Giant cells undifferentiated parotid carcinoma. Report of two cases

Tumours of the salivary glands represent a 5% of all head and neck neoplasms. Their origin is the parotid gland in about 80% of the cases. Most of them are benign. We are reporting two cases of patients diagnosed, by our ENT Department, as undifferentiated giant cells parotid carcinoma with cervical metastasis. They were operated by total parotidectomy and radical neck disection and later recieved treatment with radiotherapy. After two years-follow-up both patients are standing alive. The tumoral size is the most important pronostic factor in this histological type.     

Distant metastasis of parotid gland tumors

The incidence of distant metastasis in head and neck cancer and especially in salivary gland cancer is relatively low in comparison to other malignancies. However, the presence of distant metastasis heralds a poor prognosis in head and neck cancer, with a median survival of 4.3–7.3 months. Treatment of these patients is usually performed in a palliative setting. Patients with malignant salivary gland tumors should have an X-ray or CT scan of the chest at their initial assessment to exclude the possibility of distant metastasis. The likelihood of developing distant metastasis is associated with high-grade tumors, such as adenoid cystic carcinoma, salivary duct carcinoma, high-grade mucoepidermoid carcinoma and tumors located in the submandibular gland, posterior tongue and pharyngeal tumors. A lower risk of developing distant metastasis is known for all other histological entities of salivary gland tumors. Nevertheless all patients who have a histologically confirmed malignant salivary gland tumor should have lifelong follow-up. On the basis of a clinical case regarding a patient with metastatic parotid gland cancer we present a review of the literature.     

Distant metastasis of parotid gland tumors

The incidence of distant metastasis in head and neck cancer and especially in salivary gland cancer is relatively low in comparison to other malignancies. However, the presence of distant metastasis heralds a poor prognosis in head and neck cancer, with a median survival of 4.3-7.3 months. Treatment of these patients is usually performed in a palliative setting. Patients with malignant salivary gland tumors should have an X-ray or CT scan of the chest at their initial assessment to exclude the possibility of distant metastasis. The likelihood of developing distant metastasis is associated with high-grade tumors, such as adenoid cystic carcinoma, salivary duct carcinoma, high-grade mucoepidermoid carcinoma and tumors located in the submandibular gland, posterior tongue and pharyngeal tumors. A lower risk of developing distant metastasis is known for all other histological entities of salivary gland tumors. Nevertheless all patients who have a histologically confirmed malignant salivary gland tumor should have lifelong follow-up. On the basis of a clinical case regarding a patient with metastatic parotid gland cancer we present a review of the literature.     

17例涎腺淋巴上皮癌临床病理分析

目的：涎腺淋巴上皮癌是一种罕见的恶性肿瘤,本文主要探讨涎腺淋巴上皮癌的发病情况、病理特点及其诊治措施。方法：回顾性分析2006—2012年间于南方医科大学南方医院就诊的17例涎腺淋巴上皮癌患者,分析其临床特点与治疗方法对预后的影响。结果：17例中,男10例,女7例;Ⅱ、Ⅲ、Ⅳ期患者分别为7（41．2％）、4（23．5％）、6（35．3％）例。术后平均随访时间为2．56年,随访12例患者情况稳定;5例患者分别在术后3年内出现局部复发和（或）远处转移,其中4例已死亡。结论：涎腺淋巴上皮癌是头颈部恶性程度较高的肿瘤之一,好发于腮腺及下颌下腺。治疗应行原发灶扩大切除及根治性颈淋巴清扫术,并结合放化疗以提高疗效。     

