BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare malignant tumor and has non-specific clinical manifestations and imaging characteristics,making preoperative diagnosis challenging.Here,we report a case of PHL p...BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare malignant tumor and has non-specific clinical manifestations and imaging characteristics,making preoperative diagnosis challenging.Here,we report a case of PHL presenting primarily with fever,with computed tomography imaging showing a thick-walled hepatic lesion with low-density areas,resembling liver abscess.CASE SUMMARY The patient was a 34-year-old woman who presented with right upper abdominal pain and fever over 4 days before admission.Based on the patient’s medical history,laboratory examinations,and imaging examinations,liver abscess was suspected.Mesenchymal tumor was diagnosed by percutaneous liverbiopsy and partial hepatectomy was performed.Postoperative pathology revealed PHL.The patient is currently undergoing intravenous chemotherapy with the AD regimen and shows no signs of recurrence.CONCLUSION When there is a thick wall and rich blood supply in the hepatic lesion with a large proportion of uneven low-density areas,PHL should be considered.展开更多
BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphi...BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.展开更多
BACKGROUND Leiomyosarcoma(LMS)has a poor prognosis and rarely originates from the colon.If resection is possible,surgery is the first treatment most commonly considered.Unfortunately,no standard treatment exists for h...BACKGROUND Leiomyosarcoma(LMS)has a poor prognosis and rarely originates from the colon.If resection is possible,surgery is the first treatment most commonly considered.Unfortunately,no standard treatment exists for hepatic metastasis of LMS;although,several treatments,such as chemotherapy,radiotherapy,and surgery,have been used.Subsequently,the management of liver metastases remains controversial.CASE SUMMARY We present a rare case of metachronous liver metastasis in a patient with LMS originating from the descending colon.A 38-year-old man initially reported abdominal pain and diarrhea over the previous two months.Colonoscopy revealed a 4-cm diameter mass in the descending colon,40 cm from the anal verge.Computed tomography revealed intussusception of the descending colon due to the 4-cm mass.The patient underwent a left hemicolectomy.Immunohistochemical analysis of the tumor revealed that it was positive for smooth muscle actin and desmin,and negative for cluster of differentiation 34(CD34),CD117,and discovered on gastrointestinal stromal tumor(GIST)-1,which are characteristic of gastrointestinal LMS.A single liver metastasis developed 11 mo postoperatively;the patient subsequently underwent curative resection thereof.The patient remained disease-free after six cycles of adjuvant chemotherapy(doxorubicin and ifosfamide),and 40 and 52 mo after liver resection and primary surgery,respectively.Similar cases were obtained from a search of Embase,PubMed,MEDLINE,and Google Scholar.CONCLUSION Early diagnosis and surgical resection may be the only potential curative options for liver metastasis of gastrointestinal LMS.展开更多
BACKGROUND Leiomyosarcoma is a subtype of soft tissue sarcoma with adverse outcomes.Leiomyosarcoma accounts for nearly 70%of all uterine sarcomas and is responsible for a considerable proportion of deaths because of u...BACKGROUND Leiomyosarcoma is a subtype of soft tissue sarcoma with adverse outcomes.Leiomyosarcoma accounts for nearly 70%of all uterine sarcomas and is responsible for a considerable proportion of deaths because of uterine cancer.Clinical characteristics and relevant diagnosis of pelvic leiomyosarcoma should be further explored.AIM To identify the outcome and relevant perioperative evaluation of patients with pelvic leiomyosarcoma.METHODS The Kaplan-Meier method was used to determine progression-free survival and overall survival rates.Factors predictive of outcomes were identified using univariate and multivariate Cox proportional hazards models.RESULTS Fifty-one patients with pelvic leiomyosarcoma were enrolled and divided into two groups including uterine leiomyosarcoma and non-uterine leiomyosarcoma.Overall,28.6%and 45.5%of uterine leiomyosarcoma and non-uterine leiomyosarcoma patients,respectively,had elevated carbohydrate antigen 125 levels,whereas 45.7%and 68.8%,respectively,underwent ultrasonography.Although 68.8%of uterine leiomyosarcoma patients were initially diagnosed with hysteromyoma,72.7%of non-uterine leiomyosarcoma patients had pelvic and abdominal masses.Moreover,93.3%of the recurrent lesions were detected using ultrasonography.Patients with International Federation of Gynaecology and Obstetrics(FIGO)stages III–IV disease had poorer progression-free survival values than those with FIGO stages I–II(P=0.027)disease.FIGO stage was significantly associated with poor progression-free survival in the univariate(hazard ratio=2.64,P=0.03)and multivariate(hazard ratio=2.49,P=0.048)analyses.CONCLUSION Serum tumour biomarkers cannot be used for pelvic leiomyosarcoma diagnosis.FIGO stage is critical to predict the outcome of uterine leiomyosarcoma.Ultrasonography is more reliable for postoperative follow-up than preoperative diagnosis.展开更多
Primary hepatic leiomyosarcoma are rare tumors with less than 30 cases reported in the English literature.Non specific presentations and often diagnosis delayed until they reach a large size,is the norm with therapy l...Primary hepatic leiomyosarcoma are rare tumors with less than 30 cases reported in the English literature.Non specific presentations and often diagnosis delayed until they reach a large size,is the norm with therapy leading to an often dismal prognosis.A 67-year-old man presented complaining of abdominal pain and a palpable abdominal mass since Jan 2010.Abdominal ultrasonography and abdominal computed tomography revealed a large tumor in the left lobe of the liver.Surgical exploration was undertaken and an extended left hepatectomy with extension onto the dorsal part of segment 8 preserving the MHV with partial resection of segment 6 was undertaken.The weight of the resected specimen was 1300 g of the left lobectomy specimen and 8 g of the segment 6 partial resection specimen.The pathology report confirmed the diagnosis of leiomyosarcoma.On immunohistochemistry,the tumor cells were positive for smooth muscle actin stain.The patient is on regular follow up and is currently 9 mo post resection with no evidence of recurrence.We report the case of a resected primary hepatic leiomyosarcoma and emphasize the need for a global database for these rare tumors to promote a better and broader understanding of this less understood subject.展开更多
