DIAPH1-Deficiency is Associated with Major T, NK and ILC Defects in Humans.

Loss of function mutations in Diaphanous related formin 1 (DIAPH1) are associated with seizures, cortical blindness, and microcephaly syndrome (SCBMS) and are recently linked to combined immunodeficiency. However, the extent of defects in T and innate lymphoid cells (ILCs) remain unexplored. Herein, we characterized the primary T, natural killer (NK) and helper ILCs of six patients carrying two novel loss of function mutation in DIAPH1 and Jurkat cells after DIAPH1 knockdown. Mutations were identified by whole exome sequencing. T-cell immunophenotyping, proliferation, migration, cytokine signaling, survival, and NK cell cytotoxicity were studied via flow cytometry-based assays, confocal microscopy, and real-time qPCR. CD4+ T cell proteome was analyzed by mass spectrometry. p.R351* and p.R322*variants led to a significant reduction in the DIAPH1 mRNA and protein levels. DIAPH1-deficient T cells showed proliferation, activation, as well as TCR-mediated signaling defects. DIAPH1-deficient PBMCs also displayed impaired transwell migration, defective STAT5 phosphorylation in response to IL-2, IL-7 and IL-15. In vitro generation/expansion of Treg cells from naïve T cells was significantly reduced. shRNA-mediated silencing of DIAPH1 in Jurkat cells reduced DIAPH1 protein level and inhibited T cell proliferation and IL-2/STAT5 axis. Additionally, NK cells from patients had diminished cytotoxic activity, function and IL-2/STAT5 axis. Lastly, DIAPH1-deficient patients' peripheral blood contained dramatically reduced numbers of all helper ILC subsets. DIAPH1 deficiency results in major functional defects in T, NK cells and helper ILCs underlining the critical role of formin DIAPH1 in the biology of those cell subsets.

The refractory secondary hyperparathyroidism presenting with retro-orbital brown tumor: a case report.

BACKGROUND: Tertiary hyperparathyroidism describes the autonomous and excessive secretion of parathyroid hormone (PTH) by the parathyroid glands after longstanding secondary hyperparathyroidism in chronic kidney disease. Brown tumors are a sign of uncontrolled hyperparathyroidism. In this case, we have reported a refractory and destructive hyperparathyroidism storm. Also, it presented with atypical onset and unexpected adenoma location. CASE PRESENTATION: A 37-year-old man was diagnosed with end-stage kidney disease 22 years ago. He has been undergoing dialysis treatment since that time. Recently, he was admitted to the ophthalmology department due to the unilateral anterior bulging of the right eye and drooping of the eyelid. Magnetic resonance imaging exhibited an extraconal mass lesion located in the right orbital posterior superolateral position. Computerized tomography scans considered expansile bone lesion with peripheral calcification and originating from the sphenoid wing. The bone mass lesion was resected via craniotomy due to the compressive effect. The pathological findings were consistent with brown tumors. Plasma intact PTH level was 4557 pg/mL. The patient informed that he underwent parathyroidectomy and two leg fractures operation in a medical query. Parathyroid scintigraphy determined three distinct foci consistent with adenomas and one of them was in mediastenum. Second parathyroidectomy was recommended to the patient but the patient refused surgery. Despite his medication and dialysis regimen being revised, PTH levels were maintained at higher levels in follow-up. CONCLUSIONS: We presented a hyperparathyroidism case that was resistant to all treatments and exhibited all the severe complications in a long-term dialysis patient. Furthermore, this case has revealed the importance and difficulty of secondary hyperparathyroidism management.

High-risk human papillomavirus in Turkish patients with clinically suspicious cervical lesions analyzed by multiplex-PCR.