Eight cases of neuroendcrine carcinoma of the head and neck

Small cell neuroendocrine carcinoma of the head and neck is rare, and diagnosis may be difficult. Malignancy is high, as is the incidence of distant metastasis. We reported eight cases of stage IV small cell neuroendocrine carcinoma of the head and neck, all in men with a mean onset age of 62 years (range: 45 to 80 years). Three cases arose from the maxillary sinus, two from the ethmoid sinus, one from the parotid gland, one from the tonsil, and one from the larynx. Histological analysis by hematoxylin-eosin staining tentatively revealed malignant lymphoma and undifferentiated carcinoma in two cases each, while immunohistological and/or electron microscopy analysis confirmed histological diagnosis. All were treated by chemotherapy (VP-16, CDDP) and seven cases with radiotherapy based on the schedule of small cell carcinoma of the lung and two cases with lesional resection. Chemotherapy and radiotherapy were effective locally. Five patients died of distant metastasis to the brain, bone, lung, liver, or skin within 12 months. One is alive with liver metastasis. Two have no evidence of disease--one for eight years and the other for one year. Long-term survival thus requires the effective treatment of distant metastasis.     

Incidence of salivary gland malignancies in 12 year time period in ENT Department Medical University in Poznan

Neoplasm of salivary gland constitutes about 3% of all tumours of head and neck. Within the category we can differentiate tumours of a very different histological structure. What lies behind such great differences in the changes within the salivary glands is complex embryogenesis of the glands. This work aims at the assessment of the frequency of occurrence of malignant neoplasm in parotid gland and submandibular gland based on the material collected at the ENT Department of the Medical University in Poznan in the years 1995-2006. In the 12-year period, 103 patients suffered from malignant neoplasm. 82 tumours were diagnosed in paroid gland and respectively 21 - in submandibular gland. No neoplasm was identified in sublingual gland. It was concluded, that trends in occurrence of neoplasm of salivary glands, assessed on the basis of the number of patients operated in the years 1995-2006 show that the number of malignant neoplasm cases remains on the same level. The analysis of the epidemiological differences based on the comparison of the groups of patients treated for salivary gland neoplasm in the years 1995-2000 and 2001-2006 has shown that with regard to malignant neoplasm such parameters as sex, age, duration of symptoms, diameter of the tumour and level of advancement have not differed much from each other in the analyzed periods. Epidemiological differences were identified in respective histological structures in the two periods analyzed: adenoid cystic carcinoma was in fact more frequent in the years 1995-2000 than in the years 2001-2006 (58.8% versus 24.1%). On the other hand, metastasis to the salivary glands was less frequent in period I than in period II (11.8% and 27.6% respectively), so was cancer in mixed tumour (2.9% and 17.2%). Facial nerve paralysis was statistically more frequent in highly malignant tumours than in comparison to tumours malignant only to a slight extent.     

Salivary gland neoplasms in the years 1991-2006 in the material of the Department of Otolaryngology, Medical University in Bialystok

Complex structure of salivary glands, histological diversity of malignant salivary gland neoplasms and heterogeneous clinical image make therapeutic strategy difficult and controversial. The aim of this work was an epidemiologic analysis of 86 salivary gland tumour cases (in years 1991-2000) and 82 cases treated at the Otolaryngological Clinic of the Medical University of Bialystok (in years 2001-2006). Epidemiologic researches were conducted retrospectively. Age, sex, histological structure, location and local progression of tumour were analysed. The relationship between histological type, local progression and the presence of metastases to the cervical lymph nodes as well as remote metastases was also examined. The study was based on TNM Tumor Classification: large salivary glands (1977). It was found that malignant epithelial salivary gland tumours affect most commonly the parotid gland in men aged over 50 years. Adenoid cystic carcinoma was the most frequently represented histological type. Remote metastases were observed most commonly in patients with polymorphic adenoma.     

