Mammary Analogue Secretory Carcinoma

乳腺类似物分泌性癌
  • 文章类型: Case Reports
    分泌性癌,以前被称为乳腺类似分泌癌,是一种罕见的唾液腺恶性肿瘤.它具有多种微观模式,与其他唾液腺肿瘤相似。
    本报告介绍了一例32岁女性患者,其上唇无痛肿胀,近期有未成熟卵巢畸胎瘤手术史。显微镜切片显示一个由大囊性肿瘤组成的局限性肿瘤,乳头状囊性,和微囊型,具有平淡的泡状核和空泡状的细胞质。肿瘤细胞对乳腺珠蛋白呈强烈阳性,SOX10、GATA3、S-100和波形蛋白。诊断为涎腺分泌癌。22个月后,没有复发。
    由于分泌性癌是一种相对较新的实体,有必要了解它的特点。尽管唾液腺癌患者第二原发癌的总体发病率较低,应考虑其在此类患者中存在的可能性。
    UNASSIGNED: Secretory carcinoma, previously known as mammary analog secretory carcinoma, is a rare malignancy of salivary glands. It has a diversity of microscopic patterns and is similar to other salivary gland tumors.
    UNASSIGNED: This report presents the case of a 32-year-old female patient with a painless swelling of the upper lip and a history of recent surgery for an immature ovarian teratoma. The microscopic sections revealed a circumscribed neoplasm composed of macrocystic, papillary-cystic, and microcystic patterns with bland vesicular nuclei and vacuolated cytoplasm. Tumoral cells were strongly positive for mammaglobin, SOX10, GATA3, S-100, and vimentin. The diagnosis of salivary gland secretory carcinoma was made. After 22 months, there has been no recurrence.
    UNASSIGNED: As secretory carcinoma is a relatively new entity, it is necessary to understand its characteristics. Although the overall incidence of second primary cancer in patients with salivary gland cancers is low, the possibility of its presence in such patients should be considered.
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  • 文章类型: Case Reports
    背景:在世界卫生组织(WHO)头颈部肿瘤的第四版分类中,分泌性癌(SC)被描述为一种独特的唾液腺肿瘤。SC通常被认为是一种生长缓慢的低度恶性肿瘤,虽然已经报道了一些具有高级特征的病例,甚至直到现在文献中的转移。在这篇文章中,讨论了具有高级显微特征和神经浸润的软组织SC病例。还对具有攻击行为的唾液腺SC病例进行了审查。
    方法:一名65岁的白种人在过去的6个月里出现了左颈肿块。影像学研究表明,颈部囊性肿块非常大(46×23mm),在左颈部Vb区的前外侧有乳头状突起。他接受了左根治性颈清扫术(I-V级),并随访了12个月,诊断为分泌性癌。
    结论:尽管SC通常具有良好的结果,可能发生多次复发和异常转移,这应该由病理学家或临床医生考虑。
    BACKGROUND: Secretory carcinoma (SC) has been described as a distinct salivary gland tumor in the fourth edition of the World Health Organization (WHO) classification of head and neck tumors. SC is generally considered as a slow-growing low-grade malignant tumor, while several cases have been reported with high-grade features, and even metastases in the literature up until now. In this article, a soft tissue SC case is discussed with high-grade microscopic features and neural invasion. A review of the salivary gland SC cases with aggressive behavior is also debated.
    METHODS: A 65-year-old Caucasian man presented with a left neck mass for the past six months. The imaging studies demonstrated a very large cystic cervical mass (46 × 23 mm) with papillary projections in the anterolateral aspect of the left neck zone Vb. He underwent left radical neck dissection (level I-V) and was followed up for 12 months with the diagnosis of Secretory carcinoma.
    CONCLUSIONS: Although SC generally has a good outcome, multiple recurrences and unusual metastases may occur, which should be considered by either the pathologists or clinicians.
