Irreversible electroporation in a case of pancreatic leiomyosarcoma: a novel weapon versus a rare malignancy?

Abstract

Background: Primary pancreatic leiomyosarcoma is an extremely rare entity that needs high clinical suspicion in order to diagnose it at an early stage. Clinical characteristics, diagnosis, and management still remain challenging and controversial, especially in advanced stages, when tumor invades adjacent vessels and organs or gives distant metastases.

Case presentation: Herein, we describe a case of a 57-year-old woman suffering from advanced pancreatic leiomyosarcoma with thrombosis of the superior mesenteric vein, as well as liver lesions which were suspicious for metastasis. Multidisciplinary team decided for upfront chemotherapy to assess tumor response. Follow-up imaging after the completion of chemotherapy led tumor board to decide for subsequent surgical exploration. The patient underwent exploratory laparotomy and irreversible electroporation ablation of the pancreatic tumor. Postoperative course was uneventful, and she was discharged 10 days later with a plan to receive adjuvant therapy. To the best of our knowledge, this is the first case of pancreatic leiomyosarcoma ever reported, treated with this novel technique of irreversible electroporation that could be an alternative and feasible way for the management of these rare malignancies.

Conclusions: In conclusion, primary pancreatic leiomyosarcoma is a rare and highly malignant tumor associated with poor prognosis. Nowadays, R0 surgical resection remains the cornerstone treatment, combined with adjuvant and/or neoadjuvant chemotherapy prior to resection. In the advanced setting, when major vessel invasion and distant metastases occur, chemotherapy along with irreversible electroporation ablation could be a helpful and possibly effective modality for the management of this highly aggressive tumor.

Keywords: IRE; Irreversible electroporation; Pancreatic cancer; Pancreatic leiomyosarcoma.

Fig. 1

a MRI showing suspicious lesion in segment III of the liver prior to chemotherapy. b MRI showing pancreatic mass along with thrombosis of SMV prior to chemotherapy. c CT showing downsizing and minimal response of the segment III liver lesion after chemotherapy. d CT showing downsizing and minimal response of pancreatic mass after chemotherapy

Fig. 2

Application of IRE needles in the lesion of the head of the pancreas

Fig. 3

Extensive regression changes with nodular sarcoma remnants. Liver tissue is recognizable at the periphery (arrow), (H-E × 20)

Fig. 4

Sarcomatous tissue of high cellularity with fascicular pattern. Multinucleated giant cells can occasionally be seen (H-E × 100). Diffuse cytoplasmic immune reactivity for SMA, desmin and h-Caldesmon, and chromagen DAB (× 200)

Fig. 5

Follow-up CT scan. a Site of segment III resection. No new liver lesions detected 6 months after the operation. b Pancreatic head lesion after IRE with no obvious changes or progression of disease 6 months after the operation. c Twelve months after the operation, the liver remains free of new lesions. d Twelve months after the operation, the pancreatic mass shows no progression or enlargement