Adenoid cystic carcinoma of the salivary glands: a 20-year review with long-term follow-up

The behavior of adenoid cystic carcinoma (ACC) of the salivary glands has been shown to be unpredictable in terms of local and distant spread and mortality. We retrospectively studied 35 operations in 34 patients who had had a pathologic diagnosis of ACC of the salivary glands and who had been treated over a 20-year period and followed for a minimum of 10 years. We analyzed the effect that different factors had on outcomes. The site of origin appeared to be an important factor in survival rates; survival among patients with tumors that had originated in the parotid gland was fairly good, while survival among those with tumors that originated in the minor salivary glands was significantly worse. TNM staging was another significant factor in survival. Other poor prognostic indicators were local spread, nodal positivity, distant metastasis, and local and regional recurrence. Radiation and chemotherapy did not appear to be beneficial for patients with advanced disease. We recommend radical surgery with complete resection for all patients with ACC of the salivary glands and a careful assessment of the neck in patients with minor salivary gland tumors.     

Speichelgangkarzinome der Glandula submandibularis

BACKGROUND: Salivary duct carcinoma (SDC) is a rare adenocarcinoma of the salivary glands which usually occurs in the parotid gland (approx. 80%), but is also found in the submandibular gland (approx. 12%) and the minor salivary glands. Due to the low incidence of this tumour-particularly in terms of involvement of the submandibular and minor salivary glands-only limited clinical data is available. PATIENTS/METHOD: In a retrospective investigation, follow-ups of six patients with a SDC of the submandibular gland were analysed and the surgical measures evaluated for their efficiency. RESULTS: At the time of diagnosis, varying tumor stages were present (2xT1, 3xT2, 1xT1). Initial lymph node metastasis was observed in four (66.6%) of the six patients. In all patients, the submandibular gland was resected and neck dissection performed, in two cases (33,3%) this was followed by radiotherapy. Four patients developed a life-threatening local recurrence. Only one patient developed distant metastasis. Two thirds of the patients died due to tumor-related disease after an average of 35.5 months. CONCLUSION: The clinical course of patients with SDC of the submandibular gland exhibits a high (66.6%) rate of local recurrence, crucial for the following clinical course. The treatment of these patients, therefore, must include radical tumor resection as well as tissue resection with a wide margin.     

Distant metastases from salivary glands cancer.

Patients who present with malignant salivary glands should at their initial assessment have an X-ray of the chest to exclude the possibility of distant metastases. Patients who have other symptoms, bone pain etc., should be appropriately investigated. The likelihood of patients developing distant metastases is associated with high-grade tumors, most commonly adenoid cystic carcinoma, high-grade mucoepidermoid carcinoma, salivary duct carcinoma and tumors sited in the submandibular gland, posterior tongue and pharyngeal tumors. Patients who have had a high-grade tumor treated and survived without locoregional recurrence have the same risk of developing distant metastases as those patients who have locoregional recurrence. Other histological types of salivary tumors are associated with a lower risk of developing distant metastases but a real risk remains lifelong. It is recommended that all patients who have a malignant salivary gland tumor treated, any histology, should be followed up and clinically assessed at least once every 12 months for life.     

Multivariate analysis of risk factors for neck metastases in surgically treated parotid carcinomas

OBJECTIVE: To analyze risk factors for neck metastases in patients with parotid carcinomas. DESIGN: Cohort of patients followed up from 1 to 366.2 months at a single institution. SETTING: Referral center, private or institutional practice, hospitalized care. PATIENTS: A total of 145 patients with parotid carcinomas with complete clinical and pathological information. The histological diagnosis was reviewed according to the World Health Organization classification for salivary gland tumors. INTERVENTION: Patients were treated by surgery alone (62 cases) or with postoperative radiotherapy (83 cases). A neck dissection was performed in 80 patients. MAIN OUTCOME MEASURE: Rates of neck lymph node metastasis. Univariate and multivariate analyses were carried out using logistic regression evaluating the significance of demographic, clinical, and pathological data. RESULTS: The following variables were significantly associated to the risk of lymph node metastasis by univariate analysis: histological type (P<.001), T stage (P<.001), desmoplasia (P = .001), facial palsy (P = .02), perineural invasion (P = .01), extraparotid tumor extension (P = .02), and necrosis (P = .003). By multivariate analysis, histological type (P<.001), T stage (P = .03), and desmoplasia (P = .006) had the highest correlation with lymph node metastasis. CONCLUSION: The significant risk factors for neck metastasis in parotid carcinoma were histological type (ie, adenocarcinoma, undifferentiated carcinoma, high-grade mucoepidermoid carcinoma, squamous cell carcinoma, and salivary duct carcinoma), T stage (T3 and T4), and desmoplasia (severe).     

