Erdheim-Chester Disease with Renal Mass Presentation: Report of the First Case From Palestine and a Review of the Literature.

BACKGROUND Erdheim-Chester disease (ECD), a form of non-Langerhans-cell histiocytosis, is extremely rare. The mean age of individuals with ECD is in their 50s. Histiocytic infiltration of vital organ systems is a potential cause of substantial morbidity, which is associated with the multisystemic form of ECD. This report presents the first case of ECD with renal abnormalities in Palestine. CASE REPORT A 54-year-old woman with no medical or surgical history presented with 6 months of bilateral flank pain with no radiation or fever. A physical examination revealed only bilateral flank pain. Urine tests showed microhematuria. Laboratory test results showed increased serum creatinine levels (1.21 mg/dL) and microcytic anemia. A CT scan revealed significant multi-organ abnormalities, including renal abnormalities with a hairy kidney sign, pericardial effusion, and an osteolytic lesion of the spine. The hairy kidney sign is pathognomonic for ECD, so the renal mass was biopsied to confirm the diagnosis. The biopsy showed foamy histiocytes, lymphocytes, and plasma cells. Foamy histiocytes were CD68-positive and negative for S100, CD1a, and HMB45. PAx5 and CD3 immunostaining showed T-predominant B-lymphocyte mixtures. CONCLUSIONS In the setting of systemic symptoms and imaging abnormalities such as presence of the hairy kidney sign, pericardial effusion, and osteolytic lesion of the spine, it is necessary to examine the possibility of ECD and proceed with a biopsy for confirmation. This is the first case in Palestine to be reported and the second case worldwide with a renal mass as an atypical presentation.

Primary Malignant Rhabdoid Tumor of the Liver in a 5-Month-Old Female Who Presented with Abdominal Distension.

BACKGROUND Malignant rhabdoid tumors are rare and aggressive pediatric tumors that usually arise in the kidney and have a characteristic appearance on histology. Extrarenal malignant rhabdoid tumors originating in the liver are extremely rare. This report is of a 5-month-old girl who presented with a rapidly enlarging abdominal mass due to a malignant rhabdoid tumor of the liver. CASE REPORT A 5-month-old female patient with no known medical history had been experiencing increasing abdomen distention and less overall activity for 1 month, according to her parents. Abdominal ultrasonography was used for diagnostic purposes, and the results showed the presence of a mass with a solid and cystic appearance in the upper left quadrant of the abdomen. The patient was transferred to a tertiary care hospital for further investigations. The laboratory test results indicated a hemoglobin level of 8.2 g/dL, and the liver function tests were within the reference range. However, the serum tumor marker alpha-fetoprotein level was 1310 ng/mL, while the b-human chorionic gonadotropin was within range. Computed tomography detected a nonspecific heterogeneous mass of the liver. Histopathology revealed discohesive cells with rich eosinophilic cytoplasm, eccentric nuclei, and large central nucleoli (rhabdoid appearance). Immunohistochemistry showed negative integrase interactor 1 protein expression. The diagnosis was a primary malignant rhabdoid tumor located in the liver. CONCLUSIONS This report shows the importance of combined diagnostic imaging and histopathology analysis to confirm the diagnosis of rare pediatric tumors, including malignant rhabdoid tumor, to ensure early diagnosis and appropriate treatment.

Abnormal Presentation and Challenging Diagnosis of Mediastinal Lymphoma: A Case Report.

We describe a rare case of classical Hodgkin lymphoma (HL) in a 20-year-old female patient. She presented to our hospital with chest wall swelling after months of post-chest trauma management. The swelling was initially treated as an infected hematoma, and the patient was referred for surgical evacuation. During the surgery, the swelling was found to be a mass that extended to the mediastinum. A biopsy was taken, which later resulted in the diagnosis of a granular cell tumor (GCT). A decision on surgical resection by video-assisted thoracoscopic surgery (VATS) was taken after discussion with the multidisciplinary team of surgery, cardiothoracic surgery, oncology, pathology, and radiology. During the surgery, a frozen section biopsy was taken, which showed no features of lymphoma or granular cell tumors. The surgery was followed by a midline sternotomy to control the bleeding from an accidentally injured major vessel. The bleeding was controlled, and the mass was dissected and sent for histopathological examination. The histopathology showed nodular classical HL features, and the patient was referred for chemotherapy. In our case, the patient was initially diagnosed with GCT, but with complete resection and an adequate biopsy, the mass was found to be a classical HL. Possible cross-cellularity is questioned, and the possible correlation between the two findings encouraged us to report this case.

Noncovalent functionalization of carbon nanotubes as a scaffold for tissue engineering.

