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Liposarcoma of the base of tongue and tonsillar fossa: A possibly underdiagnosed neoplasm.

Liposarcomas of the head and neck are exceedingly rare, and fewer than 90 cases have been reported in the literature. Liposarcoma of the oral cavity is an even less common entity, and to our knowledge only nine cases have been reported to date. We report the clinical and pathologic findings of a case of well-differentiated liposarcoma of the base of tongue and tonsillar fossa. The patient is a 76-year-old white man with a long-standing history of a mass in the oral cavity and hypopharynx. The mass had been resected several times over the span of 23 years, and diagnoses of lipoma, neurofibroma, mesenchymoma, and angiofibrolipoma have been rendered on different occasions. At the last admission, a polypoid mass of the left tonsillar fossa and base of tongue was resected. The tumor was multinodular and measured 2.5 cm in greatest diameter. Histologically the tumor was ill-defined with infiltrating borders and was composed predominantly of mature adipose tissue with occasional lipoblasts. A small proportion of the tumor consisted of clusters of spindle cells and pleomorphic lipoblasts. Mitotic activity was not seen. The pleomorphic cells were positive for S100 protein and negative for muscle-specific markers. Ultrastructural analysis confirmed the nature of the lipoblasts. Our case depicts the typical natural history and histologic features of liposarcoma of the oral cavity. This tumor is usually well differentiated and has a high recurrence rate and almost no tendency for metastasis. Based on our case and review of the literature, it appears that well-differentiated liposarcoma of the oral cavity can occasionally be underdiagnosed because of the low mitotic activity and long latent period between the original diagnosis and first recurrence.

Aged↗

Pleomorphic large cell lymphomas of the mediastinum.

Nine cases of primary non-lymphoblastic, non-Hodgkin's large cell lymphomas of the mediastinum characterized by a highly pleomorphic histologic appearance are described. The patients, four women and five men, were aged 30 to 65 years. All patients presented with symptoms referable to their tumors, including cough, chest pain, dyspnea, pleural effusion, and superior vena cava syndrome. Clinical and pathologic staging in all patients showed that the bulk of the tumor was confined to the chest cavity at the time of initial diagnosis, with local infiltration into the neck, lung hilum, and surrounding mediastinal structures. Three different histological growth patterns were observed: one composed of a diffuse proliferation of pleomorphic, highly atypical cells with bizarre nuclear features that closely resembled a high grade sarcoma; another one composed of sheets of large, epithelial-appearing atypical cells suggestive of anaplastic carcinoma; and another pattern characterized by a pleomorphic proliferation of large lymphoid cells admixed with numerous scattered Reed-Sternberg-like cells reminiscent of the lymphocyte-depleted variant of Hodgkin's disease. Immunohistochemical studies on paraffin-embedded tissue sections in all cases showed positive staining of the tumor cells with CD20 and CD45 antibodies and negative staining with a large panel of markers, including broad-spectrum keratin, CAM 5.2, carcinoembryonic antigen, epithelial membrane antigen, vimentin, actin, desmin, HMB 45, S-100 protein, CD3, CD15, CD30, and CD45RO. Because of their location restricted to the anterior mediastinum, frequent lack of recognizable lymph node architecture, and bizarre cytologic features, the present group of lesions posed difficulties for diagnosis, their correct identification was achieved through the application of a panel of immunohistochemical markers. An awareness of these unusual histologic appearances of primary large cell lymphoma in the mediastinum and inclusion of a broad panel of lymphoid markers are therefore recommended for the evaluation of pleomorphic, undifferentiated malignant neoplasms of this anatomic region.

Adult↗

Polypoid spindle-cell carcinoma (pleomorphic carcinoma). Report of a case occurring on tongue and review of the literature.

