Pleomorphic Adenoma in a Young Female: Case Report / Adenoma Pleomórfico en una Mujer Joven: Reporte de Caso

ABSTRACT: Pleomorphic adenoma (PA) is the most common benign salivary gland neoplasm and it is frequently diagnosed in the third and fourth decade with predilection for women. PA is the most common benign lesion of minor salivary glands in children and adolescents, being the palate one of the most frequently affected site of minor salivary glands. Herein, we present a case report of a PA of the hard palate diagnosed in a 15-year-old female and a review of the Englishliterature of the reported cases of PA in children and adolescents in the hard palate.

RESUMEN: El adenoma pleomórfico (AP) es la neoplasia benigna más común de las glándulas salivales y se diagnostica frecuentemente en la tercera y cuarta década con predilección por las mujeres. El AP es la lesión benigna más común de las glándulas salivales menores en niños y adolescentes, siendo el paladar uno de los sitios más frecuentemente afectados de las glándulas salivales menores. En este trabajo se presenta un relato de caso de un AP de paladar duro diagnosticado en una mujer de 15 años de edad y una revisión de la literatura en inglés de los casos reportados de AP en niños y adolescentes en paladar duro.

Warthin tumor in an unusual site: a case report.

Warthin tumor (papillary cystadenoma lymphomatosum, adenolymphoma) is a benign neoplasm from salivary glands and is almost restricted to the parotid gland and the periparotid lymph nodes. Its etiopathogenesis has been associated with tobacco smoke. Several authors believe that the tumor develops from heterotopic salivary gland tissues or through the attraction of a heavy lymphoid reaction. Most cases involve the lower pole or the tail of the parotid. Since its description, a few cases have been reported in other sites, like the submandibular salivary gland. This study aimed to describe a rare presentation of a Warthin tumor and discuss its etiology and differential diagnosis. A 72-year-old male patient presented an asymptomatic, nodular mass adjacent to the submandibular salivary gland. Based on clinical diagnosis of cervical lymphadenopathy, the patient underwent a surgical excision of the lesion. Histopathological exam revealed a benign neoplasm from salivary glands composed of two components: epithelial and lymphoid. The findings were consistent with Warthin tumor. Warthin tumor from submandibular salivary gland is a rare lesion. Professionals should not misinterpret their site of origin, as if they were from the lower pole or the tail of the parotid.

Tumours of the submandibular salivary gland: a clinicopathologic review of cases over a 17-year period / Tumores de la glándula salival submandibular: una revisión clínico-patológica de casos por un período de 17 Años

OBJECTIVE: This study presents a clinicopathologic review of cases of submandibular salivary gland tumours at the Lagos University Teaching Hospital, Nigeria, over a period of 17 years. METHODS: The records of all patients with histological diagnosis of submandibular salivary gland tumours at the Lagos University Teaching Hospital over a period of 17 years (January 1990 to December 2006) were retrospectively reviewed. Parameters studied were; age and gender of patients, symptoms and duration ofsymptoms, and histological diagnosis. RESULTS: A total of 36patients with submandibular gland tumours were seen during the period. Maleto-female ratio was 1.8:1 (male = 23, female = 13). Mean age (± SD) at presentation was 43 (± 19) years (age range, 17-84 years). There were 19 malignant tumours and 17 benign ones. Pleomorphic adenoma (36.1%) was the most frequent tumours, followed by adenoid cystic carcinoma (11.1%), anaplastic carcinoma (11.1%) and malignant lymphoma (11.1%). Patients with histological diagnosis of malignant tumours were significantly older than those with benign tumours (p = 0.01). Most patients (80.6%) presented with painless swelling. CONCLUSION: Malignant submandibular salivary gland tumours were slightly more than the benign ones in the studied population. Painful swelling or ulceration is indicative ofa malignant submandibular gland tumour.

