Fibroepithelioma of pinkus in a 9-year-old boy: a case report

Fibroepithelioma of Pinkus (FEP) is a rare indolent variety of basal cell carcinoma that is typically polypoid and located on the trunk of adult males aged 40–60 years. Basal cell carcinoma (including FEP) is very rare in the pediatric population. We are reporting such a case occurring in a 9-year-old boy

Amyloid precursor-like protein 2 (APLP2) affects the actin cytoskeleton and increases pancreatic cancer growth and metastasis.

Amyloid precursor-like protein 2 (APLP2) is aberrantly expressed in pancreatic cancer. Here we showed that APLP2 is increased in pancreatic cancer metastases, particularly in metastatic lesions found in the diaphragm and intestine. Examination of matched human primary tumor-liver metastasis pairs showed that 38.1% of the patients had positive APLP2 expression in both the primary tumor and the corresponding liver metastasis. Stable knock-down of APLP2 expression (with inducible shRNA) in pancreatic cancer cells reduced the ability of these cells to migrate and invade. Loss of APLP2 decreased cortical actin and increased intracellular actin filaments in pancreatic cancer cells. Down-regulation of APLP2 decreased the weight and metastasis of orthotopically transplanted pancreatic tumors in nude mice

Primary Effusion Lymphoma: A Clinicopathological Study of 70 Cases

Primary effusion lymphoma (PEL) is a rare type of large B-cell lymphoma associated with human herpesvirus 8 (HHV8) infection. Patients with PEL usually present with an effusion, but occasionally with an extracavitary mass. In this study, we reported a cohort of 70 patients with PEL: 67 men and 3 women with a median age of 46 years (range 26-91). Of these, 56 (80%) patients had human immunodeficiency virus (HIV) infection, eight were HIV-negative, and six had unknown HIV status. Nineteen (27%) patients had Kaposi sarcoma. Thirty-five (50%) patients presented with effusion only, 27 (39%) had an extracavitary mass or masses only, and eight (11%) had both effusion and extracavitary disease. The lymphoma cells showed plasmablastic, immunoblastic, or anaplastic morphology. All 70 (100%) cases were positive for HHV8. Compared with effusion-only PEL, patients with extracavitary-only PEL were younger (median age, 42 vs. 52 years, p = 0.001), more likely to be HIV-positive (88.9% vs. 68.6%, p = 0.06) and EBV-positive (76.9% vs. 51.9%, p = 0.06), and less often positive for CD45 (69.2% vs. 96.2%, p = 0.01), EMA (26.7% vs. 100%, p = 0.0005), and CD30 (60% vs. 81.5%, p = 0.09). Of 52 (50%) patients with clinical follow-up, 26 died after a median follow-up time of 40.0 months (range 0-96), and the median overall survival was 42.5 months. The median OS for patients with effusion-only and with extracavitary-only PEL were 30.0 and 37.9 months, respectively (p = 0.34), and patients with extracavitary-only PEL had a lower mortality rate at the time of last follow-up (35% vs. 61.5%, p = 0.07). The median OS for HIV-positive and HIV-negative patients were 42.5 and 6.8 months, respectively (p = 0.57), and they had a similar mortality rate of 50% at last follow-up. In conclusion, patients presenting with effusion-only versus extracavitary-only disease are associated with different clinicopathologic features. PEL is an aggressive lymphoma with a poor prognosis, regardless of extracavitary presentation or HIV status

Pediatric-type follicular lymphoma

Background: Pediatric-type follicular lymphoma (PTFL) is a rare distinct type of mature B-cell lymphoma that has characteristic clinical and pathological features. However, it may have morphological overlap with other non-neoplastic condition like reactive follicular hyperplasia (RFH) and neoplastic condition like usual follicular lymphoma (UFL). All these conditions have different treatment considerations and prognoses and therefore they must be differentiated from each other. Aim: Aim of this study is to review the existing literature to understand the clinical, pathological, and molecular features of PTFL with a focus on microscopic and immunophenotypic features to differentiate PTFL from its histological mimicries. Methods: We searched for research papers in PubMed using the key words “pediatric” and “follicular lymphoma” and included the relevant articles for the literature review. In addition, we also reviewed the latest WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Conclusions: PTFL is an indolent tumor mainly presenting as a stage I disease in the head and neck region. Under the microscope, it is mainly composed of large, closely packed, and merging follicles which are positive for CD20, CD10 and BCL6 with a high Ki67 proliferation rate but are negative for BCL2. Particularly, PTFL does not show BCL2, BCL6, IRF4 or MYC translocations. Surgical excision is the current treatment of choice

Primary Langerhans cell histiocytosis of the vulva: Report of a case and brief review of the literature

Primary Langerhans cell histiocytosis (LCH) of the vulva is rare. Fifteen cases of primary cutaneous vulvar LCH have been reported in English literature. We report an additional case of LCH confined to the vulva. In this article, we describe the clinical presentation, histopathology and immunohistochemistry findings of vulvar LCH that are helpful to both gynecologists and pathologists in the diagnosis of this entity. We briefly discuss the pathogenesis of LCH. The debate whether LCH is a reactive or neoplastic entity is still ongoing