Background & objectives: Human papillomavirus (HPV) infection is known to be the main cause of cervical cancer. This study aimed to determine the prevalence of high-risk HPV genotypes in smear specimens taken from women who had normal or abnormal cytology using a multiplex PCR method. Methods: The study included 270 women aged between 19 and 69 yr with or without suspicious cervical abnormalities. A Pap smear sample from each patient was cytologically examined, and HPV typing was performed using a multiplex fluorescent PCR method. Those who were high-risk HPV positive and had a normal or abnormal cytology were further evaluated by colposcopy and biopsy. Results: The total HPV positivity was 43 per cent (116/270). HPV positivity in the patients with an abnormal cytology was 77 per cent (33/43), whereas it was only 37 per cent (83/227) in women with normal cytology, which showed a significant difference (P<0.05). HPV positivity was also related to the age group when all the subjects were considered (P<0.05), and the highest prevalence of HPV infection was in the 30-39 yr age group. High-risk HPV types 16, 18, 31, 35, 51 and 56 were more common in the normal cytology patients, whereas high-risk HPV types 16, 31, 35, 45, 58 and 68 were commonly found in the abnormal cytology patients. Interpretation & conclusions: The determination of high-risk HPV genotypes in women with clinically suspicious cervical lesions should be conducted during an annual follow-up, irrespective of a normal or abnormal cytology by the age of 30 years or above.

Effect of sodium selenite on synaptic plasticity and neurogenesis impaired by hypothyroidism.

AIM OF THE STUDY: We investigated protective effect of sodium selenite (Se) on hypothyroidism-induced impairments in, Morris water maze (MWM), long-term potentiation (LTP) and hippocampal neurogenesis male Wistar rats aged of 2 months. MATERIALS AND METHODS: Hypothyroidism was induced by administration of propylthiouracil (Ptu, 1 mg/kg/d) solution to the rats from postnatal day 60 for 81 days with or without Se (0.5mg/kg/d). Neurogenesis was examined by Ki-67 immunohistochemical staining. Se values on plasma and hippocampus were measured with inductively coupled plasma-mass spectrometry (ICP-MS). RESULTS: Measurement of fT3 and fT4 levels confirmed that the fT3 levels, but not fT4, in Ptu-treated rats (5435.44±816.05 fg/ml, p < 0.05) has returned to control values (8721.66±2567.68 fg/ml) by Se treatment (8661.65±711.43 fg/ml). Analysis of learning performance in water escape learning task showed that Se supplementation disappeared memory deficit in Ptu-treated rats as shown by significantly decreased time spent in the target quadrant (33.7±0.24% in control group; 26.1±0.48% in Ptu-group, p < 0.05; 33.9±0.44 in Ptu+Se group), although there was no significant difference among groups in any measurement of learning performance on the last day. Considering LTP, Se supplementation improved the deficit in synaptic plasticity in Ptu-treated rats, as shown by significant increase in the excitatory postsynaptic potential slope (% 243±31 in control group; 172±49 in Ptu-group, p < 0.05; 222±65 in Ptu+Se group) without affecting of the impairment in somatic plasticity. Se supplementation did not improve the decrease in the number of progenitor cells in the subgranular layer (SGL) of dentate gyrus (DG) of Ptu treated rats. CONCLUSIONS: These findings suggest that selenium supplementation in hypothyroid patients may improve learning and memory disorders with different physiological mechanisms.HighlightsSe increased serum fT3 levels and hippocampus Se levels in hypothyroid rats.Se attenuated impairment of population spike-LTP in hypothyroid ratsHypothyroidism disrupts neurogenesis process in the dentate gyrus of hippocampus.Se supplementation could not increase new born cells in hypothyroid rats.

A Rare Case of Activated Phosphoinositide 3-Kinase Delta Syndrome (APDS) Presenting With Hemophagocytosis Complicated With Hodgkin Lymphoma.