Spermatic cord leiomyosarcomas (LMSs) are rare tumors which may cause significant morbidity and mortality if inadequately diagnosed or treated. We report a case of a paratesticular LMS in a 60-year-old man who present...Spermatic cord leiomyosarcomas (LMSs) are rare tumors which may cause significant morbidity and mortality if inadequately diagnosed or treated. We report a case of a paratesticular LMS in a 60-year-old man who presented with a right scrotal mass. The patient was evaluated by scrotal ultrasound and computed tomography of the abdomen and pelvis (including scans of the scrotum), which revealed a large extratesticular mass. The lesion proved to be malignant and the patient underwent radical orchiectomy with high cord ligation. To improve the assignment of this lesion, we further analyze the imaging features of LMS and corre-late them with pathologic findings.展开更多
BACKGROUND Primary hepatic leiomyosarcoma is rare and reported sporadically, with less than40 such cases have been reported in the English-language literature. Although it is reported to be associated with acquired im...BACKGROUND Primary hepatic leiomyosarcoma is rare and reported sporadically, with less than40 such cases have been reported in the English-language literature. Although it is reported to be associated with acquired immune deficiency syndrome, EpsteinBarr virus infection, Hodgkin's lymphoma, immunosuppression after organ transplantation, and hepatitis C virus-related liver cirrhosis, the precise steps leading to leiomyosarcoma have not been fully identified. Therapeutic strategies include liver wedge resection or lobectomy, chemotherapy, radiotherapy and liver transplantation; however, the prognosis of primary hepatic leiomyosarcoma is dismal.CASE SUMMARY We describe here the first case of primary hepatic leiomyosarcoma successfully treated by transcatheter arterial chemoembolization(TACE). The patient was a 68-year-old woman who presented with right upper quadrant pain and weight loss over the past 5 wk before admission. Abdominal computed tomography(commonly known as CT) and ultrasonography showed a mixed echoic mass measuring about 10 cm × 7 cm occupying the right lobe of the liver. Exploratory laparotomy was performed 1 wk after admission. The tumor was unresectable and biopsy was performed. Based on rapid frozen-section and histopathological examination, a final diagnosis of primary hepatic leiomyosarcoma was established. TACE was performed 2 wk later. The postoperative course was uneventful and the patient was discharged on day 7 after the operation. Contrastenhanced CT showed that the tumor significantly shrunk with satisfactory lipiodol deposition. The patient has been followed up for 82 mo until now, and no progressive enlargement of the tumor or distal metastasis was observed.CONCLUSION TACE is a safe and effective treatment for primary hepatic leiomyosarcoma. The therapeutic effect of TACE combined with surgical resection should be further assessed.展开更多
BACKGROUND Leiomyosarcoma(LMS) of the thyroid gland is a rarely presented tumor that offers poor prognosis. To the best of the authors' knowledge, there currently exist only 28 known cases described in the literat...BACKGROUND Leiomyosarcoma(LMS) of the thyroid gland is a rarely presented tumor that offers poor prognosis. To the best of the authors' knowledge, there currently exist only 28 known cases described in the literature(limited to English).CASE SUMMARY Herein a case is reported of a 60-year-old female patient who had an LMS of the thyroid, which was accompanied by periodic dysphonia and breathing disorder as well as the feeling of pressure in the chest and neck. At the time the disease was diagnosed, no metastases were detected. Prior to the diagnosis, the patient experienced a uterine adenocarcinoma that had been treated by surgical procedure and radiotherapy. For the LMS, a total thyroidectomy was performed,followed by radiotherapy. Since metastases were also discovered in the lungs,sternum, and femur, chemotherapy was administered as well.Immunohistochemically, the tumor cells in the thyroid indicated positively for alpha smooth muscle actin, calponin, and H-caldesmon, but were negative for CD34, p63, estrogen receptor, progesterone receptor, and Epstein-Barr virus.CONCLUSION Although the etiology of the LMS is as of yet unknown, prior malignancy and radiation should be considered as risk factors.展开更多
INTRODUCTIONIt has been well known that MNNG is one of thestrong and multipotential carcinogens that havebeen frequently reported inducing malignant peptictumors.We have successfully induced rat and doggastric adenoca...INTRODUCTIONIt has been well known that MNNG is one of thestrong and multipotential carcinogens that havebeen frequently reported inducing malignant peptictumors.We have successfully induced rat and doggastric adenocarcinomas,squamous cell carcinomasof rat forestomach and gastric leiomyosarcoma展开更多
Objective： Primary uterine leiomyosarcomas （ULMS） are rare, and the optimal treatment is controversial. We aimed to assess the outcome and prognostic factors in a multicenter population of women treated for primary...Objective： Primary uterine leiomyosarcomas （ULMS） are rare, and the optimal treatment is controversial. We aimed to assess the outcome and prognostic factors in a multicenter population of women treated for primary ULMS. Methods： We retrospectively collected data of 110 women treated in 19 institutions of the Rare Cancer Network （RCN）. Inclusion criteria consisted of a pathology report confirming the diagnosis of ULMS, aged 18-80 years, complete International Federation of Gynecology and Obstetrics （FIGO） stage information, complete information on treatment, and a minimum follow-up of 6 months. Local control （LC） and locoregional control （LRC）, overall survival （OS） and disease-free survival （DFS） rates were computed using the Kaplan-Meier method. Univariate analysis was implemented using the log rank test, and multivariate analysis using the Cox model. Results： All patients underwent surgery. Seventy-five patients （68%） received adjuvant radiotherapy （RT）, including brachytherapy in 18 （I 6%）. Seventeen patients （15 %） received adjuvant chemotherapy. Median follow-up was 58 （range, 6-240） months. Five-year OS and DFS rates were 50% and 34%, and LC and LRC rates were 88% and 72%, respectively. On multivariate analysis, independent favorable prognostic factors were younger age, FIGO stage I, small tumor size, previous uterine disease, and no vascular invasion for OS and DFS. FIGO stage was the only favorable factor influencing LRC. Adjuvant local or systemic treatments did not improve the outcomes. Eight patients treated with RT presented a grade 3 acute toxicity, and only one patient with grade 3 late toxicity. Conclusions： In this large population of primary ULMS patients, we found good results in terms of LC and LRC. Nevertheless, OS remains poor, mainly due to the occurrence of distant metastases. An early diagnosis seemed to improve the prognosis of the patients. Adjuvant local or systemic treatments, or more aggressive surgical procedures such as the Wertheim procedure, did not seem to impact the outcome.展开更多
