Brief communication

Outcome following radiation treatment for high-risk pigmented villonodular synovitis☆

Pigmented villonodular synovitis is an uncommon benign neoplastic proliferation associated with the synovium, bursa, or tendon sheaths; most commonly occurring in the third to fourth decade of life. It is rare in children and may be painful or painless. Magnetic resonance imaging is the diagnostic study of choice. In this report, the radiologic, ultrasound, and magnetic resonance imaging findings of pigmented villonodular synovitis of the flexor hallucis longus in a 12-year-old girl are discussed. We briefly review the surgical findings as well. To our knowledge, this is the first case report that simultaneously synthesizes the imaging findings of 3 diagnostic imaging modalities for optimal visualization and is the youngest reported case of pigmented villonodular synovitis of the flexor hallucis longus tendon.

Pigmented villonodular synovitis (PVNS) is a rare pathology of the elbow, but presents a risk of progression with cartilage destruction. Surgical treatment consists in synovectomy, as complete as possible. Arthroscopy is an excellent tool for the exploration and treatment of intra-articular lesions in the elbow, but the results in PVNS of the elbow have never been evaluated. The aim of this study was to assess the recurrence rate of PVNS of the elbow after arthroscopic synovectomy, and secondarily to assess pain, joint range of motion, functional scores and complication rate.

We performed a retrospective study of a continuous series of 8 patients operated on between February 2012 and February 2019, with a mean age of 43.7 years. The operated side was the dominant side in 75% of cases. Surgery consisted in the most complete synovectomy possible, performed arthroscopically. Recurrence, clinical evaluation, with pain at rest and on mpvement on visual analogue scale (VAS) and joint range of motion, functional MEPS and DASH scores, and any complications were collected.

At a mean 66.4 months’ follow-up, 2 patients required revision surgery for recurrence. At the last follow-up, VAS for pain at rest was 0.9 and 1.9 for pain on movement. MEPS score was 85.6 and DASH score 82.2. No neurological, vascular or infectious complications of arthroscopic synovectomy were found in our series.

Arthroscopic synovectomy in the treatment of PVNS of the elbow was a reliable and safe therapeutic alternative, with a low complications rate and 2 cases of recurrence (25%) in our study. This was the first study to report the results of arthroscopic surgical treatment of elbow PVNS.

IV Retrospective study without control group.

La synovite villo-nodulaire pigmentée (SVNP) est une pathologie rare du coude mais présente un risque évolutif avec destruction cartilagineuse. Le traitement chirurgical consiste à réaliser une synovectomie la plus complète possible. L’arthroscopie est un excellent outil pour l’exploration et le traitement des lésions intra-articulaires du coude mais les résultats dans la SVNP du coude n’ont jamais été évalués. Le but de cette étude était d’évaluer le taux de récidive des SVNP du coude après synovectomie arthroscopique puis d’évaluer la douleur, les mobilités articulaires, les scores fonctionnels et le taux de complication.

Nous avons réalisé une étude rétrospective d’une série continue de 8 patients opérés entre février 2012 et février 2019 de 43,7 ans d’âge moyen. Le côté opéré était le côté dominant dans 75 % des cas. La chirurgie comprenait la réalisation d’une synovectomie la plus complète possible réalisée sous arthroscopie. La survenue d’une récidive, l’évaluation clinique avec l’échelle visuelle analogique (EVA) de douleur au repos et à l’effort, la mobilité articulaire et fonctionnelle avec le score de MEPS et de DASH ainsi que la recherche de complications ont été recueillies.

À 66,4 mois de suivi moyen, 2 patients ont nécessité une reprise chirurgicale pour une récidive. Au dernier recul, l’EVA au repos était de 0,9 et à l’effort de 1,9. Le score de MEPS était de 85,6 et le score DASH de 82,2. Aucune complication neurologique, vasculaire ou infectieuse du geste d’arthrolyse arthroscopique n’a été retrouvée dans notre série.

La synovectomie arthroscopique dans le traitement des SVNP du coude est une alternative thérapeutique fiable et sûre, avec un taux de complication faible et 2 cas de récidive (25 %) dans notre étude. Notre étude est la première à rapporter les résultats du traitement chirurgical arthroscopique de la SVNP du coude.

