Clinical and histopathological spectrum of cranial small round cell tumors: An experience from a tertiary care center.

BACKGROUND: Small round cell tumors (SRCTs) are a group of malignant neoplasms with minimal or no differentiation, characterized by the presence of round cells with high nuclear-cytoplasmic ratio. Although SRCTs can occur in any part of the body, involvement of central nervous system (CNS) is uncommon. AIM: We aimed to study the clinicopathological spectrum of cranial SRCT diagnosed in our institute over a period of four years (2016-2019). MATERIAL AND METHODS: A retrospective review of medical records (2016-2019) with a morphological diagnosis of cranial SRCT was made. Both intra-axial and extra-axial tumors were included. A total of 60 cases were retrieved, and the clinical and histopathological features were studied. Special cytochemical staining and immunohistochemistry were performed, where needed. RESULTS: The mean age at presentation was 18.4 years (range, 1-60 years), with a male-to-female ratio of 2.5:1. The most common site was posterior fossa of brain (n = 28, 47%), followed by dorso-lumbar spine (n = 9, 15%). The most common type of tumor was medulloblastoma (n = 29, 48.3%), followed by Ewing sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) (n = 11, 18.3%), non-Hodgkin lymphoma (NHL) (n = 9, 15%), neuroblastoma (n = 3, 5%), and CNS embryonal tumor, NOS (n = 2, 3.3%). One case each of atypical teratoid rhabdoid tumor (ATRT), rhabdomyosarcoma, pineoblastoma, melanoma, rhabdomyosarcoma, and undifferentiated pleomorphic sarcoma was also documented. CONCLUSIONS: SRCTs have a variable age of presentation. Their incidence in CNS is low as compared to other organ systems. On light microscopy, the histopathology of these lesions is overlapping, posing a great diagnostic dilemma for the pathologist. The use of ancillary techniques like immunohistochemistry helps in arriving at the correct diagnosis. Treatment strategy and tumor prognosis also vary along the entire spectrum of SRCT, thus making exact characterization essential for proper management.

Diagnostic algorithm for pathological evaluation of gliomas in a resource-constrained setting.

Introduction: Gliomas are the most common primary intracranial tumors. The current World Health Organization (WHO) classification of central nervous system tumors recommends integrated histo-molecular diagnosis of gliomas. However, molecular testing is not available in even most of the advanced centers of our country, and histopathology aided with immunohistochemistry (IHC) is still widely used for diagnosis. Immunohistochemical markers such as iso-citrate dehydrogenase1 (IDH1) and Alpha Thalassemia/Mental Retardation Syndrome X-linked (ATRX) can be reliably used for the correct diagnosis, prognosis, and treatment of gliomas. Aim: We aimed to develop a diagnostic algorithm by integrating morphology, IDH1, and ATRX status of gliomas seen in our institute for 1 year. Settings and Design: Analytical cross-sectional study. Materials and Methods: This study included 60 histopathologically confirmed cases of astrocytic (n = 51) and oligodendroglial tumors (n = 9). Clinical, radiological, and histopathological features were noted and tumor grades assigned according to the WHO recommendations. IDH1 and ATRX mutation status was evaluated using IHC. The tumors were divided into three molecular groups on the basis of their IDH1 and ATRX mutation status: (1) Group 1: IDH1 negative and ATRX positive, (2) Group 2: IDH1 positive and ATRX positive, (3) Group 3: IDH1 positive and ATRX negative. Results: The mean age of presentation was 45.0 ± 15.8 years with a male-to-female ratio of 2:1. Seizures, headache, and hemiparesis were the most common modes of presentation. The tumor subtypes studied were glioblastoma (n = 32), anaplastic astrocytoma (n = 7), diffuse astrocytoma (n = 6), oligodendroglioma (n = 6), pilocytic astrocytoma (n = 6), and anaplastic oligodendroglioma (n = 3). IDH1 mutation was present in 26 cases including anaplastic astrocytoma (n = 7), diffuse astrocytoma (n = 6), oligodendroglioma (n = 5), secondary glioblastoma (n = 5), and anaplastic oligodendroglioma (n = 3). ATRX mutation, i. e., loss of ATRX was observed in 17 cases including diffuse astrocytoma (n = 5), anaplastic astocytoma (n = 5), anaplastic oligodendroglioma (n = 3), oligodendroglioma (n = 3), and secondary glioblastoma (n = 1). All six cases of pilocytic astrocytoma were negative for IDH1 and ATRX mutation. There were 34 patients in Group 1 (IDH1- and ATRX +), nine cases in Group 2 (IDH1 + and ATRX +), and 17 patients in Group 3 (IDH1 + and ATRX-). Conclusion: Diagnosis of gliomas should be based on a detailed clinicoradiological and histopathological assessment, followed by genotypic characterization. Evaluation for IDH1and ATRX status has both diagnostic and prognostic value as it helps in differentiating gliomas from reactive gliosis, primary glioblastoma from secondary glioblastoma, and pilocytic astrocytoma (WHO grade I) from diffuse astrocytoma (WHO grade II). Tumors with IDH1 mutations have a better outcome than those with wild-type IDH. IHC can serve as a useful surrogate to conventional molecular tests in resource-constrained settings. By devising an algorithm based on morphological and IHC features, we were able to stratify gliomas into three prognostic subgroups.

