Recurrent Pleomorphic Adenoma Research Articles

Recurrent Pleomorphic Adenoma of the Parotid Gland: Intraoperative Facial Nerve Monitoring during Parotidectomy

To determine the benefit to postoperative facial function of intraoperative facial nerve monitoring (IFNM) during recurrent pleomorphic adenoma (RPA) parotid surgery. Cohort study with planned data collection. Chinese PLA General Hospital. Fifty-eight RPA parotidectomies were performed between 2004 and 2012. Recurrence was confirmed by histopathologic examination. None of the patients had preoperative facial palsy. Electromyography-based IFNM was used in 28 patients; 30 patients were unmonitored. The durations of surgery and the severity of postoperative facial nerve palsy were compared between the 2 groups. There were no significant differences between the 2 groups in the incidence of immediate or permanent facial paralysis after RPA parotidectomy (P = .95 and P = .36, respectively). However, the differences in the average duration of surgery and the severity of postoperative facial nerve palsy after total parotidectomy or wide resection were significant (P < .01 and P = .01, respectively). In contrast, these differences were not significant after superficial parotidectomies (P = .43 and P = .49, respectively). The average recovery time of temporary facial nerve paralysis was significantly shorter in the monitored group compared with the unmonitored group, independent of surgical technique (P < .01). The use of IFNM during total or wide resection RPA parotidectomy reduced the duration of surgery and the incidence of postoperative facial paralysis and enhanced recovery. However, there was little impact on facial nerve outcomes when IFNM was used during superficial RPA parotidectomy.

Recurrent salivary pleomorphic adenoma shows increased immunohistologic expression of bcl-2 oncoprotein.

Internal cell biology, including apoptotic regulation, is presumed to play a key role in the development of recurrent pleomorphic adenoma (PA). The aim of our study was to determine the relevance of B-cell lymphoma 2 (bcl-2) oncoprotein immunoexpression and distribution in primary PA, and its recurrence. Ten primary-non-recurrent, 14 primary-to-recur, and 28 recurrences of parotid PA patients aged 19-73 (mean 40.7±16.7) years were enrolled. The bcl-2 expression was compared between groups using a semi-quantitative histoscore, defined as the multiple of the percentage of cells by the intensity of immunostaining. Widely varying bcl-2 immunoreaction was found in the epithelial areas of 91.7% of primary and 85.2% of recurrent PA. Similarly varying but much less, immunopositivity was found in the myxoid areas of 62.5% of primary and 71.4% of recurrent tumours. No obvious differences in the bcl-2 staining intensity or pattern of specific epithelial morphologic structures in either the primary-non-recurrent, primary-to-recur or recurrent tumours were found. In both the mesenchymal and epithelial areas of PA, the differences in bcl-2 immunohistoscore between the primary-non-recurrent and primary-to-recur groups were not statistically significant (P=0.62, respectively 0.51). In the mesenchymal areas, the study revealed a significantly increased histoscore in recurrent tumours compared to their corresponding primaries (P=0.01). Increased bcl-2 expression in recurrent PA suggests an exaggerated aggressiveness of that tumor. The fact that a significant difference in the histoscore was found exclusively in the myxoid component seems to accord with the reported prevalence of the latter in recurrent and metastatic PA.

