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.

Physiological dead space during general anaesthesia for Caesarean section.

The physiological dead space and its components were determined during general anaesthesia for Caesarean section in seventeen patients and compared with similar values for seventeen patients undergoing abdominal hysterectomy under general anaesthesia. The physiological dead space was smaller in the pregnant patients, due to a smaller alveolar dead space. The anatomical dead space was similar in both groups. A lowered physiological dead space suggests that there will be a decrease in the pulmonary ventilation perfusion ratio (V/Q) during Caesarean section; this could result from better perfusion of the ventilated alveoli than in the non-pregnant group, due to the increased cardiac output of pregnancy. An improvement in alveolar perfusion should also produce more efficient carbon dioxide elimination in pregnant patients, when compared to non-pregnant subjects.

Arterial to end tidal carbon dioxide tension difference during caesarean section anaesthesia.

The relationship between arterial carbon dioxide tension and end tidal carbon dioxide tension was studied in 19 patients during general anaesthesia for Caesarean section. Thirteen patients scheduled for elective abdominal hysterectomy formed a nonpregnant group. There was significant correlation between arterial and end tidal CO2 tensions in both groups. During Caesarean section, this difference was significantly less than in the nonpregnant group.

Flow requirements for the Bain breathing circuit during anaesthesia for caesarean section.

We studied the relationship between arterial carbon dioxide tension (PaCO2) and fresh gas flow (FGF) during use of the Bain breathing circuit for Caesarean section anaesthesia. Thirty-one patients undergoing Caesarean section were anaesthetised using the Bain circuit with intermittent positive pressure ventilation. The PaCO2 were measured at FGF of 70 ml X kg-1 X min-1, 80 ml X kg-1 X min-1, and 100 ml X kg-1 X min-1. The FGF requirement to maintain a given PaCO2 during Caesarean section anaesthesia is the same as the requirements for nonpregnant subjects, despite the increase in carbon dioxide production associated with pregnancy. This is probably because the total FGF determined by body weight and given during Caesarean section anaesthesia is 15-20 per cent higher than nonpregnant levels, due to the weight gain associated with pregnancy. A FGF of 100 ml X kg-1 of pregnant weight/min maintains PaCO2 of 4.44 kPa predelivery, which is in the desirable range of PaCO2 during Caesarean section.

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.

Arterial to end-tidal carbon dioxide tension difference during anaesthesia in early pregnancy.

Sixteen patients requiring general anaesthesia for termination of pregnancy by dilatation and evacuation of the uterus were studied. Arterial and end-tidal carbon dioxide tensions were determined during anaesthesia. The mean arterial to end-tidal carbon dioxide tension difference was 0.07 kPa (-0.02-0.16, 5-95 per cent confidence limits). These results were similar to those observed during Caesarean section and those during anaesthesia for post-delivery tubal ligations. The physiological changes such as increased cardiac output, haemodilution, and increased blood volume which manifest by 12 weeks of gestation probably result in a reduced (a-E')PCO2 value.

Arterial to end-tidal carbon dioxide tension difference during anaesthesia for tubal ligation.

Twenty-nine patients scheduled for postnatal tubal ligation by minilaparotomy under general anaesthesia were studied. Arterial and end-tidal carbon dioxide tensions were determined during anaesthesia. The mean arterial to end-tidal carbon dioxide tension difference was 0.08 kPa (SEM 0.05). Thirty-one percent of the patients had negative values. These results were similar to those observed during Caesarean section. The physiological changes responsible for reduced arterial to end-tidal carbon dioxide values, persist into the postnatal period. It is predicted from the regression analysis of the time between delivery and anaesthesia for tubal ligation and arterial to end-tidal CO2 difference, that the values might return to normal nonpregnant levels by 8 days following delivery.

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.