Gain of function mutations in the p110δ catalytic subunit of the phosphatidylinositol-3-OH kinase (PIK3CD) classified as activated phosphoinositide 3-kinase delta syndrome (APDS) are the cause of a primary immunodeficiency characterized by recurrent sinopulmonary infections, and lymphoproliferation. Previously, autoimmunity and Epstein-Barr virus-related B-cell lymphoma have been documented for patients with APDS; here, we present a case that extends the picture, as the patient shows the full diagnostic criteria of hemophagocytic lymphohistiocytosis at 6 months of age. He experienced Hodgkin lymphoma as a 2.5-year-old baby. Next-generation sequencing returned a de novo heterozygous missense variant in PIK3CD (LRG_191t1: c.3061G>A; p.Glu1021Lys), confirming the primary immunodeficiency. After 2 courses of ifosfamide, cisplatin, and etoposide combined with brentuximab, the patient successfully underwent allogeneic hematopoietic stem cell transplantation from his HLA full matched sister, and he has been well for 18 months after that. The hematologist treating Hodgkin lymphoma and/or hemophagocytic lymphohistiocytosis should be vigilant about the possible underlying immune deficiency, and they should consider APDS in their differential diagnosis.

The role of CD133 expression of cancer stem cells on radiotherapy response in early stage glottic cancers.

PURPOSE: To investigate the relationship between CD133 positivity and radiotherapy (RT) response in early stage glottic laryngeal cancers. METHODS: Thirty seven patients with early-stage glottic laryngeal carcinoma who were treated with primary RT were evaluated. Patients with regular follow-up of at least 3 years were included in the study. Patients who had previously received chemotherapy for laryngeal surgery or underwent surgery were excluded. The patients were divided into two groups as recurrent and non-recurrent. These two groups were compared in terms of CD133 expression by immunohistochemical method. RESULTS: There were 37 patients in the study. Ten patients had recurrence and seven (70%) had CD133 positive and three had CD133 negative. Of 27 patients who had no recurrence, 16 (59%) had CD133 positive and 11 (41%) had CD133 negative. 7 (70%) of ten patients with recurrence were found to be positive for CD133; There was no statistically significant difference between recurrent and non-recurrent patient groups in terms of CD133 positivity (p > 0.05). There was no correlation between the final CD133 score and recurrence status as well (p > 0.05). CONCLUSION: There was no relationship between radiotherapy response and CD133 staining in early-stage glottic laryngeal cancers. It is the largest study about CD133 and RT sensitivity in early stage glottic carcinomas.

Cutaneous ulcerations caused by Paecilomyces variotii in a renal transplant recipient.

Skin infections caused by Paecilomyces species have been rarely described in patients with solid organ transplantation. Cutaneous manifestations are highly variable and include erythematous macules, nodules, pustules, and vesicular and necrotic lesions. The diagnosis of these infections is generally made by examination of a skin biopsy. Management of these fungal infections is difficult due to the immunocompromised state of the patients. Moreover, antifungal therapy and immunosuppressive drug interactions should be considered during treatment management. Herein, we reported a case of cellulitis caused by Paecilomyces variotii in a 56-year-old man who had undergone a kidney transplantation. Erythematous macular and nodular lesions on the left hand and left foot appeared first; within 2 months the skin lesions became ulcerated, hemorrhagic, and progressively painful and the patient was admitted to our hospital. The diagnosis was made by skin biopsy and tissue culture. The skin lesions resolved by the sixth week of the treatment with voriconazole.

The role of endobronchial and endoscopic ultrasound guided fine needle aspiration for mediastinal nodal staging of non-small-cell lung cancer.

INTRODUCTION: Mediastinal and hilar nodal staging is one of the key points for differentiating treatment modalities in patients with non-small-cell lung cancer (NSCLC). The aim of the present study was to determinate the diagnostic yields of endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA), endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) and combined EBUS-TBNA and EUS-FNA modalities for nodal staging in potentially operable NSCLC patients. MATERIALS AND METHODS: Twenty consecutive patients were prospectively enrolled in the study between March 2014 and November 2015. All patients had a potentially operable NSCLC diagnosis before endosonographic procedures. RESULT: Thirty lymph nodes were sampled by EBUS-TBNA and 17 lymph nodes were sampled by EUS-FNA in all 20 patients. The sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of F-18 fluorodeoxyglucose positron emission tomography with computed tomography (PET-CT), EBUS-TBNA, EUS-FNA and combined EBUS-TBNA and EUS-FNA were 100%, 33.3%, 64.7%, 100% and 70.0%; 81.8%, 100%, 100%, 81.8% and 90%; 81.8%, 100%, 100%, 75% and 88.2%; 90.9%, 100%, 100%, 90.0% and 95.0%, respectively. CONCLUSIONS: The combined EBUS-TBNA and EUS-FNA technique is a successful procedure for nodal staging in potentially operable NSCLC patients.

