Disseminated Carcinoma Ex Pleomorphic Adenoma in an Adolescent Confirmed by Application of PLAG1 Immunohistochemistry and FISH for <Emphasis Type="Italic">PLAG1</Emphasis> Rearrangement

A 16-year-old previously asymptomatic boy presented with complaints of fatigue, weight loss, and back pain for several months. Imaging studies revealed a large superior mediastinal mass, numerous bilateral pulmonary nodules, and multiple lytic bone lesions. A needle biopsy from a sternal lesion showed a poorly differentiated carcinoma, immunoreactive for cytokeratins and EMA and immunonegative for various organ/tissue-specific markers. His past medical history was significant for excision of a parotid gland tumor 5 years earlier. Histologic review of the salivary gland tumor revealed a pleomorphic adenoma containing a microscopic focus of invasive carcinoma (carcinoma ex pleomorphic adenoma). By immunohistochemistry, both the salivary gland tumor and the disseminated carcinoma expressed PLAG1 with a strong nuclear pattern. Fluorescence in situ hybridization (FISH), using dual-color, break-apart probes for PLAG1, showed rearrangement of the gene in both the salivary gland and the disseminated tumors. FISH demonstrated additional cytogenetic aberrations in the carcinoma, including polysomy for chromosome 8 (in both the primary salivary gland and the metastatic tumors) and PLAG1 amplification (in the metastatic tumor). We conclude that in the proper clinicopathologic setting, application of PLAG1 immunohistochemistry and FISH for PLAG1 gene rearrangement may be valuable in establishing the diagnosis of carcinoma ex pleomorphic adenoma as the source of a cancer of unknown primary site.     

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.     

Case-report: metastasizing pleomorphic adenoma of the parotid gland

CASE-REPORT: Metastasizing pleomorphic adenoma of the parotid gland. We present a case of metastasizing pleomorphic adenoma (MPA). The patient died in 2002 at the age of 64 years, following on an initial diagnosis of a pleomorphic adenoma of the right parotid gland at the age of nineteen, multiple local recurrences and finally rib metastases. Histological examination of the excised tissue from the parotid gland and the metastases showed it to be a pleomorphic adenoma without any signs of malignancy, although the metastatic deposits consisted mostly of a myoepithelial component. Both in our case as in most of the literature, MPA follows a probable incomplete removal of the first tumour. We conclude that the first operation is of great importance for the prevention of late recurrences and metastatic spread.     

Synchronous benign and malignant salivary gland tumors in ipsilateral glands: a report of two cases and a review of literature

BACKGROUND: Ipsilateral salivary gland tumors of different histologic types are rare and make up less than 0.3% of all salivary gland neoplasms. Only nine cases of synchronous benign and malignant ipsilateral parotid gland tumors have been described in the literature. METHODS: Two additional cases of synchronous benign and malignant neoplasms in the parotid gland are reported and discussed with a review of literature. RESULTS: Our first case describes a pleomorphic adenoma and a salivary duct carcinoma, an entity not previously reported in the literature. The second case documents the most common benign and malignant ipsilateral parotid gland neoplasm reported in this case series, a Warthin's tumor and a mucoepidermoid carcinoma. CONCLUSIONS: Synchronous salivary gland tumors exhibiting both benign and malignant components are uncommonly observed, with only nine cases published to date. We describe two additional cases of a synchronous benign and malignant ipsilateral parotid gland 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.     

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.     

Parotid gland's tumors in children

The tumors of the salivary glands are infrequent in children, and parotid gland is involved in 80% of them. When a salivary gland tumor is present, the chance of malignancy is greater in the child than in the adult. We reviewed 8 cases identified in patients aged 14 years and younger in our hospital, analyzing its antecedents, signs and symptoms, histological features, diagnosis, treatment and evolution. All the patients displayed preauricular painless, non-inflammatory and slow-growing masses to an age between 10 months and 14 years. Four or them were pleomorphic adenomas, two haemangiomas, one epidermal cysts and one myoepithelial carcinoma. We emphasize the exceptional nature of the carcinoma for its rareness and for the high degree of malignancy expressed. We made a fine needle aspiration biopsy in four cases but they were conclusive only in three. All were treated by surgical resection of the tumour except for the myoepithelial carcinoma and the recurrent pleomorphic adenoma that were treated by total parotidectomy. The malignant tumours of the parotid gland are clinically indistinguishable of the benign ones, thus when any palpable mass appears in the zone of the parotid gland, an accurate diagnosis should be made without delay. The treatment of choice is the surgical excision with wide margins, being other adjuvant treatments less useful to this age than in the adult age.     