Lacrimal Gland Pleomorphic Adenoma and Carcinoma ex Pleomorphic Adenoma

To study genetic alterations in lacrimal gland pleomorphic adenoma (PA) and carcinoma ex pleomorphic adenoma (Ca-ex-PA) with focus on copy number changes and expression patterns of the translocation target genes PLAG1, HMGA2, and CRTC1-MAML2 in relation to clinical data. Experimental study. A total of 36 tumors from 32 patients with lacrimal gland PA or Ca-ex-PA were included in the study. Genome wide, high-resolution array-based comparative genomic hybridization (arrayCGH) and immunohistochemistry were used to study the genomic profiles and expression patterns of the translocation targets PLAG1, HMGA2, and CRTC1-MAML2. Copy number alterations (gains/losses) and protein expression of PLAG1, HMGA2, and CRTC1-MAML2. Genome-wide arrayCGH analysis revealed normal genomic profiles in 10 of 17 PA samples. The average number of genomic imbalances per tumor was 3.25 (range, 1-7) in primary and recurrent PAs with alterations compared with 7.7 (range, 4-12) in Ca-ex-PAs. Five recurrent copy number alterations were identified in PAs, including losses of 1pter-p31.3, 6q22.1-q24.3, 8q24.22-q24.3, and 13q21.31-q21.33, and gain of 9p23-p22.3. Gain of 9p23-p22.3 also was seen in a Ca-ex-PA. In Ca-ex-PA, gain of 22q12.3-qter was the only recurrent alteration. Detailed analysis of the array data identified NFIB and PDGFB as the 2 major candidate target oncogenes that may be activated as a result of copy number gains involving 9p and 22q. Both genes have been implicated in the pathogenesis of PA and other types of salivary gland tumors. Immunohistochemical analysis revealed frequent overexpression of the translocation target gene PLAG1 in PAs and in 1 Ca-ex-PA. In contrast, overexpression of HMGA2 was observed in only a small subset of PAs. The CRTC1-MAML2 fusion oncoprotein was overexpressed in 2 mucoepidermoid Ca-ex-PAs. Lacrimal and salivary gland PAs and Ca-ex-PAs have similar genomic profiles and frequently overexpress the PLAG1 oncoprotein. Copy number gains involving 9p23-p22.3 (NFIB) and 22q12-qter (PDGFB) may be of importance for disease progression in a subset of lacrimal gland PAs.

Management of trismus by masseter myotomy

Mild trismus after parotid gland surgery may be related to inflammation and fibrosis of the masseter muscle. We present a case of long standing trismus due to masseter muscle spasm caused by a recurrent pleomorphic adenoma. As the patient did not agree for removal of the pleomaorphic adenoma, owing to the risk of injuring the facial nerve, a masseter myotomy with inter-positioning of temporalis fascia was planned for symptomatic relief. Mouth opening showed significant improvement even 1 year post-operatively.

Myoepithelial carcinoma arising in recurrent pleomorphic adenoma of the soft palate: A case report with review of literature

Myoepithelial carcinomas (MCs) are rare neoplasm of salivary glands composed predominantly of cells with myoepithelial differentiation characterized by infiltrative growth, necrosis, multinodular architecture, and potential for metastasis. Majority are seen in major salivary glands like parotid. They can arise either de novo or within a preexisting pleomorphic adenoma or myoepithelioma, the latter being very uncommon. We report a rare case of malignant myoepithelioma arising from a pleomorphic adenoma of the soft palate. A 66-year-old male patient presented with recurrent soft palate mass, with history of a pleomorphic adenoma at the same location operated 2 years ago. The recurrent tumor showed histopathological features of MC, confirmed by immunohistochemistry.

Massive multinodular head and neck recurrence of parotid gland pleomorphic adenoma: a case report.

Introduction. The optimal initial management of parotid pleomorphic adenomas reduces the risk of recurrence and malignant transformation. Surgery of recurrence can be difficult in multinodular disseminated forms. Case Report. A 67-years-old patient was referred for management of a large multifocal recurrence of a pleomorphic adenoma operated on 23 years ago. The clinical and radiological assessment found parapharyngeal, infratemporal, and prestyloid invasion, with nodules in the sternocleidomastoid muscle. Excision by transmandibular approach was performed. The pathologist found a multinodular recurrent pleomorphic adenoma without criteria of malignancy. Postoperative radiotherapy was performed. Discussion. Multinodular forms and incomplete resections are the most important factors that are thought to predispose to recurrence. A precise analysis of the extension by preoperative MRI is essential. Adjuvant radiotherapy can be given in these recurrent multifocal forms.

