Frequency, localization, and types of gastrointestinal stromal tumor-associated neoplasia

Table 1 Case reports: Overview of tumor entities

Tumor entity	Ref.
Gastrointestinal Tract
Gastric adenocarcinoma	[29-55]
Colorectal adenocarcinoma	[56-71]
Gastric high grade IEN	[72]
Esophagus SCC	[48,59,73]
Esophagus Small Cell Carcinoma	[39]
Gastro-esophageal junction adenocarcinoma	[74,75]
Duodenum adenocarcinoma	[76]
Papilla Vateri NET	[77]
Gastric Lipoma	[78]
Gastric NET	[79-82]
Doudenum NET	[67]
Ileum NET	[83]
Gastric Schwannoma	[84]
Colon NET	[85]
Jejunal Sarcomatoid Carcinoma	[86]
Pancreas adenosquamous carcinoma	[87,88]
Pancreas adenocarcinoma	[89,90]
Pancreas NET	[91-95]
Hepatocellular carcinoma	[96-99]
Cholangiocellular Carcinoma	[100,101]
Perivascular Epitheloid Cell Tumor Liver	[102]
Urogenital Tract
Renal Cell Carcinoma	[103,104]
Renal Chromophobe Cell Carcinoma	[105]
Prostate Adenocarcinoma	[40,69,82,106,107]
Transitional Cell Carcnimona Bladder	[86]
Female Genitale Tract
Ovarian Carcinoma	[108]
Ovarian Serous Adenocarcinoma	[109,110]
Uterus Leiomyoma	[91]
Breast
Sarcoma	[111]
Invasive Ductal Carcinoma	[55,61,63,92]
Blood/ Lymphatic
Acute Myeloid Leukemia	[112-114]
Acute Biphenotypic Leukemia	[100]
Chronic Lymphoid Leukemia	[115]
Low Grade B Cell Lymphoma Stomach	[116]
Extranodal DLBCL	[117,118]
Burkitt Lymphoma	[119]
Mantle Cell Lymphoma Appendix	[120]
MALT Lymphoma	[50,121]
Multiple Myeloma	[122]
Head and Neck
Oral Melanoma	[123]
Papillary Thyroid Carcinoma	[124]
Lung
Adenocarcinoma	[125-127]
SCC	[107]
NSCLC	[106]
Skin
Melanoma	[128,129]
Squamous Cell Carcinoma	[130]
Merkel Cell Carcinoma	[131]
Soft Tissue
Myxofibrosarcoma	[82]
Dermatofibrosarcoma	[132]
Ewing Sarkoma	[133]
Others
Adrenocortical Carcinoma	[134]
Peritoneal Mesothelioma	[135]
Mesenterial Fibromatosis	[136]
Granular cell tumor	[55]

IEN: Intraepithelial neoplasia; SCC: Squamous-cell carcinoma; NET: Neuroendocrine tumor; DLBCL: Diffuse large B cell lymphoma; MALT: Mucosa associated lymphatic tissue; NSCLC: Non-small cell lung cancer.

