Parapharyngeal space tumors. Our experience. I.P.O. Francisco Gentil,Lisbon

Primary parapharyngeal space tumours are rare, representing only a 0.5% of head and neck neoplasms. The authors report a case series review of 38 patients with parapharyngeal tumours who underwent surgical excision between 1975 and 1998. Twenty-six of them were female (68%) and twelve male (32%). Thirty-three tumours (87%) were benign and five (13%) were malignant being the Pleomorphic adenoma the most common neoplasm (39%). All patients were treated surgically: the trans-cervical approach was used in 19 cases, cervical-parotid in 5, the trans-parotid approach in 7 patients, transoral in 5, the cervical-parotid approach with mandibulectomy in 2 and the combined transoral-cervical approach in 1 case. Out of the 33 patients with benign neoplasms, 1 (a pleomorphic adenoma treated through a transoral approach) had a recurrence. Amongst the 5 with malignant disease, recurrence or persistent local tumour was seen in 4 cases; and of these, 3 with persistent local tumour after incomplete excision died.     

Neoplasms of the parotid gland in northern Poland, 1991–2000: an epidemiologic study

A review of patients treated for malignant and benign parotid tumors in a 10-year period (1991–2000) in the Pomeranian County of Poland revealed 417 new cases. The age-standardized incidence rate was 0.23 and 1.35 per 100,000 inhabitants for malignant and benign tumors, respectively. The following data were collected: age, sex, place of living, education, job, smoking, symptoms, duration of symptoms at the time of diagnosis, result of histopathological tumor examination, tumor size, TNM staging and distant metastases. The results were divided into two parts: malignant neoplasms and benign neoplasms. The most common malignant neoplasms were carcinomas (96.8%), and among them the most numerous were mucoepidermoid carcinoma (22.2%), carcinoma in pleomorphic adenoma (20.6%) and adenoid cystic carcinoma (15.9%). The most common benign neoplasms were adenomas (95.5%), most frequently pleomorphic adenoma (65.5%) and adenolymphoma (24.0%), but rarely basal cell adenoma (4.2%). We also observed a steady increase in the number of benign parotid tumors in the examined period of time.     

A case of pleomorphic adenoma of the parotid gland metastasizing to a mediastinal lymph node

OBJECTIVE: Pleomorphic adenomas are the most common salivary gland tumors and are typically cured with complete surgical excision. There are rare reports, however, in which these histologically benign tumors have inexplicably metastasized to distant sites. We present a case of a patient who presented, 27 years after excision of a parotid pleomorphic adenoma, with a recurrence in the parotid bed and a mediastinal metastasis. STUDY DESIGN: Case report. METHODS: A 43-year-old woman presented with a mass in the right parotid bed 27 years after excision of a pleomorphic adenoma of the parotid. The patient's presentation, workup, and final diagnosis of benign metastasizing pleomorphic adenoma will be discussed, along with a pertinent review of the literature. RESULTS: A diagnosis of recurrent pleomorphic adenoma was made from a fine needle aspiration biopsy of the right parotid mass. On subsequent computed tomographic scan, chest images revealed an incidental left mediastinal mass, which also proved to be a pleomorphic adenoma on computed tomography-guided fine needle aspiration biopsy. The patient underwent a completion parotidectomy and sternotomy with excision of the mediastinal mass. Examination of the pathology specimens confirmed a diagnosis of pleomorphic adenoma in both the parotid bed and the mediastinum. No histologic characteristics of malignancy were seen in either specimen; therefore, a diagnosis of benign metastasizing mixed tumor was rendered. CONCLUSION: Benign metastasizing pleomorphic adenoma is a rare and controversial but distinct clinical entity. Although the definition of the term benign precludes metastatic disease, these tumors do not demonstrate any malignant features yet metastasize to distant sites. It remains to be determined whether these benign metastasizing pleomorphic adenomas are really low-grade salivary malignancies.     

