Splenic hamartoma in a child in the era of PET‐CT

We present a case of a healthy 7‐year‐old female with an incidental finding of a growing splenic lesion, diagnosed as a splenic hamartoma after splenectomy. This case highlights the diagnostic challenge of splenic lesions and that the role of positron emission tomography/computerized tomography (PET/CT) in defining splenic lesions in the pediatric population remains to be defined. Pediatr Blood Cancer 2009;53:114–116. © 2009 Wiley‐Liss, Inc.     

Localised peripheral primitive neuroectodermal tumour (PNET) of the conjunctiva

A 16‐year‐old male presented with a 3‐month history of an asymptomatic, enlarging conjunctival lesion. An excisional biopsy was performed and histologic and immunohistochemical examination showed characteristic features of a peripheral primitive neuroectodermal tumour (PNET) adjacent to a benign compound naevus. FISH analysis, demonstrating a split‐signal at 22q12, confirmed the diagnosis. Staging investigations were negative confirming the primary nature of the lesion. The patient was treated with local wide re‐excision and chemotherapy. He remains alive and well 29 months after initial resection. Pediatr Blood Cancer 2009;53:669–671. © 2009 Wiley‐Liss, Inc.     

Alexander disease: An important mimicker of focal brainstem glioma

We report the case of a 6‐year‐old male who was referred to a tertiary oncology center with a focal brainstem lesion which was presumed to be neoplastic. Due to the symmetric nature of the lesion on magnetic resonance imaging, the evaluation was expanded to investigate other possible causes and eventual diagnosis of Alexander's disease (AD) was made. AD is a neurodegenerative disease which must be included in the differential for tumor‐like lesions within the posterior fossa. Pediatr Blood Cancer 2009; 53:1355–1356. © 2009 Wiley‐Liss, Inc.     

Conservative treatment of metanephric adenoma. A case report and review of the literature

Metanephric adenoma (MA) is a rare kidney tumor, especially in children, with an excellent prognosis and frequent association with polycythemia. We report the case of a 4-year-old girl presenting a MA revealed by polycythemia. Any secondary polycythemia requires checking for a possible kidney tumor even when the erythropoietin level is not elevated. MA is an exclusively epithelial tumor that can be similar to nephroblastoma or papillary renal cell carcinoma; therefore, it requires strict histopathological analysis. There are few radiological characteristics of MA: well circumscribed tumor, slightly enhanced after the injection of an intravenous contrast medium on computed tomography scan or magnetic resonance imaging. Diagnosis can be supported by core-needle kidney biopsy after hemostasis check, following two published cases of associated acquired von Willebrand disease. Chemotherapy can then be avoided and first-line conservative surgical treatment by partial nephrectomy can be considered, as in our patient's case. However, the marginal resection raised the question of frozen sections analysis of the margins. We recommend thorough follow-up visits, combining clinical examination, ultrasonography and, in the case of polycythemia, biological assays.     

Langerhans cell histiocytosis in a patient with stage 4 neuroblastoma receiving oral fenretinide

Langerhans cell histiocytosis (LCH) has previously been reported in association with other malignancies. The pathogenesis of LCH and its relationship to other malignancies is poorly understood. We present a novel case of a child who developed an LCH bone lesion while receiving a Phase I protocol therapy with oral fenretinide/Lym‐X‐Sorb (4‐HPR/LXS) powder for neuroblastoma. Pediatr Blood Cancer 2009;53:1111–1113. © 2009 Wiley‐Liss, Inc.     

Neonatal Tuberous Sclerosis with Heart and Brain Tumors

A 6-day-old neonate with tuberous sclerosis having brain and cardiac tumors is reported. The patient presented with a seizure 20 minutes after birth. Cranial ultrasonography and computed tomography on admission showed a large and round high-density tumor lesion in the left frontal lobe. Echocardiography also showed a large tumor lesion beside the left ventricle wall, and a diagnosis of tuberous sclerosis was promptly made. Two months later, a periventricular calcified lesion was found on the demonstrated portion, and the tumor lesion initially observed in the brain had disappeared. These findings suggest that the initial tumor lesion found in the brain developed into a typical calcified lesion.     