Primary pulmonary leiomyosarcoma （LMS） is a very unusual tumor.Although LMS has well-known metastatic potential,cutaneous metastasis is a remarkably uncommon.Exposure to cytotoxic agents could lead to ＂therapy-rela...Primary pulmonary leiomyosarcoma （LMS） is a very unusual tumor.Although LMS has well-known metastatic potential,cutaneous metastasis is a remarkably uncommon.Exposure to cytotoxic agents could lead to ＂therapy-related myeloid neoplasm＂ （t-MN）.Starting from 2008,the World Health Organization （WHO） has adopted the term to cover the spectrum of malignant diseases previously known as therapy-related acute myeloid leukemia （t-AML）,therapy-related myelodysplastic syndrome （t-MDS） and therapy-related myelodysplastic/myelo-proliferative neoplasm （t-MDS/MPN）.We described the onset of t-MDS and progression to t-AML in one case diagnosed as primary pulmonary LMS with cutaneous metastasis.This patient achieved complete remission （CR） after three courses of IA regimen chemotherapy （idarubicin 5 mg/d,d 1-3;cytarabine 100 mg/d,d 1-5） and 1 course of HA chemotherapy regimen （homoharringtonine 3 mg/d,d 1-3;cytarabine 100 mg/d,d 1-7）.This case presents the natural course of therapy-related neoplasm and provides therapeutic experience for t-AML.展开更多
Primary leiomyosarcoma of the nipple-areola complex is extremely rare. Less than ten such cases have been reported in English literature so far. Herein we describe a 52-year-old female presenting with a 1.5 cm×1....Primary leiomyosarcoma of the nipple-areola complex is extremely rare. Less than ten such cases have been reported in English literature so far. Herein we describe a 52-year-old female presenting with a 1.5 cm×1.1 cm×0.7 cm nodular lesion over her left nipple, and leiomyosarcoma was proved by pathological examination of the excised specimen. Positron emitted tomogram (PET) revealed no abnormal signal other than the primary site. Microscopically, this poorly circumscribed tumor was composed of interlacing bundles of smooth muscle cells with bizarre and pleomorphic nuclei, as well as prominent nucleoli. Its mitotic count was up to 7 mitoses per 10 high power fields (HPF). Immunohistochemical study of tumor cells revealed positive stain for α-smooth muscle actin and vimentin; and negative for cytokeratin, CD34 and S-100. Left simple mastectomy was undertaken and no residual mass lesion was noted on the resected specimen. Related literatures about the diagnosis and treatment for breast leiomyosarcoma will be presented here.展开更多
PURPOSE: Leiomyosarcoma of the breast is extremely rare. To date, the factors that are predictive of patient prognosis have not been identified. To clarify the nature of leiomyosarcoma of the breast, and also to estab...PURPOSE: Leiomyosarcoma of the breast is extremely rare. To date, the factors that are predictive of patient prognosis have not been identified. To clarify the nature of leiomyosarcoma of the breast, and also to establish the proper treatment strategy, we report this case We report this case while discussing The Clinical presentation, diagnosis, therapy and pathologic feature. INTRODUCION: Sarcomas comprise less than 1% of all primary breast neoplasms and only a minority of these are leiomyosarcomas. There were only 35 cases being reported in English literature. The mainstay of treatment is surgical excision with clear margins and longterm followup is essential. Its prognosis is better compared to other breast sarcoma. CASE PRESENTATION: We report a case of primary leiomyosarcoma of the breast occurring in young female patient which was successfully treated by surgery and radiotherapy. CONCLUSION: According to literature data, primary leiomyosarcoma is characterized by a better prognosis compared with other breast sarcomas, To date, the factors that are predictive of patient prognosis have not been identified. .The rarity and diagnostic difficulty imposed a multidisciplinary approach.展开更多
AIM: To describe the multidetector computed tomography features of pancreatic metastasis from leiomyosarcoma(LMS).METHODS: Between January 1995 and December 2012, 13 consecutive patients(11 women, 2 men; mean age of 5...AIM: To describe the multidetector computed tomography features of pancreatic metastasis from leiomyosarcoma(LMS).METHODS: Between January 1995 and December 2012, 13 consecutive patients(11 women, 2 men; mean age of 57 years; range, 38-78 years) with pancreatic metastases from LMS were included in our study. Imaging features including location, number, largest dimension, tumor attenuation and enhancement characteristics, presence of necrosis, pancreatic ductal dilatation, common bile duct(CBD) dilatation, presence of pancreatitis, and atrophy were documented.RESULTS: The most common site of origin of the pancreatic metastases from LMS was uterus(38.5%), followed by retroperitoneum(30.8%) and extremity(23.1%). None of the patients in our study had pancreas as the first site of metastasis. All patients developed pancreatic metastases at a median interval of 24 mo. Pancreatic metastases from LMS were solitary in 8/13 patients and multiple in 5/13 patients, had no predilection for any part of the pancreas, were hypovascular on arterial phase in 10/13 patients and associated with pancreatic duct dilatation in 3/13 patients. None had CBD dilatation. None of the pancreatic metastases in LMS cohort caused pancreatitis, and atrophy. Median duration of follow-up was 19 mo for LMS cohort during which two patients underwent resection of metastasis(median survival 45 mo) while the remaining underwent systemic therapy(median survival 13 mo).CONCLUSION: Pancreatic metastases from LMS are often solitary and hypovascular masses and less commonly associated with pancreatic ductal dilatation, CBD dilatation, pancreatitis or pancreatic atrophy. Surgical resection of solitary LMS pancreatic metastasis can be considered due to the long survival of these patients.展开更多
