Solitary Ganglioneuromatous Hamartoma Tongue in a Newborn - A Rare Entity.

Ganglioneuromatous hamartoma is a benign tumour of autonomic ganglia with very few cases reported in head and neck region. In this report, we are presenting a case of ganglioneuromatous hamartoma in a 20 day old female child who presented with a tongue mass. She underwent a surgical excision and the definitive diagnosis was made by histopathology. This case reports discusses presentation and management of a case of ganglioneuromatous hamartoma.

Paediatric Hemophagocytic Lymphohistiocytosis: A Case Series With a Diverse Spectrum From a Resource-Limited Setting.

Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterized by cytokine storms leading to multi-organ dysfunction and is a highly fatal disease. Infectious diseases are the most common cause of secondary HLH. A wide variety of infections can lead to secondary HLH.  In this case series, we report five cases of HLH which had different therapeutic approaches and varied clinical courses, with one of them diagnosed as a rare entity of coronavirus disease 2019 (COVID-19)-associated HLH of multisystem inflammatory syndrome in children (MISC) spectrum, one case each of idiopathic HLH, staphylococcal infection-associated secondary HLH, leptospirosis with secondary HLH and dengue-associated HLH. The case of idiopathic HLH required initiation of immunosuppressive therapy but had a fatal outcome while others were treated successfully with antibiotics, steroids, intravenous immunoglobulin and supportive therapy. Our case series highlights the importance of evaluating for all possible infective causes thoroughly in HLH. Most patients can be managed without chemotherapy by treating the secondary causes of HLH, including common tropical infections and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection.

Impact of COVID-19 Pandemic on Pathology Residency Program: An Experience from India.

OBJECTIVE: To study the impact of the COVID-19 pandemic on pathology residents through a questionnaire-based survey. MATERIAL AND METHOD: We designed a Google survey questionnaire with 20-questions and distributed it to the pathology residents across India via e-mail and WhatsApp. All the responses collected were analysed using appropriate statistical methods. RESULTS: We received a total of 81 responses. The majority (n=55, 68.8%) of the residents were aged 26-30 years with a male-female ratio of 1:2.2. Residents reported a significant decrease in classes as compared to pre-covid times. However, most institutions (90%) shifted to the virtual method for various teaching sessions. About 94.7% of the residents felt a fall in the quality of training due to Covid. A significant number of junior residents (76.92%) reported an inability to complete the target thesis enrolment. The residents saw a substantial decrease in the number of peripheral smears, bone marrow, cytology, and histopathology cases compared to pre-Covid times (p value < 0.001 for all). An overwhelming 83.8% of the pathology residents were posted for COVID-19 duties. About 48.8% turned Covid positive. About 77.5% (n=62) of residents felt that the necessary training period would be extended. CONCLUSION: The COVID-19 pandemic has immensely affected the training and teaching of pathology residents in India. Similarly, this pandemic must have affected pathology residents all across the globe. Therefore, institutions can consider offering an extended period of up to one year, depending upon residents' requests.

Anaplastic Rhabdomyosarcoma of Upper Lip in a 3-Year-Old Girl.

Rhabdomyosarcoma is an aggressive malignant striated muscle neoplasm commonly seen in children involving orbit, paranasal sinuses, cheek, tongue, and rarely upper lip. The anaplastic subtype is further rare and associated with poor prognosis. Herein, we report a 3-year-old female with this uncommon variant at an uncommon site.

A Retrospective Analysis of the Importance of Biochemical and Hematological Parameters for Mortality Prediction in COVID-19 Cases.

Introduction The recent coronavirus disease 2019 (COVID-19) pandemic has devastated the world's health and economy and has devastatingly affected social and emotional spheres. Although it was the older population who faced the worst, a good number of the younger population also lost their lives. It was very important to predict who will progress to the worst clinical outcome. Consequently, quick and accurate ways of forecasting mortality in COVID-19 cases are essential to save lives. In this study, 11 predictive parameters, namely, D-dimer, creatinine, C-reactive protein (CRP), lactate dehydrogenase (LDH), ferritin, neutrophil/lymphocyte ratio (NLR), platelet/lymphocyte ratio (PLR), systemic inflammatory index (SII), platelet count, absolute neutrophil count (ANC), and absolute lymphocyte count (ALC), were studied for determining their significance as predictive parameters of mortality in COVID-19-affected patients. Methods We conducted a retrospective study of essential parameters, namely, D-dimer, creatinine, CRP, LDH, ferritin, NLR, PLR, SII, platelet count, ANC, and ALC, in confirmed COVID-19 cases for one year between 2020 and 2021. The medical information was obtained from the digital storage sources of the hospital. All cases were segregated into surviving and non-surviving cases. All parameters were collected, results were tabulated, and each individual parameter was then analyzed to see if it showed any significant deviations in non-surviving cases and could help predict mortalities. Statistical analysis was conducted using the latest version of the Statistical Package for the Social Sciences (SPSS) software (IBM SPSS Statistics, Armonk, NY, USA). Results Each of the parameters was individually studied. D-dimer, creatinine, LDH, ferritin, CRP, NLR, PLR, SII, and ANC showed a statistically significant increase in non-surviving cases. Compared to surviving cases, ALC and platelets showed a statistically significant decrease in non-surviving instances. Conclusion All the studied parameters showed significant deviations in non-surviving cases and could help predict mortalities. This study also stresses the utility of readily available hematological ratios such as NLR and SII for prognosis in COVID-19 subjects.