儿童后肾腺瘤五例报告

目的 探讨儿童后肾腺瘤临床病例特点,提高对儿童后肾腺瘤的认识.方法 回顾性分析2008年5月至2016年1月收治的5例儿童后肾腺瘤的临床资料,并进行文献复习.5例患儿中,男4例,女1例;年龄小于2岁2例,8～10岁3例;肿瘤位于左侧2例,右侧3例.以腹部包块就诊1例,超声偶然发现4例.术前均行超声和增强CT,2例考虑肾母细胞瘤,2例考虑肾细胞癌,1例考虑肾囊肿合并出血.结果 肿瘤直径小于5 cm者4例.行保留肾单位的肿瘤剜除术3例,行瘤肾切除术2例.病理检查报告:后肾腺瘤4例,后肾腺瘤合并乳头状肾细胞癌1例.术后平均随访4年5个月,均无复发和转移.结论 后肾腺瘤罕见,临床表现及影像缺乏特异性,术前诊断困难,确诊依靠病理及免疫组织化学.后肾腺瘤为良性病变,手术完整切除为治疗原则.有合并恶性成分的病例报道,术后需长期随诊复查.     

Pulmonary anthracosis mimicking lung metastases in pediatric rhabdomyosarcoma

Although childhood rhabdomyosarcoma typically metastasizes to lungs, various processes may mimic metastatic etiology. Described herein is the case of an 8½‐year‐old boy with orbital embryonal rhabdomyosarcoma (RME) in whom three small foci were detected within both lungs on computed tomography. The lesion number and size, however, did not fulfil the Cooperative Weichteilsarkom Study Group 2006 protocol criteria for lung metastasis. Chemotherapy for localized RME produced primary tumor regression and vanishing of the left lung lesion. Two lesions in the right lung remained unchanged. On thoracoscopy multiple minute nodules disseminated in both lungs were detected. Histopathology excluded RME spread but indicated anthracosis in the lung parenchyma and intrapulmonary lymph nodes. Heavy smoking by parents and previous home furnace combustion appeared to be predisposing factors. Uncommon non‐malignant intrapulmonary diseases, including anthracosis, should be considered when staging pediatric cancer.     

Refractory dilated cardiomyopathy associated with metastatic neuroblastoma

A 2‐year‐old African American male presented with heart failure and an abdominal mass. Computerized tomography (CT) scan revealed a 7 cm adrenal lesion, confirmed as poorly differentiated neuroblastoma (NB). CT and meta‐iodobenzoguanidine (MIBG) scans identified multiple metastases, but cardiac MIBG imaging was absent. Cardiac ejection fraction (EF) was 8% with 7% shortening fraction. The patient underwent six cycles of chemotherapy and investigational immunotherapy. Cardiac function improved to 26% EF. However, the tumor proved unresponsive to treatment. The patient died from stage IV congestive heart failure (CHF) and progressive NB. Autopsy confirmed dilated cardiomyopathy with endocardial fibroelastosis. Pediatr Blood Cancer. 2010;55:736–738. © 2010 Wiley‐Liss, Inc.     

Lenticular lesions: not always an epidural hematoma

Ewing's sarcoma is a tumor of the bone, which occurs most often in the diaphysis of long and flat bone. The most common sites of metastasis are the lungs and bones. Less frequently, the primary site is an intracranial or pelvic lesion (either as a soft tissue or a bone lesion). We report a case of a 16-year-old female with an extraosseous intracranial lesion, who presented with a history of minor trauma, unilateral facial swelling, and head pain. Though head computed tomography scan showed a lesion consistent with an epidural hematoma, further exploration revealed Ewing's sarcoma.     