OBJETIVO: Este estudio presenta una revisión clinicopatológica de casos de tumores de la glándula salival submandibular en el Hospital Docente de la Universidad de Lagos, Nigeria, por un periodo de 17 años. MÉTODOS: Se revisaron retrospectivamente las historias clínicas de todos los pacientes con el diagnóstico histológico de los tumores de la glándula salival submandibular en el Hospital Docente de la Universidad de Lagos, correspondientes a un periodo de 17 años (de enero de 1990 a diciembre de 2006). Los parámetros estudiados incluyeron: la edad y el género de los pacientes, síntomas y duración de síntomas, y diagnóstico histológico. RESULTADOS: Un total de 36 pacientes con tumores de la glándula de submandibular fueron atendidos durante ese periodo. La proporción varón:hembra fue 1.8:1 (varones = 23, hembras = 13). La edad promedio (± SD) al momento de su presentación fue 43 (± 19) años (rango de edad, 17-84 años). Hubo 19 tumores malignos y 17 benignos. El adenoma pleomórfico (36.1%) fue el tipo de tumor más frecuente, seguido por el carcinoma cístico adenoideo (11.1%), el carcinoma anaplástico (11.1%) y el linfoma maligno (11.1%). Los pacientes con diagnóstico histológico de tumores malignos eran significativamente de más edad que los que tenían tumores benignos (p = 0.01). La mayoría de los pacientes (80.6%) presentaban hinchazón sin dolor. CONCLUSIÓN: Los tumores malignos de la glándula salival submandibular fueron ligeramente mayor en número que los benignos en la población estudiada. Hinchazón dolorosa o la ulceración dolorosas son indicios de un tumor maligno de la glándula submandibular.

Tumours of the submandibular salivary gland: a clinicopathologic review of cases over a 17-year period.

OBJECTIVE: This study presents a clinicopathologic review of cases of submandibular salivary gland tumours at the Lagos University Teaching Hospital, Nigeria, over a period of 17 years. METHODS: The records of all patients with histological diagnosis of submandibular salivary gland tumours at the Lagos University Teaching Hospital over a period of 17 years (January 1990 to December 2006) were retrospectively reviewed. Parameters studied were; age and gender of patients, symptoms and duration of symptoms, and histological diagnosis. RESULTS: A total of 36 patients with submandibular gland tumours were seen during the period. Male-to-female ratio was 1.8:1 (male=23, female=13). Mean age (+/- SD) at presentation was 43 (+/- 19) years (age range, 17-84 years). There were 19 malignant tumours and 17 benign ones. Pleomorphic adenoma (36.1%) was the most frequent tumours, followed by adenoid cystic carcinoma (11.1%), anaplastic carcinoma (11.1%) and malignant lymphoma (11.1%). Patients with histological diagnosis of malignant tumours were significantly older than those with benign tumours (p = 0.01). Most patients (80.6%) presented with painless swelling. CONCLUSION: Malignant submandibular salivary gland tumours were slightly more than the benign ones in the studied population. Painful swelling or ulceration is indicative of a malignant submandibular gland tumour

Myoepithelial carcinoma of the submandibular gland: report of a case with multiple cutaneous metastases.

Myoepithelial carcinoma is a rare and aggressive neoplasm of the salivary glands. One-third of the patients may develop regional distant metastases, and lungs and kidneys have been regarded as the most usual sites for implantation. There is, however, little information on the metastatic behavior of this malignancy. We report the first case of patient with multiple cutaneous metastases from a myoepithelial carcinoma of the submandibular gland, which depicted a very aggressive clinical course.

Sialoadenite esclerosante crônica (tumor de Kuttner): relato de caso clínico / Chronic sclerosing sialadenitis (Kuttner tumour): clinical case report

A sialoadenite esclerosante (tumor de Kuttner) representa um processo inflamatório crônico incomum de glândula salivar, que acomete com maior freqüência a glândula submandibular. O objetivo deste trabalho é relatar o caso clínico de um paciente que apresentou sialoadenite esclerosante crônica associada a quadro de sialolitíase. Essa entidade patológica é pouco freqüente, clinicamente pode ser confundida com uma neoplasia e, embora seja resolvida com a exérese da lesão, causa transtornos importantes ao paciente, como dor intensa, disfonia e disfagia.

