Dataset Information

Myeloid neoplasm with eosinophilia and rearrangement of platelet-derived growth factor receptor beta gene in children: Two case reports.

ABSTRACT:

Background

Myeloid neoplasm (MN) with eosinophilia and rearrangement of platelet-derived growth factor receptor beta (PDGFRB) shows a good therapeutic response to imatinib in adults. MN is rarely found in children, and the efficacy of imatinib on pediatric patients remain unclear.

Case summary

We report 2 pediatric cases diagnosed with MN with eosinophilia and PDGFRB rearrangement who were treated with imatinib. Case 1 was a 1-year-old girl admitted to the hospital because of "abdominal distension with hyperleukocytosis for 3 mo". She had leukocytosis, anemia, and eosinophilia (the absolute eosinophil count (AEC) was 8960/?L), and her fluorescence in situ hybridization (FISH) test revealed that PDGFRB rearrangement was detected in 70% of 500 interphase cells. Case 2 was a 2-year-old girl admitted to the hospital because of "recurrent fever and rashes for 1 mo". Her blood cell count showed an AEC of 3540/?L. The FISH test revealed that PDGFRB rearrangement was detected in 71% of 500 interphase cells. Both patients were diagnosed as MN with eosinophilia and PDGFRB rearrangement. Imatinib was added into their treatment regimen. As expected, complete hematologic remission was achieved after 1 mo of treatment, and symptoms disappeared.

Conclusion

Although MN with eosinophilia and PDGFRB rearrangement usually occurs in adults, it can be found in children. The therapeutic benefits of imatinib in these 2 pediatric patients were consistent with its reported effects in adult patients.

SUBMITTER: Wang SC

PROVIDER: S-EPMC7809671 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

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Publications

Myeloid neoplasm with eosinophilia and rearrangement of platelet-derived growth factor receptor beta gene in children: Two case reports.

Wang Shi-Chong SC Yang Wen-Yu WY

World journal of clinical cases 20210101 1

<h4>Background</h4>Myeloid neoplasm (MN) with eosinophilia and rearrangement of platelet-derived growth factor receptor beta (PDGFRB) shows a good therapeutic response to imatinib in adults. MN is rarely found in children, and the efficacy of imatinib on pediatric patients remain unclear.<h4>Case summary</h4>We report 2 pediatric cases diagnosed with MN with eosinophilia and PDGFRB rearrangement who were treated with imatinib. Case 1 was a 1-year-old girl admitted to the hospital because of "abd ...[more]

PMID: 33511186

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Project description:Background Intraductal oncocytic papillary neoplasms (IOPNs) place at the oncocytic extreme of the intraductal pancreatic neoplasm spectrum and display typical morphological features. Their identification in 1996 by Adsay et al. has been followed by a growing number of cases, paving the way for a deeper understanding of this underestimated entity. Contrarily to intraductal papillary mucinous neoplasms (IPMNs), most IOPNs run an indolent course and surgery is usually curative. Pancreatic IOPNs tend to develop from the main pancreatic duct (MPD) and their diagnosis is either incidental or subsequent to mass-related symptoms. Up to 30% of cases show concomitant areas of minimal stromal invasion and loco-regional or systemic spread are confined to a minority of cases. Biological hallmarks of IOPNs are being identified, including recurrent kinase gene rearrangements. Morphological and biological traits of IOPNs seem to overlap with those of other malignancies. A deeper understanding of these entities is needed in order to shed light upon the nature of pancreato-biliary oncocytic neoplasms. This case report describes two patients with a diagnosis of IOPN—one of them accounting for the largest IOPN ever described—and provides a brief review of recent discoveries on the subject. Case Description We describe two cases of IOPN occurring in adult male patients, respectively in their 60s and 70s. Both patients had unremarkable clinical history. In case 1 the diagnosis was coincidental to a right renal colic; case 2 complained a right lumbar pain radiating to the homolateral groin. In both cases imaging analyses revealed a voluminous pancreatic mass, posing the indication to laparoscopic pancreatectomy. Gross and histological features were consistent with the diagnosis of IOPN. Surgical margin were free from disease and the patient did not undergo further treatment. After a 10- and 7-month follow-up respectively, patients did not experience relapse. Conclusions Recent immunohistochemical (IHC) and molecular data reveal unique characteristics of IOPNs, highlighting the substantial differences from IPMNs. Further research is needed in order to identify novel prognostic and predictive markers applicable to oncocytic neoplasms of the pancreato-biliary tract.

Dataset Information

Myeloid neoplasm with eosinophilia and rearrangement of platelet-derived growth factor receptor beta gene in children: Two case reports.

Background

Case summary

Conclusion

Publications

Myeloid neoplasm with eosinophilia and rearrangement of platelet-derived growth factor receptor beta gene in children: Two case reports.

Similar Datasets

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