Leiomyosarcoma is a rare form of cancer commonly found in the retroperitoneum, uterus, stomach, small intestine and vascular tissue. Surgery with a wide margin of resection is the most effective treatment. Nevertheles...Leiomyosarcoma is a rare form of cancer commonly found in the retroperitoneum, uterus, stomach, small intestine and vascular tissue. Surgery with a wide margin of resection is the most effective treatment. Nevertheless, metastasis is common and generally occurs within the first 3 years. The liver and lungs are the most common sites of metastasis in leiomyosarcoma. Other sites of metastasis include bone, spleen, soft tissues and brain. Metastatic tumours of the clitoris are extremely rare. As cited in the literature, the most common cancers that metastasize to the clitoris are breast, bladder, renal and gastric. Here, we report a case of a clitoral mass in a 64-year-old woman who received an operation for retroperitoneal leiomyosarcoma 4 years prior. Mass resection was performed. The pathological diagnosis was a leiomyosarcoma metastasis. The patient also presented with brain and lung metastases at the time of the clitoral metastasis. This is the first case of clitoral and brain metastases originating from a retroperitoneal leiomyosarcoma.展开更多
Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive h...Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.展开更多
BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare tumor with a very low incidence of about 0.2%.CASE SUMMARY A 48-year-old diabetic,hypertensive,and morbidly obese female patient presented with a history of abdo...BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare tumor with a very low incidence of about 0.2%.CASE SUMMARY A 48-year-old diabetic,hypertensive,and morbidly obese female patient presented with a history of abdominal pain and weight loss for 2 mo.She had no history of fever,jaundice,or other liver disease(s).Clinical examination revealed a palpable mass in the epigastrium.Imaging evaluation with a contrast-enhanced computed tomography(CT)scan of the abdomen and pelvis revealed an illdefined enhancing hyper vascular hepatic mass of 9.9 cm×7.8 cm occupying the left hepatic lobe with evidence of central necrosis,compression effect on the left hepatic vein,and partial wash-out on delayed images.On further workup,the maximum standardized uptake value on positron emission computed tomography scan was 6.4,which was suggestive of malignancy.The remaining part of the liver was normal without any evidence of cirrhosis.Ultrasound-guided biopsy of the mass showed smooth muscle neoplasm suggestive of leiomyos-arcoma.After optimization for co-morbidities,an extended left hepatectomy was planned in a multidisciplinary team meeting.On intraoperative ultrasound,the left hepatic lobe was entirely replaced by a large tumor extending to the caudate lobe with a compression effect on the middle and left hepatic veins.Final histopathology showed nodular and whorled white tumor comprised of spind-led/fascicular cells with moderate to severe pleomorphism and focal necrosis.The mitotic index was greater than 20 mitoses per 10 high-power fields.The resection margins were free of tumor.Immunohistochemistry(IHC)depicted a desmin-positive/caldesmon-negative/discovered on gastrointestinal stromal tumor 1-negative/cluster of differentiation 117-negative profile,confirming the definitive diagnosis as PHL.CONCLUSION This case report highlights the rare malignant mesenchymal hepatic tumor.To confirm PHL diagnosis,one requires peculiar histopathological findings with ancillary IHC confirmation.Management options include adequate/complete surgical resection followed by chemotherapy and/or radiotherapy.展开更多
The duodenal leiomyosarcoma is a rare tumor. Its location in the duodenum makes diagnosis difficult and often delayed. Surgical treatment is not consensual because it must take into account anatomical imperatives whic...The duodenal leiomyosarcoma is a rare tumor. Its location in the duodenum makes diagnosis difficult and often delayed. Surgical treatment is not consensual because it must take into account anatomical imperatives which vary depending on the duodenal portion and oncological requirements that are not currently well known especially with regard to the utility of lymphadenectomy. We report 2 cases of leiomyosarcomas located at the angle of Treitz. This location is exceptional and only few cases were reported in the literature. From these 2 new cases and a review of the literature, we analyze the clinical features, diagnosis and treatment of duodenal leiomyosarcoma.展开更多
BACKGROUND Primary hepatic leiomyosarcoma is a very rare entity that originates from smooth muscle.Preoperative diagnosis requires a high degree of suspicion due to atypical clinical presentation and non-specific imag...BACKGROUND Primary hepatic leiomyosarcoma is a very rare entity that originates from smooth muscle.Preoperative diagnosis requires a high degree of suspicion due to atypical clinical presentation and non-specific imaging features.CASE SUMMARY We report the case of a 42-year-old man,with no relevant past medical history,accidentally diagnosed with a nodular liver lesion on a routine abdominal ultrasound.Liver function tests and hematology parameters as well as tumor markers were normal.A contrast-enhanced abdominal computed tomography scan revealed a heterogenous hepatic lesion measuring 40 mm 30 mm,adjacent to the left branch of the portal vein and the round ligament.Due to the unclear diagnosis,the patient underwent surgical resection.Histopathological and immunohistochemical examinations confirmed complete(R0)resection of a hepatic leiomyosarcoma.The patient remains without any signs of tumor recurrence for more than 2 years.CONCLUSION We report a rare case of accidentally diagnosed primary hepatic leiomyosarcoma originating from the portal vein or the round ligament.Although this tumor has aggressive metastatic potential,a tumor-free resection margin is essential to improve survival.展开更多
Primary hepatic leiomyosarcoma is a particularly rare tumor with a poor prognosis. Curative resection is currently the only effective treatment, and the efficacy of chemotherapy is unclear. This represents the first c...Primary hepatic leiomyosarcoma is a particularly rare tumor with a poor prognosis. Curative resection is currently the only effective treatment, and the efficacy of chemotherapy is unclear. This represents the first case report of a patient with primary hepatic leiomyosarcoma co-existing with metastatic liver carcinoma. We present a 59-year-old man who was diagnosed preoperatively with rectal cancer with multiple liver metastases. He underwent a curative hepatectomy after a series of chemotherapy regimens with modified FOLFOX6 consisting of 5-fluorouracil, leucovorin and oxaliplatin plus bevacizumab, FOLFIRI consisting of 5-fluorouracil, leucovorin and irinotecan plus bevacizumab, and irinotecan plus cetuximab. One of the liver tumors showed a different response to chemotherapy and was diag-nosed as a leiomyosarcoma following histopathological examination. This case suggests that irinotecan has the potential to inhibit the growth of hepatic leiomyosarcomas. The possibility of comorbid different histological types of tumors should be suspected when considering the treatment of multiple liver tumors.展开更多
基金Supported by the Lishui City Key Research and Development Project,No.2022ZDYF08.