Metanephric adenoma of the kidney: a case report

Metanephric adenoma (MA) of the kidney is an uncommon benign epithelial tumor of the kidney that presents at any age. The histology of the lesion is well established, but the imaging findings have been described in only a few cases. We report the sonographic and computed tomographic appearance of MA in a 9-year-old girl to increase awareness among radiologists of this entity. Its recognition may facilitate nephron-sparing surgery. Received: 1 April 1998 Accepted: 14 July 1998     

Medulloblastoma with epithelioid features in the cerebellar vermis

A 6‐year‐old girl was admitted with a mass lesion in the cerebellar vermis. She underwent subtotal tumor resection, and on immunohistopathology the tumor consisted of two different parts: typical medulloblastoma (MB) characteristics and atypical teratoid/rhabdoid tumor (AT/RT) features, despite positive integrase interactor 1 expression. The patient was diagnosed with MB with epithelioid features. Chemoradiation therapy was started because of tumor recurrence at the primary site and dissemination to the spinal cord, as determined on magnetic resonance imaging 2 weeks after surgery. The patient died due to tumor progression 13 months after initial diagnosis, although transient partial remission was achieved.     

Characterization of a WiT49 cell line derived orthotopic model of Wilms tumor

Wilms tumor is the most common malignant renal tumor in children. However, to date no Wilms tumor mouse model is available due to the lack of Wilms tumor cell lines. Herein for the first time we report an orthotopic xenograft mouse model utilizing the recently described Wilms tumor cell line WiT49. It has a high tumor occurrence rate (85%) without metastasis. Hematoxylin and eosin staining showed it is subcapsular in location and mainly biphasic with stromal and epithelial components while blastemal component is unappreciable. This model provides the prerequisite for the screening and development of new anti‐tumor agents for Wilms tumor. Pediatr Blood Cancer 2010;54:316–318. © 2009 Wiley‐Liss, Inc.     

Initial testing of aplidin by the pediatric pre‐clinical testing program

Aplidin was tested in vitro at concentrations ranging from from 0.1 nM to 1.0 µM and in vivo at a dose of 0.6 mg/kg administered intraperitoneally on an every 4 days × 3‐schedule that was repeated at day 21. In vitro, Aplidin was most active against acute lymphoblastic leukemia (ALL) cell lines. In vivo, Aplidin induced significant differences in EFS distribution in 12 of 28 (43%) solid tumor models and 2 of 6 evaluable ALL models. Aplidin showed potent in vitro activity and induced significant in vivo tumor growth inhibition in some xenografts, but did not induce tumor regressions. Pediatr Blood Cancer 2009;53:509–512. © 2009 Wiley‐Liss, Inc.     

Melanotic neuroectodermal tumor of infancy discovered after head trauma

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare neoplasm that generally arises in the maxilla during the first year of life. Involvement of bones of the cranial vault or brain is extremely rare. We describe a 7-month-old black female who presented after falling out of bed onto a concrete floor. Subsequently, she developed an anterior frontal mass that enlarged over several days. Radiographs of the skull at her local hospital showed a depressed right frontal skull fracture. However, computerized tomography of the head (reviewed at our institution) revealed a slightly hyperdense extra-axial mass which crossed the anterior frontal midline, widening the metopic suture and extending into the anterior subgaleal scalp. Hyperostosis of the adjacent frontal calvarium was also present. A craniotomy revealed a dark, 1.5-cm calcified epidural lesion with some features of an unusual hematoma. Microscopic evaluation revealed a chronic hematoma and MNTI. The tumor recurred within a year. MNTI should be included in the differential diagnosis of epidural and skull lesions in infants.     

Successfully treated acute lymphoblastic leukemia associated with craniopharyngioma

The authors report a 2-year-old boy with acute lymphoblastic leukemia (ALL) associated with craniopharyngioma. To our knowledge, this is the first such report. Magnetic resonance imaging showed a suprasellar tumor, an apparent cystic lesion, whereas cerebral computed tomography confirmed that the tumor exhibited calcification. Without surgical intervention for the suprasellar tumor, we initiated chemotherapy for ALL. After 1 year of chemotherapy, complete remission was achieved, and partial resection and radiation therapy were performed on the suprasellar tumor. At 4 years after completing leukemia chemotherapy, the patient remains in complete ALL remission and has had no recurrence of craniopharyngioma.     

