Jejunal sarcomatoid carcinoma:A case report and review of literature

BACKGROUND Sarcomatoid carcinoma(SCA)of the jejunum is a rare and aggressive neoplasm affecting the smooth muscle cells of the jejunum.This study presents a recent case of jejunal SCA,detailing its diagnosis and treatment,thereby providing a reference for clinical practice.CASE SUMMARY A 65-year-old male presented to Yichang Central People's Hospital with a chief complaint of hemorrhoids.A computed tomography(CT)scan incidentally revealed multiple abnormal signals in the liver.Subsequent positron emission tomography/CT at Wuhan Union Hospital indicated malignant tumor progression,with a primary duodenal tumor and multiple metastases in the upper left abdomen.Intraoperatively,a large tumor was identified on the omentum.Histopathological and immunohistochemical analyses of the resected specimen confirmed the diagnosis of jejunal SCA.The patient received a combination therapy of sintilimab,nanoparticle albumin-bound paclitaxel,and anlotinib.Follow-up imaging demonstrated significant reduction of hepatic and peritoneal lesions.The patient has remained stable for over one year postoperatively.CONCLUSION This case suggests that chemotherapy,immunotherapy,plus targeted therapy may represent an optimal treatment for intestinal SCA,meriting further investigation.

Toripalimab in combination with chemotherapy effectively suppresses local recurrence and metastatic sarcomatoid renal cell carcinoma:A case report

BACKGROUND Sarcomatoid renal cell carcinoma(SRCC)is a rare variant of renal cell carcinoma associated with an unfavorable prognosis.The efficacy of conventional chemo-therapy and targeted therapies are limited,whereas the emergence of immune checkpoint inhibitor has introduced new avenues for managing advanced SRCC.CASE SUMMARY A 77-year-old female patient was referred to our hospital following the incidental detection of a right kidney tumor without specific symptoms.The tumor was successfully resected,and subsequent pathological examination confirmed SRCC.She experienced both local recurrence and distant metastasis eight months after the initial laparoscopic resection.Following six cycles of toripalimab combined with pirarubicin chemotherapy,the patient achieved a partial response.Subse-quently,the patient attained an almost-complete continuous response to toripa-limab monotherapy maintenance for an additional six cycles.She has not experienced disease progression for 15 months,and her overall survival has reached 24 months thus far.CONCLUSION Combination therapy with programmed death 1 antibodies and cytotoxic agents may be a recommended first-line treatment approach for SRCC.

A Case Report of a Rare Sarcomatoid Poorly Differentiated Adenocarcinoma Harboring Concurrent Mutations in the ROS1, EGFR, ARID1A, and NFKBIA Genes in the Lung

ROS1 and EGFR are primary oncogenic drivers in non-small cell lung cancer (NSCLC) pathogenesis. However, EGFR mutations and ROS1 fusions are generally mutually exclusive in NSCLC, leading to a negligible probability of their co-occurrence. Consequently, clinical data and treatment strategies for their simultaneous presence are remarkably scarce. This report details the first recorded case of a sarcomatoid, poorly differentiated lung adenocarcinoma harboring both a ROS1 fusion and an EGFR mutation, alongside ARID1A and NFKBIA gene mutations. Moreover, this case study encompasses a review of instances featuring concurrent ROS1 and EGFR mutations. The identified genetic alterations in ROS1, EGFR, ARID1A, and NFKBIA are pivotal in the etiology of NSCLC. These mutations significantly influence disease progression and are essential for the development of personalized therapeutic approaches. Recognizing the unique genetic profiles in patients permits healthcare providers to devise customized treatment regimens that target these specific mutations, thereby enhancing patient outcomes in NSCLC.

