Primary pleuropulmonary synovial sarcoma

Essary, Lydia R.; Vargas, Sara O.; Fletcher, Christopher D.�M.

doi:10.1002/cncr.10188

Cited by 137 publications

(49 citation statements)

References 38 publications

(57 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…It accounts for 10% of all soft-tissue sarcomas and is slightly more common in men [4]. Four histologic subtypes are described: biphasic, monophasic (spindle), monophasic epithelial, and poorly differentiated (round cell) tumor [5]. Monophasic tumor is the most commonly observed subtype.…”

Section: Discussionmentioning

confidence: 99%

Primary Synovial Sarcoma of Lung

Çabuk

Ustuner

Akgül

et al. 2014

Korean J Thorac Cardiovasc Surg

View full text Add to dashboard Cite

Synovial sarcoma (SS) is a highly malignant tumor that accounts for 10% of all soft-tissue sarcomas. Primary SS arising from the lung is extremely rare, and the prognosis is poor. We report a case of pulmonary SS presenting with a mass lesion invading the right upper and middle lobes, extending to the mediastinum and the chest wall. After tru-cut biopsy, surgical resection was performed. The final diagnosis was SS (biphasic type) based on histological and immunohistochemical findings. There are no guidelines for optimal treatment due to the rarity of these tumors. Current treatment includes surgery and adjuvant chemotherapy and/or radiotherapy.

show abstract

Section: Discussionmentioning

confidence: 99%

Primary Synovial Sarcoma of Lung

Çabuk

Ustuner

Akgül

et al. 2014

Korean J Thorac Cardiovasc Surg

View full text Add to dashboard Cite

show abstract

“…[85][86][87] In the literature and in the experience of the IPPBR, this neoplasm is seen in adolescents and older individuals, unlike Type I PPB which occurs in very early childhood. Although usually a solid proliferation, pleuropulmonary synovial sarcoma can be cystic and present with pneumothorax, four cases of which have been brought to the attention of the IPPBR since 2006 [manuscript in preparation].…”

Section: Primary Pleuropulmonary Synovial Sarcomamentioning

confidence: 99%

Pulmonary cysts in early childhood and the risk of malignancy

Priest

Williams

Hill

et al. 2008

Pediatric Pulmonology

264

215

View full text Add to dashboard Cite

Summary. Surgery for congenital and early childhood lung cysts is often dictated by symptoms such as respiratory distress, infection or pneumothorax. Asymptomatic cysts present a therapeutic dilemma: surgical intervention and ''conservative'' observation have advocates. The risk of malignancy in such cysts is considered by some an indication for surgical intervention and is reviewed in this paper. Pleuropulmonary blastoma (PPB) is the most frequent malignancy associated with childhood lung cysts. Although rare, PPB occurs predictably in certain clinical and familial situations. This unique biology of PPB can inform the cyst management decision. The earliest manifestation of PPB is a malignant lung cyst in young children, clinically and radiographically indistinguishable from benign congenital lung cysts. Histopathologic examination differentiates cystic PPB from the benign cystic variants. Surgical excision of cystic PPB (with or without chemotherapy) cures approximately 85-90% of children. If not excised, cystic PPB evolves to cystic/solid or solid high-grade sarcoma (cure rate 45-60%) by age 2-6 years. Numerous reports of ''malignancy in a congenital lung cyst'' are now understood as the characteristic progression of cystic PPB. PPB is genetically determined in many cases. Detailed family history may reveal the hallmarks of PPB in the patient or young relatives: a unique constellation of diseases including lung cysts, cystic nephroma, childhood cancers, stromal sex-chord ovarian tumors, seminomas or dysgerminomas, intestinal polyps, thyroid hyperplasias, and hamartomas. Pneumothorax and multifocal/bilateral lung cysts also characterize PPB. These diagnoses predict that a lung cyst is more likely PPB than a benign congenital cyst. Patients fitting this pattern deserve histologic diagnosis. The genetic basis for this heritable syndrome is unknown but is being actively investigated.

show abstract

“…Spurrell et al [15] at Royal Marsden Hospital, London, between 1978 and 2003, presented analysis of 104 cases of SS, out of which only 10 were pleuropulmonary in origin. Pleuropulmonary SS seem to be more aggressive as compared to their counterparts of the extremities with less chance of complete resection and difficulty to achieve adequate wide margins and hence high percentage of local recurrence [16].…”

Section: Discussionmentioning

confidence: 98%

Primary Pleuropulmonary Synovial Sarcoma with Brain Metastases in a Paediatric Patient: An Unusual Presentation

Chirmade

Parikh

Anand

et al. 2017

Advances in Respiratory Medicine

View full text Add to dashboard Cite

Primary lung neoplasms are rare in children. The most common primary lung malignancies in children are pleuropulmonary blastoma and carcinoid tumour. Synovial sarcoma (SS) accounts for approximately 1% of all childhood malignancies. In absolute terms, the SS of the lungs and pleura are extremely rare and pose a diagnostic difficulty. Soft tissue sarcomas usually have a high potential for metastases, however, metastasis to the brain is rare, even in widely disseminated disease, and it has been described only in 3 case reports previously. Primary pleuropulmonary SS with brain metastases is even rarer. Here we present a case of an 11-year-old boy who presented with respiratory complaints, viz. fever and cough for 20 days. Initial impression was lung abscess, however, on histopathological, immunohistochemical and molecular study, the disorder was diagnosed as synovial sarcoma. After a week from the first consult, the child developed neurological symptoms, viz., an episode of convulsion and gradually worsening power of the lower limb. Computed tomography scan and Magnetic Resonance Spectroscopy was suggestive of brain metastases. Given the rarity of primary lung neoplasms in children, clinical detection remains a challenge. Delayed diagnoses are common as respiratory symptoms may be attributed to inflammatory or infective processes. Primary pleuropulmonary synovial sarcoma is a rare tumour and it is not known to commonly metastasise to the brain. Though rare, primary pleuropulmonary SS should be considered an important differential among peadiatric primary lung neoplasms due to its potential for curability if detected early, and more aggressive metastatic pattern, e.g. brain metastases making early detection imperative.

show abstract

Primary pleuropulmonary synovial sarcoma

Cited by 137 publications

References 38 publications

Primary Synovial Sarcoma of Lung

Primary Synovial Sarcoma of Lung

Pulmonary cysts in early childhood and the risk of malignancy

Primary Pleuropulmonary Synovial Sarcoma with Brain Metastases in a Paediatric Patient: An Unusual Presentation

Contact Info

Product

Resources

About