Pediatric Undifferentiated Pleomorphic Sarcoma of the Cecum.

Background: Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm typically diagnosed in older adults and localized to the extremities or retroperitoneum. Because of poor response to therapy and high rates of recurrence, this neoplasm is associated with a poor prognosis. Case Report: A 12-year-old female presented with weight loss, abdominal pain, fatigue, and diarrhea. She was profoundly anemic with occult blood-positive stools. On endoscopy, a fungating cecal mass was biopsied and diagnosed as malignant sarcomatoid neoplasm. The neoplasm was resected with clear margins during subsequent surgery, and on final pathology was diagnosed as UPS. A suspicious lung nodule was also removed via video-assisted thoracoscopic surgery and found to be a granuloma positive for Histoplasma capsulatum for which the patient received antifungal therapy. The patient did not receive additional chemotherapy or radiotherapy and was doing well without signs of recurrence at 12 months postresection. Conclusion: This report of cecal UPS in a 12-year-old is rare because of the patient's age and tumor location. We have identified only 2 other case reports of pediatric gastrointestinal UPS. This case illustrates the need for a broad differential and prompt workup in pediatric patients presenting with weight loss and abdominal complaints. More information regarding the management and outcomes in cases of gastrointestinal UPS is needed to assist providers in determining the best treatment course and to allow for better prognostication.

EBV-driven lymphoid neoplasms associated with pediatric ALL maintenance therapy.

The development of a second malignancy after the diagnosis of childhood acute lymphoblastic leukemia (ALL) is a rare event. Certain second malignancies have been linked with specific elements of leukemia therapy, yet the etiology of most second neoplasms remains obscure and their optimal management strategies are unclear. This is a first comprehensive report of non-Hodgkin lymphomas (NHLs) following pediatric ALL therapy, excluding stem-cell transplantation. We analyzed data of patients who developed NHL following ALL diagnosis and were enrolled in 12 collaborative pediatric ALL trials between 1980-2018. Eighty-five patients developed NHL, with mature B-cell lymphoproliferations as the dominant subtype (56 of 85 cases). Forty-six of these 56 cases (82%) occurred during or within 6 months of maintenance therapy. The majority exhibited histopathological characteristics associated with immunodeficiency (65%), predominantly evidence of Epstein-Barr virus-driven lymphoproliferation. We investigated 66 cases of post-ALL immunodeficiency-associated lymphoid neoplasms, 52 from our study and 14 additional cases from a literature search. With a median follow-up of 4.9 years, the 5-year overall survival for the 66 patients with immunodeficiency-associated lymphoid neoplasms was 67.4% (95% confidence interval [CI], 56-81). Five-year cumulative risks of lymphoid neoplasm- and leukemia-related mortality were 20% (95% CI, 10.2-30) and 12.4% (95% CI, 2.7-22), respectively. Concurrent hemophagocytic lymphohistiocytosis was associated with increased mortality (hazard ratio, 7.32; 95% CI, 1.62-32.98; P = .01). A large proportion of post-ALL lymphoid neoplasms are associated with an immunodeficient state, likely precipitated by ALL maintenance therapy. Awareness of this underrecognized entity and pertinent diagnostic tests are crucial for early diagnosis and optimal therapy.

Ceruloplasmin and Alpha-1-Acid Glycoprotein, but not C-Reactive Protein, Correlate With Serum Ferritin During Various Postpartum/Lactation Periods in Congolese Females.

