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Background: Consent and assent are important concepts to understand in the care of pediatric neurosurgery patients. Recently it has been recommended that although pediatric patients generally do not have the legal capacity to make medical decisions, they be encouraged to be involved in their own care. Given the paucity of information on this topic in the neurosurgery community, the objective is to provide pediatric neurosurgeons with recommendations on how to involve their patients in medical decision-making. Methods: We review the essential elements and current guidelines of consent and assent for pediatric patients using illustrative neurosurgical case vignettes. Results: The pediatric population ranges widely in cognitive and psychological development making the process of consent and assent quite complex. The role of the child or adolescent in medical decision-making, issues associated with obtaining assent or dissent, and informed refusal of treatment are considered. Conclusion: The process of obtaining consent and assent represents a critical yet often overlooked aspect to care of pediatric neurosurgical patients. The pediatric neurosurgeon must be able to distill immensely complex and high-risk procedures into simple, understandable terms. Furthermore, they must recognize when the child's dissent or refusal to treatment is acceptable. In general, allowing children to be involved in their neurosurgical care is empowering and gives them both identity and agency, which is the vital first step to a successful neurosurgical intervention. [ABSTRACT FROM AUTHOR]

The cancer stem cells deliver uncontrolled proliferative capacity within the tumor imparting to increasing size while epithelial mesenchymal transition adds to the invasive potential. Studies using specific markers elucidating the role of these phenomena may bring advancement in the targeted therapy of tumor. SOX2 and OCT4 are two among few stem cell markers indicative of proliferative potential and WNT5A is an epithelial mesenchymal transition marker indicative of invasive potential. We aimed to determine the association between expression of SOX2, OCT4 and WNT5A in oral epithelial dysplasia, oral squamous cell carcinoma and normal oral mucosa. 20 cases of oral squamous cell carcinoma, 20 cases of oral epithelial dysplasia (leukoplakia with dysplasia) and 25 normal oral mucosa tissues specimens were immunohistochemically stained to assess SOX2, OCT4 and WNT5A expression. SOX2 expression was higher in oral squamous cell carcinoma than in oral epithelial dysplasia and very low in normal oral mucosa. OCT4 was very low in oral squamous cell carcinoma and oral epithelial dysplasia when compared to SOX2, while negative in normal tissues. Co-expression of SOX2 and OCT4 showed statistically non-significant difference for tumor proliferation. WNT5A expression was found to be increasing from normal oral mucosa to oral epithelial dysplasia and oral squamous cell carcinoma. In conformity with present study, SOX2 itself can act as a potential marker for proliferation in tumor cells while OCT4 has non-significant role in regulation of tumor behavior in oral squamous cell carcinoma as well as in oral epithelial dysplasia. WNT5A can be a putative marker in studying invasive potential of oral squamous cell carcinoma. [ABSTRACT FROM AUTHOR]

Benign vocal lesions are non-malignant growths of abnormal tissue on the vocal cords. The common benign lesions of vocal cord are singer's nodule, polyps, papilloma, polypoidal degeneration and cysts. The aim of this study was to analyze the demographics such as age, sex, occupation, symptomatology, site of involvement. An objective evaluation of voice handicap was done pre and postoperatively using VHI-10 scale to see improvement in patient's symptoms. In this prospective study, a total of 50 cases were selected with benign lesions in regional hospital of India. The pre and post operative assessment for voice handicap was assessed by VHI-10. Chi square test (SPSS 20.0 version) used to analyze result; value of p < 0.05 was taken significant. In this study of 50 patients, the benign lesions were most common in 20–29 age group. Male (70%) outnumbered females (30%). Most common was Vocal polyp (56%), followed by nodule (32%), cyst (10%) and papilloma (2%) respectively. All patients showed improvement after phonosurgery and postoperative speech therapy, being assessed by VHI-10 scale. The benign lesions of vocal cords produces symptoms which can vary from hoarseness to stridor, affect social functioning, work performance. Speech therapy following microlaryngeal surgery forms an essential part of treatment, to avoid recurrence. VHI-10 scale as found to be a useful and convenient tool in measuring patient voice handicap and to see improvement after surgery. [ABSTRACT FROM AUTHOR]