Gastrointestinal mucormycosis in the pediatric age group: an evolving disease.

BACKGROUND: Mucormycosis is a devastating opportunistic fungal infection resulting in significant mortality, especially in pediatric patients with predisposing risk factors. MATERIALS & METHODS: Biopsies and surgical specimens reported and proven as Mucormycosis in children under 12 years of age were retrieved from the records for three years (January 2018 to January 2021). Complete data, predisposing factors, treatment, and clinical outcome were recorded. RESULTS: 15 cases were identified, ranging from 9 days to 5 years. The male-female ratio was 3:1; three children were preterm. Fourteen children were diagnosed with gastrointestinal Mucormycosis (14/15), and one had palatal and sinusoidal involvement. Abdominal pain with distention was the most typical complaint. On microscopy, biopsies and surgical specimens showed extensive liquefactive necrosis with broad aseptate fungal hyphae. An intraoperative diagnosis was rendered in two cases. All neonates underwent exploratory laparotomy with surgical debridement and were administered Liposomal Amphotericin B. However, only two neonates survived out of the fifteen cases, one with disease limited to the appendix and pouch colon. The others succumbed to the disease despite antifungal therapy and surgical debridement. Thus, the overall mortality in the current study was calculated to be 86%, with neonatal mortality of 75%. CONCLUSION: Gastrointestinal involvement is more common in neonates and infants with a male preponderance. The diagnosis relies on direct microscopy, histopathology, and fungal culture. Intraoperative tissue may be sent in all suspected cases for direct microscopic examination for rapid diagnosis and treatment.

An unusual case of respiratory epithelial cyst of orbit presenting as a lid swelling in a child.

This case highlights the importance of histopathological examination in all cases of choristoma undergoing excision.

Pediatric Colorectal Carcinoma: A Series of Seven Cases.

Introduction: Colorectal carcinoma (CRC) accounts for <1% of all the neoplasms of children. Unfavorable histology and delayed diagnosis often result in poor outcome. This study aimed to investigate the clinical characteristic and prognosis of pediatric patients with CRC. Materials and Methods: A retrospective review of medical records of all patients diagnosed of CRC between 2015 and 2020 was performed. Data regarding gender, age, location, and histopathology were collected. Results: Seven children (5 males and 2 females) were included in the study. Age ranged between 8.6 and 11.8 years. Abdominal pain was the most common symptom. The duration between onset of symptoms to the diagnosis ranged from 5 days to 8 months. Right-sided tumors were more common than the left side. Histopathological examination showed mucinous adenocarcinoma in four/seven patients and signet cell variant was seen in 2/7. Immunohistochemistry was positive for cytokeratin and beta-catenin, however, all microsatellite instability markers were found negative. Despite surgical treatment and chemotherapy, four of our cases faced mortality and one patient was lost to follow-up. Conclusion: CRC can present in first decade of life and often misdiagnosed. Pediatric CRC has distinct tumor biology and shares a poor prognosis. Paucity of literature from the Indian subcontinent should urge further clinicopathological trials for establishing etiology and for refining treatment recommendations in these children.

Neuroblastoma: Application of International Neuroblastoma Pathology Classification on fine needle aspiration cytology smears.

