Primitive neuroectodermal tumors of the abdominal wall and vulva in children: Report of two cases and review of the literature

Table 1 Follow-up data of two cases of primitive neuroectodermal tumor patients

Case	Age (mo)	Position	Size (cm)	Therapy	Survival (mo)	Outcome
1	66	Abdominal wall	3.4 × 6.1 × 2.0	S + Ch (VDC/IE)	66+	Survival
2	40	Vulva	3.3 × 5.0 × 2.5	S + Ch (VAC/IE)	14	Death

F: Female; S: Surgery; Ch: Chemotherapy.

Table 2 Thirteen reported cases of primitive neuroectodermal tumor in the abdominal wall

Ref.	Age	Sex	Size (cm)	Immuno-histo-chemistry	Molecular/cyto-genetic analysis	MATOD	Therapy	Follow-up	Relapse	Outcome
Roncati et al[9], 2015	45 yr	M	1.5	NSE, CD99 (+)	FISH (+)	NA	NA	NA	NA	NA
Riccardi et al[18], 2010	15 yr	M	2.5-3.0	CD99, NB84a, vimentin (+)	FISH, RT-PCR (-)	No	S + Ch	NA	NA	NA
Betal et al[19], 2009	61 yr	F	NA	CD99, CD56, cytokeratins, S100 (+)	NA	No	S + Ch (6 cycles of VDC)	NA	No	NA
Taylor et al[8], 2000	33 mo	M	3.5 × 3.5 × 2.5	CD99 (+)	NA	No	S	10 yr	No	Survival
Soma et al[20], 2015	21 yr	F	6 × 4	CD 99, vimentin (+)	NA	No	S + Ch (VDC)	6 mo	No	Survival
Somers et al[21], 2004	16 yr	F	1.5	CD99, CD56, S100 (+)	FISH and RT-PCR (-)	No	Before metastasis: S + Ch (6 cycles of VAC); after metastasis: S + RT + CT (1 cycle of IE + CBP)	NA	Yes	Death
Savić et al[22], 2017	15 yr	M	3.8 × 2.6 × 3.7	CD99, vimentin, synaptophysin (+)	RT-PCR (+)	No	S + Ch (VAC) + RT	8m	No	Survival
Askri et al[23], 2008	35 yr	F	6.5 × 4	CD99 (+)	NA	NA	S + Ch (3 cycles of VDC )	NA	NA	NA
Gurria et al[24], 2011	23 yr	F	14 × 10 × 7	CD99, PAS (+)	NA	No	S + RT + Ch (VAC/IE)	8 mo	No	Survival
Aydinli et al[25], 2009	65 yr	M	5	CD99 (+)	NA	NA	S + Ch (6 cycles of VDCE)	1 yr	No	Survival
Wang et al[26], 2017	21 yr	F	5 × 4	CD99, vimentin, NSE (+)	NA	Yes	S + RT + Ch (VAC)	7 mo	Yes	Death
Zhan et al[27], 2012	2 yr	F	5.0 × 3.8 × 5.1	positive CD99, NSE, Ki67 (+)	NA	No	S + Ch (CTX + ADM + DDP)	1 yr	No	Survival
Present case, 2019	66 mo	F	3.4 × 6.1 × 2	CD99, Syn (+)	NA	No	S + Ch (VDC/IE)	66 mo	No	Survival

+: Positive; MATOD: Metastatic at the time of diagnosis; S: Surgery; Ch: Chemotherapy; RT: Radiotherapy; NA: Not available; FISH: Fluorescence in situ hybridization; RT-PCR: Reverse transcription-PCR; VAC: Vincristine and actinomycin D, cyclophosphamide; IE: Ifosfamide and etoposide; VDC: Vincristine, doxorubicin, and cyclophosphamide.

Table 3 Thirty-seven reported cases of primitive neuroectodermal tumor in the vulva

