Post-Paget telangiectatic osteosarcoma of the skull

Abstract

Sarcomatous transformation is the most dreaded complication of Paget's disease. We report on a case of post-Paget telangiectatic osteosarcoma of the skull, a variant of osteogenic osteosarcoma, in a 79-year-old woman. We discuss the radiological pattern in relationship to the differential diagnosis.

Primary telangiectatic osteosarcoma of occipital bone: A case report and review of literature

“…[7] Extragnathic TOSs have been rarely reported at unusual sites such as sphenoid bone [4] and frontal bone. [3] The hallmark of TOS is expansile lytic lesion without sclerosis [6] and often exhibits heterogenous high signal intensity on T2WI and fast spin T2WI and may also show zones of high signal intensity on T1WI and proton density (PDWI) corresponding to areas of hemorrhage. These tumors show prominent gadolinium enhancement.…”

“…All cases of skull bone TOS, so far reported including our case, have shown characteristic radiological features. [3,4] Histopathological studies in correlation with imageological findings are essential for the diagnosis of TOS. [8] The histological and radiological diagnostic criteria proposed by the World Health Organization include: 1) predominantly lytic bone mass with minimal sclerosis; 2) grossly cystic medullary mass with no or minimal solid or sclerotic component; and 3) histologic features consisting of bone-forming tumor with notable blood-filled spaces separated by septae lined by, and/ or containing, malignant tumor cells with prominent nuclear atypia and limited osteoid deposition.…”

“…Telangiectatic osteosarcoma (TOS), an uncommon variant of osteosarcoma, involving skull bones has been rarely reported and to date, only 5 cases of TOS involving the skull bone have been reported in the world literature. [1][2][3][4] Case Report A 30-year-old woman was brought to the Emergency Department with a 15-day history of occipital headache and 2-day history of nausea and two episodes of vomiting. Clinical examination revealed fine nystagmus and bilateral papilledema.…”

Telangiectatic osteosarcoma, a rare complication of Paget's disease of bone

“…5,9,10 Historically, first recognised by Paget in 1853 and classified as a distinct variant of osteosarcoma by Ewing in 1922, telangiectatic osteosarcoma predominantly affects male patients (male to female ratio of 2:1), and is seen more often in younger patients. [10][11][12] When considering the clinical, radiological and histopathological features of telangiectatic osteosarcoma, it is important to differentiate it from an aneurysmal bone cyst and giant cell tumours of the bone. 12,13 Distinguishing pathological features of this case were the destructive growth pattern (cortical destruction and infiltration of soft tissue), gross appearance (predominantly cystic lesion filled with blood), and the presence of malignant cells on microscopic examination.…”

“…12,13 Distinguishing pathological features of this case were the destructive growth pattern (cortical destruction and infiltration of soft tissue), gross appearance (predominantly cystic lesion filled with blood), and the presence of malignant cells on microscopic examination. [9][10][11][12] Despite advances in neoadjuvant chemotherapy, the prognosis remains poor in the setting of all secondary osteosarcomas arising in PDB (a five-year survival rate of 10%). The poor outcome of osteosarcoma in this clinical setting suggests a different biological potential which may relate to underlying genetic susceptibility or environmental factors.…”