Clinical and histopathological profile of dysembryoplastic neuroepithelial tumor: An experience from a tertiary care center.

INTRODUCTION: Dysembryoplastic neuroepithelial tumor (DNT) is a rare benign brain tumor predominantly involving children and young adults. Histologically, it corresponds to WHO Grade I tumors; however, it may masquerade aggressive neural tumors such as oligodendroglioma, oligoastrocytoma, pilocytic astrocytoma, and ganglioglioma. The literature on clinical, radiological, and pathological spectrum of DNT is described mostly in the form of case reports, with only a few case series reported till date. METHODS: A retrospective review of files with diagnosis of DNT (2016 to 2018) was made in the Department of Pathology, National Institute of Pathology, New Delhi. A total of ten cases were retrieved, and their clinical, radiological, and histopathological features were reviewed and studied. Special stains and immunohistochemistry were done, wherever required. RESULTS: The mean age was 14.8 (±7.9) years, with a male-to-female ratio of 1.5:1. The most common mode of presentation was recurrent, intractable seizures. The most common site of lesion was parietal lobe followed by temporal and frontal lobes of the brain. On histology, mucoid matrix admixed with floating neurons and oligodendrocyte-like cells was a consistent feature; however, the presence of specific glioneuronal elements was observed in only a few cases. CONCLUSIONS: DNT is a benign, low-grade, nonrecurrent neuroepithelial neoplasm. It is important to differentiate this rare entity from other mimickers, as it is surgically curable and carries an excellent prognosis without the need for adjuvant chemotherapy and radiotherapy. The study helps to enrich the clinicopathological aspects of this rare but important entity.

Cranial Mycetoma: A Rare Case Report with Review of Literature.

Mycetoma is a chronic granulomatous infection caused by fungi or bacteria, known as eumycetoma and actinomycetoma, respectively. Mycetoma commonly affects young males belonging to low socioeconomic strata, usually barefooted agricultural workers. It mainly affects lower and upper limbs presenting as a painless swelling with discharging sinus. Rarely, is it encountered in the intracranial location. The diagnosis relies on the clinical presentation and identification of the etiological agents within the tissue, by histology and special stains. It is important to specify the fungal or bacterial etiology, because the treatment of each is completely different. The management of such infections is challenging and should involve early diagnosis, the use of antibacterials or antifungals, and surgical removal of the lesion. To the best of our knowledge, only seven cases of intracranial mycetoma have been reported. The present case highlights the rarity of this lesion, thereby contributing to the existing literature and presenting its diagnostic implications.

Bilateral rhabdoid meningioma mimicking glioma: an unusual occurrence.

Rhabdoid meningioma is an infrequent variant of meningioma, introduced for the first time in the 2000 World Health Organization's classification of tumors of the nervous system. Owing to its aggressive clinical course and high proliferative index, it has been classified as a grade III neoplasm. We describe a fifty-year-old male with headache, weakness of limbs, and altered sensorium. CT showed hetero-dense enhancing mass lesions in both right and left parietal areas raising suspicion of high grade glioma. Histopathologic and immunohistochemical examination revealed a tumor with features of rhabdoid meningioma. A review of literature did not reveal any bilateral occurrence of this tumor.

Synthesis and evaluation of a series of benzothiophene acrylonitrile analogs as anticancer agents.

