Undifferentiated high-grade pleomorphic sarcoma of the common bile duct:A case report and review of literature

BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a rare malignant mesenchymal tumor with a poor prognosis.It mainly occurs in the extremities,trunk,head and neck,and retroperitoneum regions.Owing to the lack of specific clinical manifestations and imaging features,UPS diagnosis mainly depends on pathological and immunohistochemical examinations for exclusive diagnosis.Here we report an extremely rare case of high-grade UPS in the common bile duct(CBD).There are limited available data on such cases.CASE SUMMARY A 70-year-old woman was admitted to our department with yellow eyes and urine accompanied by upper abdominal distending pain for 2 wk.Her laboratory data suggested significantly elevated hepatorenal function levels.The imaging data revealed calculous cholecystitis,intrahepatic and extrahepatic bile duct dilation with extrahepatic bile duct calculi,and a space-occupying lesion at the distal CBD.After endoscopic biliary stenting and symptomatic support therapy,CBD exploration and biopsy were performed.The frozen section indicated malignant spindle cell tumor of the CBD mass,and further radical pancreaticoduodenectomy was performed.Finally,the neoplasm was diagnosed as a high-grade UPS combined with the light-microscopic morphology and immunohistochemical results.CONCLUSION This extremely rare case highlighted the need for increasing physicians'vigilance,reducing the odds of misdiagnosis,and providing appropriate treatment strategies.

Argon-helium cryoablation treatment of undifferentiated pleomorphic sarcoma of the thyroid:A case report and literature review

Undifferentiated pleomorphic sarcoma is an extremely rare malignant thyroid tumor.Thyroid sarcoma differs from common malignant thyroid tumors,such as thyroid follicular cell carcinoma.It is usually highly malignant,progresses rapidly,and is prone to remote metastasis.Currently,there is no standard protocol for the treatment of thyroid sarcomas,and most treatment effects are unsatisfactory.Argon-helium cryoablation is an important method of local treatment that is widely used in patients with unresectable advanced tumors.However,owing to the low incidence of thyroid sarcomas,there are no relevant literature reports on the treatment of thyroid sarcomas using cryoablation in China.This study reports the case of a patient with undifferentiated pleomorphic sarcoma of the thyroid gland who was treated with argon-helium cryoablation,and the immediate outcome was good.Based on a review of relevant literature,we discussed the effectiveness and safety of argon-helium cryoablation treatment,to provide clinical guidance and references for the treatment of patients with thyroid sarcoma.

Undifferentiated liver embryonal sarcoma in adults: A report of four cases and literature review

AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.

Undifferentiated embryonal sarcoma of the liver:Clinical characteristics and outcomes

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.

Undifferentiated embryonal sarcoma of liver in an old female:Case report and review of the literature

The clinical characteristics of undifferentiated(embryonal) sarcoma of the liver(UESL) were investigated and the best treatment modalities were recommended.Both histology and immuno-histochemistry demonstrated the cellular features of this peculiar tumor.The tumor size was 12 cm × 9 cm × 8 cm in the right liver lobe.The patient underwent surgical resection of the tumor.The postoperative recovery was uneventful and she died eight months after diagnosis.The tumor showed mixed spindle and polygonal cells within the myxoid matrix.Some tumor cells contained eosinophilic hyaline globules that were positive for resistant diastase.Immunohistochemistry showed positive vimentin.Stellate and spindle cells were positively stained with alpha-1-antichymotrypsin(AACT) and CD68.This case indicates that UESL is not obviously differentiated in old-aged adults.

Undifferentiated pleomorphic sarcoma of the liver presenting as a unilocular cyst

BACKGROUND:A malignant fibrous histiocytoma,recently referred to as an undifferentiated pleomorphic sarcoma (UPS),is very rare in the liver,and only 34 cases of primary hepatic malignant fibrous histiocytoma have been reported in the English literature. METHODS:We report a rare case of a hepatic UPS presenting as a unilocular cystic lesion with respect to histopathologic features,the newly revised diagnostic criteria,and the differential diagnosis. RESULTS:A 60-year-old man presented for evaluation of epigastric pain of 7 months duration.Abdominal computed tomography revealed a hypodense,unilocular cystic mass in the right lobe of the liver,measuring 14.0×11.1 cm.A right lobectomy was performed.Grossly,the cut surface showed a single,well-circumscribed unilocular cystic tumor mass containing dark red-brown necrotic debris and blood clots, which occupied most of the mass.Microscopically,the tumor consisted of haphazardly arranged mononuclear pleomorphic tumor cells,admixed with abundant osteoclast- like multinucleated giant cells.Immunohistochemically,the tumor cells expressed vimentin only.The histopathologic and immunohistochemical findings were compatible with a UPS.The patient is alive and well 41 months after surgery without recurrence. CONCLUSIONS:Clinically,most of the hepatic UPSs are solid masses.Only two cases have presented as multilocular cystic masses.A primary hepatic UPS presenting as a unilocular cyst has never been reported.A UPS should be included in the differential diagnosis of unilocular cystic lesions in the liver.

Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare and aggressive mesenchymal tumor in children.Herein,we describe our experience in neoadjuvant therapy(NAT)and subsequent surgery for the treatment of UESL in children.AIM To evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.METHODS We retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019.The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava.The NAT included preoperative transcatheter arterial chemoembol ization(TACE)and systemic chemotherapy.The patients were 4 boys and 2 girls with a mean age of 7 years.The longest tumor at presentation ranged from 8.6 to 14.8 cm(mean,12 cm).Extrahepatic metastases were present in 2 cases.Preoperative systemic chemotherapy was administered 3 wk after TACE.Tumor resection was performed 3 wk after one or two cycles of NAT.The patients received systemic chemotherapy after surgery.RESULTS All patients successfully underwent NAT and complete resection.The tumor volumes decreased by 18.2%–68.7%,with a mean decrease of 36%after 1 cycle of NAT(t=3.524,P=0.017).According to the Response Evaluation Criteria In Solid Tumors criteria,4 patients had a partial response and underwent surgery,while 2 had stable disease and received another cycle of NAT before surgery.Massive tumor necrosis was seen on pathological examination of the surgical specimen:>90%necrosis in two,>50%necrosis in three,and 25%necrosis in 1,with an average of 71.8%.Post-NAT complications included fever,nausea and vomiting,and mild bone marrow suppression.Elevated alanine transaminase levels occurred in all patients,which returned to normal within 7–10 d after treatment.No cardiac or renal toxicity,severe hepatic dysfunction,bleeding and nontarget embolization were observed in the patients.The median follow-up period was 8 years with an overall survival of 100%.CONCLUSION NAT effectively reduced tumor volume,cleared the tumor margin,and caused massive tumor necrosis.This may be a promising choice for successful surgery of UESL in children.

Undifferentiated (embryonal) sarcoma of liver in adult: A case report

We report a case of undifferentiated (embryonal)sarcoma of the liver (UESL), which showed cystic formation in a 20-year-old man with no prior history of any hepatitis or liver cirrhosis. He was admitted with abdominal pain and a palpable epigastric mass. The physical examination findings were unremarkable except for a tenderness mass and the results of routine laboratory studies were all within normal limits. Abdominal ultrasound and computed tomography (CT) both showed a cystic mass in the left hepatic lobe. Subsequently, the patient underwent a tumor excision and another two times of hepatectomy because of tumor recurrence.Immunohistochemical study results showed that the tumor cells were positive for vimentin, alpha-1-antichymotrypsin (AACT) and desmin staining, and negative for alphafetoprotein (AFP), and eosinophilic hyaline globules in the cytoplasm of some giant cells were strongly positive for periodic acid-Schiff (PAS) staining. The pathological diagnosis was UESL. The patient is still alive with no tumor recurrence for four months.

Recurrent undifferentiated embryonal sarcoma of the liver in adult patient treated by pembrolizumab: A case report

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a neoplasm that rarely develops in adults.The main treatments for UESL are upfront gross total surgical resection and adjuvant multiagent chemotherapy.Here,we report a case of recurrent UESL in an adult treated with pembrolizumab and discuss a method to identify proper candidates for antibody of programmed cell death protein 1(anti-PD-1)treatment.CASE SUMMARY A 69-year-old woman was admitted for abdominal pain that developed for 1 wk.Computed tomography showed a 16 cm mass in the right lobe of the liver.Right hemihepatectomy and lymphadenectomy were performed,and histological diagnosis was UESL.Six months later,the patient suffered from painless obstructive jaundice,and positron emission tomography-computed tomography revealed multiple metastases.Then,percutaneous transhepatic cholangial drainage was applied to reduce jaundice,and radiofrequency ablation was used to control the lesion near the hepatic hilum.However,the patient suffered from a serious fever caused by the tumor.The patient received treatment with pembrolizumab,and the prescribed dosage was 2 mg/kg every 3 wk.After the seventh dose,positron emission tomography-computed tomography revealed that the multiple metastases had nearly disappeared.Radiologic exam was used to evaluate the disease state,and no new lesions were found.Next-generation sequencing and immunohistology were applied to determine the reason why the patient had such a favorable response to pembrolizumab.Tumor mutation burden,microsatellite instability,and programmed death ligand 1 expression can be combined to predict the effect of PD-1 antibodies. When every one of thesebiomarkers are detected in a tumor patient, the patient may be a proper candidatefor PD-1 antibodies.CONCLUSION Anti-PD-1 treatment for tumors needs further research to identify indications andproper biomarkers.