Salivary duct carcinoma--a clinicopathological analysis of five cases

The 5 cases of salivary duct carcinoma (SDC); very rare, but distinct group of highly malignant salivary gland tumor are presented, and difficulties with pathological and clinical diagnosis is discussed. The SDC developed in single cases in parotid salivary gland, submandibular salivary and in mucosa of maxillary sinus, pyriform fossa and oral cavity (check). In 3 cases the second malignant tumor was present--synchronously (SDC + pleomorphic adenoma in parotid gland; SDC + squamous cell carcinoma in hypopharynx) or metachroneously (squamous cell carcinoma of upper lip followed by SDC). In one case the high levels of PSA suggesting of metastases from unknown primary within the prostate gland, or PSA expression related to SDC was observed. The four patients received radical treatment - surgical resection followed by radiotherapy; in one case only palliative treatment was applied, due to patient's poor general condition and high advancement of the primary disease. The observation ranged from 10 to 77 months (average time--31 months). The one patient died 13 months after diagnosis and palliative treatment. The three patients are alive with distant metastases to the lung and bones (77, 38 and 18 months after primary treatment was completed). Only one patient with 10 months observation after treatment is living without symptoms of recurrence or metastases.     

Malignant salivary gland tumors: squamous cell carcinoma of the submandibular gland in a child.

PURPOSE: Malignant neoplasms of the salivary glands are extremely unusual in the pediatric age group. METHODS: We report an 11-year-old boy who presented with a mass in the left submandibular region and a second mass in the jugularfacial venous angle. RESULTS: Histologic evaluation determined that this was a squamous cell carcinoma of the submandibular gland with metastasis to a cervical node. Review of the literature was undertaken to identify the rate of malignant salivary gland tumors in children. Malignant salivary gland tumors are extremely rare in children. When malignancy does occur, nearly 90% are present in the parotid gland. The submandibular gland is effected in 7.7% of cases. The most common malignancy in salivary glands of children is the mucoepidermoid carcinoma. Squamous cell carcinoma occurs in less than 2% of cases. CONCLUSIONS: Salivary gland tumors are very rare in children in contrast to adults. The proportion of malignant tumors in children is higher than that observed in adults; however, it is not possible to draw conclusions concerning treatment from the few case reports that exist. A higher rate of local recurrence and cervical lymph node metastasis may be expected in children.     

头颈肌上皮癌的生物学特征及诊疗分析

目的 探讨头颈肌上皮癌的临床表现,病理、免疫组化特点及诊疗方法,提高对该病的诊疗水平。方法 回顾性分析经病理确诊的5例头颈肌上皮癌患者的临床资料,结合文献并从生物学、诊疗和预后方面进行分析。结果 5例患者均经病理和免疫组化确诊,其中腮腺3例,硬腭1例,鼻、鼻窦1例。5例患者行根治性手术治疗4例,1例放弃手术而行放、化疗。3例术后局部复发,其中2例出现颈淋巴结转移,行二次手术或加颈淋巴结清扫术,2例出现远处转移;至末次随访,4例死亡,1例无瘤生存（术后10个月）。结论 头颈肌上皮癌是预后极差的上皮性恶性肿瘤,好发于腮腺,鼻、鼻窦者极为罕见;颈淋巴结和远处转移率较高,治疗采用手术为主的综合治疗,术后复发率高。     

Squamous cell carcinoma in the area of the parotid gland. Metastasis or primary tumor?