文摘BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare malignant tumor and has non-specific clinical manifestations and imaging characteristics,making preoperative diagnosis challenging.Here,we report a case of PHL presenting primarily with fever,with computed tomography imaging showing a thick-walled hepatic lesion with low-density areas,resembling liver abscess.CASE SUMMARY The patient was a 34-year-old woman who presented with right upper abdominal pain and fever over 4 days before admission.Based on the patient’s medical history,laboratory examinations,and imaging examinations,liver abscess was suspected.Mesenchymal tumor was diagnosed by percutaneous liverbiopsy and partial hepatectomy was performed.Postoperative pathology revealed PHL.The patient is currently undergoing intravenous chemotherapy with the AD regimen and shows no signs of recurrence.CONCLUSION When there is a thick wall and rich blood supply in the hepatic lesion with a large proportion of uneven low-density areas,PHL should be considered.
文摘BACKGROUND Pleomorphic leiomyosarcomas make up around 8.6%of all leiomyosarcomas.They behave aggressively and often have poor prognoses.They can affect the gastrointestinal tract and retroperitoneum.To date,pleomorphic leiomyosarcoma involving the mesocolon have been reported in nine patients.CASE SUMMARY The patient was a 44-year-old man with a history of pleomorphic leiomyosarcoma of the left maxilla with metastasis to the lung and liver.His most recent positron emission tomography-computed tomography(PET-CT)scan showed uptake in the ascending and transverse colons.A colonoscopy revealed a 5.0 cm×3.5 cm×3.0 cm pedunculated polyp in the ascending colon.The polyp was removed using hot snare polypectomy technique and retrieved with Rothnet.Histopathologic examination of the polyp showed a metastatic pleomorphic leiomyosarcoma.CONCLUSION Uptake(s)on PET-CT in a patient with pleomorphic leiomyosarcoma should raise suspicion for metastasis.
文摘BACKGROUND Leiomyosarcoma(LMS)has a poor prognosis and rarely originates from the colon.If resection is possible,surgery is the first treatment most commonly considered.Unfortunately,no standard treatment exists for hepatic metastasis of LMS;although,several treatments,such as chemotherapy,radiotherapy,and surgery,have been used.Subsequently,the management of liver metastases remains controversial.CASE SUMMARY We present a rare case of metachronous liver metastasis in a patient with LMS originating from the descending colon.A 38-year-old man initially reported abdominal pain and diarrhea over the previous two months.Colonoscopy revealed a 4-cm diameter mass in the descending colon,40 cm from the anal verge.Computed tomography revealed intussusception of the descending colon due to the 4-cm mass.The patient underwent a left hemicolectomy.Immunohistochemical analysis of the tumor revealed that it was positive for smooth muscle actin and desmin,and negative for cluster of differentiation 34(CD34),CD117,and discovered on gastrointestinal stromal tumor(GIST)-1,which are characteristic of gastrointestinal LMS.A single liver metastasis developed 11 mo postoperatively;the patient subsequently underwent curative resection thereof.The patient remained disease-free after six cycles of adjuvant chemotherapy(doxorubicin and ifosfamide),and 40 and 52 mo after liver resection and primary surgery,respectively.Similar cases were obtained from a search of Embase,PubMed,MEDLINE,and Google Scholar.CONCLUSION Early diagnosis and surgical resection may be the only potential curative options for liver metastasis of gastrointestinal LMS.
基金Supported by National Natural Science Foundation of China,No81501530Scientific Research Projects of Hubei Health Commission,No. WJ2019M130Scientific Research Fund of Tongji Hospital,No. 2018B02
文摘BACKGROUND Leiomyosarcoma is a subtype of soft tissue sarcoma with adverse outcomes.Leiomyosarcoma accounts for nearly 70%of all uterine sarcomas and is responsible for a considerable proportion of deaths because of uterine cancer.Clinical characteristics and relevant diagnosis of pelvic leiomyosarcoma should be further explored.AIM To identify the outcome and relevant perioperative evaluation of patients with pelvic leiomyosarcoma.METHODS The Kaplan-Meier method was used to determine progression-free survival and overall survival rates.Factors predictive of outcomes were identified using univariate and multivariate Cox proportional hazards models.RESULTS Fifty-one patients with pelvic leiomyosarcoma were enrolled and divided into two groups including uterine leiomyosarcoma and non-uterine leiomyosarcoma.Overall,28.6%and 45.5%of uterine leiomyosarcoma and non-uterine leiomyosarcoma patients,respectively,had elevated carbohydrate antigen 125 levels,whereas 45.7%and 68.8%,respectively,underwent ultrasonography.Although 68.8%of uterine leiomyosarcoma patients were initially diagnosed with hysteromyoma,72.7%of non-uterine leiomyosarcoma patients had pelvic and abdominal masses.Moreover,93.3%of the recurrent lesions were detected using ultrasonography.Patients with International Federation of Gynaecology and Obstetrics(FIGO)stages III–IV disease had poorer progression-free survival values than those with FIGO stages I–II(P=0.027)disease.FIGO stage was significantly associated with poor progression-free survival in the univariate(hazard ratio=2.64,P=0.03)and multivariate(hazard ratio=2.49,P=0.048)analyses.CONCLUSION Serum tumour biomarkers cannot be used for pelvic leiomyosarcoma diagnosis.FIGO stage is critical to predict the outcome of uterine leiomyosarcoma.Ultrasonography is more reliable for postoperative follow-up than preoperative diagnosis.
