A Unique Case of Primary EBV-Positive,HPV-Negative Nasopharyngeal Carcinoma Located in the Tonsil

Nasopharyngeal carcinomas (NPC) are non-keratinizing squamous cell carcinomas of the nasopharynx associated with Epstein-Barr virus (EBV). When occurring outside of the nasopharynx, they are referred to as lymphoepithelioma-like carcinomas (LELCs) and present the same morphology as NPC. LELC have been described in other head and neck regions such as the salivary glands and the soft palate. LELC can also occur in the oropharynx, are associated with human papillomavirus (HPV) and are typically negative for EBV. We herein present a unique case of a 78-year-old Chinese male with EBV-positive, HPV-negative NPC of the left tonsil. His presenting symptom was a left-sided lymph node. There was no evidence of nasopharyngeal lesion seen on physical examination, PET and MRI. The patient was treated with curative-intent external beam radiotherapy which delivered 70 Gy (Gy) to the gross tumour and lymph nodes, and 56 Gy electively to the ipsilateral neck using a volumetric modulated arc therapy technique. This is the first case of primary tonsil EBV-positive NPC described in the literature.     

Lymphoepithelioma-like carcinoma of the renal pelvis

Lymphoepithelioma-like carcinoma (LELC), best known to occur in the nasopharynx, can arise in a variety of sites, such as the salivary gland, thymus, lung, stomach, skin and uroepithelium. Primary LELC of the uroepithelium is very rare and there is only limited information in the published reports. We managed a case of a 75-year-old woman who presented with nausea and gross painless hematuria. She was treated with laparoscopic nephroureterectomy and was diagnosed with a T1N1M0 LELC of the renal pelvis. Unlike nasopharyngeal lymphoepithelioma, immunohistochemical analysis of this urinary LELC was negative for the Epstein-Barr virus. Herein we report on one more case of primary LELC of the renal pelvis and review of the published reports, particularly those concerning Epstein-Barr virus expressions. Recognition of this tumor and complete resection are essential for saving patients.     

Lymphoepithelioma-Like Carcinoma in the Trachea: Report of a Case

Lymphoepithelioma-like carcinoma (LELC) is a non-nasopharyngeal undifferentiated carcinoma with prominent lymphoid infiltration. To our knowledge, only two cases of LELC in the trachea have ever been reported. This tumor has a strong association with the Epstein–Barr virus (EBV), which is especially prevalent in Asians and absent in Caucasians. We report a case of tracheal LELC with EBV-association in a 22-year-old man. The patient was treated with tracheal resection and anastomosis, followed later by adjuvant radiotherapy.     

原发性输尿管淋巴上皮样癌一例报告并文献复习

目的 探讨原发性上尿路淋巴上皮样癌的临床表现、病理特点、治疗和预后. 方法 原发性输尿管淋巴上皮样癌患者1例.女,81岁.间断无痛性全程肉眼血尿1个月就诊.CT尿路造影提示左输尿管盆腔段腔内可见长约2.5 cm类圆形软组织密度影.行腹腔镜下左输尿管癌根治术. 结果大体标本:纵行打开输尿管,距输尿管远端3.5 cm处见2.5 cm×2.0 cm隆起肿物,切面灰白,质稍硬,实性.镜检:肿瘤细胞呈巢状分布,体积较大,胞质丰富,核大,圆形,空泡状,核仁明显,间质内大量小淋巴细胞浸润.病理诊断:输尿管淋巴上皮样癌,浸及深肌层,病理分期pT2N0M0.原位杂交技术EBER为阴性.随诊3个月未见肿瘤复发及转移. 结论原发性上尿路淋巴上皮样癌罕见,需经病理组织学检查确诊.根治手术为主要治疗手段,预后优于其他类型尿路上皮癌.     

Lymphoepithelioma-like cholangiocarcinoma: an Epstein-Barr virus-associated tumor

Epstein-Barr virus (EBV) has been linked to carcinomas of several body sites, especially of the nasopharynx, salivary gland, lung, and stomach. We present five cases of lymphoepithelioma-like cholangiocarcinoma, including one that had been previously reported. Two patients were men and three were women. Their ages ranged from 42 to 66 years. Histologically, all five tumors were composed of variable proportions of undifferentiated epithelial cells and glandular components in a lymphocyte-rich stroma. EBV was detected in all five tumors by in situ hybridization for EBER-1 in both lymphoepithelioma-like carcinoma (LELC) and glandular parts, but not in 36 cases of cholangiocarcinoma without the LELC component. Taken together, these observations indicate that lymphoepithelioma-like cholangiocarcinoma is strongly linked to EBV. The LELC type of cholangiocarcinoma, like LELC of other body sites, may be more common in areas with endemic EBV infection.     

