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Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a distinct type of lung cancer with histological profiles similar to nasopharyngeal carcinoma. The development is associated with EBV and plasma EBV DNA has predictive value in the progression and prognosis of PPLELC. PPLELC is different from some other types of lung cancer in that it has a low mutation rate of the classical lung cancer driver genes and targeted therapy is ineffective for it. Chemotherapy combined with immunotherapy may be the best first-line treatment option for patients with advanced PPLELC.
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Lymphoepithelioma is a rare clinical entity, first was described in nasopharynx, and have been reported in another organs. Lymphoepithelioma-like carcinoma (LEC) of the breast is an undifferentiated carcinoma. Approximately 32 cases have been described, and differential diagnoses include primary breast lymphoma and medullary carcinoma. We present a 57-year-old woman, diagnosed with LEC of the breast, treated with mastectomy, followed by adjuvant chemotherapy, radiotherapy, and hormone therapy, with a disease-free interval of 2 years. There is scarce evidence related to the best treatment choice in this histological type.
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Resumen El carcinoma similar al linfoepitelioma (LELC) del cuello uterino es una variante infrecuente del carcinoma de células escamosas. Se presenta el caso de una mujer con un tumor exofítico en el cuello uterino de consistencia aumentada, sangrado al tacto y parametrios libres de tumor clasificándose como etapa clínica IBI; el estudio histopatológico reportó LELC con recurrencia a distancia al año de seguimiento. Debido a su buen pronóstico existen pocos casos reportados de recurrencia posterior al tratamiento inicial.
Abstract Lymphoepithelioma-like carcinoma (LELC) of the cervix is an uncommon variant of squamous cell carcinoma. We present the case of a woman with an exophytic tumor on the cervix of increased consistency, bleeding to the touch and tumor-free parametriums classified as clinical stage IBI; the histopathological study reported LELC with distant recurrence at one year of follow-up. Due to its good prognosis, there are few reported cases of recurrence after initial treatment.
Subject(s)
Humans , Female , Carcinoma, Squamous Cell , Cervix Uteri , Women , NeoplasmsABSTRACT
Objective To investigate CT,clinical and pathological features of primary pulmonary lymphoepithelioma-like carcinoma (LELC). Methods The clinical,CT and pathological data of 22 patients with pulmonary LELC confirmed by pathology were analyzed retrospectively, including 8 males and 1 4 females whose ages were from 1 8 to 88 years old.Results Among 22 cases,1 2 patients were found by physical examination,2 patients had cough and sputum,7 patients had cough hemoptysis and 1 patient was found by re-examination after breast cancer surgery.On CT,all cases manifested single mass in the lung,and the longer diameters ranged from 1.5 cm to 1 1 cm,in which there were central type tumors in 8 cases and peripheral type tumors in 14 cases.Nineteen masses showed homogeneous density in non-enhanced scan,and the cavity,irregular calcification and small patchy necrosis were seen in each 1 case.After contrast-media injected,masses were mild-moderately or evidently enhanced in 7 cases and 1 5 cases respectively.Eighteen cases had well defined border and unclear border in 4 cases.Vascular encasement were found in 6 central type tumors,8 tumors squeezed peripheral vascular and 8 cases had clear demarcation between tumors and vascular.Nineteen tumors were surgically removed,in which 7 cases appeared metastasis of lymph node.Histopathology showed that the tumor cells had large volumes,unclear boundaries,large nuclei,growing in syncytial nest-like patches.There were abundant lymphocyte and plasma cell infiltration in tumor’s mesenchyma.EBER were done in 1 6 cases and the results were all positive.Conclusion CT feature of pulmonary LELC manifestes that the masses are mostly single,large size, homogeneous density,clearly boundary,shallow lobulated and significantly enhancement.Vascular encasement is the feature of central pulmonary LELC,and peripheral LELC is more likely to occur in the sub-pleural region.Final diagnosis should be combined with clinical and pathological diagnosis.