Neither expression of VEGF-C/D nor lymph vessel density supports lymphatic invasion as the mechanism responsible for local spread of recurrent salivary pleomorphic adenoma

Recent research suggests that multinodular recurrent pleomorphic adenoma (PA) might result from cell migration through lymphatics. Lymphangiogenesis in malignancies is mediated by vascular endothelial growth factors C and D (VEGF-C/D). We studied the expression of VEGF-C/D in PA by immunohistochemistry as well as lymphatic vessel density (LVD). In 6 non-recurrent, 4 primary-to-recur, and 10 recurrent PAs, VEGF-C/D expression was assessed by immunohistochemistry. Staining was scored in terms of staining intensity (0 = absent to 3 = strong), and the percentage of positive tumor cells (scored as 0 (0-19 %), 1 (20-39 %), 2 (40-50 %), and 3 (60-100 %)) and a sum score were calculated. Intra- and peritumoral LVD was assessed by counting of LV after immunostaining, using the D2-40 antibody. All but one sample were VEGF-C negative. The differences in VEGF-D expression between non-recurrent, primary-to-recur, and recurrent PAs were not significant (p>0.05). VEGF-D expression did not correlate with peritumoral LVD (p>0.05). Our study revealed a significant difference between intra- and peritumoral LVD values when comparing individual and all sample groups (p=0.01). The lack of VEGF-C expression and of significant differences in VEGF-D expression and peritumoral LVD between patients with non-recurrent, primary-to-recur, and recurrent PAs does not support the lymphangiogenic local spread hypothesis

Recurrent Pleomorphic Adenoma

Recurrent Pleomorphic Adenoma

Carcinoma Ex Pleomorphic Adenoma in the Oral Cavity

Carcinoma ex pleomorphic adenoma (CEPA) is a rare, aggressive, poorly understood malignancy. In CEPA, an epithelial malignancy develops in association with a primary or recurrent benign pleomorphic adenoma. Carcinoma ex pleomorphic adenoma is very difficult to identify before surgery because the clinical presentation of many cases is similar to that of pleomorphic adenomas. The risk for malignancy increases with the duration of a mixed tumor. Treatment of CEPA must be individualized on the basis of the tumor location, involvement of adjacent structures, histologic subtype, and grade. The authors recently experienced a case of CEPA arising in the oral cavity with neck metastasis. The patient was a 70-year-old man presenting a huge mass that was present for 20 years and that slowly grew on the left side of the neck. We treated it with a total excision with wide margins and neck dissection. There was no recurrence during the follow-up period of 5 years up until now. We present a case of an unusually huge CEPA in the oral cavity.

Prognostic factors for secondary recurrence of pleomorphic adenoma: a 20-year, retrospective study

To evaluate the results of treatment of parotid pleomorphic adenoma, and the risk factors for secondary recurrence. Single-centre, retrospective study of 32 patients with pleomorphic adenoma recurrence managed between 1988 and 2008. The mean age at diagnosis of primary pleomorphic adenoma recurrence was 43.4 years. Twenty-eight per cent of patients had secondary recurrence; 32 per cent had undergone two or more surgical resections and external adjuvant radiotherapy. An age of less than 25 years was significantly associated with an earlier primary recurrence (p = 0.008). The most significant histopathological risk factor for secondary recurrence was the presence of a multifocal tumour (p = 0.019). Other histopathological criteria (i.e. cellularity and capsule rupture) were not significant. Radiotherapy was not associated with a decrease in recurrence. Nine per cent of patients progressed to malignancy. The main surgical complication was definitive facial palsy (14 per cent). Pleomorphic adenoma recurrence requires surgery, with greatly increased risk to the facial nerve. Resection with clear surgical margins is required, especially in young patients with multifocal tumours. Radiotherapy may delay second recurrence in cases of multifocal tumour.

