Clinical spectrum and clinicopathological correlation of pediatric orbital tumors: 20 years' experience from a tertiary eye care center.

PURPOSE: To study the epidemiological profile of various pediatric orbital tumors and determine their clinicopathological correlation over 20 years. METHODS: A retrospective analysis of all pediatric patients with orbital tumors from the medical records between 1995 and 2015 was done. Tumors were categorized based on their cellular origin. Demographic details, clinical and histopathological diagnoses, frequency, and clinicopathological correlation were studied. RESULTS: A total of 265 pediatric patients with orbital tumors were analyzed, of which 145 (54.7%) patients were males and 120 (45.3%) were females. The mean age was 7 years (median: 6 years; range: 10 days-16 years). The distribution of lesions in each group was as follows: vasculogenic, 76 (28.7%); lipogenic and myxoid, 66 (25%); myogenic, 31 (11.7%); optic nerve and meningeal, 22 (8.3%); lymphoid and lymphoproliferative, 18 (6.8%); secondary orbital tumors, 14 (5.2%); histiocytic, 9 (3.4%); miscellaneous, 9 (3.4%), lacrimal gland, 8 (3%); mesenchymal with neural differentiation, 6 (2%); osseous, 4 (1.5%), and fibrocystic, 2 (0.8%). The most common benign and malignant pediatric orbital tumors were dermoid cysts (65, 24.5%) and rhabdomyosarcoma (31, 11.7%). A statistically significant ( P < 0.0001) clinicopathological correlation was observed in 208 (83.2%) out of 250 biopsy-proven cases. CONCLUSION: Pediatric orbital neoplasms include a broad spectrum of benign and malignant lesions. Vasculogenic tumors constitute the majority of them, followed by lipogenic and myxoid tumors. A thorough knowledge of the incidence, clinical profile, imaging features, and histopathology of specific orbital tumors aids in accurate diagnosis and their successful management.

Integrative management of anaplastic astrocytoma through a combination of Ayurveda and conventional care: A case report.

Anaplastic Astrocytoma (AA) is a relatively rare cancer, and is associated with a median life expectancy of 3 years after conventional therapy. Complete cure of the highly infiltrative AA is uncommon, and reports of positive outcome in cases of partial resection of AA are rare. Further, integrative approaches to the management of AA remain underexplored. This paper contributes to the limited literature in this domain by presenting a case that was successfully treated through integrative conventional and Ayurvedic interventions. A patient diagnosed with AA in the left parieto-occipital lobe underwent partial lesion resection followed by post-operative radiation and chemotherapy. The patient was given a conservative post-surgical life expectancy of two years, and was referred to an Ayurveda hospital for further treatment. The Ayurvedic intervention was focussed on redressal of radiation and chemotherapy side-effects, improvement of quality of life, and improving the patient's strength and immunity. Following this novel integrative model of care, the patient was able to resume all personal and professional routines, and a contrast MRI revealed absence of residual lesion allowing the patient to outlive his initial prognosis by several years till date. We posit that the findings of this report merit further examination in the interest of potential improvements to existing models of care.

The spectrum and clinicopathological correlation of eyelid lesions: Twenty years' experience at a tertiary eye care center in South India.

PURPOSE: : To study the epidemiological pattern and diagnostic accuracy of histopathologically proven eyelid lesions over a period of two decades. METHODS: : A retrospective study of all histopathologically proven eyelid lesions from April 1996 to March 2016 was conducted. The lesions were broadly categorized as benign or malignant. Inflammatory and infectious lesions were included under the benign category. The percentage and diagnostic accuracy of each lesion was calculated. RESULTS: : There were a total of 994 (M = 551, F = 443) cases. The mean age of the patients was 43.5 ± 19.9 years. There were 809 (81.4%) benign and 185 (18.6%) malignant lesions. Benign lesions were commonly seen in the fourth decade, while the malignant ones in the late fifth decade. The upper lid was the most common site in both groups (n = 481, 48.4%). The commonest benign lesion was chalazion (n = 484, 59.8%). Dermal nevus (n = 94, 11.6%) was the most common benign neoplasm, while Molluscum contagiosum (n = 25, 3.09%) was the most common infectious lesion. Sebaceous gland carcinoma (SGC) (n = 103, 55.7%) was the leading malignant lesion followed by basal cell carcinoma (n = 39, 21.1%). Eleven malignant cases were misdiagnosed as benign (5.9%). Chalazion (99.1%) and SGC (65%) had the highest diagnostic accuracy, while Molluscum (40%) and squamous cell carcinoma (40%) were the most misdiagnosed lesions in the respective groups. CONCLUSION: : Benign eyelid lesions are far more common than malignant ones. Atypical and rare presentations may lead to misdiagnosis. Knowledge of epidemiological patterns and clinical features can help in achieving higher diagnostic accuracy.