Epigenetic patterns on the level of DNA methylation have already been shown to separate clinically relevant subgroups of meningiomas. We here set out to identify potential prognostic implications of epigenetic modification on the level of histones with focus on H3K27 trimethylation (H3K27me3). H3K27me3 was assessed by immunohistochemistry on 232 meningiomas from 232 patients. In 194 cases, trimethylation was detected in tumor cells. In 25 cases, staining was limited to vessels while all tumor cells were negative. Finally, 13 cases yielded equivocal staining patterns. Reduced abundance of H3K27me3 in cases with staining limited to vessels was confirmed by mass spectrometry on a subset of cases. Lack of staining for H3K27me3 in all tumor cells was significantly associated with more rapid progression (p = 0.009). In line, H3K27me3-negative cases were associated with a DNA methylation pattern of the more aggressive types among the recently introduced DNA methylation groups. Also, NF2 and SUFU mutations were enriched among cases with complete lack of H3K27me3 staining in tumor cells (p < 0.0001 and p = 0.029, respectively). H3K27me3 staining pattern added significant prognostic insight into WHO grade II cases and in the compound subset of WHO grade I and II cases (p = 0.04 and p = 0.007, respectively). However, it did not further stratify within WHO grade III cases. Collectively, these data indicate that epigenetic modifications beyond DNA methylation are involved in the aggressiveness of meningioma. It also suggests that H3K27me3 immunohistochemistry might be a useful adjunct in meningioma diagnostics, particularly for cases with WHO grade II histology or at the borderline between WHO grade I and II. [ABSTRACT FROM AUTHOR]

Various studies have demonstrated that the lymphatic system is the additional route for solid tumor metastasis. Lymph nodes metastasis in Head and neck squamous cell carcinoma (HNSCC) is a major prognostic indicator for disease progression and a guide for therapeutic strategies. We conducted a study to compare intratumoral (IT) and peritumoral (PT) lymphatic vessel density (LVD) in HNSCC using lymphatic marker D2-40 and its correlation with lymph node metastasis, histological grading and other clinicopathological parameters. Fifty specimen of HNSCC with modified radical neck dissection tissue were included in the study group. Tissue from tumor, peritumoral tissue, tumor margin and all the lymph nodes were processed for paraffin wax blocks and histopathological diagnosis. Immunohistochemical profile of lymphatic vessels in intratumoral and peritumoral tissue was assessed by subjecting one section each from the tumor and peritumoral tissue to D2-40 immunostain. To determine LVD, four fields with the highest LVD (hot spots) were identified. The mean values were calculated by taking an average of all the measurements. The comparison of LVD between peritumoral and intratumoral area revealed significantly higher PT-LVD (P = 0.001). No significant association was seen between LVD, IT-LVD and PT-LVD and different age groups, gender, site of tumor, risk factors, size of tumor, tumor inflammation, pushing/infiltrating margin and stage of tumors. Significantly higher LVD, IT-LVD and PT-LVD was seen in association with lymph node metastasis. Both high intratumoral and peritumoral LVD were found significantly associated with the presence of lymph node metastasis, however lymphatic vessels were found to be significantly more numerous and larger in peritumoral areas as compared to intratumoral lymphatics. The specificity of D2-40 as a lymphatic endothelial marker was also confirmed. The results of our study support the possibility of using the determination of tumor lymphangiogenesis to identify patients of HNSCC who are at risk of developing the lymph node metastasis. [ABSTRACT FROM AUTHOR]

Background: Primary benign and malignant tumors of the proximal fibula are not very common. Upper fibula being an expendable bone; the majority of the primary bone tumors at this site are usually treated with en bloc proximal fibulectomy. There is scarce literature on functional results, difficulties faced during dissection when to preserve or sacrifice common peroneal nerve and importance of lateral collateral ligament repair after proximal fibulectomy. The present study attempts at assessing these variables. Materials and Methods: This retrospective study included 46 patients; 30 males and 16 females with age ranging from 12 to 44 years (average: 26 years) operated between 2003 and 2014. There were 34 benign and 12 malignant tumors. All were treated with proximal en bloc fibulectomy as indicated and decided by the operating surgeon keeping in view its extent on magnetic resonance imaging. Peroneal nerve sacrifice or preservation was decided as per the type (benign/malignant), its involvement by the tumor and the extent of the tumor. In 14 (for 12 malignant and two benign giant cell tumors [GCTs]) patients, the peroneal nerve required resection for the margins. Partial upper tibial resection was performed in cases of malignant tumors and three GCTs. The followup ranged between 24 and 120 months (median: 48 months). Results: Patients with peroneal nerve resection had inferior functional outcome than those without peroneal nerve resection. There was no higher risk of tibia fracture in patients with partial tibial resection. Lateral collateral reconstruction yielded better results and should be performed in all cases. Functional outcome was significantly better in patients with benign tumors than in patients with malignant tumors as these required neither resection of the peroneal nerve nor large amount of muscle excision. The functional results were evaluated using Musculoskeletal Tumor Society (MSTS) score, and clinical outcomes were evaluated using knee and ankle movements and stability. The overall average MSTS score was 26.50. Conclusions: With good reconstruction of lateral ligament we can achieve good results after proximal fibulectomy for benign as well as malignant tumor without much instability. With partial upper tibial resection (i.e., the extra-articular resection of proximal tibiofibular joint) adequate margins are feasible even in malignant tumors. [ABSTRACT FROM AUTHOR]