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  • 文章类型: Journal Article
    唾液腺肿瘤占头颈部肿瘤的3-6%。70-80%的腮腺肿瘤在组织病理学上是良性的。FNAC在诊断腮腺肿瘤方面的总体准确率为95%。然而,剩下的5%仍然构成组织病理学的奥秘,可能需要手术切除腮腺。我们报告了一例42岁的男子,有腮腺肿胀史,FNAC报告显示有低度粘液表皮样癌或导管腺癌的可能性。手术切除后的最终组织病理学诊断揭示了腮腺乳腺类似物分泌性癌的新病理实体。
    Salivary gland neoplasms account for 3-6% of head and neck tumours. 70-80% of parotid tumours are benign in histopathology. FNAC has an overall accuracy of 95% in diagnosis of parotid neoplasms. However, the remaining 5% still pose a histopathological mystery which may require a surgical excision of the parotid. We report a case of a 42 year-old-man with a history of parotid swelling and a FNAC report which showed a possibility of a low-grade mucoepidermoid carcinoma or a ductal adenocarcinoma. Final histopathological diagnosis after surgical excision revealed a new pathological entity in mammary analogue secretory carcinoma of parotid.
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  • 文章类型: Journal Article
    分泌性癌(SC)是一种罕见的唾液腺肿瘤,最近已被列入世界卫生组织第4版头颈部肿瘤分类。目的了解1例23岁女性下颌下腺乳腺类似物分泌癌(MASC)的组织病理学表现和临床表现。MASC是一种有趣且罕见的恶性唾液腺肿瘤,于2010年首次描述。它有相同的组织学,乳腺分泌性癌的免疫组织化学和遗传特征。MASC的临床行为范围从缓慢生长的肿瘤到可引起广泛转移的侵袭性肿瘤。在以前分类为多形性腺瘤的存档病例中发现了许多MASC病例,腺泡细胞癌,粘液表皮样癌,和腺癌。在颌下腺中只有少数报道。MASC是唾液腺恶性肿瘤的一种新发现的变体。需要进一步的研究来更好地描述其总体患病率,并为这种新的临床实体定义适当的治疗算法。
    Secretory carcinoma (SC) is a rare salivary gland tumor and has been recently included in the fourth edition of the World Health Organization classification of head and neck tumors. To understand the histopathologic findings and clinical behavior of mammary analogue secretory carcinoma (MASC) of the submandibular gland in a 23 year old female. MASC is an intriguing and rare malignant salivary gland tumor first described in 2010. It shares histologic, immunohistochemical and genetic features with secretory carcinoma of the breast. The clinical behavior of MASC ranges from slowly growing tumors to aggressive tumors that can cause widespread metastasis. Many cases of MASC were discovered in archived cases previously classified as pleomorphic adenoma, acinic cell carcinoma, mucoepidermoid carcinoma, and adenocarcinoma. They are only a few reported in submandibular gland. MASC is a newly recognized variant of salivary gland malignancy. Further research is needed to better delineate its overall prevalence and to define an appropriate treatment algorithm for this new clinical entity.
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  • 文章类型: Case Reports
    乳腺类似物分泌型唾液腺癌(MASC)是头颈部唾液腺的低度癌。它与乳腺分泌性癌和腮腺腺泡细胞癌(ACC)具有组织学相似性。其临床行为和侵略性在个体之间有所不同,并且下颌下腺MASC的经验有限。
    我们报告了一名16岁的女性,其颌下区域颈部肿胀。下颌下区域的硬肿胀是MASC,而软囊性肿块是颈部的先天性淋巴囊肿。我们报告了两个不寻常的特征,颌下腺MASC极为罕见,主要肿瘤肿块附近区域存在先天性淋巴囊肿。通过手术切除颈部肿块和同侧选择性颈部淋巴结清扫术(I-IV级)进行治疗。
    虽然MASC的组织学模式在以前的研究中已经描述过,它的临床表现很少被记录。本报告旨在阐明这种诊断不足的实体的临床表现和术前检查期间所需的积极管理方案。术中疾病清除和术后随访。颌下腺的MASC是青少年年龄组颈部肿胀的罕见原因,但是由于其偶尔的侵略性,应牢记作为唾液腺肿瘤的可能鉴别诊断。
    UNASSIGNED: Mammary Analogue Secretory Carcinoma of salivary glands (MASC) is a low-grade carcinoma of salivary glands of the head-neck region. It bears histological resemblance to Secretory Carcinoma of the breast and Acinic Cell Carcinoma (ACC) of the parotid gland. Its clinical behaviour and aggressiveness vary amongst individuals and experience in MASC of the submandibular gland are limited.
