synovial sarcoma is a rare soft tissue sarcoma accounting for approximately 8% of the soft tissue sarcomas . it is a mesenchymal tumor with varied epithelial differentiation and is frequently associated with a translocation between chromosomes x and 18 . though synovial sarcoma predominantly affects the extremities of young adults , primary lesions have also been identified in the head and neck , abdominal wall , and thorax ( including the mediastinum , pericardium , pleura , and lung ) [ 2 , 3 , 4 ] . a recent medline search revealed approximately 60 cases of primary pulmonary synovial sarcoma reported worldwide in the last 30 years . we report the case of a female patient with primary pulmonary synovial sarcoma diagnosed and treated with neoadjuvant chemotherapy during pregnancy . a 26-year - old caucasian female , g2p1 , at 21 weeks gestation with no other significant medical history presented to a community hospital with a dry cough and right - sided pleuritic chest pain , which radiated to her right shoulder . she reported no family history of unusual or early - onset malignancies , she never smoked and she worked as a receptionist at a wastewater management company . her previous pregnancy 2 years ago was uncomplicated and resulted in a healthy baby boy . on presentation , the patient was afebrile , tachypneic , and tachycardic with normal pulse oximetry . her complete blood count was remarkable only for a low - grade normocytic anemia and her comprehensive metabolic panel was normal . her symptoms and pregnancy - associated risk were suggestive of a pulmonary embolus , which prompted a ct of the chest with iv contrast . the ct scan revealed a smooth , lobulated 6.5-cm mass in the right hilum ( fig . one week after her initial presentation , the patient underwent bronchoscopy , which disclosed a mass arising in the airway . h&e staining of the biopsied specimens revealed malignant spindle cells infiltrating endobronchial tissue ( fig . additional stains for epithelial membrane antigen pancy - tokeratin ae1/3 , cam5.2 , desmin , smooth muscle actin , s-100 , and hmb45 were all negative . fluorescence in situ hybridization performed on the formalin - fixed paraffin embedded tissue revealed two populations : a predominant population ( > 90% ) of polypoid cells with multiple copies of syt probe of which > 80% had the break - apart signal and a small population ( 10% ) of diploid cells showing the break - apart signal for syt probe . the combined histopathology , immunohistochemistry , and fluorescence in situ hybridization studies confirmed the diagnosis of monophasic spindle cell synovial sarcoma . it was unclear at presentation if this represented a metastatic or primary pulmonary synovial sarcoma . due to her pregnancy and possible fetal risk , the patient declined whole body imaging ( i.e. , pet - ct ) to survey for a possible primary source . based on these clinical observations and testing , it was assumed that the lung mass was a primary pulmonary synovial sarcoma . surgical resection , the only possible immediate cure , was deferred due to pregnancy as well as the size and proximity of the mass to vital structures . neoadjuvant chemotherapy would necessarily focus on containing the growth of the mass , with hopes of inducing tumor necrosis , without causing undue harm to the fetus . a literature review yielded data supporting clinical benefit and acceptable third - trimester fetal risk associated with ifosfamide [ 7 , 8 , 9 ] . she was given 2 cycles of ifosfamide as follows : 2 g / m iv bolus over 4 h followed by 2 g / m / day as continuous iv infusions for 6 days ( the total dose given was 14 g / m / cycle ) each along with mesna . the patient was then rushed to the operating room for an emergency c - section . she delivered a baby boy , who , aside from typical prematurity - associated concerns , was healthy . the patient 's follow - up chest ct after delivery showed approximately 30% decrease in the size of the primary mass ( fig . after delivery , the patient was treated with two more cycles of standard neoadjuvant chemotherapy with mesna , adriamycin , and ifosfamide . she also underwent a pet - ct , which showed increased metabolic activity solely at the site of the known lung malignancy and an estimated 30% decrease in the size of the primary mass . she was then scheduled for right total pneumonectomy and mediastinal lymphadenectomy approximately 8 weeks after the birth of her son . gross pathology revealed a large , white - tan , necrotic tumor mass in the right upper lobe extending inferiorly down into the interlobar fissure . all 5 resected lymph nodes as well as the bronchial and vascular margins were free of tumor . adjuvant mesna , adriamycin , and ifosfamide were offered , but the patient declined due to concerns about toxicity . a pet - ct scan performed 5 months later revealed recurrent sarcoma and a 4.7-cm right hilar and hypermetabolic mass compressing the right main pulmonary artery and indenting the left atrium . bronchoscopy with endobronchial ultrasound delineated a right hilar / paratracheal mass 34 cm in size , causing extrinsic compression of the left mainstem bronchus . the patient developed fistulae involving the trachea , bronchus , pleura , and esophagus , followed by cardiomyopathy and pneumonia . she eventually succumbed to her disease and passed away approximately 13 months after her diagnosis . primary pulmonary sarcomas accounted for approximately 0.2% of all lung cancers in the usa between 1975 and 2007 . immunohistochemistry can be helpful in diagnosis , but due to the variability in surface antigen expression , demonstration of the t(x;18)(p11.2;q11.2 ) is critical as a confirmatory test as it is present in 95% of all synovial sarcomas . the diagnosis of cancer occurs once in every 1,000 pregnancies , with breast , uterine , and cervical cancer , lymphoma , and melanoma being the most common . to date the first case was reported by sakurai et al .   in 2006 and describes a 33-year - old female who presented with acute back pain and a left hemothorax in her fifth month of pregnancy . reported the case of a young woman in the 34th week of gestation who presented with a pneumothorax . since the patient was close to term , delivery was induced , allowing for expedited pneumonectomy and adjuvant chemotherapy . most synovial sarcomas arise in the extremities and have a predilection to metastasize to the lung . thus , when this neoplasm is found in the lung , a thorough evaluation for a remote primary lesion is needed . this presents a challenge during pregnancy since standard whole - body imaging modalities are potentially harmful to the fetus . this necessitated the reliance on a thorough physical exam for a primary lesion , which was not found . complete surgical resection of the primary tumor , in this case via a right pneumonectomy , was also contraindicated due to pregnancy . to our knowledge , this is the first case of a pregnant patient with primary pulmonary synovial sarcoma treated with chemotherapy .
synovial sarcoma is a rare soft tissue sarcoma that typically arises in the extremities of young adults . 
 we report a case of a 26-year - old pregnant woman with biopsy - proven primary synovial sarcoma of the lung that was treated with chemotherapy with radiographic response . 
 this is only the third documented case of primary pulmonary synovial sarcoma occurring during pregnancy and the first case where chemotherapy was given .