The Dilemma of Coexisting Nevoid Hyperkeratosis of the Nipple and Areola in Mycosis Fungoides: A Report of Three Cases

Nevoid hyperkeratosis of the nipple and areola (NHNA) is a rare clinicopathological entity showing persistent and strictly localized hyperkeratotic lesions of the nipple, areola or both with unknown etiopathogenesis. A similar clinical appearance may also be seen in different diseases with specific histopathological features. There are a few anecdotal reports on the association of NHNA with mycosis fungoides (MF), but they do not describe a uniform condition. In this report, we present 3 patients with hyperkeratotic lesions of the nipple and areola associated with MF but showing different histopathological features. We also review similar cases in the literature and discuss possibilities concerning this association. Two of our cases represent the association between MF and NHNA without histopathological features of MF on the nipple-areola complex. The other case represents hyperkeratosis of the nipple and areola with specific histological and immunohistochemical features of MF. Hence, we would like to hypothesize that MF may involve the nipple and areola and have an appearance similar to NHNA. Intriguingly, however, NHNA may occasionally also be seen in association with MF. However, this peculiar association requires further explanation

Diagnosis and management of vulvar Darier disease: A case report

Darier disease is an autosomal dominant disorder with hyperkeratotic papules affecting primarily seborrheic areas of the upper chest, back, forehead, scalp, nasolabial folds, ears, and, less frequently, the oral mucosa. A typical eruption consists of keratotic and crusted skin-colored papules and plaques. Pruritus occurs in 80% of patients, and pain is rare. Lesions can be triggered by exposure to ultraviolet light, heat, or stress. Secondary infections of the lesions are a common complication. A definitive diagnosis is obtained by a biopsy showing histological features such as acantholysis, suprabasal clefts, and “corps rond and grains”. Here we present a 37-year-old woman admitted to the gynecology department with pruritic lesions she had noticed on her vulva and perineum for three months. A vulvar biopsy led to the diagnosis of Darier disease. She was referred to the dermatology department and treated with oral acitretin since systemic retinoids are offered as the first-line treatment of the disease

Underrecognized clinical features of folliculotropic mycosis fungoides: a large clinical series.

Background and Objective: A rare variant of mycosis fungoides (MF), folliculotropic MF (FMF) is characterized by a broad clinical spectrum that primarily includes follicle-based lesions but also many atypical clinical manifestations. The objective of the present study was to conduct a clinical analysis of patients with FMF, with a particular focus on highlighting underrecognized dermatological features

Ki67 Scoring in Pancreatic Neuroendocrine Tumors By a New Method.

Ki67 scoring is required for the grading of pancreatic neuroendocrine tumors. Ongoing debate exists about the best scoring method in terms of accuracy and practicality. Manual counting of cells in camera-captured/printed images is a widely used and accepted method and considered the most reliable one among the manual methods. It requires counting 500 to 2000 cells to determine the Ki67 score accurately and it is time and energy consuming. We investigated the possibility of achieving the same results by counting only a particular fraction of tumor cells in a printed image in a series of 45 (24 grade 1 and 21 grade 2) pancreatic neuroendocrine tumors. After counting Ki67-positive tumor cells in the whole image, the tumor cells were counted within one-tenth of the same image with the aid of a previously prepared grid on an acetate sheet. The cell number obtained was multiplied by 10 to estimate the total cell count and the Ki67 score was calculated. The agreement between the results of the acetate grid and conventional whole-image counting method was assessed. Near-perfect agreement was achieved regarding the total cell count and Ki67 score. The agreement on tumor grade between the two methods was perfect. The time spent on the process was significantly less than that spent on the conventional method. Although it needs to be validated in a larger series, the acetate grid method might be considered an alternative method for Ki67 scoring in neuroendocrine tumors

Papillary Renal Cell Carcinoma Arising in a Lymph Node Metastasis of a Testicular Teratoma: A Very Rare Occurrence

We present a case of a teratoma with somatic type malignancy (TSM) in the form of papillary renal cell carcinoma (pRCC) within supraclavicular and retroperitoneal lymph node metastases of a testicular pure teratoma. Resection of both masses revealed a teratoma without any other germ cell tumor component. A papillary carcinoma component was also detected intermingled with the teratomatous elements. The carcinoma cells displayed eosinophilic cytoplasm and prominent nucleoli. Groups of foamy histiocytes in the fibrovascular cores was a striking finding that brought pRCC to mind. Immunoreactivity for CK7, PAX8, AMACR, CD10, napsin, and vimentin along with morphologic findings confirmed renal cell differentiation. No radiological evidence of a primary renal cell carcinoma was found in the kidney. Consequently, pRCC arising in a teratoma was diagnosed. TSM is described as teratoma with a malignant component that is typically encountered in other organs and tissues. TSM in the form of pRCC is an extremely rare entity. Our case is the second example of a testicular germ cell tumor metastasis with a somatic malignancy in the form of pRCC. In conclusion, carcinomas of renal cell differentiation should be kept in mind as a rare form of TSM, especially in metastatic germ cell tumors

Mixed Epithelial and Stromal Tumor of the Kidney with Sarcomatous Transformation Metastatic to the Lung A Case Report

BACKGROUND: Malignant transformation of mixed epithelial and stromal tumor (MEST) of the kidney is a very rare entity. We report an unusual case with a review of the literature