胰腺腺泡细胞癌1例报告

胰腺腺泡细胞癌(pancreatic acinar cell carcinoma, ACC)属胰腺外分泌肿瘤,比较罕见,有关其治疗和诊断方面的信息有限,文献仅是零星的个案报道。我科近期对一例胰头占位性病变实施手术后病理证实为胰腺腺泡细胞癌,其诊治经过如下。     

食管小细胞癌的诊断与治疗进展

正原发性食管小细胞癌属于食管神经内分泌肿瘤,是一种罕见的发生于食管的高度恶性肿瘤。小细胞癌可发生于体内的任何组织器官,但主要发生于肺。仅有约2.5%发生于肺外器官。食管小细胞癌是最为常见的肺外小细胞癌,约占53.0%~71.0%~([1]),恶性程度高,易早期转移,预后差。一、食管小细胞癌的流行病学食管小细胞癌的发生年龄60~70岁,男性发病率高于女性2倍以上~([2])。西方人群中原发性食管小细胞     

乳腺涎腺源性肿瘤研究进展

乳腺和涎腺均为管泡状外分泌腺,乳腺部分肿瘤具有涎腺类肿瘤的形态特征[1].Hamperl等[2]把乳腺内涎腺类肿瘤分为两型:肌上皮分化的肿瘤和非肌上皮分化的肿瘤.前者占多数,包括良性腺肌上皮瘤、多形性腺瘤、腺样囊性癌、低度恶性腺鳞癌、恶性肌上皮瘤,后者包括腺泡细胞癌、嗜酸细胞癌和黏液表皮样癌,这些肿瘤都是乳腺内少见的肿瘤.     

原发性食管小细胞癌3例报道及文献复习

肺外表现小细胞癌甚为少见，原发于食管小细胞癌更为少见，文献报告较少。其发生率占食管癌的0．05％-7．60％。原发性食管小细胞癌(PESC)是一种恶性肿瘤。其临床表现、X线征象等与食管鳞腺癌相似，但其生物学行为及治疗等有别于食管鳞癌及腺癌，鉴于该肿瘤罕见，在治疗方面尚未受到应有的重视，治疗方案比较混乱，大多数套用食管鳞癌或腺癌的治疗方法，预后极差。本文报道我院近5年收治的3例PESC并结合文献对该病的生物学特征及治疗进行讨论。     

原发性睾丸类癌1例报告并文献复习

原发性睾丸类癌是一种少见、低度恶性的肿瘤,多发于中老年人,以40～60岁多见.1954年Simon首次报道以来,目前国外文献共报道90余例睾丸原发性类癌,至2009年,国内文献报道近10例.本院于2009年收治1例原发性睾丸类癌患者.     

肝脏血管周上皮样细胞肿瘤临床分析

目的 提高对罕见的肝脏原发性肿瘤——肝脏血管周上皮样细胞肿瘤(PEComa)的诊疗水平.方法 采用文献检索收集1989 ～ 2013年间报道的包括北京大学人民医院1例在内资料齐全的原发性肝脏PEComa患者41例,总结其病例资料,对原发性肝脏PEComa的临床特点、影像学资料、病理特点、治疗及预后进行回顾性分析.结果 肝脏PEComa无特异性临床表现,影像学特征不易识别,主要依靠术后病理确诊.手术是目前公认的主要治疗方法.41例患者中34例为良性,7例为恶性,恶性者预后较差.结论 原发性肝脏PEComa是一种肝脏罕见肿瘤,应提高对其认识和诊疗水平,术后应长期密切随访.     

前列腺小细胞癌诊治特点分析(附2例报告并文献复习)

小细胞癌多发生在肺部,肺外较罕见,原发于前列腺的小细胞癌罕见,肿瘤分化低、易转移,无有效治疗手段,预后极差[1].2006年至2010年我院收治2例,现报道如下.     

