molecular pathology of intraductal papillary mucinous neoplasms of the pancreas

Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.

DNA methylation and carcinogenesis in digestive neoplasms

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European vs 2015-World Health Organization clinical molecular and pathological classification of myeloproliferative neoplasms

The BCR/ABL fusion gene or the Ph^1-chromosome in the t(9;22)(q34;q11)exerts a high tyrokinase acticity,which is the cause of chronic myeloid leukemia(CML).The1990 Hannover Bone Marrow Classification separated CML from the myeloproliferative disorders essential thrombocythemia(ET),polycythemia vera(PV)and chronic megakaryocytic granulocytic myeloproliferation(CMGM).The 2006-2008 European Clinical Molecular and Pathological(ECMP)criteria discovered 3variants of thrombocythemia:ET with features of PV(prodromal PV),"true"ET and ET associated with CMGM.The 2008 World Health Organization(WHO)-ECMP and 2014 WHO-CMP classifications defined three phenotypes of JAK2^(V617F)mutated ET:normocellular ET(WHO-ET),hypercelluar ET due to increased erythropoiesis(prodromal PV)and ET with hypercellular megakaryocytic-granulocytic myeloproliferation.The JAK2^(V617F)mutation load in heterozygous WHO-ET is low and associated with normal life expectance.The hetero/homozygous JAK2^(V617F)mutation load in PV and myelofibrosis is related to myeloproliferative neoplasm(MPN)disease burden in terms of symptomaticsplenomegaly,constitutional symptoms,bone marrow hypercellularity and myelofibrosis.JAK2 exon 12mutated MPN presents as idiopathic eryhrocythemia and early stage PV.According to 2014 WHO-CMP criteria JAK2 wild type MPL^(515)mutated ET is the second distinct thrombocythemia featured by clustered giant megakaryocytes with hyperlobulated stag-horn-like nuclei,in a normocellular bone marrow consistent with the diagnosis of"true"ET.JAK2/MPL wild type,calreticulin mutated hypercellular ET appears to be the third distinct thrombocythemia characterized by clustered larged immature dysmorphic megakaryocytes and bulky(bulbous)hyperchromatic nuclei consistent with CMGM or primary megakaryocytic granulocytic myeloproliferation.

PVSG and WHO vs European Clinical,Molecular and Pathological Criteria for prefibrotic myeloproliferative neoplasms

The Polycythemia Vera Study Group(PVSG),World Health Organization(WHO) and European Clinical,Molecular and Pathological(ECMP) classifications agree upon the diagnostic criteria for polycythemia vera(PV) and advanced primary myelofibrosis(MF). Essential thrombocythemia(ET) according to PVSG and 2007/2008 WHO criteria comprises three variants of JAK2V617 F mutated ET when the ECMP criteria are applied. These include normocellular ET,hypercellular ET with features of early PV(prodromal PV),and hypercellular ET due to megakaryocytic,granulocytic myeloprolifera-tion(ET.MGM). Evolution of prodromal PV into overt PV is common. Development of MF is rare in normocellular ET(WHO-ET) but rather common in hypercellular ET.MGM. The JAK2V617 F mutation burden in heterozygous mutated normocellular ET and in heterozygous/homozygous or homozygous mutated PV and ET.MGM is of major prognostic significance. JAK2/MPL wild type ET associated with prefibrotic primary megakaryocytic and granulocytic myeloproliferation(PMGM) is characterized by densely clustered immature dysmorphic megakaryocytes with bulky(bulbous) hyperchromatic nuclei,which are never seen in JAK2V617 F mutated ET,and PV and also not in MPL515 mutated normocellular ET(WHO-ET). JAK2V617 mutation burden,spleen size,LDH,circulating CD34+ cells,and pre-treatment bone marrow histopathology are mandatory to stage the myeloproliferative neoplasms ET,PV,PMGM for proper prognosis assessment and therapeutic implications. MF itself is not a disease because reticulin fibrosis and reticulin/collagen fibrosis are secondary responses of activated polyclonal fibroblasts to cytokines released from the clonal myeloproliferative granulocytic and megakaryocytic progenitor cells in ET.MGM,PV and PMGM.