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.     

Carcinosarcoma (malignant mixed tumor) of the parotid: Report of a case with a pure rhabdomyosarcoma component

Background. Carcinosarcoma or true malignant mixed tumor of the parotid gland is extremely rare, accounting for <1% of all salivary gland malignancies. Methods. A 63-year-old woman presented with a 5-cm right parotid mass which was resected with a radical parotidectomy and infratemporal fossa dissection. Results. The tumor contained two distinctive histologic patterns, that of a poorly differentiated ductal carcinoma and a pleomorphic rhabdomyosarcoma. Additionally, a residual focus of pleomorphic adenoma was present peripherally, Immunohistochemical and electron microscopic studies confirmed the skeletal muscle differentiation. Conclusions. The pattern of combined rhabdomyosarcoma and ductal carcinoma has not been previously reported and adds further evidence to the myoepithelial derivation of these tumors. © 1994 John Wiley & Sons, Inc.     

Recurrent pleomorphic adenoma of the palate in a child.

A rare case of recurrent pleomorphic adenoma of the palate in a 9-year-old boy is presented. Pleomorphic adenoma is relatively rare in children compared with its incidence in adults. However, it is the most common benign epithelial tumour of the salivary glands. The majority of pleomorphic adenomata in children occur in the major salivary glands, mainly the parotid gland. Pleomorphic adenomata of the minor salivary glands are rare in children and mainly occur in the palatal glands. Of the few cases of pleomorphic adenoma of the palate reported in children, only one case showed recurrence of the tumour after primary excision. We present the second case of recurrent pleomorphic adenoma of the palate in a child.     

Primary Unilateral Multifocal Pleomorphic Adenoma of the Parotid Gland: Molecular Assessment and Literature Review

Multiple separate tumors developing in a single salivary gland is rare in previously untreated patients. Tumors that can be multicentric include Warthin tumor, oncocytoma, basal cell adenoma and acinic cell carcinoma. The incidence of multiple primary unilateral pleomorphic adenomas is extremely rare in patients with no prior history of trauma or surgery. We report two cases of primary multicentric pleomorphic adenoma and review the literature. We also subjected one of our cases to a molecular clonality test. The molecular results suggested that the separate nodules were clonally related, arguing against an independent origin.     

Parotid gland metastasis from renal carcinoma

The salivary gland most frequently involved with secondary cancer is the parotid gland. Metastases are responsible for 21-42% of malignant parotid tumors. Malignant melanoma and squamous cell cancer are the two most common tumors to metastasize to the parotid gland. Metastatic adenocarcinoma to this site has rarely been reported. Secondary renal cell carcinoma has been reported in many structures, including the brain, sinonasal tract, lungs, abdomen, genitourinary tract, bone, soft tissue, and lymphatics. Histologically proven parotid metastasis has been previously reported in only one patient. Two patients have recently been diagnosed and treated at our institution for this rare disease. The clinical presentation of each patient was quite different. One patient presented with parotid and pulmonary metastases seven years after resection of a renal tumor. Another patient had resection of a parotid mass revealing an occult metastasis from a renal cell carcinoma. Further evaluation revealed a locally extensive asymptomatic hypernephroma. The survival from the time of discovery of the parotid metastasis was 46 months for the former patient, while the latter patient is alive after 20 months. Differentiation of these tumors from vascular disorders (aneurysm or arteriovenous fistula) required selective angiography and computed tomography. Surgical excision via superficial parotidectomy with facial nerve preservation is necessary for palliation, particularly to avoid massive hemorrhage which may occur upon tumor extension into the oropharynx.     

Pleomorphic adenoma with extensive lipometaplasia: report of three cases

We report a series of three cases of pleomorphic adenoma with extensive lipometaplasia, a recently described subtype of pleomorphic adenoma of salivary gland origin. Two patients were female and one male, ranging in age from 30 to 45 years. Two occurred in the minor salivary glands of the lip and palate, respectively, and one in the parotid. Typical histologic findings are presented. In addition, one case consists of a proliferation of spindle cells with an interesting combination of mature adipose tissue, hyaline cartilage, and bone in the absence of ductal structures. The differential diagnosis, as it pertains to other fat-containing tumors (such as lipoadenoma, spindle cell lipoma, interstitial lipomatosis, and benign mesenchymoma), is discussed. It is likely that the ability of myoepithelial cells to undergo various metaplasias is the cause of the unusual histologic appearances of this tumor.     