    UNASSIGNED: We report a 16-year-old female with binary neck swelling in the submandibular region. The hard swelling in the submandibular region was a MASC and the soft cystic mass was a synchronous congenital lymphatic cyst in the neck. We report two unusual features, an extremely rare involvement of MASC of submandibular salivary gland and the presence of a congenital lymphatic cyst in the area adjacent to the main tumour mass. Treatment was done by surgical excision of both the neck masses in-toto and ipsilateral selective neck dissection (Level I-IV).
    UNASSIGNED: While MASC\'s histological pattern has been described in previous studies, its clinical picture is rarely documented. This report aims to shed light on the clinical presentation of this under-diagnosed entity and the aggressive management protocol required during preoperative workup, intraoperative disease clearance and post-operative follow up of such patients. MASC of the submandibular salivary gland is an uncommon cause of neck swelling in the adolescent age group, but due to its occasional aggressive nature, should be borne in mind as a possible differential diagnosis of salivary gland tumours.
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  • 文章类型: Case Reports
    Mammary analogue secretory carcinoma (MASC) is a rare malignant tumour of the salivary glands, with only few cases reported in the literature to date. Initial preoperative staging is crucial for all patients with an oral malignancy to visualize the tumour, detect lymph node or distant metastases and plan therapeutic interventions. In the case presented herein, radiological imaging revealed a tumour of the right hard palate with suspected positive contralateral lymph nodes. Therefore, local tumour resection comprising hemimaxillectomy and bilateral neck dissection was performed. The diagnosis of MASC was finally based on characteristic histopathological and immunohistochemical findings, such as S100 protein and mammaglobin positivity. The diagnosis of MASC may be challenging, as such findings lack specificity. To confirm the diagnosis, molecular genetic examinations may be performed to detect a highly specific ETV6-NTRK3 fusion gene. Depending on the results of these examinations, surgery, alone or combined with adjuvant radiation or chemoradiation, is the recommended approach. In summary, MASC should be treated similarly to other low-grade salivary gland tumours, such as acinic cell carcinoma, as they exhibit biological and histopathological similarities.
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  • 文章类型: Case Reports
    BACKGROUND: Mammary analogue secretory carcinoma (MASC) is a recent discovered entity of salivary glands tumors, reported for first time in 2010. The presence of a translocation encodes the ETS variant transcription factor 6-neurotrophic tyrosine receptor kinase (ETV6-NTRK3) gene fusion differences MASC from other tumors.
    METHODS: A 68-year-old male showed a non-painful right parotid enlargement, came from dermatology service, and followed by some facial squamous cell carcinomas. A computed tomography (CT) scan showed a 1.7×1.6 cm right parotid enlargement in superficial lobe. The patient underwent a right superficial parotidectomy. The final pathology confirmed the presence of ETV6-NTRK3-positive MASC. Complete right deep parotidectomy and functional cervical emptying were performed.
    CONCLUSIONS: It is necessary to establish an appropriated differential diagnosis between salivary gland tumors. MASC is a low-grade malignancy cancer that sometimes can evolve to a high-grade tumor that might produce local and distance dissemination. Most times, these tumors are only treated by surgical resection and evaluating by a multidisciplinary team the need of more treatments. In our case, the patient showed a primary parotid tumor, removed surgically with free edges, and being identified as MASC. We decided to underwent neck dissection and discovered a second MASC focus on cervical salivary gland; however, there was no nodal dissemination. The patient remains disease-free after 14 months from last surgery. It is important to keep studying genetic therapy targets to ETV6-NTRK3 to obtain a new therapy line to treat those cases that require.