Progression of hepatic hyperperfusion disorders revealed during follow-up CT scan of digestive system neoplasm

Objective： The aim of this study was to investigate progression of hepatic hyperperfusion disorders revealed during follow-up contrast material-enhanced multi-slice spiral computed tomography （MSCT） scan of digestive system neoplasm. Methods： Three-phase contrast material-enhanced MSCT were performed during the follow-up in patients with digestive system malignant tumor confirmed histologically. The progression of hepatic hyperperfusion disorders revealed on contrast material-enhanced CT image were investigated at the 2 years follow-up with approximately 6 months interval. Results： The hepatic hyperperfusion disorders were showed in 39 patients on follow-up contrast material-enhanced MSCT scans. Among the 39 patients, initial hyperperfusion disorders were revealed in 6 （15.38%）, 26 （66.67%）, and 7 （17.95%） patients in 6, 12, and 18 months during follow-up respectively. The initial hyperperfusion disorders revealed in 12 months were more frequent than those revealed in 6 months （X2 = 14.82, P 〈 0.05） and 18 months （X2 = 15.02, P 〈 0.05）. Among the 39 patients, the hyperperfusion disorders progressed into liver metastasis based on typical CT findings in 37 （94.87%） patients, and were not obvious changes in 2 （5.13%） patients. Among the 37 patients, the hyperperfusion disorders progressed into metastasis in 10 （25.64%） patients in 6 months after the hyperperfusion disorders were revealed, and in 27（69.23%） patients in 12 months. The hyperperfusion disorders developing into metastasis were more in 12 months than those in 6 months （X2= 14.98, P 〈 0.05）. Conclusion： Most hepatic hyperperfusion disorders revealed at the follow-up of digestive system neoplasm may be early manifestations of liver metastasis. The careful follow-up of hepatic hyperperfusion disorders is necessary.

Cavitary Pulmonary Metastases: CT Features and Their Correlation with the Pathology of the Primary Malignancy

Objective: To study CT features of cavitary pulmonary metastases and to investigate the pos- sible relationship between CT features and the pathology of the primary lesions. Methods: CT ?ndings of 131 cavitary metastatic nodules in 40 patients with pathologically-proved pulmonary metastases were retrospectively analyzed. A comparison between CT signs and the pathologic types of the primary tumors was made. Results: Cavitary metastases and multiple solid nodules coexisted in all patients. Cavitary metastases presented as bubble (n=41), irregular (n=33), cystic (n=26) or small circular (n=31) cavities, with even (n=61) or uneven (n=70) thickness of the cavity wall. Of 131 cavitary nodules, diameter less than 15 mm was seen in 44, between 15–25 mm in 66, 25–40 mm in 17 and larger than 40 mm in 4 respectively. And the wall thickness of the cavity below 4 mm, between 4–15 mm and over 15 mm was respectively seen in 69, 44 and 18 metastatic nodules. Cavitary pulmonary metastases mainly occurred in patients whose primary malignancy was squamous cell carcinoma (n=13) or adenocarcinoma (n=22). Both squamous cell carcinoma and adenocarcinoma had its own CT characteristics. The occurrence of cavity bore no relationship to its site in the lung. Conclusion: Cavitary pulmonary metastases carries certain CT features and its occurrence is related to the pathologic type of the primary malignancy.

Practical hints for the diagnosis of mixed neuroendocrine-nonneuroendocrine neoplasms of the digestive system

In this editorial,a comment on the article by Díaz-López et al published in the recent issue of the 2024 is provided.We focus on the practical implications critical for providing a correct and complete diagnosis of mixed neuroendocrine-nonneuroendocrine neoplasm(MiNEN)in the gastrointestinal system.The diagnosis of MiNEN begins with the recognition of neuroendocrine features in one component of a biphasic tumor.The non-neuroendocrine counterpart can be virtually represented by any neoplastic type,even though the most frequent histologies are glandular and squamous.However,qualification of the neuroendocrine component requires histological and immunohistochemical confirmation.Neuroendocrine tumors are characterized by a peculiar architectural organization and bland nuclei with granular“salt and pepper”chromatin.Although neuroendocrine carcinomas have multiple and variable presentations,they typically show a solid or organoid architecture.The histological aspect needs to be confirmed by immunohistochemistry,and a diagnosis is confirmed whenever the expression of keratin and neuroendocrine markers is observed.Once both histopathological and immunohistochemical features of neuroendocrine neoplasms are identified,it is important to consider the three major pitfalls of MiNEN diagnostics:(1)Entrapment of neuroendocrine non-neoplastic cells within the tumor mass;(2)Differential diagnosis with amphicrine neoplasms;and(3)Differential diagnosis of tumors that partially express neuroendocrine markers.According to the current guidelines for diagnosing digestive MiNEN,each component must represent at least 30%of the entire neoplastic mass.Although the high-grade histopathological subtype frequently determines disease prognosis,both components can significantly affect prognosis.Thus,if one of the components,either neuroendocrine or non-neuroendocrine,does not fulfill the volumetric criteria,the guidelines still encourage reporting it.These strict criteria are essential for correctly recognizing and characterizing digestive MiNENs.This task is essential because it has prognostic relevance and substantial potential value for guiding further studies in this field.In the future,systematic analyses should be performed to validate or reconsider the current 30%cutoff value.