文摘Primary hepatic leiomyosarcoma are rare tumors with less than 30 cases reported in the English literature.Non specific presentations and often diagnosis delayed until they reach a large size,is the norm with therapy leading to an often dismal prognosis.A 67-year-old man presented complaining of abdominal pain and a palpable abdominal mass since Jan 2010.Abdominal ultrasonography and abdominal computed tomography revealed a large tumor in the left lobe of the liver.Surgical exploration was undertaken and an extended left hepatectomy with extension onto the dorsal part of segment 8 preserving the MHV with partial resection of segment 6 was undertaken.The weight of the resected specimen was 1300 g of the left lobectomy specimen and 8 g of the segment 6 partial resection specimen.The pathology report confirmed the diagnosis of leiomyosarcoma.On immunohistochemistry,the tumor cells were positive for smooth muscle actin stain.The patient is on regular follow up and is currently 9 mo post resection with no evidence of recurrence.We report the case of a resected primary hepatic leiomyosarcoma and emphasize the need for a global database for these rare tumors to promote a better and broader understanding of this less understood subject.
文摘Spermatic cord leiomyosarcomas (LMSs) are rare tumors which may cause significant morbidity and mortality if inadequately diagnosed or treated. We report a case of a paratesticular LMS in a 60-year-old man who presented with a right scrotal mass. The patient was evaluated by scrotal ultrasound and computed tomography of the abdomen and pelvis (including scans of the scrotum), which revealed a large extratesticular mass. The lesion proved to be malignant and the patient underwent radical orchiectomy with high cord ligation. To improve the assignment of this lesion, we further analyze the imaging features of LMS and corre-late them with pathologic findings.
基金Yinzhou Young Investigator Award,NO.Yin Ren She 2017-133Ministry of Science of Yinzhou District,No.Yin Ke 2017-110
文摘BACKGROUND Primary hepatic leiomyosarcoma is rare and reported sporadically, with less than40 such cases have been reported in the English-language literature. Although it is reported to be associated with acquired immune deficiency syndrome, EpsteinBarr virus infection, Hodgkin's lymphoma, immunosuppression after organ transplantation, and hepatitis C virus-related liver cirrhosis, the precise steps leading to leiomyosarcoma have not been fully identified. Therapeutic strategies include liver wedge resection or lobectomy, chemotherapy, radiotherapy and liver transplantation; however, the prognosis of primary hepatic leiomyosarcoma is dismal.CASE SUMMARY We describe here the first case of primary hepatic leiomyosarcoma successfully treated by transcatheter arterial chemoembolization(TACE). The patient was a 68-year-old woman who presented with right upper quadrant pain and weight loss over the past 5 wk before admission. Abdominal computed tomography(commonly known as CT) and ultrasonography showed a mixed echoic mass measuring about 10 cm × 7 cm occupying the right lobe of the liver. Exploratory laparotomy was performed 1 wk after admission. The tumor was unresectable and biopsy was performed. Based on rapid frozen-section and histopathological examination, a final diagnosis of primary hepatic leiomyosarcoma was established. TACE was performed 2 wk later. The postoperative course was uneventful and the patient was discharged on day 7 after the operation. Contrastenhanced CT showed that the tumor significantly shrunk with satisfactory lipiodol deposition. The patient has been followed up for 82 mo until now, and no progressive enlargement of the tumor or distal metastasis was observed.CONCLUSION TACE is a safe and effective treatment for primary hepatic leiomyosarcoma. The therapeutic effect of TACE combined with surgical resection should be further assessed.
文摘BACKGROUND Leiomyosarcoma(LMS) of the thyroid gland is a rarely presented tumor that offers poor prognosis. To the best of the authors' knowledge, there currently exist only 28 known cases described in the literature(limited to English).CASE SUMMARY Herein a case is reported of a 60-year-old female patient who had an LMS of the thyroid, which was accompanied by periodic dysphonia and breathing disorder as well as the feeling of pressure in the chest and neck. At the time the disease was diagnosed, no metastases were detected. Prior to the diagnosis, the patient experienced a uterine adenocarcinoma that had been treated by surgical procedure and radiotherapy. For the LMS, a total thyroidectomy was performed,followed by radiotherapy. Since metastases were also discovered in the lungs,sternum, and femur, chemotherapy was administered as well.Immunohistochemically, the tumor cells in the thyroid indicated positively for alpha smooth muscle actin, calponin, and H-caldesmon, but were negative for CD34, p63, estrogen receptor, progesterone receptor, and Epstein-Barr virus.CONCLUSION Although the etiology of the LMS is as of yet unknown, prior malignancy and radiation should be considered as risk factors.
