Follicular Basaloid Hamartoma and Associated Cutaneous Tumors: Clinical, Histopathological and Dermoscopic Aspects.

Background: Basaloid follicular hamartoma is an uncommun benign neoplasm of the hair follicule. The linear form of basaloid follicular hamartoma can be associated with basal cell carcinoma.OBJECTIVE: We report a case of a patient with multiple basal cell carcinomas developing on a congenital lesion allowing the diagnosis of basaloid follicular hamartoma on histopathology. Methods: Each lesion was evaluated by two dermatologists. All biopsy specimens were routinely stained with hematoxylin-eosin. Results: A 76 year-old-man consulted our dermatology departement for erythematous papules spread over the left lower limb. The lesion had been evolving since birth with flesh-colored to pinkish papules following Blaschko's lines from the root of the thigh to the foot. Three months before consultation, the papules increased in size leading to nodules with sometimes an ulcerated center. Dermoscopy under polarized mode displayed for ulcerated lesions (A) yellow-brownish crusts and ulceration surrounded by blue-grey ovoid nests, subtle short white streaks, brown dots and linear/arborising vessels. For nodular lesions (B), dermoscopic features are white pinkish hue, dotted and linear vessels, brown dots, blue-grey structureless areas and white prominent shiny streaks. There were some more erythematous inflamed and eroded areas in the background with a reversed honeycomb white network on dermoscopy (C), polymorphous vessels, whitish scales, ulcerations and milia-like cysts. The background lesion showed varied dermoscopic structures on a flesh colored slightly pinkish bottom (D). Histolopathology concluded for lesions A to an infiltrating and nodular basal cell carcinoma, (B) and (C) to fibroepithelioma of Pinkus and (D) to basaloid follicular hamartoma. Conclusion: Several case reports have documented dermoscopic features of a solitary basaloid follicular hamartoma. However, further studies are required to specify any reproducible features.

Complexity of diagnosing and treating langerhans cell sarcoma: A case report.

INTRODUCTION: Langerhans cell sarcoma (LCS) is a very rare malignant tumor of Langerhans cells that may metastasize to many organs. The diagnosis of this tumor is difficult and its prognosis is poor. AIM: To report the difficulty to diagnose LCS, and discuss therapeutic management of this rare entity. CASE PRESENTATION: We report a case of LCS in a 52-year-old man who presented with an axillar lymphadenopathy. The diagnosis of nodular sclerosis type Hodgkin's disease was established after histologic examination. The patient was treated with chemotherapy (ABVD regimen: Doxorubicin, Bleomycin, Vinblastine, Dacarbazine) and radiotherapy with a partial response. However, disease recurrence was observed and histological analysis confirmed the diagnosis of Langerhans cell sarcoma. A revision of the initial histological examination concluded to the diagnosis of sarcoma from the beginning. We chose the ESHAP (Etoposide, Methylprednisolone, Aracytine, Cisplatin) regimen and clinical improvement of LCS was obtained after 2 cycles but the patient had a fatal outcome and died by disease progression. CONCLUSION: Because of its rarity, diagnosis is difficult and an optimal treatment strategy for this disease has not yet been identified. Polychemotherapy can be an effective modality for the treatment of LCS.

The Prognostic Significance of CD10 Expression in Invasive Breast Carcinoma in Tunisian Patients.

BACKGROUND: Breast cancer is the first female cancer worldwide. Its prognosis depends mainly on pathological stage and histological grade. These classical prognostic factors are essential but may be insufficient to predict the outcome of the disease. Research focuses on identifying new prognostic factors such as CD10, which is a cell surface metalloproteinase. OBJECTIVE: This study aims to evaluate CD10 expression on stromal and tumor cells in invasive breast carcinomas and its correlations with other clinicopathological factors and survival. METHODS: A series of 100 cases of breast carcinoma of no special type diagnosed from 2009 to 2011, was investigated in this study. CD10 expression was detected by immunohistochemistry. Stromal CD10 expression (=10% stromal positivity was considered positive) and tumor cells expression (=1% stained carcinomatous cells) were noted. Statistical correlations were analyzed with different known prognostic parameters; survival analysis were performed using SPSS 22.0. RESULTS: Stromal CD10 expression was seen in 60% of the cases. It showed positive correlation with high tumor grade (p=0,012) and distant metastasis (p=0,02). CD10 expression on tumor cells was observed in 10% of the cases. It was associated with high tumor grade (p=0,009), hormone receptor negativity (estrogen receptor: p<0,0001), progesterone receptor: p=0,005), triplenegative phenotype (p=0,001), and Ki67 overexpression (p=0,046). Stromal CD10 expression was significantly associated to a shorter overall survival (p=0,029) and disease-free survival (p=0,05) in univariate analysis. CONCLUSION: Given these results, it can be concluded that CD10 expression predict an aggressive behavior of breast cancer. This marker can be introduced as a determinant prognostic factor.

Concomitant primary angiosarcoma and invasive carcinoma of the breast: a case report.

