19 yo presented to provider at 15 wks gestation for painful thigh lesion, clinically diagnosed as lipoma. Lesion grew throughout second and third trimesters. Referred for further evaluation postpartum.
Large biphasic synovial sarcoma invading surrounding fatty tissue, fascia, and tendons. IHC: vimentin+, B-cell lymphoma (bcl)+, epithelial membrane antigen (EMA) +; MIC occasionally +; Ki67 20%+. t (X; 18) (p11; q11) translocation unable to be tested.
Imaging evaluation for distant metastases was negative. Proceeded with left lower limb amputation with negative resection margins. Staged pT2bNx G3. Adjuvant chemotherapy with ifosfamide 5 g/m2 and doxorubicin 50 mg/m2 weekly x6 weeks. External radiotherapy 10 Gy/week for total dose of 50 Gy. Patient currently undergoing radiation, no evidence of local recurrence.
Full-term cesarean delivery. No treatment/evaluation during pregnancy.
28 yo pregnant HIV+ female noted to have a mass on her left thigh. Ultrasound of thigh suspicious for sarcoma. MRI demonstrated large mass within left medial thigh () replacing distal fibers of left adductor longus muscle belly, encasing femoral vessels, and infiltrating into left sartorius muscle.
Biopsy confirmed synovial sarcoma.
CT scan no evidence of distant metastases. Stage pT2bN0M0 G2. Initial surgery (hip disarticulation) at 30 wk gestation. 4 wks postoperative represented with sepsis and infected wound stump. Required three wound debridements and long-term IV antibiotics. Surveillance plan: clinical review and chest X-ray every 3 months x2 years then every 6 months x3 years, then annually x5 years.
Antenatal steroids given prior to initial surgery at 30 wk gestation. Presented at 34 wks with sepsis arising from infected leg stump. Fetal growth restriction suspected (% ile). Emergent cesarean and wound exploration and debridement at 34 wks. Delivered a female neonate weighing 1756 g.
32 yo G1P0 presented for evaluation of large left kidney mass after experiencing gross hematuria starting 3 weeks earlier. Renal ultrasound demonstrated abnormal mass concerning for neoplasm. MRI confirmed findings of a large tumor in the left lower renal pole. No lymphadenopathy, adrenal involvement, or distant metastases were noted.
Monomorphic cellular pattern with large cells and dark nucleoli. IHC vimentin+, bcl-2+, low growth ratio (). CD 99-, estrogen receptor-, progesterone receptor-, AFP-, HCG-. RT-PCR confirmed t (X; 18) translocation with SYT-SSX2 fusion transcript confirming monophasic variant of synovial sarcoma.
Nephrectomy at time of cesarean delivery. Adjuvant chemotherapy with ifosfamide (5 g/m2) and doxorubicin (75 mg/m2) for two cycles followed by 54 Gy radiation to region. After radiation therapy, 2 additional cycles of ifosfamide/doxorubicin were given. Eight-month follow-up shows no recurrence.
34-week cesarean delivery with left nephrectomy after administration of antenatal corticosteroids.
38 yo presented at 26 wk gestation with dyspnea, upper right back pain, and orthopnea. Chest X-ray and CT angiogram revealed large 12 cm mass in the right chest. CT-guided needle biopsy demonstrated high-grade neoplasm.
Right pneumonectomy and lymph node dissection performed at 28 wk gestation. Negative margins not obtained due to tumor burden. Patient presented 8 wks later with worsening dyspnea and new pulmonary embolism. Repeat CT revealed tumor growth and near complete occlusion of SVC. Radiation therapy administered. Unable to undergo chemotherapy due to liver dysfunction. Patient expired 2 weeks after readmission.
Antenatal steroids prior to 28 wk pneumonectomy. Fetal growth restriction noted at 30 wks with subsequent poor interval growth leading to repeat cesarean section at 32 wks. Newborn required pulmonary artery banding but was discharged home on day of life 50.
G2P1 presented at 34 wks gestation with worsening dyspnea and left-sided cheat pain for 1 month. Chest X-ray revealed a left apical pneumothorax treated with pigtail chest tube. Repeat chest X-ray concerning for mass in this area. CT revealed left pneumothorax with significant complex mass in the left lung. Cardiothoracic surgery proceeded with video-assisted thoracoscopy.
Poorly differentiated synovial sarcoma within left pleural debris. IHC vimentin+, AW1/3+, CK7+, CK56+, K903+, S-100-, calretinin-.
Postpartum left pneumonectomy followed by adjuvant chemotherapy. Patient expired 13 months after resection.
Induction of labor after pathology findings confirmed neoplasm leading to a vacuum-assisted vaginal delivery of a 2125 g male neonate.