基金the National Natural Science Foundation of China,No.39270305
文摘INTRODUCTIONIt has been well known that MNNG is one of thestrong and multipotential carcinogens that havebeen frequently reported inducing malignant peptictumors.We have successfully induced rat and doggastric adenocarcinomas,squamous cell carcinomasof rat forestomach and gastric leiomyosarcoma
文摘Objective： Primary uterine leiomyosarcomas （ULMS） are rare, and the optimal treatment is controversial. We aimed to assess the outcome and prognostic factors in a multicenter population of women treated for primary ULMS. Methods： We retrospectively collected data of 110 women treated in 19 institutions of the Rare Cancer Network （RCN）. Inclusion criteria consisted of a pathology report confirming the diagnosis of ULMS, aged 18-80 years, complete International Federation of Gynecology and Obstetrics （FIGO） stage information, complete information on treatment, and a minimum follow-up of 6 months. Local control （LC） and locoregional control （LRC）, overall survival （OS） and disease-free survival （DFS） rates were computed using the Kaplan-Meier method. Univariate analysis was implemented using the log rank test, and multivariate analysis using the Cox model. Results： All patients underwent surgery. Seventy-five patients （68%） received adjuvant radiotherapy （RT）, including brachytherapy in 18 （I 6%）. Seventeen patients （15 %） received adjuvant chemotherapy. Median follow-up was 58 （range, 6-240） months. Five-year OS and DFS rates were 50% and 34%, and LC and LRC rates were 88% and 72%, respectively. On multivariate analysis, independent favorable prognostic factors were younger age, FIGO stage I, small tumor size, previous uterine disease, and no vascular invasion for OS and DFS. FIGO stage was the only favorable factor influencing LRC. Adjuvant local or systemic treatments did not improve the outcomes. Eight patients treated with RT presented a grade 3 acute toxicity, and only one patient with grade 3 late toxicity. Conclusions： In this large population of primary ULMS patients, we found good results in terms of LC and LRC. Nevertheless, OS remains poor, mainly due to the occurrence of distant metastases. An early diagnosis seemed to improve the prognosis of the patients. Adjuvant local or systemic treatments, or more aggressive surgical procedures such as the Wertheim procedure, did not seem to impact the outcome.
文摘Primary pulmonary leiomyosarcoma （LMS） is a very unusual tumor.Although LMS has well-known metastatic potential,cutaneous metastasis is a remarkably uncommon.Exposure to cytotoxic agents could lead to ＂therapy-related myeloid neoplasm＂ （t-MN）.Starting from 2008,the World Health Organization （WHO） has adopted the term to cover the spectrum of malignant diseases previously known as therapy-related acute myeloid leukemia （t-AML）,therapy-related myelodysplastic syndrome （t-MDS） and therapy-related myelodysplastic/myelo-proliferative neoplasm （t-MDS/MPN）.We described the onset of t-MDS and progression to t-AML in one case diagnosed as primary pulmonary LMS with cutaneous metastasis.This patient achieved complete remission （CR） after three courses of IA regimen chemotherapy （idarubicin 5 mg/d,d 1-3;cytarabine 100 mg/d,d 1-5） and 1 course of HA chemotherapy regimen （homoharringtonine 3 mg/d,d 1-3;cytarabine 100 mg/d,d 1-7）.This case presents the natural course of therapy-related neoplasm and provides therapeutic experience for t-AML.
文摘Primary leiomyosarcoma of the nipple-areola complex is extremely rare. Less than ten such cases have been reported in English literature so far. Herein we describe a 52-year-old female presenting with a 1.5 cm×1.1 cm×0.7 cm nodular lesion over her left nipple, and leiomyosarcoma was proved by pathological examination of the excised specimen. Positron emitted tomogram (PET) revealed no abnormal signal other than the primary site. Microscopically, this poorly circumscribed tumor was composed of interlacing bundles of smooth muscle cells with bizarre and pleomorphic nuclei, as well as prominent nucleoli. Its mitotic count was up to 7 mitoses per 10 high power fields (HPF). Immunohistochemical study of tumor cells revealed positive stain for α-smooth muscle actin and vimentin; and negative for cytokeratin, CD34 and S-100. Left simple mastectomy was undertaken and no residual mass lesion was noted on the resected specimen. Related literatures about the diagnosis and treatment for breast leiomyosarcoma will be presented here.
文摘PURPOSE: Leiomyosarcoma of the breast is extremely rare. To date, the factors that are predictive of patient prognosis have not been identified. To clarify the nature of leiomyosarcoma of the breast, and also to establish the proper treatment strategy, we report this case We report this case while discussing The Clinical presentation, diagnosis, therapy and pathologic feature. INTRODUCION: Sarcomas comprise less than 1% of all primary breast neoplasms and only a minority of these are leiomyosarcomas. There were only 35 cases being reported in English literature. The mainstay of treatment is surgical excision with clear margins and longterm followup is essential. Its prognosis is better compared to other breast sarcoma. CASE PRESENTATION: We report a case of primary leiomyosarcoma of the breast occurring in young female patient which was successfully treated by surgery and radiotherapy. CONCLUSION: According to literature data, primary leiomyosarcoma is characterized by a better prognosis compared with other breast sarcomas, To date, the factors that are predictive of patient prognosis have not been identified. .The rarity and diagnostic difficulty imposed a multidisciplinary approach.
文摘AIM: To describe the multidetector computed tomography features of pancreatic metastasis from leiomyosarcoma(LMS).METHODS: Between January 1995 and December 2012, 13 consecutive patients(11 women, 2 men; mean age of 57 years; range, 38-78 years) with pancreatic metastases from LMS were included in our study. Imaging features including location, number, largest dimension, tumor attenuation and enhancement characteristics, presence of necrosis, pancreatic ductal dilatation, common bile duct(CBD) dilatation, presence of pancreatitis, and atrophy were documented.RESULTS: The most common site of origin of the pancreatic metastases from LMS was uterus(38.5%), followed by retroperitoneum(30.8%) and extremity(23.1%). None of the patients in our study had pancreas as the first site of metastasis. All patients developed pancreatic metastases at a median interval of 24 mo. Pancreatic metastases from LMS were solitary in 8/13 patients and multiple in 5/13 patients, had no predilection for any part of the pancreas, were hypovascular on arterial phase in 10/13 patients and associated with pancreatic duct dilatation in 3/13 patients. None had CBD dilatation. None of the pancreatic metastases in LMS cohort caused pancreatitis, and atrophy. Median duration of follow-up was 19 mo for LMS cohort during which two patients underwent resection of metastasis(median survival 45 mo) while the remaining underwent systemic therapy(median survival 13 mo).CONCLUSION: Pancreatic metastases from LMS are often solitary and hypovascular masses and less commonly associated with pancreatic ductal dilatation, CBD dilatation, pancreatitis or pancreatic atrophy. Surgical resection of solitary LMS pancreatic metastasis can be considered due to the long survival of these patients.