Myoepithelial carcinoma ex-pleomorphic adenoma of the parotid gland

INTRODUCTION: Myoepithelial carcinoma accounts for less than 1% of salivary gland malignant tumors. It may develop de novo or, in approximately 50% of the cases, from a pleomorphic adenoma. OBSERVATION: A 57-year-old man with a history of a pleomorphic adenoma of the parotid treated surgically 6 years earlier, presented with a 2 cm tumor mass, palpable in the scar region. The histological examination and immunohistochemical study revealed a low-grade myoepithelial carcinoma. The patient had no complementary treatment. He presented 5 years later with a 2 cm parapharyngeal recurrence which was resected. There was no evidence of recurrent tumor or of distant metastasis at 4 year follow-up. DISCUSSION: Although myoepithelial cells are often predominant in pleomorphic adenomas, carcinomas developing in pleomorphic adenomas show in most cases a glandular differentiation and myoepithelial carcinomas are rare. Currently the diagnostic criteria, the prognosis, and the management of myoepithelial carcinomas are not well documented. Invasive carcinomas developing in pleomorphic adenoma are known to be highly aggressive neoplasms and myoepithelial carcinomas ex-pleomorphic adenoma seem to have a better prognosis. Our reported case was characterized by the weak aggressiveness of the lesion.     

Myoepithelial neoplasia of the submandibular gland: case report and therapeutic considerations

Tumors of the submandibular gland typically arise from the seromucinous acini, which make up the majority of the gland. The most common benign tumor of this structure is the pleomorphic adenoma, whereas the most common malignancy of the submandibular gland is adenoid cystic carcinoma. We describe an unusual case of a neoplastic process of the myoepithelial cells of the submandibular gland in a middle-aged woman. This rare tumor is most commonly diagnosed in the parotid gland and in the minor salivary glands of the hard palate; a review of the literature uncovered only 5 previous reports of myoepithelioma of the submandibular gland. Distinguishing myoepithelioma from benign pleomorphic adenoma and malignant myoepithelial carcinomas can be challenging. Immunohistochemical staining can help to distinguish between the benign neoplasms, but histologic features remain the "gold standard" for diagnosing the malignant tumors. Increasing use of immunohistochemistry panels to assess parotid neoplasms also suggests that myoepithelioma may be underrecognized.     

Primary pleomorphic adenoma of the external ear canal. Report of a case and literature review

INTRODUCTION: Primary neoplasms of the external ear canal are rare, and 5% of these tumors are of glandular origin. Ceruminal glands are modified sweat glands of the skin of the external auditory meatus that may give rise to (a) benign tumors such as ceruminous adenoma, pleomorphic adenoma, and syringocystadenoma papilliferum, and (b) malignant tumors such as ceruminous adenocarcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma. An alternative theory holds that pleomorphic adenomas may well arise from ectopic salivary tissue present in the external ear canal. These tumors are very rare and usually have a benign course. In this report, we describe an unusual case of pleomorphic adenoma of the external auditory canal associated with chronic infection of the middle ear. CASE REPORT: A 60-year-old woman presented with exacerbation of left otalgia over a 6-month period. She had been affected with chronic otitis media and aural polyps for the last 13 years, for which she had received medical treatment only. A canal-filling aural polyp was noted on clinical examination. Subsequent biopsy and histologic examination revealed pleomorphic adenoma of the external ear canal, possibly with malignant elements. Magnetic resonance imaging showed no intracranial extension or any association with the adjacent parotid gland. The patient underwent modified radical mastoidectomy and complete resection of the tumor and the entire skin of the external auditory canal. Final histology and immunohistochemistry confirmed the absence of malignancy, and no recurrence has been reported 1 year postoperatively. CONCLUSIONS: Pleomorphic adenoma is an extremely rare tumor arising from the ceruminal glands of the external ear canal. Nonspecific presentation and difficult histologic diagnosis characterize this benign neoplasm. Wide local excision is the mainstay of treatment.     

