A case report of a rare nasopharyngeal myoepithelial dominant pleomorphic adenoma

IntroductionPleomorphic adenoma is the most common benign salivary gland neoplasm. The majority of cases occur in the major salivary glands; however, they can also originate from the minor salivary glands. The nasopharynx is an uncommon site, but it has been reported in the literature. The characteristic difference of our report from the literature is the young age of our patient and his previous unremarkable medical or surgical history.The objective of this report is to present a case of salivary gland pleomorphic adenoma, discuss radiological and histopathological findings and treatment options.Case presentationHerein we report a case of a 25-year-old male patient presenting with a long-term history of snoring, mouth breathing, and progressive left nasal obstruction. After examining the patient and confirming the diagnosis, patient successfully underwent tumor resection using a combined transnasal/transoral endoscopic approach with no complications following surgery and significant improvement of the previously reported symptoms.DiscussionPleomorphic adenoma of the minor salivary glands can occur anywhere throughout the distribution sites of these glands such as: along the upper aerodigestive tract, parapharyngeal fat spaces, soft palate, the sinonasal, and nasopharyngeal areas.ConclusionThe occurrence of pleomorphic adenoma in uncommon sites has been reported in the literature, and the nasopharynx is considered to be one of these uncommon sites. The mainstay of treatment for nasopharyngeal pleomorphic adenoma is surgical excision as they can grow to giant sizes if left untreated.     

Recurrent pleomorphic adenoma of the parotid gland

BACKGROUND: Surgery of recurrent pleomorphic adenoma presents an increased risk of facial nerve injury and a considerable re-recurrence rate. METHODS: A series of 33 patients with first recurrence of pleomorphic adenoma of the parotid gland was analyzed. The data were derived from medical records as well as from interviews and clinical examinations of all living patients. Histologic material of the initial and recurrent tumor were reviewed. RESULTS: Multifocal recurrence and carcinoma in pleomorphic adenoma were observed in 73% and 9% of patients, respectively. The incidence of permanent partial facial nerve injury after surgery was 23% in patients with initial enucleation and 14% in those with initial superficial parotidectomy (including 1 patient with facial nerve resection and 1 patient with a partial facial paresis before recurrence surgery). A subsequent recurrence occurred in 6 patients, all with initial enucleation after a mean time interval of 9 years. CONCLUSIONS: The preservation of the facial nerve was possible in all but 1 patient treated for the first recurrence with a relatively low rate of permanent partial facial paresis because of the use of the operating microscope and facial nerve monitor. To evaluate the re-recurrence rate, a follow-up of at least 10 years is necessary.     

Myoepithelial Carcinoma Ex Pleomorphic Adenoma of the Maxillary Sinus: A Case Report and Review of Literature

Myoepithelial carcinoma ex pleomorphic adenoma is defined as a malignant epithelial neoplasm arising from a primary or recurrent benign pleomorphic adenoma. This type of tumor comprises 3.6% of all salivary gland tumors and 12% of malignant ones. Clinically, it most commonly presents as a firm mass in the parotid gland. The development of this neoplasm in the sinonasal and nasopharyngeal regions is extremely rare and only few cases are reported in the literature. The prognosis of myoepithelial carcinoma is variable. Marked cellular pleomorphism, high mitotic rate, and high proliferative activity correspond to a poor prognosis. In this article, the authors report the histopathological features of a clinical case of a 64-years-old patient with a large median maxillary neoplasm diagnosed as myoepithelial carcinoma/ex-pleomorphic adenoma. The tumor was resected and subjected to secondary reconstruction using a revascularized free fibula flap. The myoepithelial derivation of neoplastic cells was demonstrated by immunohistochemical positivity for S-100 protein (strong and diffuse), cytokeratin 14 (strong and diffuse), and GFAP (focal).     

