Carcinosarcoma ex Pleomorphic Adenoma of the Submandibular Gland in a 64-Year-Old Man: A Case Report.

Carcinosarcoma (CS) is a rare tumor, consisting of both carcinomatous and sarcomatous components. In this paper, we present a case of CS arising from a pleomorphic adenoma (PA) of the submandibular gland. A 64-year-old Japanese man presented with a left submandibular mass that had developed for 20 years with complaints of pain for the last 3 months. Magnetic resonance imaging showed a lesion involving the left submandibular gland. The patient underwent total dissection of the left submandibular gland and left cervical lymph nodes. Upon gross examination, the mass appeared completely covered by fibroadipose tissue measuring 46×42×45 mm; sectioning revealed a solid-white nodule with central bleeding and necrosis, invading into the surrounding adipose tissue. Microscopically, the presence of carcinomatous and sarcomatous components in the fibro-myxomatous stroma was detected, suggestive of pre-existing PA. The carcinoma component was diagnostic of salivary adenocarcinoma, not otherwise specified, whereas the sarcomatous component exhibited features of osteosarcoma characterized by formation of osteoid. As the border between the carcinomatous and sarcomatous components was not evident, CS may have occurred via transformation of the carcinoma into sarcoma. Tumor metastasis was detected in the cervical lymph nodes. Immunohistochemically, AE1/AE3 expression was noted in the carcinomatous component, but not in the osteosarcoma component. Both components were diffusely positive for vimentin. Four months after the operation, the patient developed a metastatic CS lesion in the lung, suggesting tumor aggression.

Siringoma condroide en el labio superior: reporte de un caso / Chondroid syringoma of the upper lip: report of a case

Paciente masculino de 81 años de edad que acudió a consulta con un cirujano dentista por presentar una lesión nodular, asintomática, firme y móvil en el labio superior del lado derecho. El sujeto refi rió tener la lesión desde la infancia, pero notó crecimiento lento en el último año. El diagnóstico clínico indicaba un adenoma pleomorfo, por lo que el individuo se sometió a biopsia escisional bajo anestesia local. El espécimen se envió a estudio histopatológico. La evolución del paciente fue satisfactoria, sin reincidencia de la lesión. El examen microscópico reveló la presencia de una neoplasia bien delimitada constituida pormúltiples estructuras ductales pequeñas en la dermis. Estos ductos se encontraban revestidos por dos hileras de células epiteliales planas y contenían cantidades variables de material amorfo en la luz. El estroma presentaba diferenciación condroide.

An 81-year-old male patient consulted a dental surgeon due to his presenting a fi rm, mobile, asymptomatic nodular lesion on the upper lip. The patient stated that he had had the mass since childhood but that it had grown slowly over the course of the previous year. The clinical diagnosis was pleomorphic adenoma, for which reason the patient underwent an excisional biopsy under local anesthesia. The specimen was sent for histopathological examination. The patient’s progress was satisfactory and there was no recurrence of the lesion. Micro-scopic examination revealed the presence of a well-defi ned neoplasm consisting of multiple small ductal structures located in the dermis. These ducts were lined with two rows of fl attened epithelial cells and contained varying amounts of amorphous material in the lumen. The stroma exhibited chondroid differentiation.

Salivary duct carcinoma with rhabdoid features: report of 2 cases with immunohistochemical and ultrastructural analyses.

BACKGROUND: Salivary duct carcinoma with rhabdoid features is extremely rare. METHODS: We report 2 cases of salivary duct carcinoma with rhabdoid features treated at our institution. RESULTS: Case 1 was a 44-year-old Japanese man who had swelling in the left parotid region. This tumor consisted of residual pleomorphic adenoma and widely invasive carcinoma, which showed a diffuse growth pattern by atypical rhabdoid cells. Case 2 was a 66-year-old Japanese man who had swelling of the right cervical region. This submandibular tumor was also composed of both residual pleomorphic adenoma region and invasive adenocarcinoma components, whereas some metastatic lesions were purely composed of rhabdoid cells. Such cells were strongly and diffusely positive for cytokeratins (CKs), gross cystic disease fluid protein-15 (GCDFP), and androgen receptor (AR). Case 1 was also positive for Her-2 and p53. CONCLUSION: Both patients were diagnosed with carcinoma ex pleomorphic adenoma and their carcinomatous components were composed of salivary duct carcinoma with rhabdoid features, which is a highly aggressive tumor, similar to salivary duct carcinoma.