Tumors of the parotid glands in the material of the Department of otolaryngology of the Medical Academy in Warsaw in 1986-1995

In the Department of Otolaryngology, Medical Academy in Warsaw, 332 patients were surgically treated between 1986 and 1995 because of parotid gland tumors. In 275 cases benign tumors, in 57 cases--malignant tumors were observed. The majority of benign tumors were pleomorphic adenoma (61.45%) and lymphadenoma (22.54%); the majority of malignant tumors were adenocarcinoma (28%), adenoid cystic carcinoma (21%), mucoepidermoid carcinoma (15.78%). All patients with benign tumors were surgically treated, patients with malignant tumors were operated on and irradiated. The surgical procedure used superficial parotidectomy and total parotidectomy with preservation of facial nerve where possible. Ten patients required total parotidectomy with facial nerve resection because of malignant tumors, six patients had neck dissection performed. Of the 57 patients treated on account of malignant tumors 43 survived; 14 patients died of a local recurrence or generalization neoplasm disease.     

Malignant myoepithelioma arising from recurrent pleomorphic adenoma of the soft palate

Malignant myoepithelioma is a rare salivary gland neoplasm that can arise either de novo or within a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma arising from a pleomorphic adenoma of the soft palate. A 70-year-old woman presented in our department with a very large tumor of the soft palate. The patient had a history of a pleomorphic adenoma at the same location that was surgically removed 2 years ago. A second operation, with intraoral excision of the tumor was performed. Histological examination of the recurrence revealed a malignant spindle cell neoplasm with an infiltrative growth pattern and a high mitotic rate. Immunohistochemical investigation confirmed the diagnosis of a low-grade carcinoma of myoepithelial origin with free surgical margins. The patient remains free of disease for more than twelve months after the end of treatment.     

Salivary gland tumors in children.

Fewer than 5% of all primary salivary gland neoplasms occur in children, but if benign supporting tissue tumors are excluded, a higher proportion than in adults are malignant. The first decade of life, and particularly the first 2 years of life, has a preponderance of benign neoplasms. Commencing with the second decade, carcinomas rise in incidence and are most often mucoepidermoid and acinic cell carcinomas. The pleomorphic adenoma is the most common epithelial salivary tumor throughout childhood. The embryoma may be a uniquely childhood epithelial salivary gland tumor.     

Malignant mixed tumor of the nasal cavity

Parotid gland is the most common location of malignant mixed tumors. Three different subtypes of these tumors exist: carcinoma ex pleomorphic adenoma, carcinosarcoma, and metastasizing mixed tumor. Carcinoma ex-pleomorphic adenoma is by far the most common subtype. Although unusual sites of these tumors have been published, we report an extremely rare case of a malignant mixed tumor located in the nasal cavity.     

Carcinosarcoma of the submandibular gland: an autopsy case

Malignant mixed tumors of salivary glands are classified as three types: carcinoma arising in a pleomorphic adenoma, the most common; benign metastasizing pleomorphic adenoma; and carcinosarcoma (true malignant mixed tumor), which is very rare [Ann. Otol. Rhinol. Laryngol. (1982) 91 342]. In carcinoma, both epithelial and soft tissue elements are malignant. In the context of previous reports, we discuss a case of carcinosarcoma of the submandibular gland including the results of postmortem examination.     

Malignant myoepithelioma arising from recurrent pleomorphic adenoma of minor salivary gland.

Malignant myoepitheliomas of the salivary gland are very rare tumors which may either arise de novo or develop in a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma of the minor salivary gland that progressed from benign pleomorphic adenoma. The original pleomorphic adenoma was resected in 1979 (the '79 tumor). The first recurrent tumor was operated in 1995 (the '95 tumor). The '95 tumor was diagnosed as pleomorphic adenoma. Although the myoepithelial tumor component was more prevalent in the '95 tumor, histological features of the first recurrent tumor were similar to the '79 tumor. The second recurrent tumor showed more aggressive clinical features (the '98 tumor). It also showed massive growth of myoepithelial tumor cells with bone invasion, which led to the diagnosis of the '98 tumor as malignant myoepithelioma. With adequate surgical margin, the patient has been free from tumor recurrence for 20 months.     