Background: Giant cell tumor (GCT) of the bone is known for its locally aggressive behavior and tendency to recur. It is an admixture of rounded or spindle-shaped mononuclear neoplastic stromal cells and multinucleated osteoclast-like giant cells with their proportionate dispersion among the former. Zoledronic acid (a bisphosphonate) is being used in various cancers such as myelomas and metastasis, for osteoporosis with an aim to reduce the resorption of bone, and as an adjuvant treatment for the management of GCT of bone for reduction of local recurrence. We have carried out a prospective comparative study to assess the effect of intravenous infusion of zoledronic acid on histopathology and recurrence of GCT of bone. Materials and Methods: The study was carried out in the biopsy proven GCTs in 37 patients; 15 males and 22 females, in the age range from 17 to 55 years. They were treated with extended curettage. Of these 37 patients, 18 were given three doses of 4 mg zoledronic acid infusion at 3-week intervals and extended curettage was performed 2 weeks after the last infusion whereas the other 19 were treated with extended curettage without zoledronic infusion. The post infusion histopathology of the curetted material was compared with the histopathology of initial biopsy. All the patients were evaluated at 3-month intervals for the first 2 years and then six monthly thereafter, for local recurrence and functional outcome of limb using the Musculoskeletal Tumor Society (MSTS) score. Results: In postzoledronic infusion cases, the histopathology of samples showed abnormal stromal cells secreting matrix leading to fibrosis and calcification. The type of fibrosis and calcification was different from pathological calcification and fibrosis what is usually observed. There was a good marginalization and solidification of tumors which made surgical curettage easier in six cases in the study group. There was noticeable reduction in the number of giant cells and alteration in morphology of stromal cells to the fibroblastic-fibrocytic series type in comparison to preinfusion histopathology. Recurrence occurred in one case out of 18 patients in infusion group whereas in four cases among 19 patients in control group. The functional results were assessed, and the overall average MSTS score was 27.50 (range 24-30) and 27.00 (range 23.50-30) in the study and control groups, respectively. Conclusions: We observed that bisphosphonates reduce osteoclast activity and affects stromal cells in GCT, resulting in the reduction of their numbers and noticeable apoptosis. This results in better marginalization of the lesions and reduced recurrence. Extended curettage of friable GCT became easier and adequate which otherwise might not have been possible. [ABSTRACT FROM AUTHOR]

To compare the role of fresh and dry preserved amniotic membrane transplantation (AMT) in partial limbal stem cell deficiency (LSCD). 30 Patients of partial LSCD involving <180° of cornea of different age group were divided into 2 groups; group A and B. Clinical diagnosis was made on the basis of presence of conjunctival pannus over cornea and loss of limbal palisades of Vogt. After taking impression cytology from the corneal surface, group A patients underwent conjunctival pannus resection followed by fresh AMT while group B patients underwent dry preserved AMT after resection of pannus. Resected specimens were sent for histopathological confirmation of the conjunctival tissue. Serial follow-ups were done at 1, 2, 4, 8, 12 and 24 weeks interval post-operatively. Pre-operative impression cytology revealed goblet cells in 40 and 53.33 % cases of group A and group B respectively. However, histopathological examination confirmed the presence of conjunctival tissue in all cases. All cases in both the groups maintained stable corneal epithelial surface with amelioration of clinical symptoms except for 1 case in group A. At the end of 24 weeks improvement in visual acuity was observed in 40 % cases in both the groups which was not more than 2 Snellen's line in any case. Complete regression of superficial vascularisation was observed in 60 and 53.33 % cases in group A and B respectively while deep vascularisation persisted in all at the end of study period. Recurrence of conjunctival pannus at the same site was observed in 13.33 % in both the groups. One patient (6.66 %) developed post-operative infectious keratitis in group A while no major complications were observed in group B. Both fresh and dry AMT is equally effective in the treatment of partial LSCD. However, the utility of AMT is limited in corneas with long standing tissue scarring and vascularisation secondary to partial LSCD. [ABSTRACT FROM AUTHOR]