Retrospective study on mixed neuroendocrine non-neuroendocrine neoplasms from five European centres

BACKGROUND Mixed neuroendocrine non-neuroendocrine neoplasm(MiNEN)is a rare diagnosis,mainly encountered in the gastro-entero-pancreatic tract.There is limited knowledge of its epidemiology,prognosis and biology,and the best management for affected patients is still to be defined.AIM To investigate clinical-pathological characteristics,treatment modalities and survival outcomes of a retrospective cohort of patients with a diagnosis of MiNEN.METHODS Consecutive patients with a histologically proven diagnosis of MiNEN were identified at 5 European centres.Patient data were retrospectively collected from medical records.Pathological samples were reviewed to ascertain compliance with the 2017 World Health Organisation definition of MiNEN.Tumour responses to systemic treatment were assessed according to the Response Evaluation Criteria in Solid Tumours 1.1.Kaplan-Meier analysis was applied to estimate survival outcomes.Associations between clinical-pathological characteristics and survival outcomes were explored using Log-rank test for equality of survivors functions(univariate)and Cox-regression analysis(multivariable).RESULTS Sixty-nine consecutive patients identified;Median age at diagnosis:64 years.Males:63.8%.Localised disease(curable):53.6%.Commonest sites of origin:colon-rectum(43.5%)and oesophagus/oesophagogastric junction(15.9%).The neuroendocrine component was;predominant in 58.6%,poorly differentiated in 86.3%,and large cell in 81.25%,of cases analysed.Most distant metastases analysed(73.4%)were occupied only by a poorly differentiated neuroendocrine component.Ninety-four percent of patients with localised disease underwent curative surgery;53%also received perioperative treatment,most often in line with protocols for adenocarcinomas from the same sites of origin.Chemotherapy was offered to most patients(68.1%)with advanced disease,and followed protocols for pure neuroendocrine carcinomas or adenocarcinomas in equal proportion.In localised cases,median recurrence free survival(RFS);14.0 months(95%CI:9.2-24.4),and median overall survival(OS):28.6 months(95%CI:18.3-41.1).On univariate analysis,receipt of perioperative treatment(vs surgery alone)did not improve RFS(P=0.375),or OS(P=0.240).In advanced cases,median progression free survival(PFS);5.6 months(95%CI:4.4-7.4),and median OS;9.0 months(95%CI:5.2-13.4).On univariate analysis,receipt of palliative active treatment(vs best supportive care)prolonged PFS and OS(both,P<0.001).CONCLUSION MiNEN is most commonly driven by a poorly differentiated neuroendocrine component,and has poor prognosis.Advances in its biological understanding are needed to identify effective treatments and improve patient outcomes.

Telomere,telomerase and digestive cancer

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Intraductal papillary-mucinous neoplasia of the pancreas:Histopathology and molecular biology

Intraductal papillary-mucinous neoplasm(IPMN) of the pancreas is a clinically and morphologically distinctive precursor lesion of pancreatic cancer,characterized by gradual progression through a sequence of neoplastic changes.Based on the nature of the constituting neoplastic epithelium,degree of dysplasia and location within the pancreatic duct system,IPMNs are divided in several types which differ in their biological properties and clinical outcome.Molecular analysis and recent animal studies suggest that IPMNs develop in the context of a field-defect and reveal their possible relationship with other neoplastic precursor lesions of pancreatic cancer.