Histology of submandibular gland tumours, 10 years' experience

Introduction and objectivesThere are few reports focused on neoplasms in the submandibular gland because they are uncommon and are frequently grouped with the other salivary glands tumours. In the classical studies, the proportion of malignancy reported in these cases is around 50%.ObjectivesDetermining the proportion of malignancy, the most frequent histological types, the gender distribution and average age at diagnosis in patients with submandibular gland neoplasms who were treated in our hospital from 2000 year to 2010.MethodsA retrospective review of our department database of the patients who underwent surgery for submandibular gland neoplasm in a ten-year period was performed.ResultsTwenty-two patient records were included, in which 19 (86%) patients presented benign disease and 3 were of a malignant type (adenocarcinoma, well-differentiated epidermoid carcinoma, follicular lymphoma). Two of the 3 cases of malignant neoplasms were in men.ConclusionsA greater proportion of benign neoplasm was found in submandibular tumours. The most frequent benign tumour is the pleomorphic adenoma. Women are more commonly affected (76%). Benign tumours appear in younger patients than do malignant ones.     

Carcinoma ex pleomorphic adenoma of the salivary gland: an immunohistochemical study

The proliferative activity of the tumor cells and the expression of tumor-associated genes and sex steroid hormone receptors were investigated immunohistochemically in ten cases of carcinoma ex pleomorphic adenoma (Ca-ex-PA) of the salivary glands. These were analyzed in benign and malignant components separately, and then were compared with ten cases of the other malignant tumors [adenocarcinomas, not otherwise specified (ACN) and salivary duct carcinomas (SDC)] and ten cases of pleomorphic adenomas (PA). The results obtained in this study were as follows: (1) malignant component of Ca-ex-PA showed a higher incidence of PCNA and Ki67 than benign component of Ca-ex-PA. A significant difference between benign component of Ca-ex-PA and PA was not observed. (2) A significant difference in the incidence of p53, c-erbB-2, EGFR overexpression was observed only between malignant component of Ca-ex-PA and benign component of Ca-ex-PA. (3) The incidence of PCNA, Ki67, p53, c-erbB-2 overexpression in malignant component of Ca-ex-PA showed the highest data among the four groups. These results suggest that Ca-ex-PA acquired the particular biological behavior in contrast to the other salivary neoplasms in the long-standing process while PA undergoes malignant transformation.     

Submandibular gland tumors. Adverse histologic factors and therapeutic implications

We reviewed our 41-year experience with tumors of the submandibular gland to determine what factors influence outcome and their implications for treatment. The most common benign neoplasm was pleomorphic adenoma (21), while among malignant tumors the adenoid cystic variety (37) predominated. For the 86 patients who had malignant tumors, the 2- and 5-year survivals by the life table method were 82% and 69%, respectively. For patients with malignant tumors, histology, size, perineural invasion, and prior treatment did not affect overall survival. Factors adversely affecting outcome were extraglandular soft-tissue extension and lymph node metastasis. Local-regional control was enhanced in patients with soft-tissue extension if they were treated by surgery followed by radiotherapy rather than by surgery alone.     

Malignant myoepithelioma of the soft palate

Malignant myoepitheliomas (MM) (myoepithelial carcinomas) are rare tumors representing <1% of salivary gland tumors. They are characterized as being locally aggressive. Rarely do they present distant metastases; however, when they do metastasize the sites most affected are the lungs, liver, pleura, peritoneum and skin. They may originate de novo in a pleomorphic adenoma or a benign myoepithelioma. We report the case of a patient with a submucosal lesion of the soft palate measuring ∼4 cm × 3 cm. The patient underwent transoral resection with a microscope and CO₂ laser. Histopathological report was MM originating in a pleomorphic adenoma. Management of this neoplasm is controversial. Myoepithelial carcinoma is a rare neoplasm whose diagnosis includes immunohistochemical (IHC) studies. Surgery is the cornerstone of treatment. Management with laser surgery may preserve the function of the soft palate without deterioration of the quality of life in these patients.     