Familial bilateral acinic cell carcinoma of the parotid synchronous with pituitary adenoma: case report

BACKGROUND: Acinic cell carcinoma is a common neoplasm of the salivary glands that occurs predominately in the parotid. Only one case of a familial recurrence of such a neoplasm and 16 cases of bilateral tumors have been reported. METHODS: History files and histologic reports of a patient with bilateral multifocal acinic cell carcinoma of the parotid and a synchronous pituitary adenoma, and of the patient's sister and his father, also treated for parotid tumours, were retrieved. RESULTS: There was one recurrence of acinic cell carcinoma in the family. A pituitary tumor was a chromophobe gonotrophic adenoma. CONCLUSIONS: This is the 17th case of bilateral acinic cell carcinoma of the parotid gland and the second reported case with a familial recurrence. It is the first with a synchronous pituitary adenoma.     

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).     

Salivary tumors in north Jordanians: a descriptive study.

OBJECTIVE: To evaluate the types and distribution of tumors of salivary glands in north Jordanians. STUDY DESIGN: The records of the Department of Pathology at Jordan University of Science and Technology were reviewed for patients who were treated for salivary gland tumors from 1991 to 2002. The tumors were analyzed for age of patient, sex of patient, tumor site, and tumor type. RESULTS: One hundred two true neoplasms (70% benign and 30% malignant) were found. The most frequent benign and malignant neoplasms found were pleomorphic adenoma (54%) and adenoid cystic carcinoma (13%), respectively. The most common major and minor salivary gland sites were the parotid (51%) and palatal glands (20%), respectively. Although most of major gland tumors were adenomas, carcinomas of the minor glands were only slightly less frequent than adenomas. The most frequent malignant parotid tumors were adenoid cystic carcinoma and mucoepidermoid carcinoma. The most frequent minor salivary gland malignant tumors were palatal adenoid cystic carcinoma. Age ranged from 1 to 94 (mean 40) years, with a male to female ratio of 1:1.2. CONCLUSION: North Jordanians with salivary gland tumors were found to have similar characteristics with patients of other countries with regard to tumor type, tumor site distribution, and age and sex of patients.     

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.     

Schwannoma-Like Pleomorphic Adenoma: A Case Report with Review of the Literature

Pleomorphic adenoma is a common benign salivary gland tumor, which represents about 66 % of benign neoplasms of the salivary glands. Although it can occur in any salivary gland, it is most frequently found in the parotid. Pleomorphic adenomas are renowned for their cytomorphological and architectural heterogeneity that are characterized by intermixed epithelial and mesenchymal-like components. We report a rare case of pleomorphic adenoma of the parotid gland with prevalent schwannoma-like features mimicking a benign schwannoma. Microscopically the tumor showed a prevalence (about 95 %) of schwannoma-like areas with focal (about 5 %) epithelial component with tubular organization. The tumor showed positive immunoexpression for cytokeratin, S-100 protein, and focal expression of p63, CD10 and smooth muscle actin. To the best of our knowledge only six cases of schwannoma-like pleomorphic adenoma have been reported in the literature. The differential diagnosis between this entity and neurogenic and myogenic tumors is discussed.     

Recurrent pleomorphic adenomas of the parotid gland

Twenty-seven patients with recurrent pleomorphic adenomas of the parotid gland were seen. This study confirms that superficial parotidectomy with identification and dissection of the facial nerve greatly decreases the incidence of recurrent benign pleomorphic adenoma, and, in the small number that recur, are easier to cure. All lumps in the parotid area should be approached in the same manner, with the surgeon prepared to isolate the facial nerve and perform superficial parotidectomy. This technique is successful because it avoids dissection near the tumor capsule, which is the major cause of recurrence. The procedure recommended for treatment of patients to remove the first recurrent tumor is total parotidectomy and excision of the previous scar. This vigorous approach is dictated by the increasing difficulty of removing further recurrent tumors. Only 67% of patients with recurrent tumors ultimately achieve a tumor-free status. Experienced surgeons can preserve the facial nerve even after multiple operations.     

Stereotactic radiosurgery for recurrent pleomorphic adenoma invading the skull base--case report--

A 38-year-old man presented with a recurrent pleomorphic adenoma in the parapharyngeal space invading the skull base 19 years after the first operation for a parotid gland tumor. Stereotactic radiotherapy was performed to control the tumor growth using a marginal dose of 8 Gy and maximum dose of 18 Gy with care taken to minimize the dose to nearby structures. The symptoms were reduced within a few months. Magnetic resonance imaging over 5 years showed that the tumor was controlled with no regrowth. Stereotactic radiotherapy is a therapeutic option for the treatment of pleomorphic adenomas.