Identificación de colágeno I y III con picrosirius red/ polarización en el estroma de tumores salivales / Identification of type I and III collagen by picrosirius red/polarization of tumoral salivary stroma

El estroma juega un rol importante en los procesos tumorales de invasión y metástasis. Las fibras de colágeno tipo I son el principal componente estructural del estroma en distintos tumores. Sin embargo, hay muy pocos estudios en los tumores de glándulas salivales. Basándonos en estos antecedentes el objetivo de la presente comunicación fue estudiar las características del colágeno con Picrosirius red/polarización en tumores benignos y malignos de glándulas salivales para evaluar su posible rol en los mecanismos de progresión tumoral. Cortes histológicos de adenoma pleomórfico, carcinoma adenoide quístico y carcinoma epitelial mioepitelial se colorearon con H/E y Picrosirius red y se examinaron con microscopio de polarización. La birrefringencia del colágeno con Picrosirius/polarización resultó diferente en el estroma de los tumores malignos (carcinoma adenoide quístico y carcinoma epitelial mioepitelial), con predominio de colágeno I, en comparación con el tumor benigno (adenoma pleomórfico), con predominio de colágeno III. El diferente perfil de coloración en las fibras colágenas producidas en el estroma de los tumores analizados podría relacionarse con diferentes mecanismos de expansión tumoral, los que fueron poco estudiados en los tumores de glándulas salivales. Más estudios son necesarios para obtener resultados más concluyentes que contribuyan al diagnóstico, pronóstico y tratamiento.

The stroma plays an important rol in tumoral invasion and metastasis. Type I collagen is the main structural component of the stroma in several tumors. However, there are few studies on salivary gland tumors. Based on this background the objective of the present communication was to study collagen characteristics with picrosirius red/polarization on malignant and benign tumors of salivary glands to evaluate its posible rol in the tumoral progression mechanism. Histological sections of pleomorphic adenoma, adenoid cystic carcinoma and epithelial/myoepithelial carcinoma were stained with H/E and picrosirius red and were studied with polarization microscope. Collagen birefringence with Picrosirius/polarization was different in the malignant tumor stroma (adenoid cystic carcinoma and epithelialmyoepithelial carcinoma), with predominance of type I collagen, compared with a benign tumor (pleomorphic adenoma), with predominance of type III collagen. The different staining profile in collagen fibers produced in the benign and malignant stroma tumors analized could be related with different tumoral expansion mechanism, which were scarce studied on the salivary glands tumors. More studies are needed to obtain more conclusive results to contribute to diagnosis, prognosis and treatment.

Ultrastructural immunolocalization of a cartilage-specific proteoglycan, aggrecan, in salivary pleomorphic adenomas.

A pleomorphic adenoma (PA) is the most common epithelial tumor in the salivary glands, but it frequently shows a mesenchyme-like histology, including the presence of myxoid and chondroid areas. Cartilage-specific matrix proteins are deposited in PA. Aggrecan is a major component of cartilage-specific proteoglycans. The present study examined the ultrastructure of the stromal areas in ten salivary PA specimens and investigated the distribution of aggrecan by immunoelectron microscopy. Aggrecan was deposited in the myxoid and chondroid stroma of PA. Ultrastructural observations revealed many proteoglycan cores and fibrils in the myxoid stroma and some spindle-shaped neoplastic myoepithelial cells with vacuoles and actin filaments in the myxoid areas. By immunoelectron microscopy, positivity for aggrecan was observed in the vacuoles of neoplastic myoepithelial cells, which coexisted with the viscous materials, and it was also frequently seen in electron-dense crystals in the myxoid stroma. These findings suggest that neoplastic myoepithelial cells produce aggrecan and release it from vacuoles, and aggrecan is then deposited in the myxoid stroma. Aggrecan deposition is therefore considered to play an important role in the formation of the mesenchyme-like stroma, especially the myxoid stroma.