Thoracodorsal nerve graft for reconstruction of facial nerve branching

ObjectSurgical treatment of parotid malignancies may frequently involve facial nerve amputation to achieve oncological radical resection. The entire facial nerve branching from its exit from the stylomastoid foramen to the periphery of the gland is often sacrificed. The first reconstructive strategy is the immediate reconstruction of the facial nerve by directly anastomosing the trunk of the facial nerve to its distal branches by interpositional nerve grafting.The present study was performed to determine the adequacy of thoracodorsal nerve grafting for immediate repair of the facial nerve. The anatomical features of the thoracodorsal nerve make it particularly appropriate to match its trunk to the stump of the facial nerve at its exit from the stylomastoid foramen. Up to seven branches of the thoracodorsal nerve may be distally anastomosed to the severed distal branches of the facial nerve. More complex reconstruction may be addressed simultaneously by contemporary harvesting a de-epithelialized free flap from the same site based on thoracodorsal vessel perforators and preparing a rib graft from the same donor site. MethodsBetween October 2003 and August 2010, seven patients affected by parotid tumors (6 with parotid malignancies and 1 with multiple recurrences of pleomorphic adenoma) underwent radical parotidectomy with intentional sacrifice of the facial nerve to obtain oncological radical resection. In all patients, the facial nerve was reconstructed with an interpositional thoracodorsal nerve graft. In four patients, a de-epithelialized free flap based on the latissimus dorsi was transposed to cover soft tissue defects. Moreover, two of these patients also required a rib graft to reconstruct both the condyle and ramus of the mandible. With the exception of one patient affected by recurrent pleomorphic adenoma, all patients underwent radiotherapy after surgical treatment. ResultsAll patients in our study recovered mimetic facial function. Facial muscles showed clinical signs of recovery within 5–14 (mean: 7.8) months, with varying degrees of mimetic restoration, and almost complete facial symmetry at rest in all patients. The House–Brackmann final score was I in two patients, II in two patients, and III in three patients. ConclusionsA thoracodorsal nerve graft to replace extratemporal facial nerve branching is a valid alternative technique to multiple classical nerve grafts, with good matching at both the proximal and distal anastomoses.

Myoepithelial carcinoma arising in recurrent pleomorphic adenoma in maxillary sinus

Myoepithelial carcinoma is characterized by nearly exclusive myoepithelial differentiation and evidence of malignancy. It may arise de novo or in preexisting benign tumors including pleomorphic adenoma and benign myoepithelioma. A 39-year-old lady presented with painless progressive swelling on the right cheek and right side of palate. On surgery, there was a mass in right maxillary sinus which was surgically excised and diagnosed on histopathology as pleomorphic adenoma. Subsequently, there were two recurrences. The first recurrence was in the right maxilla after 2 years that was removed surgically and diagnosed as pleomorphic adenoma. One year later, she came with rapidly progressive swelling in bilateral cheeks and face. Intraoperatively, there was a large tumor in both maxillary sinuses with extensive local infiltration. Histologically, it was diagnosed as myoepithelial carcinoma. Carcinoma ex pleomorphic adenoma is usually a high grade malignancy. It occurs most commonly in parotid gland followed by submandibular glands, minor salivary glands and occasionally in sublingual gland. To the best of our knowledge, this is the first case of myoepithelial carcinoma arising in a recurrent pleomorphic adenoma in the maxillary sinus.

Comparison of ultrasound-guided core biopsy versus fine-needle aspiration biopsy in the evaluation of salivary gland lesions

Ultrasound-guided core biopsy provides many benefits compared with fine-needle aspiration cytology and has begun to emerge as part of the diagnostic work-up for a salivary gland lesion. Although the increased potential for tumor-seeding and capsule rupture has been extensively discussed, the safety of this procedure is widely accepted based on infrequent reports of tumor-seeding. In fact, a review of the literature shows only 2 cases of salivary tumor seeding following biopsy with larger-gauge needle characteristics, with 2 reported cases of salivary tumor seeding following fine-needle aspiration cytology. However, the follow-up interval of such studies (<7 years) is substantially less than the 20-year follow-up typically necessary to detect remote recurrence. Studies on tumor recurrence of pleomorphic adenoma, the most common salivary gland lesion, suggest that as many as 16% of tumor recurrences occur at least 10 years following initial surgery, with average time to recurrence ranging anywhere from 6.1 to 11.8 years postoperatively. Despite the benefits of ultrasound-guided core biopsy over fine-needle aspiration biopsy, which include both improved consistency and diagnostic accuracy, current studies lack adequate patient numbers and follow-up duration to confirm comparable safety profile to currently accepted fine-needle aspiration cytology. In this report we: (1) compare the relative benefits of each procedure, (2) review evidence regarding tumor seeding in each procedure, (3) discuss time course and patient numbers necessary to detect tumor recurrence, and (4) describe how these uncertainties should be factored into clinical considerations.

