Cutaneous T‐cell lymphoma in a 21‐year‐old male with Wolf–Hirschhorn syndrome

Abstract

We describe a case of cutaneous T-cell lymphoma occurring in a 21-year-old male with Wolf-Hirschhorn syndrome (WHS) due to a chromosome 4p16.3 deletion. This is the first documented case report of malignancy occurring in an adult with WHS. We also review the literature regarding patients with WHS and the joint occurrence of malignancy and discuss genetic changes involving chromosome 4 which may have contributed to the genesis of our patient's lymphoma.

Hepatoblastoma and Wolf–Hirschhorn syndrome: Coincidence or a new feature of a rare disease?

“…We could not identify a direct relationship between functional loss of genes in WHS and hepatoblastoma development, but genes in the 4p 16.3 region play various roles in the cell cycle and in cell growth. It is possible that, as noted in previous reports, loss of heterogeneity in WHSCR genes or incomplete genetic defect might be associated with the development of hepatoblastoma in the present case. Prematurity and very low‐birthweight might be additional predisposing factors in the development of hepatoblastoma …”

“…Patients usually have seizures until 3 years of age, and majority of them have mental or motor retardation . Cancer is not a frequent feature of the syndrome, but hematopoietic system malignancies and hepatic adenoma have been reported in a few cases . Herein, we report a pediatric patient with WHS who developed hepatoblastoma.…”

Two cases of hepatic adenomas in patients with wolf‐hirschhorn syndrome: A new rare complication?

“…Oncologic complications are a rare finding in patients with WHS; in 2003 two children with WHS and myelodysplastic syndrome (MDS) were reported [Sharathkumar et al, ]. Another report described a case of cutaneous T‐cell lymphoma, occurring in a 21‐year‐old male with WHS [Batton et al, ]. These are the only descriptions of an association between WHS and tumors.…”

Inflammatory Myofibroblastic Bladder Tumor in a Patient with Wolf-Hirschhorn Syndrome

“…To our knowledge, this is the first reported case of IMT of bladder urinary in a pediatric patient with WHS. A case of multiple hemangiomas, one case of cutaneous T-cell Lymphoma in a 21 year-old-male, and two cases of myelodysplastic syndrome in children are only reported [17, 18]. In our case, karyotype analysis of peripheral blood was normal as test fish of chromosome 4.…”