乳腺原发性鳞状细胞癌1例报道

目的 乳腺原发性鳞状细胞癌病例报道及文献综述。 方法 对作者近期发现右乳腺原发性鳞状细胞癌病例进行分析。结果 增生的瘤细胞呈多角形,胞浆丰富,明显核异型,核分裂易见,细胞角化,可见明显角化珠和细胞间桥,肿瘤旁见多个癌细胞弥漫浸润,部分呈小叶癌化,符合乳腺鳞状细胞癌病理诊断。结论 乳腺原发性鳞状细胞癌是一种罕见的乳房恶性肿瘤,同其它乳腺肿瘤一样,早期发现、早期诊断、及早治疗是提高本病疗效的关键。     

Lymphoepithelioma-Like Carcinoma of the Urinary Bladder: A Case Report and Discussion of Differential Diagnosis

Lymphoepithelioma-like carcinoma (LELC) of the urinary bladder is very rare. It is mandatory to make differential diagnosis among lymphoma, chronic cystitis and LELC because of different therapeutic approach. A bladder tumor was found in a 90-year-old patient suffering from hematuria. After transurethral resection, undifferentiated tumor with prominent lymphoid infiltration was seen on light microscopy. Immunohistochemical examination demonstrated positive staining of tumor cells with cytokeratin (CK), epithelial membrane antigen and CK-20. We presented the case because of its rarity and related literature was reviewed.     

Primary pulmonary lymphoepithelioma-like carcinoma with positive expression of Epstein-Barr virus and PD-L1: A case report

Introduction and importancePulmonary lymphoepithelioma-like carcinoma (LELC) is a rare type of non-small cell lung cancer (NSCLC) that is classified as a subtype of unclassified carcinoma by the WHO. LELC is usually associated with Epstein-Barr virus (EBV) infection. LELC has often been observed in Southeast Asia; however, it is extremely rare in Japan.Case presentationA 60-year-old Japanese woman presented with an abnormal shadow in the left lung on chest radiography. Chest computed tomography showed a nodule located between the lingular and basal anteromedial segments. A blood test suggested an existing EBV infection, and LELC was suspected preoperatively in the transbronchial lung biopsy. She underwent a lingular and basal bi-segmentectomy. The EBV-encoded small ribonucleic acid in-situ hybridization (EBER-ISH) was positive, and she was diagnosed with LELC. Moreover, programmed death-ligand 1 (PD-L1) expression was moderately positive. No recurrence was observed for 30 months.Clinical discussionAlthough LELC has been reported as a low-grade malignancy with a good prognosis, the frequency of PD-L1 expression in LELC seems to be higher than that in other NSCLCs. Moreover, it has been reported that LELC patients with high PD-L1 expression are likely to have early recurrence/metastasis and poor prognosis.ConclusionAn investigation of PD-L1 expression for LELC would be useful considering the benefit of PD-1/PD-L1 blockade in patients with pulmonary LELC with high PD-L1 expression. The present case is the first report of LELC with positive expression of EBER-ISH and PD-L1 in Japan.     

Myoepithelial Carcinoma: A Rare Neoplasm of the Breast

BACKGROUND: Malignant myoepitheliomas of the breast are extremely rare. There has been a limited number of published reports of myoepithelial carcinomas originating from the breast. CASE REPORT: We describe a malignant myoepithelioma of the breast in a 56-year-old woman. Histological examination showed polygonal epithelioid cells and spindle cells with moderate to marked nuclear atypia. Immunohistochemistry showed reactivity in the spindle cells for smooth muscle actin, cytokeratin (AE1/AE3), and p63, indicating a myoepithelial cell lineage of tumor cells. The patient underwent radical surgical excision of the lesion and axillary lymph node dissection. She demonstrated no evidence of recurrence over an 11-month follow-up. CONCLUSIONS: We suggest myoepithelial carcinomas of the breast be managed with appropriate surgical clearance. A multidisciplinary approach is usually required.     

Primary lymphoepithelioma-like carcinoma of the urinary bladder: Case report and literature review

A lymphoepithelioma-like carcinoma (LELC) is a rare neoplasm of the urinary bladder. A 71-year-old man presented with gross hematuria for several weeks. On cystoscopy, a solid sessile tumor was observed in the bladder dome. A partial cystectomy was performed. Histopathological findings showed that the lesion was a pure LELC of the urinary bladder. The patient was followed up for 24 months without tumor recurrence. We present the case to highlight the significance of differentiating this rare tumor and discuss the prognosis and treatment options.     