Orbital and adnexal amyloidosis: Thirty years experience at a tertiary eye care center.

Purpose: The aim of this work was to study the clinical presentation, management and outcomes of orbital and adnexal amyloidosis. Methods: This retrospective analysis included all the patients diagnosed with orbital and adnexal amyloidosis between January 1990 and December 2019. Positive staining with Congo Red and apple-green birefringence on polarized light microscopy established the diagnosis. Data analyzed included demographic profile, varied presentations, management, and outcome. Results: Thirty-three eyes of 26 patients were included. The male:female ratio was 1:1. The mean age of the study population was 42.6 ± 16 years. The median duration of symptoms was two years. Unilateral involvement was seen in 19 eyes (right = 11, left = 8). The most common presenting feature was acquired ptosis. Eyelid was the most commonly affected site followed by orbit and conjunctiva. Two patients had systemic involvement in the form of multiple myeloma and lymphoplasmacytic lymphoma. Complete excision was done in seven (26.9%) cases while 19 (73.1%) cases underwent debulking. Three patients underwent ptosis surgery. The median duration of follow-up was 1.5 years. Three cases had recurrence and underwent repeat surgery. Conclusion: Orbit and adnexa is a rare site for amyloidosis. It is usually localized; however it can occur as a part of systemic amyloidosis. Eyelid is the most common site of involvement and patients usually present as eyelid mass or ptosis. Complete excision is difficult and most of the patients usually undergo debulking surgery. All patients should undergo screening for systemic amyloidosis.

Carvacrol Promotes Cell Cycle Arrest and Apoptosis through PI3K/AKT Signaling Pathway in MCF-7 Breast Cancer Cells.

OBJECTIVE: To examine the role of carvacrol in modulating PI3K/AKT signaling involved in human breast cancer pathogenesis using in vitro experimental model MCF-7 cells. METHODS: MTT and lactate dehydrogenase assays were performed with cells treated with different doses of carvacrol (0-250 p mol/L) at different time points (24 and 48 h). The nuclear morphology was assessed in MCF-7 cells with propidium iodide (PI) and acridine orange/ethidium bromide (AO/EB) staining and analyzed by fluorescence microscopy. Events like cell cycle arrest, apoptosis was observed by flow cytometric analysis and expressions of p-Rb, cyclin D1, cyclin-dependent kinase 4 (CDK4), CDK6, Bax, Bcl-2, PI3K/p-AKT was analyzed by immunoblot. RESULTS: Carvacrol significantly reduced cell viability with the half maximal inhibitory concentration value of 200 µmol/L at 24 and 48 h (P<0.05). importantly, there was a significant increase in the accumulation of the G0/G1 phase upon treatment with carvacrol in MCF-7 cells (P<0.05 or P<0.01). A remarkable decrease in protein expressions of p-Rb, cyclin D1, CDK4 and CDK6 denotes cell cycle arrest (P<0.05 or P<0.01). In addition, carvacrol treatment significantly inhibited PI3K/p-AKT protein expressions leading to induction of apoptosis mediated by decreased Bcl2 and increased Bax protein expressions. Further, Annexin V/PI staining by FACS analysis, dual staining by AO/EB and PI staining studies suggests induction of apoptosis by carvacrol through PI3K/Akt signaling pathway in MCF-7 cells. CONCLUSION: Carvacrol significantly inhibited the breast cancer MCF-7 cell proliferation and induced apoptosis via suppressing PI3/AKT signaling pathway.

Complete Excision of Intradural-Extraforaminal Spinal Tumors Using a Minimally Invasive 2-Incision Technique With Fixed Tubular Retractors.