[Some particular aspects in cases of pleomorphic adenoma of the parotid gland]. / Unele aspecte particulare intâlnite in cazuri de adenom pleomorf al parotidei.

Twenty three cases of parotid gland pleomorphic adenoma from Surgical Oro-Maxillo Facial Clinic of "Sf. Spiridon" Hospital, Iasi, have been evaluated. In order to reveal the particular microscopic aspects, several histological techniques have been used: HE, Van Gieson, PAS, Alcian Blue and Gordon Sweet. The microscopic evidences points out the variety of microscopic forms even within the same case, and also the prevalence of some particular forms such as myo-epithelial cell predominance, squamous metaplasia and cystic degeneration. Our observations, corroborated with the one from other research, support the hypothesis of an unique ectodermal cell origin for the lesion histogenesis.

The ultrastructural aspects of neoplastic myoepithelial cell in pleomorphic adenomas of salivary glands.

The purpose of this study has been to establish the major ultrastructural aspects of the myoepithelial cell and the myoepithelial-like cells proliferated in the pleomorphic adenomas of salivary glands. Thus, twelve benign pleomorphic adenomas of salivary glands have been studied by electron-microscopy transmission techniques. Our analysis has proved the proliferation of two major cellular populations, one of ductal type and one of myoepithelial type, which tried to reproduce the tubulo-acinar cytoarchitecture from the normal salivary glands. We have also noticed the key role of the so-called 'modified' myoepithelial cells from the periphery of the proliferating epithelial units in the genesis of the myxoid and chondromyxoid tumoral stromal areas. All these ultrastructural aspects have explained the great histological diversity of these salivary gland neoplasms as well as the key role of the myoepithelial cell in its histogenesis.

The frequency and distribution of mast cells in pleomorphic adenomas of salivary glands.

AIM: To investigate whether the frequency and distribution of mast cells (MCs) in pleomorphic adenomas (PAs) of major and minor salivary glands justifies the suggestion that there exists an association between MCs and mucoid stromal changes in PAs. METHODS: The material consisted of 22 cases of pleomorphic adenoma (eight arising in major and 14 in minor salivary glands) and a control group represented by five cases of monomorphic adenoma (MA). Representative 3-microm thick, paraffin-embedded sections were stained with H&E and Azur A. Computer-aided image analysis was performed in order to evaluate the relative surface area occupied by epithelial and connective tissue components, as well as the absolute number of MCs. RESULTS: According to our findings, PAs from minor salivary glands contain significantly greater numbers of mast cells compared with tumours from major glands. Additionally, the distribution of MCs within the stromal connective tissue appeared not to be random. CONCLUSION: It is possible that differences in the pattern of connective tissue might influence the actual concentration of MCs and that these differences are responsible for the observed variations between major and minor gland PAs.

Oncocytic tumor in myoepithelioma arising from the grossopalatine gland.

Oncocytoma or oncocytic change in salivary glands normally occurs in old patients and mostly in the parotid gland, but those arising from the grossopalatine gland in young patients are extremely rare. The present case shows that oncocytic ductal structures were observed in myoepithelioma, consisting of spindle, plasmacytoid or epithelioid cells. The oncocytic tumor contained large amounts of eosinophilic granular cytoplasm and small nuclei.

Adenoma pleomorfo: caso clínico y revisión de la literatura / Pleomorphic adenoma: clinical case and review of the literature

Se presenta el caso de un paciente de sexo masculino de 48 años de edad, portador de una turmoración intrabucal de gran tamaño (10x7x5 cm), de consistencia firme, que no permitía al enfermo cumplir las funciones mínimas de deglución y fonación, además dificultaba seriamente la respiración normal del enfermo. Esta lesión tenía 20 años de evolución. El paciente fue examinado y diagnosticado en el hospital Félix Bulnes Cerda e intervenido en conjunto con el equipo del Hospital Traumatológico, ambos en Santiago de Chile. La lesión fue enucleada de acuerdo al diagnóstico clínico e histopatológico

Pleomorphic adenocarcinoma of the ciliary epithelium: a clinicopathological, immunohistochemical, ultrastructural, DNA-ploidy and comparative genomic hybridization analysis of an unusual case.