Background: Serum ferritin usually correlates positively with acute phase proteins (APPs), but limited information is available on this association during various postpartum/lactation periods. The objective of this study was to assess the association between serum ferritin and APPs in Congolese females during different postpartum/lactation periods. Methods: Serum ferritin, C-reactive protein (CRP), alpha-1-acid glycoprotein (AGP), ceruloplasmin (Cp), and transferrin saturation (TS) were measured during various postpartum/lactation periods (0.5 to 6, 6.1 to 12, 12.1 to 18, and 18.1 to 24 months) in 131 Congolese females aged 15 to 45 years. Results: Mean serum ferritin concentrations were lower in females in the 0.5- to 6-month postpartum/lactation subgroup than in the other 3 subgroups (P<0.05). Mean concentrations of hemoglobin, APPs, and TS were not different among the 4 subgroups. While serum ferritin concentrations correlated with Cp (r=0.514) and AGP (r=0.795) during the 0.5- to 6-month and the 6.1- to 12-month postpartum/lactation periods, respectively (P<0.05), they did not correlate with CRP. Multiple regression analysis suggested that Cp explained 25% of serum ferritin variance in the 0.5- to 6-month postpartum/lactation period (39.3% at 0.5 to 4 months) and AGP explained 60.5% of the variance in the 6.1- to 12-month period (3.7% at 0.5 to 4 months). CRP explained <5% of the serum ferritin variance at these postpartum/lactation periods. APPs explained ≤15.1% of serum ferritin variance at postpartum/lactation periods >12 months. Conclusion: Data suggest that the association between serum ferritin and inflammation is dependent on APP type and lactation time. This association may affect the diagnosis of iron deficiency in lactating females. The positive association between serum ferritin and Cp at 0.5 to 6 months postpartum may be necessary to increase liver iron release and erythropoiesis after childbirth.

In Vitro Lymphocyte Functions in Undernourished Children With Sickle Cell Anemia.

Background: Children with sickle cell disease (SCD) often suffer from growth deficits and impaired immunity. However, the association between mild to moderate malnutrition and in vitro lymphocyte function has not been well studied. The goal of this study was to investigate the effects of undernutrition on lymphocyte functions in children with SCD. Methods: Weight; height; plasma concentrations of albumin (Alb), prealbumin (PA), transferrin (Tf), retinol-binding protein (RBP), α1-acid glycoprotein (AGP), C-reactive protein (CRP), and ceruloplasmin (Cp); and lymphocyte proliferation and interleukin (IL)-2 in phytohemagglutinin-treated blood lymphocytes were measured in 90 children with SCD (59 SS, 4 Sß°, 27 SC hemoglobin genotypes). Results: The mean age of the children included in the analysis was 7.65 years. Four of the 90 children had weight and height below the fifth percentile. A higher percentage of children with HbSS/HbSß° (61.4%) than of those with HbSC (44%) had ≥2 plasma protein concentrations below normal (Alb <35 g/L, PA <160 mg/L, Tf <2.0 g/L, and RBP ≤20 mg/L). Mean anthropometric measurements, hemoglobin, and hematocrit were lower in children with HbSS/HbSß° than in those with HbSC (P<0.05). Lymphocyte proliferation was reduced by 20% to 27% in children with HbSS/HbSß° with undernutrition plus inflammation (AGP >1 g/L, CRP >5 mg/L, Cp >600 mg/L) compared to children with neither. Regardless of inflammatory status, lymphocyte proliferation was reduced by 29% to 49% in children with HbSS/HbSß° and undernutrition defined by PA or Alb plus RBP (P<0.05) compared to those with RBP within normal range. Neither undernutrition nor inflammation reduced lymphocyte proliferation in children with HbSC. Mean IL-2 activity was reduced by undernutrition, defined as PA <160 mg/L, in both groups. PA, RBP, and hemoglobin concentrations positively correlated with in vitro lymphocyte functions (P<0.05). Conclusion: Undernutrition altered in vitro lymphocyte function in children with the HbSS/HbSß° genotypes. Dietary supplements may improve the altered functions in these children.

Fever and Leg Pain: Consider ALL the Diagnoses.

Background: Acute lymphoblastic leukemia (ALL) is the most common childhood cancer diagnosed in the United States. The disease causes a decrease in hematopoiesis, so children often present with symptoms related to anemia, thrombocytopenia, and leukopenia. Symptoms for this malignancy may have significant overlap with other conditions such as osteomyelitis. Case Report: A 2-year-old male with no significant medical history presented with lower extremity pain and fever. Initial investigations, including imaging and complete blood count, led physicians to diagnose bilateral osteomyelitis. The patient was prescribed a course of antibiotics; however, his symptoms returned. Eventually, a bone marrow aspiration showed CD99 membrane-positive small round blue cell tumors. The patient was diagnosed with ALL. He was successfully treated with chemotherapy and is now in remission. Conclusion: This case demonstrates the importance of a broad differential diagnosis for a child presenting with leg pain and fever.