E0078 - Primary Pancreatic Undifferentiated Pleomorphic Sarcoma

Tuesday, October 25, 2022

3:00 PM – 5:00 PM ET

Location: Crown Ballroom

Has Audio

Presenting Author(s)

Mehrie Harshad Patel, MBBS

Mayo Clinic
Rochester, MN

Mehrie Harshad Patel, MBBS¹, Upasana Agrawal, MBBS², Daniela Guerrero Vinsard, MD¹, Santhi Swaroop Vege, MD¹, Seth Sweetser, MD¹
¹Mayo Clinic, Rochester, MN; ²LSU Health Sciences Center, Shreveport, LA

Introduction: Primary pancreatic sarcomas are rare malignancies with an incidence of 0.1%[1, 2]. Sarcomas are frequently seen in the surrounding pancreatic tissue, hence it’s important to differentiate primary pancreatic sarcomas from other peripancreatic tumors[3]. We report a case of high-grade primary undifferentiated pleomorphic sarcoma (UPS) of pancreas.

Case Description/Methods: A 48-year-old man presented with acute lower abdominal pain and weight loss. He had a family history of colorectal cancer. He was tachycardic on presentation and physical examination revealed tenderness in LLQ. Laboratory studies showed a mild anemia and a white blood cell count of 13.3/mm^3. Liver panel and CA 19-9 level were normal. Abdominal CT scan revealed a 6.8 cm complex solid-cystic pancreatic lesion (Figure1A). Abdominal MRI showed a 9.2 cm macrolobulated mass involving the pancreas without local extension or metastasis. PET CT showed large fluorodeoxyglucose (FDG) avid centrally necrotic pancreatic mass without metastasis. Subsequent endoscopic ultrasound revealed a 6.5 cm solid-cystic mass in the pancreatic body with no vascular involvement and fine needle biopsies revealed malignant spindle cells. Immunohistochemistry (IHC) failed to characterize lineage and differential included carcinoma or sarcoma. He underwent distal pancreatectomy and splenectomy (Figure 1B). Intraoperative frozen section showed epithelioid and spindled malignancy with IHC negative for EMA, broad spectrum keratins (AE1/AE3 and OSCAR), and high molecular weight keratins. S-100 and other tumor markers were negative. Final pathology revealed high grade undifferentiated pleomorphic sarcoma measuring 10.5 cm with negative margins (Figure 1C&D). He underwent adjuvant chemotherapy with 4 cycles of doxorubicin/ifosfamide. Surveillance with CT chest/abdomen/pelvis every 3 months was recommended. Follow-up imaging was unremarkable.

Discussion: This case highlights a rare case of primary pancreatic UPS in a middle-aged man. A search of literature revealed 16 cases of UPS with a similar clinical presentation (Table 1). A step-wise approach with cross-sectional imaging and biopsy is important to appropriately manage these rare malignancies.

Baylor,S.M.andJ.W. Berg,Cross‐classification and survival characteristics of 5,000 cases of cancer of the pancreas.1973.p.335-358.

Ambe,P.,et al., Primary sarcoma of the pancreas, a rare histopathological entity.A case report with review of literature.2011.p.85

Varela,A.B.,et al.,Primary pancreatic sarcoma.2009.p.285-287.

Figure: Figure 1:
A: CT Scan
B: Post pancreatectomy and splenectomy
C: Undifferentiated pleomorphic sarcoma (UPS) of the pancreas, showing extension into peripancreatic adipose tissue (H+E, x100)
D: Higher power view, showing highly malignant-appearing, pleomorphic spindled cells and an atypical mitotic figure (center) (H+E, x200)

Author	Age	Sex	Histologic type	Location	Treatment	Preoperative diagnosis	Postoperative therapy	Follow-up (months)
Ishiguchi, et al.	44	M	Pleomorphic	Body-tail	Left pancreatectomy, splenectomy	Pancreatic neoplasms	NA	15, NED
Garvey, et al.	77	M	Storiform-pleomorphic	Uncinate lobe	Enucleation	Pancreatic head mass	NA	48, NED
Pascal, et al.	39	M	Storiform-pleomorphic	Head	Pancreaticoduodenectomy	Mesenchymal tumor	NA	0, DOC
Allen, et al.	46	M	Storiform-pleomorphic	Body-tail, local invasion	Pancreatectomy, splenectomy, subtotal gastrectomy	NA	Chemotherapy	5, DOD
Tsujimura, et al.	43	F	Storiform-pleomorphic	Tail	Pancreatectomy, splenectomy	Pancreatic cystadenoma	Chemotherapy	5, NED
Ben Jilani, et al.	72	M	Storiform-pleomorphic	Body-tail	Left pancreatectomy, splenectomy	Pancreatic mass	NA	12, DOD
Balen, et al.	37	M	Pleomorphic	Body-tail	Extended left pancreatectomy	Pancreatic mass	Radiotherapy and chemotherapy	7, DOD
Haba, et al.	70	M	Storiform-pleomorphic	Head	Pancreaticoduodenectomy	Tumor of pancreatic head	Chemotherapy	22, NED
Bastian, et al.	67	M	Storiform-pleomorphic	Body	Left pancreatectomy, splenectomy, transverse colectomy, subtotal gastrectomy	Pancreatic cancer	NA	34, NED
Darvishian, et al.	74	M	Storiform-pleomorphic	Head	Pancreaticoduodenectomy	Pancreatic head cancer	NA	4, NED
Akatsu, et al.	67	M	Storiform-pleomorphic	Body-tail	Left pancreatectomy, splenectomy, transverse colectomy, total gastrectomy	Pancreatic cancer	NA	35, NED
Mizukami, et al.	44	F	Pleomorphic	Body-tail	Total gastrectomy, left pancreatectomy	Pancreatic tumor	NA	20, NED
Yu, et al.	67	M	Storiform-pleomorphic	Head	Pancreaticoduodenectomy	Pancreatic head cyst	NA	11, DOD
Jarry, et al.	45	M	Storiform-pleomorphic	Head	Pancreaticoduodenectomy	Pancreatic cancer	Radiotherapy and chemotherapy	36, NED
Sanei, et al.	72	F	Pleomorphic	Head and neck	Pancreaticoduodenectomy	Pancreatic head cancer	NA	22, NED
Liang, et al.	37	F	Pleomorphic	Body-tail	Distal pancreatectomy	Pancreatic mass	NA	12, DOD
Own case	48	M	Pleomorphic	Body	Pancreatectomy with splenectomy	Pancreatic mass	Chemotherapy	12, NED

Table: Table 1. Summary of all Undifferentiated Pleomorphic Sarcoma of Pancreas report to date (references of the table will be displayed in the poster due to the abstract character count limit)

Disclosures:

Mehrie Harshad Patel indicated no relevant financial relationships.

Upasana Agrawal indicated no relevant financial relationships.

Daniela Guerrero Vinsard indicated no relevant financial relationships.

Santhi Swaroop Vege indicated no relevant financial relationships.

Seth Sweetser indicated no relevant financial relationships.

Mehrie Harshad Patel, MBBS¹, Upasana Agrawal, MBBS², Daniela Guerrero Vinsard, MD¹, Santhi Swaroop Vege, MD¹, Seth Sweetser, MD¹. E0078 - Primary Pancreatic Undifferentiated Pleomorphic Sarcoma, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.