Spinal tumors are rare, of which intradural-extramedullary lesions form the majority of primary spinal tumors. Occasionally these may even be large, dumbbell shaped, with both intraspinal and extraspinal components. Complete gross total resection is the gold standard in the removal of these tumors since most are benign in nature. Traditionally this has been achieved using large open midline approaches that involve significant muscle dissection, extensive laminectomy, and even facetectomy. This may lead to instability, requiring stabilization to prevent deformity. Minimally invasive surgical approaches using fixed tubular retractors may obviate this need by minimizing the amount of muscle stripping and bony resection required for complete tumor excision. By utilizing facet sparing corridors, the authors describe a novel 2-incision minimally invasive surgical technique that combines a paramedian and a far-lateral approach to access both the intraspinal and extraforaminal, paraspinal portions of the tumor for achieving complete excision. Three illustrative cases are discussed with tumors in 2 different spinal locations that highlights the versatility of this technique-1 in the cervical region and the other 2 in the thoracolumbar region.

Combined medical and surgical management for cicatricial ectropion in lamellar ichthyosis: A report of three cases.

Ichthyosis is a rare inherited skin disorder characterized by abnormal keratinization of the epidermis. Cicatricial ectropion is the most common ophthalmic feature of congenital ichthyosis. Progressive subepithelial cicatrization and abnormal cornification of eyelid skin cause progressive ectropion in both eyelids, leading to lagophthalmos and corneal exposure. Surgical correction of cicatricial ectropion in these cases is challenging with unsatisfactory results. Proper processing of the donor and recipient site with lubricants and topical retinoids before surgery makes grafting easier and its survival better. We present three cases of lamellar ichthyosis with cicatricial ectropion managed with combined preoperative topical therapy followed by surgery. All patients had extremely good surgical outcomes, with none of them requiring repeat surgery.

Clinical spectrum and management outcomes of Langerhans cell histiocytosis of the orbit.

Purpose: To describe the clinical spectrum and management outcomes of Langerhans cell histiocytosis (LCH) involving the orbit. Methods: Retrospective review of patients with orbital LCH presenting at the Sankara Nethralaya, Chennai, India, over the past 15 years. Demographic details, presenting features, radiology, histopathology, immunohistochemistry, and management outcomes were analyzed. Results: Nine patients were reviewed. The mean age of presentation was 10.12 ± 14.31 years (range: 6 weeks to 35 years). Eyelid swelling was the most common presenting feature (4, 44.4%), followed by proptosis (3, 33.3%). The mean duration of the presentation was 2.21 ± 2.77 months. Radiological investigations revealed orbital roof osteolytic defects in six (66.6%) patients. Six patients underwent near-complete excision of the mass while three underwent incisional biopsy. Histopathology revealed histiocytes with nuclear grooving and numerous eosinophils characteristic of LCH. The cells were positive for CD1a and S 100 antigens. None of the patients had any systemic involvement. Three received systemic steroids and four received systemic chemotherapy. At a mean follow-up of 17.85 ± 23.46 months, all had complete remission without any signs of recurrence. One patient was lost to follow-up after near-complete excision while one adult patient with a mass in the intraconal space had no recurrence after near-complete excision. Conclusion: LCH is a rare disorder of the orbit that commonly occurs in children and should be considered a differential for osteolytic lesions involving the orbit. All patients should undergo a systemic evaluation to rule out multifocal disease. The treatment depends upon disease extent and risk factors.

Periocular Basal Cell Carcinoma: 20-Years Experience at a Tertiary Eye Care Center of South India.

PURPOSE: To study the clinical profile and management outcomes of periocular basal cell carcinoma (BCC). MATERIALS AND METHODS: A retrospective analysis of all histopathologically proven cases of BCC between 1995 and 2015 was done. The demographic data, clinical presentation, histopathological subtype, and management outcomes were analyzed. RESULTS: We had a total of 185 malignant eyelid tumors during the study period, out of which 37 (20%) were BCC. Thirty-two (86.5%) out of 37 cases were primary BCC. The mean age was 63.3 years. Pigmented ulcerative lesion (14, 38%), lower eyelid (19, 51%), and nodular BCC (15, 40%) were the most common presentation, periocular site, and histological subtype, respectively. 28 patients underwent excision under frozen section with an average tumor-free margin of 3.5 mm. Mean follow-up period was 18.78 months. Recurrence rate of 0% and 3.1% was noted in primary and recurrent BCC, respectively. CONCLUSION: Periocular BCC commonly presents as pigmented ulcerative lesion in the lower lid. Excision biopsy under frozen section with an average tumor-free margin of 3.5 mm offers cure in most cases.

Mesenchymal chondrosarcoma of the orbit: A case report with 5 years of follow-up.