Tissue engineering is one of the hot topics in recent research that needs special requirements. It depends on the development of scaffolds that allow tissue formation with certain characteristics, carbon nanotubes (CNTs)-collagen composite attracted the attention of the researchers with this respect. However, CNTs suffer from low water dispersibility, which hampered their utilization. Therefore, we aim to functionalize CNTs non-covalently with pyrene moiety using an appropriate hydrophilic linker derivatized from polyethylene glycol (PEG) terminated with hydroxyl or carboxyl group to disperse them in water. The functionalization of the CNTs is successfully confirmed by TEM, absorption spectroscopy, TGA, and zeta potential analysis. 3T3 cells-based engineered connective tissues (ECTs) are generated with different concentrations of the functionalized CNTs (f-CNTs). These tissues show a significant enhancement in electrical conductivity at a concentration of 0.025%, however, the cell viability is reduced by about 10 to 20%. All ECTs containing f-CNTs show a significant reduction in tissue fibrosis and matrix porosity relative to the control tissues. Taken together, the developed constructs show great potential for further in vivo studies as engineered tissue.

Enhancement of wound healing by single-wall/multi-wall carbon nanotubes complexed with chitosan.

BACKGROUND: Impaired wound healing is commonly associated with many health problems, including diabetes, bedsores and extensive burns. In such cases, healing often takes a long time, which subjects patients to various complications. This study aims to investigate whether single-wall or multi-wall carbon nanotubes complexed with chitosan hydrogel can improve wound healing. MATERIALS AND METHODS: Initially, the effects of the complexes on the viability and functionality of fibroblasts were investigated in engineered connective tissues. Then, their activity on wound healing was investigated in a mouse model with induced full-thickness wounds, in which the wounds were treated daily with these complexes. Finally, the effect of the complexes on collagen deposition by fibroblasts was investigated in vitro. RESULTS: The engineered connective tissue studies showed that fibroblasts were viable in the presence of the complexes and were still able to effectively organize and contract the extracellular matrix. In vivo data showed that both types of complexes improved the re-epithelialization of the healing wounds; however, they also increased the percentage of wounds with higher fibrosis. In particular, the chitosan-multi-wall carbon nanotube complex significantly enhanced the extensiveness of this fibrosis, which is in line with in vitro data showing a concentration-dependent enhancement of collage deposition by these complexes. These observations were associated with an increase in inflammatory signs in the wound bed. CONCLUSION: Single-wall and multi-wall carbon nanotubes complexed with chitosan improved the re-epithelialization of wounds, but an increase in fibrosis was detected.

Topical aqueous extract of Ephedra alata can improve wound healing in an animal model.

PURPOSE: Ephedra alata (E. alata) is perennial tough shrub plant that grows in Palestine and other regions. It is used often in folk's medicine for the treatment of various diseases. In this project, E. alata extract was tested for its ability to improve wound and burn healing. METHODS: An aqueous extract of E. alata was prepared and underwent several phytochemical analyses for the presence of the major classes of phytochemical compounds. After that, a polyethylene glycol-based ointment containing the extract of E. alata was prepared and its wound and burn healing activities were tested in-vivo using an animal model for deep wound and full thickness skin burn. The effect was compared against a placebo ointment. Skin biopsies were evaluated by a blinded clinical histopathologist, in addition to digital analysis. RESULTS: Phytochemical analysis demonstrated the presence of the major classes of phytochemical compounds in the prepared extract including flavonoids, alkaloids, phytosteroids, phenolic compounds, volatile oils and tannins. As compared to placebo ointment, E. alata ointment significantly improved the healing of the wound ulcers, whereas it showed no advantage on the quality of the healing of burn ulcers. CONCLUSION: E. alata extract is rich in phytochemical compounds and can improve wound healing when applied topically.

Epstein-Barr virus and Hodgkin lymphoma in Jordan.

BACKGROUND AND OBJECTIVES: This study aims to examine the possible association of Epstein-Barr virus (EBV) in Classical Hodgkin lymphoma (cHL) and to shed light on the epidemiology of Hodgkin lymphoma (HL) in Jordan. PATIENTS AND METHODS: We examined 100 consecutive cases of HL for the presence of EBV in tumor cells by immunohistochemistry for latent membrane protein-1 (LMP-1). We collected patient data on age, sex and histologic subtype. We reviewed the pathological findings in each case and confirmed diagnosis. RESULTS: Nodular lymphocyte predominant HL was diagnosed in 6% of the cases and these were negative for EBV LMP-1. Of the 94 cases of cHL, 65% were males, the most common subtype was nodular sclerosis (NS), representing 70% of the cases, and 45.7% of cHL (43% of all HL) cases were positive for EBV LMP-1. The positive cases were significantly related to age: ⩽15 years and >51 years (p: 0.009 and 0.014 respectively), male gender (p: 0.03) and mixed cellularity (MC) subtype (p: <0.0001). In line with other developing countries, there also appears to be a trend towards a decreasing association of EBV with cHL and a subtype switch from MC to NS in Jordan. CONCLUSION: The epidemiology of HL in Jordan and some developing countries is approaching that of developed countries.