Table 2 Retrospective and prospective studies used for this investigation

				Patient characteristics					Organ systems affected by associated NPL
Au-thor	Year	Date of diag-nosis	N	Age average (median)		Sex m/f		Nu-mber (rate) of pati-ents with ass. NPL	GI-tract	Liver/ gall bla-dder/ pan-creas	Uro geni-tal tract	Fem-ale geni-tal tract	Bre-ast	Blo-od/ lymp-hatic sys-tem	Head and neck	Lung	Skin/ mela noma	Soft	Other
				Total pop-ula-tion	Pat. with ass. NPL	Total pop-ula-tion	Pat. with ass. NPL
Ad-im et al[137]	2011	1997-2009	78		62		9/4	13 (17%)	9	2	1		1
Aga-imy et al[14]	2005	1997-2004	97	65	72	42/55	6/12	18 (17%)	6	3	2	3	2	2		2
Agh-das-si et al[138]	2018	1993-2011	104	67		54/50		44 (42%)	16	4	8		7	2				1
Am-aad-our et al[139]	2013	1998-2006	43	62	65	15/28	1/5	6 (14%)	5				1
Fer-nan-dez et al[140]	2018	1999-2016	104		64	62/42	22/10	32 (31%)	9	4	7		3	3	2	2	1	1
Ferr-eira et al[141]	2010	1998-2006	43	62	61	15/28	1/5	6 (14%)	5				1
Giu-liani et al[142]	2012	2002-2010	24	66	69	12/12	5/3	8 (33%)	6		1		1
Gon-alves et al[143]	2010	1998-2008	101		68		5/9	14 (14%)	8		2	2	1						1
Hec-htm-ann et al[144]	2015	2009-2013	260		65	142/118	30/20	50 (19%)	2		21	1	8	8	7	1	3	3	6
Kra-mer et al[17]	2015	2006-2011	836	68	69	423/413	148/119	422 (51%)	118	20	60	22	26	23	6	8	23	2	9
Lai et al[145]	2016	1995-2015	749		68		77/59	136 (18%)	64	27	12	10	7	1	9	13	4	3	10
Lis-zka et al[146]	2007	1989-2006	82		64	38/44	12/10	22 (27%)	17	5				1
Ma-yr et al[15]	2019	1998-2017	188		69			70 (37%)	23	1	17	9	5	5		2	5		3
Mur-phy et al[12]	2015	2001-2011	6112			3252/2860	727/481	1047 (17%)	208	34	432		144	80	54	99	52	48	72
Pan-dur-eng-an et al[147]	2010	1995-2007	783	57		444/339	91/62	153 (20%)	40	8	48	14	15	12	1	10	9	9	20
Ponti et al[148]	2010	1988-2007	141	66	67	77/64	20/26	46 (33%)	18	1	7	2	6	1		2	1	2	3
Ric-hter et al[149]	2008	1993-2005	54	65		28/26		13 (20%)	7		3		1	1					1
Rod-riq-uenz et al[150]	2016	2002-2014	128	65	68	59/69	19/27	46 (34%)	24	5	3	4	2	1	1	3		3	1
Rub-io-Cas-ade-vall et al[151]	2015	1996-2006	132	65		67/53		30 (23%)	8	2	3	1	3			3
Sevi-nc et al[152]	2011	2002-2009	200		67		20/12	32 (16%)	16		5	1	2	1	2			1	4
Smi-th et al[13]	2016	2001-2009	1705	63	69	885/820	95/86	181 (11%)
Vas-sos et al[153]	2014	2000-2009	86	66	70	50/36	27/10	37 (43%)	19	7	5			4	3		3	1
			12050	63	68	1.1 : 1	1.4 : 1	2426 (20,1%)	751 (32%)		706 (30%)		236 (10%)	145 (6%)	85 (4%)	145 (6%)	101 (4%)	74 (3%)	130 (5%)

Table 3 Age and sex distribution

	Total study population	GIST with associated neoplasia	No. of patients available for calculation
Age (median)	63	68	4176/1139
Sex male: Female	1.1:1	1.4:1	10444/2080

GIST: Gastrointestinal stromal tumors.

Table 4 Characteristics of gastrointestinal stromal tumors and associated neoplasias

Parameter	Quantification		No. of patients available for calculation
Designtion of associated NPL, n (%)	Benigne 253 (11)		2248
	Malignant 1995 (89)
Chronological presentation, n (%)	Synchoronous 366 (50)		732
	GIST first 179 (24)
	Ass. NPL first 187 (26)
Histological subtypes of GIST, n (%)	Spindle	Total population 409 (78)	525; 185
		GIST + ass. NPL 149 (80)
	Epitheloid	Total population 43 (8)
		GIST + ass. NPL 11 (6)
	Mixed	Total population 73 (14)
		GIST + ass. NPL 25 (14)

GIST: Gastrointestinal stromal tumors.

Table 5 Distribution of risk scores, n (%)

Risk score	GIST without associated neoplasia	GIST with associated neoplasia	Total
Low and very low	123 (35)	230 (65)	353
Intermediate and high	373 (69)	165 (31)	538
Total	496	395	891

GIST: Gastrointestinal stromal tumors.