文摘Leiomyosarcoma is a rare form of cancer commonly found in the retroperitoneum, uterus, stomach, small intestine and vascular tissue. Surgery with a wide margin of resection is the most effective treatment. Nevertheless, metastasis is common and generally occurs within the first 3 years. The liver and lungs are the most common sites of metastasis in leiomyosarcoma. Other sites of metastasis include bone, spleen, soft tissues and brain. Metastatic tumours of the clitoris are extremely rare. As cited in the literature, the most common cancers that metastasize to the clitoris are breast, bladder, renal and gastric. Here, we report a case of a clitoral mass in a 64-year-old woman who received an operation for retroperitoneal leiomyosarcoma 4 years prior. Mass resection was performed. The pathological diagnosis was a leiomyosarcoma metastasis. The patient also presented with brain and lung metastases at the time of the clitoral metastasis. This is the first case of clitoral and brain metastases originating from a retroperitoneal leiomyosarcoma.
文摘Coronary fistulae and ventricular septal perforation are very rare clinically, and even less caused by cardiac leiomyosarcoma. A case is reported that a 67-year-old female had cardiac leiomyosarcoma with progressive heart failure and coronary fistulae and ventricular septal perforation. This case was special since all ante-mortem examinations and cardiac surgery failed to detect the presence of any abnormal car-diac mass. Therefore, the malignant cardiac tumors could appear in an invasive form without mass and be one of the causes of the coronary fistulae and ventricular septal perforation.
文摘BACKGROUND Primary hepatic leiomyosarcoma(PHL)is a rare tumor with a very low incidence of about 0.2%.CASE SUMMARY A 48-year-old diabetic,hypertensive,and morbidly obese female patient presented with a history of abdominal pain and weight loss for 2 mo.She had no history of fever,jaundice,or other liver disease(s).Clinical examination revealed a palpable mass in the epigastrium.Imaging evaluation with a contrast-enhanced computed tomography(CT)scan of the abdomen and pelvis revealed an illdefined enhancing hyper vascular hepatic mass of 9.9 cm×7.8 cm occupying the left hepatic lobe with evidence of central necrosis,compression effect on the left hepatic vein,and partial wash-out on delayed images.On further workup,the maximum standardized uptake value on positron emission computed tomography scan was 6.4,which was suggestive of malignancy.The remaining part of the liver was normal without any evidence of cirrhosis.Ultrasound-guided biopsy of the mass showed smooth muscle neoplasm suggestive of leiomyos-arcoma.After optimization for co-morbidities,an extended left hepatectomy was planned in a multidisciplinary team meeting.On intraoperative ultrasound,the left hepatic lobe was entirely replaced by a large tumor extending to the caudate lobe with a compression effect on the middle and left hepatic veins.Final histopathology showed nodular and whorled white tumor comprised of spind-led/fascicular cells with moderate to severe pleomorphism and focal necrosis.The mitotic index was greater than 20 mitoses per 10 high-power fields.The resection margins were free of tumor.Immunohistochemistry(IHC)depicted a desmin-positive/caldesmon-negative/discovered on gastrointestinal stromal tumor 1-negative/cluster of differentiation 117-negative profile,confirming the definitive diagnosis as PHL.CONCLUSION This case report highlights the rare malignant mesenchymal hepatic tumor.To confirm PHL diagnosis,one requires peculiar histopathological findings with ancillary IHC confirmation.Management options include adequate/complete surgical resection followed by chemotherapy and/or radiotherapy.
文摘The duodenal leiomyosarcoma is a rare tumor. Its location in the duodenum makes diagnosis difficult and often delayed. Surgical treatment is not consensual because it must take into account anatomical imperatives which vary depending on the duodenal portion and oncological requirements that are not currently well known especially with regard to the utility of lymphadenectomy. We report 2 cases of leiomyosarcomas located at the angle of Treitz. This location is exceptional and only few cases were reported in the literature. From these 2 new cases and a review of the literature, we analyze the clinical features, diagnosis and treatment of duodenal leiomyosarcoma.
文摘BACKGROUND Primary hepatic leiomyosarcoma is a very rare entity that originates from smooth muscle.Preoperative diagnosis requires a high degree of suspicion due to atypical clinical presentation and non-specific imaging features.CASE SUMMARY We report the case of a 42-year-old man,with no relevant past medical history,accidentally diagnosed with a nodular liver lesion on a routine abdominal ultrasound.Liver function tests and hematology parameters as well as tumor markers were normal.A contrast-enhanced abdominal computed tomography scan revealed a heterogenous hepatic lesion measuring 40 mm 30 mm,adjacent to the left branch of the portal vein and the round ligament.Due to the unclear diagnosis,the patient underwent surgical resection.Histopathological and immunohistochemical examinations confirmed complete(R0)resection of a hepatic leiomyosarcoma.The patient remains without any signs of tumor recurrence for more than 2 years.CONCLUSION We report a rare case of accidentally diagnosed primary hepatic leiomyosarcoma originating from the portal vein or the round ligament.Although this tumor has aggressive metastatic potential,a tumor-free resection margin is essential to improve survival.
文摘Primary hepatic leiomyosarcoma is a particularly rare tumor with a poor prognosis. Curative resection is currently the only effective treatment, and the efficacy of chemotherapy is unclear. This represents the first case report of a patient with primary hepatic leiomyosarcoma co-existing with metastatic liver carcinoma. We present a 59-year-old man who was diagnosed preoperatively with rectal cancer with multiple liver metastases. He underwent a curative hepatectomy after a series of chemotherapy regimens with modified FOLFOX6 consisting of 5-fluorouracil, leucovorin and oxaliplatin plus bevacizumab, FOLFIRI consisting of 5-fluorouracil, leucovorin and irinotecan plus bevacizumab, and irinotecan plus cetuximab. One of the liver tumors showed a different response to chemotherapy and was diag-nosed as a leiomyosarcoma following histopathological examination. This case suggests that irinotecan has the potential to inhibit the growth of hepatic leiomyosarcomas. The possibility of comorbid different histological types of tumors should be suspected when considering the treatment of multiple liver tumors.
