Primary Intraosseous Synovial Sarcoma with Molecular Confirmation: Expanding and Clarifying the Spectrum of This Rare Neoplasm

Table 1

Reported cases of primary intraosseous synovial sarcoma with molecular confirmation of the diagnosis.

Author	Year	No. cases	Age	Sex	Anatomic site	Extraosseous component	Morphology	Molecular confirmation	Clinical management	Clinical follow-up
Cohen et al.	1997	1	22	M	Proximal tibia	Yes	Biphasic	FISH	NA	NA
Hiraga et al.	1999	1	67	M	Distal radius	No	Monophasic	RT-PCR	Neoadjuvant chemotherapy; wide resection	Pulmonary metastases 8 months postoperative; metastases in radius treated with XRT; NED 2.5 years
O’Donnell et al.	2006	1	37	M	Proximal ulna	Yes	Monophasic	RT-PCR	Amputation	NED 1 year
Jung et al.	2007	1	21	F	Distal tibia	Yes	Monophasic	RT-PCR	Neoadjuvant chemotherapy	NA
Verbeke et al.	2010	1	73	F	Fibula	No	Monophasic	FISH	Amputation	Pulmonary metastases after 0.25 years; ultimately DOD
Beck et al.	2011	1	53	M	Proximal tibia	Yes	Biphasic	FISH	Proximal tibial resection; adjuvant chemotherapy	Local recurrence at 3 years; AKA & chemotherapy; NED 8 months
Cao et al.	2014	1	26	M	Thoracic spine	Yes	Biphasic	RT-PCR	En bloc resection; adjuvant chemotherapy & XRT	NED 1 year
Fujibuchi et al.	2019	1	77	F	Distal ulna	No	Monophasic	RT-PCR	Wide resection	NED 2 years
Horvai et al.	2019	2	33	M	Tibia diaphysis	No	Monophasic	FISH	Curettage	Local recurrence & metastasis to inguinal lymph node; adjuvant chemotherapy; NED 7 years
			36	M	Tibia diaphysis	No	Monophasic	FISH	En bloc resection	Local recurrence; NED 8 years
Caracciolo et al.	2019	1	33	M	Proximal femur	Yes	Monophasic	RT-PCR	Pulmonary metastases at initial presentation; neoadjuvant chemotherapy; proximal femoral resection	Progressive pulmonary metastases; adjuvant chemotherapy; no additional follow-up provided
McHugh et al.	2019	2	45	F	Humerus	No	Monophasic	FISH	Proximal humeral resection; adjuvant chemotherapy	NED 2 months
			36	M	Metatarsal	Yes	Monophasic	FISH	NA	NA

Total cases: 13. Poorly differentiated (the remainder of the monophasic tumors is of the spindle cell/fibrous type). Abbreviations: M: male; F: female; FISH: fluorescent in situ hybridization; RT-PCR: reverse-transcription polymerase chain reaction; NA: not applicable (not reported); XRT: radiation therapy; NED: no evidence of disease; DOD: dead of disease.