Pediatric central nervous system tumors in the first 3 years of life: pre-operative mean platelet volume, neutrophil/lymphocyte count ratio, and white blood cell count correlate with the presence of a central nervous system tumor.

PURPOSE: The aim of this study is to describe the relationship of pre-operative complete blood count parameters [mean platelet volume (MPV), neutrophil/lymphocyte count ratio (NLCR), and white blood cell count (WBC)], with the clinical, radiological, and histopathological features and the management options for patients under 3 years of age with a newly diagnosed central nervous system tumors. METHODS: Children with central nervous system (CNS) tumors in the first 3 years of life admitted in the Erciyes University Hospital between April 2004 and April 2014 were enrolled in this study. The CBC parameters were compared with those of an age- and sex-matched normal control group. RESULTS: In the study group, the means of MPV and WBC were 8.00 ± 1.24 fl, and 10,855 ± 3642/mm3 respectively; the median (25-75%) of NLCR was 0.98 (0.66-1.46). For the control group, the means of MPV and WBC were 6.8 ± 0.73 fl and 8565 ± 2522/mm3; the median (25-75%) of NLCR was 0.52 (0.36-0.70). The MPV, WBC, and NLCR were higher in the study group. The median overall survival (OS) of the patients was 60 months (range 0-81.6 months); and median event free survival (EFS) was 24 months (range 0-70.1 months). The formulation of MPV, NLCR, and WBC was found to be predictive for the diagnosis of CNS tumor in children with nonspecific symptoms. The univariate and multiple binary regression analyses showed a positive association of MPV, NLCR, and WBC and the risk of a diagnosis of CNS tumor. There was no relationship between MPV, WBC, NLCR, and histological subgroups. However, there were no associations between CBC parameters and OS or EFS of the patients. CONCLUSIONS: By causing suspicion, MPV, NLCR, and WBC may provide both an earlier radiological investigation decision and thereby an early diagnosis of CNS tumor in children with nonspecific symptoms in the first 3 years of life.

A case of multiple myeloma with navicular bone involvement.

Multiple myeloma is a haematological disease caused by proliferation of malignant plasma cells in bone marrow. It frequently has lytic bone lesions. However, involvement of the small bones of the hands and feet is extremely rare. We report a unique multiple myeloma patient with first recurrence in navicular bone after allogenic stem cell transplantation.

Predictive value of E-cadherin and Ep-CAM in cervical lymph node metastasis of supraglottic larynx carcinoma.

OBJECTIVE: To evaluate the value of E-cadherin and epithelial cell adhesion molecule (Ep-CAM) expression in laryngeal biopsy materials for predicting cervical lymph node metastasis in patients with supraglottic laryngeal carcinoma. METHODS: All patients participating in the study were selected from among the surgically treated patients at the department of Otolaryngology, Head and Neck Surgery, Erciyes University School of Medicine between 1991 and 2005. The study consisted of thirty patients who had pathologically metastatic lymph nodes (pN+ group) and 30 age-, sex-, T value- and differentiation matched patients without pathologically metastatic lymph nodes (pN0 group). Immunohistochemical studies were performed with E-cadherin and Ep-CAM antibodies on representative tumor sections collected from paraffin sections of laryngeal biopsy materials. The expression of E-cadherin and Ep-CAM was compared between the pN0 and pN+ groups. The association between immunostaining of E-cadherin and Ep-CAM was also evaluated. RESULTS: There was no significant difference between the two groups in terms of E-cadherin and Ep-CAM expression. There was also a very poor agreement between the expression of E-cadherin and Ep-CAM. CONCLUSION: Multi-institutional and multidisciplinary immunohistochemical studies conducted with standardized methodology and also with more patient participation may help to obtain more specific results.