Endoscopic submucosal tunnel dissection for largesuperficial esophageal squamous cell neoplasms

Endoscopic submucosal dissection(ESD)is a wellestablished treatment for superficial esophageal squamous cell neoplasms(SESCNs)with no risk of lymphatic metastasis.However,for large SESCNs,especially when exceeding two-thirds of the esophageal circumference,conventional ESD is time-consuming and has an increased risk of adverse events.Based on the submucosal tunnel conception,endoscopic submucosal tunnel dissection(ESTD)was first introduced by us to remove large SESCNs,with excellent results.Studies from different centers also reported favorable results.Compared with conventional ESD,ESTD has a more rapid dissection speed and R0 resection rate.Currently in China,ESTD for large SESCNs is an important part of the digestive endoscopic tunnel technique,as is peroral endoscopic myotomy for achalasia and submucosal tunnel endoscopic resection for submucosal tumors of the muscularis propria.However,not all patients with SESCNs are candidates for ESTD,and postoperative esophageal strictures should also be taken into consideration,especially for lesions with a circumference greater than three-quarters.In this article,we describe our experience,review the literature of ESTD,and provide detailed information on indications,standard procedures,outcomes,and complications of ESTD.

Clinical significance of CA_(19-9) in diagnosis of digestive tract tumors

IM To evaluate the clinical value of CA199 in diagnosing and differentiating the gastrointestinal tumors and in monitoring the patients treated surgically.METHODS Gastric cancer (n=70), colorectal cancer (n=90), pancreatic cancer (n=7), esophageal cancer (n=10), benign disorder (n=30), and normal adults (n=111) as control were studied. Fasting blood samples were obtained from each patient and normal adult. The serum CA199 concentration was measured with radioimmunoassay.RESULTS The mean CA199 level in gastric cancer (17069kU/L±9145kU/L) and in colorectal cancer (8721kU/L±3955kU/L) was significantly higher than that in the control (11254kU/L±600kU/L). Compared with the normal adults, the CA199 level was much higher in pancreatic cancer patients (126658kU/L±52131kU/L) (P<001). However, the CA199 concentrations in nonrecurrent gastric (1263kU/L±362kU/L) and colorectal cancers (1414kU/L±326kU/L) and benign disorders (1423kU/L±260kU/L) were similar to the control. The differences were not statistically significant (P>005). The demarcation value of CA199 between the negative and positive was <310kU/L. The sensitivity of CA199 for gastric, colorectal, pancreatic and esophageal cancers and gastrointestinal benign disorders was 473%, 500%, 833%, 200% and 0%, respectively. The specificity of CA199 for digestive system malignant diseases was all 100%.

Characterics of upper digestive tract diseasesin fishermen of the Bohai Bay

AIM To study the characteristics of upper digestive tract diseases (UDTDs) in the fishermen of the Bohai Bay. METHODS An investigation was carried out in 1488 fishermen with symptoms of UDTDs except liver, biliary and pancreatic diseases during the period from December 1991 to February 1995, which included medical history inquiry, physical, gastroscopic and pathological examinations, detection of Helicobacter pylori ( H. pylori) infection, and nitrate content in their drinking water. RESULTS Among the 1488 subjects investigated, 1467 suffered from one or more diseases among 14 kinds of UDTDs, most of which were chronic atrophic gastritis (CAG, 1103 cases), peptic ulcer (268 cases), and cancer of the upper digestive tract (25 cases). CONCLUSION Incidence rate of UDTDs tends to be high among the fishermen, especially CAG, peptic ulcer and upper digestive tract cancer, and complicated state of illness due to their special life habits, and high nitrate content in their drinking water, etc, and UDTDs in fishermen is significantly different from that in inland residents in clinical manifestations.

The accuracy of magnetic resonance imaging and ultrasound in evaluating the size of early-stage breast neoplasms

Objective Breast cancer is the most frequently diagnosed cancer in women. Accurate evaluation of the size and extent of the tumor is crucial in selecting a suitable surgical method for patients with breast cancer. Both overestimation and underestimation have important adverse effects on patient care. This study aimed to evaluate the accuracy of breast magnetic resonance imaging(MRI) and ultrasound(US) examination for measuring the size and extent of early-stage breast neoplasms.Methods The longest diameter of breast tumors in patients with T_(1–2)N_(0–1)M_0 invasive breast cancer preparing for breast-conserving surgery(BCS) was measured preoperatively by using both MRI and US and their accuracy was compared with that of postoperative pathologic examination. If the diameter difference was within 2 mm, it was considered to be consistent with pathologic examination.Results A total of 36 patients were imaged using both MRI and US. The mean longest diameter of the tumors on MRI, US, and postoperative pathologic examination was 20.86 mm ± 4.09 mm(range: 11–27 mm), 16.14 mm ± 4.91 mm(range: 6–26 mm), and 18.36 mm ± 3.88 mm(range: 9–24 mm). US examination underestimated the size of the tumor compared to that determined using pathologic examination(t = 3.49, P < 0.01), while MRI overestimated it(t =-6.35, P < 0.01). The linear correlation coefficients between the image measurements and pathologic tumor size were r = 0.826(P < 0.01) for MRI and r = 0.645(P < 0.01) for US. The rate of consistency of MRI and US compared to that with pathologic examination was 88.89% and 80.65%, respectively, and there was no statistically significant difference between them(χ~2 = 0.80, P > 0.05).Conclusion MRI and US are both effective methods to assess the size of breast tumors, and they maintain good consistency with pathologic examination. MRI has a better correlation with pathology. However, we should be careful about the risk of inaccurate size estimation.

