Primary Pancreatic Leiomyosarcoma Research Articles

Pancreatic leiomyosarcoma: a case report

Primary pancreatic leiomyosarcoma belongs to a rare group of malignant soft tissue tumors. we present a clinical case of a 61-year-old patient who underwent hemipancreatectomy with splenectomy for a pancreatic tumor in 2018. Histological and immunohistochemical studies confirmed the diagnosis of leiomyosarcoma. for three years, the patient has been observed at the N.N. Blokhin National Medical Research Center of Oncology of ministry of Health of Russia without signs of progression.

S1693 A Case of Primary Pancreatic Leiomyosarcoma: An EUS Diagnosis

S1693 A Case of Primary Pancreatic Leiomyosarcoma: An EUS Diagnosis

Leiomiosarcoma primario de páncreas. A propósito de un caso

Primary Pancreatic Leiomyosarcoma is a rare tumour. It probably originates from the smooth muscle of the pancreatic ducts or the small pancreatic vessels. Given its rarity and the scarce published cases, only few data are available regarding its epidemiological characteristics, evolution and therapeutic strategies. Common elements of the previously published cases were poor prognosis and aggressive course with early haematogenous metastasis. Case report. A 61-year-old man presented with subacute abdominal pain and weight loss. Abdominal computerized tomography showed a large solid mass that encompasses the body and head of the pancreas in addition to multiple solid nodular lesions in the liver compatible with metastases. Pathology report. Primary pancreatic leiomyosarcoma, immunologically-stained positive for caldesmon, smooth muscle actin. Chemotherapy was given with initial improvement. The patient survived for 27 months. Conclusion. The rarity and the ominous course of primary pancreatic leiomyosarcoma impose great medical challenge. It also lacks specific clinical and imaging characteristics and evidence-based treatment strategy. The immunohistochemically diagnosis proves to be essential, due to the important differential diagnosis. Its ominous prognosis corresponds to its aggressive course and early haematogenous metastases.

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

BackgroundPrimary 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 presentationHerein, 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.ConclusionsIn 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.

Primary leiomyosarcoma of the pancreas: report of a case treated by local excision and review of the literature.

BackgroundFirst described by Ross in 1951, primary pancreatic leiomyosarcoma is a rare mesenchymal tumour of the pancreas, with nonspecific clinical and radiological features and a poor prognosis, if unresectable.Case reportA 60-year-old woman presented with abdominal pain. Magnetic resonance imaging (MRI) and computed tomography (CT) scan detected a dishomogeneous egg-shaped 8-cm mass, arising from the pancreatic head, with duodenal compression, without dilation of the Wirsung duct. 18F-FDG positron-emission tomography (PET)-CT showed a moderate tracer uptake, and the endoscopic ultrasound (US) showed a hypoechoic lesion, arising from the duodenal wall, suspected to be a gastrointestinal stromal tumour (GIST). CEA, CA19-9, NSE, and chromogranin A were normal. At the surgical exploration, a 10-cm mass, adherent to the anterior aspect of the pancreatic head, was found. The lesion was easily separable from the duodenal wall and was totally excised. The frozen intraoperative examination showed a mesenchymal tumour, with spindle-shaped cells, suggesting that a GIST diagnosis was likely. Postoperative course was uneventful. Histology and immunohistochemistry demonstrated a well-differentiated leiomyosarcoma, with five to six mitotic counts per 10 high-power field (HPF) and proliferative index (MIB-1) 10 % (grade 2 according to Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC)), with positive smooth muscle actin, desmin, and caldesmon but negative CD117 (c-kit) and S-100. The patient is alive and asymptomatic 19 months after surgery, without evidences of disease.ConclusionsIn the English literature, only 44 cases of primary pancreatic leiomyosarcoma have been reported. If a pancreatic mass suspected for primary pancreatic leiomyosarcoma has no adjacent organ/vessel invasion or distant metastases, surgical resection is the therapy of choice.

