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Med One. 2016 Oct 25;1:e160020. DOI:10.20900/mo.20160020.
1Division of Pediatric Oncology, Catholic University of Rome, Largo A. Gemelli 8, Roma 00168, Italy
Correspondence: Antonio Ruggiero, MD, Catholic University of Rome, Italy. Phone: + 39-06-3058203; Fax: + 39-06-3052751; E-mail: email@example.com
Background: Hematodermic tumours are rare neoplasms of the hematolymphoid system, consisting of a heterogeneous group of tumours with an aggressive course. The early recognition of the tumor may allow physicians to avoid making misdiagnosis.
Objectives: To report a case occurring in an eight-year-old child, that was initially diagnosed as haematoma secondary to child abuse.
Results:A child with an initially misdiagnosed hematodermic tumour located on the posterior region of the left thigh underwent, few months after the clinical onset, a skin biopsy on affected tissue for histopathology which was diagnostic for CD4+/CD56+ hematodermic tumour.
Conclusion: Although hematodermic tumors are relatively rare, clinicians should broaden the differential diagnosis, including malignancies, when unexplained clinical pictures are brought to their attention.
We describe a child with a primary CD4+/CD56+ hematodermic tumour characterized by a skin lesion with a bruise-like aspect, initially diagnosed as having an haematoma of the thigh secondary to child abuse.
He presented to his local hospital with a 5-month history of haematoma located on the posterior region of the left thigh. He was initially investigated for a bleeding diathesis but the coagulation screening tests and an haematoma drainage were inconclusive. Therefore the physicians concluded for a battered child syndrome and treated the patient conservatively.
Four months later, he was admitted to our hospital for further investigations. On examination, an ecchymosis on the left posterior thigh and multiple purple lesions on both legs were present (Figure 1 a-b). During his stay in hospital, the boy developed a left groin lymph node enlargement. He underwent a lymph node and cutaneous biopsy which were diagnostic for CD4+/CD56+ hematodermic tumour. Bone marrow aspirate was negative for neoplastic infiltration. The patient was treated with chemotherapy, he went into remission and the haematoma gradually disappeared. Two years after initial presentation, he is well and disease free.
The disease spectrum of CD4+/CD56+ hematodermic tumour is expanding and only a few paediatric cases have been documented [1,2,3]. It has been described as a distinct clinicopathologic entity, with aggressive course and poor outcome. CD4+/CD56+ hematodermic malignancies are a rare entity of the hematolymphoid system, consisting of neoplasms with a high incidence of cutaneous involvement and risk of rapid leukaemic dissemination. These neoplasms mainly involve skin at diagnosis with often indolent presentation, but manifest an aggressive and rapidly fatal course without delay .
Knowledge of this particular clinical presentation may allow physicians to avoid making misdiagnosis. In our case, the initial erroneous assertion led to a delay in correct diagnosis and determined an extensive social service investigation that found the child's parents completely free of any charge or suspect of child abuse.
The diagnosis of CD4+/CD56+ hematodermic tumour may be complex, especially when an isolated extramedullary involvement is the presenting sign [5,6]. Since clinical findings may be non-specific and histopathology is only suggestive, misdiagnosis is likely. Clinicians should broaden the differential diagnosis, including malignancies, when unexplained or bad-definite clinical pictures are brought to their attention. Early recognition and appropriate treatment are essential in improving the chance of cure for these patients.
No conflict of interests and financial disclosure is present. Each author has substantially contributed to the acquisition and analysis of data.