Metastatic Malignant Ectomesenchymoma Initially Presenting as a Pelvic Mass: Report of a Case and Review of Literature

Table 1

Review of malignant ectomesenchymoma cases reported after 1998.

Case number	Age^a	Sex	Primary site	Histology^b	Recurrence; or metastasis^a	Treatment^c	Follow-up^a
1 [4]	13 yr.	M	Scrotum	ERMS and GCs	Retroperitoneum Met. after 2 yr.	DS, CT, and RT	NA
2 [5]	10 yr.	F	Intracranial	US with rhabdoid features and NB	Local Rec. after 5 wk.	TSR	NED after 12 mo.
3 [6]	19 mo.	M	Pelvic	ERMS and NB	Local Rec. and BM Met. after 8 yr.	TSR and CT	NED for 8 yr., NA after Met.
4 [7]	11 mo.	M	Intra-abdomen	US with rhabdoid features and NB	Liver, lung, and BM Met. at the time of presentation	DS, CT, and RT	DOD after 9 mo.
5 [8]	61 yr.	M	Retroperitoneum with invasion to vertebral bone	ERMS and GN	No	DS and RT	DOD after 14 mo.
6 [9]	1.5 yr.	M	Upper lip	ERMS and GCs	Local Rec. after 1 yr.	TSR and CT	NED for 1 year, NA after Rec.
7 [10]	4 mo.	M	Pelvic	ERMS, GCs, and schwannoma	NA	TSR and CT	NA
8 [11]	17 mo.	M	Left wrist	RMS, CRS, GNB	No	TSR and CT	NED after 4 yr.
9 [12]	10 yr.	F	Intracranial	ERMS and NB	No	TSR, CT, and RT	NED after 6 yr.
10 [13]	10 d.	F	Face	RMS and GCs	No	Biopsy and CT	DOD, after a few days
11 [14]	4 yr.	F	Intracranial	US with focal rhabdomyoblastic diff. and GCs	Lung Met. at the time of presentation	TSR and CT	DOD after 10 wk.
12 [15]	8 mo.	M	Scrotum	ERMS and GC	NA	TSR and CT	NA
13 [16]	10 yr.	M	Intracranial	US and GCs	No	TSR, CT, and RT	NED after 20 mo.
14 [17]	36 yr.	F	Ethmoid sinus and orbit	RMS and NB	No	Biopsy, CT, and RT	NED after 28 mo.
15 [18]	6 mo.	F	Vagina	ERMS and GCs	Abdomen-pelvic Met. after 4 mo.	DS and CT	DOD after 15 mo.
16 [19]	43 yr.	F	Nasal cavity	RMB and NB	No	Biopsy, CT, and RT	NED after 10 mo.
17 [20]	6 yr.	M	Intracranial, frontal lobe	US and GCs	No	TSR, CT, and RT	NED after 2 years
18 [2]	4 yr.	F	Orbit	ERMS and NB	No	TSR, CT, and RT	NED after 12.9 years
19 [2]	2.5 mo.	F	Upper arm	ARMS and pPNET	No	TSR and CT	NED after 13.7 years
20 [2]	13.5 yr.	M	Buttock	ARMS and NB	Local Rec. and lungs Met. after 1.1 yr.	DS, CT, and RT	DOD after 1.3 years
21 [2]	1 yr.	M	Groin	ERMS and NB	No	TSR and CT	NED after 5 years
22 [2]	7 mo.	F	Sole	ERMS and NB	Local Rec. after 5 mo.	TSR and CT	NED after 2.3 years
23 [2]	8 mo.	M	Intra-abdomen	ERMS and NB	Local Rec. after 1.4 yr.	TSR and CT	NED after 2.1 years
24 [21]	5 mo.	M	Mediastinum with invasion into lung and SVC	RMS and pPNET	No	DS and CT	DOD after 11 mo.
25 (Our case)	6 mo.	M	Inguinal and pelvic	ARMS and NB	Cerebellum Met. after 21 mo.	Biopsy, CT, and RT	NED after 3 yr.

ARMS, alveolar rhabdomyosarcoma; BM, bone marrow; CRS, chondrosarcoma; CT, chemotherapy; diff., differentiation; DOD, dead due to disease; DS, debulking surgery; ERMS, embryonal rhabdomyosarcoma; F, female; GC, ganglion cell; GN, ganglioneuroma; GNB, ganglioneuroblastoma; M, male; Met., metastasis; mo., month(s); NA, no data available; NB, neuroblastoma; NED, no evidence of disease; pPNET, peripheral primitive neuroectodermal tumor; Rec., recurrence; RMB, rhabdomyoblastoma; RMS, rhabdomyosarcoma; RT, radiation therapy; SVC, superior vena cava; TSR, total surgical resection; US, undifferentiated sarcoma; wk, week(s); yr., year(s); ^aage, recurrence/metastasis and follow-up since first diagnosis; ^bitdescribes which tumor components were present in respect to diagnosis of MEM; ^citdescribes type of treatment on the primary tumor.