Whole‐lung irradiation in the treatment of metastatic synovial sarcoma

Gerber, Naamit K.; Meyers, Paul A.; LaQuaglia, Michael P.; Wolden, Suzanne L.

doi:10.1002/pbc.25114

Cited by 2 publications

(2 citation statements)

References 18 publications

(20 reference statements)

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“…PPSS comprises 14.7-18% of all primary lung sarcomas and constitutes around 0.5% of all lung malignancies ( 5 , 6 ). Despite it is rareness most synovial sarcoma is present in extremities that favor metastasizing to the lung forming secondary pulmonary synovial sarcomas ( 7 , 8 ). Synovial sarcoma (SS) is more common in adolescents and young adults ( 8 ).…”

Section: Discussionmentioning

confidence: 99%

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report

AlQatari,

Ahmed,

AlHije

et al. 2023

Med Arch

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Background: Synovial sarcoma is a rare and aggressive soft tissue malignancy most commonly arises from periarticular tissue of the extremities. Although several cases in the literature have reported different origins, primary pulmonary synovial sarcoma (PPSS) is an exceedingly rare and underrecognized entity, accounting for 0.5% of all lung malignancies. Clinical presentation includes chest pain, dyspnea, cough, and hemoptysis. The finding of hemothorax is a rare presentation and was barely reported in the literature. Due to its rarity and aggressive nature, the optimal treatment is unclear, while the mainstay remains surgical resection with chemo- and/or radiation therapy. Objective: To report a case of hemorrhagic effusion subsequently diagnosed with primary pulmonary synovial sarcoma with the main objective of enriching the literature regarding this rare malignancy. Case report: A 52-year-old male smoker with a background of coronary artery disease, hypertension, and diabetes mellitus was referred to our hospital. The patient presented with a history of chest pain, dyspnea, and massive right-sided pleural effusion. Laboratory investigations were unremarkable except for anemia. Chest x-ray showed a complete opacity on the right lower zone with right-sided pleural effusion. Thoracentesis was done and revealed hemorrhagic exudative effusion. Computed tomography (CT) scan showed a right heterogeneous lung mass compressing the medial segment of the middle lobe. Subsequently, the patient underwent bronchoscopy, which showed compression and edema on the right middle lobe bronchus with traces of blood coming from the right lower lobe. The patient underwent a right posterolateral thoracotomy, a fungating mass eroding the medial segment of the middle lobe was resected that was diagnosed as high-grade primary pulmonary synovial sarcoma. Radiotherapy was instituted. The patient died after two years due to recurrence. Conclusion: PPSS is an aggressive disease with poor prognostic outcomes, and Its presentation is almost similar to other lung malignancies. Meanwhile, there is no definitive management guideline, and most management depends on surgical resection if feasible with adjuvant chemo-radiation therapy.

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Section: Discussionmentioning

confidence: 99%

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report

AlQatari,

Ahmed,

AlHije

et al. 2023

Med Arch

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“…Tüdőáttétek kialakulásakor egy esetismertetés szerint teljes tüdőbesugárzást alkalmaztak jó effektivitással [26]. Az NCT02181829 klinikai vizsgálat a teljes tüdő intenzitásmodulált radioterápia hatását határozza meg.…”

Section: Kezelés éS Kimenetelunclassified

A sarcoma synoviale kezelési lehetőségei

Deme¹,

Telekes

2015

Orvosi Hetilap

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A sarcoma synoviale a lágyrész-sarcomák 5-10%-ában, az összes malignus daganatok 0,05-0,1%-ában fordul elő. Főként a végtagokon keletkezik, azonban bárhol kialakulhat. A betegek 50%-ában 3-5 éven belül metasztázisok jelennek meg (tüdő, nyirokcsomó, csont), sőt akár 20 év elteltével is számítani lehet a sarcoma synoviale kiújulására. Az 5 éves teljes túlélés lokalizált betegség esetén 76%, metasztatikus esetben 10%. Az életkor, daganatméret, szö-vettani altípus és a radioterápia megtörténte befolyásolja a prognózist. Az adjuváns kemoterápia szerepe nem bizonyított. Számos klinikai vizsgálat van folyamatban a lokálisan előrehaladott, relaptálódott/refrakter és metasztatikus sarcoma synoviale kezelésére. A sarcoma synoviale biológiai viselkedésének jobb megértésével a célzott kezelés és a konvencionális terápia kombinációja válhat a jövő útjává. Orv. Hetil., 2015, 156(22), 875-880.Kulcsszavak: sarcoma synoviale, kemoterápia, radioterápia, célzott kezelés Therapeutic options for synovial sarcomaSynovial sarcomas account for approximately 5 to 10% of soft tissue sarcomas and 0.05 to 0.1% of all malignant neoplasms. They predominantly affect the extremities but can occur in any part of the body. More than 50% of the patients are expected to develop metastatic disease within 3-5 years. In some patients disease recurrence may develop after 20 years. The 5-year overall survival rate is 10% for patients with metastatic disease and 76% for patients with localized one. Age, tumour size, histological subtype, and adjuvant radiotherapy infl uence prognosis. The role of adjuvant chemotherapy has not been proven yet. There are several ongoing clinical trials to determine the effi cacy of active agents used for therapy of locally advanced, relapsed/refractory or metastatic disease. Better understanding of the biological behaviour of synovial sarcomas would provide the future way for the targeted therapy in combination with conventional treatments. Rövidítések CCND1 = ciklin D1; CR = komplett remisszió; DRFS = távoli kiújulásmentes túlélés; EFS = eseménymentes (gyógyszertoxi-citás, -intolerancia, egyéb) túlélés; EpSSG = European Pediatric Soft Tissue Sarcoma Group; HDAC = hiszton-deacetiláz; IGF-1R = inzulinszerű növekedési faktor receptor-1; KT = kemoterápia; LRFS = lokális kiújulásmentes túlélés; MD = metasztatikus betegség; MFS = áttétmentes túlélés; OS = teljes túlélés; PD = progresszív betegség; PFS = progressziómentes túlélés; PR = parciális remisszió; RT = radioterápia; SD = stabil betegség; SEER = Surveillance, Epidemiology, and End Results; SS = sarcoma synoviale A sarcoma synoviale (SS) a ritka malignitások közé tartozik. A malignus daganatok 0,05-0,1%-át és a lágyrész-sarcomák 5-10%-át alkotja [1,2]. Az SS leggyakrabban a serdülőkorúak és fi atal felnőttek 15-40 éves korosztá-lyát érinti, azonban előfordulhat 10 éven aluli gyermekekben, sőt újszülöttekben is [3]. Prognosztikai faktorokTöbb munkacsoport vizsgálta az SS-ben szenvedő pá-ciensek túlélését befolyásoló prognosztikai faktorokat, amelyek közül az 5 cm vagy ann...

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Whole‐lung irradiation in the treatment of metastatic synovial sarcoma

Cited by 2 publications

References 18 publications

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report

Primary Pulmonary Synovial Sarcoma with Hemothorax: a Case Report

A sarcoma synoviale kezelési lehetőségei

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