Carcinomas ex monomorphic adenoma of salivary glands

A clinicopathological analysis of eight examples of carcinomas arising from salivary gland monomorphic adenomas, carcinomas ex monomorphic adenoma, is presented. These uncommon to rare neoplasms have a predilection for the parotid glands, are diagnosed about a decade later than their benign precursors, and most often arise from the dermal analogue type of monomorphic adenoma. As judged by follow-up periods of two to 16 years, carcinomas ex monomorphic adenoma are locally aggressive neoplasms with the clinical course marred by recurrences but without regional or distant metastases.     

Parapharyngeal space tumors: Fifty-one cases managed in a single tertiary care center

Conclusions: Pre-operative planning for parapharyngeal tumors must include meticulous analysis. Factors such as tumor size, distance to cranial base, and relation to neurovascular structures must guide the selection of a surgical approach. Objective: To summarize experience in diagnosis and surgical management of parapharyngeal tumors, analyzing the frequencies of various tumoral types, clinical presentation, choice of surgical approach and outcomes. This study also compares the results with the most relevant case series in the literature. Methods: A retrospective review was performed of the records of 51 patients treated by the team, from 1984–2012. Only primary tumors were included, excluding invasion from adjacent spaces and metastatic disease. All patients underwent imaging studies and surgical resection of the neoplasm. Cytological analysis and arteriography were used on an individualized basis. Surgical excision was performed via different approaches, predominantly through a cervicoparotid route. Results: Benign neoplasms were predominant (80%), and the most frequent tumor was pleomorphic adenoma. FNAC had a 100% accuracy to differentiate benign vs malignant tumors. The most common post-operative sequel was compromise of a cranial nerve, and three patients presented local complications after surgery. After follow-up, only three of 41 patients with benign tumors had recurring disease.     

'Metastasizing pleomorphic salivary adenoma' should now be considered a low-grade malignancy with a lethal potential

PURPOSE OF REVIEW: To review the current knowledge on the clinical entity of metastasizing pleomorphic adenoma. RECENT FINDINGS: Using case studies, the literature continues to explore the potential to identify a chromosomal abnormality that might explain the clinical entity, and may constitute a submicroscopic malignant transformation with molecular genetic alterations. Presently there are no agreed histopathological parameters that can identify or confirm a difference in behavior between the 'common' pleomorphic adenoma from the metastasizing pleomorphic adenoma. SUMMARY: The evidence supports that metastasizing pleomorphic adenoma is most likely to be an unrecognized and as yet unclassified malignant neoplasm and as such must be considered a low-grade, potentially lethal malignant disease when it presents, which may constitute a submicroscopic malignant transformation with molecular genetic alterations. Further research is required to answer the many questions.     

Carcinosarcoma arising from a preexisting pleomorphic adenoma--true malignant mixed tumor of the parotid gland

Carcinosarcoma (true malignant mixed tumor) is rare in salivary gland neoplasms. Even rarer is a carcinosarcoma arising from a preexisting pleomorphic adenoma. Case: a 49-year-old woman was admitted with left neck pain. Computed tomography and USG-examination revealed a mass originating from left parotid gland. The patient undergone total parotidectomy with facial nerve preservation and then irradiation and chemioteraphy. Clinical and histologic date revealed it had developed from a preexisting pleomorphic adenoma. Conclusion: microscopic examination showed and neoplasm comprised of sarcomatous and carcinomatous cells. This tumor seemed to consist of two histogenetically different populations of cells. We also review the literature and dicuss the histogenetic origin of the carcinosarcoma of salivary gland.