Pleomorphic adenoma of the breast should be excised with a cuff of normal tissue

Pleomorphic adenoma is a benign tumor found rarely in the breast but commonly in the salivary gland. Unlike the salivary gland variant management guidelines are poorly defined in the breast. We describe the first case of pleomorphic adenoma of the breast that has recurred for the second time following previous surgical excisions, and review the available literature. Due to the risk of recurrence and malignant transformation, we recommend complete excision of the lesion with a cuff of normal tissue, as is the practice in the salivary gland. Clinicians should be aware of the condition, as preoperative diagnosis will facilitate adequate surgery. Patients should be informed about the risk of recurrence. We recommend follow-up for at least a period of 5 years with yearly clinical examinations.     

Neutron radiotherapy for recurrent pleomorphic adenomas of major salivary glands.

INTRODUCTION: Pleomorphic adenoma is the most common neoplasm arising in the salivary glands. Surgical management is the primary therapeutic modality. With the use of modern surgical techniques, recurrence is infrequent, and facial nerve sparing is the norm. However, for patients with recurrent disease, the risk of further relapses is increased with surgical resection alone, particularly for those patients in whom multiple recurrences have already occurred. The role of adjuvant radiotherapy in this setting remains uncertain. Although neutron radiotherapy is superior to conventional radiotherapy for malignant salivary gland tumors, its role in the treatment of pleomorphic adenomas is less well defined. We report our experience using this modality for high-risk, recurrent pleomorphic adenomas. METHODS: Sixteen patients were treated with neutron radiotherapy for recurrent pleomorphic adenomas of major salivary glands from 1986 through 1993. The median age at diagnosis was 33 years (range, 11-77 years); median age at the time of neutron radiotherapy was 52 years (range, 22-77 years); median number of prior surgical procedures was 3 (range, 1-6); median duration from initial diagnosis to radiotherapy was 14.5 years (range, 3 months-30 years); median follow-up was 83 months (range, 9-144 months). The median period at risk for survivors was 96 months (defined as the interval from completion of neutron radiotherapy to last follow-up). Ten patients had evidence of gross residual disease at the time of treatment as determined by imaging studies, with nine patients having multinodular disease. RESULTS: The 10-year actuarial survival was 79%. One patient died from lung metastases 9 months after treatment; one patient died from a liver tumor of uncertain origin, but the histology could not rule out a metastasis from the previous pleomorphic adenoma; and one patient died from recurrent disease at the base of skull. The 15-year actuarial locoregional control rate was 85%. One of the two patients with locoregional recurrence had a malignant transformation into an adenocarcinoma. No statistical difference in 15-year actuarial survival (75% vs 83%, p =.82) was found comparing patients with gross residual disease vs microscopic residual disease. The actuarial 15-year locoregional control was 76% for patients with gross residual disease vs 100% for those with microscopic disease. The 15-year actuarial risk of RTOG/ESTRO nonaudiologic grade III/IV complications was 21%. No facial nerve injuries were observed as a direct consequence of neutron radiotherapy. CONCLUSIONS: Neutron radiotherapy offers both excellent local control rates and survival rates in patients with multiply recurrent pleomorphic adenomas that are not candidates for surgical resection, even in the presence of gross residual disease. The treatment-related morbidity is acceptable. Malignant transformations and metastases, although uncommon, may be observed in this tumor.     

Benign metastasizing pleomorphic adenoma of the parotid gland: a clinicopathologic puzzle

BACKGROUND: Pleomorphic adenoma constitutes the most common benign parotid gland tumor. Local recurrence after surgical treatment (lateral or total parotidectomy) has been described in 1% to 5% of cases. Malignant degeneration has been reported in 2% to 9% of cases of pleomorphic adenoma of salivary gland origin. Metastasizing pleomorphic adenomas without histologic evidence of malignancy have rarely been reported. Metastatic lesions have been discovered in bone, lymph nodes, the lung, oral cavity, pharynx, skin, liver, retroperitoneum, kidney, calvarium, and central nervous system. To the best of our knowledge, we hereby report the first case of pleomorphic adenoma of the parotid gland metastasizing to the ipsilateral maxilla. METHODS: We simultaneously examined apoptosis-related protein expression and markers of cell-proliferation activity in our case of benign pleomorphic adenoma metastasis and compared outcome with a control group of primary parotid pleomorphic adenomas. RESULTS: Analysis of p53, Bcl-2, MIB1, CD 105, p27, and p21 expression did not reveal significant differences between metastasizing pleomorphic adenoma of the salivary gland and the control group of primary parotid pleomorphic adenomas. CONCLUSIONS: Clinical rather than pathologic evidence seems to justify inclusion of metastasizing salivary pleomorphic adenoma in the group of low-grade malignant salivary tumors.     