Merkel细胞癌一例

Merkel细胞癌（merkel cell carcinoma,MCC）也称小梁癌（trabecular carcinoma），原发性小细胞癌或皮肤神经内分泌癌，是一种少见的高度恶性的肿瘤，属于APUD（amime precursor uptake and decarboxylation）系统肿瘤，临床上较为少见。我科近期收治1例右面颊部MCC患者，现报道如下。     

原发性前列腺印戒细胞癌伴腺泡状腺癌变异型1例报告

正原发性前列腺印戒细胞癌(signet-ring cell carcinoma,SRCC)伴腺泡状癌变异型临床罕见,国内外仅少量报道,是一种特殊的、恶性程度较高的肿瘤,病情进展较快、预后较差。昆明医科大学第二附属医院泌尿外科收治1例,现结合文献报道如下。1病例资料患者男性,58岁,因进行性排尿困难1年,加重2月余于2019年8月6日入院。近2个月以来尿线变细明显,伴排尿踌躇及尿不尽,无尿失禁、排尿中断及肉眼血尿的情况发生,偶伴尿频、尿急、尿痛。查体:     

小肠原发鳞状细胞癌导致肠穿孔1例

在消化道肿瘤中,原发性小肠肿瘤发病率较低,多发生于十二指肠,其次为空肠及结肠,发病隐匿,严重影响患者生命健康.因来自小肠的肿瘤比较罕见,大多数是腺癌和类癌 [1].原发于小肠的鳞状细胞癌更为罕见,国内外无系统的研究和报道,仅有少数小肠鳞状细胞癌的罕见病例在文献中报道 [1].小肠肿瘤占胃肠道肿瘤的1％～2％,虽然小肠癌...     

Successful treatment of adrenocortical carcinoma with pulmonary metastasis in a child: Report of a case

Adrenocortical carcinoma (ACC) in childhood is rare: in 2002, only three new cases were reported in Japan. Although there is no established therapeutic management system, margin-free complete surgical excision of the tumor is very important to a satisfactory outcome. We report a case of ACC with bilateral pulmonary metastasis in a 10-year-old boy. Preoperative chest computed tomography (CT) showed pulmonary metastasis with near-water density. The patient was treated successfully with complete surgical resection of the primary tumor and adjuvant chemotherapy, including mitotane. After bilateral lung surgery for the metastatic lesions, the plasma dehydroepiandrosterone sulfate level became negative.     

Primary Pituitary Adenoid Cystic Carcinoma: A Rare Salivary Gland-Like Tumor in the Sella

Adenoid cystic carcinoma (ACC) is a tumor of the exocrine glands that originates primarily from the minor and major salivary glands, nasopharynx, and lacrimal glands. ACC grows slowly but is locally aggressive and prone to recurrence. It is uncommon for ACCs to develop in the pituitary gland as a primary tumor. We present a case of primary pituitary ACC extending to the sphenoid sinus resembling an invasive adenoma in a 71-year-old woman with a history of nasal epistaxis. We reviewed other reported cases of pituitary ACCs with retrospective validation of whether the tumor was primary or not. The intrasellar tumor exhibited MYB rearrangement with enlargement and destruction of the sella turcica and dural tears toward the sphenoid sinus, which would be consistent for a tumor originating from the pituitary gland. Including our case, only four intrasellar and one suprasellar ACC have been confirmed as primary tumors. All intrasellar ACCs had the characteristic of some form of invasion of neighboring structures with evidence of hyperprolactinemia. ACC could develop in the pituitary gland as a form of salivary gland-like tumor derived from the ectopic salivary gland rests. ACCs rarely arise from the pituitary gland; however, the accurate determination of primary occurrence is difficult because of the invasive nature of these tumors, and the anatomical relationship with the sphenoid sinus and the cavernous sinus. Excessive bleeding from the tumor or unexpected internal carotid artery injury should be avoided during surgery for primary and secondary pituitary ACC.     

