Primary cutaneous Rosai-Dorfman-Destombes disease with features mimicking IgG4-related disease: A challenging case report and literature review.

Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic disorder affecting lymph nodes as well as extranodal sites. Although cutaneous involvement in RDD is common, primary cutaneous RDD is a distinct and not well-documented entity with unknown aetiology and non-specific clinicopathological features. We report a case of a 57-year-old patient, who presented with an indolent skin nodule in the left sub-nipple area. Surgical excision was performed. Histological examination concluded to the diagnosis of cutaneous RDD with histological features mimicking IgG4-related disease. The diagnosis of systemic RDD with cutaneous involvement was ruled out after exclusion of extracutaneous involvement. No recurrence or systemic progression was observed during follow-up. The diagnosis of primary cutaneous RDD is very uncommon and hence is challenging for pathologist and dermatologist especially with features of IgG4-related disease. Careful systemic and microscopic examinations help in establishing the appropriate diagnosis.

Discordance in receptor status between primary and metastatic breast cancer and overall survival: A single-center analysis.

BACKGROUND: The tumor phenotype may change between primary and metastatic breast cancer. We compared the expression of estrogen receptor (ER), progesterone receptor (PR), and HER2 in a series of primary breast carcinomas (PBC) with their metastatic relapses and analyzed the impact of any changes on survival. MATERIALS AND METHODS: It was a single-center retrospective study, collecting consecutive cases of metastatic breast carcinoma diagnosed in the pathology and medical oncology departments at Habib Bourguiba University Hospital in Sfax, Tunisia. An immunohistochemical study was used to assess ER, PR, and HER2 expression. Overall survival (OS) and post-metastasis survival (PMS) were evaluated using multivariable Cox regression analysis. RESULTS: Our study included 68 patients. ER and PR status changed in 29.4 % and 39.7 % of cases, respectively. Conversions were mainly from positive to negative status (22 % and 23.5 % for ER and PR, respectively). Differences in HER2 status were observed in 19.6 % of cases, with loss of overexpression in 6 patients (10.7 %). Adjuvant trastuzumab therapy and PBC molecular subtype (HR-, HER2+) were associated with HER2 status discordance (p = 0.02 and 0.03, respectively). On multivariable analysis, HR-negative conversion tumors were significantly associated with a worse OS (p = 0.042) and PMS (p < 0.001), compared to HR-concordant positive tumors. CONCLUSION: This study establishes that HR and HER2 status discordance between primary and metastatic breast carcinoma has a prognostic impact on patient outcome. Analyzing these receptors' status in all newly diagnosed cases of metastatic breast carcinoma is strongly recommended and would provide information for changing treatment strategies.

Trichofolliculoma: A Case Series.

Trichofolliculoma (TF) is a rare benign adnexal follicular tumor, described as hamartoma with follicular differentiation according to some authors. It typically appears during adulthood on the face or scalp as an isolated nodule with protrusion of central tufted hairs. We present a retrospective series of eleven patients with histologically confirmed TF to evaluate epidemiological, clinical, and histopathologic characteristics. The mean age at excision was 46 years with extremes ranging from 20 to 75 years. The sex ratio M/F was 0.37. Clinical presentation was a papule or nodule with an average diameter of 6,7mm (2-15 mm), firm with central pit in 54 percent and visible emerging vellus hairs in 18 percent of cases only. The localization was on the face in seven cases (63.6%) and only four cases were located outside the face (scalp [n=2], sub mammary fold [n=1] and shoulder [n=1]). Histologically, a cystically dilated hair follicle containing keratinous material with several mature and immature branched follicular structures is described in all cases. According to our series, TF occurs predominantly in women without age predilection, in the face. Central tufted hairs are only found in a minority of cases corresponding histologically to many secondary vellus hair follicles. In fact, histopathological examination is the gold standard for the diagnosis because clinical diagnosis could be challenging. Histology and dermoscopy may vary according to the age of the lesion. To date, only few case series have been published.

Alveolar Adenoma: A Rare Benign Tumour of the Lung with A Challenging Diagnosis.

Alveolar adenoma is a rare lung benign tumour originating from type II pneumocytes. It presents as a well-defined nodule. In some cases, it is difficult to differentiate from lung cancer. Few cases of this tumour have been reported. We describe here a case of alveolar adenoma in a 63-year-old man discovered incidentally on chest X-ray. The lesion was reported as lepidic adenocarcinoma in bronchoscopic biopsy. The patient underwent a thoracoscopic left lower lobectomy. The histopathological and immunohistochemical examinations resulted in a diagnosis of alveolar adenoma. We report this case to describe its morphological and immunohistochemical characteristics and to emphasize its diagnostic difficulties.

Atypical spindle cell lipomatous tumor of the tongue: A rare entity arising in an unusual location.

Atypical spindle cell/pleomorphic lipomatous tumor (ASCPT) constitutes an emerging entity of lipomatous tumors. It is a benign tumor. It occurs typically in limbs and limb girdles. The occurrence in oral cavity is unusual. The diagnosis of this neoplasm is challenging. Herein, we report a case of ASCPT arising in the tongue.

Immunohistochemical Overexpression of Cyclin D1 in Tunisian Invasive Breast Carcinoma Women.

BACKGROUND: Breast cancer represents the most frequent cancer and cause of death in women worldwide and in Tunisia. Cyclin D1 is a gene of cell cycle regulation. It represents a potential oncogene in invasive breast cancer; however; the results are conflicting. We performed a retrospective study aiming to analyze the prognostic impact of cyclin D1 expression in patients with invasive breast carcinoma of no special type and its relation with clinical-pathological features. METHODS: One hundred cases of invasive breast carcinoma of no special type diagnosed between 2009 and 2011 were included in this study. Immunohistochemical (IHC) staining was performed for cyclin D1 in all cases. Results were analyzed statistically. RESULTS: Cyclin D1 positivity was seen in 74 cases (74%), of which 32 cases (32%) showed strong immunoreactivity. Cyclin D1 staining was statistically significantly associated with estrogen receptor (ER) and progesterone receptor (PR) positivity (P<0.0001) and with low grade SBR (P=0.007). None of the clinical data and other pathological features had any association with cyclin D1 expression (P>0.05). Univariate analysis revealed that expression of cyclin D1 was not statistically associated with overall survival (OS) and disease-free survival (DFS) (P=0.459 and P=0.564, respectively). CONCLUSION: These results confirm that cyclin D1 overexpression can be employed as a beneficial prognostic marker and suggest that anti-cyclin D1 therapy may be efficient, especially for ER positive tumors.