Oral Adult Rhabdomyoma

This report describes a case of an adult rhabdomyoma (ARM) occurring in the oral cavity. A 47-year-old man was referred for the diagnosis of a painless, well-circumscribed, submucous nodule located on the floor of the mouth, measuring approximately 6.0 cm in length. Computed tomography revealed a well-defined, solid, and hypodense mass. A benign salivary gland or mesenchymal tumor were the main diagnostic hypotheses. Under local anesthesia, the patient underwent surgical excision. Microscopically, the tumor comprised large polygonal well-defined cells with abundant, eosinophilic granular cytoplasm with cross striations. No atypia or mitosis was observed. The cells were positive for muscle-specific actin, desmin, and sarcomeric alpha-actin. Based on these features, a diagnosis of ARM was established. No recurrence was observed after 48 months. Although rare, ARM should be considered in the differential diagnosis of oral submucosal nodules, especially those located on the floor of the mouth.     

Intraductal growth of malignant mammary myoepithelioma.

This report describes the histologic and immunohistologic features of an intraductal myoepithelial tumor that developed in the breast of a 61-year-old woman. Histologically, the tumor proliferated intraductally, with both a comedo or doughnut pattern and a solid pattern containing narrow fibrovascular cores, mimicking what appeared to be a conventional intraductal carcinoma. No fine papillary or arborizing growth or cribriform formation was observed. Tumor cells at the ductal peripheral zone were polygonal and clear with abundant glycogen in the cytoplasm; they were transformed into nonclear cells with slightly eosinophilic cytoplasm toward the center of the involved ducts. Occasionally, nonclear cells were elongated, with a centrally located cigar-shaped nucleus. These elongated or spindle cells tended to show a fascicular and streaming pattern similar to that of a smooth muscle tumor. Immunohistochemically, alpha smooth muscle actin (alpha-SM-actin) and S-100 protein were expressed in most of the nonclear cells. While clear cells also had a positive reaction for S-100 protein, they were mostly negative or barely positive for alpha-SM-actin. Epithelial membrane antigen (EMA) was also positive in a certain number of polygonal cells. These results support the myoepithelial nature of the present tumor, and some cells might also be immunologically differentiated into ductal epithelial cells. In addition to cytological atypia, frequent mitoses, and central necrosis within ducts, there was a minimal but evident stromal invasion, suggesting histological malignancy in this peculiar tumor.     

Is there a spectrum of cytologic atypia in type a thymomas analogous to that seen in type B thymomas? A pilot study of 13 cases

Thirteen cases of type A thymoma are reported showing the characteristic architectural attributes of this tumor type (World Health Organization), such as lobulation, perivascular spaces, gland-like formations, and cystic changes, but also displaying atypical features, as defined by increased mitotic activity, mild to moderate nuclear atypia, and/or scattered small foci of necrosis. The tumors were similar to type B3 thymomas in many respects, except for the fact that the tumor cells were spindled instead of round/polygonal. The existence of these cases suggests the desirability of an expansion of the type A thymoma category, analogous to that currently used for type B tumors. The various nomenclature options that could be used to name these tumors have been discussed.     

Intrahepatic Cholangiocarcinoma With Lymphoepithelioma-like Carcinoma Components Not Associated With Epstein-Barr Virus: Report of a Case

A carcinoma displaying undifferentiated features with dense lymphoplasmacytic infiltration is defined as lymphoepithelioma-like carcinoma (LELC). Intrahepatic cholangiocarcinoma (ICC) with LELC components is rare, and most LELCs are associated with Epstein-Barr virus (EBV). We report here on a case of ICC with LELC components not associated with EBV. A 65-year-old woman was incidentally found to have a hepatic tumor in the caudate lobe. An extended right hepatectomy with lymphadenectomy was performed. Histologically, the tumor was mainly composed of large undifferentiated epithelial cells with vesicular nuclei, prominent nucleoli, indistinct cell borders, and heavy small lymphocytic infiltration, which are the characteristic features of LELC. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin 19 but were negative for glypican 3. In situ hybridization using EBV-encoded RNA was negative. Therefore, a diagnosis of ICC with LELC components not associated with EBV was made. Because there is limited information available regarding the prognosis and treatment of ICC with LELC components because of the limited number of reported cases, additional studies will be needed to clarify the clinicopathologic features of this disease.Key words: Lymphoepithelioma-like carcinoma, Cholangiocarcinoma, Epstein-Barr virus, LiverLymphoepithelioma-like carcinomas (LELCs) are tumors that are composed of undifferentiated carcinoma with an intense lymphocytic infiltrate, and they have histomorphologic features identical to those of undifferentiated nasopharyngeal carcinomas. These tumors have been reported in various anatomic sites, such as the stomach, salivary gland, and thymus.¹ The occurrence of LELC in the hepatobiliary tract is rare. Most LELCs have been reported to be associated with the Epstein-Barr virus (EBV).^1–4 To the best of our knowledge, only 6 cases of intrahepatic cholangiocarcinoma (ICC) with LELC components not associated with EBV infection have been reported,^5–10 and only 2 cases with lymph node metastases at the time of surgery have been reported.^5,9 In this paper, we report on an unusual case of an ICC with LELC components accompanied by lymph node metastases not associated with EBV infection.     