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Objective: To investigate CT findings of pulmonary lymphoepithelioma-like carcinoma (LELC). Methods CT findings of 244 patients with pathologically proved pulmonary LELC were analyzed retrospectively. All patients underwent nonenhancement CT scan, 226 of them also underwent contrast-enhanced CT scan. Results: Totally 132 (132/244, 54.10%) central tumors and 112 (112/244, 45.90%) peripheral tumors were identified. The maximum diameter of lesion was ranged from 1.2 cm to 13.1 cm, with average (5.42±2.64)cm. The lesions were characterized with 49 (49/244, 20.08%) nodules and 195 (195/244, 79.92%) masses. Totally 169 (169/244, 69.26%) tumors were found with well-defined borders, 183 (183/244, 75.00%) showed lobulations, and 104 (104/244, 42.62%) showed spiculation. The tumor located closely with the mediastinum in 184 cases (184/244, 75.41%), while pleural change of tail sign was observed in 111 cases (111/244, 45.49%). The tumor showed homogeneous density on nonenhancement CT in 158 cases (158/244, 64.75%), moderate enhancement in 160 cases (160/223, 71.75%), gradually enhancement in 145 cases (145/193, 75.13%), while 108 cases (108/193, 55.96%) showed abnormal small vessels and 81 cases (81/193, 41.97%) showed vascular encasements. Bronchial encasement and/or stenosis was found in 185 cases (185/244, 75.82%). Ground-glass opacity around the lesion was detected in 124 cases (124/244, 50.82%), when interstitial thickening was noticed in 81 cases (81/244, 33.20%) and obstructive pneumonia or pulmonary atelectasis was found in 132 cases (132/244, 54.10%). Hilar and/or mediastinal lymph node enlargement was found in 167 cases (167/244, 68.44%). Conclusion: Most pulmonary LELC occurres in right middle lobe and bilateral lower lobe of lung, and appeares as well-defined and slight lobulated tumor with enlarged lymph nodes. Vascular encasements, closely associated with mediastinum, pleural change of tail sign, ground-glass opacity and interstitial thickening are comparative characteristics of pulmonary LELC.
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@#Primary pulmonary lymphoepithelioma-like carcinoma (PPLELC) is a rare type of lung cancer with special characteristics of epidemiology, clinical diagnosis, treatment and prognosis. It has close relationship with Epstein-Barr virus (EBV) infection and has prominent regional feature. Most patients are young and non-smoking. There is no specificity of clinical manifestation. Most patients are asymptomatic at the time of diagnosis. As for treatment, the standard treatment for early stage disease is complete resection. Platinum-based doublet chemotherapy has been applied in locally advanced disease as the first line therapy. Due to its rarity, the treatment of advanced PPLELC is still lack of evidence of large sample randomized controlled trial. Whether target therapy or immunotherapy is effective is worth further study. This article reviews the research progress of PPLELC, to give a particular sight for clinicians and provides a better understanding of this rare tumor for researchers.
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A 69-year-old male smoker was referred to ¹⁸F-f luorodeoxyglucose (FDG) pos i t r on emi s sion tomography-computed tomography (PET/CT) with the indication of a suspicious solitary pulmonary nodule. FDG PET/CT determined a 2.5 × 2-cm soft-tissue lesion (SUVmax, 16.2) with spiculated margins in the hilum of the right lung. A 1-cm diameter lymphadenopathy on the right hilum with an SUVmax of 3.2 was also determined. Transbronchial biopsy of the right hilar lymphadenopathy did not reveal any malignant features in histopathologic examination, and it was determined to be reactive. The patient underwent a right upper lobe segmentectomy, and the histopathologic evaluation revealed that the tumor was a primary lymphoepithelioma-like carcinoma (LELC) of the lung with negative Epstein-Barr virus (EBV) on in situ hybridization studies. In this case report, we mainly focus on the FDG avidity of this very rare kind of tumor comparatively with previous reports and possible explanations of discordancy in FDG avidity in relation to histopathologic characteristics.
Subject(s)
Aged , Humans , Male , Biopsy , Herpesvirus 4, Human , In Situ Hybridization , Lung , Lung Neoplasms , Lymphatic Diseases , Mastectomy, Segmental , Positron Emission Tomography Computed Tomography , Solitary Pulmonary NoduleABSTRACT
Purpose To study the clinicopathological characteristics,treatments and prognosis of primary pulmonary lymphoepithelioma-like carcinoma (LELC).Methods Twenty cases of LELC were collected and the clinicopathological characteristics,immunohistochemical expression,survival data and clinical treatments were analyzed,with review of the literatures.Resuits Histopathologically,it was characterized by the syncytial pattern of growing with large vesicular nuclei,conspicuous nucleoli,and a marked lymphocytic infiltrate in the stroma that was similar to undifferentiated nasopharyngeal carcinoma.The immunohistochemical staining of CKpan showed positive expression in all cases (100％).Also the positive expression of in situ hybridization of Epstein-Bar virus-encoded RNA (EBER) were in all cases (100％) while the positive cases of CK5/6,p63 and p40 were 19,18,and 16 respectively.But gene mutations such as EGFR,K-RAS were negative.All the LELC patients in this cohort were survived until the deadline of follow-up.Comprehensive treatments were done such as surgical resection,radiotherapy and chemotherapy.Conclusion Primary pulmonary LELC is a rare disease that histomorphological analysis and immunohistochemistry are the main methods of diagnosis for it.It must be excluded the metastasis carcinoma from other locations.While both EGFR mutation and anaplastic lymphoma kinase (ALK)mutation could be commonly detected in non-small cell lung cancer,but seldom in LELC of lung.The high expression of PD1/PD-L1 may provide a rationale for immunotherapy in this subtype of lung cancer.Currently,experimental therapy is still taken in the pulmonary LELC.