Ref.	Age	Size (cm)	Immunohistochemistry	Molecular/ cytogenetic analysis	MATOD	Therapy	Follow-up	Relapse	Outcome
Present case, 2019	40 mo	3.3 × 5 × 2.5	Vimentin, CD99 (+)	NA	No	S + Ch (VAC/IE)	14 mo	YES	Death
Pei et al[28], 2018	33 yr	0.5 × 0.5	PAS, CD99, vimentin (+)	EWSR1 gene (+)	Yes	S + RT + Ch	15 mo	NO	Survival
Chiang et al[29], 2017	65 yr	NA	CD99, NSE, SYN, CD56, S100, FLI-1 (+)	FISH (+)	NA	NA	NA	NA	NA
Kakoti et al[30], 2017	16 yr	15 × 10	CK, vimentin, CD99, FLI-1 (+)	NA	No	Ch (VDC/IE)	NA	NA	Death
Tunitsky-Bitton et al[31], 2015	15 yr	5	CD99 (+)	RT-PCR (+)	No	S + Ch (VDC/IE 14 cycles)	29	NO	Survival
Huang et al[32], 2015	20 yr	8 × 10 × 10	CD99, vimentin, NSE (+)	NA	Yes	S	2 wk	NA	Death
Rekhi et al[33], 2015	10 yr	12 × 8	MIC2/CD99, FLI-1(+)	FISH (+)	NA	S + Ch (VIME 5 cycles)	18 mo	YES	Survival
Narayanan et al[34], 2014	17 yr	3 × 2 × 2	MIC2 (+)	NA	NA	S + RT + Ch (VAC/IE)	22 mo	YES	Death
Matsuda et al[35], 2014	60 yr	NA	MIC-2, synaptophysin, NSE, neurofilament antibodies (+)	NA	No	S + RT + Ch (VACI)	48 mo	YES	Survival
Xiao et al[36], 2014	20 yr	NA	CD99, NSE (+)	NA	NA	Not done	NA	NA	Death
Xiao et al[36], 2014	36 yr	NA	CD99, Syn, NSE (+)	NA	NA	S + Ch (PEI, 4 cycles; PAC, 2 cycles)	13 mo	YES	Death
Che et al[37], 2013	37 yr	5 × 3.5 × 3; 3 × 2 × 1.2	CD99, vimentin, FLI-1 (+)	NA	NA	S + Ch (VAC)	12 mo	YES	Survival
Tang et al[38], 2012	17 yr	5.5 × 5 × 5	CD99 and FLI-1 (+)	NA	NA	S	NA	NA	LS
Tang et al[38], 2012	25 yr	2 × 2 × 2	CD99 and FLI-1 (+)	NA	NA	S	NA	NA	LS
Yang et al[39], 2012	20 yr	20 × 10 × 7	CD99 and NSE (+)	RT-PCR (+)	Yes	Ch	NA	NA	Death
Kelling et al[40], 2012	18 yr	1.7 × 9 × 1.5	CD99 and vimentin (+)	RT-PCR (+)	Yes	S + RT + Ch	3 mo	NA	Survival
Anastasiades et al[41], 2012	28 yr	3	CD99 (+)	NA	No	S + RT + Ch	12 mo	YES	Death
Dong et al[42], 2012	20 yr	11 × 7.7 × 6.5	CD99, NSE, CK (AE1/AE3) and Syn (+)	NA	Yes	S	3 mo	NA	Death
Dong et al[42], 2012	12 yr	3.1	CD99, NSE, CK (AE1/AE3) and Syn (+)	NA	Yes	NA	13 mo	NA	Survival
Dong et al[42], 2012	35 yr	NA	CD99 and NSE (+)	NA	Yes	S + Ch	20m	YES	Death
Halil et al[43], 2011	14 yr	NA	CD99 (+)	NA	NA	S + RT + Ch	9 mo	YES	Death
Boldorini et al[44], 2010	52 yr	NA	CD99, CK(AE1/AE3) and vimentin (+)	FISH (+)	No	S + RT + Ch(VAI/IE)	12 mo	NO	Survival
Dadhwal et al[45], 2010	20 yr	20 × 15 × 10	CD99 (+)	NA	Yes	S	20 d	YES	Death
Cetiner et al[46], 2009	23 yr	4 × 4	CD99 and vimentin (+)	RT-PCR (+)	Yes	S + R + Ch (VDC/IE)	7 yr	NO	Survival
Cetiner et al[46], 2009	29 yr	NA	CD99 and vimentin (+)	RT-PCR (-)	NA	S + Ch	51 mo	NO	Survival
Fong et al[47], 2008	17 yr	0.7 × 0.6 × 0.2; 2.1 × 1.7 × 1.5	CD99 and Fli-1 (+)	RT-PCR (+)	No	S + Ch (VDC)	48 mo	NO	Survival
McCluggage et al[48], 2007	19 yr	4	CD99 and FLI-1 (+)	RT-PCR and FISH (-)	NA	S + Ch	NA	NA	NA
McCluggage et al[48], 2007	20 yr	6.5	CD99 and FLI-1 (+)	FISH (+)	NA	S	NA	NA	Death
McCluggage et al[48], 2007	40 yr	3	CD99, FLI-1 (+)	FISH (+)	NA	S + Ch	12 mo	NA	Survival
Moodley et al[49], 2005	26 yr	4 × 5	NA	NA	No	Ch + RT	NA	YES	Death
Takeshima et al[50], 2001	45 yr	4 (at recurrence)	Neuron specific enolase, vimentin, HBA 71 (+)	NA	No	S	1 yr (at recurrence)	YES	Survival
Lazure et al[51], 2001	15 yr	20	CD99 (+)	RT-PCR (+)	NA	S + Ch	7 mo	NO	Survival
Vang et al[52], 2000	28 yr	0.9	CD99 (+)	RT-PCR (+)	NA	S + Ch	18 mo	NA	Survival
Paredes et al[53], 1995	29 yr	5	Vimentin (+)	NA	NA	S + RT + Ch (6 cycles of VAC)	8 mo	NO	Survival
Nirenberg et al[54], 1995	20 yr	12	PAS (+)	NA	NA	S + RT + Ch (VA)	10 mo	YES	Death
Scherr et al[55], 1994	10 yr	6.5 × 5.5 × 2.0	HBA-71 (+)	NA	No	S	NA	NA	NA
Habib et al[56], 1992	23 yr	1.5	CK, EMA (+)	NA	NA	NA	NA	NA	NA

+: Positive; MATOD: Metastatic at the time of diagnosis; S: Surgery; Ch: Chemotherapy; RT: Radiotherapy; NA: Not available; FISH: Fluorescence in situ hybridization; RT-PCR: Reverse transcription-PCR; PEI: Cisplatin, ifosfamide, and etoposide; PAC: Cisplatin, cyclophosphamide, and actinomycin D; VAC: Vincristine, actinomycin D, and cyclophosphamide; IE: Ifosfamide and etoposide; VDC: Vincristine, doxorubicin, and cyclophosphamide; VIDE: Vincristine, ifosfamide, doxorubicin, and etoposide; VIME: Vincristine, ifosfamide, mesna, and etoposide; LS: Loss to follow-up.