Cancer of the Nasopharynx: Functional Surgical Salvage

The primary treatment modality for nasopharyngeal carcinoma is radiotherapy (RT). When tumor persists or recurs at the nasopharynx or in the neck after irradiation, then further courses of radiotherapy are associated with recognized morbidities. The swallowing, speech abilities as well as the cervical musculoskeletal functions of the patient may be significantly affected. Surgical salvage of neck disease with radical neck dissection contributes to better tumor control with acceptable morbidity. For localized tumor in the nasopharynx, adequate extirpation of the disease together with the removal of paranasopharyngeal tissue is possible with the anterolateral approach. The operation is not difficult and the associated morbidity is minimal. After a median follow up of 3 years, the 82 patients who underwent this operation demonstrate that with adequate exposure and surgical salvage, satisfactory tumor control rate with good functional outcome is possible.     

肾脏淋巴上皮瘤样癌(附1例报告并文献复习)

目的提高对肾脏淋巴上皮瘤样癌(LELC)的认识,探讨其诊治方法。方法诊治1例LELC患者,并复习有关文献进行讨论。结果行根治性肾切除,病理证实为LELC;免疫组织化学染色:CK( ),CD68(-),LMP( );原位杂交:EBER(-)。拒绝化疗,术后3个月死于肿瘤复发并转移。结论LELC比较罕见,其临床表现无特异性,确诊依靠病理组织学检查;早发现、及早手术、术后联合化疗有望提高其疗效。     

The giant cavernous hemangioma of the neck]

A 67-year-old female who had complains of dyspnea and giant mass in the right neck. The plain CT scan and selective angiography showed an giant mass in the neck and extended into antero-superior mediastinum which pressed intrathoracic trachea. The tumor was resected completely by oblique neck incision and partial median sternotomy. It was 15 x 9 x 5 cm in size. The histological diagnosis was cavernous hemangioma. The giant cavernous hemangioma in neck extended into mediastinum is rare.     

Epithelioid angiomyolipoma of the kidney

Epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, which is characterized by the presence of polygonal cells with densely eosinophilic cytoplasm and varying degrees of nuclear atypia. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported in the literature. We report a case of epithelioid angiomyolipoma of the kidney that occurred in a 38-year-old woman. The tumor was composed of diffuse sheets of epithelioid cells, adipocytes and only scattered thick-walled blood vessels. The epithelioid cells had pleomorphic and hyperchromatic nuclei with densely eosinophilic cytoplasm. Hemorrhage, necrotic foci and clusters of foamy macrophages were present. HMB-45, CD117 (c-kit) and CD68 were detected in the epithelioid cells. There was no expression of cytokeratin, epithelial membrane antigen or desmin. The patient showed no evidence of recurrence or metastatic disease 9 months after nephrectomy.     

Ciliated Adenosquamous Carcinoma: Expanding the Phenotypic Diversity of Human Papillomavirus-Associated Tumors

This study describes a unique subset of ciliated, human papillomavirus (HPV) related, adenosquamous carcinomas (AsqCA) of the head and neck that in contrast to most AsqCA, often show areas with lower grade cytonuclear features. They are comprised of largely non-keratinizing squamous cell carcinoma components with cystic change, gland formation, mucin production, and cilia in tumor cells. Seven cases of ciliated AsqCA were retrieved. Site distribution was as follows: palatine tonsil—3/7, base of tongue—1/7, and neck (unknown primary site)—3/7. Despite the occasional resemblance to mucoepidermoid carcinoma (MEC), the tumors showed focal keratinizing morphology and atypia, and all tumors were negative for MAML2 rearrangements. Oropharyngeal and neck tumors were uniformly p16 positive and showed punctate staining by in situ hybridization for high risk HPV DNA. There were two distant metastases (lung), and one tumor related death. Thus, ciliated AsqCA are HPV-associated lesions that pose unique pitfalls, closely mimicking MEC and other salivary gland tumors. These tumors add to the list of those which defy the dogma that ciliated epithelium always equates to a benign process.     

Lymphoepithelioma-like carcinoma of the lung

Primary lymphoepithelioma-like carcinoma (LELC) of the lung is a very rare disease. There is very little long term follow-up data about this disease. A 60-year-old woman was found to have abnormalities according to a routine chest X-ray examination. She was admitted to our hospital in March 1998. Since a malignant tumor of the right lung was suspected, surgical resection was performed in April of the same year. Pathological diagnosis was LELC of the lung. Its pathological stage was T₁N₀M₉ stage I_A. It has been reported that this carcinoma, is associated with Epstein-Barr virus (EBV) infection. However, the patient's tumor cells were negative for EBV as examined with RNAin situ hybridization technique. She is alive and has been free from recurrence of the disease over 7 years postoperatively.