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Lymphoepithelioma-like carcinoma (LELC) of the uterine cervix is exceedingly uncommon. We herein report a rare case of cervical LELC. A 45-year-old woman was admitted to gynecology department with vaginal bleeding for one month. Liquid-based cytology revealed atypical endometrial cells, not otherwise specified on her cervix. On a hysteroscopy, an endocervical mass was identified and the pathologic result was consistent with poorly differentiated squamous cell carcinoma. Magnetic resonance imaging and positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro-D-glucose integrated with computed tomography revealed a 3.1-cm endocervical mass without distant metastasis or enlarged lymph nodes. The International Federation of Gynecology and Obstetrics stage was IB1. A radical hysterectomy and bilateral pelvic lymph node dissection were performed. The pathologic diagnosis was a poorly differentiated carcinoma, showing features of LELC. She has been followed for 8 months without adjuvant treatment since the surgery, during which time there has been no evidence of tumor recurrence or metastasis.
Subject(s)
Female , Humans , Middle Aged , Carcinoma, Squamous Cell , Cervix Uteri , Diagnosis , Gynecology , Herpesvirus 4, Human , Hysterectomy , Hysteroscopy , Lymph Node Excision , Lymph Nodes , Magnetic Resonance Imaging , Neoplasm Metastasis , Obstetrics , Positron-Emission Tomography , Recurrence , Uterine Cervical Neoplasms , Uterine HemorrhageABSTRACT
Lymphoepithelioma-like carcinoma (LELC), an undifferentiated carcinoma with intense lymphoplasmacytic infiltrates, is commonly reported in the nasopharynx and occasionally in other organs. Pure type of LELC has previously been reported in the gallbladder. Mixed type could be reportable in comparison with other organs. Here we present a case of an 83-year-old man with mixed LELC and adenocarcinoma in the gallbladder. To the best of our knowledge, this is the first case of mixed LELC and adenocarcinoma in the gallbladder.
Subject(s)
Aged, 80 and over , Humans , Adenocarcinoma , Carcinoma , Gallbladder , NasopharynxABSTRACT
Lymphoepithelioma-like carcinoma (LELC) of the liver is uncommon, only 20 cases have been reported in the English-language literature so far, and the majority has been identified as cholangiocarcinomas, only four cases were hepatocellular LELC. Here we described a rare case of lymphoepitheliomalike hepatocellular carcinoma (HCC). A 42-year-old Chinese female who was incidentally found to have a liver-occupying lesion during a routine medical examination. Ultrasonography revealed a 47 mm × 33 mm × 36 mm hypoechoic mass in the left lobe. Computed tomography and magnetic resonance imaging displayed a nodular lesions in the left liver lobe. The patient underwent a left-side hepatectomy. Histopathological examination of the resected specimen revealed an undifferentiated carcinoma with a dense lymphocytic infiltrate, predominantly composed of CD3(+) T cells, morphologically similar to nasopharyngeal carcinoma. Immunohistochemically, the tumor cells were positive for CK, EMA, Glypican-3 and hepatocyte, but negative for alpha-fetoprotein, CK19, CK7 and CK20. Epstein–Barr virus (EBV) in situ hybridization was negative. The final histopathological diagnosis was lymphoepithelioma-like HCC without EBV infection.
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Objective To assess the diagnostic value of 18 F?FDG PET/CT imaging for primary pul?monary lymphoepithelioma?like carcinoma (LELC). Methods A total of 19 patients (10 males, 9 fe?males;average age 56.4 years) with suspected primary pulmonary LELC undergone whole?body 18F?FDG PET/CT and chest contrast CT scan were enrolled in this retrospective study. The SUVmax and uptake pat?terns of FDG, the histological types and the clinical stages were recorded. Image characteristics of contrast CT and FDG PET/CT were observed, and the diagnostic efficiency was calculated. Two?sample t test and one?way analysis of variance were used to analyze the results. Results The diagnostic sensitivity and spe?cificity of LELC were 12/13 and 4/6 for FDG PET/CT. The SUVmax of LELC lesions was significantly differ?ent from that of other lung malignant tumors or lung benign lesions (F=3.67, P<0?05). The lesion had longer diameter, lower CT density, higher SUVmax in stage Ⅳ patients, compared with that in stageⅠ-Ⅲpatients ( F=7.01, P<0.05) . The significant difference of SUVmax was found between lesions with ring up?take and lesions with other uptake types (3.94±0.67 vs 2.86±0.35; t=4.07, P<0?05). The SUVmax was higher in Schmincke tumors when compared with that in tumors of Regaud subtype ( 3. 61 ± 0. 71 vs 2. 76 ± 0?29; t=2.99, P<0.05) . Conclusion 18 F?FDG PET/CT could be an accurate method in differential diag?noses of primary pulmonary LELC.