Sarcomatoid carcinoma of the pancreas: A case report

BACKGROUND Sarcomatoid carcinoma of the pancreas(SCP) is a rare and aggressive epithelial tumor that has both epithelial and mesenchymal features. It is characterized by sarcomatous elements with evidence of epithelial differentiation. And the term"sarcomatoid carcinoma" is often confused with "carcinosarcoma".CASE SUMMARY We present a case of SCP with lymph node metastasis in a 59-year-old male patient. He had experienced darkening of the urine, scleral icterus, and fatigue for 4 weeks. Computed tomography and magnetic resonance imaging revealed a mass in the pancreatic head, and laboratory tests revealed elevated serum bilirubin levels. The patient underwent pancreaticoduodenectomy after biliary decompression. Histologically, spindle cells with marked nuclear atypia and brisk mitotic activity arranged in a storiform or fascicular pattern were present in the bulk of the tumor. Immunohistochemical analysis found that the spindle cells exhibited strong diffuse positivity for epithelial markers, indicative of epithelial differentiation. Accordingly, the pathologic diagnosis of the pancreatic neoplasm was SCP.CONCLUSION Although sarcomatoid carcinomas and carcinosarcomas have different pathologic features, both have epithelial origin.

Achievement of complete response to nivolumab in a patient with advanced sarcomatoid hepatocellular carcinoma:A case report

BACKGROUND Sarcomatoid hepatocellular carcinoma(SHC)is a rare subtype of hepatocellular carcinoma(HCC),with a high recurrence rate after surgery.In addition to limited effective treatment for the advanced stage of SHC,the prognosis of patients with this malignancy is worse than that of patients with conventional HCC.CASE SUMMARY We present the case of a 54-year-old man with SHC who underwent radical segmental hepatectomy,which relapsed 4 mo after surgery due to lymphatic metastasis in the porta hepatis.Although a second surgery was performed,new metastasis developed in the mediastinal lymph nodes.Therefore,sorafenib and lenvatinib were sequentially administered as first-and second-line systemic therapies,respectively.However,progressive disease was confirmed based on a recurrent hepatic lesion and new metastatic lesion in the abdominal cavity.Percutaneous transhepatic cholangial drainage was performed to alleviate the biliary obstruction.Because the tumor was strongly positive for programmed death-ligand 1,the patient was started on nivolumab.Imaging studies revealed that after two cycles of immunotherapy,the metastatic lesions decreased to undetectable levels.CONCLUSION The patient experienced continuous complete remission for 8 mo.Immune checkpoint inhibitors are useful for the treatment of advanced SHC.

Evidence for colorectal sarcomatoid carcinoma arising from tubulovillous adenoma

Sarcomatoid carcinomas of the colorectum are rare tu- mors that display both malignant epithelial and stromal components. Clinically, they are aggressive tumors with early metastasis. Due to their infrequent occurrence, the pathogenesis is poorly understood. We report a case of a 52-year-old woman who presented with a rectal mass and intermittent hematochezia. Superficial biopsies during colonoscopy revealed a tubulovillous adenoma with high-grade dysplasia. Endoscopic ultra- sonography confirmed an invasive nature of the mass, and deeper biopsies revealed the presence of neoplasm with mixed histological components. The surgically- excised specimen demonstrated the presence of poorly differentiated spindle cells underneath the tubulovillous adenoma and an intermediate stage of invasive adeno- carcinoma. Based on the histological appearance and immunohistochemical studies, a diagnosis of sarcoma- toid carcinoma was made. Only nine cases of sarcoma- toid carcinomas of the colorectum have been reported to date. As a result, the terminology and pathogenesis of sarcomatoid carcinoma remain speculative. To the best of our knowledge, this is the first report of co- existence of sarcomatoid carcinoma and invasive ad- enocarcinoma with tubulovillous adenoma; all stages represented within the same tumor. This observation supports the "monoclonal theory" of pathogenesis with an adenoma-sarcoma progression with or without an intermediate stage of carcinoma.

Sarcomatoid carcinoma of the pancreas—a rare tumor with an uncommon presentation and course:A case report and review of literature

BACKGROUND Sarcomatoid carcinoma of the pancreas(SCP)is a rare type of pancreatic neoplasm,and only a few cases have been described in the literature.Histologically,it is composed mostly of atypical spindle cells with apparent sarcomatous features.CASE SUMMARY This is a report of a 61-year-old Chilean woman who underwent medical investigation for acute abdominal pain.Computed tomography identified a solid tumor in the tail of the pancreas with features suspicious of malignancy.En-bloc distal pancreatectomy and splenectomy were performed to excise the tumor.Histopathology and immunohistochemistry were confirmatory of sarcomatoid carcinoma with lymphovascular invasion.After surgery,the patient did not receive chemotherapy.Previous studies indicate a poor prognosis for this type of malignancy.However,our patient has survived for 35 mo with no recurrence to date.CONCLUSION The case presented herein is a patient with an SCP with a rare presentation and long-term survival after surgery despite not receiving adjuvant chemotherapy.

