Cutaneous metastases from Ewing's sarcoma: report of two cases

Abstract

Ewing's sarcoma is a malignant osseous neoplasm that affects mostly children and young adult males. Clinically, the neoplasm presents with oedema, swelling, and pain of the involved area. Histopathologically, Ewing's sarcoma consists of solid sheets of small round cells, with vesicular nuclei and scant cytoplasm, arranged in irregular masses separated by strands of fibrous tissue, with areas of necrosis en masse intermingled with intratumoural haemorrhage. Ewing's sarcoma is an extremely aggressive neoplasm and metastases to sites such as lung, pleura, other bones, central nervous system, liver, and regional lymph nodes frequently develop in early stages of the disease. Surprisingly, despite the highly aggressive biological behaviour of this neoplasm, cutaneous metastases from Ewing's sarcoma are very uncommon. We report two patients with Ewing's sarcoma of the bone who developed cutaneous metastases. As in other internal malignancies, the onset of cutaneous metastases in patients with Ewing's sarcoma indicates a poor prognosis.

Multifocal Ewing’s sarcoma of the brain, calvarium, leptomeninges, spine and other bones in a child

“…Regional lymph nodes are frequently involved in early stages of the disease. 1,2,4 Spreading to the central nervous system (CNS) is uncommon and is usually due to direct extension of the sarcoma into the extradural space and meninges. 4 Isolated CNS lesions are seen extremely rarely in Ewing's sarcoma patients, with an incidence ranging between 1.1% and 4.3%.…”

“…A diagnosis of ES is established on the basis of the light microscopic features of immunohistochemical findings for a biopsy of the involved bone. 1,2,5,14 The histopathologic hallmark of ES is sheets of densely packed, small, round cells with scant clear cytoplasm and vesicular, hyperchromatic regular nuclei. Intracytoplasmic glycogen is present, and the perimembranous region is positive for CD-99 positivity.…”

“…1,2,5,14 ES and PNET are closely related tumours that may occur as skeletal or extraskeletal soft tissue neoplasms. Both tumours express the MIC-2 antigen 17 and peripheral PNETs contain the same t (11,22) translocation that is found in the great majority of cases of classic osseous ES 18 .…”

“…[1][2][3][4][5] In 1921, James Ewing first described this entity as 00endothelioma of the bone00, and he suggested that it arose from the endothelial cells of blood vessels within the bone. 6 ES usually presents as a solitary bone lesion.…”

Cutaneous Hematopathology

“…It is most frequently encountered on the extremities, followed by the trunk and the head and neck (Boland and Folpe 2013 ;Collier et al 2011 ). Cutaneous metastases from deep-seated Ewing sarcoma are rare but have been described (Izquierdo et al 2002 ).…”