Published online Oct 16, 2022. doi: 10.12998/wjcc.v10.i29.10763
Peer-review started: June 14, 2022
First decision: July 29, 2022
Revised: August 9, 2022
Accepted: August 30, 2022
Article in press: August 30, 2022
Published online: October 16, 2022
Malignant giant cell tumor of the tendon sheath (MGCTTS) is an extremely rare malignant tumor originating from synovial and tendon sheath tissue with highly aggressive biological behavior and a high rate of local recurrence and distant metastasis which should be considered a highly malignant sarcoma and managed aggressively. How to systemically treat MGCTTS remains a challenge. In this case, a patient with MGCTTS suffered a recurrence after 2 surgical resections received adjuvant chemotherapy and radiation therapy, but the treatment outcome remained poor. More clinical trials and better understanding of the biology and molecular aspects of this subtype of sarcoma are needed while novel medicines should be developed to efficiently target particular pathways.
A 52-year-old man presented with persistent dull pain in the right groin accompanied by limited right hip motion starting 6 mo ago. Two months before his attending to hospital, the patient's pain worsened, presenting as severe pain when standing or walking, limping, and inability to straighten or move the right lower extremity. Surgical excision was performed and MGCTTS was confirmed by pathology examination. Two recurrences occurred after surgical resection, moreover, the treatment outcome remained poor after adjuvant chemotherapy and radiation therapy. The patient died only 10 mo after the initial diagnosis.
MGCTTS is characterized by a joint mass with pain and limited motion. It typically grows along the tendons and infiltrated into the surrounding muscle and bone tissue, with a stubborn tendency to relapse, as well as pulmonary metastasis. Radically surgical resection provides a choice of treatment whereas post-operation care should be taken to preserve the function of the joint. Chemotherapy and radiotherapy can be used as alternative treatments when radical resection cannot be performed.
Core Tip: This case demonstrates the highly aggressive biological behavior of Malignant giant cell tumor of the tendon sheath (MGCTTS), which mostly appears in Computed tomography (CT) images as a large mass that grows around the joint with poorly defined borders. CT can show details of bone destruction surrounding the lesion caused by extensive invasion. Magnetic resonance imaging clearly shows the histological features of the tumor and its relationship to the surrounding tissue. There is a stubborn tendency for MGCTTS to relapse and a possibility of pulmonary metastasis. Postoperative follow-up, especially long-term follow-up for evaluating recurrence, is necessary. Meanwhile systemic examination should be undertaken to evaluate distant metastasis.