Blastic plasmacytoid dendritic cell neoplasm in Jinhua,China:Two case reports

BACKGROUND Blastic plasmacytoid dendritic cell neoplasm(BPDCN)is a rare and clinically aggressive hematologic malignancy originating from the precursors of plasmacytoid dendritic cells.BPDCN often involves the skin,lymph nodes,and bone marrow,with rapid clinical progression and a poor prognosis.The BPDCN diagnosis is mainly based on the immunophenotype.CASE SUMMARY In this paper,we retrospectively analyzed 2 cases of BPDCN.Both patients were elderly males.The lesions manifested as skin masses.Morphological manifestations included diffuse and dense tumor cell infiltration of the dermis and subcutaneous tissues.Immunohistochemistry staining showed that cluster of differentiation CD4,CD56,CD43,and CD123 were positive.CONCLUSION In this paper,we retrospectively analyzed 2 cases of BPDCN.Both patients were elderly males.The lesions manifested as skin masses.Morphological manifestations included diffuse and dense tumor cell infiltration of the dermis and subcutaneous tissues.Immunohistochemistry staining showed that cluster of differentiation CD4,CD56,CD43,and CD123 were positive.

Clinico-pathologic significance of neuroendocrine cells in gastric carcer tissue

AIMS To elucidate the biological and clinicopathological signifi- cance of neuroendocrine(NE)cells in gastric carcinoma(GC). METHODS One hundred and eighty-nine cases of various histo- logical types of GC were observed using light microscopy, histochemistry,immunohistochemistry and electron microscopy. Of them 127 patients were followed up. RESULTS Chromogranin A(CgA)positive GC was demonstrat- ed in 85 cases(45.0%).The typos of NE cells in GC were probed using 9 kinds of hormone antibodies 49 cases(67.2%) contained more than one hormone.NE cells were found more often in poorly differentiated GC than in well differentiated ones(P<0. 01).Expression of some kinds of hormone Was relative to the dif- ferentiation and histological types of GC.BOM,CT(P<0.01) GAST and 5-HT(P<0.05)were more expressed in poorly differ- entiated cases than in well defferentiated ones.Nineteen cases of the matastatic foci in regional lymph nodes were found to have CgA positive cancer cells.The presence of HCG in matastatic lymph nodes was more often observed than that of other hormone (P<0.01).The survival rate of patients with NE positive GC was 38.9% and negative GC 52.7%.Five of 7 patients(71. 4%)with somatostatin positive GC still survived at follow up of 33-66 months,but 4 patients with HCG positive GC died in 12-29 months. CONCLUSIONS The NE cells occur more frequently in poorly differentiated GC.Certain hormones appear to be related to the metastasis and prognosis.

Histopathological study of hepatocellular carcinoma after transcatheter hepatic arterial embolization

AIMS To study the histopathological changes in hepatocellular carcinoma (HCC) after transcatheter arterial embolization (TAE). METHODS Histopathological analysis was made in 39 cases of liver neoplasms after TAE and 11 cases of liver neoplasms after digital selective angiography (DSA), including pathological type, histological grade, necrotic degree, capsule, times of treatment, injured vessel and lymphocyte infiltration. RESULTS Six cases with 100% necrosis, 14 cases with 30% 95% necrosis, 19 cases with 0% 5% necrosis after TAE and 11 cases without necrosis after DSA were found histologically. The necrosis was related to the pathological type, capsule, injured vessels, but not to the histological grade, time of treatment and lymphocyte infiltration of the liver neoplasms. CONCLUSIONS TAE is an effective therapy for the late stage HCC. The encapsulated HCC is a preferable indicator for TAE.