Papillary thyroid carcinoma occurring with undifferentiated pleomorphic sarcoma: A case report

BACKGROUND Papillary thyroid cancer(PTC)is the most common malignant tumor of the thyroid.However,the coexistence of PTC and sarcoma in one patient is rare.In this article,we report the case of a patient who presented with both PTC and undifferentiated pleomorphic sarcoma(UPS),which has not been previously reported in the online Medline database(PubMed).CASE SUMMARY A 71-year-old man was admitted to our hospital for a mass on the right side of his neck for one month,which rapidly enlarged within 2 wk with distending pain.The patient was diagnosed with a thyroid malignancy by fine-needle aspiration and underwent total thyroidectomy and bilateral central lymph node dissection.Histology and immunohistochemistry revealed features of both PTC and UPS.The thyroid cancer 8 gene detection kit results showed BRAF and telomerase reverse transcriptase mutations.The disease progressed rapidly,and the patient died four months after surgery from extensive lung metastasis.CONCLUSION Our report highlights the patient’s pathological characteristics and related genetic mutations.Due to the rapid development and poor prognosis of cooccurring PTC and sarcoma,it is important for clinical physicians and pathologists to raise awareness of this type of tumor.

Local hyperthermia combined with chemotherapy for the treatment of multiple recurrences of undifferentiated pleomorphic sarcoma:A case report

BACKGROUND Undifferentiated pleomorphic sarcoma(UPS)is a type of soft tissue sarcoma,the histologic origin and differentiation direction of which are still unclear.There are few treatment options for UPS other than surgery.Herein we describe a patient who had multiple recurrences of UPS postoperatively,but R0 resection was achieved by local hyperthermia combined with chemotherapy,thus providing a new treatment approach for similar situations.CASE SUMMARY A 65-year-old man sought evaluation from a physician for a mass on his right back.After surgery,the pathologic diagnosis was fibrosarcoma.During the follow-up evaluations until 2021,the patient had four relapses of varying degrees.Postoperative pathology confirmed the recurrence of UPS on the right back.In March 2021,he underwent local hyperthermia combined with two cycles of chemotherapy for recurring lesions.After magnetic resonance imaging reexamination and preoperative examination,the patient chose surgery again.During the operation,the tumors were easy to excise,the amount of bleeding decreased significantly,and the pathologic evaluation confirmed that one of the specimens was an R0 excision.CONCLUSION Local hyperthermia combined with chemotherapy enables R0 resection to be achieved in patients with advanced UPS recurrence.

Surgical treatment outcomes of primary hepatic sarcomas: A singlecenter experience

BACKGROUND Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue.There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing.Unlike hepatocellular carcinoma,outcome of primary hepatic sarcoma is not well-known due to it’s rarity.However,with development of medical technology,surgical treatment may lead to better survival.AIM To investigate the surgical outcomes of primary hepatic sarcoma,we gathered and analyzed the cases of a single institute.METHODS From August 2001 to September 2016,a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure,early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma.Baseline characteristics,tumor characteristics such as tumor pathology,size and number,surgical and adjuvant treatments were reviewed.Tumor recurrence,and patient survival were analyzed with retrospective approach.RESULTS The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma.All patients experienced tumor recurrence at a median of 52 post-operative days.Only two patients survived and the 5-year survival rate was 29.6%.One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years.One patient with undifferentiated sarcoma received Rt.lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor.Two patients who received living donor liver transplantation due to angiosarcoma died.Only adjuvant therapy was associated with survival gain(P=0.002).CONCLUSION Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy,even though the outcome remains relatively poor.

Soft tissue sarcoma with metastasis to the stomach: A case report

Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleeding.

Diagnosis and therapy of primary undifferentiated embryonal sarcoma of the liver

Primary undifferentiated embryonal sarcoma （UES） of the liver, also called malignant mesenchymoma of the liver, is a rare hepatic malignancy. It arises from the mesoderm and comprises more than two types of sarcomas. UES can occur at any site of the body; common sites include the retroperitoneum and the lower extremities. Only a few cases of UES have been reported worldwide.1 In this study, we retrospectively examined the clinical data of 11 patients with UES between January 2000 and November 2011 at the People＇s Liberation Army General Hospital.

Adult primary undifferentiated embryonal sarcoma of the liver： a case report

Undifferentiated embryonal sarcoma of the liver （UESL） was first described by Stocker and Ishak in 1978. It is a rare tumor that most often presents in childhood between 6 to 10 years of age.2 The adult patients diagnosed with UESL often show nonspecific symptoms with normal laboratory results. Ultrasonography, CT, or MRI often reveals a cystic mass. Complete surgical resection plus pre- and/or post-operative systemic chemotherapy offers the possibility of a cure. We report a case of primary UESL in an adult with no specific symptoms, tumor markers, or image results. The diagnosis of the case was based on the resulting pathology from surgery.

Liver transplantation in malignant disease

Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology.Following the implementation of the Milan criteria,hepatocellular carcinoma(HCC)was the first generally accepted indication for transplantation in patients with cancer.Subsequently,more liberal criteria for HCC have been developed,and research on this topic is still ongoing.The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies.Regarding perihilar cholangiocarcinoma,more and more evidence supports the use of liver transplantation,especially after neoadjuvant therapy.In addition,some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma.Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer.In contrast,patients with hepatic angiosarcoma are currently not considered to be optimal candidates.In secondary liver tumors,neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation.Recently,some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases.This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.