Carcinoma ex-pleomorphic adenoma: A case report

Carcinoma ex pleomorphic adenoma (CXPA) is a rare and aggressive malignancy that usually develops in primary or recurrent pleomorphic adenoma. It occurs in 6% of all pleomorphic adenomas, of which only 7% of cases occur in minor salivary gland tumors. The rate of occurrence and malignant change increases with time if a case is left untreated. Intraorally, it is mostly seen in the palate, followed by the upper lip and buccal mucosa. CXPA of the hard palate typically presents as a painless, slow-growing swelling that frequently involves the periosteum and underlying palatal bone. Imaging of CXPA is usually noncontributory, leading to frequent misdiagnosis. Longstanding cases may extend up to the parapharyngeal space and cavernous sinus. We describe a rare case of CXPA of the palate with non-significant clinical and radiological findings. Early diagnosis for better prognosis is stressed.

Massive Transcranial Parotid Pleomorphic Adenoma: Recurrence after 30 Years

Pleomorphic adenoma, also known as benign mixed tumor, is the most common tumor affecting the parotid gland and can reach massive size; however, intracranial invasion is rare. Recurrence of pleomorphic adenoma after excision is a well-known phenomenon and can present decades after resection of the primary tumor. Here we present the case of a 53-year-old man who presented to our clinic with ear fullness, otalgia, and hearing loss 30 years after undergoing total parotidectomy and external beam radiotherapy for pleomorphic adenoma. Magnetic resonance imaging revealed a massive transcranial tumor invading the mastoid cavity, the dura of the posterior fossa, the fallopian and semicircular canals, the jugular foramen, the lateral infratemporal fossa skull base, the sigmoid and transverse sinuses, and the superior parapharyngeal region. Gross examination and histopathological studies confirmed that the mass was a recurrent pleomorphic adenoma. Here we discuss the features of recurrent pleomorphic adenoma and review the current literature.

Radiotherapy for pleomorphic adenoma

PurposeThe aim of this study was to update our experience in treating pleomorphic adenoma with radiotherapy (RT). Materials and MethodsThis is a retrospective analysis of 25 patients treated with RT alone (2 patients) or combined with surgery (23 patients), with follow-up ranging from 1.8 to 34.9 years (median, 10.5 years). ResultsLocal control was achieved in 13 (75%) of 16 patients with subclinical disease and 5 (56%) of 9 patients with gross disease. Overall local control was achieved in 18 (72%) of 25 patients. The 5-, 10-, and 15-year overall local control rates were 76 %, 76%, and 68%, respectively. Ten patients died of the following causes: recurrent disease, 2; malignant transformation, 2; and intercurrent disease, 6. At last follow-up, 14 patients were alive without evidence of disease, and 1 patient was alive with disease. Dental carries and transient facial nerve deficits were the most common complications. No patients developed severe complications subsequent to RT. ConclusionsIn patients at high risk for developing recurrent pleomorphic adenoma after surgery, RT is effective in controlling subclinical disease.

Recurrent Pleomorphic Adenoma of the Parotid Gland

Recurrent pleomorphic adenoma (RPA) of the parotid gland represents a challenging task for maxillofacial surgeons. The role of radiotherapy in the treatment of RPA of the parotid gland has been studied in previous experiences, and its use has been considered questionable. The aims of our article were to analyze and illustrate a case of RPA, initially treated with enucleations at another institution, showing a multinodular pattern with positivity for S-100 protein and cytokeratin, managed with conservative parotidectomy and neutron radiotherapy.