Background: Neuroblastoma (NB) is the fourth most common tumor of childhood. There is a paucity of literature on its subtyping of cytology and prognostic utility. Aims: We aimed to study the cytopathological features of NB on the aspirated material, subtype it, and assess the role of International Neuroblastoma Pathology Classification (INPC) classification on cytology smears in the preoperative prognosis of NB. Materials and Methods: Fifteen cases of NB reported on fine-needle aspiration cytology (FNAC) in the past 3 years were included. Detailed clinical, radiological, and cytological features were noted. Smears were assessed for characteristics such as cellularity, neuroblasts (cytoplasmic, nuclear details), rosettes, neuropil, Schwann cells, fibroblasts, calcification, and necrosis. Afterward, cases were categorized as undifferentiated (UD), poorly differentiated (PD), and differentiating (D) subtypes. Mitotic-karyorrhectic index (MKI) was calculated and correlated with histopathology. Follow-up was done to date. Results: The age ranged from 19 days to 10 years with an M: F ratio of 3:1. Twelve cases were retroperitoneal, two cervical, and one mediastinal. Metastatic disease was seen in six cases, one to the cervical, four to the bone marrow, and two to the scalp. The International Neuroblastoma Risk Group (INRG) staging system was available in all cases, out of which three were in stage L1, six in stage L2, four in stage M, and two in stage Ms. On cytology, four cases were differentiating NB, five PD NB, and six UD NB. The MKI was high (>4%) in 80% of UD, intermediate (2-4%) in 100% of PD, and low (<2%) in 75% of D cases. MKI corroborated in both histology and cytology, except in one case. Conclusion: NB can be subtyped on cytology on the basis of characteristics of neuroblasts, presence of neutrophils, rosettes, and necrosis. UD NB has a high MKI and is associated with a poor prognosis. A preoperative comprehensive reporting of NB on cytology can be very useful in guiding appropriate chemotherapy with some increment in survival. However, larger studies are needed to validate the calculation of MKI on FNA smears.

Neonate with Congenital Duodenal Obstruction and Ectopic Hepatic Parenchyma.

BackgroundThe presence of hepatic parenchyma at ectopic locations is infrequently reported in neonatal age. Case details: A male neonate presented with clinical signs and symptoms of duodenal obstruction. At exploration, an annular pancreas was found as the causative factor and he underwent a Kimura's duodeno-duodenostomy. A pedicled cyst was attached to the stomach's greater curvature, was excised, and histologically was a mesothelial-lined cyst with ectopic liver, complete with bile ducts, in the cyst wall. Conclusion: Ectopic liver tissue may be clinically silent and found within the wall of a mesothelial cyst. Long-term complications of this ectopic tissue are not known.

Peripheral Lymphadenopathy in Children: Cytomorphological Spectrum and Interesting Diagnoses.

OBJECTIVE: Peripheral lymphadenopathy is a common complaint in the pediatric outpatient department. Fine needle aspiration cytology is the first investigation of choice with a high sensitivity for diagnosis but cytology may be challenging in some cases. The study was planned to study the cytomorphological spectrum and discuss a few interesting cases. MATERIAL AND METHOD: 1890 paediatric subjects' up to 12 years of age with significant peripheral lymph node enlargement and an adequate cytology specimen were included in the study. Inadequate aspirates were excluded. RESULTS: The majority of children presented within 4-8 years of age with a male to female ratio of 1.7:1. The anterior cervical group was most commonly affected, followed by the posterior cervical, axillary and inguinal. Reactive lymphadenitis constituted the majority of the diagnoses, followed by Tuberculosis, acute suppurative, BCG-induced lymphadenitis, Kimura disease, Rosai-Dorfmann disease and Kikuchi-Fujimoto disease. Lymphomas and metastatic malignancies were less common, and mainly consisted of Hodgkin lymphoma, non-Hodgkin lymphoma, anaplastic large cell lymphoma, and Langerhans cell histiocytosis. Cytomorphological features of a few challenging and interesting cases have been discussed. CONCLUSION: Non neoplastic causes of lymphadenopathy predominate in the pediatric age group. A definitive diagnosis rests upon a complete clinical, radiological, microbiological, and cyto-histopathological correlation with the use of ancillary techniques wherever necessary.

Association of Anti-tissue Transglutaminase Antibody Titers and Duodenal Biopsy Findings in Pediatric Patients of Celiac Disease.

Aims & Objectives To ascertain the association of serum anti-tissue transglutaminase (anti-tTG) antibody titers with the severity of duodenal mucosal damage on histology andto predict a possible cut-off value of anti-tTG antibody titers for the diagnosis of Celiac disease. Marsh grading greater than two in conjunction with clinical assessment, which may help avert an invasive endoscopic procedure, especially in medically unfit children. Materials & Methods A retrospective study was designed wherein demographic and laboratory data of children aged less than 12 years with raised anti-tTG antibody titers with available histopathology of duodenal biopsies were extracted from the hospital medical records and reviewed. Results A total of 134 children were included in the study, which showed female preponderance. Histopathological changes, characteristic of Celiac disease, were observed in 116 cases; seven among the rest showed evidence of Giardiasis, and 13 could be considered potential Celiac. Of the 116 patients, 1.7% belonged to Marsh grade I, 5.2% grade II and 8.6%, 26.7%, and 57.7% to grade IIIA, IIIB, and IIIC, respectively. A significant association was found between anti-tTG antibody titers and Marsh grading. The cut-off value of anti-tTG antibody titer levels for diagnosing Celiac disease using receiver operating characteristics (ROC) curve in predicting Marsh greater than two at histology was observed to be 84.6 U/ml with sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of 91.7%, 68.4%, 94.2%, and 59%, respectively. Conclusion An anti-tTG antibody titer greater than 10 times the upper limit of normal (≥84 U/ml) is significantly associated with Marsh grade greater than two. Standard stool microscopy may be used as a simple tool in the workup of all children with raised anti-tTG antibody levels to rule out Giardiasis to avert unnecessary endoscopic evaluation for Celiac disease in such cases.

