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.

Renal pelvis sarcomatoid carcinoma with renal vein tumor thrombus:A case report and literature review

BACKGROUND Renal pelvis sarcomatoid carcinoma(RPSC)is a rare and aggressive malignancy whose diagnosis is difficult because radiological imaging results can lead to misclassification as a more common type of renal tumor.In addition,clinical management of patients with RPSC is difficult because of the limited efficacy of available treatments.In this study,we present a comprehensive description of a patient who presented with RPSC and a simultaneous renal vein tumor thrombus.CASE SUMMARY During April,2020,a 64-year-old female presented with an isolated episode of hematuria accompanied by abdominal pain.Computed tomography(CT)and magnetic resonance imaging(MRI)showed a lesion in the right renal pelvis.We therefore performed a radical nephrectomy of the right kidney.The subsequent histopathological and immunological results verified the diagnosis of RPSC.Despite administration of 6 cycles of a gemcitabine-cisplatin regimen,the patient's condition progressively deteriorated,and she died about 15 mo after the nephrectomy.CONCLUSION We performed a comprehensive analysis of a patient with RPSC that included CT,MRI,immunohistochemistry,and genetic testing.The insights from our detailed analysis of this patient and our concomitant review of the literature may assist clinicians in their diagnosis and treatment of RPSC.

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)

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.

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.

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.

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.

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.

Significant benefits of pembrolizumab in treating refractory advanced pulmonary sarcomatoid carcinoma: A case report

BACKGROUND Pulmonary sarcomatoid carcinoma(PSC),a rare subtype of non-small cell lung cancer(NSCLC),is poorly differentiated and highly aggressive.Treatment is limited,and the prognosis is poor.Pembrolizumab is an anti-programmed death(PD)-1 antibody with good efficacy in NSCLC.Recent studies have demonstrated that PD-ligand 1(PD-L1)overexpression is common in PSCs,which suggests that anti-PD-L1 treatment is an ideal option.However,the response to pembrolizumab in PSC has not been studied.CASE SUMMARY We present a PSC case with PD-L1 overexpression that significantly benefited from pembrolizumab.A 73-year-old Chinese male was detected with a right lung lesion.Pathological analysis of the right upper lobectomy confirmed PSC.The PDL1 test revealed overexpression(TPS:90%).Multiple metastases occurred 1 mo after surgery,representing stage IV PSC.Neither first-line chemotherapy nor second-line antiangiogenic agents showed any benefit.Radiotherapy(1200 cGy)was administered to relieve chest wall pain.The patient received the PD-1 inhibitor pembrolizumab(100 mg)as third-line therapy;however,because of fever and severe infection,he refused to receive immunotherapy any longer.Thus,only one dose of pembrolizumab was administered.Deep sustained remission of most of the metastases was achieved except for lesions in the right adrenal gland,which first shrank and then progressed.The patient died because of disease progression in the right adrenal gland.He achieved a progression-free survival time of 8 mo and an overall survival time of 9 mo with third-line pembrolizumab.CONCLUSION Our findings highlight and offer direct evidence of the efficacy of pembrolizumab in PD-L1-overexpressing PSCs.Combined radiotherapy and immunotherapy may enhance treatment efficacy.

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 Mandible: A Case Report and Literature Review

Background: Sarcomatoid carcinoma is a rare biphasic malignancy with an epithelial and mesenchymal component. Given its histological particularity, the diagnosis of this tumor still represents a challenge for pathologists and surgeons. Sarcomatoid carcinoma of the mandible is a very rare and aggressive entity. To date, only sporadic cases have been reported in the literature. Aim: To report a rare case of aggressive mandibular sarcomatoid carcinoma treated with radical surgery and adjuvant chemoradiation, and discuss the related literature. Case Report: We introduce a rare case of mandibular sarcomatoid carcinoma occurring in a 34-year-old woman. Treatment consisted of radical surgery and re-excision for positive margins, adjuvant radiation therapy (66 Gy in 33 fractions in simultaneous integrated boost delivered using Volumetric Modulated Arc Therapy) and Cisplatin-based concurrent chemotherapy. The tumor displayed aggressive behavior and high metastatic potential, causing tumour recurrence and extensive lung metastasis. Conclusion: Sarcomatoid carcinoma of the mandible is an extremely rare malignant entity, with a high tendency for local recurrence, distant metastasis. Treatment involves radical surgery and adjuvant chemoradiation, although the outcome and prognosis might be poor.

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.

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.