Salivary Gland Tumor: A Review of 599 Cases in a Brazilian Population

Salivary gland tumors consist of a group of heterogeneous lesions with complex clinicopathological characteristics and distinct biological behaviors. Worldwide series show a contrast in the relative incidence of salivary gland tumors, with some discrepancies in clinicopathological data. The main aim of this study was to describe demographic characteristics of 599 cases in a population from Central Brazil over a 10-year period and compare these with other epidemiological studies. Benign tumors represented 78.3% of the cases. Women were the most affected (61%) and the male:female ratio was 1:1.6. Parotid gland tumors were the most frequent (68.5% of cases) and patient age ranged from 1 to 88 years-old (median of 45 years old). The most frequent tumors were pleomorphic adenomas (68.4%) and benign tumors were significantly more frequent in the parotid (75.9%), while malignant tumors were more frequent in the minor salivary glands (40%) (P < 0.05). In conclusion, women and the parotid gland were the most affected and pleomorphic adenoma was the most frequent lesion, followed by adenoid cystic carcinoma and Warthin’s tumor.     

Giant submandibular gland tumor: mucoepidermoid carcinoma within the pleomorphic adenoma (mixed tumor)

Pleomorphic adenoma (mixed tumor) is the most common type of salivary gland tumors, most often involving the parotid glands. The study presented a case of a giant pleomorphic adenoma of the submandibular gland in a 78-year old woman. Due to the initially slow and progressive growth (during a period of 25 years) without treatment, the tumor attained a size of 25×19×16 cm and a weight of 4 kg. The patient underwent surgery. The histopathological examination showed that the tumor was subject to malignant transformation: within the pleomorphic adenoma one observed mucoepidermoid carcinoma lesions of high-degree malignancy. In spite of the good postoperative esthetic and functional effect the patient was diagnosed with distant metastases to the lungs.     

Carcinoma ex pleomorphic adenoma of the submandibular gland with distant metastases

A case of metastatic carcinoma ex pleomorphic adenoma (PA) of the submandibular gland is reported. While reports of local recurrence of this cancer are numerous, few cases of distant metastases have been reported. We report a case of a 52-year-old male patient with a history of PA diagnosed ten years previous. The patient presented reporting that the mass had increased size and pain during the previous six months. Needle biopsy results of this mass were compatible with a poorly differentiated carcinoma of the salivary ducts. Radical surgery with wide right neck dissection was performed with curative intent. Three years post-surgery liver and bony metastases were detected. In conclusion, the potential for malignant transformation of PA demands close follow-up of younger patients.     

The Diagnostic Dilemma of a Parasympathetic Schwannoma in the Submandibular Gland

Schwannomas are benign soft tissue tumours that arise from nerve sheath cells. They are rare in the salivary glands and are thought to arise from the parasympathetic nerves. We report the case of a 69-year-old man who presented with a painless right submandibular mass that appeared to resemble a pleomorphic adenoma on ultrasonography and, on this basis, proceeded to surgical excision of the gland. We review the relevant literature and highlight the diagnostic pitfalls.     

Carcinoma ex Pleomorphic Adenoma of the Nasal Cavity

Background  

Carcinoma ex pleomorphic adenoma (CXPA) is a malignant epithelial neoplasm arising in a benign mixed tumor (i.e., pleomorphic adenoma or PA); it accounts for approximately 3–4% of all salivary gland neoplasms. CXPA is exceedingly rare in the nasal cavity, with only three cases previously documented.     

A case of carcinosarcoma arising in the submandibular gland.