Cranial metastatic alveolar rhabdomyosarcoma mimicking hematological malignancy in an adolescent boy.

BACKGROUND: Widespread alveolar rhabdomyosarcoma (ARMS) with bone marrow involvement and with an unknown primary tumor, especially presenting with acute tumor lysis syndrome can be easily misdiagnosed as a hematological malignancy. Furthermore, brain metastasis of ARMS is rare seen in children. CASE REPORT: Herein, we report a 14-year-old boy presenting with acute tumor lysis syndrome due to bone marrow invasion of ARMS, who was diagnosed after abdominal paraaortic lymph node biopsy. Despite radiological and nuclear medicine imaging, the primary tumor site could not be found. He was treated with vincristine, topotecan, and cyclophosphamide for 42 weeks. Six months after the completion of treatment, he suffered from severe headache, blurred vision, right hemiplegia, and severe bone pain. Cranial magnetic resonance imaging showed multiple hemorrhagic infarctions. Brain biopsy showed brain metastasis with PAX3-FKHR fusion transcript. CONCLUSION: The clinicians must be vigilant about solely brain metastasis in ARMS without additional metastasis.

Effect of angiostatin on 1,2-dimethylhydrazine-induced colon cancer in mice.

Antiangiogenic therapy is supposed to be an attractive approach for antitumor treatment. Human plasminogen-derived angiostatin K1-3 is one of the most potent antiangiogenic agents known currently. However, it is unclear whether angiostatin has got protective effects on colon cancer. So we investigated the protective effects of angiostatin on 1,2-dimethylhydrazine (DMH)-induced colon cancer in mice. Thirty Balb/C male mice, weighing 25-30 g and 8 weeks of age, were used. Twenty of the mice were treated with DMH subcutaneously (20 mg/kg) once a week for 12 weeks. Six mice died during the DMH injection and surviving mice were divided into two groups (7 mice in DMH and 7 mice in DMH + angiostatin groups). In the angiostatin group, 6 weeks after the last DMH injection the animals were first treated with angiostatin (20 µg/mouse) intraperitoneally and then subcutaneously every 48 h (5 µg/mouse) throughout a period of 12 weeks. The animals were killed after 30 weeks for histopathological examination. When we look at the distribution of lesions in the colon, they mainly occurred in the distal colon. The incidence of mean colonic lesions in a tumor-bearing mouse was 9.85 ± 4.91 in those treated with DMH and 8.71 ± 3.49 in those treated with angiostatin. The incidence of colon tumors was not significantly affected by low dose of angiostatin, and we noticed that the number of lesions decreased by 12% in DMH + angiostatin group compared to the number of the lesions in DMH group, but this decrease was not statistically significant (p > 0.05). The administration period of angiostatin corresponds to the precancerous period and the reduction in the number of lesions could be important for the protective function of angiostatin in DMH + angiostain group. We assume that therapeutic effects of angiostatin are related to its doses, route of administration, frequency and administration period. In addition, we believe that combination of high doses of angiostatin with radiation, gene therapy or chemotherapy might be successful in proper tumor model.

Influence of gilaburu (Viburnum opulus) juice on 1,2-dimethylhydrazine (DMH)-induced colon cancer.

