Limb Amputation after Multiple Treatments of Tenosynovial Giant Cell Tumour: Series of 4 Dutch Cases

Abstract

In Tenosynovial Giant Cell Tumours (TGCT), previously named Pigmented Villonodular Synovitis (PVNS), a distinction is made between a single nodule (localized-type) and multiple nodules (diffuse-type). Diffuse-type is considered locally aggressive. Onset and extermination of this orphan disease remain unclear. Surgical resection is the most commonly performed treatment. Unfortunately, recurrences often occur (up to 92%), necessitating reoperations and adjuvant treatments. Once all treatments fail or if severe complications occur, limb amputation may become unavoidable. We describe four cases of above-knee amputation after TGCT diagnosis.

Does CSF1 overexpression or rearrangement influence biological behaviour in tenosynovial giant cell tumours of the knee?

“…Within our well‐defined patient cohort, all patients had a minimum follow‐up of 3 years. However, patients without recurrent disease at the time of analysis could still develop this in due course, as it is known that local recurrence might develop years after initial surgery . Verspoor et al .…”

Long‐term outcomes of pexidartinib in tenosynovial giant cell tumors

“…Diffuse-type TGCT, formerly called pigmented villonodular synovitis (PVNS), constitutes 10% to 20% of cases, usually occur in large joints (eg, knees, ankles, and hips), and show a higher tendency toward recurrence. 6,7 The diffuse variant often causes debilitating symptoms, including pain, swelling, a limited range of motion, and stiffness. 1,3 Although surgery cures the vast majority of localized TGCT cases, the diffuse type shows a high tendency toward local recurrence, which occurs in approximately 50% of resected cases; therefore, limiting the value of surgery for this subtype.…”

“…Localized‐type TGCT is a locally aggressive disease, and this type accounts for 80% to 90% of TGCT cases and most commonly occurs in the digits. Diffuse‐type TGCT, formerly called pigmented villonodular synovitis (PVNS), constitutes 10% to 20% of cases, usually occur in large joints (eg, knees, ankles, and hips), and show a higher tendency toward recurrence 6,7 . The diffuse variant often causes debilitating symptoms, including pain, swelling, a limited range of motion, and stiffness 1,3 .…”

Gibt es eine Magic Bullet für Sarkome?

“…Auch für Riesenzelltumoren der Sehnenscheiden vom diffusen Typ wird der Einsatz von maßgeschneiderten Therapeutika evaluiert [26]. Diese Tumoren werden grundsätzlich als benigne eingestuft, können jedoch einen aggressiven und rezidivierenden biologischen Verlauf aufweisen, der bis hin zur Amputation führen kann [18,26]. Zielstruktur der therapeutischen Ansätze ist in diesem Fall der Monozytenkolonien-stimulierende Faktor ([M-]CSF-1; "colony stimulating factor"-1) [26].…”