A total of 61 patients with a histological diagnosis of squamous cell carcinoma of the parotid gland were studied. The patients were classified into three categories. There were 34 patients with a metastasis to the parotid gland from a squamous cell carcinoma elsewhere within the head and neck who presented on average 2.1 years (range 3 months to 7 years) after diagnosis of the primary tumour; in one case a salivary gland tumor presented 32 years after irradiation of a squamous cell carcinoma of the temple. Six patients had histological evidence of a metastasis within the parotid gland, but no evidence of a primary tumour. Twenty-one patients presented with a primary epidermoid carcinoma of the parotid gland. Two patients showed a primary squamous cell carcinoma of the parotid arising in myoepithelial sialadenitis.     

Ocena ekspresji receptora estrogenowego,progesteronowego i receptora HER2 w wybranych nowotworach złośliwych ślinianek

Introduction

The incidence of the malignant tumors of the salivary glands is relatelively low. However, rapid locoregional progression, distant metastases occurrence and high local recurrence rate occurring despite radical surgical treatment, significantly affect the outcome. Malignant tumors of the salivary glands are characterized by low radiosensitivity and poor response to systemic therapy. Therefore there is a need for new targeted therapies which may improve the prognosis. In some aspects, malignant tumors of the salivary glands are similar to breast cancers. Efficacy of hormonal replacement therapy and the treatment with the monoclonal antibody (trastuzumab) in the treatment of breast cancer is well known and confirmed. Criterion determining the qualification for treatment is the presence of hormone receptors and HER2 receptors in the tumor.

Aim

The aim of this study was to evaluate the expression of estrogen receptor, progesterone receptor and HER2 in selected salivary gland malignancies.

Materials and methods

Study group consisted of 51 patients with the salivary glands cancer. In the study group there were 42 tumors in the parotid gland, 7 tumors of the submandibular gland, 1 in the mucosa of the cheek and 1 tumor in the tongue. Histologically material comprised of muco-epidermoid carcinoma (10), adenoid-cystic carcinoma (8), salivary duct (7), adenocarcinoma (6), squamous cell carcinoma (6), mioepithelial carcinoma (5) and other (9). In all cases immunohistochemical analysis of the estrogen receptor, progesterone receptor and HER2 receptor was performed.

Results

The expression of the estrogen receptor was found in 18% of tumors (9 patients). The expression of the progesterone receptor was found in 20% (10 patients). HER2 expression was found in 10% of tumors and was related to only one histological type of tumor – salivary duct carcinoma.

Conclusion

HER2 expression in malignant tumors of the salivary glands, especially in salivary duct carcinoma may be of use in future implementation of new targeted therapies based on monoclonal antibodies     

Malignant lymphoma in the head and neck associated with benign lymphoepithelial lesion of the parotid gland

Lymphoepithelial lesion is a benign lymphoproliferative disease occasionally arises in the salivary glands, but association with malignant diseases or autoimmune diseases has also been discussed. We herein present three cases of malignant lymphoma arose in the parotid gland and the lacrimal gland, following parotid surgery for benign lymphoepithelial lesion (BLEL) of the parotid gland. Two cases had mucosa associated lymphoid tissue (MALT) lymphoma in the parotid gland; one arose in the ipsilateral parotid gland as a recurrent swelling, and the other arose in the contralateral parotid gland of the previous BLEL surgery. The third case of malignant lymphoma arose in the lacrimal gland on the ipsilateral side, and the following contralateral parotid gland remained BLEL. All three patients were female, and one patient had a past history of Sj?gren's syndrome and Hashimoto's disease. All three patients were treated by chemotherapy and one patient received additional radiotherapy. To follow-up lymphoproliferative diseases in the salivary glands such as BLEL, careful observation should be made on the same gland, other major salivary glands, and other organs in the head and neck, especially in females with autoimmune diseases.     