26 yo G2P1 at 21 wk gestation presented with dry cough and right sided pleuritic chest pain. CT revealed smooth, lobulated 6.5 cm mass in the right hilum. Bronchoscopy with multiple biopsies performed.
Malignant spindle cells infiltrating endobronchial tissue. IHC CD99+, bcl-2+, CD34-, AE1/3-, Cam5.2-, desmin-, smooth muscle actin-, S-100-, HMB45-. FISH revealed predominant population of polypoid cells with multiple copies of SYT probe. Findings consistent with monophasic spindle cell synovial sarcoma.
Primary surgical management deferred given pregnancy. Patient given 2 cycles of neoadjuvant chemotherapy: ifosfamide 2 g/m2 IV bolus over 4 h followed by 2 g/m2/day continuous IV for 6 days (total dose 14 g/m2/cycle) given with mesna. Cycles given at 26 and 29 wk gestation. CT scan postdelivery showed a 30% decrease in size of primary mass. Two additional neoadjuvant cycles with doxorubicin, ifosfamide, and mesna were given. Right total pneumonectomy and mediastinal lymphadenectomy performed 8 wk postpartum. Lymph nodes and margins negative. Patient declined adjuvant chemotherapy. 5 months later, recurrence occurred. Patient given palliative radiation. Patient expired 13 months after diagnosis.
Patient experienced decreased fetal movement at 31 wks and was diagnosed with oligohydramnios. She had a nonreassuring fetal heart tracing and was delivered via emergent cesarean delivery.
21 yo G1 presented with slow growing mass in anterior abdominal wall. MRI revealed soft tissue mass attached to anterior abdominal wall without metastasis. FNA cytology revealed spindle cell tumor. Total excision of mass was then completed.
Crowded spindle cells in wavy, short fascicular pattern. IHC cytokeratin+, EMA+, Mic-2+, bcl-2+, calponin-. Pathological diagnosis of biphasic synovial sarcoma.
Patient declined local radiation therapy and chemotherapy. She presented 6 months later with local recurrence. She declined further treatment and was lost to follow-up.
23 yo at 28 wk gestation presenting with fever, cough, and dyspnea. Ultrasound revealed a large mass in left hemi-thorax. Initial biopsy inconclusive, repeat CT-guided biopsy revealed synovial sarcoma.
Information not available.
Advised for surgical resection and chemo-radiation therapy but further information not available.
25 yo at 8 wk gestation presented with abdominal distension, left flank pain, and hematuria x10 months. 10 cm flank mass palpated. CT/MRI consistent with renal cell carcinoma without evidence of metastases.
Malignant spindle cells arranged in sheets, fascicles, and whorls. IHC bcl2+, Mic2+, vimentin+. RT-PCR for fusion transcript SYT-SSX1 negative. Final diagnosis monophasic primary synovial sarcoma of the kidney.
Left radical nephrectomy referred to oncology for adjuvant chemotherapy.
23 yo at 22 wk gestation presented with palpable lesion in the left cheek. She underwent surgical resection at 23 wks gestation with rupture of capsule intra-op.
Post-op MRI revealed possible residual disease and concern for high rate of recurrence given capsule rupture. Decision made to proceed with radiation therapy at 30 wk gestation with shielding of the abdomen/uterus. Patient received 48 Gy (24 of 25 fractions planned) prior to delivery with remainder after delivery. Eight months posttreatment without evidence of recurrent disease.
Planned cesarean delivery at 36 wks. 2800 g newborn male. Newborn healthy at 8 months of age.
25 yo G2P1 at 10 wk 4 d gestation was incidentally noted to have a left maternal renal mass on perinatal ultrasound. MRI revealed a cm mass arising from the left kidney suggestive of renal cell carcinoma.
Tumor completely replaced the left kidney. Well circumscribed neoplasm of round to oval to spindle cells in sheets, fascicles, and perivascular arrangement. IHC TLE-1+, CD56+, CD99+, vimentin+, desmin-, myogenin-, EMA-, CK-, WT1-. Molecular analysis confirmed presence of SYT-SSX2 translocation.
Open left radical nephrectomy. Adjuvant chemotherapy: ifosfamide (1800 mg/m2) and doxorubicin (25 mg/m2) for 3 cycles. Disease free at 3-year follow-up.
33 yo female 5 months pregnant presented with acute onset back pain and was diagnosed with pleural effusion. Thoracentesis revealed frank blood. She deteriorated and required video-thoracoscopic evaluation with evacuation of 2000 cc of blood and multiple tumors attached to the parietal and visceral pleura including one that was bleeding and was resected for hemostasis and diagnosis.
Monophasic cells in plump, spindle-shaped appearance arranged in fascicles and herringbone pattern. Chromosomal translocation t (X; 18) (p11; q11) confirmed by FISH. Final diagnosis monophasic fibrous synovial sarcoma.
Patient refused further treatment after initial emergent surgery.