A new library of small molecules with structural features resembling combretastatin analogs was synthesized and evaluated for anticancer activity against a panel of 60 human cancer cell lines. Three novel acrylonitrile analogs (5, 6 and 13) caused a significant reduction in cell growth in almost all the cell lines examined, with GI50 values generally in the range 10-100 nM. Based on the structural characteristics of similar drugs, we hypothesized that the cytotoxic activity was likely due to interaction with tubulin. Furthermore, these compounds appeared to overcome cell-associated P-glycoprotein (P-gp)-mediated resistance, since they were equipotent in inhibiting OVCAR8 and NCI/ADR-Res cell growth. Given that antitubulin drugs are among the most effective agents for the treatment of advanced prostate cancer we sought to validate the results from the 60 cell panel by studying the representative analog 6 utilizing prostate cancer cell lines, as well as exploring the molecular mechanism of the cytotoxic action of this analog.

Anesthesia outside the operating room for emergency procedures.

Non-anesthetists usually provide sedation and anesthesia outside the operating room for emergency procedures. Techniques vary from no sedation to deep sedation using drugs with a good safety profile and few side effects. Newer methods of airway control may allow volatile agents such as sevoflurane to be used. Anesthetists may need to join sedation teams if they are to maintain control of their specialty.

Anaesthesia in Barbados.

PURPOSE: To describe the anaesthesia services in Barbados: to present the major challenges confronting the Anaesthesia Department of the government-owned Queen Elizabeth Hospital (QEH): and to describe the Department's approaches to optimise safety and cost-effectiveness of anaesthesia at QEH. SOURCE OF INFORMATION: Authors (KBS, HSLM, RAH), who collectively provided more than 50 yr of anaesthesia at QEH; the Dean (ERW) of the University of West Indies Medical School (Barbados campus); archives of Barbados; and records of QEH. PRINCIPAL FINDINGS: The government of Barbados provides modern health care services to all of its citizens, primarily at QEH. Barbados, however, has tight financial constraints, infrastructural limitations, and a bureaucratic administration that predispose QEH's Anaesthesia Department to unexpected depletions of drugs and disposable supplies, sporadic shortages of personnel and functioning equipment, and occasional quality assurance problems. To deal with such problems, the Anaesthesia Department has implemented several pro-active measures: establishing an audit system to prevent depletion of imported drugs and supplies: training local personnel to maintain equipment: purchasing an oxygen concentrator to reduce oxygen costs: decreasing nitrous oxide use (expensive in Barbados): and initiating its own quality and safety standards. CONCLUSION: Continuous delivery of high quality, cost-effective anaesthesia care requires thoughtful planning by administrators and judicious resource allocations. Health care administrators and clinical departments need to work together closely to establish a framework that enables departments to play a major role in determining how the institution's limited financial resources are best allocated to meet the departmental priorities.

Total intravenous anaesthesia for day-care surgery using diclofenac for analgesia - abstract

The trend in modern surgery is to do more and more cases as "Day Care". This approach saves money by promoting the efficient use of available bed space and the time of hospital-based health professionals. The main objective of the anaesthetic technique is to produce conditions satisfactory to surgeon and patient, using agents that allow for the safe and rapid discharge of the patient from the hospital. Inhalation techniques with halothane and by using narcotics for post-operative analgesia are the standard techniques used in the Queen Elizabeth Hospital in Barbados. However, studies have shown that the combination of various narcotics such as Fentanyl and Alfentanyl for analgesia in total intravenous anaesthesia techniques, using continuous intravenous Propofol, will produce satisfactory anaesthesia for many types of surgical procedures with short recovery periods. Substitution of analgesics in the non-steroidal anti-flammatory drug group (NSAIDs), in combination with Propofol, offers the opportunity for avoiding the side-effects of narcotics. Accordingly, the effectiveness of total intravenous anaesthesia, using Propofol with the NSAID Diclofenac for analgesia in patients undergoing laparoscopic gynaecological surgery as day-care patients was compared with the standard inhalation anaesthetic technique, using a narcotic (Fetanyl) analgesia. The thirty-eight female patients studied were induced with Propofol (2.5 mg/kg-1) for muscle relaxation. Nineteen received Fentanyl (1-1.5 ægm kg-1) i.v. immediately prior to induction and anaesthesia was continued, using 50 percent nitrous oxide (2-1 min-1) in oxygen and Halothane (1-1.5 percent). The other nineteen received Diclofenac (75 mg i.m.) one hour before surgery and anaesthesia was continued with an infusion of Propofol, using an Ohmeda 9000 syringe pump at 12 mg. kg-1.h-1. Conditions for surgery were satisfactory in both groups and there was no signidicant difference in the degree of post-operative analgesia or the time to awaken under anaesthesia and all patients said they would be happy to have the anaesthetic technique used again. However, the total intravenous technique is expensive and therefore its use is recommended only when inhalation methods or the use of narcotics in undesirable (AU)