Złośliwe gruczolaki wielopostaciowe ślinianki przyusznej w materiale Kliniki Otolaryngologii Warszawskiego Uniwersytetu Medycznego

Pleomorphic adenoma (PA), or benign mixed tumor, is the most common salivary gland neoplasm. One of the factors influencing the management strategy in parotid PA is the risk of malignant transformation of this tumor. The objective of this study was the clinical and pathological analysis of malignant mixed tumors treated surgically at the Department of Otolaryngology, Medical University of Warsaw. Retrospectively analysed clinical material included 12 patients with malignant mixed tumors selected from the group of 423 patients who underwent surgical treatment for parotid pleomorphic adenoma at the Department of Otolaryngology, Medical University of Warsaw, between January 1988 and June 2010. There were 7 women and 5 men; age ranged from 39 to 67 years, with a mean of 56 years. The following parameters were analysed: demographic data, symptoms and disease duration, results of diagnostic tests, intraoperative data, results of histopathological examination, and adjunctive therapy. Four patients with a follow-up shorter than 5 years were excluded from the analysis of treatment results. A group of 12 patients with malignant mixed tumours included 10 (83.3%) cases of a primary tumor and 2 (16.7%) cases of a recurrent tumor. In the complete study sample (n = 423), patients with malignant mixed tumor comprised: 2.8% of all patients, 2.5% of patients with primary PA and 5.1% of patients with recurrent PA. Patients with malignant mixed tumor were significantly older than patients with benign PA (an average patient age was 56.0 and 43.2 years, respectively p < 0.001). 83.3% (n = 10) patients were older than 50 years. An average disease duration was longer in patients with malignant tumors. Considering all data from preoperative patient assessment (medical history, physical examination, FNAB, imaging studies), a preliminary diagnosis of malignant disease was made in 6 (50%) patients. All patients were treated surgically and 10 also had radiation therapy. The group of patients (n = 8), subjected to evaluation of treatment results, included three patients with non-invasive carcinoma and five patients with invasive carcinoma. All patients with non-invasive carcinoma were free from disease symptoms during the follow-up of 9 to 21 years after the treatment. Five-year survival was 80% (4 of 5 patients) in a group of patients with invasive carcinoma. The rate of death for the underlying disease was the same (80%). An overall 5-year survival in patients with malignant mixed tumor was 87.5%. 1. Malignant transformation of pleomorphic adenoma more frequently occurs in recurrent tumors and in older patients. 2. Uncharacteristic clinical picture of malignant mixed tumors allows to make an accurate initial diagnosis only in half of the patients. 3. Extracapsular cancer invasion is a vital prognostic factor. Overall 5-year survival for invasive and noninvasive carcinoma was 80% and 100%, respectively.

An Unusual Case of Carcinoma Ex Pleomorphic Adenoma of the Parotid Metastasizing to the Mandible: Case Report and Review of the Literature

Carcinoma ex pleomorphic adenoma (CXPA) is a rare neoplasm that represents carcinomatous transformation within a primary or recurrent pleomorphic adenoma.1,2 A review summarizing published cases of CXPA between 1953 and 1991 found that it accounts for approximately 3.6% of all salivary gland neoplasms and 11.6% of all malignant salivary gland neoplasms.1 CXPAs are most frequent in the sixth to eighth decades of life, with a slight female gender predominance. Although the major salivary glands are the most common index tumor site, CXPAs have been reported in the minor salivary glands,3 lacrimal glands,4 breast,5 trachea,6 and nasal cavity.

Low-grade malignant myoepithelioma arising in a pleomorphic adenoma: a rare case

Malignant myoepithelioma is a very rare salivary gland tumor that can arise de novo or within a pre-existing pleomorphic adenoma. We report a case of malignant myoepithelioma most probably arising in a pre-existing pleomorphic adenoma of the left parotid gland. The patient was a 60-year-old man who presented with a multinodular mass lesion over left side of the face and neck. He had undergone removal of a pleomorphic adenoma of the left parotid gland twice (8 and 22 years ago). Histological examination showed locally concentrated highly invasive myoepithelial cells with bland-looking morphology and no evidence of mitosis or necrosis. Immunohistochemistry confirmed the myoepithelial differentiation (S-100+, SMA+) and a low Ki-67 labeling index (<5%).