Marie E. Steiner

Pleuropulmonary blastoma the so-called pulmonary blastoma of childhood

The authors studied 11 pediatric intrathoracic neoplasms that share clinicopathologic features and constitute a specific tumor in children. These neoplasms were intrapulmonary, mediastinal, or pleural‐based masses. A common histologic feature was the presence of small, primitive cells with blastematous qualities separated by an uncommitted stroma. Focal rhabdomyosarcomatous, chondrosarcomatous, and liposarcomatous differentiation was observed. Epithelial components had bland cytologic features and probably represented entrapped benign epithelium and/or mesothelium. The prognosis for these patients was grave; seven patients died of their disease 5 months to 2 years after diagnosis. Two patients have survived diseasefree for 10 and 12 years after diagnosis. Two recent cases are alive 14 and 32 months after diagnosis. This neoplasm constitutes a distinct entity which has been reported in the literature as pulmonary blastoma in children. It differs from pulmonary blastema in adults because of its variable anatomic location, primitive embryonic‐like blastema and stroma, absence of a carcinomatous component, and potential for sarcomatous differentiation. The designation of pleuropulmonary blastoma is suggested by the authors for these intrathoracic neoplasms of childhood rather than pulmonary blastoma for histogenetic and anatomic reasons. The clinicopathologic features, immunophenotypic and ultrastructural characteristics, possible histogenesis, and differential diagnosis of these neoplasms from other thoracopulmonary tumors in children serve as the basis for this report.

Effects of Red-Cell Storage Duration on Patients Undergoing Cardiac Surgery

BACKGROUND Some observational studies have reported that transfusion of red-cell units that have been stored for more than 2 to 3 weeks is associated with serious, even fatal, adverse events. Patients undergoing cardiac surgery may be especially vulnerable to the adverse effects of transfusion. METHODS We conducted a randomized trial at multiple sites from 2010 to 2014. Participants 12 years of age or older who were undergoing complex cardiac surgery and were likely to undergo transfusion of red cells were randomly assigned to receive leukocyte-reduced red cells stored for 10 days or less (shorter-term storage group) or for 21 days or more (longer-term storage group) for all intraoperative and postoperative transfusions. The primary outcome was the change in Multiple Organ Dysfunction Score (MODS; range, 0 to 24, with higher scores indicating more severe organ dysfunction) from the preoperative score to the highest composite score through day 7 or the time of death or discharge. RESULTS The median storage time of red-cell units provided to the 1098 participants who received red-cell transfusion was 7 days in the shorter-term storage group and 28 days in the longer-term storage group. The mean change in MODS was an increase of 8.5 and 8.7 points, respectively (95% confidence interval for the difference, -0.6 to 0.3; P=0.44). The 7-day mortality was 2.8% in the shorter-term storage group and 2.0% in the longer-term storage group (P=0.43); 28-day mortality was 4.4% and 5.3%, respectively (P=0.57). Adverse events did not differ significantly between groups except that hyperbilirubinemia was more common in the longer-term storage group. CONCLUSIONS The duration of red-cell storage was not associated with significant differences in the change in MODS. We did not find that the transfusion of red cells stored for 10 days or less was superior to the transfusion of red cells stored for 21 days or more among patients 12 years of age or older who were undergoing complex cardiac surgery. (Funded by the National Heart, Lung, and Blood Institute; RECESS ClinicalTrials.gov number, NCT00991341.).

Pediatric ovarian tumors: A review of 67 cases

Ovarian tumors are uncommon but important childhood neoplasms.

Bleeding risks are higher in children versus adults given prophylactic platelet transfusions for treatment-induced hypoproliferative thrombocytopenia

Age-group analyses were conducted of patients in the prophylactic platelet dose trial (PLADO), which evaluated the relation between platelet dose per transfusion and bleeding. Hospitalized patients with treatment-induced hypoproliferative thrombocytopenia were randomly assigned to 1 of 3 platelet doses: 1.1 × 10(11), 2.2 × 10(11), or 4.4 × 10(11) platelets/m(2) per transfusion, given for morning counts of ≤ 10 000 platelets/μL. Daily hemostatic assessments were performed. The primary end point (percentage of patients who developed grade 2 or higher World Health Organization bleeding) was evaluated in 198 children (0-18 years) and 1044 adults. Although platelet dose did not predict bleeding for any age group, children overall had a significantly higher risk of grade 2 or higher bleeding than adults (86%, 88%, 77% vs 67% of patients aged 0-5 years, 6-12 years, 13-18 years, vs adults, respectively) and more days with grade 2 or higher bleeding (median, 3 days in each pediatric group vs 1 day in adults; P < .001). The effect of age on bleeding differed by disease treatment category and was most pronounced among autologous transplant recipients. Pediatric subjects were at higher risk of bleeding over a wide range of platelet counts, indicating that their excess bleeding risk may be because of factors other than platelet counts.

Does red blood cell storage affect clinical outcome? When in doubt, do the experiment

Q uestions about the efficacy, safety, and availability of blood products are major concerns facing the FDA and the National Heart, Lung, and Blood Institute (NHLBI) as well as health care providers and administrators at hospitals. One of the basic questions that has been posed episodically but repeatedly for decades has been whether or not the changes that occur in red blood cells (RBCs) during storage affect clinical outcomes. Although the urgency of the question has waxed and waned over the years, it remains unanswered. This issue of TRANSFUSION offers two articles that address this issue. Lelubre and coauthors review our current level of understanding of the impact of RBC storage on patient outcomes and point out some of the difficulties of interpreting the data from these clinical studies. The clinical design feasibility study of Bennett-Guerrero and coauthors highlights some of the challenges of conducting definitive clinical trials in this area. Together, they help us focus on the direction we need to move, to address the questions that persist about the clinical impact of RBC storage._02265 1286..1290

Pulmonary cytolytic thrombi: a newly recognized complication of stem cell transplantation.