The interesting case -- case no. 68. Metastasis of a small-cell bronchial carcinoma to the parotid gland

We present a case of a metastasis to the parotid gland from a small cell carcinoma of lung. The patient noted swelling in right parotid region without pain and saw an otorhinolaryngologist. There was no facial nerve palsy. He was admitted to our hospital. The total parotidectomy with facial nerve preserving was performed. The pathological findings indicated small cell carcinoma. After receipt of this report, we examined the lungs. An abnormal shadow could be found in the chest X-rays. CT revealed a lung tumor on a left site which was determined to be small cell carcinoma by transbronchial lung biopsy. Metastasis to the parotid glands from any distant primary site is quite unusual. In most cases of secundary parotid involvement of malignant tumors primary tumors originated from the head and neck region. Primary tumors in the neighborhood are the skin of the head and neck and the mucosa of the upper airway and digestive tract. Distant metastases to the parotid gland have been reported to arise from bronchial carcinoma, renal carcinoma, colonic carcinoma, prostat and breast. The distinction between primary salivary glands tumors and metastases of other primary tumors is difficult often. Histologic and immunhistological methods can be helpful.     

Alveolar rhabdomyosarcoma of the parotid gland

INTRODUCTION: Alveolar rhabdomyosarcoma of salivary glands is a rare malignant neoplasm, presenting a striated muscular differentiation. It is typically observed in children, adolescents, and young adults but can sometimes be diagnosed in older people. OBSERVATION: We report the case of a 57 year-old man presenting with a primary alveolar rhabdomyosarcoma of the parotid gland. The patient was treated by surgery and radiotherapy. DISCUSSION: According to literature data, alveolar rhabdomyosarcoma of the parotid gland is exceptionally described after 40 years of age. In that case it has the worst prognosis and its lethality is due mainly to its very early metastasis.     

Salivary duct carcinoma of the sublingual gland--a case report

RATIONALE: Pleomorphic adenoma is the most common neoplasm of major and minor salivary gland origin, followed by infectious reasons and sialolithiasis. Less common are adenocarcinomas presented. The salivary duct carcinoma represents a rare variant of the group of adenocarcinomas originated from the salivary glands, especially extremely rare from minor salivary gland origin. CASE REPORT: We report about a 52 year old male patient presenting with painless, non-ulcerating tumor at the floor of the mouth. Since 2 weeks ingestion was painful. Further ENT-investigations including endoscopy and scans (MRT, ultrasound) showed no pathology. In particular, growth of cervical lymph nodes and distant metastases were not revealed. A biopsy showed a rare salivary duct carcinoma of comedo-type originated from the left sublingual salivary gland. The patient underwent an operation: the tumor was extirpated in toto, and, as the pathohistological investigation confirmed, in sano. Additionally a neck dissection at the left side was performed, followed by the radiation of the tumor region and the lymphatic neck regions, after sufficient reconvalescence. The tumor-classification was pT3, pN0. Since 3 years no recurrence of the tumor was confirmed. DISCUSSION: Salivary duct carcinoma is a rare, high-grade malignant epithelial neoplasm, which occurs almost exclusively in the parotid. As presented, it can also occur in minor salivary glands. Histologically it is composed of structures that resemble expanded salivary glands. Comedonecrosis of these structures is a frequent feature. There is some histologic similarity to intraductal carcinoma of the breast. Differential diagnosis comprises adenoid cystic carcinoma, metastases of other adenocarcinomas, such as the carcinoma of the thyroid gland and the comedocarcinoma of the breast. Because of its poor prognosis a rapid and radical therapy is recommended. CONCLUSION: A rare case of a salivary duct carcinoma of sublingual salivary gland origin is presented. Usually the prognosis is poor. The adequate therapy is the complete surgical extirpation including the surgery of the regional lymphatic nodes and the additional radiotherapy.