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  • 文章类型: Case Reports
    背景:乳腺类似物分泌癌(MASC)是一种罕见的低度恶性涎腺肿瘤。MASC的形态学和免疫组织化学特征与乳腺分泌性癌非常相似。病变的关键特征是缺乏疼痛和缓慢生长。临床表现和影像学特征无明显特异性。该病的诊断主要依赖于MASC特异性ETV6-NTRK3融合基因的检测。
    方法:本报告描述了一例罕见的32岁男性患者,该患者表现为逐渐增长的病变,最初被诊断为右侧腮腺的乳样分泌癌。影像学和组织学检查用于克服诊断困难。病变采用右腮腺切除术治疗,面神经保护,生物补片植入以恢复由此产生的缺陷,和术后放疗。术后随访,患者报告轻度面部畸形,无并发症,面瘫的迹象,或弗雷综合征。
    结论:讨论了MASC的影像学和组织学诊断挑战。
    BACKGROUND: Mammary analogue secretory carcinoma (MASC) is a rare low-grade malignant salivary gland tumor. The morphological and immunohistochemical features of MASC closely resemble those of breast secretory carcinoma. The key characteristics of the lesion are a lack of pain and slow growth. There is no obvious specificity in the clinical manifestations and imaging features. The diagnosis of the disease mainly depends on the detection of the MASC-specific ETV6-NTRK3 fusion gene.
    METHODS: This report describes a rare case of a 32-year-old male patient who presented with a gradually growing lesion that was initially diagnosed as breast-like secretory carcinoma of the right parotid gland. Imaging and histological investigations were used to overcome the diagnostic difficulties. The lesion was managed with right parotidectomy, facial nerve preservation, biological patch implantation to restore the resulting defect, and postoperative radiotherapy. On postoperative follow-up, the patient reported a mild facial deformity with no complications, signs of facial paralysis, or Frey\'s syndrome.
    CONCLUSIONS: The imaging and histological diagnostic challenges for MASC are discussed.
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  • 文章类型: Case Reports
    分泌性癌(SC)在2010年首次被认为是一种独特的唾液恶性肿瘤。在它被认可的九年里,已经有多个主要和次要唾液腺SC的报告,还有一例舌根受累。在这里,我们介绍了第一例报道的双侧SC。第一个肿瘤,在识别SC之前诊断,被归类为粘液表皮样癌。2016年对侧腮腺肿瘤诊断为SC后,对两种组织学进行了比较,粘液表皮样癌被重新分类为SC。在这份报告中,我们描述了患者的临床过程,并回顾了SC文献,重点是病理诊断和临床预后。
    Secretory carcinoma (SC) was first recognized as a distinct salivary malignancy in 2010. In the nine years since its recognition, there have been multiple reports of SC of the major and minor salivary glands, as well one case of tongue base involvement. Here we present the first reported case of bilateral SC. The first tumor, diagnosed before the recognition of SC, was classified as mucoepidermoid carcinoma. After the contralateral parotid tumor was diagnosed as SC in 2016, the two histologies were compared, and the mucoepidermoid carcinoma was reclassified as SC. In this report, we describe our patient\'s clinical course and review the SC literature, with a focus on pathologic diagnosis and clinical prognosis.
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  • 文章类型: Case Reports
    BACKGROUND: Secretory Carcinoma is a recently discovered head and neck tumour. Surgical resection is generally the first line of treatment with neck dissection, radiation, and chemotherapy decided based on clinical, radiological, and histological parameters. We present a case of a Secretory Carcinoma presenting in the maxillary and ethmoid sinuses.
    METHODS: A 39-year-old male presented with left nasal obstruction and chronic rhinosinusitis beginning after surgical repair of the mandible and maxilla due to facial trauma. A CT scan of the head showed soft tissue density in the left maxillary sinus extending through the ethmoid sinus and posterior nasal cavity. Functional endoscopic sinus surgery (FESS) was performed to remove the obstructing lesion. Biopsy was analysed and the main differential diagnosis was between a non-intestinal type adenocarcinoma of nasal origin and a salivary carcinoma.
    CONCLUSIONS: To our knowledge, there have only been two cases of Secretory Carcinoma reported in the sinuses: one case in the ethmoid sinus and the other in the maxillary sinus. This is the first to report follow up at 1 year.
    CONCLUSIONS: Both MRI and exam showed no evidence of recurrent disease at one year follow up. This report aims to further the understanding of Secretory Carcinoma tumours in the sinuses. Our report could be used to further understand diagnostic criteria for Secretory Carcinoma in the sinuses as well as treatment outcomes for surgical resection without adjuvant treatment.
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