Aplastic anaemia (AA), the paradigm of bone marrow failure syndromes, is most simply defined as peripheral blood cytopenia and a hypocellular bone marrow. Parvovirus B19 (B19V) which is the best characterized amongst the four different parvoviruses infecting humans has a variety of clinical manifestations depending upon the clinical and hematological status of the host. We report a case of parvovirus B19-associated chronic aplastic anaemia in a 34-year-old female patient who presented with gum bleeding and ecchymotic patches over both legs and curdy vaginal discharge. She had pancytopenia on peripheral blood film examination and was subjected to bone marrow biopsy as the marrow aspirate showed peripheral blood only hence was not conclusive. Bone marrow biopsy was reported as AA, severe grade. The serological analysis performed later on revealed parvovirus B19 infection. Acute infection with B19V should be considered as a cause of acquired aplastic anaemia in individuals where other etiological factors are not elucidated. [ABSTRACT FROM AUTHOR]

Human Parvovirus B19 has been linked to a variety of diseases. One of the most common complications is transient aplastic crisis in patients with chronic hemolytic anemia. Very few case reports have implicated this virus as a putative etiology behind hepatitis and severe aplastic anemia in immuno competent individuals. We report a case of severe aplastic anemia in a previously healthy adult female due to acute parvovirus B19 infection. Laboratory examination showed pancytopenia in peripheral blood and severe hypoplastic bone marrow on biopsy. Serological analysis (ELISA) revealed acute Parvovirus B19 infection. In the face of unavailable HLA matched bone marrow donor, immuno-supressive therapy was contemplated, but could not be given because of financial constraints. Pancytopenia persists till date, 4 months after the diagnosis, with the patient requiring repeated packed red cell and irradiated platelet transfusions. Thus, acute infection with this virus must be considered a cause of acquired aplastic anemia even in individuals without underlying disease. [ABSTRACT FROM AUTHOR]

Gelatinous bone marrow transformation (GTBM) also known as serous atrophy is a degenerative change in the hematopoietic bone marrow and is a rare well recognized pathological entity. It was earlier described mainly in association with anorexia nervosa and psychiatric eating disorders, but recently it has been reported in ulcerative colitis, tuberculosis, chronic renal diseases, immuno suppressed states (mainly HIV infection), malignancies and Kala azar. Treatment is based on treating the underlying disease. Our objective was to study the epidemiology and pathogenesis of diseases causing gelatinous transformation of bone marrow, at a tertiary center level. A prospective study was conducted on 732 samples of bone marrow aspirate with the aim of identifying gelatinous transformation in bone marrow aspirates which was confirmed by Alcian blue stain at pH 2.5. The presence of this material was correlated with the hematological profile of the patient, the presenting signs and symptoms and provisional clinical diagnosis at initial visit. Incidence of gelatinous transformation was calculated to be nearly 4.8 % and the condition was more common in males (23) as compared to females (12) (Male:Female = 2:1). Forty percent of the cases were seen in children followed by 37 % in adolescents and young adults. The older individuals comprised only 23 % of the cases. The bone marrow was hypocellular in 21 (60 %), normocellular in 10 (28.5 %) and hypercellular in four cases (11.5 %). Five cases with GTBM progressed to aplastic anaemia of which three were in children. [ABSTRACT FROM AUTHOR]

A case of an unusual occurrence of mature solid teratoma of the rectum is reported with a review of the literature. A 30-year-old woman presented with bleeding per anum without any alteration in her bowel habits. Sigmoidoscopy revealed a pedunculated polypoidal mass arising from the posterior wall of the rectum. A polypectomy specimen on microscopy showed the components of all the three germ layers. This may be the largest polypoidal type of rectal teratoma reported in the English literature to date. A review of the literature indicated only 50 previously reported cases, with all except one being female. The common presentations include prolapse and bleeding per anum. [ABSTRACT FROM AUTHOR]