A Case Report of a Sarcomatoid Carcinoma Arising in the Renal Pelvis with Exuberant Osteosarcomatous Element

Sarcomatoid carcinoma is a rare malignant tumor that has both malignant epithelial and mesenchymal components. We describe a sarcomatoid carcinoma arising in the right renal pelvis of a 68-year-old man. The dominant component of the tumor was osteosarcomatous, but there were also focal carcinomatous areas. The sarcomatous tumor cells produced abundant osteoid matrix surrounded by osteoblastic cells. The carcinomatous tumor cells consisted of papillary urothelial carcinoma. Immunohistochemical assay showed that the sarcomatous tumor cells were positive for vimentin and negative for cytokeratin. The papillary urothelial carcinoma was positive for cytokeratin and negative for vimentin. After surgery, the patient underwent adjuvant chemotherapy. Four months later, he presented with recurrence in the right subphrenic area and metastasis in the right middle lobe of the lung.

Sarcomatoid Carcinoma of the Urinary Bladder

The patient was a 62-year-old female with bladder carcinoma. TUR-BT was performed in March 2010, and the pathological finding was UC, G2 > G1, pTa. However, the cancer recurred in the urinary bladder 3 months after TUR-BT. Radical cystectomy was performed, and the disease was considered to have been cured, but metastasis developed in the skin, lung, liver, and bone several months after surgery. Chemotherapy was ineffective, and the patient died. On pathological examination at the time of radical cystectomy, the lesion was sarcomatoid carcinoma of the urinary bladder. Although the prognosis associated with this carcinoma is known to be poor, the possibility of underestimation on preoperative staging cannot be ruled out from the cause, in addition to the insufficiency of the current therapeutic strategy.

Sarcomatoid carcinoma of the penis

We encountered a 63-year-old male with a sarcomatoid carcinoma of the penis accompanied by metastasis to the bilateral inguinal lymph nodes and lungs. He noticed a penile mass, but neglected it. The mass rapidly increased in size, forming an ulcer, and began to disintegrate. He visited our hospital 4 months after noticing the mass. After cystostomy and blood transfusion, surgical resection was performed. A diagnosis of a squamous cell carcinoma with a sarcomatoid carcinoma of the penis was made. After the operation, best supportive care (BSC) was selected. We made efforts to maintain his quality of life (QOL), and he died 3 months after the operation.

Integrative genomic and transcriptomic profiling of pulmonary sarcomatoid carcinoma identifies molecular subtypes associated with distinct immune features and clinical outcomes

Background:Pulmonary sarcomatoid carcinoma(PSC)is a rare and aggressive subtype of non-small cell lung cancer(NSCLC),characterized by the presence of epithelial and sarcoma-like components.The molecular and immune landscape of PSC has not been well defined.Methods:Multiomics profiling of 21 pairs of PSCs with matched normal lung tissues was performed through targeted high-depth DNA panel,whole-exome,and RNA sequencing.We describe molecular and immune features that define subgroups of PSC with disparate genomic and immunogenic features as well as distinct clinical outcomes.Results:In total,27 canonical cancer gene mutations were identified,with TP53 the most frequently mutated gene,followed by KRAS.Interestingly,most TP53 and KRAS mutations were earlier genomic events mapped to the trunks of the tumors,suggesting branching evolution in most PSC tumors.We identified two distinct molecular subtypes of PSC,driven primarily by immune infiltration and signaling.The Immune High(IM-H)subtype was associated with superior survival,highlighting the impact of immune infiltration on the biological and clinical features of localized PSCs.Conclusions:We provided detailed insight into the mutational landscape of PSC and identified two molecular subtypes associated with prognosis.IM-H tumors were associated with favorable recurrence-free survival and overall survival,highlighting the importance of tumor immune infiltration in the biological and clinical features of PSCs.

Sarcomatoid carcinoma of the jejunum presenting as obscure gastrointestinal bleeding in a patient with a history of gliosarcoma

Small bowel malignant tumors are rare and sarcomatoid carcinomas have rarely been reported at this site.We report a 56-year-old woman,with history of an excised gliosarcoma,who presented with recurrent obscure gastrointestinal bleeding.She underwent endoscopy and colonoscopy,which failed to identify the cause of the bleeding.The abdominal computed tomography scan located a tumor in the small bowel.Pathology revealed a jejunal sarcomatoid carcinoma.She developed tumor recurrence and multiple liver metastases shortly after surgery.Immunohistochemistry is required for accurate diagnosis.Sarcomatoid carcinoma is a rare cause of obscure gastrointestinal bleeding,which is associated with a poor prognosis.

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.

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.