Collision tumor is a rare entity composed of two different tumors that occur in close to one another and maintain distinct borders. Only few cases have been reported in the breast. We report the first case of concomitant and adjacent primary angiosarcoma (PBAS) and invasive carcinoma of the breast (IBC), in a 45-year-old patient which presented with a lump in her right breast. Biopsy revealed PBAS. She underwent mastectomy. Gross examination showed a hemorrhagic and spongy tumor in contact with a second small grayish-white mass. Histologically, the hemorrhagic tumor was consistent with a high grade (HG) PBAS; the second mass was consistent with an IBC with no images of histological admixture. The diagnosis of a collision tumor composed of HG PBAS and IBC was established. During follow-up, the patient developed ovarian angiosarcomatous metastasis. The diagnosis of breast collision tumor is very uncommon and hence is challenging for pathologist. Careful gross and microscopic examinations help in establishing the appropriate diagnosis.

A verrucous tumor of the leg: Atypical case of Syringocystadenoma Papilliferum.

Syringocystadenoma Papilliferum (SCAP) is a benign adnexal tumor. Most of the cases present with a solitary lesion in the head and neck region at birth or in early childhood. In fact, only two cases of SCAP on the lower leg have been reported in the literature. We report an atypical case by its location and its clinical aspect.

Erysipeloid cutaneous leishmaniasis: a study of 40 cases of an unusual variant.

BACKGROUND: Erysipeloid cutaneous leishmaniasis (ECL) is known as the chronic form of cutaneous leishmaniasis (CL). However, keeping its clinical presentation in view, there is a need to revisit this form of the disease. AIMS: To describe ECL in view of clinical features and treatment modalities. METHODS: We include a case series seen in Sfax (Southern Tunisia) from January 2017 to January 2021. All patients clinically suggestive and laboratory confirmed with a diagnosis of CL were registered. Patients of all age groups and of either gender having cutaneous lesions resembling erysipela on the face were included in the study. Different demographic features of the patients and clinical aspects were identified. Descriptive statistics were used for analysis. RESULTS: Of 1300 registered patients with CL, 40 (3%) were diagnosed as ECL. Ages ranged from 15 to 65 years, and duration of lesions varied from 15 to 180 days. All patients had lesions over the face. Clinically, a painful infiltrated inflammatory placard of the central facial area with a butterfly shape was observed in 14 cases, as well as zones of the cheekbone (11 cases), cheekbone and nose (5 cases), cheekbone and eyelid (8 cases), and cheekbone with ear (2 cases). Several therapeutic methods were prescribed with a sufficient result with no recurrence. CONCLUSION: ECL is a rare presentation that typically occurs on the face, looking like erysipelas, in patients who are native from an endemic region of CL.

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.

Compound blue nevus: A misleading pigmented melanocytic tumor.

Compound blue nevus had clinical and histological similarities with other heavily pigmented melanocytic tumor, like the pigmented epithelioid melanocytoma. Distinctive genomic aberrations have allowed differentiating it. The defining characteristic of blue nevi family is the presence of activating mutations in the G protein α-subunits, GNAQ and GNA11.

Identification of novel candidate genes by exome sequencing in Tunisian familial male breast cancer patients.

Male Breast Cancer (MBC) is a rare and aggressive disease that is associated with genetic factors. Mutations in BRCA1 and BRCA2 account for 10% of all MBC cases suggesting that other genetic factors are involved. The aim of the present study is to screen whole BRCA1 and BRCA2 exons using the Ampliseq BRCA panel in Tunisian MBC patients with family history. Furthermore, we performed exome sequencing using the TruSight One sequencing panel on an early onset BRCA negative patient. We showed that among the 6 MBC patients, only one (MBC-F1) harbored a novel frameshift mutation in exon 2 of the BRCA2 gene (c.17-20delAAGA, p.Lys6Xfs) resulting in a short BRCA2 protein of only 6 amino-acids. We selected 9 rare variants after applying several filter steps on the exome sequencing data. Among these variants, and based on their role in breast carcinogenesis, we retained 6 candidate genes (MSH5, DCC, ERBB3, NOTCH3, DIAPH1, and DNAH11). Further studies are needed to confirm the association of the selected genes with family MBC.

RIP140 and LCoR expression in gastrointestinal cancers.

The transcription coregulators RIP140 and LCoR are part of a same complex which controls the activity of various transcription factors and cancer cell proliferation. In this study, we have investigated the expression of these two genes in human colorectal and gastric cancers by immunohistochemistry. In both types of tumors, the levels of RIP140 and LCoR appeared highly correlated. Their expression tended to decrease in colorectal cancer as compared to adjacent normal tissues but was found higher in gastric cancer as compared to normal stomach. RIP140 and LCoR expression correlated with TNM and tumor differentiation. Significant correlations were observed with expression levels of key proteins involved in tumor progression and invasion namely E-cadherin and Cyclooxygenase-2. Survival analysis showed that patients with LCoRlow/RIP140high colorectal tumors have a significant prolonged overall and disease-free survival. In gastric cancer, high LCoR expression was identified as an independent marker of poor prognosis suggesting a key role in this malignancy. Altogether, these results demonstrate that RIP140 and LCoR have a prognostic relevance in gastrointestinal cancers and could represent new potential biomarkers in these tumors.