A 54-year-old man presented with an 8-year history of a hard asymptomatic mass of the left submandibular area. Total excision of the left submandibular gland with radical neck dissection was performed under a diagnosis of a submandibular tumor, probably a malignant mixed tumor. The pathologic diagnosis was carcinosarcoma consisting of carcinomatous and sarcomatous elements. The epithelial component was composed of squamous cell carcinoma, undifferentiated carcinoma, and adenocarcinoma. The nonepithelial component was composed of chondrosarcoma, osteosarcoma, spindle cell sarcoma, rhabdomyosarcoma, and liposarcoma. In the central area of the tumor, a few remnants of benign pleomorphic adenoma were identifiable. The finding suggested that in our patient, the carcinosarcoma arose from a preexisting pleomorphic adenoma. In view of the expected aggressive nature of the tumor, the patient was treated with postoperative radiotherapy of 60 Gy total, in 30 daily fractions of 2 Gy, and chemotherapy. He currently remains well and free of disease 24 months after treatment.     

Pleomorphic Adenoma of the Trachea Mimicking Bronchial Asthma: Report of a Case

Benign tracheal tumors are rare and difficult to diagnose. A delay in diagnosis is common because the symptoms mimic those of bronchial asthma. We report the case of a 42-year-old woman who was treated for asthma for 2 years before a diagnosis of pleomorphic adenoma of the trachea was made. To our knowledge, only 33 patients with pleomorphic adenoma of the trachea have been reported. We treated our patient by performing circumferential segmental tracheal resection and end-to-end anastomosis. A dynamic Y-stent was implanted for tracheal stenosis soon after the operation and it was removed 2 years later. The patient is now asymptomatic and no recurrence has been detected in the 5 years since diagnosis.     

Primitive neuroectodermal tumor of the adrenal gland

We report a rare case of primitive neuroectodermal tumor arising from adrenal gland in adulthood, diagnosed preoperatively as having non-functional adrenocortical adenoma. Laparoscopic adrenalectomy was performed. Immunohistological examination revealed the definite diagnosis as primitive neuroectodermal tumor of the adrenal gland. Although primitive neuroectodermal tumor is a highly malignant neoplasm, there is no evidence of local recurrence and distant metastasis 16 months after surgery.     

Pleomorphic adenoma of the breast: Report of a case

We present herein the case of a 74-year-old woman found to have a pleomorphic adenoma, or so-called mixed tumor, of the breast. The patient presented with a hard mass, 3 cm in diameter, located just beneath the left areola. Physical examination suggested a diagnosis of breast carcinoma; however, the findings of both ultrasonography and mammography indicated a benign neoplasm. Aspiration biopsy cytology was evaluated as class III and the diagnosis of pleomorphic adenoma was finally confirmed by examination of an excisional biopsy specimen. As it is a rare benign tumor, pleomorphic adenoma of the breast has been mistaken for a malignant tumor clinically, mammographically, cytologically, and even by frozen section. Thus, an awareness of this disease in the breast will help to prevent misdiagnosis and overaggressive surgery.     

Familial mixed tumors of the parotid gland

BACKGROUND: The most common neoplasm of the parotid gland is the pleomorphic adenoma. The familial occurrence of such tumors arising within the parotid gland is rare, with only 3 previous reports in the literature. Bilateral synchronous pleomorphic adenomas of the parotid gland are also uncommon. We report 2 siblings with pleomorphic adenomas of the parotid gland, 1 of whom had bilateral synchronous mixed tumors. Patients and Methods Chromosomal analysis of tumor cells from the sibling with bilateral adenomas revealed the translocation t(3;12)(p21;q15). Chromosome 12q breakpoints have previously been identified in a wide variety of solid tumors including pleomorphic adenomas of the parotid gland. CONCLUSIONS: We discuss bilateral mixed tumors, familial parotid tumors, and the potential for a genetic predisposition for the recurrence of such parotid tumors, as suggested by characteristic chromosomal translocations associated with mixed tumors.     

