Primary retroperitoneal extraskeletal mesenchymal chondrosarcoma involving the vena cava: A case report

Abstract

The current study presents a case of extraskeletal mesenchymal chondrosarcoma (ESMC) involving the vena cava that originally occurred in the retroperitoneum of a 61-year-old female. Following excision of the masses, pathological examination confirmed a diagnosis of primary ESMC. Mesenchymal chondrosarcomas are extremely rare in comparison to conventional chondrosarcomas and even more so when arising in an extraskeletal location. In the current report, the major characteristics of ESMC are discussed and a review of the current knowledge regarding this rare disease entity is presented.

Primary Intracranial Extraskeletal Mesenchymal Chondrosarcoma: Clinical Mimicry as Glomus Jugulare

“…The most common sites of origin are long limbs, orbital wall, and rarely spinal meninges. It has also been reported in axillary and shoulder region as isolated case reports [1, 3,4] . ESMCS is a very rare pathologic entity with no specific clinical or radiologic characteristics to distinguish them from other neoplastic pathologies.…”

“…ESMCS is a rare neoplasm first described in clinical literature in 1959 by Lichenstein and Berstein [3,4] . There are 2 histological variants, mesenchymal and myxoid, out of which former is rarer, more aggressive, and carries poorer prognosis.…”

“…There are 2 histological variants, mesenchymal and myxoid, out of which former is rarer, more aggressive, and carries poorer prognosis. In about 30-40% cases, MCS have an extraskeletal origin [1, 3,4] . The most common sites of origin are long limbs, orbital wall, and rarely spinal meninges.…”

“…Unlike other variants, MCS is a fast growing tumor with frequent systemic metastases [1,2] . Extraskeletal MCS (ESMCS) is an extremely rare variant of MCS with no evidence of any primary intracranial ESMCS till date [3,4] . We describe our experience with the case of middle-aged female of primary ESMCS, managed as glomus jugulare tumor (GJT) by stereotactic gamma knife radiosurgery (GKRS).…”

Radiofrequency ablation of metastatic chondrosarcoma-associated refractory ventricular tachycardia originating from the right ventricular outflow tract: A case report and literature review

“…ESMC may appear in any location that contains mesenchymal cells; primary sites include limb extremities, the torso, the head and neck, and rarely the heart (23)(24)(25). Compared with the myxoid subtype, the ESMC subtype is rare, more aggressive and with poor prognosis due to the high probability of metastases (26), which may occur several years following the initial treatment. Cardiac metastasis of ESMCs is infrequent; it has been reported that the most common site of cardiac metastasis are the right atrium and left intracavity, leading to pulmonary embolism and systemic embolism (27,28).…”