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Mediastinal masses
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Brent R. Weil, Robert C. Shamberger
Solid lesions require further histopathologic diagnosis. The most common solid tumor in the anterior mediastinum is Hodgkin lymphoma followed by non-Hodgkin lymphoma. Other areas of lymph node involvement besides the mediastinum, particularly the neck, should be sought where biopsy could be more easily performed. In those rare instances where only the mediastinum is involved, a germ cell tumor should be suspected and serum alpha-fetoprotein (AFP) and human chorionic gonadotrophin (hCG) levels should be obtained. In cases where there is no extrathoracic tumor, either a needle biopsy with radiographic guidance or a limited anterior thoracotomy or thoracoscopy may be required to establish a tissue diagnosis. The rare chloroma of leukemia presenting as a mediastinal mass can be diagnosed with the initial complete blood count and bone marrow biopsy. Serum metanephrine levels should be obtained for suspected paragangliomas. For suspected neuroblastomas, urine catecholamine measurement, MIBG scan, and/or bone marrow biopsy should be considered.
Cranial Neuropathies I, V, and VII–XII
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Cerebellopontine angle: Vestibular schwannoma compressing CN V (although CN VII is most commonly affected).Trigeminal schwannoma (second most common cause of schwannomas affecting CNs).Meningioma (multiple CNs are usually affected).Glossopharyngeal schwannoma (rare).Epidermoid/dermoid tumor.Chordoma.Chloroma.Metastases.Aberrant vessels, basilar artery ectasia, or aneurysm.
Haematological malignancy
Published in Peter Hoskin, Peter Ostler, Clinical Oncology, 2020
Chloroma or isolated granulocytic sarcoma is a localized tumour of myeloblasts. Around two-thirds will progress to widespread AML; localized treatment with surgery or radiotherapy is not appropriate and these tumours should be treated as for AML.
A phase I study of panobinostat in children with relapsed and refractory hematologic malignancies
Published in Pediatric Hematology and Oncology, 2020
John Goldberg, Maria Luisa Sulis, Julia Bender, Sima Jeha, Rebecca Gardner, Jessica Pollard, Victor Aquino, Theodore Laetsch, Naomi Winick, Cecilia Fu, Leigh Marcus, Weili Sun, Anupam Verma, Michael Burke, Phoenix Ho, Thomas Manley, Rajen Mody, Wendy Tcheng, Blythe Thomson, Julie Park, Richard Sposto, Yoav Messinger, Nobuko Hijiya, Paul Gaynon, Julio Barredo
Eligible subjects were ≥ 1 and ≤ 21 years of age at the time of enrollment. For the leukemia cohort, they had either relapsed or refractory AML or ALL with ≥ 5% blast in the bone marrow or biopsy confirmed relapsed extramedullary disease (e.g. chloroma). Subjects may have had prior CNS disease; however, isolated CNS relapses were not eligible. Subjects with secondary AML were eligible. For the lymphoma cohort, subjects had relapsed or refractory NHL or HL with no CNS disease. A performance status of a Karnofsky score > 50% for subjects > 16 years of age or a Lansky score of > 50% for subjects < 16 years of age was required, as was a life expectancy of at least 8 weeks. Subjects were required to have had at least 1 prior therapeutic regimen for their initial diagnosis of relapsed or refractory disease and at least 7 days must have elapsed since the completion of cytotoxic therapy with the exception of hydroxyurea, which was permitted prior to beginning protocol therapy and could continue during the first cycle of therapy per investigator discretion. Renal, hepatic and cardiac functions had to be within acceptable ranges, and subjects were required to employ contraception if of age while participating in the study.
Clinical features and prognosis of normal karyotype acute myeloid leukemia pediatric patients with WT1 mutations: an analysis based on TCGA database
Published in Hematology, 2020
Jing Xu, Yaofang Zhang, Jinjun Hu, Yan Ren, Hongwei Wang
The clinical characteristics were compared between the WT1- mutated group and the WT1 wild-type group (Table 1). There are 28(12.7%) cases mutated in WT1 gene. There was no significant difference in age (P = 0.075), white blood cell (WBC) count at diagnosis (P = 0.169), percentage of bone marrow leukemic blast cells (P = 0.411) and percentage of peripheral blood blast cells (P = 0.565). What’s more, the WT1-mutated and WT1 wild-type AML patients were equally distributed over protocols (P = 0.240), gender (P = 0.061), race (P = 0.313), central nervous system (CNS) disease (P = 0.219) and chloroma (P = 0.743). However, the distribution of FAB type was different (P = 0.044) and WT1 mutations were more common in patients with French–American–British (FAB) class M4 (28.6% vs. 15.1%). And such mutations were also less frequent in a low-risk group (7.1% vs. 41.1%, P = 0.002). The positive rate of FLT3-ITD in the WT1-mutated group was higher than that in the WT1 wild-type group (67.9% vs. 36.5%, P = 0.002). In contrast, WT1 mutation and NPM1 mutation were negatively correlated (P = 0.013). There was no significant difference in the rate of FLT3 point mutation (7.1% vs. 8.9%, P = 1.000) between the two groups. In addition, patients with WT1 mutations had a lower mutation rate of CEBPA, but this difference was not statistically significant (3.6% vs. 19.4%, P = 0.073).
Distinguishing Benign from Malignant Circumscribed Orbital Tumors in Children
Published in Seminars in Ophthalmology, 2018
Yufei Tu, Frederick A. Jakobiec, Katherine Leung, Suzanne K. Freitag
Orbital infiltration by leukemia could be also a presenting sign, manifesting as a solid, unifocal orbital mass (also known as myeloid sarcoma or chloroma because of esterase and myeloperoxidase).15 It is most commonly associated with acute myelogenous leukemia.16 The lesions tend to mold to contiguous orbital structures, and have predilection for the lateral orbit.17 The leukemic deposits on MRI demonstrate hypointensity on T1-weighted images and isointensity or intermediate signal intensity on T2-weighted images, whereas tumors such as rhabdomyosarcoma, isolated schwannoma, and neurofibroma tend to be hyperintense on T2-weighted images.18 The tumor is composed of undifferentiated, large blastic blue cells, which can confer a greenish tinge to the mass at gross examination (thus, a name of chloroma). The diagnosis can be confirmed with cytochemical reactions or immunophenotypic cell studies for myeloperoxidase, esterase (Leder’s stain), muramidase, lysozyme, and CD56.19