Sarcomatoid carcinoma of the prostate with bladder invasion shortly after androgen deprivation:Two case reports

BACKGROUND To summarize the imaging,morphological and biological characteristics of sarcomatoid carcinoma(SC)of the prostate with bladder invasion not long after castration.CASE SUMMARY Our two cases were initially diagnosed with adenocarcinoma of the prostate due to dysuria.However,prostate SC was diagnosed after transurethral resection of the prostate(TURP)and castration after only 5 and 10 mo,respectively.Distinctive liver-like tissues appeared in the second TURP procedure in case 1,while a white,fish flesh-like,narrow pedicled soft globe protruded from the prostate to the bladder in case 2.CONCLUSION The sarcomatoid component of SC may arise from one of the specific groups of cancer cells that are resistant to hormonal therapy.Morphological characteristics of SCs can present as“red hepatization”and“fish flesh”.SCs grow rapidly and have a poor prognosis,and thus,extensive TURP plus radiation may be the treatment of choice.

Surgical therapy and prognosis of sarcomatoid carcinoma of the gallbladder

BACKGROUND: Sarcomatoid carcinoma of the gallbladder is rare and its characteristics are poorly understood. This study aimed to understand the behavior and prognosis of sarcomatoid carcinoma of the gallbladder as well as its clinical manifestations and survival rate of patients after radical or palliative surgery, and to review the reported data worldwide and our 10 patients. METHODS: From 2004 to 2009, ten patients were pathologically diagnosed with sarcomatoid carcinoma of the gallbladder and underwent operation at our center. These characteristics, clinical presentations, tumor-node-metastasis (TNM) staging, surgical modes, and prognosis were reviewed, retrospectively. We collected the data of 46 patients reported in the English-language literature worldwide and analyzed the survival with ours. The survival rate was estimated using the Kaplan-Meier method, and was compared using the log-rank test. RESULTS: The median age of the 10 patients was 67 years (inter-quartile range 59-74 years), and the size of tumor inter-quartile ranged from 3.1 to 7.9 cm. In this series, 9 patients received radical surgery, and one undewent palliative surgery. There was no surgical mortality, and one patient underwent a second operation because of liver metastasis. The median survival time of the patients was 9 months (inter-quartile range 6-12 months), with 3 patients still being alive until follow-up; however, two patients had tumor recurrence. The data from the 56 patients (10 patients in our series and 46 reported elsewhere) statistically indicated that the median age was 66 years (inter-quartile range 61-74.5 years) and the overall median survival was 5.5 months (inter-quartile range 2.5-10 months). The survival time in the patients undergoing radical surgery (n=42) was significantly longer than that in the patients undergoing palliative surgery (n=14) (P=0.031). CONCLUSIONS: The survival of the patients with sarcomatoid carcinoma of the gallbladder is poor. Some patients may die shortly after the surgery because of recurrence or metastasis. However, radical surgery is still necessary if possible. (Hepatobiliary Pancreat Dis Int 2010; 9: 175-179)

A huge adenosquamous carcinoma of the pancreas with sarcomatoid change:An unusual case report

Adenosquamous carcinoma rarely occurs in the pancreas,and is characterized by the presence of cellular components from both duct adenocarcinoma and squamous carcinoma.Here,we describe a rare case of pancreatic adenosquamous carcinoma with sarcomatous change.Immunohistochemistry showed that the sarcomatous lesion lost the epithelial marker and aberrantly expressed of acquired mesenchymal markers,which indicated that this special histological phenotype may be attributed to epithelial-mesenchymal transition.This case also indicated that a routine radical surgery without aggressive treatment strategies was still appropriate for adenosquamous carcinoma of the pancreas with sarcomatoid change.