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Due to differences in prognosis and management, it is important to subclassify esophageal carcinoma. Esophageal lymphoepithelioma-like carcinoma (LELC) is extremely rare, with only a few cases reported to date. Review of the literature revealed case reports describing lesions with similar histology. We present a 69-year-old man with a giant pedunculated-polypoid lesion of the esophagus shrinking the lumen. Endoscopic excision of the tumor was performed and final histopathological diagnosis was confirmed to be LELC. In contrast to a previous case with a more aggressive course and a recurrent lesion, our patient died of his disease within 8 months of diagnosis. Here we discuss the endoscopic and radiologic findings of the case and a review of the literature.
Subject(s)
Aged , Humans , Diagnosis , Esophagus , PrognosisABSTRACT
Objective To analyze and summarize the clinical-pathological features,immunophenotype and prognosis of the lymphoepithelioma-like gastric carcinoma (LELGC).Methods The clinical,radiographic and histological data of four patients with LELGC were retrospectively analyzed.The expression of cytokeratin (CK),CD20,CD3,CD4,CD8,E-cadherin,β-catenin,bcl-2,p16,p53,p63,c-erbB-2,cyclin D1,Ki67 and DNA methyl-transferase 1 (DNMT1) in tumor was detected by Epstein-Barr virus-encoded small RNA (EBER) in situ hybridization and immunohistochemical methods.Results The size of four tumor was 1.8 cm× 1.6 cm,1.5 cm× 2.0 cm,2.5 cm× 2.0 cm and 4.0 cm× 2.5 cm.Under light microscope,tumor cells appeared like cords,small lumps or scattered single infiltration with vacuolized nucleus; clear nucleoli and obvious interstitial lymphocytic infiltration.The results of immunohistochemical examination indicated that in four tumors CK was positive in membrane of all the tumor cells,while EBER was positive in all cell nucleus.The number of lymphocytes with CD3 positive was over those with CD20 positive,which was mainly CD8 positive lymphocytes.The percentage of E cadherin and β-catenin positive in the cell membrane of four tumors was between 10 ％ and 90 ％,and two cases with β-catenin positive in cytoplasm.The expressions of DNMT1,cyclin D1 and bcl-2 were all positive,while p16 and c-erbB-2 were all negative.The expression of p63 was positive in only one case,and p53 was negative in one case.The percentage of Ki67 positive was 40％,15％,60％ and 40％,respectively.Conclusions LELGC is a rare neoplasm with better prognosis.The features of clinical pathology and immunohistology may help to make a correct diagnosis.
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Purpose To study the clinic-pathologic features, differential diagnosis and prognosis of lymphoepithelioma-like carcinoma of cervix. Methods Eight cases of lymphoepithelioma-like carcinoma of cervix were retrieved from our institution. HE staining, im-munohistochemistry and in situ hybridization were perform on those cases. Results Of the 8 cases of lymphoepithelioma-like carcino-ma,the median age was 44 years old ( ranged from 29-67 years) and all of the clinical stage belonged toⅠB. Grossly, 5 cases presen-ted a cauliflower-like appearance, 1 case grew as a polyp protrusion, and the other 2 cases showed a non-specified thicken change of cervix. Histologically, the tumors were composed of undifferentiated large cells, either loosely or closely-packed, with the nested and trabecular growth pattern. The tumor cells were generally oval or polygonal in shape, while the demarcation between the cells was indis-tinct and form the syncytial appearance. The nuclei were vesicular and had one or more prominent nucleoli. There were abundant infil-trates of lymphocyte and plasma cells among the tumor nests. The tumor cells showed positive expression of CK in 100% of cases, p63 in 37. 5% of cases, p16 in 62. 5% of cases, CK5/6 in 75% of cases, Ki-67 in 10% ~30% of cases. The stromal lymphocytes mainly expressed CD3 and CD8. EBER was negative in the entire tumor cells while was weakly positive in the stromal lymphocytes in one case. The follow-up data, which terminated at June 2013, demonstrate 7 cases were alive and 1 case died of the disease. Conclusions Lymphoepithelioma-like carcinoma occurred of cervix is rare. They have distinctive morphologic features and overall favorable prog-nosis. The accurate diagnosis is dependant on the combination of the histologic and immunohistochemical examination.