A case of spindle-cell carcinoma (pleomorphic carcinoma), a rare polypoid tumor of the tongue, is reported. The characteristic clinical, gross, and microscopic features of this peculiar lesion and its common sites of location are presented. Origin and pathogenesis of the sarcoma-like elements of the lesion are discussed and the literature on the subject is reviewed. Sites of apparent transition between the spindle cell elements of the tumor and the overlying epithelium were observed in light microscopic sections, and the electron micrographs of the spindle-cell portion of the lesion demonstrated the presence of tonofilaments and desmosomes in many tumor cells. These findings support the concept that this group of lesions are pleomorphic variants of squamous-cell carcinoma with predominantly spindle-cell pattern. Current evidence in the literature also suggests that metaplastic transformation of the tumor cells into mesenchymal elements may take place in some of these lesions, and that "metaplastic carcinoma" may probably be a more proper designation for them.

Aged↗

Pleomorphic giant cell carcinoma of the pancreas: a review of nine cases.

Nine cases of pleomorphic giant cell carcinoma of the pancreas collected over a 26-year period are analyzed. The tumor presents a distinctive histology. The head of the pancreas appears to be the predominant site of the lesions in this study, and the cell origin is considered to be the ductal epithelium. Its clinical manifestation and rapid, fatal course of usually two months are almost indistinguishable from the more conventional pancreatic adenocarcinoma.

Adult↗

Pleomorphic giant cell carcinoma of the esophagus with coexpression of cytokeratin and vimentin and neuroendocrine differentiation.

A pleomorphic (giant cell) carcinoma of the esophagus is reported in a 52-year-old man who had dysphagia and weakness. The 8-cm-high vegetating tumor consisted of solid sheets of poorly cohesive epithelioid cells broken into clusters by strands of stroma. Numerous giant cells showing phagocytic phenomenon were present. Immunochemical analyses demonstrated the epithelial origin of the neoplasm, although most of the tumor cells strongly expressed vimentin. Numerous tumor cells expressed synaptophysin. Neurosecretory granules were detected in some tumor cells on electron microscopic examination. The patient died 4 months after he became symptomatic. As far as we can ascertain, this is the first case report describing a pleomorphic carcinoma arising in the esophagus. This poorly differentiated carcinoma might be of neuroendocrine differentiation. In the esophagus, pleomorphic carcinoma must be distinguished from polypoid tumors such as carcinosarcoma and malignant melanoma.

Biomarkers, Tumor↗

Low-grade malignant Triton tumor of the oral cavity: a case report.

Malignant Triton tumor (MTT) is a malignant peripheral nerve sheath tumor showing rhabdomyoblastic differentiation. It is considered a high-grade neoplasm with poor outcome. This report describes an MTT appearing in the oral cavity. On histologic examination the encapsulated lesion was composed of interlacing fascicles of spindle cells and scattered, large, strap-like pleomorphic cells with abundant eosinophilic cytoplasm. No cross striations were seen. Examination of levels through the tissue showed a total of only 4 normal mitoses and no necrosis. Immunohistochemistry demonstrated diffuse S100 positivity in the spindle cells. The large pleomorphic cells were weakly positive for alpha-sarcomeric actin and myoglobin, although variably but strongly positive for desmin. Management involved a small en bloc resection of the maxilla. After 33 months there was no sign of recurrence or distant metastasis. It was concluded that low-grade variants of MTT occur that do not have an aggressive clinical course.

Actins↗

[Sarcomalike patterns in laryngeal carcinoma (pseudosarcoma, carcinosarcoma, spindle-cell-carcinoma, pleomorphic carcioma (author's transl)].

Report on 5 cases of so called carcinosarcomas of the larynx and hypopharynx and review of literature. These polypoid tumors have a small epidermoid carcinomatous part and a major part with sarcoma like appearance. The sarcomatoid tissue resembles to fibrosarcomas or some undifferentiated pleomorphic sarcoma. Observations of a gradual transition from the epithelial tumor cells to cells with a mesodermal appearance show that these tumors are varieties of squamous cell carcinomas (spindle cell or pleomorphic variety) and not "combination"--or "collision-tumors", "carcinosarcomas" or "seudosarcomas". These tumors appear in the larynx predominantly in man, at the same sites and in the same ageclasses as common squamous cell cancers and are not less malignant. The sarcomatoid tissue can arise at the beginning of the disease or at a later stage it can appear or disappear after operation or irradiation. The reason for this change of cellular structure is unknown.