Chronic sclerosing sialadenitis (Kuttner tumour) is an unusual chronic inflammatory process of the salivary gland that affects principally the submandibular gland. The aim of this work is to report a case of chronic sclerosing sialadenits associated with sialolithiasis. Kuttner tumour is an uncommon entity that clinically may not be distinguished from neoplasia and though it can be resolved by surgical excision of the lesion, it causes important upset for the patient, as intense pain, dysphonia and dysphagia.

Biomarker analysis in carcinoma ex pleomorphic adenoma at an early phase of carcinomatous transformation.

Diagnostic criteria for intracapsular carcinoma ex pleomorphic adenoma (CXPA) are subjective and vary among authors. Biomarker analysis, which could provide more objective evaluation of these tumors, has rarely been studied in intracapsular CXPA. Immunohistochemical evaluation of c-erbB-2, p53 protein, bcl-2, and Ki-67 was performed in 8 cases of CXPA at an early phase of malignant transformation (4 intracapsular and 4 minimally invasive) and in 17 pleomorphic adenomas (PA). In all cases of CXPA, p53 and Ki-67 were demonstrated predominantly in luminal cells of benign and malignant areas, significantly more in the latter. Few benign myoepithelial cells were p53 positive. c-erbB-2 reactivity was strongly associated with atypical luminal cells. Bcl-2 expression was weak and focal in malignant areas from 2 cases. In conclusion, both p53 and c-erbB-2 proteins appear to be involved at an early stage of malignization of PA. In PA with atypical cells, evaluation of the expression of these 2 markers provides more objective criteria for the diagnosis of intracapsular CXPA.

Macroglossia secondary to systemic amyloidosis: case report and literature review.

Amyloidosis is characterized by an abnormal extracellular deposition of amyloid in different tissues and organs, where it usually causes some type of dysfunction. Its cause is unknown. The two main forms of amyloidosis are systemic and localized; the latter is rare. No satisfactory treatment for systemic amyloidosis has been discovered, and mean survival is poor, ranging from 5 to 15 months depending on the presence or absence of multiple myeloma. We report a case of primary systemic amyloidosis in a 71-year-old man. The diagnosis of amyloidosis was established by tongue biopsy, and its systemic nature was identified by analysis of aspirated abdominal fat. At the 1-year follow-up, the patient's clinical condition had not changed, and he was thereafter lost to follow-up.

Pleomorphic adenoma of the submandibular gland: clinicopathological and immunohistochemical features of 60 cases in Brazil.

The submandibular gland is involved in only 5% to 10% of the salivary gland tumors, and pleomorphic adenoma (PA) is the most common tumor affecting it. This study describes the clinicopathological features and immunohistochemical expression of Ki-67 and p53 in 60 cases of submandibular salivary gland PAs. Most of the patients were in the third and fifth decades of life and 37 (62%) of them were women. Tumor sizes varied from 1 to 10 cm and the mean time between symptom onset and treatment was 52 months. Only 1 patient experienced local recurrence, 3 years after treatment. Histologically, most tumors consisted chiefly in a chondromyxoid stroma. Stroma-rich PAs were larger than stroma-poor ones (P<.02). All PAs were found negative for Ki-67 and p53. These results show that PAs of the submandibular gland are histologically similar to PAs of other salivary glands, and that they have a low proliferative rate and a good prognosis.

[Chronic sclerosing non-specific sialadenitis of the submandibular gland (Küttner tumor). Report of one case]. / Sialadenitis crónica esclerosante inespecífica de la glándula submandibular (tumor de Küttner). Caso clínico.