Analysis of intrahepatic sarcomatoid cholangiocarcinoma:Experience from 11 cases within 17 years

BACKGROUND Intrahepatic sarcomatoid chonalgiocarcinoma(s-CCC) is an extremely rare disease, accounting for less than 1% of hepatobiliary system malignancies, and its pathophysiology is not well known. On the hypothesis that its clinical, serologic,or radiologic diagnosis are not fully understood and its prognosis is poor, we investigated the distinguishing features of s-CCC compared with those of intrahepatic bile duct adenocarcinoma [cholangiocellular carcinoma(CCC)] in patients from a single center.AIM To analyze the clinical, serologic, imaging, and histopathologic characteristics of intrahepatic s-CCC patients diagnosed in a single center.METHODS The clinical, serologic, imaging, and histopathologic features of 227 patients diagnosed with intrahepatic cholangiocarcinoma(IHCC) in a single medical center during the last 17 years were analyzed. The characteristics of 11 patients with s-CCC were compared with those of 216 patients with CCC.RESULTS The number of patients with s-CCC who presented fever and abdominal pain and past history of chronic viral hepatitis or liver cirrhosis(LC) was higher than that of patients with CCC. In imaging studies, patients with s-CCC showed relatively aggressive features. However, no clear distinction was observed between s-CCC and CCC based on other clinical, serologic or radiologic examination results. An accurate diagnosis could be made only via a histopathologic examination through immunohistochemical staining. The clinical course of s-CCC was generally aggressive, and patients had a relatively poor prognosis.CONCLUSION In patients with s-CCC, early diagnosis through biopsy and aggressive treatment,including surgical resection, are important.

Unusual early-stage pancreatic sarcomatoid carcinoma

Sarcomatoid carcinoma of the pancreas(SCP)is a very rare pathological type of carcinoma that usually has a poor prognosis.Its pathogenesis has not been elucidated.We herein report a case of an early-stage SCP involving successful treatment and a good prognosis.The patient was a 48-year-old Chinese man with a5-mo history of vague abdominal pain.Ultrasonography revealed a 93 mm×94 mm×75 mm mass of mixed echogenicity in the tail of the pancreas.Laboratory test results were within the normal range,with the exception of an obviously increased pretreatment neuron-specific enolase level.The plasma transforming growth factor(TGF)β1 and interleukin-11 levels were obviously increased according to enzyme-linked immunosorbent assay.Microscopically,the excised tumor tissue comprised cancer cells and mesenchymal cells.Immunohistochemical analysis was positive forα-1-antichymotrypsin,pan-cytokeratin,cytokeratin 19,cytokeratin 8/18,and vimentin and negative for CD68and lysozyme.The pathogenetic mechanism of this case shows that TGFβ1 may regulate the epithelial-tomesenchymal transition in SCP.With early eradication of the tumor and systemic therapy,this patient has been alive for more than 3 years without tumor recurrence or distant metastasis.This case is also the first to show that TGFβ1 may regulate the epithelial-tomesenchymal transition in early-stage SCP.

Pembrolizumab-emerging treatment of pulmonary sarcomatoid carcinoma: A case report

BACKGROUND Few studies have addressed the efficacy of pembrolizumab in pulmonary sarcomatoid carcinoma(PSC),a rare,previously rapidly fatal subtype of nonsmall-cell lung cancer.CASE SUMMARY We report the case of a 69-year-old man presented with respiratory distress caused by a large left upper lung lobe mass diagnosed as PSC with programmed death-ligand 1 expressed on more than 50 percent of tumor cells.The patient was started on pembrolizumab and,after 5 cycles,there was a more than 80 percent decrease in the size of the tumor mass.Further decrease was seen at the end of 10 cycles.The patient has been tolerating pembrolizumab well,with no limiting side-effects.Fourteen months after first coming into the hospital,he remains asymptomatic.CONCLUSION Pembrolizumab appears as a viable emerging treatment for PSC.