In this study, the effects of gilaburu (Viburnum opulus) juice on colon tumorogenesis were investigated. Eight weeks old Balb-C male mice received subcutaneous injections of 1,2-dimethylhydrazine (DMH) (20 mg/kg body weight) once a week for 12 weeks. Both the sham control (group 1) and the DMH control (group 2) groups received drinking water alone, whereas the mice of groups 3 and 4 received gilaburu juice for 30 weeks (started with first DMH injection) and for 18 weeks (started after last DMH injection), respectively. Eighteen weeks after the last DMH injection, all mice were killed and the histogenesis of colon tumors was investigated from the paraffin-embedded sections of colon, which were stained with hematoxylin-eosin. The sites and incidences of tumoral lesions (low-grade dysplasia, high-grade dysplasia, intramucosal carcinoma and invasive carcinoma) were analyzed and compared with control. The results showed that the body weights of the mice were similar in all the groups. No tumoral lesions were found in group 1. Colon tumors developed in all DMH-treated mice (groups 2, 3 and 4). In these groups, the greatest numbers of tumor lesions were detected in the distal colon, followed by the mid-colon and only a few in the proximal colon. There was a reduction in the mean total number of tumor lesion in groups 3 (8.5) and 4 (8.3), when compared to group 2 (11.3). The incidence of invasive carcinoma in group 3 was significantly lower than group 2 (p < 0.05). On the basis of these results, we conclude that gilaburu juice may be useful for the prevention of colon cancer at the initiation stage.

Should Core Needle Lymph Node Biopsy be a Relevant Alternative to Surgical Excisional Biopsy in Diagnostic Work Up of Lymphomas?

OBJECTIVE: Surgical excisional biopsy is accepted as the standard of care approach in the diagnosis of lympho- mas. Financial issues related to the increased cost and the invasive nature of the procedure forced physicians to use some alternative diagnostic methods. Percutaneous core needle biopsy, which gained a reputation for the diagnosis of lymphomas with the advent of improved pathological, immunohistochemical, and molecular analysis, made it possible to have an accurate diagnosis with limited tissue samples. In this retrospective study, we aimed to compare the diagnostic yield of surgical excisional biopsy and core needle biopsy. MATERIALS AND METHODS: This study included 131 patients who were diagnosed with lymphoma with a nodal biopsy which was acquired via surgical excisional biopsy or core needle biopsy between 2014 and 2020 in our center. Around 68 patients underwent surgical excisional biopsy and the remaining 63 underwent core needle biopsy. Samples that allowed to the identification of the exact tumor type and/or subtype were accepted as fully diagnostic. Sufficient amount of tissue that the pathologist could have any suspicious findings considering malignant lymphoma was classified as partial diagnostic group. Inadequate samples were the ones who were not enough to report any final diagnosis. RESULTS: The patients who underwent a core needle biopsy were significantly older than the patients who underwent to surgical excisional biopsy (56.8 vs. 47.6, P = .003). Despite the full diagnostic ability of surgical excisional biopsy outperformed core needle biopsy (95.2 % vs. 83.8 %, P=.035), in 92.6% of the patients whose tissue samples were obtained via core needle biopsy were accepted to have a sufficient diagnosis to initiate the treatment and not required a second biopsy, which was comparable with the ones achieved by surgical excisional biopsy (92.6% vs. 95.2%, P = .720). CONCLUSION: According to the results obtained in our study, we may conclude that core needle biopsy is a viable and comparable alternative to surgical excisional biopsy, offering a less invasive and less-expansive approach.

Molecular epidemiology and antifungal susceptibilities of Aspergillus species isolated from patients with invasive aspergillosis.

OBJECTIVE: The aim of this study was to evaluate the demographic data, molecular epidemiology, and in vitro antifungal susceptibility results of patients with Aspergillus isolated from various clinical specimens. METHODS: A total of 44 Aspergillus strains were studied. The definition of invasive aspergillosis in patients was made according to European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) criteria. Strains were phenotypically and molecularly identified. Demographic characteristics of patients and genotypes of strains were evaluated. Phylogenetic analysis was done by the The Unweighted Pair-Group Method with Arithmetic Mean (UPGMA). Antifungal susceptibility of strains was determined according to The Clinical and Laboratory Standards Institute (CLSI)-M61-Ed2 and The European Committee on Antimicrobial Susceptibility Testing (EUCAST). RESULTS: A total of 11 patients were classified as proven and 33 as probable invasive aspergillosis. There was a statistically significant difference in age groups, subdisease, neutropenic, and receiving chemotherapy between groups. A total of 23 strains were identified as Aspergillus fumigatus, 12 as Aspergillus niger, 6 as Aspergillus flavus, and 3 as Aspergillus terreus. Phylogenetic analysis revealed five different genotypes. No statistical difference was found in the comparisons between patients groups and genotype groups. There was a statistically significant difference between genotype groups and voriconazole, posaconazole, and itraconazole Minimum Inhibition Concentration (MIC). CONCLUSION: Accurate identification of strains and antifungal susceptibility studies should be performed due to azole and amphotericin B resistance. Genotyping studies are important in infection control due to identifying sources of infection and transmission routes.