IV. Étude rétrospective sans groupe contrôle.

Tenosynovial giant cell tumor (TGCT) is a rare proliferative disorder of synovial membrane that previously was known as pigmented villonodular synovitis. Primary treatment involves surgical resection; however, complete removal of all disease involvement is difficult to achieve. Radiation may be useful to reduce the risk of recurrence. We report and update our institutional experience treating diffuse and recurrent TGCT with postsurgical external beam radiation therapy.

We performed a retrospective chart review of 30 patients with TGCT from 2003 to 2019 treated with radiation therapy. Each patient was evaluated for demographics, radiation treatment parameters, surgical management, complications, and outcome.

With mean follow-up of 82 months (range, 3-211), 24 patients (80%) who underwent surgery followed by radiation therapy did not experience any further relapse, and all 30 patients achieved local control (100%) with additional salvage therapy after radiation therapy. The most common site of disease was the knee (n = 22, 73%), followed by the ankle (n = 5, 16%) and the hand (n = 3, 10%). Seven patients (24%) presented at time of initial diagnosis and 23 (76%) presented with recurrent disease after surgical resection, with an average of 2.6 surgical procedures before radiation therapy. After resection, 18 of 30 patients (67%) demonstrated residual TGCT by imaging. The median radiation therapy dose delivered was 36 Gy (range, 34-36 Gy) in 1.8 to 2.5 Gy/fractions for 4 weeks. In the assessment of posttreatment joint function, 26 sites (86%) exhibited excellent or good function, 2 (7%) fair, and 2 poor (7%) as determined by our scoring system. There were no cases of radiation-associated malignancy.

Among patients with diffuse or recurrent TGCT, postsurgical external beam radiation therapy provided excellent local control and good functional status, with minimal treatment-related complications. Postsurgical radiation therapy is a well-tolerated noninvasive treatment that should be considered after maximal cytoreductive resection to prevent disease progression and recurrence.

Pigmented Villonodular Synovitis (PVNS) is a kind of extremely rare and not easily diagnosed disease, while the occurrence following the anterior cruciate ligament (ACL) rupture is comparably high.

Here we reported 2 patients presenting knee swelling, pain, and giving away symptoms following a rupture of ACL history. Clinical and radiological findings were supportive of ACL rupture and PVNS, prompting the patient to undergo surgery. The synovium was completely removed with the help of an arthroscope. Reconstruction of the ruptured ACL was carried out a few months later. At the last follow-up visit, both the patients were doing well with no recurrence of pain, swelling, or give way sensation.

The diagnosis of PVNS is often difficult because in the early stages, symptoms are often non-specific and pathological examination remains the gold standard for the diagnosis of PVNS. We have reviewed some of the previously reported literature on PVNS, suggesting that joint instability may be an important risk factor for PVNS and summarizing the treatment options for PVNS.

More attention should be paid to the incidence of PVNS in patients with joint instability, particularly in patients with cruciate ligament injuries. Early and reliable stabilization of the joint may significantly prevent PVNS and ensure a better clinical outcome.

Among all Soft Tissue sarcomas there are some subtypes with low incidence and/or peculiar clinical behaviour, that need to be consider separately. Most of them are orphan diseases, whose biological characteristics imply a clearly different diagnostic and therapeutic approach from other more common sarcoma tumors. We present a brief and updated multidiciplinary review, focused on practical issues, aimed at helping clinicians in decision making. In this second part we review these subtypes: Alveolar Soft Part Sarcoma, Epithelioid Sarcoma, Clear Cell Sarcoma, Desmoplastic Small Round Cell Tumor, Rhabdoid Tumor, Phyllodes Tumor, Tenosynovial Giant Cell Tumors, Myoepithelial Tumor, Perivascular Epithelioid Cell Neoplasms (PEComas), Extraskeletal Myxoid Chondrosarcoma, NTRK-fusions Sarcomas. Most of them present their own radiological and histopathological feautures, that are essential to know in order to achieve early diagnosis. In some of them, molecular diagnosis is mandatory, not only in the diagnosis, but also to plan the treatment. On the other hand, and despite the low incidence, a great scientific research effort has been made to achieve new treatment opportunities for these patients even with approved indications. These include new treatments with targeted therapies and immunotherapy, which today represent possible therapeutic options. It is especially important to be attentive to new and potential avenues of research, and to promote the conduct of specific clinical trials for rare sarcomas.