Over the past 5 years we have recognized a new pulmonary complication of hematopoietic stem cell transplantation (HSCT) associated with fever and pulmonary nodules termed ‘pulmonary cytolytic thrombi’ (PCT). Retrospective analysis of medical and radiographic records and pathologic material from 13 HSCT recipients with PCT and a review of the Blood and Marrow Transplant Database for all patients with radiographic evidence of pulmonary nodules or who underwent open-lung biopsy from 1 January 1993 to 31 December 1998 (n = 1228) were performed. The median age of patients with PCT was 11.9 years (range, 1.3–29.7 years). All patients developed fever at a median of 72 days (range, 8–343 days) post transplant, followed by pulmonary nodules on chest CT. Eleven patients were receiving therapy for active GVHD (acute, grades I–IV (n = 10); extensive chronic (n = 1)). Biopsy of the pulmonary nodules revealed a unique pattern of necrotic, basophilic thromboemboli with amorphous material suggestive of cellular breakdown products. This was descriptively labeled ‘pulmonary cytolytic thrombi’. Immunohistochemical staining revealed entrapped leukocytes and disrupted endothelium, but was negative for histiocytes. Cultures and immunohistochemical stains were negative for infectious agents. Empiric therapy included systemic corticosteroids (n = 9) and amphotericin (n = 7). Nine patients survive with resolution of PCT at a median follow-up of 1.5 years. Bone Marrow Transplantation (2000) 25, 293–300.

Pulmonary Nodular Lesions in Bone Marrow Transplant Recipients Impact of Histologic Diagnosis on Patient Management and Prognosis

Bone marrow transplantation is associated with numerous pulmonary complications, which may manifest as nodules. We studied 33 bone marrow transplant (BMT) recipients in whom pulmonary nodular lesions (PNLs) developed during a 5-year period and who underwent open lung biopsy (OLB) for diagnosis. Of 33 patients with PNL, 15 (45%) had pulmonary cytolytic thrombi (PCT), a recently described condition characterized histologically by occlusive vascular lesions and hemorrhagic infarcts and clinically by a favorable outcome. Clinical symptoms and radiologic abnormalities disappeared during a period of a few weeks. None of the patients died of PCT; 10 were alive at last contact. The second most common cause of PNL (8/33 [24%]) was Aspergillus infection, which was the cause of death in 6. OLB is an effective way of obtaining diagnostic tissue in BMT recipients with PNLs. Histologic examination is accurate in determining the cause of PNLs and identifying lesions that have a favorable outcome and those that require a change in treatment.

Undifferentiated (embryonal) sarcoma of the liver. A clinicopathologic study of a survivor treated with combined technique therapy

Undifferentiated (embryonal) sarcoma is one of the less common primary malignant tumors of the liver that typically, although not exclusively, presents in later childhood. It has come to be recognized as an aggressive neoplasm with the potential for local recurrence and distant metastasis, despite a seemingly successful total surgical resection. This report documents our experience with an 8‐year‐old boy who had an 80% hepatectomy for an undifferentiated (embryonal) sarcoma, followed by cisplatin (90 mg/m2), doxorubicin (20 mg/m2/d), and radiation therapy (1950 cGy) to the tumor bed. Residual microscopic tumor was very likely present in the region of the inferior vena cava, but exploratory laparotomies for the purpose of “second‐look” biopsies were performed with negative results after the completion of each course of chemotherapy. The patient is off of treatment and disease‐free 30 months after the diagnosis. Because the survival at this time has been so poor for this highly malignant tumor, we suggest that the combination of therapeutic techniques and second‐look laparotomy(s) to identify any subclinical tumor may be a reasonable approach. Ultrastructural and immunohistochemical studies by us and other investigators indicate that the neoplastic cells are primitive mesenchymal cells by electron microscopic study and have immunoreactivity limited to vimentin, alpha‐1‐antitrypsin, and alpha‐1‐antichymotrypsin.

Adnexal masses in infancy and childhood

The presentation of adnexal masses in childhood differs from that in adult women. Children may present with poorly localized symptoms or precocious puberty. Ovarian cysts occur throughout development; ovarian tumors are less frequent but occur in all age groups. Congenital malformations may present with signs or symptoms of an adnexal mass. Occasionally adnexal findings may suggest the presence of an underlying syndrome. Assessment of the patients developmental, hormonal, and pubertal status is necessary to ensure an accurate diagnosis. Treatment options must consider risks to ovarian function and future fertility.

Hepatic mesenchymal hamartoma in a neonate : A case report and review of the literature

To describe an unusual presentation of mesenchymal hamartoma in a critically ill neonate necessitating a novel therapeutic embolization before definitive resection. An unusual presentation of a large hepatic mass in a newborn complicated by pulmonary hypertension and vascular “steal” with renal insufficiency is presented. The mass was initially successfully embolized, but then revascularized, necessitating resection in an attempt to improve the clinical status of the critically ill neonate. The resected mass was a mesenchymal hamartoma with a necrotic center and extensive arterial collateralization. The patient began improving immediately after resection. Mesenchymal hamartoma may present in the neonate as a diagnostic dilemma. This is the first case report describing persistent pulmonary hypertension and renal compromise from this tumor. Embolization as a therapeutic modality to address this tumor is described. The cause of the persistent and severe pulmonary hypertension remains unclear, but may be related to the tumor.