Clinicopathological characteristics and surgical outcomes of sarcomatoid hepatocellular carcinoma

BACKGROUND Hepatocellular carcinoma(HCC)is the sixth most common type of cancer and the fourth leading cause of cancer-related death worldwide.Sarcomatoid HCC,which contains poorly differentiated carcinomatous and sarcomatous components,is a rare histological subtype of HCC that differs from conventional HCC.It is highly aggressive and has a poor prognosis.Its clinicopathological characteristics,surgical outcomes and underlying mechanisms of its highly aggressive nature have not been fully elucidated.AIM To examine the clinicopathological characteristics and surgical outcomes of sarcomatoid HCC and explore the histogenesis of sarcomatoid HCC.METHODS In total,196 patients[41 sarcomatoid HCC and 155 high-grade(Edmondson-Steiner grade III or IV)HCC]who underwent surgical resection between 2007 and 2017 were retrospectively reviewed.The characteristics and surgical outcomes of sarcomatoid HCC were compared with those of patients with high-grade HCC.The histological composition of invasive and metastatic sarcomatoid HCCs was evaluated.RESULTS Sarcomatoid HCC was more frequently diagnosed at an advanced stage with a larger tumor and higher rates of nonspecific symptom,adjacent organ invasion and lymph node metastasis than high-grade HCC(all P<0.05).Compared with high-grade HCC patients,sarcomatoid HCC patients are less likely to have typical dynamic imaging features of HCC(44.4%vs 72.7%,P=0.001)and elevated serum alpha-fetoprotein levels(>20 ng/mL;36.6%vs 78.7%,P<0.001).The sarcomatoid group had a significantly shorter median recurrence-free survival(5.6 mo vs 16.4 mo,log-rank P<0.0001)and overall survival(10.5 mo vs 48.1 mo,log-rank P<0.0001)than the high-grade group.After controlling for confounding factors,the sarcomatoid subtype was identified as an independent predictor of poor prognosis.Pathological analyses indicated that invasive and metastatic lesions were mainly composed of carcinomatous components.CONCLUSION Sarcomatoid HCC was associated with a more advanced stage,atypical dynamic imaging,lower serum alpha-fetoprotein levels and a worse prognosis.The highly aggressive nature of sarcomatoid HCC is perhaps mediated by carcinomatous components.

Sarcomatoid intrahepatic cholangiocarcinoma mimicking liver abscess: A case report

BACKGROUND Sarcomatoid intrahepatic cholangiocarcinoma(SICC) is an extremely rare and highly invasive malignant tumor of the liver. To our knowledge, the imaging findings of sarcomatous cholangiocarcinoma have been rarely reported; and radiological features of this tumor mimicking liver abscess have not yet been reported.CASE SUMMARY We present a case of SICC mimicking liver abscess. The patient, a 43-year-old male, complained of repeated upper right abdominal discomfort and intermittent distension over a period of one month. Radiology examination revealed a huge focal lesion in the right liver. The lesion was hypointense on computed tomography with honeycomb enhancement surrounded by enhanced peripheral areas. It showed a hypo-signal on non-contrast T1-weighted images and a hypersignal on non-contrast T2-weighted images. Radiologists diagnosed the lesion as an atypical liver abscess. The patient underwent a hepatectomy. After surgery, he survived another 2.5 mo before passing away. A search of Pub Med and Google revealed 43 non-repeated cases of SICC reported in 20 published studies. The following is a short review in order to improve the diagnostic and therapeutic skills in cases of SICC.CONCLUSION This report presents the clinical and radiological features of SICC and imaging features which showed hypovascularity and progressive enhancement. SICC can present as a multilocular cyst on radiological images and it is necessary to distinguish this lesion from an atypical abscess. Simple surgical treatment is not the best treatment option for this disease.