High Grade Transformation in Adenoid Cystic Carcinoma of the Parotid: Report of a Case with Cytologic, Histologic and Immunohistochemical Study

Adenoid cystic carcinoma (ACC) constitutes about 4% of salivary epithelial tumors and is the second common malignant epithelial salivary gland tumor involving both the major and minor salivary glands. High grade transformation in ACC is a recently recognized entity with only a few cases reported in literature. We report the first case of ACC with high grade transformation involving the parotid. A 54-year-old man with a history of right parotid painful swelling from 1.5 years presented with recent increase in size of the swelling and facial paresis of 2 months duration. The locally invasive salivary neoplasm was composed predominantly of an undifferentiated carcinoma with foci of conventional ACC occupying less than 10% of tumor area. Immunohistochemical study of the undifferentiated component as compared to conventional ACC showed greater over-expression of p53 and Ki-67. Her-2/Neu was negative in both components. Recognition of occasional clusters of basaloid cells and hyaline globules in association with larger poorly differentiated malignant cell population in aspiration smears can help in cytological diagnosis. The acquisition of high proliferation index and over-expression of p53 may be the probable factors involved in the pathogenesis of high grade transformation in a conventional ACC.     

Primary intraosseous ACC of mandible of possible salivary origin: A rare clinical entity

INTRODUCTION

Adenoid cystic carcinoma (ACC) is a malignant tumor mainly of salivary origin which is well known for its deceptively benign histologic appearance characterized by indolent, locally invasive growth with high propensity for local recurrence and distant metastasis.

PRESENTATION OF CASE

An unusual case of a 23-year-old woman was reported in our hospital. After investigations, it showed that it is a second primary intraosseous lesion of mandible that occurred subsequently after ACC of parotid gland. After diagnosis was established, resection of tumor and reconstruction with a free fibula flap was performed. Ten months follow-up showed no signs of recurrence or metastasis.

DISCUSSION

Among the salivary neoplasms, adenoid cystic carcinoma is very rare and intraosseous lesions are even rarer. We found a total of 26 cases of primary ACC of the mandible reported in the literature. Pain and swelling were the most frequent symptoms.

CONCLUSION

This case illustrates two key facts. First, not all cystic lesions are necessarily metastatic or recurrence. Second is, even though the exact origin of this tumor is unknown, central salivary gland tumors should be considered in the differential diagnosis of cystic lytic lesions in the mandible.     

Primary acinic cell carcinoma of the breast

Acinic cell carcinoma (ACC) is an uncommon neoplasm of the salivary glands with low malignant potential. Even rarer is the occurrence of primary ACC of the breast, there is only one previously documented case. We now describe the second case of a primary carcinoma of the breast with morphological, ultrastructural, and immunohistochemical features consistent with an ACC. In contrast to the previous case report we report the lack of encapsulation of the tumor and a spectrum of cell types within the tumor including cells resembling well-differentiated infiltrating ductal carcinoma. Additionally we did not observe the abundant nuclear abnormalities noted in the previously published case. We report for the first time the mucicarmine-staining pattern of this tumor as well as immunohistochemistry directed against cytokeratin 7, the estrogen receptor, and the progesterone receptor.     

Adenoid Cystic Carcinoma of Trachea

Adenoid cystic carcinoma (ACC) is the second most common primary malignant tumor of the trachea, after squamous cell carcinoma. Patients present with upper airway obstructive symptoms and signs including dyspnoea, cough, haemoptysis, and stridor which are often insidious, delaying diagnosis and optimal management. Described here is an unusual case of primary ACC cervical trachea with concomitant micropapillary thyroid carcinoma (microPTC) in a middle-aged lady presenting with cough and breathing difficulty since 3 months.     

Imatinib mesylate as treatment for adenoid cystic carcinoma of the salivary glands: report of two successfully treated cases

BACKGROUND: Adenoid cystic carcinoma (ACC) is a malignant neoplasia of the salivary glands that is treated primarily by surgery. Local control and survival are usually compromised despite surgery. Expression of KIT tyrosine kinase is involved in the pathogenesis of ACC. Imatinib mesylate is a potent inhibitor of KIT tyrosine kinase, so we explored the possibility that ACC could be a potential target for this drug. METHODS: We report two cases of unresectable ACC treated with imatinib mesylate in the context of recurrent disease (case 1) and locally advanced tumor at its initial presentation (case 2). RESULTS: Both patients responded well to treatment with imatinib mesylate. Significant regression of recurrent disease (case 1) resulted in a successful salvage surgical resection; the locally advanced tumor (case 2) had an excellent response to treatment, but, unfortunately, the patient refused salvage resection. CONCLUSION: This is the first time ACC is reported to respond to imatinib mesylate. Studies in which more patients are enrolled in controlled clinical trials are needed to confirm this observation.     