Aged↗

Keratin 14 immunoreactive cells in pleomorphic adenomas and adenoid cystic carcinomas of salivary glands.

Our recent study of developing myoepithelial cells (MECs) in rat salivary glands demonstrated that developing MECs begin to express alpha-smooth muscle actin (alphaSMA) first and, thereafter, keratin 14. Therefore, it is unlikely that duct basal cells expressing keratin 14 alone are immature or undifferentiated MECs. In this study we carried out immunohistochemistry of pleomorphic adenomas and adenoid cystic carcinomas including normal salivary glands using monoclonal antibodies to keratin 14, smooth muscle proteins and keratin 19. The smooth muscle proteins examined included alphaSMA, h-caldesmon and h1-calponin; h1-calponin was observed in keratinocytes and nerve fibers, indicating that the protein is not specific to smooth muscle, whereas alphaSMA and h-caldesmon turned out to be highly specific markers for smooth muscle cells in normal tissues. In normal glands, MECs were positive for both keratin 14 and smooth muscle proteins (alphaSMA and h-caldesmon). Non-MEC cells were essentially devoid of smooth muscle proteins. Non-MEC duct basal cells expressed keratin 14 with or without keratin 19, and luminal cells keratin 19 with or without keratin 14. This suggests that the keratin 14-positive, smooth muscle proteins-negative duct basal cells are luminal cell progenitors. Luminal cells in tubular structures of both tumors were positive for keratin 19 with or without keratin 14. Nonluminal peripheral cells of pleomorphic adenomas were mostly positive for keratin 14, and a small fraction of them expressed smooth muscle proteins. Conversely, peripheral cells of adenoid cystic carcinomas were mostly positive for smooth muscle proteins, and some of them expressed keratin 14. These results strongly suggest (1) that the luminal cell progenitors transform into major constituents of pleomorphic adenoma cells with keratin 14 but not smooth muscle proteins, and (2) that the peripheral cells of adenoid cystic carcinoma are derived from undifferentiated MECs. Solid structures of pleomorphic adenomas were formed by proliferation of the peripheral cells. MECs were observed only occasionally in the periphery. Solid and cribriform structures of adenoid cystic carcinomas were formed by proliferation of the luminal cells. MECs were observed in the periphery and around the pseudocyst.

Actins↗

Aspiration cytodiagnosis of breast carcinoma in pregnancy and lactation with immunohistochemical and electron microscopic study of an unusual mammary malignancy with pleomorphic giant cells.

We have reviewed our experience with 8,706 needle aspiration cytology (NAC) of the breast which were done from January 1983 to June 1991. During our study we diagnosed four cases of breast carcinomas (three ductal type and one with pleomorphic giant cells) in pregnant or lactating women; in all of these the cytologic findings corresponded with the subsequent tissue diagnosis and cell block preparations. Considering that carcinoma of breast during pregnancy and lactation is rare and is second only to cervical cancer, it was felt that its diagnosis by NAC was useful for timely management. In the case in which pleomorphic giant cells were present as an integral component of the tumour, immunohistochemical and electron microscopic study was done. This is discussed in view of our recent experience with this unusual tumour.

Adult↗

Establishment of human parotid pleomorphic adenoma cells in culture: morphological and biochemical characterization.