Chronic sclerosing unspecific sialadenitis or Küttner tumor, is an infrequent inflammatory lesion of submandibular gland. We report a 60 years old male presenting with a slowly growing, painless, bilateral submandibular tumor of two years of evolution. Pathological examinations showed marked atrophy of glandular parenchyma with increased fibrous connective tissue and an intense lymphocytic infiltration with lymphoid follicle formation. Lymphocyte population study with kappa, lambda, CD20 and CD45RO antibodies was similar to that observed in reactive lymph nodes. There was no over expression of Bcl-2 gene protein, involved in the phenomenon of apoptosis of glandular tissue, that could explain the pathogenesis of atrophy. This protein was positive only in lymphoid cells and glandular conducts. An immune etiology, with replacement of glandular tissue by lymphoid and fibrous connective tissue is suggested.

Sialadenitis crónica esclerosante inespecífica de la glándula submandibular, tumor de Kuttner: caso clínico / Chronic sclerosing unspecific sialadenitis of submandibular gland, Kuttner tumor: report of case

Chronic sclerosing unspecific sialadenitis or KYttner tumor, is an infrequent inflammatory lesion of submandibular gland. We report a 60 years old male presenting with a slowly growing, painless, bilateral submandibular tumor of two years of evolution. Pathological examinations showed marked atrophy of glandular parenchyma with increased fibrous connective tissue and an intense lymphocytic infiltration with lymphoid follicle formation. Lymphocyte population study with kappa, lambda, CD20 and CD45RO antibodies was similar to that observed in reactive lymph nodes. There was no over expression of Bcl-2 gene protein, involved in the phenomenon of apoptosis of glandular tissue, that could explain the pathogenesis of atrophy. This protein was positive only in lymphoid cells and glandular conducts. An immune etiology, with replacement of glandular tissue by lymphoid and fibrous connective tissue is suggested

Paraganglioma de glomus carotídeo: un caso clínico de presentación inusual / Paraganglioma of carotid glomus: a clinical case of unusual presentation

Se presenta un caso clínico de un paraganglioma benigno, no cromafínico, del glomus carotídeo, en un hombre de 51 años que debutó con dolor torácico y al que posteriormente tras una punción aspiración con aguja fija (PAAF), el estudio citológico fue informado como compatible con oncocitoma de la glándula submaxilar

Paraganglioma de glomus carotídeo: un caso clínico de presentación inusual / Paraganglioma of carotid glomus: a clinical case of unusual presentation

Se presenta un caso clínico de un paraganglioma benigno, no cromafínico, del glomus carotídeo, en un hombre de 51 años que debutó con dolor torácico y al que posteriormente tras una punción aspiración con aguja fija (PAAF), el estudio citológico fue informado como compatible con oncocitoma de la glándula submaxilar (AU)

[Nucleolus organizer regions (NORs) in metastasizing pleomorphic adenoma]. / Regiones de organización nucleolar (NORs) en adenoma pleomórfico metastasiante.

The metastasizing pleomorphic adenoma of the salivary glands is a rare variant of pleomorphic adenoma with a benign microscopical appearance, but malignant biological behaviour and production of metastasis. The histopathological study with the routine techniques is not enough for disclosing the nature of these tumors and they are most of the times underdiagnosed as benign. The correct diagnosis, almost always too late, is evident after several recurrences and the detection of metastatic foci. The number of nucleolar organizing regions (NORs) detected with the silver colloid method can be used in histopathology to determine the degree of cell activity. We report the findings in a case of metastasizing pleomorphic adenoma of the submandibular gland. This technique demonstrated to be useful for the diagnosis and characterization of the metabolism of these tumors.

Carcinoma oncocítico / Oncocitic carcinoma

Apresentaçäo de um caso de Carcinoma Oncocítrico, que se faz ainda mais raro por sua provável origem (glândula submandibullar) e a idade do paciente (28 anos), na época