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El cáncer linfoepitelial del cuello uterino es excepcional en nuestro continente, el propósito de la presente publicación es evaluar la etiología, diagnóstico y tratamiento de un caso clínico. El estudio anátomo-patológico ofreció ciertas dificultades, por lo que se recurrió a la inmunohistoquímica para hacer el diagnóstico diferencial con el carcinoma epidermoide, el carcinoide de células grandes y el linfoma anaplásico. El compromiso del virus de Epstein-Barr y el virus del Papiloma Humano, con el cáncer linfoepitelial, no fueron con-cluyentes. Se encontró tinción positiva granular en el citoplasma de células epiteliodeas aisladas en relación al primero, e indicios de daño coilocitico en el epitelio pavimentoso en relación al segundo. La enfermedad se presentó como lesión única sangrante en el labio anterior del cuello uterino. El tratamiento fue quirúrgico con erradicación completa, lo que favorece el pronóstico.
Linfoepitelial cancer of the cervix is exceptional in our continent. The purpose of this publication is to evaluate the etiology, diagnosis and treatment. Anatomical and pathological study offered certain difficulties; immunohistochemistry was used to make the differential diagnosis with squamous carcinoma, large cell carcinoid and anaplastic lymphoma. Compromise of Epstein - Barr virus and virus of Human Papilloma, with linfoepitelial cancer, were not conclusive. We found positive granular staining in the cytoplasm of isolated epithelioid cells in relation to the first virus and koilocytic damage of the squamous epithelium in regard to the second. The disease was presented as unique, bloody injury, in the anterior lip of the cervix. The treatment was surgical with complete eradication, which favors the prognosis.
Subject(s)
Humans , Female , Adult , Carcinoma, Squamous Cell/surgery , Carcinoma, Squamous Cell/diagnosis , Uterine Cervical Neoplasms/surgery , Uterine Cervical Neoplasms/diagnosis , Diagnosis, Differential , ImmunohistochemistryABSTRACT
Gastric lymphoepithelioma-like carcinoma is a very rare cancer of the stomach. It has undifferentiated malignant cells with rich lymphoid stroma histologically and its prognosis has known to be relatively fair. Recently, there were some reports on the successful removal of the lesion by endoscopic submucosal dissection technique. We present a case of 70 years old man who had 2 cm sized early gastric cancer of lymphoepithelioma-like carcinoma with perigastric lymph node metastasis, and also review the relevant medical literature.
Subject(s)
Lymph Nodes , Neoplasm Metastasis , Prognosis , Stomach NeoplasmsABSTRACT
Gastric lymphoepithelioma-like carcinoma is a rare carcinoma among gastric malignant tumor but has a good prognosis. The carcinoma has histologic feature characterized by small nest of cancer cells mixed with lymphoid stroma. We report a case with lymphoepithelioma-like carcinoma of stomach initially presenting as panperitonitis because of spontaneous tumor perforation. A 56-year-old man visited our emergency room because of epigastric pain. A preoperative abdominal CT scan showed a massive pneumoperitoneum in the upper abdomen, and the presence of gastric cancer in the lesser curvature of the stomach. An emergent laparotomy was performed followed by radical subtotal gastrectomy. Pathologic examination revealed that the tumor was a lymphoepithelioma-like gastric carcinoma.
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Humans , Male , Middle Aged , Carcinoma/diagnosis , Combined Modality Therapy , Lymphoma/diagnostic imaging , Pneumoperitoneum/etiology , Rupture, Spontaneous , Stomach Neoplasms/complications , Stomach Rupture/complications , Tomography, X-Ray ComputedABSTRACT
Lymphoepithelioma-like carcinoma is a neoplasm at extranasal sites with striking histological similarity to lymphoepithelioma arising in the nasopharynx. The tumor is composed of islands of pleomorphic cells with a lymphocytic infiltrate. The head and neck are the principal locations for this tumor, which occurs in the skin. Differential diagnoses include squamous cell carcinoma, Merkel cell tumor, lymphoma, cutaneous lymphadenoma, malignant melanoma, and metastatic lymphoepithelioma. Here, we report a case of lymphoepithelioma-like carcinoma of the skin in a 78 year-old male who had a firm nodule on the right upper eyelid. He was treated with wide excision and local flap coverage. The patient had been disease-free for more than 1 year.