Massive benign osteoblastoma of the suboccipital bone and foramen magnum region

Benign osteoblastoma is an uncommon primary bone tumor frequently found in the vertebral column and long tubular bones, and rarely occurring in the calvarium. A case of a massive benign osteoblastoma of the suboccipital bone and foramen magnum region in a 9-year-old boy is reported. He presented with progressively worsening nuchal pain and headaches secondary to a bony lesion in the suboccipital and foramen magnum region. Computed tomography (CT) of the brain showed a large midline occipital/suboccipital bony lesion extending to either side (R > L) and extending from the torcula till the foramen magnum region, causing moderate obstructive hydrocephalus. The atlas was uninvolved by the tumor. In addition, the cerebellum was pushed anteriorly squashing the fourth ventricle. The tumor was completely resected with wide margins via a suboccipital route. At follow-up after 7 years, the patient was asymptomatic, and CT imaging demonstrated no recurrence. The occurrence of benign osteoblastoma in the suboccipital bone and foramen magnum region has not been reported earlier in the pediatric population. Surgical extirpation of the lesion with wide margins is advocated and can produce an excellent long-term outcome. Serial vigilant follow-up along with sequential imaging is advocated even in cases with complete resection to detect early recurrence and possible malignant transformation.     

Phosphaturic Mesenchymal Tumor-Induced Rickets

We describe two prepubertal girls with oncogenic rickets. The first patient, 9 years of age, presented with recent-onset lower-extremity pain. The second girl, presented at 4 years of age following a 9-month period of muscle weakness, bone pain, and poor linear growth. Laboratory analyses in both patients revealed hypophosphatemia and hyperphosphaturia; elevated circulating alkaline phosphatase activity was present in one of them. Radiographic evidence of a generalized rachitic process was evident in both cases. Computerized tomography of the paranasal sinuses and facial bones in patient 1 revealed a small lesion eroding through the inner table of the left mandibular ramus. Microscopic examination of this mass revealed a spindle cell neoplasm with chondroid material, dystrophic calcification, and both osteoclast-like and fibroblast-like cells. Prominent vascularity and marked atypia were present. These features are consistent with a phosphaturic mesenchymal tumor of the mixed connective tissue variant. In the second patient, computerized tomography revealed a lytic lesion located in the right proximal tibia, with histologic features consistent with a phosphaturic mesenchymal tumor of the nonossifying fibroma-like variant. Resection of each tumor resulted in rapid correction of the phosphaturia and healing of the rachitic abnormalities. A careful search for small or occult tumors should be carried out in cases of acquired phosphaturic rickets. Received November 25, 1998; accepted February 25, 1999.     

Solitary,extracutaneous, skull‐based juvenile xanthogranuloma

We report a case of an 18‐month‐old female who presented an occipital bone lesion with progressive growth. Imaging studies showed a left extradural, skull‐based tumor partially occupying the posterior fossa. Histopathological and immunohistochemical studies confirmed a juvenile xanthogranuloma (JXG). Partial surgical resection, chemotherapy, and conformational radiotherapy were used. Exclusive extracutaneous JXG with an intracranial, vertebral, or skull‐based localization is extremely rare. Pediatr Blood Cancer. 2010;55:380–382. © 2010 Wiley–Liss, Inc.     

Ex vivo‐expanded donor CD4+ lymphocyte infusion against relapsing neuroblastoma: A transient graft‐versus‐tumor effect

High‐risk neuroblastoma has a poor prognosis despite multimodal treatment including high‐dose chemotherapy. A 7‐year‐old male with neuroblastoma received ex vivo‐expanded donor CD4⁺ T lymphocyte infusion (CD4⁺ DLI) after recurrence in the bone marrow following allogeneic hematopoietic stem cell transplantation from his HLA‐identical mother. The disease transiently responded to CD4⁺ DLI with reduction of tumor cells and a decrease of serum neuron‐specific enolase. The response was associated with development of continued high fever and an increase of cytotoxic T lymphocytes in peripheral blood. This case suggests a possibility of a graft‐versus‐tumor effect against neuroblastoma. Pediatr Blood Cancer 2009;52:895–897. © 2009 Wiley‐Liss, Inc.