Gallbladder sarcomatoid carcinoma:Seven case reports

BACKGROUND Gallbladder sarcomatoid carcinoma is a rare and aggressive tumor,and little is known about its clinical behavior,prognosis,and optimal treatment.CASE SUMMARY From 1997 to 2017,we collected seven cases of gallbladder sarcomatoid carcinoma at our institution.The median patient age was 68.5 years.Six(85.7%)patients were female.Overall,85.7%(6/7)of the tumors had a maximal diameter greater than 7 cm.Late TNM stage was associated with a significantly poor prognosis.All patients with advanced-stage(III/IV)disease died from metastases or disease progression shortly after surgery.One patient with stage IIIB disease who received adjuvant chemoradiotherapy(gemcitabine and capecitabine)achieved a progression-free survival(PFS)of 12 mo and overall survival of 15 mo,which might be the longest PFS reported among patients who ultimately experienced recurrence or metastasis.CONCLUSION Sarcomatoid carcinoma is a unique and aggressive gallbladder malignancy.Surgery is suggested as the first and only recognized treatment.There is a significant difference in prognosis between patients with early-stage and advanced-stage disease.Postoperative adjuvant therapy may bring survival benefits for locally advanced patients.Gemcitabine combined with fluorouracil and radiotherapy could be a potential strategy.

Tongue swelling as a manifestation of tongue metastasis from pulmonary sarcomatoid carcinoma: A case report

BACKGROUND Sarcomatoid carcinoma is a rare subtype of non-small-cell lung cancer,commonly associated with locally advanced disease,early metastasis,and poor prognosis.Tongue metastasis from lung cancer is a rare condition that may occur in advanced stage of the disease.CASE SUMMARY The patient was a 70-year-old female with a history of resected pulmonary sarcomatoid carcinoma(PSC)who presented with subacute tongue swelling,imparting the clinical impression of a lingual abscess.However,histologic examination of the partial glossectomy revealed a high-grade,poorly differentiated spindle and epithelioid carcinoma consistent with metastatic PSC.CONCLUSION Although uncommon,clinicians should be cognizant of the possibility of a metastatic process to the tongue mimicking a benign or inflammatory process.A high index of suspicion for metastatic disease should be maintained when tongue swelling is observed in patients with a known history of PSC.

Primary squamous cell carcinoma with sarcomatoid differentiation of the kidney associated with ureteral stone obstruction: A case report

BACKGROUND Primary squamous cell carcinoma(SCC)with sarcomatoid differentiation of the kidney was rarely reported.This disease is usually related to renal stones,and due to a lack of symptoms and radiological features,patients usually attend the hospital with late stage disease.CASE SUMMARY A 54-years-old female presented with left flank pain and an abdominal mass for 6 mo.Imaging studies revealed that the left kidney was enlarged and massive hydronephrosis was present.A stone was seen in the ureteropelvic junction.The patient subsequently underwent left radical nephrectomy,and histopathological examination of the mass revealed a poorly differentiated renal SCC with sarcomatoid differentiation.After primary surgery,the patient received four cycles of tirelizumab.Four months later,the patient developed adrenal,lymph,and uterine appendage metastases.CONCLUSION SCC of the kidney has a poor prognosis,and should be considered in patients with a renal mass,long-standing urinary calculi and massive hydronephrosis.

Giant pedunculated sarcomatoid hepatocellular carcinoma: a case report

We report a case who was a 74-year-old female with hepatitis B virus （HBV） carrier for more than 30 years. The serum levels of AFP, CEA and CA199 were within normal range. CT scan demonstrated that a huge pedunculated mass of the right hepatic lobe presenting as Iobulated and cystic-solid appearance and heterogeneous density of the solid portion composed of multiple nodules. The patient was performed surgery, and the tumor was removed. Sarcomatoid hepatocallular carcinoma （SHC） was diagnosed by pathological and immunohistochemical test. The patient has been living for 4 months without tumor recurrence, which indicates that pedunculated SHC might have a high probability for resection and acquire better survival.

Pyonephrosis as a sign of sarcomatoid carcinoma of the renal pelvis

We report the case of an urgent nephrectomy because of a pyonephrosis and sepsis due to an unsuspected sarcomatoid transitional cell carcinoma, an infrequent subtype with a bad oncological prognosis. We present a 58-year-old man assessed by internal medicine for a general syndrome and weakness many months previously. A pyonephrotic kidney was observed at abdominal computed tomography in the context of septic shock, without suspecting the underlying cause. The pathology report described a sarcomatoid transitional cell carcinoma. Sarcomatoid transitional cell carcinoma is an invasive and infrequent subtype of urothelial tumors. The symptoms are often the same as other renal masses; however, in this case, sepsis and pyonephrosis were the rare initial symptoms.