Infantile hepatic hemangioendothelioma:A clinicopathologic study in a Chinese population

AIM:To investigate whether the clinicopathologic features of infantile hemangioendothelioma(IHE) of the liver in a Chinese population are similar to the features observed in other races.METHODS:The clinical data,radiological findings,histopathological changes and outcome of 12 cases of IHE diagnosed by the Department of Pathology,West China Hospital over the last 10 years were analyzed retrospectively.Immunohistochemical studies were carried out using antibodies against CD31,CD34,Factor Ⅷ,cytokeratin 8 and cytokeratin 18.RESULTS:The 12 patients were aged from fetal to 5 years(three males and nine females).The tumor was presented with different clinical manifestations,mainly as an asymptomatic,palpable,upper abdominal mass,except for the two fetuses who were detected antena-tally by ultrasound.In one patient,this presentation was accompanied by an initial severe pneumothorax.No symptoms of congestive heart failure were present and neither congenital abnormalities nor vascular tumors in the skin or other organs were found.Laboratory abnormalities included leukocytosis(40%),anemia(60%),thrombocytosis(60%),hyperbilirubinemia(16.7%),abnormal liver function(50%) and increased α-fetoprotein(80%).Based on radiological findings and gross specimens,the tumor presented as a solitary lesion or a multifocal space-occupying lesion.The tumor size ranged from 5.0 cm × 3.5 cm × 2.0 cm to 13.8 cm × 9.0 cm × 7.7 cm,and the 0.2-1.1 cm nodules were diffusely distributed within the multifocal tumor.Seven cases were surgically resected,three cases underwent biopsy and the two fetuses were aborted.Histologically,nine cases were classified as typeⅠ and three as type Ⅱ,presenting aggressive morphologic features,immature vessels,active mitosis and necrosis.An inflammatory component,predominantly eosinophilic granulocytes,sometimes obscured the nature of the tumor.Ten patients are alive after a follow-up of 1-9 years.Based on immunohistochemistry,the endothelial cells in all cases were positive for CD31,CD34 and polyclonal factor Ⅷ antigen,whereas the scattered hyperplasia bile ducts were positive for cytokeratin 8 and cytokeratin 18.CONCLUSION:The clinical manifestations of IHE are non-specific.There is no significant correlation between histological type and prognosis.The clinicopathologic features of IHE in Chinese patients may provide a clue to further evidence-based studies.

Basaloid squamous carcinoma of esophagus:a clinicopathological,immunohistochemical and electron microscopic study of sixteen cases

AIM To further clarify the clinicopathological, immunohistochemical and electron microscopic features, and prognostic aspect of basaloid squamous carcinoma (BSC), a rare esophageal carcinoma.METHODS We reviewed 763 documented cases of esophageal malignancies from year (1977-1996) from our hospital, and discovered 16 (2.1%) cases of BSC. The clinicopathological features of these cases were evaluated. Immunohistochemistry (S-P method), histochemical stains, and electron microscopy were used to further characterize the neoplasm.RESULTS The tumors were classified into stages Ⅰ (n=1), ⅡA (n=6), ⅡB (n=2), Ⅲ (n=5), and Ⅳ (n=2) according to the criteria of the UICC TNM classification system of malignant tumors (1987). Most neoplasms were located in the mid third of the esophagus. Grossly, they had a similar appearance of conventional esophageal carcinoma, but showed a typical cytoarchitectural pattern of BSC histologically. The most important histologic feature of this tumor is carcinoma with a basaloid pattern, intimately associated with squamous cell carcinoma, dysplasia, or focal squamous differentiation. The basaloid cells were round to oval in shape with scant cytoplasm, arranged mainly in the form of solid, smooth-contoured lobules with peripheral palisading. A panel of immunostains were used for the basaloid component of the tumor with the following results: CK(Pan) 14/16 (+); EMA 16/16 (+); Vimentin 4/16 (+); S-100 protein 7/16 (+). CEA and smooth muscle actin were negative. Electron microscopy (EM) revealed that the basaloid cells were poorly differentiated, with a few desmosomes and fibrils, and numerous free and polyribosome. Of the 11 patients with adequate follow-up 8 died within 2 years, with an average survival time of 16.2 months. No stage Ⅱ, Ⅲ or Ⅳ cases survived beyond 5 years. The one-year survival rate was 60% and two-year 20%.CONCLUSION The BSC of esophagus is a distinct clinicopathological entity with poor prognosis. The cellular differentiation and biologic behavior of esophageal BSC were assumed to occupy a station intermediate between that of conventional squamous cell carcinoma and small undifferentiated cell carcinoma.