Sarcomas of the scalp: A comparison of two lesions

Sarcomas of the scalp: A comparison of two lesions

Primary Pancreatic Leiomyosarcoma: A Retrospective Analysis of Clinical Characteristics and Prognosis of this Rare Disease

Primary pancreatic leiomyoarcoma (PLMS) is a rare disease; its clinical characters and prognosis are poorly demonstrated. This study aimed to investigate the tumor's clinical characters and to reveal the true outcome. A retrospective review of both Chinese and worldwide PLMS patients were performed. Data was collected and analyzed and overall survival was described by Kaplan-Meier curve. In total, there were 9 cases identified in China and 55 cases identified in the worldwide literature. Incidence rate was general equal in gender and had an increased trend with age(mean +SD: 55+14 years). Abdominal pain, mass and weight loss were the most common presentations, mean tumor size was 10cm (SD 7.2cm) and most of them presented as a solid mass. Forty six cases accepted operation, 33 of which obtained a radical resection. Median overall survival time was 27 months.The patients who accepted radical resection had an obviously improved prognosis over non-resectable patients. PLMS commonly occurred in the mid-age people and there was lack of specific non-invasive methods for precise preoperative diagnosis.PLMS has a much better prognosis compared with pancreatic ductal adenocarcinoma. Radical resection is the only hope for improving the outcome of this malignancy.

Primary Pancreatic Pleomorphic Leiomyosarcoma

Primary pancreatic leiomyosarcoma is a rare mesenchymal tumour that is believed to arise from the walls of the pancreatic blood vessels or the pancreatic duct. A 56-year-old female was referred with epigastric pain and abdominal mass. Preoperative computed tomography showed a large soft tissue mass in the pancreatic body and tail. Fine needle aspiration biopsy indicated a spindle cell type tumour. The patient received distal pancreatectomy with no adjuvant treatment. Histology revealed a pleomorphic spindle cell neoplasm with an immunoprofile suggestive of smooth muscle origin. The absence of other lesions in the body was consistent with the diagnosis of primary pleomorphic leiomyosarcoma. The patient was well and tumour-free 14 months after surgery. Detailed immunohistochemical analyses are necessary in the diagnosis of this highly malignant tumour. Radical resection offers the only chance of long-term survival.

Leiomyosarcoma Arising in the Pancreatic Duct: A Case Report and Review of the Current Literature

Context. Leiomyosarcomas are rare malignant smooth muscle tumors that may arise in any organ or tissue that contains smooth muscle, commonly within the gastrointestinal tract. They are most often found in the stomach, large and small intestines, and retroperitoneum. Primary pancreatic leiomyosarcoma is extremely rare, and to the best of our knowledge only 30 cases have been reported in the world literature since 1951. Our case represents the first to have a clear origin from the main pancreatic duct. Case Report. This case was diagnosed in a large, tertiary care center in Tampa, Florida. Pertinent information was obtained from chart review and interdepartmental collaboration. A mass in the tail of the pancreas was identified with large pleomorphic and spindle-shaped cells. Immunohistochemistry for vimentin, smooth muscle actin, and desmin was positive. All remaining immunohistochemical markers performed were negative. The tumor clearly originated from the pancreatic duct wall, filled and expanded the duct lumen, and was covered with a layer of benign biliary epithelium. Conclusion. Leiomyosarcoma of the pancreas is an extremely rare malignancy with few reported cases in the literature. The prognosis is poor, and treatment consists of alleviating symptoms and pain management. To our knowledge, this represents the first reported case demonstrating clear origin of a leiomyosarcoma from the pancreatic duct.

Primary pancreatic leiomyosarcoma: a case report

BackgroundLeiomyosarcoma are malignant tumours of the soft tissues. It is most commonly found in the stomach, small intestine and retroperitoneum. Primary pancreatic leiomyosarcoma is extremely rare and only 22 cases have been reported in the literature since 1951. Its prognosis is poor and the palliative treatment is only aimed at symptomatic improvement.Case presentationWe report case of primary pancreatic leiomyosarcoma with liver metastases in a 73 year old man who presented with weight loss, epigastric pain, anorexia, abdominal fullness and obstructive jaundice. Histological examination and Immunohistochemical analysis of liver biopsy specimen confirmed the diagnosis of metastatic leiomyosarcoma. The patient died of disease 3 months after the initial diagnosis.ConclusionLeiomyosarcoma of the pancreas is an extremely rare malignancy with a poor prognosis. Treatment is aimed at symptomatic improvement and pain management.