Mesenchymal chondrosarcoma is a rare orbital tumor. Several case reports of this rare tumor have been published in the literature but only 6 cases have documented a follow up of 5 years or more. We report a case of 28 year-old female who presented with left orbital mass. Computed Tomography (CT) revealed a lobulated mass in the superior extraconal space with dense intralesional calcification. Patient underwent complete resection of the mass and histopathology was suggestive of mesenchymal chondrosarcoma. He was given adjuvant radiotherapy and there was no recurrence or metastasis at 5 years of follow-up. The case highlights that a complete resection with adjuvant radiotherapy in cases of orbital mesenchymal chondrosarcoma offers excellent prognosis.

Orbital angioleiomyoma: A rare orbital neoplasm.

A 44-year-old male patient presented with painless progressive proptosis of left eye for the last 20 years. Examination revealed a purplish vascular mass extending from the medial orbital region to the surface of the globe. He underwent complete excision of the mass via an anterior orbitotomy approach. Histopathology and immunohistochemistry revealed a diagnosis of angioleiomyoma. No recurrence was noted at 1 year of follow-up. Angioleiomyomas are benign smooth muscle tumors with an additional vascular component. Their occurrence in the orbit is extremely rare with only three cases reported in literature till date. We report a fourth case of angioleiomyoma of the orbit with the longest duration of presentation of 20 years.

Intracerebellar malignant nerve sheath tumor in a child: case report and review of literature.

PURPOSE: Intracerebellar malignant nerve sheath tumor (ICMNST) is an extremely rare entity, only two cases have been reported previously, and this is the first case to be reported in a child. The histogenesis, diagnosis, and management of this entity are very ambiguous, and natural history in a child is unknown. METHODS: The authors report a 7-year-old girl who presented with ataxia and signs of raised intracranial pressure and discuss the challenges in diagnosis, surgical strategy, and treatment. RESULTS: Following gross total resection and radiation to tumor bed, the patient had unremarkable recovery and is recurrence free at 1-year follow-up. CONCLUSION: ICMNSTs are extremely rare tumors of the cerebellum. Preoperative radiological diagnosis is not possible due to its close radiological resemblance to other common posterior fossa tumors. Immunohistochemistry plays a pivotal role in clinching the diagnosis. Though the reported adult counterparts have shown dismal prognosis, the pediatric counterparts may fare better with good surgical resection followed by radiotherapy.

Prediction of promiscuous epitopes in the e6 protein of three high risk human papilloma viruses: a computational approach.

A najor current challenge and constraint in cervical cancer research is the development of vaccines against human papilloma virus (HPV) epitopes. Although many studies are done on epitope identification on HPVs, no computational work has been carried out for high risk forms which are considered to cause cervical cancer. Of all the high risk HPVs, HPV 16, HPV 18 and HPV 45 are responsible for 94% of cervical cancers in women worldwide. In this work, we computationally predicted the promiscuous epitopes among the E6 proteins of high risk HPVs. We identified the conserved residues, HLA class I, HLA class II and B-cell epitopes along with their corresponding secondary structure conformations. We used extremely precise bioinformatics tools like ClustalW2, MAPPP, NetMHC, EpiJen, EpiTop 1.0, ABCpred, BCpred and PSIPred for achieving this task. Our study identified specific regions 'FAFR(K)DL' followed by 'KLPD(Q)LCTEL' fragments which proved to be promiscuous epitopes present in both human leukocyte antigen (HLA) class I, class II molecules and B cells as well. These fragments also follow every suitable character to be considered as promiscuous epitopes with supporting evidences of previously reported experimental results. Thus, we conclude that these regions should be considered as the important for design of specific therapeutic vaccines for cervical cancer.

Cryptophthalmos: reconstructive techniques--expanded classification of congenital symblepharon variant.

PURPOSE: To report the varied presentation and management of cryptophthalmos and further categorize grades of the congenital symblepharon variant based on the severity of the defect. METHODS: The records of 34 eyes of 25 patients with cryptophthalmos who sought treatment at the authors' tertiary eye care center over a period of 22 years were analyzed. RESULTS: Of the 34 eyes, 25 belonged to the congenital symblepharon variant, which could distinctively be further classified in medial/mild (4), moderate (9), and severe (11) subgroups and the surgical management for each has been outlined. The associated corneal and facial anomalies have also been highlighted along with their management modalities when indicated. CONCLUSIONS: Although a rare clinical entity, this is the largest series of cryptophthalmos reported so far with a proposed classification scheme for the congenital symblepharon variant.