Kaposiform haemangioendothelioma of duodenum in a neonate.

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.

Reliable cerebrospinal fluid cytology reporting over an extended period: Is it possible by adding formol saline and ethylenediaminetetraacetic acid as cell preservative?

BACKGROUND: Reporting cerebrospinal fluid (CSF) cytology within a narrow time frame is crucial as it is often indicated in critically ill patients and moreover, the cells in CSF are highly labile and tend to decline rapidly on standing. However, due to various logistic issues, delay in reporting is inevitable at times, especially if ancillary tools are required. In this study, we examine the effect of using formol saline and ethylenediaminetetraacetic acid (EDTA) as a preservative on the cellular composition of CSF at 18, 24, and 48 hours of preservation. METHODS: Thirty CSF specimens were examined within 2 hours of collection and this reading was recorded as time zero reading. The CSF specimens were then divided in three tubes with: (a) preservative:CSF ratio of 1:1; (b) preservative:CSF ratio of 1:5; and (c) no preservative. Total and differential leucocyte counts and immunocytochemistry were performed on the three specimens at 18, 24, and 48 hours and were compared with the readings at 0 hour. RESULTS: Preserved CSF (in the ratio of 1:5) showed no significant decrease in the number of cells at 18 hours (P = .4), 24 hours (P = .3), and 48 hours (P = .1). Cellularity decreased by 8.5%, 22%, and 40% at 18, 24, and 48 hours, respectively. Cell morphology and antigenicity were well preserved at all the three time intervals. CONCLUSION: Formol saline and EDTA, when mixed with the CSF in the ratio of 1:5, can preserve significant cellularity for up to 24 hours.

Spindle cell lipoma, dorsum of hand in a 4 year old child: A rare entity.

Lipogenic tumors are an uncommon entity in children. There are several variants of lipoma, of which spindle cell lipoma is an extremely rare and accounts for only 1.5% of all lipoma diagnosis. Spindle cell lipoma rarely involves distal extremities. We report a 4-year old boy with swelling over dorsum of hand operated for aesthetic purposes. The report also highlights the salient cytological features, which can help differentiate this entity from other closer mimics on aspiration cytology, guiding the surgeon for appropriate management.

Benign vascular anomalies: A transition from morphological to etiological classification.

The International Society for the Study of Vascular Anomalies (ISSVA) devised a multidisciplinary etiopathogenesis based approach to classify benign vascular anomalies into tumors and malformations. This classification scheme has major therapeutic and prognostic implications as treatment modalities differ for both the categories. Inappropriate usage of the term "hemangioma" for etiopathogenetically distinct entities is commonly seen in clinical practice leading to delivery of incorrect treatment to the patients. We aimed to study the histomorphological and immunohistochemical features of benign vascular anomalies for their precise histopathological classification. A total of 48 cases diagnosed over a period of 3.5 years were reviewed and reclassified into vascular tumors and malformations based on ISSVA classification and prototypical histopathological features. Biopsies were reviewed based on 5 histopathological criteria viz. endothelial morphology, mitotic activity, intralesional nerve bundles, intralesional inflammation, and prominent vessel type. A panel of GLUT-1, WT-1, and Ki-67 was performed in each case. Seven cases of infantile hemangioma, 4 cases each of non-involuting congenital hemangioma and pyogenic granuloma, and 33 cases of vascular malformations were diagnosed. Endothelial cell morphology (p < 0.001), mitotic activity (p < 0.001), and intralesional nerve bundles (p < 0.001) were found to be statistically significant in differentiating hemangioma from malformations. GLUT-1 (p < 0.001) and Ki-67 labeling index (p < 0.001) were useful to distinguish infantile hemangioma from vascular malformations. To conclude, the ISSVA classification of benign vascular anomalies can be reliably done on histopathology. However, every case must be interpreted in the light of clinical and radiological features.