Small Cell Carcinoma ex-Pleomorphic Adenoma of the Parotid Gland

Small cell carcinoma (SCC) is a high-grade malignancy usually encountered in the lungs but also seen in almost any site including the salivary glands. SCC is important to recognize because it often metastasizes widely and is treated with systemic chemotherapy. Carcinoma ex pleomorphic adenoma is a malignant epithelial neoplasm arising in a pre-existing benign mixed tumor (i.e., pleomorphic adenoma, PA). While virtually any salivary carcinoma can arise from a PA, to our knowledge SCC ex-PA has not been described. We report a case of a woman presenting with fullness of the right neck and a parotid gland mass. The tumor was resected and evaluated grossly, by light microscopy, and by immunohistochemistry. Grossly, a 1.6 cm well-circumscribed nodule was identified within the parotid. Microscopic examination revealed foci of SCC associated with high-grade adenocarcinoma, in the background of a PA. The SCC was immunoreactive for cytokeratin in a dot-like pattern and neuroendocrine markers synaptophysin and CD56. Despite the focal nature of the SCC in the parotid, a pure SCC metastasis was present in one neck level II lymph node. The patient was free of disease with 8 months of follow-up. Our case illustrates that: (1) although rare, in the salivary glands SCC may arise from lower grade neoplasms including PAs; (2) SCC ex PA may metastasize as pure SCC even if the primary SCC component was focal; (3) adequate sampling of PAs is crucial to prevent missing a rare SCC that must be treated with chemotherapy.     

Surgical treatment for pulmonary metastasis from submandibular gland cancer

We reviewed surgical result of 5 patients with pulmonary metastasis from submandibular gland cancer. Pathological diagnosis was adenoid cystic carcinoma in 2 patients, carcinoma in pleomorphic adenoma in 1, epidermoid carcinoma in 1, and mucoepidermoid tumor in 1. Disease free interval from resection of the primary lesion to diagnosis of pulmonary metastasis was ranging from 7 to 76 months. Lobectomy was performed in 2 patients and partial lung resection in 3. Three patients died of tumor recurrence 12 to 28 months after lung resection and 2 patients with a single metastatic lesion have survived as long as 42 and 150 months after the operation. This study suggests that surgical treatment may be effective for pulmonary metastasis, especially for a single metastatic lesion, from submandibular gland cancer.     

Tumors of the major salivary glands in children

During the 30-year period 1955-1985, 21 children with neoplasms of the major salivary glands were treated at the Johns Hopkins Hospital. A thorough review of the English literature revealed an additional 472 cases. The cases were studied as to age, sex, site, histopathologic characteristics, mode of treatment, and results. The majority of the patients were between 8 and 20 years of age. There was a female preponderance of 1.42:1. The parotid gland was the site of origin in 85.1%, the submandibular in 11.7%, and the sublingual in 3.2%. An equal incidence of benign (50.1%) and malignant (49.9%) lesions is noted. Pleomorphic adenoma was the most common benign neoplasm and mucoepidermoid carcinoma the most common malignant neoplasm. The final prognosis seems to be similar with superficial or total parotidectomy, provided the tumor has been completely removed at the initial surgery. Re-excision is recommended for tumor recurrence, and the use of radiation therapy should be individually determined in cases of aggressive malignancies.     

Pleomorphic adenoma of the parotid gland: histopathologic analysis of the capsular characteristics of 218 tumors

BACKGROUND: Histopathologic features of the capsule may have an impact on the recurrence rate of pleomorphic adenomas. METHODS: Retrospective (n = 154) and prospective (n = 64) histologic analysis of the capsular characteristics such as incompleteness, tumor penetration, pseudopodia, and satellite tumors of 218 pleomorphic adenomas. RESULTS: In 160 of the 218 (73%) pleomorphic adenomas, 1 or more capsular characteristics such as incomplete capsule (33%), capsule penetration (26%), pseudopodia (40%), and satellite nodules (13%) were detected. Incomplete capsule and satellite tumors were most frequently seen in the stroma-rich (myxoid) subtype. Capsular penetration and pseudopodia were significantly more common in the prospective group than in the retrospective group (p < .05/<.05). CONCLUSION: Pseudopodia and satellite tumors were more common than reported in the literature. If left in the surrounding salivary gland tissue at surgery, they can lead to recurrences of pleomorphic adenomas of the parotid gland.