Reconstructions of eyelid defects.

Eyelids are the protective mechanism of the eyes. The upper and lower eyelids have been formed for their specific functions by Nature. The eyelid defects are encountered in congenital anomalies, trauma, and postexcision for neoplasm. The reconstructions should be based on both functional and cosmetic aspects. The knowledge of the basic anatomy of the lids is a must. There are different techniques for reconstructing the upper eyelid, lower eyelid, and medial and lateral canthal areas. Many a times, the defects involve more than one area. For the reconstruction of the lid, the lining should be similar to the conjunctiva, a cover by skin and the middle layer to give firmness and support. It is important to understand the availability of various tissues for reconstruction. One layer should have the vascularity to support the other layer which can be a graft. A proper plan and execution of it is very important.

An unusual case of intraorbital foreign body and its management.

Intraorbital foreign bodies are usually the result of accidental trauma and can lead to considerable morbidity. We report an unusual case of an industrial injury in a plastic manufacturing unit wherein hot molten plastic splashed and solidified inside the orbit. The resultant increased intraorbital pressure led to loss of vision in that eye. The extreme temperature of the foreign body caused extensive thermal damage to the surrounding adnexal structures. Staged reconstructive surgery was undertaken to repair the damage, with an acceptable final cosmetic outcome. Employment of protective eye wear to prevent such accidents in high-risk occupations should be made mandatory.

APACHE-II scoring system in perforative peritonitis.

BACKGROUND: Prospective assessment of the Acute Physiology and Chronic Health Evaluation-II (APACHE-II) scoring system of stratification of disease severity has been shown to provide objective discrimination between low-risk and high-risk groups of patients with intra-abdominal sepsis. The current study was undertaken to evaluate the performance of APACHE-II score in prediction of mortality risk in patients with peritonitis due to hollow viscus perforation. STUDY DESIGN: Fifty patients admitted to a teaching hospital with peritonitis due to hollow viscus perforation were prospectively studied over a 2-year period. APACHE-II points were assigned to all patients in order to calculate their individual risk of mortality before undergoing emergency surgery. The accuracy in outcome prediction of the APACHE-II system was assessed by means of receiver operating characteristic (ROC) curve and the Pearson correlation coefficient and its significance test. RESULTS: Of the 50 patients admitted during the study period, there were 42 (84%) survivors and 8 (16%) nonsurvivors. Mean APACHE-II score of the study population was 11.38 with a range of 1 to 23. The predicted death rate was 23% and the observed death rate was 16%. Mean APACHE-II score in survivors was 9.88, whereas in nonsurvivors it was 19.25. Using ROC analysis, the area under the curve was found to be .984. Correlation of APACHE-II score and predicted death rate showed perfect correlation, with r = .99 and P <.001 [R2 = .9993]. APACHE-II score between 11 and 15 showed a sensitivity and specificity of 100% and 73.8%, respectively, and APACHE-II score of 16 to 20 had a sensitivity and specificity of 87.5% and 100%, respectively. CONCLUSION: APACHE-II score between 11 and 20 was shown to be a better predictor of risk of mortality in patients with peritonitis due to hollow viscus perforation. Predicted mortality did not correlate with observed mortality in patients with APACHE-II scores of 1 to 10 and greater than 20. The APACHE-II scoring system can be used to assess group outcomes in patients with peritonitis due to hollow viscus perforation. However, it does not provide sufficient confidence for outcome prediction in individual patients.

Cystic schwannoma of the orbit-a case series.

A schwannoma is an uncommon benign orbital tumor that arises from Schwann cells in the peripheral nervous system. Schwannoma with cystic degeneration is an even more rarely reported entity. Clinical examination alone is inadequate for the diagnosis. Radiological examination, like computed tomography (CT) scans, can help in the diagnosis; however, the diagnosis can only be confirmed by histopathological examination (HPE) after excision biopsy. Here, the authors report four cases of orbital schwannoma with cystic degeneration that presented with proptosis and decreased vision. CT scans showed a well-defined non-enhancing intraconal mass with cystic spaces. The histopathological examination was diagnostic for orbital schwannoma with cystic degeneration. Schwannoma should be included in the differential diagnosis of cystic orbital lesions.