PURPOSE: To describe detailed phenotypic and genotypic analysis of a pleomorphic adenocarcinoma of the ciliary epithelium (CE). CASE REPORT: An 86-year-old white woman developed an enlarging mass protruding from her previously eviscerated left eye 2 months postoperatively. Based on light and ultrastructural microscopy, the final diagnosis was a pleomorphic adenocarcinoma of the ciliary epithelium (CE). DISCUSSION: Cell proliferation indices confirmed the unusually rapid growth rate of this tumor; the peridiploid DNA content might explain the relatively low incidence of distant metastases. An imbalance of the chromosome 6 was also found by Comparative Genomic Hybridization (CGH).

Centrosomal abnormalities, multipolar mitoses, and chromosomal instability in head and neck tumours with dysfunctional telomeres.

Carcinomas of the head and neck typically exhibit complex chromosome aberrations but the underlying mutational mechanisms remain obscure. Evaluation of cell division dynamics in low-passage cell lines from three benign and five malignant head and neck tumours revealed a strong positive correlation between multipolarity of the mitotic spindle and the formation of bridges at anaphase in both benign and malignant tumours. Cells exhibiting a high rate of mitotic abnormalities also showed several chromosome termini lacking TTAGGG repeats and a high frequency of dicentric chromosomes. Multicolour karyotyping demonstrated a preferential involvement in structural rearrangements of chromosomes with deficient telomeres. The majority of malignant, mitotically unstable tumours expressed the reverse transcriptase subunit of telomerase. These data indicate that some of the genomic instability in head and neck tumours is initiated by telomere dysfunction, leading to the formation of dicentric chromosomes. These form chromosome bridges at mitosis that could prevent the normal anaphase-telophase transition. In turn, this may cause an accumulation of centrosomes and mitotic multipolarity. Telomerase expression does not confer total stability to the tumour genome but could be crucial for moderating the rate of chromosomal evolution.

Lipomatous mixed tumour of the skin: a histological, immunohistochemical and ultrastructural study.

BACKGROUND: Mixed tumours are composed of an admixture of an epithelial/myoepithelial and usually a myxochondroid stromal component. Adipocytes are found more rarely, and account for a minor part of the tumour. To date, only three cases of mixed tumour/pleomorphic adenoma of the salivary gland have been described, showing an extensive adipocyte content of more than 90% of the tumour tissue. Owing to this peculiarity, some authors have defined it as 'lipomatous pleomorphic adenoma'. We are not aware of previously reported similar lesions in the skin. OBJECTIVES: We report a case of a tumour that occurred as a 2 x 2 x 1.5 cm nodule in the scalp of a 65-year-old man. Analogies with salivary lipomatous pleomorphic adenoma, as well as histogenesis and differential diagnoses are discussed here. METHODS: A histological, immunohistochemical and ultrastructural study was performed. RESULTS: The tumour was well-circumscribed and showed a substantial mature adipose tissue component intermingled with epithelial cells arranged in ducts and branching tubules, embedded in a fibromyxoid stroma, which was diagnostic of a chondroid syringoma/mixed tumour. Adipocytes strongly expressed S-100 protein and cytokeratin 14. Transitional elements from epithelial/myoepithelial cells into adipocytes were observed. They coexpressed cytokeratin 14, S-100 protein and vimentin, and showed lipid droplets, desmosome-type junctions, cytoplasmic tonofilaments and basal lamina. CONCLUSIONS: The tumour differed from lipomas with myxoid stroma and from lipoadenomas, which show non-proliferating normal sweat glands admixed with adipose tissue. Because of the similarity to lipomatous pleomorphic adenoma/mixed tumour of salivary glands, we suggest that it should be called 'lipomatous mixed tumour of the skin'.

Adenoma pleomorfo / Pleomorphic adenoma

El adenoma pleomorfo es una neoplasia benigna y es el tumor más frecuente de aquellos que derivan de los tejidos que forman las glándulas salivales. En años recientes se han publicado casos de transformación maligna de esta neoplasia. En este trabajo se exponen los pincipales resultados de diferentes investigaciones sobre este tema. Asimismo, se revisarán las características clínicas, histogénesis, hallazgos microscópicos, inmunología, transformación maligna, tratamiento y pronóstico

Hyaline cell-rich chondroid syringoma: case report and review of the literature.

