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Midline vascular abdominal wall lesions are likely to be mistaken for vascular malformations in young children. We report a case of large yolk sac tumor located in the anterior abdominal wall just below xiphisternum in a 20-month-old girl diagnosed by raised serum alpha fetoprotein levels and fine-needle aspiration cytology. Preoperative chemotherapy helped in reducing its size allowing wide resection and primary wound closure. This case is reported for the unusual location and role of chemotherapy in management.

Background: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. Materials and Methods: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. Results: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11–16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75–5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5–12 years (median 3 years) follow-up including cellular mesoblastic nephroma. Conclusions: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

Background: Lumbar puncture (LP) is frequently performed in patients with advanced intraocular retinoblastoma. However, this may not be necessary in a significant proportion of patients. Materials and Methods: A file review of patients who were diagnosed with retinoblastoma over a 13-year-period was performed. Patients who underwent LP as part of staging were included in the study. Results: The study included 223 patients. One-third had bilateral retinoblastoma. The grouping was C, D, and E in 4 (2.9%), 41 (29.9%), and 92 (67.2%) patients, respectively. The stage was 0, I, II, III, and IV in 14 (6.3), 123 (55.2%), 13 (5.8%), 70 (31.4%), and 3 (1.3%) patients, respectively. Eight (3.6%) patients had a positive cerebrospinal fluid (CSF) cytology. None of the patients with intraocular disease and 7 (10%) patients with extraocular disease had a positive CSF. Conclusions: A diagnostic CSF is not indicated in patients with intraocular retinoblastoma.

A 14-year-old child with acute lymphoblastic leukemia who had completed induction chemotherapy presented with fever and diffuse musculoskeletal pains which was thought to be a constellation of myositis, arthralgias and arthritis. Investigations revealed initially showed normal peripheral blood counts but had pancytopenia and pre-terminally blasts were seen in the peripheral blood smear. He had bone marrow necrosis. Disseminated intravascular coagulation was suspected with a positive fungal serology. At autopsy, he had evidence of disease relapsed in lymph nodes, liver, spleen, testes and kidneys. There was extensive pseudomembranous colitis and appendicitis with changes of toxic megacolon.

Background: Accurate diagnosis of Non-Hodgkin′s Lymphoma (NHL) on fine-needle aspiration (FNA) specimen is challenging and requires ancillary testing. Aim: The feasibility of flow cytometric immunophenotyping (FCI) along with cell block immunocytochemistry (CB-ICC) as adjunct techniques in the diagnosis of NHL as per the current World Health Organization (WHO) classification was evaluated. Materials and Methods: All cases of suspected lymphoma underwent FNA, and the sample was triaged for light microscopic evaluation, FCI, and CB-ICC, and each case was classified as per the current WHO classification. Results: A total of 65 cases was analyzed which included 40 B-cell, 21 T-cell, and 4 unclassifiable lymphomas. Of 61 cases, FCI alone was contributory in 74% (45/61) cases whereas CB-ICC alone was contributory in 65.5% (40/61) cases in typing the lymphoma. In 11.4% (7/61) cases, the lymphoma could not be classified by either technique. Thus, in a total of 88.5% (54/61) cases a combination of FCI and CB-ICC from FNA enabled a diagnosis of lymphoma with its subtyping. Conclusion: Flow cytometric immunophenotyping and ICC on CBs are feasible on FNA material and are very useful in a suspected case of NHL especially when a biopsy may not be possible or feasible.

Background: Children presenting with typical clinical features of celiac disease (CD) are diagnosed relatively easily, however, diagnosis remains challenging and is often delayed when they present with ′difficult to treat anemia′ without overt gastrointestinal manifestations. Index study was undertaken to report profile of patients referred to pediatric hematology unit with ′difficult anemia′ who subsequently were diagnosed with CD. Materials and Methods: The records of 83 patients (1988-2008) with CD were scrutinized retrospectively who had presented with predominant hematological manifestations. Results: CD was confirmed histologically in 31 (37%), while 52 (63%) were diagnosed by serology alone. The mean age at diagnosis was 8.0 ± 2.8 years. The mean duration of symptom-diagnosis interval was 40.9 ± 30.6 months. Eighty-one (98%) children had anemia (Hb < 11 g/dl) and 55 (66%) had received iron supplements without discernible benefit. Thirty-nine (47%) patients received a blood transfusion. Thirty-six (43%) patients did not have diarrhea. Majority of the patients had either a microcytic-hypochromic (48%) or dimorphic (43%) anemia. Twenty-four (33%) had thrombocytosis, while 5 (7%) had thrombocytopenia. Mean duration of follow-up for patients on roll in the clinic for more than six months was 17.7 ± 20.9 months. Conclusion: Pediatricians and hematologists need to be aware of the extra-intestinal manifestations of CD. Prolonged duration of symptoms and a diagnosis at a relatively older age is striking in children presenting with predominantly hematological manifestations. Investigations for CD are recommended in children presenting with iron deficiency anemia refractory to hematinics or who have coexisting growth retardation. Necessity for biopsy in overtly symptomatic cases is discussed.