We report a case of a newborn male who had mongoloid slant and was hypotonic at birth. Routine investigations revealed leucocytosis (WBC > 70,000/cmm) with 50% blasts in peripheral blood film. Marrow examination confirmed the excess of blasts. Karyotyping revealed 47, XY + 21 chromosomes. Due to absence of clinical symptoms, the baby was kept on follow-up without treatment. Within 7 weeks, PBF and bone marrow findings returned to normal, and the child was diagnosed as having Transient leukaemia with Down syndrome. [ABSTRACT FROM AUTHOR]

Oral manifestations of tuberculosis are quite rare with an incidence rate of 1.4%. The tongue is the most common site of oral tuberculosis having varied presentations. Lingual tuberculosis is a diagnostic dilemma because of its rarity, particularly in immunocompetent patients. We are reporting a rare case of primary tuberculosis of the tongue in a 38 year old male diagnosed on fine needle aspiration cytology. [ABSTRACT FROM AUTHOR]

Salivary duct carcinoma (SDC) is a recently described highly malignant tumor, seen most commonly in the parotid gland & occurs in the 6 th and 7 th decade of life. Rarely, it occurs in the minor salivary glands. The case is presented because of its rare occurrence and unusually presenting as a swelling predominantly involving the maxillary bone. The tumor bears histological homology with breast carcinoma, prostatic carcinoma and sweat duct carcinoma. [ABSTRACT FROM AUTHOR]

An unusual case of segmental defect of ileal musculature with terminal ileal atresia and ventricular septal defect is reported. Clinically the patient was diagnosed as ileal atresia. On exploration, there was type I atresia at ileocaecal junction with attendant ileal dilatation and an adjoining 5 cm x 4 cm segment resembling segmental dilatation of ileum, about 15 cm proximal to ileocaecal junction. Pathological examination of segmentally dilated ileum revealed focal absence of circular and longitudinal muscle coat without any evidence of necrosis or inflammation. The possible etiology and its relationship with congenital segmental dilatation of intestine is discussed. [ABSTRACT FROM AUTHOR]

We encountered an extremely unusual presentation of proptosis in a 7-yr-old boy due to psammomatoid juvenile ossifying fibroma of frontoethmoidal region. The tumor also led to development of mucocele in the frontal sinus not yet fully developed as on opposite side. [ABSTRACT FROM AUTHOR]

This report presents features of mesoblastic nephroma, a rare benign tumor of kidney observed in perinatal period of life and highlights the role of imaging investigations in the proper management of this tumor. Awareness of this tumor may facilitate prevention and management of severe obstetric and neonatal complications and inadvertent and vigorous therapy compatible with that of Wilms' tumor can be avoided. [ABSTRACT FROM AUTHOR]

Background: Morphological diagnosis of non-Hodgkin lymphoma (NHL) is usually based on lymph node biopsy. Bone marrow biopsy (BMB) is important for staging, and morphology alone can be challenging for subtyping. Immunohistochemistry (IHC) allows a more precise diagnosis and characterization of NHL using monoclonal antibodies. However, there is a need for a minimal panel that can provide maximum information at an affordable cost., Methods: All newly diagnosed cases of B-cell NHL with bone marrow infiltration between 2017 and 2019 were included. BMB was the primary procedure for diagnosing B-cell NHL. Subtyping of lymphomas was performed by immunophenotyping using a panel of monoclonal antibodies on IHC. The primary diagnostic panel of antibodies for B-cell NHL included CD19, CD20, CD79, CD5, CD23, CD10, Kappa, and Lambda. The extended panel of antibodies for further subtyping included CD30, CD45, CD56, Cyclin D1, BCL2, and BCL6., Results: All cases of B-cell NHL were classified into the chronic lymphocytic leukemia (CLL) and non-CLL groups based on morphology and primary IHC panel. In the CLL group, the most significant findings were CD5 expression, CD23 expression, dim CD79 expression, and weak surface immunoglobulin (Ig) positivity. In the non-CLL group, they were CD5 expression, positive or negative CD23 expression, strong CD79 expression, and strong surface Ig expression. An extended panel was used for further subtyping of non-CLL cases, which comprised CD10, Cyclin D1, BCL2, and BCL6., Conclusion: We propose a two-tier approach for immunophenotypic analysis of newly diagnosed B-cell NHL cases with a minimum primary panel including CD5, CD23, CD79, Kappa, and Lambda for differentiation into CLL/non-CLL group and Kappa and Lambda for clonality assessment. An extended panel may be used wherever required for further subtyping of non-CLL.