Cerebellar hemangioblastoma associated with diffuse neonatal hemangiomatosis in an infant.

INTRODUCTION: Hemangioblastomas (HBLs) comprise approximately 2% of all primary central nervous system (CNS) tumors. Although histological features of this rare tumor are generally benign, its outcome is often unfavorable due to high risk of recurrence and multifocal localization. HBLs can be detected as sporadic or associated with Von Hippel-Lindau disease. Diffuse neonatal hemangiomatosis (DNH) presents with multiple, progressive, rapidly growing cutaneous hemangiomas associated with widespread visceral hemangiomas in the liver, lungs, gastrointestinal tract, brain, and meninges. DNH with predominant CNS involvement is rarely reported. Herein, we present a neonatal case of cerebellar HBL associated with DNH. CASE REPORT: A 5-day-old male baby was referred with complaints of multiple cutaneous lesions. Purple papules were noted on the trunk, extremities, and the head. Thoracic magnetic resonance imaging demonstrated multiple hyperintense lesions on the chest wall and apex of the right lung. On MRI, a 3×2-cm mass lesion in the right cerebellar hemisphere was detected. Total resection of the mass and ventriculoperitoneal shunting was performed. Histopathologic examination confirmed the diagnosis of HBL. Steroid therapy was administered for disseminated hemangiomatosis, and the lesions showed regression; the patient showed good clinical recovery. The parents refused further treatment, and he was out of our control when he was 9 months old. CONCLUSION: According to our knowledge, the presented newborn is the second case of cerebellar HBL associated with diffuse skin and visceral hemangiomas in the English medical literature. Clinicians must be vigilant about the predictive value of visceral and/or cutaneous hemangioma for an associated intracranial HBL.

Effect of endostatin on 1,2-dimethylhydrazine-induced colon tumor in mice.

Endostatin, one of the most potent negative regulators of angiogenesis, is naturally occurring as an inhibitor of angiogenesis capable of inhibiting tumor growth and their metastases. We aimed to investigate the in vivo activities of low dose of recombinant human endostatin on 1,2-dimethylhydrazine (DMH)-induced mice colon cancer. Thirty male Balb-c mice were injected with DMH (20 mg/kg/week) subcutaneously once a week for 12 weeks to induce colon cancer. Twelve weeks after the last DMH injection, 7 µg rh-endostatin was injected every day for 6 weeks. The animals were killed after 30 weeks for histopathological examination. The weight of the animals, tumor inhibition rates, death rates and the distribution of the lesions in colon were evaluated after the mice were killed. The mean colonic lesions incidence in single tumor bearing mice was 11 ± 4.0 in those treated with DMH and 8.1 ± 3.7 in those treated with endostatin. When we look at the distribution of lesions in the colon, they occurred in the distal colon. At the end of our study, we noticed that the number of lesions decreased by 25% in the group of endostatin, considering the number of the lesions in the group of DMH. But there was no statistical difference between the mice treated with endostatin and those treated with DMH. It will be very significant to identify endostatin therapeutic effects as long as proper dose of endostatin is administrated at the proper time, duration and proper tumor model.

Malignant proliferating trichilemmal tumor of the scalp: report of 4 cases and a short review of the literature.

Malignant proliferating pilar tumors are very rare adnexial lesions that can be confused with other skin neoplasms. The authors present four patients with malignant proliferating trichilemmal tumors located on the scalp. A review of the literature search for malignant proliferating pilar tumors and treatments was performed.