Hyaline cell-rich chondroid syringoma (HCRCS) is a rare benign cutaneous neoplasm composed of cells with eosinophilic hyaline cytoplasm and plasmacytoid features, the origin of which remains elusive. To the best of our knowledge, only eight cases of this entity have been reported so far, and none of them was submitted to a large panel of myoepithelial markers. We report on a case of a previously healthy 29-year-old male patient who presented with a slowly enlarging flesh-colored nodule on the palmar aspect of the tenar region of his left hand, measuring 2 cm in maximum diameter. The nodule was "shelled-out" and submitted to light microscopy, immunohistochemistry, and ultrastructural examination. Histopathologic analysis disclosed a lobulated neoplasm composed of hyaline cells with plasmacytoid features showing ovoid nuclei, with occasional invaginations, finely granular chromatin, and discrete nucleoli; the cytoplasm was deeply eosinophilic with occasional dot-shaped paranuclear hyaline inclusions. On immunohistochemical evaluation, hyaline cells were strongly and diffusely positive for S-100 protein, vimentin, pan (CAM 5.2) and high molecular weight (34betaE12) cytokeratins; these cells were focally positive for GFAP, maspin, neuron-specific enolase, and cytokeratin 14. Alpha-smooth muscle actin, epithelial membrane antigen, carcinoembryonic antigen, collagen IV, Gp100 (HMB-45), and p63 were negative in neoplastic hyaline cells. Ultrastructural analysis disclosed cells with ovoid nuclei showing occasional invaginations and nuclear pockets; the cytoplasm was rich in meshworks of non-bundling intermediate filaments and a variable amount of rough endoplasmic reticulum cisternae. Based on our findings and those previously reported, hyaline cells of HCRCS might posses an aberrant myoepithelial differentiation. Most importantly, pathologists need to be aware of the histologic and immunohistochemical features of HCRCS to avoid a misdiagnosis of highly malignant neoplams, such as malignant melanoma and extra-skeletal myxoid chondrosarcoma.

DNA cytometry in pleomorphic adenomas with cytologic atypia.

OBJECTIVE: To investigate the nuclear DNA content of pleomorphic adenomas with cytologic atypia. STUDY DESIGN: Analysis was performed on new, fuchsin-stained samples of 10 selected cases of pleomorphic adenoma with cytologic atypia. Morphometric analysis was done by a computer-assisted image cytometry system and consisted of the determination of DNA indices, Auer DNA histogram types, and 3c and 5c exceeding rates. RESULTS: Eight cases were diploid and two cases aneuploid according to the DNA index. The Auer histogram was type I in five cases and type III in the others. In the two aneuploid cases the 3c exceeding rate was > 10% and the 5c exceeding rate > 1%. CONCLUSION: Atypical cells in pleomorphic adenomas with cytologic atypia carry abnormal amounts of DNA. Image cytometry can make detecting very low numbers of aneuploid cells easier due to its higher resolution as compared to that of flow cytometry.

Recurrent malignant chondroid syringoma of the foot: a case report and review of the literature.

Malignant chondroid syringoma, or mixed tumor of the skin, salivary gland type, is an uncommon neoplasm believed to originate in sweat glands. This neoplasm occurs mostly in women and is typically seen in the extremities and torso. A case of recurrent malignant chondroid syringoma of the right foot in a man aged 34 years is described with a review of pertinent literature. The surgically excised neoplasm was evaluated by routine histology, immunohistochemistry, and transmission electron microscopy. The malignant chondroid syringoma showed microscopic dermal satellite tumor nodules. Immunohistochemical staining was positive for keratin and S100 and negative for actin and p53. Ki-67 showed <10% positive staining. Ultrastructurally, the neoplasm was composed of epithelial cells with tonofilaments, cell junctions, and electron-dense amorphous keratin-like substance in the intercellular spaces. No evidence of myoepithelial differentiation was noted. Given the tumoral size, acral location, and histologic findings, the neoplasm was classified as a malignant chondroid syringoma. After reviewing the literature, it became apparent that wide surgical excision, adjuvant radiation therapy as well as patient education are critical in facilitating long-term survival.