Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

Gatt, Raphael; Casingena, Luca; Pisani, David; Agius, Rachel; Cassar, Noel

doi:10.1186/s40792-023-01734-4

Surgical Case Reports

Table 1 Demographics, treatment modality, spleen size and initial adjuvant chemoradiotherapy response in all 41 cases identified within literature

From: Undifferentiated pleomorphic sarcoma of the spleen: a case report and literature review

First author	Year of publication	Age	Gender	Distant metastasis at diagnosis	Surgical management	Chemoradiotherapy	Spleen size	Initial response using RECIST evaluation
Govoni [10]	1982	51	F	No	Open splenectomy	n/a	21 × 25 × 10.5 cm	n/a
Wick [11] (Case No.1)	1982	48	M	Yes—liver	Open splenectomy	Unspecified adjuvant chemotherapy	N/S	n/s
Wick [11] (Case No.2)		51	F	No	Open splenectomy	n/a	N/S	n/a
Wick [11] (case No.3)		54	M	No	Open splenectomy	n/a	N/S	n/a
Jinno [12]	1987	53	M	Yes—liver, vertebrae, peritoneum	n/a	n/a	N/S	n/a
Bruneton [13]	1988	54	M	No	Open partial splenectomy	n/a	N/S	n/a
Sieber [14]	1990	41	M	Yes—omentum	Open splenectomy + omentectomy	Unspecified adjuvant chemoradiotherapy	28 × 17 × 12 cm	Progressive disease
Bonilla [15]	1994	42	F	No	Open splenectomy Surgical removal of lesions after recurrence	Adjuvant radiotherapy 5,300 cGy over surgical field + doxorubicin × 4 cycles	N/S	Progressive disease
Kimura [16] (Case No.1)	1998	29	M	No	Open splenectomy	Unspecified adjuvant chemoradiotherapy	17 × 13 × 8 cm	Progressive disease
Kimura [16] (Case No. 2)	1998	60	M	No	Open splenectomy	Adjuvant methylprednisolone Adjuvant cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) × 2 cycles Adjuvant mitoxantrone, etoposide, carboplatin and prednisolone (MECP) × 1 cycle Adjuvant oral etoposide	8 × 8 × 8 cm	Progressive disease
Kimura [16] (Case No.3)	1998	66	F	No	Open splenectomy	Neoadjuvant prednisolone and gamma-globulins + spleen radiotherapy Adjuvant prednisolone + severe courses of different chemotherapy	N/S	Progressive disease
Mallipudi [17]	1998	73	F	No	Open splenectomy	n/a	15 × 10 × 8 cm	n/a
Gong [18]	1999	34	M	Yes—liver, omentum	Open splenectomy, distal pancreatectomy, retroperitoneal lymphadenectomy & liver biopsy	Adjuvant adriamycin × 3 days	20 × 16 × 10 cm	Progressive disease
Colovic [19]	2001	45	F	Yes—liver	Open splenectomy + liver biopsy	Adjuvant cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) × 6 cycles	14 × 12 × 7 cm	Progressive disease^a
Agha [20]	2002	57	F	No	Open splenectomy	n/a	10 × 10.7 × 9.5 cm	n/a
Audouin [21]	2003	71	F	No	Open splenectomy	Unspecified adjuvant chemotherapy	14 × 12 × 10 cm	Progressive disease^b
Ozaras [22]	2003	51	F	No	Open splenectomy	n/a	N/S	n/a
Moloney [23]	2004	19	F	No	Emergency open splenectomy	Adjuvant doxorubicin and ifosfamide × 2 cycles	22 × 17 × 13 cm	Progressive disease
Katsuura [24]	2006	82	M	No	Open splenectomy	n/a	13 cm maximal diameter	n/a
Low [25]	2006	63	F	Yes—liver, lung, myocardial	n/a	n/a	N/S	n/a
Koboyashi [26]	2008	82	F	Yes—liver	n/a	Palliative radiotherapy—10 Gy in 10 fractions to the spleen over 2 weeks	19 × 13 × 9 cm	Progressive disease
Oka [27]	2008	58	F	No	Open splenectomy	n/a	14.5 × 8.5 × 9.5 cm	Progressive disease
Hashmi [7]	2010	76	M	No	Laparoscopic splenectomy	n/a—patient refused adjuvant treatment	N/S	n/a
Amatya [28]	2011	77	M	Yes—renal, adrenal, femur	n/a	n/a	N/S	n/a
Ji-Feng [29]	2011	48	F	No	Laparoscopic splenectomy + fenestration of hepatic cysts	n/a	11 × 10 × 8 cm	n/a
Liang He [30]	2011	35	M	No	Emergency open splenectomy	n/a	13 × 11 × 8 cm	n/a
Gupta [31]	2012	30	M	No	Open splenectomy	n/a—patient failed to turn up after hospital discharge	15 × 12 × 6 cm	n/a
Yamada [32]	2012	67	F	No	Open splenectomy	n/a	14.5 × 12 × 8 cm	n/a
Yamamoto [33]	2012	69	M	Yes—liver	Open splenectomy	Neoadjuvant paclitaxel, carboplatin and etoposide × 2 cycles Adjuvant mitomycin C and etoposide via hepatic arterial infusion × 6 cycles	N/S	Complete response
Das [34]	2013	30	M	Yes—liver	Open splenectomy + deroofing of hepatic cysts + liver biopsy	n/a	N/S	n/a
Rakic [35]	2013	57	M	No	Laparotomy—removal of large solitary mass in left upper abdomen, partial gastrectomy, partial resection of left crus of diaphragm, distal pancreatectomy, left hemicolectomy	n/a	N/S	n/a
Yamamoto [33]	2013	81	F	No	Laparoscopic splenectomy	n/a	21 × 12 × 5 cm	n/a
Batra [36]	2016	17	M	No	Open splenectomy	Adjuvant cyclophosphamide, doxorubicin, vincristine, prednisolone (CHOP) × 3 cycles Adjuvant ifosfamide, carboplatin and etoposide (ICE) × 3 cycles Autologous stem cell transplant using carmustine, etoposide, cytarabine and melphalan (BEAM)	24 × 14 × 11 cm	Complete response
Makis [37]	2017	63	M	Yes—liver	n/a	n/a	N/S	n/a
Mantas [9]	2016	66	F	No	Open splenectomy	n/a	18 × 13 × 10 cm	n/a
Ashmoore [38]	2020	56	F	No	Open splenectomy	n/a	15 cm maximal diameter	n/a
Huang [39]	2020	40	F	No	Laparoscopic splenectomy	Neoadjuvant prednisolone	12 × 8 × 3.6 cm	n/a
Darwish [40]	2022	49	n/s	Yes—lung	Delayed elective open splenectomy	Adjuvant doxorubicin and ifosfamide × 3 cycles	16 cm (craniocaudal)	Progressive disease
Koboyashi [41]	2022	61	F	No	Open splenectomy	n/a	19 × 13 × 9 cm	n/a
Luo [44]	2022	60	F	No	Laparoscopic splenectomy	Adjuvant oral imatinib	N/S	Complete response
Wijebandara [42]	2022	52	F	No	Open splenectomy	n/a	N/S	n/a

^aInitial good response not considered as it was based on ultrasound findings
^b“Good initial clinical results” not considered as it was not based on any imaging or tumour markers

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