has not yet been established. Recent studies have looked at concurrent tumor deposits in lymph nodes of MELTUMP, mostly of atypical Spitzoid lesions, and shown that these lesions rarely progress to overt malignancy. In our study, sentinel node metastasis was found in only one case. The follow-up period of this case and of the others has shown that the clinical outcome of MELTUMP tends to be favorable.

An unusual pathological finding of chronic lymphocitic leukemia and adenocarcinoma of the prostate after transurethral resection for complete urinary retention: case report.

BACKGROUND: We describe a patient who underwent transurethral resection of the prostate for urinary obstructive symptoms and had histological findings of adenocarcinoma of the prostate with prostatic localization of chronic lymphocitic leukemia (CLL). The contemporary presence of CLL, adenocarcinoma of the prostate and residual prostatic gland after transurethral resection has never been reported before and the authors illustrate how they managed this unusual patient. CASE PRESENTATION: A 79-years-old white man, presented with acute urinary retention, had a peripheral blood count with an elevated lymphocytosis (21.250/mL) with a differential of 65.3% lymphocytes and the prostate-specific antigen (PSA) value was 3.38 ng/mL with a percent free PSA of 8.28%. The transrectal ultrasound (TRUS) indicated an isoechonic and homogenic enlarged prostate of 42 cm3 and the abdomen ultrasound found a modest splenomegaly and no peripheral lymphadenophaty. The patient underwent transurethral resection of the prostate and had a pathological finding of adenocarcinoma in the prostate with a Gleason Score 4 (2+2) of less than 5% of the material (clinical stage T1a), associated with a diffused infiltration of chronic lymphocitic leukemia elements. CONCLUSIONS: The incidental finding of a prostatic localization of a low-grade non-Hodgkin's lymphoma does not modify eventually further treatments for neither prostate cancer nor lymphoma. The presence of a low-grade and low-stage lymphoma, confirmed by a hematological evaluation, and the simultaneous evidence of an adenocarcinoma after transurethral resection of the prostate for acute urinary retention do not require any immediate treatment due to its long-term survival rate and the follow-up remains based on periodical PSA evaluation and complete blood count.

Coexistence of atypical adenoma, adenoma with bizarre nuclei and follicular variant of papillary carcinoma of the thyroid.

Coexistence of atypical adenoma, adenoma with bizarre nuclei and follicular variant of papillary carcinoma is described in the same thyroid, with study of p53 expression. A 72-year-old woman presented to the endocrinology out-patient unit for a routine follow-up visit. Patient underwent a total thyroidectomy. Histological examination revealed a solid nodular neoplasm with the longest diameter of 0.8 cm in the upper pole of the left lobe. The neoplasm showed the histological features of follicular variant of papillary carcinoma with moderate diffuse immunoreactivity for p53. The lower pole of the right lobe showed two adjacent nodules with histological features respectively of atypical adenoma and adenoma with bizarre nuclei, with strong diffuse immunoreactivity for p53.

HMB-45 negative clear cell perivascular epithelioid cell tumor of the skin.

The first case of cutaneous clear cell perivascular epithelioid cell tumor (PEComa) with negative HMB-45 marker is presented. The tumor was a nodule 3x2 cm in size, located on the right foot in a 60-year-old man. The lesion consisted of large irregularly shaped cells with clear cytoplasm, negative for S-100 protein, HMB-45, Melan-A, pancytokeratin, epithelial membrane antigen and CAM5.2. Multifocal positivity for desmin, microphthalmia transcription factor and tyrosinase was found. The diagnosis of cutaneous PEComa of clear cell type was made. Clear cell change is a very unusual finding in PEComa and may pose problems in diagnostic differentiation from other clear cell cutaneous lesions that may be excluded with immunohistochemistry. In our case, the HMB-45 negativity may be explained by extensive clear cell change. Additional studies are necessary to accept the clear cell cutaneous HMB-45 negative PEComa as a new variant of perivascular epithelioid cell tumor.

Peritoneal Malignant Psammomatous Mesothelioma.

Psammoma bodies (PBs) are observed most commonly in papillary thyroid carcinoma, meningioma, and papillary serous cystadenocarcinoma of the ovary. We report one case of peritoneal malignant mesothelioma (PMM) with massive deposition of PBs. A 72-years-old man presented with abdominal swelling and marked weight loss. Contrast enhanced computed tomography showed fluid diffuse in peritoneal recesses, thick septa with micronodules in the greater omentum and adjacent enhancement of the thickened peritoneum. The explorative laparoscopy showed diffuse minute parietal peritoneal nodules. The peritoneal biopsy revealed a superficial papillary growth of malignant epithelial-like cells with diffuse involvement of submesothelial tissues. Massive deposition of PBs was observed. Nuclear and cytoplasmic calretinin immunoreactivity was present in neoplastic cells along with membranous D2-40 and membranous/cytoplasmic cytokeratin 5 staining. The patient was treated with chemotherapy (gemcitabine, vinorelbine, cisplatin). PBs may represent an active biologic process ultimately leading to degeneration/death of tumor cells and retardation of growth of the neoplasm. It may also serve as a barrier against the spread of tumor. Psammomatous malignant mesothelioma may simulate serous psammocarcinoma of the peritoneum. The behavior of serous psammocarcinoma is more closely similar to borderline serous tumor than to serous carcinoma. Further studies are necessary to establish if massive deposition of PBs may define a new variant of psammomatous malignant mesothelioma with a favorable impact to the prognosis of usual psammomatous malignant mesothelioma, as well as in serous psammocarcinoma of the peritoneum.