This study reports the establishment of alpha-amylase-producing human parotid pleomorphic adenoma cell lines (2HP and 2HP1) which have been maintained in culture for over 1 yr. The procedures required preparation of cellular clumps from tumor tissue and plating them on plasma clot or precoated dishes. During the initial phase of growth they required modified MCDB-153 medium without serum. When cells showed signs of degeneration they were changed to MCDB-153 medium containing first 2% and then 10% heat inactivated fetal bovine serum. Although cells grew well in MCDB-153 containing 10% serum, the epithelial cell morphology was not distinct. Therefore, the growth and morphology of cells grown in MCDB-10% serum were compared with those in RPMI growth medium containing 10% fetal bovine serum and F12 containing 10% agammaglobulin newborn bovine serum. Although the growth of cells was a little slower in F12 medium than those in MCDB and RPMI, the epithelial cell morphology was maintained better than in other growth media. The cells of 2HP and 2HP1 produce low levels of alpha-amylase and relatively high levels of alpha-amylase mRNAs of 1176 and 702 bp and contain neurofilament-160, a neuronal-specific marker. The cells of 2HP1 are tumorigenic when tested in athymic mice, but the cells of 2HP are not. The establishment of amylase-producing human parotid adenoma cell lines of different characteristics in culture provides a new opportunity to study the mechanisms of differentiation and transformation, and regulation of alpha-amylase in these cells.

Adenoma, Pleomorphic↗

Angiomyofibroblastoma of the male inguinal region.

We present a case of benign angiomyxoid tumor arising in the inguinal region of a 27-year-old man. The tumor was a gelatinous mass completely encapsulated by a thin fibrous capsule with no hemorrhage or necrosis. Histologically, a proliferation of spindle cells as well as occasional pleomorphic cells was observed within the myxofibrous stroma, intermingled with abundant capillary-sized blood vessels. Immunohistochemical staining of the tumor demonstrated spindle, oval, and pleomorphic cells equally positive for vimentin, desmin, and CD34, but not for alpha-smooth muscle actin. Based on these histologic and immunophenotypic features, we conclude that this angiomyxoid tumor of the male inguinal region is indistinguishable from the female angiomyofibroblastoma of the pelvic and perineal regions.

Actins↗

A femorotibial joint swelling with popliteal lymph node enlargement in a Rottweiler.

A 9-year-old male Rottweiler was presented to the Veterinary Medical Hospital Montenegro, Porto, Portugal with a large mass medial to the left stifle and radiographic signs of bone lysis involving the proximal tibia, fibula, and distal femur. A fine-needle aspiration was obtained from left popliteal lymph node, which was markedly enlarged. Cytologic examination revealed a highly cellular sample consisting of pleomorphic cells with marked anisocytosis and anisokaryosis and other criteria of malignancy. Some cells contained intracytoplasmic, granular, dark brown material, consistent with hemosiderin. Histologic evaluation of the surgically-excised lymph node revealed a neoplastic proliferation of histiocytic cells, with marked pleomorphism; occasional cells were erythrophagocytic. Mitotic figures were frequently observed, and many were atypical. Histologic findings were consistent with malignant histiocytosis. Necropsy examination confirmed the diagnosis of malignant histiocytosis with systemic involvement affecting most organs examined. This case of malignant histiocytosis case had an atypical clinical presentation, mimicking a musculoskeletal disorder. It underlines the importance of cytology as a simple, inexpensive, rapid and noninvasive complementary exam in routine clinical practice that can permit early diagnosis of the disease and timely selection of the most adequate therapy.

Animals↗

Clinical specular microscopy of posterior polymorphous endothelial dystrophy.

By means of clinical specular microscopy in 27 patients with posterior polymorphous corneal endothelial dystrophy diagnosed by slit lamp examination and 26 relatives with normal findings by slit-lamp and specular microscopic examinations, we identified two clinical and specular microscopic patterns of posterior polymorphous dystrophy. (1) The localized vesicular or band pattern consisted of clear corneas with localized lesions resembling craters that were lined by abnormal cells and surrounded by enlarged, pleomorphic cells. (2) The geographic pattern was associated with Descemet's membrane haze and deep stromal haze, occasional peripheral iridocorneal adhesions, and glaucoma. By specular microscopy, this type had large, well circumscribed areas of abnormal pleomorphic cells with indistinct borders and increased reflective highlights. These areas were surrounded by normal-sized and smaller polygonal cells. The former cells appear to represent epithelial cells and the latter endothelial cells.

Adult↗

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.

Adenoma, Pleomorphic↗

[Morpho-immunologic phenotypes of T-cell non-Hodgkin's malignant lymphoma in baboons and their connection with with T-lymphotropic retrovirus STLV-1].

