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Irreversible Electroporation in a Case of Pancreatic Leiomyosarcoma: a Novel Weapon Versus a Rare Malignancy? Alexandros Papalampros1†, Michail G

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Irreversible Electroporation in a Case of Pancreatic Leiomyosarcoma: a Novel Weapon Versus a Rare Malignancy? Alexandros Papalampros1†, Michail G Papalampros et al. World Journal of Surgical Oncology (2019) 17:6 https://doi.org/10.1186/s12957-018-1553-9 CASE REPORT Open Access Irreversible electroporation in a case of pancreatic leiomyosarcoma: a novel weapon versus a rare malignancy? Alexandros Papalampros1†, Michail G. Vailas1*† , Ioanna Deladetsima2, Demetrios Moris3, Maria Sotiropoulou4, Athanasios Syllaios1, Athanasios Petrou5 and Evangelos Felekouras1 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: Irreversible electroporation, IRE, Pancreatic cancer, Pancreatic leiomyosarcoma Background nature, and 25% of patients suffer from distant metasta- Pancreatic leiomyosarcoma is an extremely rare malig- ses at the time of diagnosis [2]. Median survival is re- nant entity with only few reported cases in the medical ported to be around 48 months with a 5-year associated literature. Therefore, the current evidence on diagnosis survival of about 43.9% [3]. A complete resection is con- and management of these rare tumors is based mostly sidered the sole and gold standard treatment for this on single case reports and small case series. Pancreatic type of malignancy [1]. However, in cases of advanced leiomyosarcomas represent 0.1% of all malignant pancre- and/or metastatic pancreatic leiomyosarcoma, an estab- atic tumors. They seem to be more prevalent in the fifth lished consensus for the management of these tumors decade of life, with nonspecific symptoms at their initial does not exist, whereas efficacy of adjuvant and presentation [1]. The tumor is usually aggressive in neo-adjuvant treatment with chemotherapy and/or radi- ation remains questionable. * Correspondence: [email protected] We describe a case of a 57-year-old woman who pre- †Alexandros Papalampros and Michail G. Vailas contributed equally to this sented in outpatient clinic suffering from advanced pan- work. creatic leiomyosarcoma, causing thrombosis of the 11st Surgical Department, Athens University School of Medicine,“Laiko” General Hospital, Agiou Thoma 17, 11527 Athens, Greece superior mesenteric vein (SMV) and portal vein, along Full list of author information is available at the end of the article © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Papalampros et al. World Journal of Surgical Oncology (2019) 17:6 Page 2 of 6 with multiple liver lesions, which were suspicious for ma- and portal vein infiltration (Fig. 1a, b). The decision was lignancy. Multidisciplinary team decided for upfront to undergo an endoscopic ultrasound (EUS) biopsy in chemotherapy in order to assess tumor response. order to determine the exact nature of the lesion. EUS Follow-up imaging after the completion of chemotherapy report was indicative of pancreatic leiomyosarcoma. led tumor board to decide for subsequent surgical explor- Multidisciplinary team’s decision was to use gemcita- ation. Following that, the patient underwent exploratory bine- and docetaxel-based chemotherapy as up-front laparotomy and irreversible electroporation (IRE) ablation treatment to assess tumor response. Follow-up CT scan of the pancreatic tumor as well as local excision and and magnetic resonance imaging (MRI) after the com- microwave ablation of liver lesions which were suspicious pletion of chemotherapy regimen showed downsizing of for metastasis. To the best of our insight, this is a unique the pancreatic mass, as well as downsizing of suspicious case of pancreatic leiomyosarcoma treated with IRE, a for malignancy segment III liver lesion (Fig. 1c, d). novel and innovative weapon for the treatment of several Based on the response to chemotherapy, tumor char- malignancies at advances stages. acteristics, and physical status of the patient, multidis- ciplinary team’s decision was to proceed to surgical Case presentation exploration. Due to local expansion of the pancreatic A previously healthy 57-year-old woman, with no signifi- tumor, its relation with the superior mesenteric and por- cant past medical history, presented to the surgical de- tal vein, and the underlying SMV thrombosis, excision partment of our hospital for definite management of a of the pancreatic tumor was not feasible. Intraopera- primary pancreatic leiomyosarcoma, after being treated tively, a small piece of tumor was excised in order to be with adjuvant chemotherapy. sent for histopathology. Surgeon’s decision was to ablate One year before her last admission, she was initially the tumor with irreversible electroporation (Fig. 2). admitted to our emergency department due to abdom- Metastatic liver lesions were identified with the use of inal pain, fatigue, and weight loss. She was totally intraoperative ultrasound. Segment III liver lesion was healthy prior to these symptoms. She then underwent resected, while smaller lesions of the right lobe were ab- magnetic resonance imaging (MRI) that was indicative lated using microwave ablation. of a pancreatic head lesion along with possible meta- The patient had an uneventful postoperative recovery static liver lesions, superior mesenteric vein occlusion, and complete resolution of her symptoms. 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 Papalampros et al. World Journal of Surgical Oncology (2019) 17:6 Page 3 of 6 histopathological report, tumor board decided that the patient should be treated with adjuvant therapy for leio- myosarcoma after surgery. A regimen with anthracycline and olaratumab was used for 3 months. Follow-up im- aging in 6 and 12 months showed no progression of the disease (Fig. 5a–d). Discussion It is well known that pancreatic adenocarcinoma is the most common histologic type of malignancy of the pan- creas, representing more than 90–95% of all pancreatic tumors. Pancreatic sarcomas are encountered with a re- ported incidence of about 0.1% of all malignant pancre- atic tumors, with leiomyosarcoma being the most common type reported [1]. Pancreatic leiomyosarcoma, which was first described by Ross in 1951, is an ex- tremely rare tumor with nonspecific clinical and radio- logical features and also poor prognosis [2]. Fig. 2 Application of IRE needles in the lesion of the head of Pancreatic leiomyosarcoma seems to be more preva- the pancreas lent in female patients in the fifth decade of life with a variable size at the time of their presentation between 1 and 30 cm [2]. There is no predilection regarding the Histopathological examination of pancreatic lesion as tumor location (body-tail or head of the pancreas) [1]. It well as segment III liver lesion revealed sarcomatous tis- is considered to originate either from the wall of small sue of high cellularity with fascicular pattern, increased intra-pancreatic vessels or from smooth muscle cells of mitotic activity, and diffuse cytoplasmic immune reactiv- the pancreatic duct [2]. At the time of diagnosis, 25% of ity for SMA, desmin and h-Caldesmon, and chromagen patients suffer from metastatic disease, whereas invasion DAB (Figs. 3 and 4). Surprisingly enough, pathological to adjacent
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