Recurrent adenoid cystic carcinoma in the liver: a repeated laparoscopic surgical approach

Adenoid cystic carcinoma (ACC) is characterized by a particularly aggressive behavior even many years after resection of primary tumor. The evolution of metastasis dramatically affects the final outcome but resection should always be evaluated. Herein is described a case of aggressive ACC of the parotid gland in a 30-year-old female. She developed local recurrence and lung metastases; then, she also developed two liver metastasis 112 and 132 months after the resection of the primitive cancer of the parotid gland. Both lesions were successfully managed by a laparoscopic approach. Intra-abdominal adhesions after the first surgery were mild, allowing an easier access for the second laparoscopic liver resection. At 1 year follow-up, the patient is liver disease free with a stable lung disease. To our knowledge, this is the first report of a double laparoscopic liver resection for parotid gland's ACC metachronous metastases. Patients with resected ACC need a strict and lifelong follow-up after the resection of the primitive cancer. Also for ACC, a laparoscopic approach to liver metastasis should always be considered as a viable alternative to open surgery. In our experience of over 90 cases, laparoscopic surgery causes less adhesions, allowing an easier approach for repeated resections.     

Primary acinic cell carcinoma of mandible,report of a case and literature review

Introduction and importanceAcinic cell carcinoma (ACC) is a rare low-grade salivary gland malignancy that accounts for approximately 17% of all salivary gland malignancies. The most common site affected by ACC is the parotid gland followed by the submandibular glands, minor salivary glands, and sublingual glands. Also, it could hardly be observed in unusual sites such as the jaw bones.Case presentationThis case is an example of a central acinic cell carcinoma in a 73-year-old man who came up with a painless gradual swelling for 15 months. Based on clinico-radio-pathologic findings, the diagnosis of a solid variant Intraosseous Acinic Cell Carcinoma was established. Subsequently, the patient underwent hemimandibulectomy and modified radical neck dissection, followed by postoperative radiotherapy. Within a six-month follow-up period, no evidence of residual tumor was found.Clinical discussionCentral salivary gland carcinoma is a rare entity and intraosseous ACC is more scarcely observed. Based on our findings, a total of 17 cases of primary intraosseous ACC have been reported so far. Etiology and clinical presentations of this tumor are still vague due to its rarity.ConclusionDentists and oral surgeons must be aware of such a rare malignant lesion when encountering a radiolucent lesion within the jaws. The early diagnosis and a complete surgical excision to achieve tumor-free surgical margins and a long-term follow-up could result in significantly improved survival rates.     

Acinic Cell Carcinoma with Extensive Neuroendocrine Differentiation: A Diagnostic Challenge

Primary salivary gland carcinoma with neuroendocrine differentiation is of rare occurrence, especially so in the parotid gland. Amongst the various reported primary tumors with neuroendocrine differentiation, acinic cell carcinoma (ACC) one such tumor. A 48 year old lady presented with a gradually increasing right infra-auricular swelling for a period of 1 year which enlarged suddenly in a short period. Contrast Enhanced Computed Tomography (CECT) suggested diagnosis of Pleomorphic Adenoma. Fine Needle Aspiration Cytology (FANC) yielded a cystic fluid suggesting a possibility of Warthin’s tumor or Oncocytic lesion. Intraoperative findings were suggestive of a Warthin’s tumor. Initial histopathological examination of the tumor was suggestive of neuroendocrine carcinoma. However, extensive sectioning revealed peripheral islands of ACC. Immunoexpression of S-100, Neuron specific Enolase (NSE), Chromogranin A and Synaptophysin confirmed the diagnosis. The possibility of neuroendocrine differentiation in a primary salivary gland tumor should be kept in mind whenever a salivary gland tumor shows only neuroendocrine histology.