8 cytological types were detected among 58 cases of T-NHL of Papio hamadryas according to human updated Kiel classification (1988). 5 of them were of low grade (lymphocytic, prolymphocytic, T-zone, angioimmunoblastic, small cell pleomorphic) and 3-of high grade (medium and large cell pleomorphic, immunoblastic, large cell anaplastic CD30/Ki-1+). There are differences in a tumor structure and generalization in baboon and human lymphomas in spite of significant similarities. STLV-1 presence (antibodies in blood and virus genome fragments in the lymphoma cell DNA) detected by PCR-amplification in 7 investigated types of baboon T-NHL. CD4+ immune phenotypes were predominant in T-cell baboon lymphomas, while CD8+ phenotypes were rare. Abnormal phenotypes in some cases were also detected in the three-color FACS-analyses.

Animals↗

Needle aspiration cytology, immunocytochemistry, and electron microscopic study of unusual pancreatic carcinoma with pleomorphic giant cells.

Needle aspiration cytology, immunocytochemistry, and electron-microscopic findings are presented in three cases of an unusual pancreatic carcinoma in which pleomorphic giant cells formed an integral part of the tumour. All three patients were elderly males (age range 66-83 years) and had pancreatic masses. Notable cytologic features in all cases were the presence of bizarre mononucleated and multinucleated, poorly cohesive tumour giant cells, rare spindle cells, with occasional cannibalism and cytophagocytosis. Immunocytochemical study of aspirated material showed diffuse staining of cytokeratin and EMA within the tumour cells, while B 72.3 was seen as focal trace stain. Electron microscopy of aspirated material demonstrated epithelial features and these were characterised by the presence of tonofilaments and surface microvilli. Based on our findings, it is felt that the bizarre giant cells in this unusual variant of pancreatic carcinoma are of epithelial origin. The differential diagnosis of other tumours that may be associated with predominant giant cells in pancreatic aspirates is appropriately discussed.

Aged↗

Fine-needle aspiration cytopathology of malignant fibrous histiocytoma.

BACKGROUND: Most pathologists generally accept malignant fibrous histiocytoma (MFH) as the most common soft tissue sarcoma in adults. This study examines the authors' aspiration cytopathology experience with this tumor, describes its cytomorphology, and determines the reliability of such a diagnosis by fine-needle aspiration biopsy (FNAB). METHODS: The authors' files were reviewed for cases diagnosed as MFH by FNAB, and for surgical pathology cases of MFH previously aspirated but not diagnosed as such by cytology. RESULTS: Fifty-two cases of MFH (by FNAB or histology) were recovered from the combined files; 42 aspirates had tissue confirmation. Patient age ranged from 15-88 years (mean, 63 years); the male:female ratio was 1.2. Thirty aspirates were from primary tumors, and 12 were from recurrences or metastases. From the 29 aspirates diagnosed as MFH, 24 (83%) were determined to be MFH on subsequent surgical excision. Four of the remaining cases were other sarcoma subtypes, and there was one organizing thrombus (false-positive). The remaining 13 aspirates were identified as unqualified sarcoma (11 cases) or a different sarcoma subtype (2 cases). Eleven of these were histologically diagnosed as MFH, and 2 as other sarcomas. No single cytologic feature or combination of features distinguished MFH. Patterns ranged from single cells to large storiform fragments. Spindled, plasmacytoid, and pleomorphic cell shapes were found; pleomorphic cells were often multinucleated. All cases of MFH had malignant nuclear morphology. Diagnostic pitfalls included low cellularity, obscuring blood and inflammation, and inadequate clinical and/or radiologic information. CONCLUSIONS: The diagnostic role of FNAB in soft tissue lesions remains controversial. FNAB is important in the initial triage of patients with soft tissue tumors, and is particularly accurate for confirming recurrent or metastatic disease. Although making an initial diagnosis of sarcoma by FNAB is reliable, specific subtyping of them as MFH is more problematic.

Adolescent↗